Neonatal Certification Review for the CCRN and RNC High-Risk Examination

NEONATAL

CERTifiCATiON REviEw for the CCRN and RNC HigH-Risk EXAMiNATiONs

Keri r. rogelet, rN, MSN, MBA/HCM, CCrN CFo, Director of Clinical Development Pro ed Menifee, California

ANN J. BrorSeN, rN, MSN, CCrN, CeN Coo, Director of Clinical Applications Pro ed Menifee, California

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Library of Congress Cataloging-in-Publication Data Rogelet, Keri R. Neonatal certification review for the CCRN and RNC high-risk examinations / Keri R. Rogelet, Ann J. Brorsen. p. ; cm. Includes bibliographical references. ISBN 978-0-7637-8005-0 (pbk.) 1. Neonatal intensive care—Examinations, questions, etc. 2. Intensive care nursing—Examinations, questions, etc. 3. Newborn infants—Diseases—Nursing—Examinations, questions, etc. I. Brorsen, Ann J. II. Title. [DNLM: 1. Neonatal Nursing—methods—Examination Questions. 2. Critical Illness—nursing—Examination Questions. 3. Intensive Care, Neonatal—methods—Examination Questions. 4. Nursing Assessment—Examination Questions. 5. Pregnancy Complications—nursing—Examination Questions. 6. Pregnancy, High-Risk— Examination Questions. WY 18.2 R721n 2011] RJ253.5.R64 2011 618.92’01076—dc22 2010008078 6048 Printed in the United States of America 14 13 12 11 10 10 9 8 7 6 5 4 3 2 1

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Contents

About the Authors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . iv Contributor. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v Reviewers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v Acknowledgments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vi Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii The RNC Credential. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix The CCRN Credential. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi Registering for the Neonatal CCRN Examination. . . . . . . . . . . . . . . xiii Section 1:

The Synergy Model. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1

Section 2:

Test-Taking Strategies. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11

Section 3:

Maternal Risk Factors and Delivery Issues . . . . . . . . . . . . . . . . . . . . 15

Section 4:

Cardiovascular System. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69

Section 5:

Pulmonary System. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117

Section 6:

Gastrointestinal System. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161

Section 7:

Genitourinary System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183

Section 8:

Hematology/Immunology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213

Section 9:

Neurology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241

Section 10: Endocrinology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277 Section 11: Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303 Section 12: RNC Practice Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 321 Section 13: Neonatal CCRN Practice Examination. . . . . . . . . . . . . . . . . . . . . . 381

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About the Authors

Keri R. Rogelet, RN, MSN, MBA/HCM, CCRN Keri has presented national programs for adult health issues, the neonatal CCRN, pediatric CCRN, and developmental care. Keri is a regional NRP trainer for the American Academy of Pediatrics and a lead instructor for the S.T.A.B.L.E. program. Keri’s professional associations include Sigma Theta Tau, the American Association of Critical-Care Nurses, and the Academy of Neonatal Nurses. In addition, she works as a consultant for pediatric and neonatal product applications. She is currently the Chief Financial Officer and Director of Clinical Development for Pro Ed in Menifee, California.

Ann J. Brorsen, RN, MSN, CCRN, CEN Ann is a nationally known speaker and has presented certification review courses for the adult and pediatric CCRN, PCCN, and CEN. Ann has presented programs as diverse as advanced hemodynamics to best practice models for hospital corporations. Ann is a member of Sigma Theta Tau, the American Association of Critical-Care Nurses, the Society of Critical Care Medicine, and the Emergency Nurses Association. Ann also works as a consultant for educational program development and management training for healthcare facilities. She is currently the Chief Operating Officer and Director of Clinical Applications for Pro Ed in Menifee, California.

Contact Information Web site: www.forproed.com Email: [email protected]

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Contributor

Melissa R. Christiansen, RN, MSN, NP-C, CCRN, CNRN Melissa has over 24 years of experience as a critical care nurse in neurological, cardiac, and trauma ICUs. She is currently working as a family nurse practitioner in Southern California. Melissa has presented programs on neurological and neuroscience topics, adult critical care certification reviews, and courses in postanesthesia nursing. Melissa is a member of Sigma Theta Tau, the American Association of Critical-Care Nurses, the American Association of Neuroscience Nurses, and the American Academy of Nurse Practitioners. Melissa is also on the faculty for the BSN, MSN, and Nurse Practitioner programs at the University of Phoenix.

Reviewers Mary A. Cowett, RN, RNC-BC Riverside, California Vicki L. Hirschel, RN CPQ Data Coordinator CPeTS Coordinator Moreno Valley, California

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Acknowledgments

Mary Margaret Forsythe, RN, and Nancy O. Roberts, RN Two instructors who were ultimate professionals and passed before their time.

Karen S. Ehrat, RN, PhD For seeing potential in a new grad and making education a joy and a privilege. You will be missed.

Damien, Minerva, and M’Leah Angels who taught me every life touches another and reminds me why I do what I do.

A. J. First my teacher, then my mentor, most important—my friend. My life is forever changed. Thank you.

D • d We are grateful to all the nurses who provided suggestions for content of this book. We are indebted to Melissa Christiansen for her dedication and long hours toward making this work a success. Thank you to the reviewers who gave their time, effort, and suggestions to enhance the content of this manuscript. We would also like to express our gratitude to the editorial staff at Jones and Bartlett Publishers. K. R. R. and A. J. B.

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Preface

Congratulations! You are one step closer to achieving certification as a neonatal nurse. If you plan to use this book as a study guide for neonatal care nursing, this will be an invaluable resource. This book will present an introduction to the neonatal CCRN and the RNC credential. We will guide you through the process of registering for the neonatal CCRN and RNC exams and offer some test-taking strategies. We will even provide you with the resources you will need to complete the process. This book contains test questions with rationales that will cover a broad range of topics and will be representative of the type of questions you will find on the actual examinations. In addition, two written practice tests with rationales appear at the end of the book. These practice tests will also be available on the book’s online TestPrep (see below for access information). Most nurses take the RNC examination. For this reason, we prepared the questions in this book to have three responses, to closely simulate the actual test. However, the practice CCRN examination will have four possible responses and be structured to match an actual CCRN examination. We are dedicated to helping you successfully pass this exam and achieve certification as a neonatal critical care nurse. Please feel free to contact us if you have any questions or you would like to schedule a CCRN or RNC review course for your facility or group. Keri and Ann

New Online Interactive Testing Program Enclosed within this book you will find a printed “access code card” containing an access code providing you access to the new online interactive testing program, JB TestPrep. This program will help you prepare for the CCRN and RNC exams. The online program includes the same multiple-choice questions that are printed in this book. You may sort the questions by chapter or randomize them. You can choose a “practice exam” that allows you to see feedback on your response immediately, or a “final exam,” which hides your results until you have completed all the questions in the exam. Your overall score on the questions you have answered is also compiled. Here are the instructions on how to access JB TestPrep, the Online Interactive Testing Program: 1. Find the printed access code card bound in to this book. 2. Go to www.JBLearning.com/usecode. 3. Enter in your 10-digit access code, which you can find by scratching off the protective coating on the access code card.

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viii  y  Preface

4. Follow the instructions on each screen to set up your account profile and password. Please note: Only select a course coordinator if you have been instructed to do so by an institution or an instructor. 5. Contact Jones and Bartlett Publishers technical support if you have any questions: • Call 800-832-0034 • Visit www.jbpub.com and select “Tech Support” • Email [email protected]

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The RNC Credential

This book was primarily written for nurses seeking the RNC credential in Neonatal Intensive Care Nursing (RNC-NIC) or the neonatal CCRN credential. This book would also be a good study guide for nurses seeking the Low Risk Neonatal Nursing (RNC-LRN) credential. The RNC credential is administered by the National Certification Corporation (NCC). Since its inception in 1975, NCC has awarded certification or certificates of added qualification to more than 83,000 licensed healthcare personnel.

Eligibility Requirements • •

Current licensure as an RN in the United States or Canada and 24 months of specialty experience as a U.S. or Canadian RN comprised of a minimum of 2000 hours. Employment in the specialty some time in the last 24 months

Registering for the RNC Examination In order to take the RNC examination, the first thing you should do is make certain you meet the testing criteria. Next, download an application and candidate guide for the exam from the NCC Web site at www.nccwebsite.org. Allow 4 weeks for the application to be processed. When the application has been processed, you will receive a postcard stating you must register for and complete the exam within 90 days. Tests are given at Applied Measurement Professional (AMP) testing sites around the country. You will schedule your own exam appointment with AMP, and exams will be administered by appointment only Monday through Friday at 9:00 a.m. and 1:30 p.m. Make certain to follow all the guidelines for the test and arrive early. Candidates arriving after the scheduled start of the exam will not be admitted. After the test, you will be given a preliminary score. The certification does not become official until NCC has reviewed the score and sends you appropriate documentation. Our goal is to assist you in obtaining certification. Good luck!

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The CCRN Credential

Traditionally, nurses have worked in a variety of roles and environments. For most of the twentieth century, when nurses graduated from their programs, they had spent many hours in clinical situations and were prepared to practice in any area. Nursing eventually had to adjust from the general practitioner to a nurse who would concentrate practice in one area. Nurses worked in emergency rooms, operating rooms, recovery rooms, obstetrics, and medical surgical units. Those nurses who worked in operating rooms or who administered anesthesia were considered specialized. With the advent of emerging technology, the post–World War II population boom, and a trend toward increasingly more acute patients, nursing and hospitals adjusted by placing patients in more subspecialized areas. One critical issue that arose in the twentieth century related to patients with poliomyelitis, who were increasing both in number and special needs. The iron lung had been around for years. In the 1930s, the machine cost $1,500, which was also the median cost for a home at that time. Patients who could afford such treatment began to recover, only to develop sequelae that required specialized care. Tilt beds and hot pack treatments were initiated. At one time, even curare was used to combat the severe muscle spasms suffered by polio victims. All of these treatments required time and resources, including larger numbers of nurses. In 1931, the American Association of Nurse Anesthetist (no s on the end) formed. On June 4, 1945, the organization held the first-ever certification examination for a nursing specialty. In 1952, the first accredited program for nurse anesthetists was started. In 1955, Jonas Salk announced the discovery of a vaccine for polio. The vaccine would help prevent spread of the disease, but thousands of victims still required care. Technology in general was improving and becoming more broadly available. Although the first EKG machines were available in the United States as early as 1909, they were not widely used until the late 1950s. In the 1960s, many patients required around-the-clock, specialized care that required resources and practitioners who were experts or who had a great deal of experience with the particular condition or disease process. Veterans of previous wars and the escalating Vietnam War required increasingly more medical resources. Hospitals began placing cardiac, trauma, burn, and acute medical patients in areas of the hospital designated as providing more “intensive” care. Patient survival rates improved, so the numbers of specialized areas increased. In 1967, nurses from Nashville Baptist Hospital were frustrated at the lack of educational opportunities for continuing education for intensive care nurses, so they sent inquiries to other nurses to see if interest existed to form an association or some type of national association to provide education to other intensive care nurses. At that time, most of the ICUs served cardiac patients. A year later, more than 400 nurses attended a symposium and affirmed the need for an organization. In 1969, the American Association of Cardiovascular Nurses was formed. In 1971, the name was changed to the

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xii  y  The CCRN Credential

American Association of Critical-Care Nurses (AACN) in recognition of the broad area covered by critical care. AACN is now the largest specialty nursing organization in the world. In 1975, the AACN Certification Corporation was established and began offering the CCRN examination. We invite you to visit the AACN Web site at www.aacn.org to learn more about the CCRN credential. Thousands of nurses have successfully attained the CCRN credential and we are here to help you become successful. The next section will explain the registration procedure for the examination. You can do it!

References American Association of Critical-Care Nurses. http://www.aacn.org American Association of Critical-Care Nurses. History of AACN. http://www.aacn.org/AACN/ mrkt.nsf/vwdoc/HistoryofAACN?opendocument National Museum of American History. How polio changed us. http://americanhistory.si.edu/ polio/howpolio/index.htm Anesthesia Nursing & Medicine Web site. What is a CRNA? http://www.anesthesia-nursing.com/ wina.html National Certification Corporation. http://www.nccwebsite.org

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Registering for the Neonatal CCRN Examination

Before registering to take the CCRN examination, you must first determine if you are eligible for the exam. To be able to take the neonatal CCRN exam, you must not have any encumbrances on your current registered nursing license in any state—in other words, no restrictions, disciplinary actions, attached conditions, or provisions of any kind that would affect your ability to practice as a nurse. You need to have completed 1,750 hours of direct bedside care for a critically ill neonatal patient population during the past 2 years. For you to qualify for the neonatal CCRN exam, all of your practice hours must involve care of neonatal patients. You may not split the hours between, say, a neonatal ICU and an adult ICU. Of the 1,750 hours of direct care, 875 hours must have been completed in the past year. If you are an educator, a CNS, or a manager, you can still qualify. If you directly supervise students or nurses at the bedside, you will qualify. You must, however, participate in the care of the patient. For example, if you demonstrate a procedure or supervise a nurse or student performing the procedure, that activity would be acceptable. Nurses must have a certain level of experience to qualify for the CCRN exam. Many questions require integration of knowledge and critical thinking, and the test covers advanced concepts such as hemodynamics and ventilator management. It would be difficult, though not impossible, to pass the test if you have not had experience with either of these clinical situations. Other questions test your familiarity with technology, such as umbilical lines and ECMO. Passing the test would certainly be easier if you have experience with this technology, but not every critical care area utilizes these therapies. For example, some nurses do not have direct experience with patients undergoing open heart surgery or neurosurgery. In such cases, the relevant concepts can be learned from reading appropriate critical care texts or by consulting another nurse with experience in the particular area. Nursing is a mobile profession, and quite often a person you work with may have experiences and qualifications unknown to you. Some nurses have the opportunity to work via registries or as travelers and have a wide base of experience. Other nurses will seek out new experiences within their own facility or work part-time in other facilities. We are not trying to scare you. Thousands of nurses have passed this exam, and it is certainly feasible to do so even without experience in certain areas. Candidates for the CCRN also come from various levels of neonatal ICUs. To see what the CCRN exam covers, you can download a copy of the neonatal CCRN Test Plan from AACN at www.aacn.org. The Test Plan is an outline, or blueprint, of the major areas to be tested. The Test Plan also indicates the percentage of questions tested in that particular section or system. Specific conditions and pathophysiologies are listed on the Test Plan, allowing you to better focus your study time and resources. If you would like us to send you an application and current neonatal CCRN Test Plan, please email us your name and address (please do not forget your ZIP code!).

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xiv  y  Registering for the Neonatal CCRN Examination

When you sign up for the CCRN exam, you can sign up as either a member or a nonmember of AACN. The cost is lower if you are a member. You can join AACN at the time you register to receive the member discount. Just email us if you have any questions. After your documents are received by AACN, you must allow several weeks for processing. You will then be sent a postcard stating that, from the date on the postcard, you will have 90 days to complete the exam. Once you register for the exam, you will be provided with a list of examination locations near you, based on your ZIP code. The test is administered at Applied Measurement Professional (AMP) sites around the United States, and you may take the exam at any AMP testing center. Your next step is to call the AMP testing center you select and make an appointment for the exam. The testing center will authorize the time and day when you will take the test. Visit the AACN Web site or the AMP Web site for updated information. Please note: You will be sent a specific authorization code to access the AMP Web site to register for the exam. Specific rules apply when you want to change an appointment, so make sure you have the most current information on this process. The CCRN exam is now computer based and given year round. You may take the CCRN exam the old-fashioned way, by pencil and paper. These written exams are given only a couple times a year and require special registrations and arrangements. Please contact AACN directly if you wish to test in this manner. Remember, thousands of nurses have passed this examination. You can, too!

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Section 1 The Synergy M ­ odel

The Synergy Model was originally developed to match clinical competency with patient needs and outcomes. The nurse and the care provided are driven by the needs of the patients and their families. Nurses begin practice as novices and as they gain experience, may reach the expert level. The expert nurse is able to synthesize information, integrate that information, and then formulate a plan of care based on the patient’s needs. The patient may have simple needs or very complex needs. The patient also has characteristics that reflect their physical, emotional, cultural, family, financial, and social resources. The nurse must take all of the patient’s characteristics into ­consideration. The patient must be the focus of the care and the patient’s needs must come first. When the needs and characteristics of the patient are matched with the competencies of the nurse, synergy is the result. For example, it would not be in the best interest of a critically ill patient who requires multiple medications and hemodynamic monitoring to assign them to a new graduate who lacks the experience and knowledge to care for such a complex ­patient. Synergy is applicable to education, the corporate world, small business, and anywhere the characteristics of the individual must be matched by the competencies of another entity. In nursing, we are looking for optimal outcomes for the patient and the ­nurse. The CCRN examination used to be entirely based on clinical judgment. If you could memorize a lot of facts, you would probably be successful on the exam. Now, 80% of the exam is clinical judgment, but the other 20% is covered by “Professional Caring and Ethical Practice.” There is no question that the Synergy Model can be confusing and intimidating. There are excellent resources to help you understand the model and the concepts. At the end of this section is a list of those resources and we hope you will take the time to investigate each of them. AACN has posted on their Web site the information about nurse and patient characteristics that appear in these tables. There are two excellent books about the Synergy Model. First, we invite you to read, “Critical Care Nursing: Synergy for Optimal Outcomes” by Roberta Kaplow, RN, PhD, CCRN, CCNS, CCNS and Sonya R. Hardin, RN, PhD, CCRN. The second book is “Synergy for Clinical Excellence: The AACN Synergy Model for Patient Care” by Sonya R. Hardin, RN, PhD, CCRN and Roberta Kaplow, RN, PhD, CCRN, CCNS, ­CCNS. Recently, AACN expanded the information about the Synergy Model patient and nurse characteristics and we thank them for the information that appears in Table 1-1, Patient Characteristics, and Table 1-2, Nurse ­Competencies. Remember, the neonatal CCRN exam does not test on the terminology, but rather on your ability to integrate these concepts into the care of ­patients.

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Table 1-1  Patient Characteristics Resiliency

The capacity to return to a restorative level of functioning using compensatory/coping mechanisms; the ability to bounce back quickly after an insult.

Level 1—Minimally resilient

Unable to mount a response; failure of compensatory/ coping mechanisms; minimal reserves; brittle

Level 3—Moderately resilient

Able to mount a moderate response; able to initiate some degree of compensation; moderate reserves

Level 5—Highly resilient

Able to mount and maintain a response; intact compensatory/coping mechanisms; strong reserves; endurance

Vulnerability

Susceptibility to actual or potential stressors that may adversely affect patient outcomes.

Level 1—Highly vulnerable

Susceptible; unprotected, fragile

Level 3—Moderately vulnerable

Somewhat susceptible; somewhat protected

Level 5—Minimally vulnerable

Safe; out of the woods; protected, not fragile

Stability

The ability to maintain steady-state equilibrium.

Level 1—Minimally stable

Labile; unstable; unresponsive to therapies; high risk of death

Level 3—Moderately stable

Able to maintain steady state for limited period of time; some responsiveness to therapies

Level 5—Highly stable

Constant; responsive to therapies; low risk of death

Complexity

The intricate entanglement of two or more systems (e.g., body, family, therapies).

Level 1—Highly complex

Intricate; complex patient/family dynamics; ambiguous/ vague; atypical presentation

Level 3—Moderately complex

Moderately involved patient/family dynamics

Level 5—Minimally complex

Straightforward; routine patient/family dynamics; simple/ clear cut; typical presentation

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The Synergy ­Model  y  3

Table 1-1  Patient Characteristics (continued) Resource availability

Extent of resources (e.g., technical, fiscal, personal, psychological, and social) the patient/family/ community bring to the situation.

Level 1—Few resources

Necessary knowledge and skills not available; necessary financial support not available; minimal personal/ psychological supportive resources; few social systems resources

Level 3—Moderate resources

Limited knowledge and skills available; limited financial support available; limited personal/psychological supportive resources; limited social systems resources

Level 5—Many resources

Extensive knowledge and skills available and accessible; financial resources readily available; strong personal/ psychological supportive resources; strong social systems resources

Participation in care

Extent to which patient/family engages in aspects of care.

Level 1—No participation

Patient and family unable or unwilling to participate in care

Level 3—Moderate level of

Patient and family need assistance in care

Level 5—Full participation

Patient and family fully able to participate in care

Participation in decision-making

Extent to which patient/family engages in decision-making.

Level 1—No participation

Patient and family have no capacity for decision-making; requires surrogacy

Level 3—Moderate level of participation

Patient and family have limited capacity; seeks input/advice from others in decision-making

Level 5—Full participation

Patient and family have capacity, and makes decision for self

Predictability

A characteristic that allows one to expect a certain course of events or course of illness.

Level 1—Not predictable

Uncertain; uncommon patient population/illness; unusual or unexpected course; does not follow critical pathway, or no critical pathway developed

Level 3—Moderately predictable

Wavering; occasionally-noted patient population/illness

Level 5—Highly predictable

Certain; common patient population/illness; usual and expected course; follows critical pathway

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Table 1-2  Nursing Competencies Clinical Judgment

Clinical reasoning, which includes clinical decisionmaking, critical thinking, and a global grasp of the situation, coupled with nursing skills acquired through a process of integrating formal and informal experiential knowledge and evidence-based guidelines.

Level 1

Collects basic-level data; follows algorithms, decision trees, and protocols with all populations and is uncomfortable deviating from them; matches formal knowledge with clinical events to make decisions; questions the limits of one’s ability to make clinical decisions and delegates the decisionmaking to other clinicians; includes extraneous detail

Level 3

Collects and interprets complex patient data; makes clinical judgments based on an immediate grasp of the whole picture for common or routine patient populations; recognizes patterns and trends that may predict the direction of illness; recognizes limits and seeks appropriate help; focuses on key elements of case, while shorting out extraneous details

Level 5

Synthesizes and interprets multiple, sometimes conflicting, sources of data; makes judgment based on an immediate grasp of the whole picture, unless working with new patient populations; uses past experiences to anticipate problems; helps patient and family see the “big picture;” recognizes the limits of clinical judgment and seeks multidisciplinary collaboration and consultation with comfort; recognizes and responds to the dynamic situation

Advocacy and Moral Agency

Working on another’s behalf and representing the concerns of the patient/family and nursing staff; serving as a moral agent in identifying and helping to resolve ethical and clinical concerns within and outside the clinical setting.

Level 1

Works on behalf of patient; self assesses personal values; aware of ethical conflicts/issues that may surface in clinical setting; makes ethical/moral decisions based on rules; represents patient when patient cannot represent self; aware of patients’ rights

Level 3

Works on behalf of patient and family; considers patient values and incorporates in care, even when differing from personal values; supports colleagues in ethical and clinical issues; moral decision-making can deviate from rules; demonstrates give and take with patient’s family, allowing them to speak/represent themselves when possible; aware of patient and family rights

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Table 1-2  Nursing Competencies (continued) Level 5

Works on behalf of patient, family, and community; advocates from patient/family perspective, whether similar to or different from personal values; advocates ethical conflict and issues from patient/family perspective; suspends rules—patient and family drive moral decisionmaking; empowers the patient and family to speak for/ represent themselves; achieves mutuality within patient/ professional relationships

Caring Practices

Nursing activities that create a compassionate, supportive, and therapeutic environment for patients and staff, with the aim of promoting comfort and healing and preventing unnecessary suffering. Includes, but is not limited to, vigilance, engagement, and responsiveness of caregivers, including family and healthcare personnel.

Level 1

Focuses on the usual and customary needs of the patient; no anticipation of future needs; bases care on standards and protocols; maintains a safe physical environment; acknowledges death as a potential outcome

Level 3

Responds to subtle patient and family changes; engages with the patient as a unique patient in a compassionate manner; recognizes and tailors caring practices to the individuality of patient and family; domesticates the patient’s and family’s environment; recognizes that death may be an acceptable outcome

Level 5

Has astute awareness and anticipates patient and family changes and needs; fully engaged with and sensing how to stand alongside the patient, family, and community; caring practices follow the patient and family lead; anticipates hazards and avoids them, and promotes safety throughout patient’s and family’s transitions along the healthcare continuum; orchestrates the process that ensures patient’s/family’s comfort and concerns surrounding issues of death and dying are met

Collaboration

Working with others (e.g., patients, families, healthcare providers) in a way that promotes/encourages each person’s contributions toward achieving optimal/ realistic patient/family goals. Involves intra- and interdisciplinary work with colleagues and community.

Level 1

Willing to be taught, coached and/or mentored; participates in team meetings and discussions regarding patient care and/or practice issues; open to various team members’ contributions

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Table 1-2  Nursing Competencies (continued) Level 3

Seeks opportunities to be taught, coached, and/or mentored; elicits others’ advice and perspectives; initiates and participates in team meetings and discussions regarding patient care and/or practice issues; recognizes and suggests various team members’ participation

Level 5

Seeks opportunities to teach, coach, and mentor and to be taught, coached, and mentored; facilitates active involvement and complementary contributions of others in team meetings and discussions regarding patient care and/or practice issues; involves/recruits diverse resources when appropriate to optimize patient outcomes

Systems Thinking

Body of knowledge and tools that allow the nurse to manage whatever environmental and system resources exist for the patient/family and staff, within or across healthcare and non-healthcare systems.

Level 1

Uses a limited array of strategies; limited outlook—sees the pieces or components; does not recognize negotiation as an alternative; sees patient and family within the isolated environment of the unit; sees self as key resource

Level 3

Develops strategies based on needs and strengths of patient/family; able to make connections within components; sees opportunity to negotiate but may not have strategies; developing a view of the patient/family transition process; recognizes how to obtain resources beyond self

Level 5

Develops, integrates, and applies a variety of strategies that are driven by the needs and strengths of the patient/ family; global or holistic outlook—sees the whole rather than the pieces; knows when and how to negotiate and navigate through the system on behalf of patients and families; anticipates needs of patients and families as they move through the healthcare system; utilizes untapped and alternative resources as necessary

Response to Diversity

The sensitivity to recognize, appreciate and incorporate differences into the provision of care. Differences may include, but are not limited to, cultural differences, spiritual beliefs, gender, race, ethnicity, lifestyle, socioeconomic status, age, and values.

Level 1

Assesses cultural diversity; provides care based on own belief system; learns the culture of the healthcare environment

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Table 1-2  Nursing Competencies (continued) Level 3

Inquires about cultural differences and considers their impact on care; accommodates personal and professional differences in the plan of care; helps patient/family understand the culture of the healthcare system

Level 5

Responds to, anticipates, and integrates cultural differences into patient/family care; appreciates and incorporates differences, including alternative therapies, into care; tailors healthcare culture, to the extent possible, to meet the diverse needs and strengths of the patient/family

Facilitation of Learning

The ability to facilitate learning for patients/families, nursing staff, other members of the healthcare team, and community. Includes both formal and informal facilitation of learning.

Level 1

Follows planned educational programs; sees patient/ family education as a separate task from delivery of care; provides data without seeking to assess patient’s readiness or understanding; has limited knowledge of the totality of the educational needs; focuses on a nurse’s perspective; sees the patient as a passive recipient

Level 3

Adapts planned educational programs; begins to recognize and integrate different ways of teaching into delivery of care; incorporates patient’s understanding into practice; sees the overlapping of educational plans from different healthcare providers’ perspectives; begins to see the patient as having input into goals; begins to see individualism

Level 5

Creatively modifies or develops patient/family education programs; integrates patient/family education throughout delivery of care; evaluates patient’s understanding by observing behavior changes related to learning; is able to collaborate and incorporate all healthcare providers’ and educational plans into the patient/family educational program; sets patient-driven goals for education; sees patient/family as having choices and consequences that are negotiated in relation to education

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8  y  Section 1

Table 1-2  Nursing Competencies (continued) Clinical Inquiry (Innovator/Evaluator)

The ongoing process of questioning and evaluating practice and providing informed practice. Creating practice changes through research utilization and experiential learning.

Level 1

Follows standards and guidelines; implements clinical changes and research-based practices developed by others; recognizes the need for further learning to improve patient care; recognizes obvious changing patient situation (e.g., deterioration, crisis); needs and seeks help to identify patient problem

Level 3

Questions appropriateness of policies and guidelines; questions current practice; seeks advice, resources, or information to improve patient care; begins to compare and contrast possible alternatives

Level 5

Improves, deviates from, or individualizes standards and guidelines for particular patient situations or populations; questions and/or evaluates current practice based on patients’ responses, review of the literature, research and education/learning; acquires knowledge and skills needed to address questions arising in practice and improve patient care (the domains of clinical judgment and clinical inquiry converge at the expert level; they cannot be separated)

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The Synergy ­Model  y  9

References Becker, D., Kaplow, R., Muenzen, P. M. & Hartigan, C. (2006). Activities performed by acute and critical care advanced practice nurses: American Association of ­Critical-­Care Nurses study of practice. American Journal of Critical Care, 15(2), 130–148. Brewer, B. B., ­Wojner-­A lexandrov, A. W., Triola, N., Pacini, C. et al. (2007). AACN synergy model’s characteristics of patients: Psychometric analyses in a tertiary care health system. American Journal of Critical Care, 16(2), 158–167. Burns, S. M. (ed.). (2007). American Association of ­Critical-­Care Nurses (AACN): AACN Protocols for Practice: Healing Environments. (2nd Ed.). Sudbury, MA: Jones and ­Bartlett. Cline, M., Nottingham, M., & Lockhart, J. S. (2006). Synergy for clinical excellence: The AACN synergy model for patient care. Critical Care Nurse, 26(2), 139–140. Copstead, L., & Banasik, J. L. (2000). Pathophysiology: Biological and Behavioral Perspectives. (2nd Ed.). Philadelphia, PA: W. B. ­Saunders/Elsevier. Curley, M. A. Q. (1998). Patient Nurse Synergy: Optimizing Patients’ Outcomes. American Journal of Critical Care, 7, 64–72. Dossey, B. M., Keegan, L. & Guzzetta, C. (2003). Holistic Nursing: A Handbook for Practice. (3rd Ed.). Sudbury, MA: Jones and ­Bartlett. Edwards, D. F. (1999). The Synergy Model: Linking Patient Needs to Nurse Competencies. Critical Care Nurse, 19(1), 88–98. Hardin, S. R., & Kaplow, R. (2005). Synergy for Clinical Excellence: The AACN Synergy Model for Patient Care. Sudbury, MA: Jones and ­Bartlett. Hardin, S.R., & Kaplow, R. (eds.). (2004). Synergy for Clinical Excellence: The AACN Synergy Model for Patient Care. Sudbury, MA: Jones and ­Bartlett. Kaplow, R. (2004). Applying the synergy model to nursing education. Critical Care Nurse: AACN Critical Care Careers, 20, 22, 24–26. Kelleher, S. (2006). Providing ­patient-­centered care in an intensive care unit. Nursing Standard, 21(13), 35–40. Lipson, J. G., Dibble, S. L., & Minarik, P. A. (eds.). (1996). Culture and Nursing Care: A Pocket Guide. San Francisco, CA: UCSF Nursing ­Press. McQuillan, K. A., Von Rueden, K. T., Hartsock, R. L., Flynn, M. B., & Whalen, E. (eds.). (2002). Trauma Nursing: From Resuscitation Through Rehabilitation (3rd Ed.). Philadelphia, PA: W. B. ­Saunders/Elsevier. Smith, A. R. (2006). Using the synergy model to provide spiritual nursing care in critical care settings. Critical Care Nurse, 26(4), 41–47. Web site: AACN Certification Information. Retrieved on March 15, 2008 at http://web.aacn.org/ WD/Certifications/Content

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Section 2 Test-Taking Strategies

When preparing for the neonatal examinations, the first thing to do is be absolutely honest with yourself about how you study. If you have good study habits and plenty of time, you are very fortunate. If you are a procrastinator, studying a little bit at a time might help. Nurses have to juggle so many roles that take up their time: parent, child, employee, student, teacher, and on and on. One of the biggest struggles is simply finding time and a place to study. Discovering your learning style will help you find a better way to absorb information. Three types of learners are commonly identified: visual, auditory, and kinesthetic. There is no perfect strategy for learning because every person is unique. Not everyone has a single style of learning; you may use a mix of styles depending on your situation. Visual learners learn better from reading and writing than from hearing and talking about information. Background noise, such as music or television, is distracting to these types of learners. Finding a quiet space is a problem for some people. You may have to stay awake after family members have gone to bed. Flashcards often work, and some visual learners use colored markers to highlight important information. Auditory learners learn information effectively by listening and talking. Playing music, listening to audiotapes, or being part of a study group often works. Kinesthetic learners prefer to learn via a hands-on approach. Nurses often learn this way because we have to listen to lectures and then demonstrate skills. This approach focuses on the use of models, manikins, or patients and works well for many healthcare providers. Kinesthetic people are often antsy and cannot sit still for long periods of time, so lectures may be difficult for them without frequent breaks. If you are a kinesthetic learner, some of the things that might help while studying include taking frequent breaks, walking around, or riding a stationary cycle. No matter what your personal learning style, you can improve your test-taking skills. How? Practice! That is why we wrote this book in a question-and-answer format. Keep practicing the questions until you can answer at least 80% correctly. Research has shown that two-thirds of study time should be spent taking sample tests, and only onethird of study time should be spent reviewing content. As nurses, we must always keep abreast of the professional literature and spend time studying to keep our knowledge and skills up to date. In addition, many states require continuing education to renew a professional license. The CCRN requires 100 hours of continuing education to be obtained within a 3-year period. Anything worthwhile, such as studying, takes time, effort, and sacrifice. There is no way around studying for this certification. There are no shortcuts! If you have been out of school for a while, don’t despair! It may be slow going at first, so take things a little at a time. Just like going to the gym, you should make a plan to study in one particular place and at the same time every day if possible. This is your space and your time—claim it. Have all your books, tapes, and other study materials handy. If

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12  y  Section 2

you need snack food, make sure it is not all sugar and include some salty food. Caffeine tends to make people jittery, but if you need it, it may be right for you. The neonatal CCRN and RNC test plans are a blueprint of the exam’s content. The major sections are broken down into subheadings and topics. If you study only a little at a time, you will be fine. One day you may feel like studying cardiomyopathy; the next day, you may focus on chest tubes. You may download the current neonatal CCRN test plan from AACN at www.aacn.org. You may download the current RNC exam outline from www.nccwebsite.org. We did not include the test plans in this book because the exam changes frequently, and you should have a copy of the current information. If you study with a group, you can save a lot of time and effort by breaking up the topics for that study period and having each person present his or her topic(s) and provide handouts and practice questions for the rest of the group. When you can make up a test question about a subject, you really will be prepared. Study for short periods of time, say 30 to 45 minutes, and then take a break. Set small goals, and after you have accomplished each one, reward yourself!

Exam Content The neonatal CCRN certification exam consists of 150 multiple-choice test questions. Twenty-five of those questions do not count; they are there to be validated. In other words, every question is tried first to see if it is written well and if a certain percentage of people answer it correctly. At this point in the process, a question can still be tweaked for use on future exams. Your results are determined by how many answers you get correct. Some answers are a bit harder than others. The final score usually indicates a passing grade if you get at least 70% correct. If you do not know an answer, take your best guess, because you have at least a chance of guessing correctly. You get points only for questions answered correctly. The test plan shows the breakdown of questions by topic and section. AACN uses the Synergy Model as a basis for practice. We provide a brief overview of the Synergy Model in Section 1 of this book. Here is the good news: There are no questions on the test that deal specifically with the terminology of the Synergy Model. Instead, the answers to questions are based on best practice that utilizes and synthesizes the Synergy Model. The RNC examination contains 175 questions. There are different versions of this examination, and some versions are easier than other versions. Questions are weighted as to difficulty.

Study Tips A multiple-choice test question consists of three parts: an introductory statement, a stem (question), and options from which you must select the correct answer. The introductory statement provides information about a clinical issue, pathophysiology, or a nursing action or duty. Stems are worded in different ways. Some stems are in the form of a question; others are in the form of an incomplete statement. Additionally, a stem will usually request one of two types of responses: a positive response or a negative response. More good news: The CCRN test was recently changed so that it does not include negative stem questions.

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Test-Taking Strategies  y  13

This means stems such as “all of the following except” are no longer part of the CCRN exam. (The questions you will practice from in this book may have an occasional negative stem to facilitate learning.) Even more good news: There are no longer any multiplemultiple-choice questions on the CCRN exam. We believe that the RNC exam will also be changing format to eliminate negative stem and multiple-multiple-choice questions. Key words are important words or phrases that help focus your attention on what the question is asking. Examples of key words include always, most, first response, earliest, priority, first, on admission, common, best, least, not, immediately, and initial. You should always be looking for a therapeutic response. The nurse is always ­therapeutic. In other words, your initial response as a nurse is always the therapeutic response— you must acknowledge and validate the patient’s feelings. Communication skills learned in Nursing 101 are important components of successful test-taking strategies. More than one option may contain a therapeutic response. When in doubt, validate, validate, validate. Always validate the feelings before you present information. A medical emergency would, of course, take precedence. Who is actually the focus of the question? You need to be able to identify this person. Sometimes questions are asked about a friend, a relative, or a significant other instead of a patient. A lot of information in the question may be deliberately distracting. Also, you must, when applicable, validate that person’s feelings first.

When in Doubt When answering questions, remember Maslow’s hierarchy of needs and the ABCs (airway, breathing, circulation). When these initial goals are met, then safety is the priority. After safety, the psychological needs are a priority. Assessment always comes before diagnosis and treatment (intervention). Learning takes place only if the learner is motivated. Eliminate incorrect options. This gives you a 50% chance of guessing the correct answer. Here are some hints: • • • • • •

Select the most general, all-encompassing option. Eliminate similar options or those that contain words such as always or never. If two options say essentially the same thing, then neither is correct. If three of the four options sound similar, choose the one that sounds different. Eliminate any options that contain the words always or never. Look for the longest option. It is usually the correct answer. Watch for grammatical inconsistencies between the stem and options.

It’s Time to Take Your Certification Exam! Well, you are finally ready! The night before the exam, get a good night’s sleep. Do not cram the night before, although that is easier said than done. Do something relaxing and enjoyable, like going to a movie or out to dinner. Try to avoid caffeine or any other stimulant. Please let us know when you pass the exam so we can congratulate you.

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14  y  Section 2

Practice Questions The next several sections in this book contain over 900 practice questions. The questions are written with three responses, like you will find on the RNC examination. However, the CCRN practice examination at the end of this book is written with four responses and structured like the actual examination. Even though questions are divided into systems and issues, many topics will cross over to another section. For example, even though there is a section on genetics, you will find questions about genetic problems in several sections. The questions will have a variety of complexity. Some will be quite easy to answer and some will be quite difficult to answer and require critical thinking and integration of practices. On occasion, we ask information previously covered within the context of another patient situation. We have included material on drug dosages and medications commonly in use at time of publication. Keep practicing until you can routinely answer 80% of these questions correctly. At that point, you should be ready to take the certification examination.

Recommended Reading Kobel Lamonte, M. (2007). Test-taking strategies for CNOR certification. AORN Journal, 85(2), 315–332. Ludwig, C. (2004). Preparing for certification: Test-taking strategies. Medsurg Nursing, 13(2), 127–128.

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Section 3 Maternal Risk Factors and Delivery I­ ssues

Questions 1. You are called to the delivery of a 42-week gestation infant from a mother with gestational diabetes. Upon your arrival, the labor nurse reports that the infant is having frequent decelerations and that an amnio infusion ran over 4 hours for thick meconium. The baby is delivered vaginally with thick particulate meconium. You note that the baby has not cried and is limp in the obstetrician’s arms. Your first priority is ­to A. Rapidly dry and stimulate the baby to prevent cold ­stress B. Aggressively suction the airway with the bulb ­syringe C. Avoid stimulating the baby and assist with intubation and tracheal ­suctioning 2. You are called to a primary cesarean section for a term infant’s failure to progress. Over the last few minutes of labor, the baby experienced three 4-minute decelerations with a fetal heart rate of 68. Upon receipt of the baby, you note that the infant is apneic with no tone. After drying the baby and removing blankets, your initial assessment indicates a heart rate 70, apnea despite stimulation, and the baby remains limp and cyanotic. This baby i­ s A. In primary ­apnea B. In secondary ­apnea C. In tertiary ­apnea 3. Which of the following neonates should be intubated ­immediately? A. An apneic, term infant with poor chest rise despite positive pressure ­ventilation B. A 26-week gestation neonate crying with minimal ­retractions C. A 31-week gestation neonate with a marked inguinal ­hernia 4. You are participating in the delivery and resuscitation of a 28-week gestation neonate. The heart rate is 140 with a respiratory rate of 80 with moderate retractions and circumoral cyanosis. Your next step is ­to A. Provide positive pressure ventilation with 100% ­FiO2 B. Provide CPAP using a s­ elf-­inflating ­bag-­mask-­valve C. Provide CPAP using a T ­ -­piece ­resuscitator 5. Prioritization of care during resuscitation should focus on the ABCs. ABC stands ­for A. Airway, breastfeeding, ­crying B. Airway, breathing, ­circulation C. Appearance, breathing, ­color

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16  y  Section 3

6. Transition to extrauterine life begins ­when A. The baby’s head is d ­ elivered B. The baby takes the first ­breath C. The baby takes the first breath and the cord is ­clamped 7. You are attending the vaginal delivery of a term infant with meconium. Which of the following infant presentations would you classify as ­vigorous? A. The baby is crying and kicking, with a heart rate  100 ­bpm B. The baby is crying weakly, grabbing at the stethoscope, and the heart rate is  80 ­bpm C. The baby is apneic and floppy, with a heart rate  100 ­bpm 8. The baby you are resuscitating has persistent cyanosis despite strong respiratory efforts and a heart rate . 100 bpm. What is the next nursing action that should be performed to assist this infant’s respiratory ­status? A. Provide supplemental oxygen via CPAP using a T-­piece B. Provide ­f ree-­flow supplemental oxygen via a ­self-­inflating b ­ ag-­mask-­valve C. Provide ­blow-­by supplemental oxygen via a ­flow-­inflating ­bag-­mask-­valve 9. You are using a fl ­ ow-­inflating b ­ ag-­mask-­valve to resuscitate a 32-week ­gestation neonate. The bag fails to inflate and the chest does not rise. What action should you take first to resolve this ­problem? A. Reposition the head and verify the mask ­seal B. Verify the tubing is connected to the air ­line C. Get a new ­bag-­mask-­valve ­device 10. As a transport nurse, you have been trained to use the new ­T-­piece resuscitation device for preterm deliveries. Which of the following statements is true regarding the use of a ­T-­piece for preterm ­infants? A. The PIP and PEEP are ­pre-­set on the machine ­f ront B. The p ­ op-­off valve is fixed at 30 mm ­Hg C. The rate and inspiratory time are controlled by the staff member manipulating the T-­piece 11. Which of the following reactions is the best way to determine if ventilations with a ­bag-­mask-­valve are ­effective? A. Visible chest ­r ise B. Visible abdominal ­movement C. Spontaneous breathing occurs within 30 ­seconds 12. After assisting with the delivery of a term infant who is apneic, you provided 30 seconds of positive pressure ventilation. The infant remains apneic and the heart rate is 110. What should you do ­next? A. Continue positive pressure ventilation since the heart rate is . 100 ­bpm B. Insert an orogastric ­t ube C. Begin chest compressions if the heart rate is 80 ­bpm

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Maternal Risk Factors and Delivery ­Issues  y  17

13. You are attending the resuscitation of a 33-week gestation infant. You have dried, suctioned, and stimulated the baby. The initial assessment reveals a heart rate of 40 bpm, a spontaneous respiratory rate of 20 bpm, and the baby’s color is cyanotic. According to current NRP guidelines, what is the next action you should ­take? A. Begin chest ­compressions B. Continue stimulating and drying the ­infant C. Provide positive pressure ­ventilation 14. Today you are performing the duties of the charge nurse and are attending an emergency cesarean section. The infant is a 26-week neonate with an 18-­year-­old gravida 1 mother with placenta previa. The infant was delivered pale, floppy, and apneic with a heart rate of 50 bpm. The neonatal nurse practitioner intubated the neonate immediately and positive pressure ventilation was provided with effective chest rise. The heart rate increased to 80 bpm with minimal spontaneous effort. The patient is pale and hypotonic. What is the next step to be performed by your resuscitation t­ eam? A. Provide chest ­compressions B. Prepare to give epinephrine via the endotracheal ­t ube C. Insertion of an umbilical venous line and administration of normal ­saline 15. You are preparing to give epinephrine to a premature infant with a heart rate of 40 bpm despite ­well-­coordinated positive pressure ventilation via an endotracheal tube and adequate chest compressions. Which of the following statements regarding epinephrine administration is ­true? A. The correct epinephrine dose is 0.1 to 0.3 ml/kg via the umbilical venous ­line B. Epinephrine is just as effective whether administered endotracheally or via an umbilical venous ­line C. The endotracheal tube placement does not need to be confirmed prior to administering epinephrine via the endotracheal ­t ube 16. Your resuscitation team is preparing for the delivery of a 27-week gestation ­neonate. Which of the following endotracheal tube (ETT) combinations should you ­prepare? A. A 2.5 ETT with a #1 curved b ­ lade B. A 2.0 ETT with a #0.5 straight ­blade C. A 2.5 ETT with a #0 straight ­blade 17. Intubation of a term infant was just completed. The infant has a heart rate of 20 bpm, no spontaneous respiratory effort, and he is flaccid and mottled. The chest rises equally and breath sounds are auscultated in all lung fields. The CO2 detector does not indicate the presence of exhaled CO2 . What is the next step in this ­resuscitation? A. Continue to provide positive pressure ventilation and observe for a rise in heart ­rate B. Remove the endotracheal tube and reintubate after 30 seconds of positive pressure ­ventilation C. Remove the endotracheal tube and insert a laryngeal mask ­airway

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18  y  Section 3

18. The resuscitation team is providing positive pressure ventilation (PPV) to a premature infant without an effective chest rise. The head has been repositioned, oral secretions suctioned and the resuscitation bag verified. Which mechanical blockage of the airway should be ­considered? A. ­Pneumothorax B. Laryngeal ­webbing C. Pleural ­effusions 19. Your delivery team is resuscitating a 35-week gestation infant with severe respiratory distress. The infant has unilateral chest rise, deviated heart tones, and diminished breath sounds on the unaffected side, with a stretched to scaphoid abdomen. Which of the following actions should be taken ­immediately? A. Needle ­decompression B. Endotracheal intubation and orogastric tube ­placement C. Provide continuous positive airway ­pressure 20. You are resuscitating a term infant born to a mother who was given Stadol 2 hours ago. The infant is apneic, floppy, cyanotic and has a heart rate of 80 bpm. The team has provided stimulation, suctioned, and positioned the airway without improvement. What is the next action for your team to perform to stabilize this ­infant? A. Provide Narcan 0.1 mg/kg IM ­immediately B. Provide positive pressure ­ventilation C. Continue to stimulate until the infant “comes around” 21. While shopping at a local department store, a fellow shopper’s amniotic sac ruptures. The mother tells you she has had three previous children and this child should be due this week. You realize delivery of a single, term infant appears imminent. Emergency medical services (EMS) have been contacted and the unit en route is 5 minutes away. Before EMS arrives, a female infant is delivered. What is the first action you should ­take? A. Dry and stimulate the infant using available clothing ­articles B. Leave the cord attached and focus on establishing ­ventilation C. Place the infant on the floor and pile clothing on ­top 22. To improve temperature control of a premature infant at delivery, you s­ hould A. Use hot water bottles placed next to the ­skin B. Use a space heater next to the b ­ ed C. Use a reclosable polyethylene ­bag 23. Which of the following nursing actions prevents brain injury in the premature neonate during ­resuscitation? A. Placing the neonate in Trendelenburg ­position B. Providing positive pressure ventilation at 29 mm ­Hg C. Titrating FiO2 based on pulse oximetry to maintain saturation between 85% and 95%

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Maternal Risk Factors and Delivery ­Issues  y  19

24. You are asked to visit the parents of a premature neonate who will deliver imminently. The labor and delivery nurse tells you that they have multiple questions regarding prognosis and expected plan of care. You ­should A. Go speak with the parents ­immediately B. Ask the neonatologist to speak with the ­parents C. Wait until after the delivery to speak with the ­parents 25. The resuscitation team is called to an emergency cesarean section of a 38-week gestation infant with absent fetal movement and heart rate for 5 minutes. An apneic, flaccid, and cyanotic infant is handed to you. After drying, suctioning, and positioning, the infant remains apneic without a heartbeat and is gray. Over the next few minutes, the team intubates the infant, provides effective positive pressure ventilation, and begins w ­ ell-­coordinated chest compressions. An umbilical venous line is placed for epinephrine and volume replacement. Throughout the resuscitation, the team has never been able to establish a heartbeat in the infant. At what point may the team stop ­resuscitation? A. After 5 minutes of ­resuscitation B. After 10 minutes of ­resuscitation C. After 15 minutes of ­resuscitation 26. You are transporting a preterm infant in respiratory distress to a facility for a higher level of care. The parents question why their baby will not be eating immediately. Your best response should ­be A. “Respiratory distress leads to an increased risk for choking and lack of oxygen that may inhibit normal intestinal function.” B. “The baby will receive IV nutrition so it’s no big deal.” C. “The baby has high residual glucose from the mother and stress releases more glucose, so feeding at this time would cause hyperglycemia.” 27. Your transport team arrives at an outlying facility to pick up a 34-week gestation neonate with poor feeding tolerance and mild respiratory distress. Upon arrival, the nursery charge nurse reports that blood cultures and a CBC were drawn, a peripheral IV heparin lock was started for antibiotics only, and the baby was nipple fed 20 ml standard formula with iron 30 minutes ago. A repeat blood sugar was 52 mg/dl. You prepare a D10W IV solution at 80 ml/kg/d to hang during transport. This intravenous solution was selected ­because A. The dextrose content of D10W is 10 mg/kg/min, which will prevent the neonate from becoming hypoglycemic during ­transport B. You want to keep the IV patent during ­transport C. You want to provide a constant and consistent supply of glucose to the brain for normal physiologic function to ­continue

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20  y  Section 3

28. You are preparing a preterm neonate for transport to a higher level of care for cardiothoracic surgery. The initial bedside glucose is 28 mg/dl. Per your hospital’s protocol, a random glucose sample is sent to the lab. What should the next step be to help prepare the infant for ­transport? A. Wait for the random glucose results before notifying the ­physician B. Notify the physician and prepare to give 2 mg/kg D10W ­IV C. Nipple feed 30 ml of 24-calorie premature infant formula now and in 30 ­minutes 29. Your transport team arrives at a hospital emergency room that does not have maternal child services to transport a term infant who was surrendered to staff. The infant’s first temperature was 35.4° Celsius. You find the infant wrapped in heated blankets under a radiant warmer. During your assessment, you find the blood glucose to be 17 mg/dl. The ER physician orders Dextrose 25% at 2 ml/kg IV STAT. You ­should A. Administer the dextrose as ­ordered B. Refuse the order and hang a D10W continuous drip at 150 ­mg/kg/day C. Politely refuse the order, notify your transport neonatologist and follow standard stabilization protocol of using Dextrose 10% at 2 ml/kg ­IV 30. To stabilize a neonate for transport, the priority should always focus first ­on A. Airway, circulation, and ­temperature B. Airway, circulation, and laboratory ­results C. Airway, breathing, and ­circulation 31. Your transport team is assessing an infant for transport to your facility. Vital signs are stable and the peripheral IV to the wrist is running Dextrose 10% at 80 ml/kg/d. You note that the IV arm is pale when compared to other extremities and core color. You ­should A. Stop the IV immediately and remove it, then prepare to insert another peripheral ­IV B. Stop the IV for 10 minutes, then restart the IV at 50 ­ml/kg/d C. Place warm compresses to the arm and warm the IV ­fluid 32. You have arrived at a facility to transport a newborn with severe respiratory distress syndrome requiring high pressures and 100% FiO2 by conventional ventilation to maintain saturations. There is an umbilical venous catheter (UVC) infusing D10W at 80 ml/kg/day. During your assessment you note pink, frothy secretions in the endotracheal tube, multiple premature atrial contractions, and tachycardia. You ­should A. Check the UVC and endotracheal tube placement via chest X-­ray B. Suction and provide morphine ­sulfate C. Remove the endotracheal tube and ­reintubate

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33. While transporting a premature infant, the neonatologist orders a heparinized solution to run through the umbilical venous catheter. The order reads, “D10W with heparin 1 unit/ml to run at 8.9 ml/h.” You have heparin 100 units/ml available and a 2.5 dl bag of D10W. How much heparin would you ­add? A. 0.25 ­ml B. 2.5 ­ml C. 25 ­ml 34. Your team confirms umbilical line placement on chest ­X-­ray prior to transporting your term patient. The umbilical artery catheter is located at T4 and the umbilical venous catheter tip is just above the diaphragm. Based on the chest ­X-­ray results, which of the following choices would be an appropriate ­action? A. Nothing, the lines are properly placed and the infant is ready for ­transport B. Withdraw the arterial line to T11–T12 and the venous line to 1 cm below the diaphragm before securing the ­lines C. Confirm with the neonatologist and prepare to withdraw the arterial line back to T6–T9 and then secure the venous ­line 35. During feeding time, you have the opportunity to teach a new mother and father about taking their infant’s temperature. Which of the following statements about this process is ­true? A. Environmental factors do not impact the infant’s ability to ­self-­regulate ­temperature B. Brown fat does not need oxygen to b ­ urn C. A normal temperature for an infant is between 36.5 and 37.5° Celsius (97.7–99.5° Fahrenheit) 36. During resuscitation in an operating suite, a term infant is placed on a radiant warmer that was just turned on. The front of the baby is dried, but the wet linens are not removed. Oxygen is provided via free flow. By which mechanism is the infant losing h ­ eat? A. Conduction from being placed on a cool ­surface B. Convection from wet ­linens C. Evaporative via ­f ree-­flow oxygen d ­ elivery 37. While orienting a new nurse, you are discussing methods to stabilize and maintain temperature of a newborn. Which of the following statements is ­true? A. A premature neonate may be placed in a ­single-­walled isolette if a ­double-­walled isolette is not ­available B. A radiant warmer and a heat lamp should not be utilized at the same ­time C. In the absence of radiant warmer or heat lamp, blankets warmed in the microwave may be ­used 38. Temperature regulation is controlled by the hypothalamus with the release of which ­hormone? A. ­Epinephrine B. ­Norepinephrine C. ­Cortisol

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22  y  Section 3

39. Death of the hypothermic infant may be caused ­by A. Aerobic ­metabolism B. Hypoglycemia and ­hypoxemia C. Bradycardia 40. You are receiving report for a 26-week gestation infant with respiratory distress and feeding intolerance at day 15 of life. The neonate is developmentally positioned in a d ­ ouble-­walled isolette on ­servo-­control with the skin probe on the abdomen. The nurse reports a wide fluctuation in isolette temperature and that the skin probe reading does not always match actual temperatures taken. Which statement best explains these fi ­ ndings? A. The baby was lying on the temperature probe during some of the ­day B. The baby was wrapped too warmly in the developmental positioning ­device C. The temperature probe only records skin temperature at any given time, not core ­temperature 41. Which of the following is a correct method to take the temperature of a ­neonate? A. Rectally, with the tip of a standard thermometer inserted 5 cm into the ­anus B. In the axillary area, by holding the standard thermometer for 3 to 5 ­minutes C. Use a tympanic thermometer for 1–10 ­seconds 42. The mother of a 30-week gestation neonate is sitting at bedside when you let her know that you will be transferring the baby to an isolette. She states, “I don’t want him to go into that box thing. I’ll be afraid to touch him. Can’t he stay in this warmer thing?” What is the best response to her ­request? A. “Sure, we can leave him in the warmer for another week.” B. “No, he must be changed over to an isolette per our policy.” C. “We will assist you in touching and holding your baby as he gets better, and the isolette allows for better temperature control and reduces stress to the baby.” 43. An absolute contraindication to the use of regional anesthesia would ­be A. A history of ­meningitis B. ­Sepsis C. The use of morphine ­sulfate 44. Opioids are frequently used for regional anesthesia via the epidural route. These agents provide anesthesia by binding to receptors in the dorsal horn of the spinal cord a ­ nd A. Affect sensory neurons without affecting motor or sympathetic ­activities B. Are lipophilic and will stay in the lower spinal tract to provide ­anesthesia C. Eliminate the need for continuous ­dosing 45. Which of the following statements is true regarding the use of sedatives in the ­neonate? A. Sedatives enhance the behavioral expression of ­pain B. Sedatives will relieve ­pain C. Sedatives may increase ­pain

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46. Baby Sam is about to undergo a circumcision. A topical anesthetic that may be used for this procedure would ­be A. A Lidoderm ­patch B. EMLA ­cream C. ­Duragesic 47. Possible side effects of EMLA cream ­include A. ­Methemoglobinemia B. Transient kidney ­failure C. ­Ototoxicity 48. A possible adverse effect of morphine ­is A. ­Ileus B. ­Ototoxicity C. Increased venous ­capacitance 49. A commonly used scale for assessment of pain in the neonate is ­the A. Opioid weaning ­scale B. Bether’s ­scale C. N-­PASS 50. A possible adverse effect with the use of fentanyl ­is A. ­Hyperthyroidism B. Chest wall ­r igidity C. ­Neutropenia 51. Fentanyl administered intravenously is incompatible ­with A. ­Morphine B. ­Phenytoin C. ­Piperacillin 52. A contraindication for the use of fentanyl ­is A. ­Asthma B. A urinary tract ­infection C. Concomitant use of ­penicillin 53. A term baby with neonatal abstinence syndrome should be tested ­for A. Hemophilia ­A B. Visual d ­ eficits C. ­HIV 54. The infant you are caring for has been pharmacologically weaned from opiates. Weaning is usually deemed successful if the ­neonate A. Can be easily ­consoled B. Maintains weight for one ­week C. Scores a 10 or less on the neonatal abstinence scoring ­system

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24  y  Section 3

55. You are preparing to transport a 26-week gestation infant born at a rural hospital 3 hours ago via emergency cesarean section. The reporting nurse provides you with a complete blood count (CBC) as part of the chart. You ­would A. Put the lab result with the rest of the chart for the physician to review when you arrive at the receiving ­hospital B. Review the CBC; if the results are abnormal, begin antibiotics ­immediately C. Review the lab, complete the assessment and history, and report findings to the transport ­neonatologist 56. Sam is a 30-week gestation neonate now 14 days old. During the last 2 days, he has been having an increase in the frequency of apneas and bradycardia despite Cafcit administration. In the last 3 hours, he has required positive pressure ventilation to restore normal heart rate, spontaneous respirations, and color. You also note an increase in feeding intolerance with residuals 15–20% of the feeding and abdominal distention. After reporting your findings to the physician, a complete blood count, blood cultures, and IV fluids are ordered. You would anticipate the immature to total ratio (I/T ratio) to ­be A. ­0.05 B. ­0.5 C. ­5 57. You are admitting a 37-week gestation infant to the NICU for feeding intolerance, vomiting, hypothermia, and lethargy. The infant was delivered 18 hours ago vaginally to a homeless mother who received no prenatal care. The mother received 1 dose of penicillin prior to delivery for unknown GBS infection. The baby’s admitting temperature in nursery was 100.2° Fahrenheit, which decreased to 99 after the bath. The pediatrician ordered a complete blood count that morning and the results were: white blood cell count 10 mcgL, segmented neutrophils 40%, band neutrophils 15%, metamyelocytes 5%, lymphocytes 36%, basophils 3%, and eosinophils 2%. Calculate the ANC and I/T r­ atio. A. The ANC is 6000 and the I/T ratio is ­0.33 B. The ANC is 7000 and the I/T ratio is ­0.5 C. The ANC is 7500 and the I/T ration is ­0.9 58. The physician orders ampicillin and Claforan to be started on a 33-week gestation infant with respiratory distress and requiring regular nasal cannula at 1 L/m on 35% FiO2 to maintain saturation 88–95%. A CBC, ­c-­reactive protein (CRP), and blood culture were drawn with placement of an umbilical arterial catheter. The CBC and CRP are normal and the blood culture is pending. Although the physician suspects transient tachypnea of the newborn, why would antibiotics be ­started? A. Regardless of patient presentation, antibiotics should be started prophylactically on every admit to the intensive care ­unit B. Although the CBC and CRP are negative, the patient is experiencing respiratory distress, there may be up to a 6-hour delay before infection is reflected in these ­labs C. Premature deliveries are always due to infection and antibiotic treatment is prophylactic

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Maternal Risk Factors and Delivery ­Issues  y  25

59. The neonatal nurse practitioner ordered ampicillin 100 mg/kg/dose for your patient, who weighs 3.25 kg. How much would ampicillin would you ­administer? A. 32.5 ­mg B. 3.25 ­g C. 325 ­mg 60. You are preparing to administer ampicillin IV to your patient. Which of the following statements is ­true? A. You should administer ampicillin rapidly with a 1 ml saline flush ­afterwards B. Reconstituted ampicillin is stable for 4 hours if stored in the ­refrigerator C. Ampicillin may be infused with intralipids at the terminal injection site of venous ­access 61. Gentamicin 5 mg/kg was started on a 30-week gestation infant 20 days ago. You note that the urine output has dropped to 1 ml/kg/hour and is an amber color in the diaper. The grasp reflex is weak and the infant appears lethargic. You ­would A. Check the last gentamicin peak and ­trough B. Give a 10 ml/kg bolus of normal ­saline C. Administer Lasix 1 mg/kg ­IV 62. The new Ballard scoring system is based on assessment of what ­factors? A. 10 neurologic and 11 physical ­factors B. 10 neurologic and 6 physical ­factors C. 6 neurologic and 6 physical ­factors 63. A gravida 1 mother with no prenatal care and severe ­pregnancy-­induced hypertension treated with magnesium just gave birth to a baby girl. How will the mother’s medical treatment for PIH alter the gestational ­scoring? A. Maternal medical management has no affect on gestational ­scoring B. The neurologic assessment portion will be ­altered C. The physical assessment portion will be ­altered 64. The maximum score a neonate may receive using the New Ballard Scoring system ­is A. ­40 B. ­50 C. ­60 65. To achieve the most accurate gestational score, the neonate should be ­assessed A. Once within 6 ­hours B. Once within 12 ­hours C. Twice within 12 ­hours

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26  y  Section 3

66. You are explaining to a student nurse the correct method to assess arm recoil during the gestational assessment. Which of the following instructions is ­correct? A. Hold the infant prone in your arms to assess the flexion in the ­arms B. Place the patient supine, stretch the arms straight out, then release the arm and measure the a ­ ngle C. Place the neonate supine, hold the arms flexed for 5 seconds, then straighten and release the arms to measure the a ­ ngle 67. You are anticipating the delivery of a 24-week gestation neonate. Which of the following assessment findings would be consistent with a 24-week gestation ­neonate? A. A popliteal angle of 160° B. Thinning ­lanugo C. Flat areola with no ­bud 68. A 30-weeks gestation neonate was vaginally delivered with vacuum assist 20 minutes ago. The weight was 2000 grams, length 45 cm, and head circumference 31 cm. You would classify this neonate ­as A. Preterm small for gestational age (SGA) B. Preterm appropriate for gestational age (AGA) C. Preterm large for gestational age (LGA) 69. One of the differences between the symmetric and asymmetric intrauterine growth restriction (IUGR) ­is A. Symmetrical IUGR has a normal number of cells with reduced cell ­size B. Asymmetrical IUGR has a lower weight and shorter length compared to head ­circumference C. Asymmetrical IUGR is associated with congenital ­malformation 70. A diabetic mother is looking at her new son and is concerned that something may be wrong with her son because his head appears small for his large body. You would ­respond A. “Yes, we are testing chromosomes now.” B. “The head circumference is normal, but seems small in comparison to the body. Insulin does not cross the blood–brain barrier and creates this effect.” C. “Yes, the head is smaller due to premature sealing of the cranial sutures.” 71. The resuscitation team has been called to the vaginal delivery of a female infant to a mother with late prenatal care. The infant is placed on the warmer. The arms are extended straight with slight flexion of the legs. The elbow crosses the sternum, but not past the trunk. You note that the skin is smooth with visible veins and abundant lanugo. When you assess the soles, you note faint red marks without creases. The pinna are slightly curved and soft with slow recoil. The clitoris is prominent with an enlarged minora, and the nipples have no bud with flat areola. The findings are consistent with a neonate of which gestational ­age? A. 24 ­weeks B. 29 ­weeks C. 33 ­weeks

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72. A 47-­year-­old, gravida 10, para 5 mother is in active labor. She received late prenatal care, seeing the obstetrician only once. Estimated gestation is 21 weeks and 450 grams on ultrasound. The mother believes she is at 23 weeks. After a family conference with the obstetrician, a neonatologist, and social service, the parents decide to withhold resuscitation efforts until gestation can be assessed. Upon delivery, the infant is dried, positioned, and placed on the warmer. Weight is estimated to be 350 grams. The skin is friable and sticky, the eyelids are tightly fused, and the heel–toe length is 45 mm. These findings ­suggest A. The parents should be allowed to choose what resuscitation measures should be taken or ­continued B. The infant is viable and all resuscitation measures should be ­initiated C. The infant is not viable and resuscitation efforts should be ­withheld 73. You are called to the vaginal delivery of a 44-week gestation infant with meconium. You anticipate the skin to ­be A. Leathery and cracked with meconium stained ­nails B. Rough with peeling and few v ­ eins C. Smooth with rare ­veins 74. As gestational age increases, the square window ­angle A. ­Increases B. Stays the ­same C. ­Decreases 75. Baby Alia is a 28-week gestation infant born 24 hours ago to a 14-­year-­old mother with no prenatal care. After reviewing the chart, you note that the gestational assessment was not completed. Which of the following assessment criteria is no longer accurate if done ­now? A. The plantar ­creases B. The ­lanugo C. Square ­window 76. The parents of newborn 24-week gestation triplets ask you if they have opened their eyes. You would explain t­ hat A. The eyelids are fused at this time but will open at 25 weeks ­gestation B. The eyelids are fused at this time but will open between 26 and 30 weeks ­gestation C. You can separate the eyelids in the morning with forceful ­pressure 77. You are mentoring a nursing student and discussing the importance of obtaining a maternal history prior to delivery. Maternal health during the pregnancy is extremely important in the development of the fetus and directly affects the ­post-­delivery management of the neonate. Which are the most important maternal factors you should discuss with the nursing ­student? A. Placental function and inherent maternal ­resources B. Placental location and overall maternal ­health C. Maternal socioeconomic and physiologic ­status

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28  y  Section 3

78. You are preparing for the delivery of a 38-week gestation neonate via emergency primary cesarean section to a 29-­year-­old, gravida 1 mother with uncontrolled type 2 diabetes. The mother has been pushing for 15 hours without significant cervical changes or fetal drop. Maternal vital signs at delivery are a heart rate of 120, respiratory rate 20, blood pressure 135/85, and saturation in room air 92%. Fetal weight on last ultrasound was 4.2 kg. Which ­post-­delivery fetal complication would you ­anticipate? A. ­A nemia B. ­Hypotension C. Respiratory distress ­syndrome 79. The neonatal intensive care unit is seeing an increase in the number of teen mothers. Which of the following factors in the management of an infant of a teen mother should you consider when performing discharge ­teaching? A. Teaching the mother is the only ­priority B. Technical jargon is appropriate when explaining medical conditions and ­management C. Discharge teaching should include birth ­control 80. Your patient’s 14-­year-­old mother tells you she smoked pot. How is today’s marijuana different from the marijuana of 20 years ­ago? A. There is no ­difference B. Pot today is purer than pot was 20 years ­ago C. Pot 20 years ago was ­safer 81. You are assisting in a neonatology consultation for a 17-­year-­old in preterm labor at 25-weeks gestation who is addicted to energy drinks. What potential complications in the neonate must you ­anticipate? A. Complications similar to fetal alcohol ­syndrome B. Complications similar to Trisomy ­13 C. Complications similar to heroin ­addiction 82. The neonatal resuscitation team is called to the vaginal delivery of a 38-week gestation infant of a mother having seizures. She is reported as having headaches, hypertension, nausea, and vomiting prior to delivery. The preeclampsia ­work-­up shows normal platelet count, normal AST and ALT, and no proteinuria. What condition is the infant at risk for during ­delivery? A. ­Hyperglycemia B. ­Hypervolemia C. Cerebral vascular ­accident

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83. Addiction and abuse are terms often used incorrectly and interchangeably in conversation with parents and family members. Which statement best defines these ­terms? A. Addiction is when the user cannot stop using with mild systems of withdrawal, and abuse is when the user cannot live without the substance or ­drug B. Addiction is when the user uses the drug or substance in a way not intended and abuse is when the user uses without the knowledge of other ­people C. Addiction is when a user cannot live or function without the substance or drug without severe or ­life-­threatening withdrawal, and abuse is when the user uses the drug or substance in amounts or functions not ­intended 84. Which maternal illegal drug of addiction is the most commonly ­abused? A. ­Marijuana B. ­Heroin C. ­Toluene 85. Heather is a 22-­year-­old admitted in labor at 30-weeks gestation. She denies any drug or alcohol use throughout the pregnancy. When questioned further during the neonatology consultation, she admits to drinking a “few beers” weekly while partying at college. Knowing that most patients will underestimate drug and alcohol consumption, which fetal complications could you anticipate finding at ­delivery? A. Large for gestation, short long ­bones B. Small for gestation, ­microencephaly C. Hypotonicity, lengthened long ­bones 86. Helen tells you that she used witch hazel during her pregnancy. Your first question after hearing this should ­be A. “Why did you take witch hazel?” B. “How did you obtain the witch hazel?” C. “How much and how often did you use witch hazel?” 87. Mara is a 16-­year-­old who was brought into the emergency room after collapsing at a party. She is 25 weeks pregnant and having mild contractions. Mara’s cardiac and neurologic ­work-­up is negative. Her urine drug screen is negative. If Mara delivers in the next few hours, what immediate infant complication could you ­anticipate? A. Respiratory ­depression B. ­Irritability C. Projectile ­vomiting

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30  y  Section 3

88. You are assisting with the neonatal consultation for a 32-week gestation infant of a gravida 3, para 2, 18-­year-­old mother named Brenna. Brenna appears euphoric and confused. While looking around the room, you note a c ­ hewed-­up pacifier in the bed with her. Why would the pacifier be ­significant? A. Brenna’s other children were in the room just before you ­arrived B. Brenna may have been using the pacifier prior to you entering the ­room C. The pacifier was caught in the railing and ­torn 89. If a mother has a history of abusing solvents, what is the most likely effect on the ­fetus? A. Cardiac ­anomalies B. Limb ­deformities C. Intrauterine growth ­restriction 90. You are caring for a 36-week gestation infant born to a mother addicted to methamphetamines. Which complications are you most likely to ­see? A. Cleft palate and ­hypoglycemia B. Hyperglycemia and ­bradycardia C. Feeding intolerance and ­hypothermia 91. Rosa is 34 years old and pregnant with 33-week gestation twins. She is admitted in preterm labor. During her physical assessment, the labor nurse reports that Rosa was combative, red faced, and talking to the walls. You approach the family to obtain a prenatal history, and her husband reports that Rosa has been sick with a severe cough and allergies for 4 days. The husband had gone to the store this morning for Benadryl and Robitussin as Rosa had not slept soundly for 2 days. Rosa has taken both medications every 2 hours for the past 8 hours. You suspect the premature labor is due t­ o A. ­Dehydration B. ­Tachycardia C. ­Hypertension 92. Allison and Jake both smoke one pack of cigarettes daily. You are completing the discharge teaching for their son Aaron born at 26-weeks gestation and who is now 36 weeks adjusted age. Which of the following statements must be included in their ­teaching? A. CPR classes are optional as part of discharge ­teaching B. Hearing and vision screening should only be done at discharge and at 3 years ­old C. Aaron is at higher risk for sudden infant death ­syndrome 93. Sammy is a 35-week gestation infant just born and diagnosed with fetal alcohol syndrome. His mother reports drinking heavily while pregnant and does not want any interaction with the baby. Due to the baby’s presentation and history, you would anticipate withdrawal symptoms to ­occur A. Within the first 12 hours of ­life B. Between 24 and 48 hours of ­life C. Between 48 and 72 hours of ­life

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94. Which of the following drugs are classified as ­stimulants? A. Cocaine, marijuana, and yellow ­jackets B. Doriden, methadone, and ­ketamine C. Modafinil, caffeine, and ­amphetamines 95. Jill is a 26-­year-­old, gravida 12, para 4 presenting in preterm labor at estimated 28-weeks gestation. She has had no prenatal care and refuses to provide health history. Her admitting weight is 100 pounds. A urine drug screen is positive for opioids, oxycodone, marijuana, and cocaine. What additional testing should be ­considered? A. Gonorrhea, Hepatitis D, and ­Candida B. Syphilis, Hepatitis B and ­C C. Gonorrhea, syphilis, and ­Candida 96. Olga is a 28-­year-­old, gravida 2, para 1 in labor at 30-weeks gestation with twins. She has been suffering from postpartum depression since her last delivery 15 months ago. She reports that she has been on Paxil 20 mg/day during the pregnancy, but took extra every day because she was overwhelmed by the thought of twins. Her husband reports finding Elavil 50 mg that was prescribed to take every 8 hours on her bedside table. One fetal heart rate cannot be found using Doppler and the other fetal heart rate is 80 bpm with late decelerations. It is decided to deliver the twins via cesarean section. Which complications should be ­expected? A. Congenital heart ­defects B. ­Hyperglycemia C. ­Macrosomia 97. You are completing discharge teaching to a foster parent for a term baby treated for cocaine withdrawal. The baby still has moments of stiffness and rigidity with arching. What would you suggest to the foster parent regarding ­positioning? A. Always wrap tightly and position him supine when he is ­arching B. Use forceful hand containment to obtain a flexed ­position C. Use a hammock or sling bed for ­sleeping 98. Which of the following mothers should be advised against ­breastfeeding? A. Hazel, a 24-­year-­old nicotine ­smoker B. Nala, a 38-­year-­old who is HIV ­seropositive C. Joan, a 34-­year-­old with Hepatitis ­B 99. Developmental care is based on which of the following standards of critical ­care? A. All infants receive the same care with slight ­differences B. Parents play a critical role in infant care once the infant is ­stable C. Parent and infant interaction is continuously assessed and interactions ­supported 100. You are teaching new parents to recognize signs of stress in their 30-week gestation neonate. Which of the following is a correct statement regarding autonomic ­cues? A. Sighs are a sign of stress and ­instability B. Fisting is a common autonomic ­response C. Squirming and restlessness while in kangaroo care indicate autonomic ­stress

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101. Over the last 2 weeks you have been teaching Jan and Eric about behavioral cues and interventions when caring for their 26-week gestation neonate. You would document effective learning and application ­if A. They call you to the bedside when the baby ­cries B. They provide hand containment when they see arms and legs ­failing C. They adjust the neonate with extended extremities while providing kangaroo ­care 102. To increase parental involvement in their neonate’s care, teaching ­should A. Provide detailed pathophysiologic changes occurring at any one ­time B. Provide short bursts of developmental cues and parent ­intervention C. Provide lengthy explanations of cues and nursing ­actions 103. The best time to begin teaching parents about interpreting their neonate’s behavioral cues ­is A. At ­discharge B. When the baby is at least 30-weeks ­gestation C. Immediately on ­admission 104. While Nora is participating in kangaroo holding of her 29-week gestation son Dalton, it is time for cares. At which point would you perform cares to lessen stress on the b ­ aby? A. When Dalton is asleep without ­movement B. When Dalton is asleep with movements of the chest and ­head C. When Dalton is ­crying 105. The parents of your 31-week gestation neonate, Zeke, are in your unit to participate in kangaroo holding. Prior to placement skin to skin, Zeke’s temperature is taken and is 38° Celsius. Which parent is the better choice for performing kangaroo care at this t­ ime? A. Neither; Zeke is too ­unstable B. Zeke’s f­ather C. Zeke’s m ­ other 106. Jacob and Madison are new parents to 24-week gestation triplets who are 9 days old. Due to severe respiratory and hemodynamic instability during visits, they have been unable to participate in cares. During your update for triplet A, ­Madison remarks that “This doesn’t feel real,” and Jacob adds, “It is hard to bond with them.” An appropriate action to increase parental involvement would ­be A. Have the parents turn the babies’ heads to improve viewing of the ­face B. Have the parents assist in an umbilical line dressing ­change C. Have the parents assist in administering Aquaphor to the skin 107. Developmental positioning is crucial to prevent both skeletal and muscular deformities. Common deformities ­include A. Hip abduction and internal ­rotation B. Shoulder flexion and scapular ­abduction C. Neck extension and head ­molding

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108. Which of the following statements regarding developmental positioning is ­true? A. Functional positioning is always ­comfortable B. Developmental positioning always requires expensive tools and extensive ­training C. Developmental positioning should support midline ­alignment 109. Parents have correctly identified ­self-­regulation actions by their child if they ­report A. Grasping of the parent’s finger and movement toward the ­mouth B. Pushing the parent’s hand away with a splayed fingered ­hand C. Eye roving with head turning away from ­stimulation 110. Jayden is a 34-week gestation infant requiring a peripherally inserted central catheter (PICC) for ­long-­term antibiotic and fluid management, feeding intolerance, and bacteremia. Aliya, Jayden’s mother, is concerned about ­long-­term addiction to narcotics and asks if pain control is possible without drug therapy. You would ­respond A. “PICC insertion is cruel without narcotic administration.” B. “We can provide oral narcotics that are less addictive.” C. “We can swaddle Jayden during the procedure and provide a sucrose pacifier during insertion.” 111. Areceli and Quinton are the parents of a 25-week gestation infant named Valerie. Valerie is now 10 days old and is being extubated and changed to a ­high-­flow nasal cannula. She continues to have minor periodic apneic episodes and was started on caffeine 3 days ago. She is tolerating feedings of breastmilk and will continue to advance feedings. To foster parental involvement and bonding, the nurse ­should A. Encourage the parents to journal Valerie’s progress and their goals to provide ­care B. Limit ancillary family interruption during parental visits so they can focus on Valerie’s ­care C. Direct the parents on teaching goals set weekly during multidisciplinary ­rounds 112. Farah is a 23-week gestation neonate who is now at 33 weeks adjusted gestation. During the last 10 weeks, to appropriately transition Farah to breast and bottle feeding, the nurse should ­have A. Provided the pacifier only during ­procedures B. Placed an orogastric feeding tube for gavage feedings between bottle or breast ­feedings C. Provided oral care with lemon or vanilla flavoring during and between cares when sucking motions were ­noted 113. Damian is a 32-week gestation neonate who is now 34 weeks adjusted gestational age. You are beginning trial oral feedings because of noted increase in quiet alert state, vigorous sucking on the pacifier without desaturation, and rooting during kangaroo care. Successful transition to oral feeding with breast and/or bottle ­is A. Dependent upon nursing skill in feeding with a bottle and/or assisting with ­breastfeeding B. With assistance of speech or occupational therapy in creating a feeding ­plan C. The infant’s ability to maintain physiologic stability, a flexed posture, and a quiet, alert ­state

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114. Preston is now a 35-week gestation neonate who is alternating oral and gavage feedings. You just completed a bottle feeding using a standard or regular nipple. Preston finished 40 ml of breastmilk with human milk fortifier at 1 pack per 25 ml in 20 minutes. He required external pacing only during the first 5 ml of the feeding and no chin or cheek support. Vital signs were stable during and after the feeding. You ­would A. Recommend advancing the feedings to oral feeding x2 then gavage feeding (nipple, nipple, gavage) B. Document a poor feeding for requiring external pacing during any part of the ­feeding C. Hold advancing feedings until external pacing is not required at ­all 115. Caitlyn is the mother of a 26-week gestation neonate requiring mechanical ventilation for respiratory distress syndrome. Caitlyn was planning to breastfeed after delivery, but is concerned about completing that goal. You ­should A. Tell her that breastfeeding is a possibility after staff has established that her infant can bottle feed ­effectively B. Have Caitlyn begin pumping immediately every 2–3 hours to establish milk ­supply C. Tell Caitlyn that she may begin breastfeeding in 1 ­week 116. Which nursing action supports ­long-­term collaborative feeding practices within the neonatal care ­unit? A. Using a temporary bedside feeding care ­plan B. Using poor, fair, and good to describe feeding t­ olerance C. Establishing consistent nursing staff for feeding and general ­care 117. During discharge teaching, you notice multiple bruises on the mother’s arms and red marks on her neck. You suspect she is being physically abused. What other indicator would support your ­assessment? A. Extroverted ­behavior B. Hesitance to discuss home ­situation C. Denial of abuse when asked ­directly 118. According to the Centers for Disease Control and Prevention, which of the following women should not be treated prophylactically with ­antibiotics? A. A GBS positive woman with intact membranes with scheduled cesarean ­section B. A woman with unknown GBS status with preterm labor , 37 weeks ­gestation C. A woman with history of prior GBS positive ­birth 119. At which of the following anticipated deliveries would you expect a neonatal team to be in a ­ ttendance? A. Mother on methadone for drug ­addiction B. 37-weeks gestation ­neonate C. Abruption or maternal ­hemorrhage

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120. Admission criteria for the intermediate care nursery may ­include A. 38-week gestation with transient tachypnea of the newborn without ­oxygen B. 33-week gestation with ­apnea/bradycardia C. 40-week gestation with blood glucose of 50 ­mg/dl 121. The University of Illinois Medical Center at Chicago defines pain as, “An unpleasant sensory and emotional experience, associated with actual or potential tissue damage or describe in terms of such damage. Pain is always subjective.” Pain may be observed in the neonate ­as A. Tachycardia, tachypnea, and ­crying B. Heart rate variability, deep and even respirations, and rapid eye ­movement C. Stable vital signs as neonates do not feel ­pain 122. Cortical perception of pain is complete before A. ­16 B. ­20 C. ­24

week ­gestation.

123. Pharmacologic intervention may be used for infants of a drug- addicted mother. You would anticipate to be used as a substitute for heroin and as a substitute for c ­ ocaine. A. Fentanyl; ­morphine B. Morphine; ­Demerol C. Morphine; ­phenobarbital 124. Your patient is being weaned off IV morphine. The doctor tells you that today the patient will switch from IV morphine to oral morphine. You expect the oral dosage to ­be A. Three to five times the IV ­dose B. The same as the IV dose, but more ­f requent C. Twice the IV ­dose 125. Within a Chinese family, cultural values and practices may include which of the following ­beliefs? A. Chi is an external ­energy B. Disease is caused by disharmony with ­society C. There is wide use of medicinal ­herbs 126. Your patient has been in the second stage of labor for 2 hours and the baby weighs approximately 3900 grams. You anticipate which of the following potential c ­ omplications? A. Breech d ­ elivery B. HELLP ­syndrome C. Shoulder ­dystocia

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127. You are preparing for resuscitation of a baby with breech presentation being delivered vaginally. You anticipate p ­ ossible A. Developmental ­delay B. Lacerations of the maternal birth ­canal C. Meconium ­aspiration 128. What ultrasonographic measurement is frequently used to establish gestational ­age? A. ­Crown-­r ump ­length B. Abdominal ­circumference C. Biparietal ­diameter 129. A newborn’s visual acuity is estimated to be in the range o ­f A. ­20/400 B. ­20/200 C. ­20/100 130. An infant is determined to be healthy if he demonstrates which of the ­following? A. A flexed ­posture B. A relaxed ­posture C. ­Hypertonicity 131. Vernix should exist in skin folds at which level of ­gestation? A. 34–35 ­weeks B. 38–40 ­weeks C. 40–41 ­weeks 132. The primary reason to determine gestational age is ­to A. Provide statistical ­data B. Prevent ­complications C. Anticipate problems with ­development 133. A complication of prone positioning ­is A. Lower energy ­expenditure B. Improved lung ­excursion C. External rotation of the ­hips 134. A father notes several 1 mm papules on the baby’s chin and cheeks. He asks you what would cause this. You should offer which of the following ­explanations? A. He should consult the doctor about birth ­defects B. The spots are normal and will disappear within a few ­weeks C. They are precancerous ­lesions 135. A hypoxic infant who is cold stressed is at risk for ­developing A. Metabolic ­acidosis B. ­Hypocarbia C. ­Hyperglycemia

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136. Infants are at great risk for heat loss at a rate of three to four times that of an adult ­because A. The infant has a greater ­surface-­to-­body ­ratio B. Infants cannot ­shiver C. Infants cannot regulate body ­temperature 137. Your newborn patient’s breasts appear enlarged and have a milky discharge. This finding might ­indicate A. ­Infection B. Maternal ­estrogen C. Trisomy ­13 138. What is the major factor that causes parental stress while their infant is in the neonatal intensive care u ­ nit? A. Illness of the ­infant B. Deviation from expected ­events C. Change in family ­dynamics 139. Marilyn and Frank are the new parents of a 23-week gestation neonate who delivered via emergency cesarean section due to uterine rupture. Initially they say little while visiting and have a glazed expression while at their baby’s bedside. The nurse recognizes this ­as A. The initial response to a ­crisis B. The initial steps to ­coping C. Adaptation to intensive care ­environment 140. As parents of premature and/or sick neonates cope with stress, they must complete several psychological tasks in order to build a healthy relationship with their child. During the neonate’s course of hospitalization, nursing personnel must continually monitor and intervene to support healthy bonding. Which of the following parental actions would indicate the beginning of a negative parental-­neonate ­relationship? A. The father states that he caused the mother go into labor because he let the mother move heavy boxes the day of ­delivery B. The parents insist that clergy performs a blessing and ­baptism C. The parents visit for only 10 minutes at a time and stare at the ­monitor 141. Your facility offers parenting classes. Nathan and Emily are 16-­year-­old, ­first-­time parents of a 32-week gestation neonate. Gail and Ibrahim are 42-­year-­old, ­first-­time parents of a 34-week gestation infant. Prior to discharge home, which parents should be offered parenting ­classes? A. The 16-­year-­old ­parents B. The 42-­year-­old ­parents C. Both sets of ­parents

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142. You are preparing to update Anna and Steven about their son, Evan, a 23-week gestation neonate. He is orally intubated and was treated effectively this morning with a second course of surfactant for increasing respiratory distress and mechanical support. An echocardiogram was ordered for today to evaluate a murmur and fluctuating oxygen requirements. Trophic feedings were started using breastmilk provided by Anna. Which of the following comments or statements regarding Evan’s condition is the most therapeutic and ­correct? A. “Evan was really sick this morning, but we took care of it before you got here.” B. “An echocardiogram was ordered for today to evaluate blood flow through Evan’s heart.” C. “We are beginning trophic or enteral gavage feedings so that Evan can wean off TPN and IL within the week.” 143. Sarah and Graham are the parents of a 30-week gestation neonate admitted to the intensive care unit for respiratory distress syndrome, possible sepsis, and prematurity. Sarah and Graham ask about what to tell the three older siblings about the baby being in the unit. Which of the following statements would be ­appropriate? A. “Your 2-­year-­old daughter will not understand that the baby is in the unit, but she may have separation anxiety with you visiting for great lengths of time.” B. “Tell your 5-­year-­old daughter that the baby is sick and we are giving him shots to make him feel better and she may visit when he is better.” C. “Tell your 11-­year-­old son that since he is in school, he cannot visit because of risk of infection.” 144. Jose and Nita are parents of 24-week triplets, Juanita, Sergio, and Tierra, born 5 days ago. Tierra was diagnosed with a grade IV intraventricular hemorrhage and cardiogenic shock, and was removed from life support yesterday. Jose and Nita are crying at the bedside while visiting Juanita and Sergio today. What should you ­do? A. Tell them you are sorry for their loss and offer to listen or pray with ­them B. Do not say anything about Tierra; just update them about Juanita and ­Sergio C. Pull the curtain and give them ­privacy 145. The purpose of risk management is ­to A. Identify healthcare staff who are ­negligent B. Collect and store records regarding potential and actual injuries in the ­hospital C. Determine scope of practice within the hospital based on financial ­liability 146. To provide consistent and complete transfer of care from one healthcare professional to another, regulating bodies recommend standardized ­report-­off or ­hand-­off documentation or reports. An example of this ­is A. ­SBAR B. ­DBAR C. ­SOAR

This concludes the Maternal Risk Factors and Delivery Issues ­questions.

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Maternal Risk Factors and Delivery Issues A ­ nswers 1. Correct answer: ­C According to current NRP guidelines (5th ed.), the baby is not vigorous and should be immediately intubated and tracheally suctioned using the appropriate sized endo­tracheal tube and meconium aspirator. An additional indicator for immediate tracheal suctioning of meconium is the presence of a heart rate under 100 bpm. The priority is to open and clear the airway. If a baby is stimulated prior to clearing the airway of meconium, the baby is likely to aspirate the meconium, possibly leading to meconium aspiration syndrome. Once the airway is clear, the team should dry and stimulate the infant, using the bulb suction as appropriate. Due to the fragile mucosal membranes of the oral cavity, it is inappropriate to be aggressive while suctioning. If the baby has copious secretions, it would be appropriate to use wall suction to clear oral ­secretions. 2. Correct answer: ­B This baby is in secondary apnea as the baby did not respond to previous stimulation while drying and suctioning. Immediate action should include providing positive pressure ventilation with 100% oxygen for 30 seconds. As research becomes available, the concentration of FiO2 recommended currently at 100% may change. Please refer to the most current NRP provider manual from the American Academy of Pediatrics for any changes since publication of this ­text. 3. Correct answer: ­A Immediate intubation is indicated when a neonate cannot be ventilated effectively via positive pressure ventilation. Additional indicators for intubation include a neonate who has extreme low birth weight, an infant requiring prolonged positive pressure ventilation, an infant not responding to resuscitation efforts, or an infant who requires surfactant therapy. Intubation is also indicated for infants requiring tracheal suctioning for nonvigorousness in the presence of meconium, and those with a diaphragmatic ­hernia. 4. Correct answer: ­C A ­T-­piece resuscitator or ­flow-­inflating ­bag-­mask-­valve would be appropriate methods for providing continuous positive airway pressure (CPAP). A ­self-­inflating ­bag-­mask-­valve requires the user to squeeze the bag to provide oxygen. Current NRP guidelines recommend that the initial oxygen begin at less than 100% FiO2 during resuscitation of a premature neonate and that oxygen be titrated for saturation via pulse ­oximetry. 5. Correct answer: ­B Neonatal Resuscitation Provider guidelines focus care around the continual assessment and management of airway, breathing, and ­circulation. 6. Correct answer: ­C Transition from fetal circulation to independence requires two major changes in circulation. The first change requires that infant’s lungs fill with air, resulting in pulmonary vascular bed dilation and a subsequent drop in pulmonary vascular resistance. The second change occurs when the cord is clamped. Cord clamping initiates an

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increase in systemic vascular resistance, thus beginning the closure of fetal ­r ight-­to-­left shunts. Complete circulatory transition may take hours or days depending upon gestation at ­delivery. 7. Correct answer: ­A If the baby has strong respiratory effort and has strong tone with a heart rate . 100 bpm, as in option A, the baby is considered vigorous. If the baby presented as in option B, with a weak cry, heart rate . 80 bpm and grabbing the stethoscope, the baby should be provided stimulation and positive pressure ventilation (PPV) for 30 seconds. If the infant presented as in option C, the baby should be tracheally intubated and suctioned for meconium prior to stimulation, and PPV may be needed if apnea persists despite ­stimulation. 8. Correct answer: ­C For persistent cyanosis despite stable heart rate and strong respiratory effort, provide ­f ree-­flow ­( blow-­by) supplemental oxygen. Oxygen delivery may be by means of a ­flow-­inflating b ­ ag-­mask-­valve, cupped oxygen tubing, ­T-­piece, or neonatal simple oxygen mask for 30 ­seconds. 9. Correct Answer: ­A If a fl ­ ow-­inflating b ­ ag-­mask-­valve does not inflate during use, first reposition the baby’s head and verify the mask seal. Next, verify that the tubing is connected to oxygen, not air, and the valve is turned to between 8 and 10 Lpm. Assess the problem from the baby toward the wall connections. Use suction to clear oral and nasal secretions and reposition the head to the sniffing position. Check that the manometer is connected properly and see if the ­flow-­control valve is not open fully. Next, check for any kinks or breaks in the bag or tubing. If you are unable to rapidly determine the cause of the bag failure, immediately obtain and use another bag or ­bag-­mask-­valve device. Many equipment complications can be avoided during resuscitation by checking the bag, connections, and oxygen flow prior to ­delivery. 10. Correct answer: ­C The staff member using the ­T-­piece controls the ventilation rate and inspiratory time. The PIP or positive inspiratory pressure and ­pop-­off valve should be ­pre-­set on the ­T-­piece machine front. The PEEP or positive end expiratory pressure is set at the ­T-­piece circuit near the patient. Changes can be made with each breath to assist in preventing pneumothorax and over/under ventilation during resuscitation. The system allows the user to provide ventilatorlike support using either a mask or endotracheal ­t ube. 11. Correct answer: ­A Chest rise is the fast way to determine if ventilations with a ­bag-­mask-­valve are effective. Additional methods are to observe for a rise in heart rate, improvement in color, and audible breath sounds are ­auscultated. 12. Correct answer: ­B Despite providing positive pressure ventilation (PPV) for 30 seconds, the baby is still apneic. PPV must be continued and an orogastric tube should be inserted. If

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spontaneous respiratory effort returns and the heart rate is . 100 bpm, then you may halt PPV. If the heart rate is 60–100 bpm, then PPV must continue for an additional 30 ­seconds. 13. Correct answer: ­C The heart rate did not improve with stimulation and spontaneous respiration. The heart is likely ischemic, so the best initial treatment is to provide 30 seconds of positive pressure ventilation (PPV) to improve oxygenation to raise the heart rate. If, after 30 seconds of PPV, the heart rate remains under 60 bpm, then chest compressions should be initiated with continued PPV and an orogastric tube should be ­placed. 14. Correct answer: ­C The history indicates hypovolemia related to maternal blood loss and poor neonatal response to resuscitation. Because the airway has been secured with a moderate heart rate increase, volume expanders are now indicated. Initial resuscitation with 10 ml­/­kg of normal saline over five to ten minutes is appropriate if packed red blood cells are not immediately ­available. 15. Correct answer: ­A A 1:10,000 concentration of epinephrine should be administered at 0.1–0.3 ml/kg via the umbilical vein and 0.3–1 ml/kg via the endotracheal tube. Absorption rates of epinephrine via the endotracheal tube are unreliable in the neonate and may not be as effective. Umbilical venous administration of epinephrine is the preferred route of administration. Epinephrine increases oxygen demand within tissues. To prevent ischemia and infarction of cardiac tissue, it is critical that oxygen be correctly delivered and confirmed prior to epinephrine administration. If the patient is intubated, use both primary and secondary assessment tools to verify endotracheal tube ­placement. 16. Correct answer: ­C A 27-week gestation infant would be estimated to weigh , 1000 g. The appropriate endotracheal tube (ETT) size would be a 2.5 with a #0 straight ­blade. 17. Correct answer: ­A If neonatal circulation is compromised, the lungs may not have exhaled adequate quantities of CO2 to register a change in the device. The resuscitation team must use primary confirmation (equal chest rise, audible breath sounds in all lung fields, absent air movement over the stomach, rise in heart rate, and visualization of the tube between the vocal cords) to determine positive endotracheal tube ­placement. 18. Correct answer: ­B Laryngeal webbing is a mechanical blockage that is usually noted during intubation. The tissue growth may cover the tracheal airway, cover the vocal cords, or grow between the cords, making intubation difficult or impossible. Immediate tracheostomy may be required to secure an airway. Pneumothorax and pleural effusions are examples of functional airway complications seen during and after ­resuscitation.

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19. Correct answer: ­B The clinical presentation is consistent with diaphragmatic hernia. Bowel tones may or may not be auscultated in the thoracic cavity if hypoxemia occurred during the labor process or resuscitation. Auscultation of sounds over the epigastrium may reflect air passage through the esophagus to the stomach rather than gastric motility. The degree of respiratory distress, unilateral chest rise, deviated heart tones, and abdominal appearance is dependent upon the severity and extent of the hernia. Although hernias are typically located on the left side, ­r ight-­sided hernias do occur. Endotracheal intubation and the placement of an orogastric tube to ­low-­intermittent wall suction are required to maximize ventilation and oxygenation. To decrease the risk of pneumothorax related to elevated airway pressures, the physician may purposely intubate the unaffected side to allow for lower ventilation pressures. Transillumination and chest ­X-­ray may be used to rule out secondary ­pneumothorax. 20. Correct answer: ­B The priority is to initiate ventilation using positive pressure ventilation (PPV) to stabilize heart rate and color. PPV for 30 seconds will assist in differentiating the cause of neonatal depression from secondary apnea or opioid administration. If PPV does not restore respirations and tone but stabilizes heart rate and color, then Narcan is indicated. Another requirement is a history of opiate administration within the last 4 hours. If the mother is a suspected addict or if you are unsure of the history, do not administer Narcan, as seizures may occur. Continue to provide PPV and support until causative factors can be identified. If the mother is receiving magnesium sulfate, Narcan will not restore respiratory function, so continue to provide PPV and support until sufficient neonatal clearance of excessive magnesium can ­occur. 21. Correct answer: ­A The steps of resuscitation are the same regardless of location. Upon delivery of the infant, begin drying, stimulating, and clearing the airway using available articles of clothing. The cord should be clamped using a tie, shoelace, or other material that clamps, but does not cut, the cord. Do not cut the cord as this may provide an entry of bacteria into neonatal circulation. Wait for EMS personnel to cut the cord using sterile equipment. Clamping the cord is instrumental in transitioning neonatal circulation. The infant should be placed skin to skin with the mother and face up on the mother’s chest, then covered to maintain temperature. The floor is cold and the infant would lose heat via conduction if she were placed on the ­floor. 22. Correct answer: ­C For premature neonates under 28 weeks gestation, use a reclosable polyethylene bag that comes to the neck and a hat while the infant is under a heat lamp, radiant warmer, or transporter. If these are not available, use warmed blankets, the mother’s skin, turn up the ambient temperature to the room, and place a warming pad under the neonate’s blankets or towels. Do not place heated water bottles directly next to the skin, as this may result in a burn and increase the risk of infection and injury. A space heater may be used to raise the room temperature, but do not place it immediately next to the bed where the air can blow against the ­skin.

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23. Correct answer: ­C The fragile vasculature of the premature neonate’s brain requires gentle handling and positioning in a neutral or reverse Trendelenburg’s bed angle. The high airway pressures should be avoided and fluid resuscitation be given slowly. Oxygen concentration delivery should be titrated based on pulse oximetry with accurate correlation with heart rate to maintain saturations between 85% and 95%. 24. Correct answer: ­B The best staff member to speak with the parents is the neonatologist or nurse practitioner with or without the obstetrician present to answer questions regarding prognosis. The nurse can only speak to nursing plan of care and should not be discussing the medical plan of care with the parents. It is important to establish effective communication and trust with the parents prior to delivery if time is available to improve overall understanding and patient ­outcomes. 25. Correct answer: ­B According to current NRP guidelines (5th ed.) from the American Academy of Pediatrics, if, after 10 minutes of resuscitation, there is no heartbeat and each resuscitation step has been completed correctly and verified, then the team may stop resuscitation efforts. More time may be needed to ensure that each step of resuscitation was completed correctly and effectively. Additional time may also be needed to fully treat identified causes of fetal arrest such as hemorrhagic or hypovolemic shock and asphyxia. Ethical dilemmas are frequent challenges during resuscitation as providers balance legal, ethical, and moral elements in delivering care. If the prognosis is unknown, continual communication with the parents by the physician is critical in determining best outcome for the infant, even if that choice is to withdraw support. After any resuscitation, it is vital for staff members to discuss the resuscitation to identify team strengths and opportunities for improvement and any ethical challenges. Discussion may identify more effective ways to communicate and improve competence among various disciplines, departments, and ancillary ­staff. 26. Correct answer: ­A When caring for any infant in respiratory distress, it is important to hold oral feedings until gastric function has been determined. If the infant is tachypneic, there is an increased risk of aspiration. Aspiration may occur as a result of air trapped in the stomach from oxygen delivery devices, poorly coordinated suck, swallow, and breathing due to hypoxia and diminished activity tolerance. Additional reasons to delay oral feedings include hypoxic effects and hypotension on the intestines that may inhibit or stop peristalsis. Although IV nutrition will be initiated, the answer given in B does not acknowledge or address the parent’s question. Increased stress and physiologic distress increase glucose utilization by the infant, resulting in initial hypoglycemia, not ­hyperglycemia. 27. Correct answer: ­C Neonates with respiratory distress and feeding intolerance may have delayed gastric emptying due to intestinal hypoxia. This neonate is at risk for aspiration during transport due to recent feeding and an existing feeding intolerance. To provide a consistent

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and constant supply of glucose to the brain, a maintenance drip of glucose should be provided. D10W or dextrose 10% in water at 80 ml/kg/day provides 5.5 mg/kg/min of ­glucose—­similar to glycogen levels (4–6 mg/kg/min) released by a healthy term liver when patients are not ­eating. 28. Correct answer: ­B A glucose level of 28 mg/dl is insufficient to maintain normal neurologic function. Although the physician should be notified immediately, the nurse should prepare a glucose bolus in anticipation of the order. Glucose replacement should be initiated immediately to raise the blood glucose level to at least 50 mg/dl. Waiting for random glucose results from the laboratory causes an unnecessary delay in treatment and could result in a further drop in glucose. As the infant is being transported for surgery, the neonate should remain NPO and a maintenance IV of a glucose solution should be ­started. 29. Correct answer: ­C It is the responsibility of the transport team to function and care for the neonate within the standards of care for stabilization. The ER physician may not be aware of or current to the standards of care in newborn stabilization. It would be appropriate to politely decline the order and contact the accepting neonatologist for orders while following standard stabilization practice. Dextrose concentrations of 25% and 50% provide a bolus of glucose that would cause profound hyperglycemia followed by rebound hypoglycemia, as well as severe damage to the ­vessels. 30. Correct answer: ­C Stabilization of a neonate for transport within a facility or between facilities cannot begin until the patient’s airway has been established and protected, effective breathing (spontaneous or assisted) has been initiated, and circulation (heart rate over 100 bpm and color improved) is confirmed. These three criteria are essential to patient outcome, and failure of any of these steps is life ­threatening. 31. Correct answer: ­A The peripheral IV is likely in the artery, not a vein, as evidenced by the blanching of that extremity. The nurse should stop the IV fluid immediately and remove the catheter. The physician should be notified while another peripheral IV is ­placed. 32. Correct answer: ­A Pink, frothy secretions may indicate multiple factors such as traumatic intubation, carina irritation, and pulmonary hemorrhage. The presence of ectopy on the monitor indicates a misplaced UVC. If the UVC is inserted too deeply, the catheter may travel into the right atrium and then into the ventricles or left atrium, causing cardiac irritation and ectopy. The chest ­X-­ray will assist in determining the UVC and the endotracheal tube position. Additional complications of a deep UVC placement may include emboli formation, infarction, pericardial effusions, endocarditis, pulmonary infarction, and pulmonary hemorrhage. The physician should be notified of your findings, and you should anticipate the physician ordering the line pulled back to a position just above the diaphragm and a ­follow-­up chest X-­ray.

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33. Correct answer: ­B You would draw up 2.5 ml of heparin 100 units/ml to add to D10W to make the final concentration of heparin 1 unit/ml in a 2.5 dl bag of D10W. To calculate how much heparin to add to the D10W, first figure how many milliliters there are in a 2.5 dl bag. Because 1 dl5100 ml, there are 250 ml in 2.5 dl. Next you can calculate that 250 ml/100 units per ml 5 2.5 ml. To prevent potential medication problems during transport, make sure to double check all calculations, concentrations, and drawn medications prior to constitution, mixing, or ­administration. 34. Correct answer: ­C Prior to starting any medications or fluids, confirm line placement with the physician or radiologist to avoid injury to the patient. The umbilical artery catheter tip should be located within T6–T9 for a high line and between L3 and L4 for a low line. The umbilical venous catheter tip should be located just above the diaphragm or only 2–5 cm in depending on gestation and weight. Never advance a catheter if the sterility has been compromised. This patient should have the arterial line withdrawn to T6–T9. Repeat the chest ­X-­ray to confirm the new tip placement. Originating from the aortic arch, the coronary and subclavian arteries (above T5), celiac and superior mesenteric arteries (T11–T12), renal artery (L1), the inferior mesenteric arteries (L2) and iliac arteries (L4–L5) may be damaged by fluids and medications administered via a misplaced umbilical artery catheter. A venous catheter tip in the liver may cause irreversible damage to the ­liver. 35. Correct answer: ­C Normal temperature for an infant is between 36.5 and 37.5° C (97.7–99.5° F). Mild hypothermia begins when neonatal temperature falls below 36.4° C or 97.6° F. Moderate hypothermia begins at temperatures below 35.9° C or 96.6° F. Severe hypothermia and death result when the temperature drops below 32° C or 89.6° F. Premature infants are at greater risk of temperature instability at considered normal ranges if there is concurrent sepsis, cardiac anomalies, or respiratory distress. Brown fat deposits are incomplete in the premature infant as the majority of brown fat deposits occur during the third trimester. Similar to a match, brown fat burns fast and hot and requires oxygen to be metabolized. If the neonate is hypoxic, then metabolism of brown fat is further restricted. Environmental factors greatly impact the neonate’s ability to conserve heat and ­self-­regulate temperature. Bed type, humidity, moisture, ambient temperature, air drafts, and the temperature of items touching the skin (equipment, fluids, clothing) all influence neonatal ­temperature. 36. Correct answer: ­A This baby is losing heat multiple ways. Heat loss is by conduction from being placed on a cold surface. The radiant warmer was not on long enough to warm the bed prior to the infant being placed on the surface. Conduction heat loss may also occur if the staff’s hands or stethoscope are cold when touching the infant. Heat loss via convection occurred when the unheated oxygen was delivered and by the cold temperature of the room. Convective heat loss is accelerated if a draft blows on or near the infant. Evaporative heat loss occurs when there is loss of heat through moisture, such as skin left wet in a cold room. Radiant heat loss is the final mechanism not mentioned in the

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scenario. This is a loss of heat between solid objects/surfaces not in direct contact with the infant, such as windows, cold walls, and a ­single-­walled ­isolette. 37. Correct answer: ­B The radiant warmer should be set to servocontrolled temperature regulation based on neonatal skin temperature. If a heat lamp is utilized at the same time, the servo probe may register the heat lamp and not accurately interpret the infant’s temperature, resulting in a failure to heat the warmer properly, and the neonate may become cold. Premature neonates should always be placed in ­double-­walled isolettes to minimize heat loss via convection and radiation. Never use any blanket or fluid warmed in the microwave. The heat element heats unevenly and cannot be regulated from unit to unit. Hot spots in the blanket that come in direct contact with skin will cause burns to the fragile dermal layers. This is also true of water bottles, gloves filled with hot tap water, and chemical mattresses in direct contact with ­skin. 38. Correct answer: ­B Norepinephrine is released by the hypothalamus in response to chemical and temperature receptors in the skin, face, and along the spinal column. Norepinephrine triggers a cascade of actions within the body to retain or create heat in the core. The efficiency of this cascade is impaired by gestation, weight, disease process, and respiratory function. Peripheral vasoconstriction shunts blood flow to internal organs to control heat loss through the massive surface area of the infant. Unfortunately, in attempting to raise temperature, the metabolic rate is also increased, resulting in a significant increase and consumption of oxygen and glycogen to burn brown fat or cause movement and flexion of extremities to generate heat. Norepinephrine also causes pulmonary vasoconstriction that inhibits normal blood flow through the lungs, inhibiting blood oxygenation. In premature and sick neonates, hypoglycemia and hypoxia may be compounded by a lack of brown fat, muscle tone, or sympathetic nervous system control. The best treatment for hypothermia or cold stress is appropriate and timely ­prevention. 39. Correct answer: ­B Infants experiencing severe hypothermia are dying from a lack of sufficient oxygen supply for demand by the tissues and hypoglycemia related to an increase in glycogen metabolism with diminishing supply. In the absence of both oxygen and glucose, anaerobic metabolism is used to create energy at great cost to tissues as lactic acid builds up in the body. As pH decreases and lactic acid increases, tissues throughout the body are destroyed. Bradycardia, apnea, renal failure, and altered neurologic function worsen as tissues are destroyed, and hypoxia and hypoglycemia ­continue. 40. Correct answer: ­C Although answers A and B are probably true, they do not explain the reason that the temperature probe used on servocontrolled beds and warmers only reads the skin temperature and not the core temperature. When using temperature probes, it is important to continue to monitor core temperatures to determine the effectiveness of the bed or warmer in maintaining a neutral thermal environment. Infants who are lying on temperature probes, bundled tightly, or dressed in clothing may artificially read too warm, and the isolette or radiant warmer may decrease the temperature in response,

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leading the air surrounding the infant to cool. Any portion of the body still exposed to the air, such as the head and extremities, may cause the hypothermic cascade to be initiated. To counter this issue, move the temperature probe location with position changes to prevent the infant from lying on the ­probe. 41. Correct answer: ­B The safest method for temperature assessment is using the thermometer in the axilla for 3 to 5 minutes with skin touching skin. Many hospitals have halted rectal temperatures as a standard method for assessing temperature in most neonates because of the danger of inserting the tip too far into the colon, resulting in perforation where the sigmoid colon angles sharply and/or vaginal stimulation. Because of its flexibility, a temperature probe may be inserted into the anus up to 5 cm to obtain core temperatures. A tympanic thermometer is difficult to use and less reliable in the neonatal population because the ear canal is often smaller than the tympanic tip and cannot make a complete seal with the ­skin. 42. Correct answer: ­C The best approach to parent concerns is to address them directly and then provide the reason for your actions. Isolettes may be seen as a clear but physical barrier to the parents as compared to an ­open-­access radiant warmer. To encourage parent bonding, support open communication, and provide teaching, the nurse’s best response is, “We will assist you in touching and holding your baby as he gets better, and the isolette allows for better temperature control and reduces stress to the baby.” Leaving an infant in the warmer may meet the mother’s needs, but it does not address the infant’s need for a stable, neutral thermal environment. Stating policy and ignoring parent concerns closes communication and removes parent involvement in patient care and further compounds barriers to ­bonding. 43. Correct answer: ­B Absolute contraindications to the use of regional anesthesia would be sepsis, active meningitis, bleeding disorders, malformations of the spinal column, hydrocephalus with increasing ICP, seizures, or ­allergy. 44. Correct answer: ­A Opioids are frequently used for regional anesthesia via the epidural route. These agents provide anesthesia by binding to receptors in the dorsal horn of the spinal cord and affect sensory neurons without affecting motor or sympathetic activities. Morphine is actually hydrophilic and will stay in the cerebrospinal fluid longer and tends to travel toward the ­brain. 45. Correct answer: ­C Sedatives given to neonates may actually increase pain. Sedatives suppress the behavioral expression of pain and do not have analgesic ­effects. 46. Correct answer: ­B EMLA cream is frequently used for topical anesthesia for procedures such as circumcisions, arterial punctures, venipunctures, and PICC line placement. EMLA contains

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lidocaine 2.5% and prilocaine 2.5%. EMLA works by releasing lidocaine and prilocaine from the cream into the epidermal and dermal layers of the skin and by the accumulation of lidocaine and prilocaine in the area of dermal pain receptors and nerve ­endings. 47. Correct answer: ­A Possible side effects of EMLA cream include methemoglobinemia, thrombocytopenia, rash, liver failure, fever, erythema, and transient local ­blanching. 48. Correct answer: ­A Possible adverse effects of morphine include respiratory depression, ileus, abdominal distention, delayed gastric emptying, hypotension, bradycardia, and urine ­retention. 49. Correct answer: ­C N-­PASS stands for Neonatal Pain Agitation and Assessment Scale. Correct use of this scale will give you two scores—­one for the assessment of pain and another for the assessment of ­sedation. 50. Correct answer: ­B Possible adverse effects of fentanyl include respiratory depression, chest wall rigidity, apnea, laryngospasm, abdominal distention, loss of bowel sounds, and generalized muscle ­r igidity. 51. Correct answer: ­B Fentanyl administered intravenously is incompatible with phenytoin, azithromycin, and ­pentobarbital. 52. Correct answer: ­A Contraindications for the use of fentanyl include increased intracranial pressure, severe respiratory disease or depression including acute asthma (unless the patient is mechanically ventilated), and seizures. Additional contraindications include CNS depression, paralytic ileus, severe liver or renal insufficiency, and hyperglycemia (fentanyl may elevate blood glucose). 53. Correct answer: ­C A term baby with neonatal abstinence syndrome should be tested for HIV. You must adhere to the policy of your institution and obtain the mother’s consent. There is the possibility that HIV screening is a routine part of the admission procedure for your ­facility. 54. Correct answer: ­A Weaning is usually deemed successful if the neonate can be consoled, scores an 8 or less on the neonatal abstinence scoring system, maintains a rhythmic sleep and feeding cycle, and has steady weight ­gain.

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55. Correct answer: ­C It is important to review all lab work as compared to patient presentation prior to reporting findings to the physician. This allows the nurse to provide the most comprehensive assessment of the patient to the physician when obtaining orders. Depending on patient clinical presentation, antibiotics may or may not be initiated. Patient presentation may prompt the nurse to calculate the absolute neutrophil count (ANC) to determine potential for bacterial ­infection. 56. Correct answer: ­B The immature to total ratio (I/T ratio) assesses the neutrophil storage pool (NSP) of infants . 60 hours old. The calculation should be done any time the percentage of neutrophils exceeds 20%. High neutrophil counts indicate a response to a bacterial infection. The number of immature cells is divided by the number of total neutrophils. An I/T ratio . 0.25 indicates suspected infection. An I/T ratio . 0.8 indicates high risk of death from ­sepsis. 57. Correct answer: ­A Along with the clinical presentation, an ANC of 6000 and an I/T ratio of 0.33 indicates that this infant is responding to a bacterial infection. The ANC is calculated by adding the segmented neutrophils, the band neutrophils, and the metamyelocytes as a percentage (60%). That percentage is then multiplied by the total number of white blood cells (10,000 3 0.6 5 6000). This number is evaluated using a Manroe chart. An ANC of 6000 is below the normal range for an infant who is 18 hours old. The I/T ratio is calculated by dividing the sum percentages of immature neutrophils by the total number of neutrophils (20 4 60 5 0.33). 58. Correct answer: ­B Antibiotics should be started prophylactically on any patient with respiratory distress requiring oxygen therapy despite having an initially normal CBC and CRP. There may be up to a 6-hour delay before infection is reflected in the CBC and/or CRP. Insufficient blood draw for the blood culture may delay a positive blood culture result until sufficient growth is present. In 6 hours, the infant may become overwhelmingly septic and may be unresponsive to treatment. Other indicators of possible infection would include temperature instability, feeding intolerance, and altered ­perfusion. 59. Correct answer: ­C The correct dosage would be 325 mg of ampicillin per dose. Calculation: 100 mg 3 3.25 kg 5 325 ­mg. 60. Correct answer: ­C Ampicillin is a common ­broad-­spectrum antibiotic used in prophylactic and ongoing treatment for bacterial infections. Ampicillin is compatible at the terminal injection site with intralipids, but not with dextrose/amino acid solution. Once reconstituted, ampicillin should be administered within 1 hour by IV push slowly over 3–5 ­minutes.

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61. Correct answer: ­A The nurse should check the last peak and trough values prior to contacting the physician. The infant is exhibiting signs and symptoms of nephrotoxicity and neurotoxicity as presented by a drop in urine output and muscular weakness. Although a patient may recover from gentamicin nephrotoxicity, the ototoxic and neurotoxic effects are ­irreversible. 62. Correct answer: ­C The New Ballard Scoring system is based on 6 neurologic and 6 physical factors. The original Dubowitz system was based on 10 neurologic and 11 physical factors, but was cumbersome and required significant skill and time to complete correctly. The first Ballard scoring system simplified the scoring system by eliminating the active tone scoring. The tool still requires skill to perform, but less time. The New Ballard system further refined the scoring tool to include scoring from 20 to 44 weeks ­gestation. 63. Correct answer: ­B The magnesium sulfate used to treat the mother’s p ­ regnancy-­induced hypertension will cause the neurologic portion of the assessment to be underscored. Other treatments that cause sedation, muscle relaxation, or neurologic depression may have similar affects on gestation assessment. Neonatal factors that may alter scoring include neonatal neurologic disorders, breech presentation, deformities, and ­asphyxia. 64. Correct answer: ­B The maximum gestational score assigned to a neonate is 50, representing 44 weeks gestation. The minimum score at this time is 212, representing a neonate under 20 weeks gestation. The scoring is within 6 2 weeks gestation. For each additional 5 points assigned to the neonate, the gestational age increases by 2 ­weeks. 65. Correct answer: ­C To achieve the most accurate gestation score, the neonate should be assessed twice within 12 hours. To limit preconceived expectations of a neonate’s gestation from altering the score, the assessment should be done by two different evaluators. Before conducting the assessment, be cognizant of physical and neurologic factors that may influence ­scoring. 66. Correct answer: ­C In order for you to measure arm recoil during gestational assessment, the neonate should be supine. Hold the arms flexed for 5 seconds, then straighten the arms down and quickly release. The angle the arm flexes indicates maturity. The more mature the neonate, the less degree of angle between the forearm and the upper ­arm. 67. Correct answer: ­A A popliteal angle of 160° would be consistent with a gestational age of 24 weeks. Thinning lanugo and flat areola with no bud are associated with gestations between 28 and 30 ­weeks.

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68. Correct answer: ­C At 30-week gestation, this neonate is preterm. The weight, length, and head circumference are all above the 90th percentile and would classify this neonate as preterm LGA (large for gestational age). 69. Correct answer: ­B Asymmetrical IUGR has a lower weight and shorter length compared to head circumference while symmetrical IUGR has a proportional decrease in growth. With symmetrical IUGR, cells have a decreased growth potential or there are fewer cells. Asymmetrical IUGR results in a normal number of cells, but with reduced size. Symmetrical IUGR is associated with congenital infections, malformations, and errors in chromosomes. Asymmetrical IUGR is associated with external factors such as placental function and maternal ­malnutrition. 70. Correct answer: ­B Infants of diabetic mothers may appear to have a smaller head in proportion to the body due to the inability of insulin to cross the blood–brain barrier. Despite the large body, head circumference is ­normal. 71. Correct answer: ­B A neonate at 29 weeks will have poor resting tone with only slight flexion of the knees and ankles. Additional findings include smooth skin with visible veins and abundant lanugo; the soles will be smooth with only faint red marks. The pinna will be slightly curved and soft with slow recoil. The nipples will not have buds and the areolas are flat. Females of this gestation will have prominent clitorises and enlarged minora, and the males will have rare rugae on the scrotum and the testes are noted in the upper ­canal. 72. Correct answer: ­C The infant assessment indicates a neonate who is less than 23 weeks gestation weighing under 400 grams. Per NRP guidelines, this infant is not viable and resuscitation measures should be withheld. Estimated gestational dates provided by the mother may be inaccurate by 6 2 weeks. The findings are more consistent with the ultrasound assessment of 21 weeks. The family conference before delivery provided the opportunity for the practitioners to discuss viability and outcomes with the parents to prepare them for the potential outcomes. Care should now be focused on family grieving and emotional ­support. 73. Correct answer: ­A A 44-week gestation infant would be expected to have leathery and cracked skin. If meconium is present, then the nail beds may be stained. Smooth skin would have visible veins and be expected of a 29-week gestation infant, and rough skin with few veins would be expected in a 34-week gestation ­infant. 74. Correct answer: ­C As gestational age increases, the square window angle of the wrist decreases. To assess the square window, flex the wrist at a right angle toward the ­forearm.

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75. Correct answer: ­A Due to the drying effects of the environment, the soles begin to dry, changing the appearance of creases inconsistent with the gestational age. Gestational assessments should be completed within the first 12 hours after birth to increase ­accuracy. 76. Correct answer: ­B The eyelids will open normally between 26 and 30 weeks gestation. Each day, the eyelids should be assessed visually for the development of a crease between the upper and lower lids. To determine degree of fusion, use slight or gentle traction to the upper and lower lids. The lids should not be forced open because this causes injury to the ­eye. 77. Correct answer: ­A Placental function and inherent maternal resources are critical maternal factors to consider when planning care of the neonate prior to and after delivery. Placental function determines the rate and flow of nutrients and oxygen to the fetus as well as cellular waste products flowing away from the fetus. The placenta also plays a critical role in the physiologic changes within the mother that increase glucose and essential elements to the fetus during development. Maternal resources comprise the mother’s ability to obtain and maintain adequate and appropriate nutrition, cardiovascular and respiratory function in order to transport oxygen, carbohydrates, fats, vitamins, minerals, amino acids, and more to the fetus. If maternal resources are compromised, then fetal health becomes compromised. Additional complications to adequate maternal resources include preexisting medical and mental health conditions, drug ingestion, and ­exercise. 78. Correct answer: ­C Based on the maternal and fetal history and vital signs provided, the resuscitation team should anticipate respiratory distress syndrome. Elevated maternal glucose levels inhibit the normal production of surfactant in the fetus resulting in respiratory distress syndrome. Additional postdelivery complications of a macrosomic infant could include pronounced hypoglycemia due to a fetal hyperinsulinemia, hypocalcemia, hypomagnesemia, and polycythemia resulting in hyperbilirubinemia and ­hypertension. 79. Correct answer: ­C Teen mothers are more likely to become pregnant again within 2 years if not educated regarding contraception and birth control measures. This may include abstinence, oral contraceptives, intrauterine devices, hormone injections, and vaginal contraception. Information should be presented without judgment or pressure, but in conversation regarding future planning. Teens with infants may find it difficult to complete schooling and obtain employment while raising a child. Additional topics and education should include parenting classes, day care options, home health visits, community support groups, and ­follow-­up clinics. It may be easier for the nurse to plan all the ­follow-­up visits, but this does not help the mother in building confidence in becoming a mother or provide information to learn to manage the healthcare system. Teen education should focus on providing information, building confidence and knowledge, and guiding the teen through care. Teaching sessions should be in clear, understandable terminology and should include the mother, father if involved, and grandparents/support ­persons.

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80. Correct answer: ­C Pot (marijuana) produced 20 years ago was more natural and purer with fewer additives. Pot grown and sold in today’s market contains 20 times more ammonia and 5 times more hydrogen cyanide, formaldehyde, and PCP. The goal of today’s brand of pot is to obtain a more profound, faster, and sustained high. Within a few minutes of ingestion or inhalation, the heart rate may increase from 20 to 80 beats per minute above baseline, and memory loss will occur. There will also be failure of the ability to concentrate, confusion, dry mouth, and an increased appetite. The effects may be compounded and varied if other drugs are cut or mixed with the ­marijuana. 81. Correct answer: ­A Energy drinks contain massive amounts of caffeine and a variety of ingredients that will break down into methamphetamines and amphetamines with effects on the fetus similar to fetal alcohol syndrome. Immediate presentation includes small for gestation, dehydration, poor coordination for suck and swallow, hyperactive behavior, and sleep disturbances. ­Long-­term effects include learning disabilities, speech and language delays, poor judgment, and low intelligence ­quotient. 82. Correct answer: ­C The mother’s condition indicates this baby has been exposed to cocaine during the pregnancy and prior to delivery. Cocaine increases the risk of fetal cerebral vascular accident and intraventricular hemorrhage due to the stress of the vaginal delivery. Cocaine use should be considered with any mother presenting with eclampsia without proteinuria. Maternal urine should be tested for benzoylecgonine, which is a metabolite only seen with ­cocaine. 83. Correct answer: ­C The correct definition of addiction is when the user cannot live or function without the use of a substance or drug. Addiction withdrawals can be severe or life threatening. Abuse is when the user uses a substance or drug in an amount or function that was not originally intended. An individual who abuses a drug might not be an addict. For example, an individual who uses Benadryl to fall asleep when no allergy symptoms are present is abusing the drug. An addict is usually an abuser by both function and amount of the substance or drug used. Substances or drugs abused range from the obvious (alcohol, marijuana, oxycodone, heroin, and cocaine), to the unexpected (glue, spray paint, nail polish remover, energy drinks, ibuprofen, and cough syrups). 84. Correct answer: ­A Marijuana is the most common illegal drug in the world. In many countries and some states, medicinal marijuana has been legalized but closely regulated. For more information regarding statistics for a specific area and current ranking, the U.S. government Web sites at www.dea.gov and www.nida.nih.gov are updated frequently. Toluene is the addictive chemical in ­glue. 85. Correct answer: ­B This infant is at risk for fetal alcohol syndrome and the delivery staff should anticipate an infant who is small for gestation with possible microencephaly, facial anomalies

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with broad nasal bridge, cardiac defects, kidney defects, hearing defects, and growth restriction with short long bones. Common facial features include short palpebral fissures, maxillary hypoplasia, long and smooth philtrum (space between the nose and upper lip), and a thin upper lip. Common cardiac defects are ventricular septal defect and atrial septal defect. The most profound effects are l­ong-­term damage to the central nervous system with learning difficulties, memory and judgment failure, irritability, and hyperactivity during infancy and early ­childhood. 86. Correct answer: ­A Although you may ask all of these questions, the first question should be used to determine if Helen was using actual witch hazel for acne or facial cleaning, or if she is using the slang term witch hazel for heroin. Many street drugs may present with innocuous names. Heroin may also be referred to as smack, nose drops (liquid heroin), dragon rock (mixed with cocaine), ­A-­bomb (mixed with marijuana), big H, brown sugar, brown tape, diesel, and old navy. Monitor for additional signs and symptoms of heroin abuse such as venous tracks, bruising, and burnt fingertips from holding a roll lighter to smoke the ­heroin. 87. Correct answer: ­A The infant should be treated for respiratory depression and the airway supported with positive pressure ventilation and intubation if needed. Time will resolve the respiratory depression. The sudden collapse of a young woman at a party without explanation is suspicious for gamma hydroxybutyrate (GHB) or ketamine ingestion. These are known date or party rape drugs. Due to the anesthetic and amnesic effects of the drug, an affected female may be unable to describe events leading up to the collapse. To determine exposure, the drug screen should be repeated, looking specifically for Gamma hydroxybutyrate and/or ­ketamine. 88. Correct answer: ­B A c­ hewed-­up pacifier and maternal presentation of euphoria and confusion should lead the staff to consider ecstasy addiction and abuse. Ecstasy or methylenedioxymethamphetamine (MDMA), causes trismus, or teeth grinding. Users may chew on pacifiers to preserve teeth and hide the grinding from others. Most children using pacifiers will not chew, but suck on ­pacifiers. 89. Correct answer: ­C Intrauterine growth restriction is the most common fetal effect if the mother abuses and is addicted to solvents. Infants may also deliver prematurely, have low birth weights, and have potential acid–base ­imbalances. 90. Correct answer: ­A Methamphetamines have an increased risk for cleft palate/lip, hypoglycemia, seizures, poor sucking, and feeding intolerance. Cardiac rhythm disturbances such as supraventricular tachycardia may occur. In addition, the baby may have hyperthermia, agitation, tremors, intraventricular hemorrhages, and a ­high-­pitched ­cry.

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91. Correct answer: ­A Diphenhydramine (Benadryl) and guaifenesin (Robitussin) both contain antihistamines and have anticholinergic effects. Rosa appears to have overdosed on cough and cold remedies, causing her to become severely dehydrated, hyperthermic, and having hallucinations. The classic presentation of anticholinergic overdose is, “dry as a bone, red as a beet, hot as a pistol, mad as a hatter, and loony as a toon.” Due to hallucinations, mothers who present with anticholinergic overdose must be protected from others and from themselves. They may be very agitated and difficult to treat. Emergency management is with fluids and benzodiazepines. Fetal heart rate may be tachycardic. If the baby is delivered, fluid resuscitation is necessary while monitoring for electrolyte imbalances, seizures, tachycardia, hypertension, and urinary ­retention. 92. Correct answer: ­C Infants of smokers are at higher risk for sudden infant death syndrome (SIDS) with the risk increasing if the parents smoke with the infant in the same room and with the number of cigarettes smoked. Parents should be given literature and referrals for smoking cessation classes and support prior to discharge. If the parents smoked regularly during the pregnancy, additional ­follow-­up teaching should include keeping appointments to monitor growth (due to intrauterine growth restriction), neurologic function, vision and hearing screening due to malformations, and speech therapy if language delays are ­noted. 93. Correct answer: ­A Alcohol withdrawal symptoms may occur within the first 12 hours of life. Presentation includes hypertonia, tremors, feeding intolerance with poor coordination and weak suck, and opisthotonos (severe arching of the back and neck). Comfort measures should include decreased external stimulation with a darkened and quiet room, containment, and grouped ­cares. 94. Correct answer: ­C Modafinil, caffeine, and amphetamines are all classified as stimulants. Other stimulants include cocaine cathinone, ephedrine, phentermine, and theophylline. Effects of marijuana range from stimulation to depression depending upon the amount and frequency used as well as what chemicals are cut or mixed in for smoking. Yellow jackets, methadone, and ketamine are ­depressants. 95. Correct answer: ­B Jill is presenting as a polydrug user without providing any medical history. Polydrug users are at very high risk for sexually transmitted diseases such as syphilis, ­hepatitis B and C, gonorrhea, and chlamydia. It is recommended that rapid screening be done as soon as possible and prophylactic treatments begin prior to delivery. Fetal monitoring is necessary as risk for spontaneous abortion is elevated in this ­high-­risk group. Should the infant deliver, complications include intrauterine growth restriction, low birth weight, hypoxia, withdrawal symptoms, congenital infections, and ­microencephaly.

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96. Correct answer: ­A Paroxetine (Paxil) is a selective serotonin reuptake inhibitor (SSRI) that has been linked to congenital heart defects when more than 25 mg daily is taken during the first trimester. SSRIs also have been linked to respiratory distress, seizure activity, hypoglycemia, omphalocele, and craniosynostosis. Elavil is a tricyclic antidepressant associated with respiratory distress, endocrine and metabolic imbalances, hypoglycemia, temperature instability, and seizures. Social services will need to investigate whether Olga reported to the prescribing physicians that she was pregnant or taking other drugs. ­Follow-­up maternal care should include counseling and close prescription ­management. 97. Correct answer: ­C The best position for an infant who is arching is to use a sling or ­hammock-­style bed to promote flexion. Although swaddling may decrease arching, it is best to avoid placing the infant supine while arching to avoid the head pressing against the mattress and the scapula from tightening. Additional methods this parent can use include hydrotherapy and massage. As arching may occur until age 1 year, it is recommended that this infant not be placed in a walker, as this promotes extension and not ­flexion. 98. Correct answer: ­B HIV poses complex care issues when the mother is considering breastfeeding. At this time, due to transmission risks, breastfeeding is contraindicated for any seropositive HIV mother. Mothers who are at risk, but test HIV seronegative, yet wish to breastfeed must be given information regarding risks and be tested regularly to determine serology status. Although breastfeeding while smoking is discouraged, it does not prohibit breastfeeding. Hepatitis B is not a contraindication for breastfeeding. Hepatitis C is contraindicated for breastfeeding. Mothers who are actively using alcohol, cocaine, amphetamines, marijuana, and heroin should not breastfeed due to risk of transmission via breastmilk to the ­infant. 99. Correct answer: ­C Standards of critical care should focus on both infant and parent response and interaction. Interventions of care should be individualized for each infant–parent group. Interventions are continually adjusted based on ongoing assessment of infant cues and response as well as parental involvement and interaction. Parental support and bonding should be encouraged and facilitated at every ­opportunity. 100. Correct answer: ­A Sighs, periodic breathing, tachypnea, mottling, hiccups, tremors, and straining are all autonomic signs of stress in infants. Fisting, squirming, frantic movements, and extended extremities are all signs of motor stress. Parents should be taught to identify these behavioral cues and interventions to increase comfort, knowledge, and independence in caring for their child. Common interventions include repositioning, hand containment, decreasing identified negative stimulation, and calm ­holding. 101. Correct answer: ­B After your teaching, parents should have the knowledge and confidence to identify and intervene when negative cues are present. Additional teaching is needed if the

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parents only call the nurse’s attention to the behaviors, but fail to become involved. Teaching during kangaroo holding should also include positioning in the flexed position with ­skin-­to-­skin touching from chest to umbilicus to maximize ­benefits. 102. Correct answer: ­B Parental involvement is crucial at every stage of care. The neonatal intensive care setting may be overwhelming and frightening to parents. The nurse needs to offer short bursts of information with appropriate parent intervention to increase parental confidence and knowledge. Detailed and lengthy explanations at the physiologic level may be overwhelming and intimidating to new parents and hinder interaction. Parents can be taught about all stages of sleep and wake cycles during each visit by pointing out both positive and negative behavioral cues when present. Parents should then be taught what actions would assist in establishing stability. While at bedside, encourage and direct parent interactions without doing an action for ­them. 103. Correct answer: ­C Even if the nurse teaches one behavioral cue, the parents will begin to identify and interpret their neonate’s behavioral language. During a neonate’s short stay, the parents must learn their baby’s language in order to reach independent care for discharge. Although this learning can be intimidating, the nurse can explain that staff will teach the parents one “word” or behavior at a time until they are comfortable in reading the baby’s needs. During each parent interaction, engaging the parent to identify neonate behaviors and clarify parent actions increases confidence and ­bonding. 104. Correct answer: ­B It is best to perform cares when Dalton is asleep with core movement of the head and chest. This indicates wakefulness and awareness of his surroundings without stress. The patient is quiet and ready for stimulation without negative effects. Crying indicates profound stress and cares would only increase the amount and severity of the negative stress as seen with increased cortisol levels. When an infant is in deep sleep without movement, brain maturation is occurring. This baby should be left alone until spontaneous movement is noted. Brain maturation, the development and myelination of neurons, during kangaroo holding may last 5 to 45 ­minutes. 105. Correct answer: ­C The best choice for kangaroo care for a hyperthermic neonate is the mother. Maternal breast tissue can thermoregulate by both increasing and decreasing skin temperature. Paternal breast tissue can only increase temperature and would not be preferred when providing kangaroo care for a hyperthermic neonate. Current research supports kangaroo holding whenever possible to increase and sustain stability faster and more efficiently than an isolette or radiant warmer ­use. 106. Correct answer: ­C It is difficult for parents to bond with their very premature and/or very sick children. It is important to involve them in any appropriate care as soon as possible to establish a bond. If Aquaphor has been ordered for skin protection, take the opportunity to demonstrate and support correct application to the parents. This simple task gives the

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parents a tangible opportunity to become an active participant in making their child more comfortable and healthier. Umbilical dressing changes may require additional skills and comfort beyond what a new parent may handle. Head repositioning also requires additional skill to protect the airway of an intubated patient and should be performed by trained personnel during early and unstable ­periods. 107. Correct answer: ­C Due to neonatal instability and/or lack of developmental positioning, common neonatal positioning deformities include neck extension and head molding to maintain and maximize the airway patency. Without positioning devices, gravity exerts resistance greater than muscle strength, causing hips to adduct with external rotation and shoulder extension with scapular adduction. This presents as the classic “frog” appearance. Boundaries should allow some resistance to active extension of limbs, yet provides passive flexion when at ­rest. 108. Correct answer: ­C Developmental positioning should support midline alignment with symmetric positioning of limbs while maintaining an optimal airway. Developmental positioning may not always be comfortable to the neonate and leads to challenges in maintaining symmetry and alignment with hands near the face and legs flexed. Multitudes of positioning devices are available in the market today with a variety of costs. Unit preferences and training should include the correct use of the positioning device to meet midline and symmetric position with the ability to allow for active and passive ­motion. 109. Correct answer: ­A Self-­regulatory actions that may be observed and reported by the parents include grasping of objects with movement toward the face, “praying”-like hand positioning, trunk flexion, bracing a foot or feet against nesting or boundaries, and controlled head position changes. Splayed hands, pushing away movements, arching, aversion, and roving eyes indicate stress and should be addressed by the nurse and/or ­parents. 110. Correct answer: ­C Many interventions in the neonatal unit can be painful, and parents are concerned about narcotic addiction and ­long-­term effects. It is important to address parental concerns and provide alternatives if possible or additional information if applicable. Swaddling, nonnutritive sucking, sucrose pacifiers, nesting, and breastmilk administration in place of sucrose are all nonpharmacologic methods of pain control that can be used during procedures to provide comfort. Stating that failure to provide narcotics is cruel and changing the method of administration does not address parental concerns. The parent may become defensive and close ­communication. 111. Correct answer: ­A Areceli and Quinton should be encouraged to journal Valerie’s progress during her stay in the intensive care unit. As her status improves, the parents and staff should discuss strategies to increase parental involvement and document weekly goals set by both parents and staff. Journaling allows parents to visualize and track progress towards

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discharge and provide opportunities to teach and clarify parent knowledge. Ancillary family should be encouraged to visit with parents especially if those family members will also be participating in home care. Parents are given the chance to teach what they have learned to family members and strengthen confidence and ­independence. 112. Correct answer: ­C Extremely premature or ­low-­birth-­weight neonates may spend weeks or months with negative oral stimulation from oral gastric tubing and endotracheal tubes. To successfully transition to oral feedings, it is important to introduce positive oral stimulation throughout management. Oral care with lemon or vanilla flavor both while intubated and extubated gives positive oral stimulation despite ­life-­sustaining technology. If possible, nasogastric feeding tube placement should be considered for use between oral feedings to decrease negative oral stimulation. Pacifiers should be used for pleasure as well as during procedures to support comfort and suck, swallow, and breathing ­patterns. 113. Correct answer: ­C The greatest indicators of successful transition to oral feedings via breast and/or bottle are determined by the infant’s physiologic stability, ability to maintain a flexed posture during feeding, and the presence of a quiet alert state. Although we might wish it was all nursing or ancillary support that results in successful oral feedings, the major component is the neonate. Staff involvement in feedings comes from our ability to recognize instability, support flexed position, and identify quiet, alert states that impact feeding tolerance. Feeding plans, techniques, and advancement should first evaluate infant cues and then incorporate parent involvement to increase ­long-­term feeding ­success. 114. Correct answer: ­A The appropriate nursing action would be to recommend advancement in feeding to oral feeding 3 2 then gavage feedings (nipple, nipple, gavage). Preston exhibited tolerance to the oral feeding with physiologic stability and stamina to complete the entire feeding in less than 30 minutes despite minimal external pacing initially to establish an effective suck, swallow, and breathing pattern. Allowing Preston to nipple feed back to back will allow for evaluation of stamina and ability to maintain physiologic stability with the added stress of another oral feeding. Initial pacing is expected for premature infants to establish initial suck, swallow, and breathing pattern. Coordination of suck pattern indicates brain development and neurologic growth. Feedings should continue to be monitored for any deviation in physiologic baseline (heart rate, respiratory pattern, and rate and oxygen requirements) during and after feeding. Additional monitoring for weight changes, ability to complete feeding in 30 minutes or less, and muscle tone are also ­necessary. 115. Correct answer: ­B To increase breastfeeding success for the preterm infant, it is important that the mother begin pumping breastmilk to obtain colostrum and establish the milk supply. Success varies from mother to mother, but nursing actions can increase success by providing the mother with literature on proper nutrition, providing a quiet and private location to pump, educating the mother and father regarding proper pumping and

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milk storage, and providing access to lactation consultants for education and support during neonatal stay. Breastfeeding can be effectively established with and without bottle feeding. Teaching infant readiness cues, providing positive oral stimulation, and nonnutritive sucking during development all assist in increasing oral feeding success. Providing specific time frames for breastfeeding prior to physiologic stability and maturity is inadvisable. Time frames set up the staff and mother for failure and disappointment if the infant remains unstable and is not neurologically mature to establish even nonnutritive ­sucking. 116. Correct answer: ­C To support ­long-­term collaborative feeding practices, it is recommended that a core group of staff provide general and feeding care to establish trust and a bond with the parents and patient that provides insight to patient progress and stability. Documentation of feeding stability, cues, tolerance, and techniques should be incorporated into the chart so that all providers have consistent and easy access to feeding plans and progress. Documentation between caregivers should use consistent terminology that is not subjective, is clearly understood, and is supported with objective ­statements. 117. Correct answer: ­C Abuse may be suspected if the mother denies abuse in the presence of bruising, injury to bones and around the throat. Many abused women and children have stories of a positive home life and excuses to explain injuries due to clumsiness. Due to the likely introverted personalities of abused individuals, it is important to establish a safe zone and trust to encourage honest communication and to begin assistance to escape the ­abuse. 118. Correct answer: ­A Women who have intact membranes and schedules for cesarean section do not need prophylactic antibiotics for positive Group ­beta-­strep (GBS). Women with unknown or known positive GBS status with ruptured membranes and prior positive GBS birth should receive prophylactic antibiotic therapy to decrease the risk of transmission to the infant at ­delivery. 119. Correct answer: ­C The neonatal team should be in attendance for any delivery where there is known or suspected maternal blood loss such as with abruption or maternal hemorrhage. The risk of fetal hypovolemic shock may predict poor response to resuscitation measures and the need for volume expanders and hemodynamic support. Drug addiction is not always a requirement for neonatal team attendance unless the substance of abuse may cause respiratory depression and/or seizure activity. Infants at 37-week gestation are often stable and do not require advanced support at delivery unless another risk factor has been ­identified. 120. Correct answer: ­B Any infant experiencing apnea and bradycardia postdelivery should be evaluated with likely admission to the intermediate care nursery for observation and treatment. Transient tachypnea of the newborn, especially without oxygen requirement, should be monitored for at least 4 to 6 hours for additional signs of distress such as grunting,

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nasal flaring, and increasing retractions. Blood glucose of 50 mg/dl in a term infant requires ongoing monitoring in the newborn nursery if signs and symptoms of hypoglycemia develop or if the patient is an infant of a diabetic ­mother. 121. Correct answer: ­A Pain can be observed in the neonate as tachypnea, tachycardia, and crying. Neonatal pain remains controversial, but its importance should not be underestimated when performing painful and invasive procedures. Neurologic research continues to describe the effects of what could be painful stimuli altering normal neurologic development. Comfort measures may include nonpharmacologic as well as pharmacologic measures to reduce neonatal distress and injury as caused by painful ­stimuli. 122. Correct answer: ­C Research reveals that cortical perception of pain is complete before 24-week gestation and should be considered and the neonate should be treated for ­it. 123. Correct answer: ­C You should anticipate morphine to be used as a substitute for maternal heroin addiction and phenobarbital as a substitute for maternal cocaine addiction. During treatment, abstinence scoring may assist in determining and managing withdrawal symptoms and directing ­treatment. 124. Correct answer: ­A Oral morphine doses may be three to five times greater than the intravenous dose as absorption and onset times differ. Ongoing, visual (including via monitor) respiratory assessment should be continuous to evaluate for response to treatment and respiratory ­depression. 125. Correct answer: ­C Many cultures have beliefs and practices that may influence medical management of the neonate in the intensive care setting. The Chinese culture will often use medicinal herbs and religious beliefs regarding internal energy known as a person’s chi to manage health. Cultural beliefs must be acknowledged, regardless of staff beliefs, as influential in patient care. Provide education and explanation if cultural beliefs interfere with or inhibit medical ­treatment. 126. Correct answer: ­C Although the open cranial sutures allow molding to permit passage via the vaginal canal, the shoulders are limited in movement. Large infants with delayed passage through the vaginal canal should be suspected of having shoulder ­dystocia. 127. Correct answer: ­C Breech presentation during a vaginal delivery increases the risk of aspiration, including meconium aspiration if the infant is stressed prior to or during delivery. Likely aspiration occurs once the body is delivered and the infant makes an effort to breathe while its head is still within the vaginal ­canal.

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128. Correct answer: ­C Biparietal diameter is frequently used to establish gestational age between 12 and 20 weeks ­gestation. 129. Correct answer: ­A Neonatal visual acuity is estimated at 20/400 for term infants. It is important to teach parents that due to vision at 20/400, the infant best sees or focuses in on objects that are placed 6–10 inches from the ­face. 130. Correct answer: ­A A flexed posture indicates a healthy, term infant with sufficient oxygen delivery to all organs, including the muscles. A floppy or hypotonic posture may indicate central nervous system depression and/or oxygen ­deprivation. 131. Correct answer: ­C Vernix, or the waxy coating on baby’s skin, will be present in the skin folds at 40 to 41 weeks gestation. Vernix begins to be secreted by the sebaceous glands during the 20th week of ­gestation. 132. Correct answer: ­C Gestational age assessment is primarily utilized to anticipate problems related to gestation and development. Multiple evaluation systems exist, but each is based on both physical presentation and neurologic ­development. 133. Correct answer: ­C External rotation of the hips during prone positioning poses a major developmental complication for the premature neonate. External rotation promotes extensor muscle development over flexor muscle development and may lead to difficulty crawling and walking within the first year of ­life. 134. Correct answer: ­B Papules about 1 mm in diameter around the chin and cheeks may concern parents, but are ­self-­resolving milia found in approximately 40% of term births. The milia are epidermal inclusion cysts of keratin. It is important to teach the parents not to pop or break these cysts, as doing so increases the risk of ­infection. 135. Correct answer: ­A Hypoxic infants will produce lactic acid during cold stress, thus causing metabolic acidosis. The most effective treatment for metabolic acidosis caused by cold stress is prevention. Rapidly establish and maintain a consistent neutral thermal environment with warmed blankets, servocontrolled bed, and ­vigilance. 136. Correct answer: ­A Due to the greater surface to body ratio of infants, heat loss is three to four times greater or faster for infants than for ­adults.

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137. Correct answer: ­B Elevated maternal estrogen levels may result in breast discharge in the infant, both female and male. This condition is ­self-­limiting as estrogen levels fall over ­time. 138. Correct answer: ­B The unpredicted situation, or deviation from expected events, that occurs with delivery of a premature or neonate with congenital anomalies, is the major negative factor in parental stress. Preconceived expectations allow parents to adjust to changes in lifestyle and family dynamics. When anticipated events do not occur, parents are often left without sufficient coping mechanisms and are rapidly overwhelmed by circumstances, creating a crisis. The birth of a new family member and the resulting change in family dynamics are positive stress factors for a new ­parent. 139. Correct answer: ­A With the early and traumatic delivery of a sick and premature neonate, these parents are experiencing the initial response to a crisis. The situation is sudden, unexpected, and involves an environment and circumstances beyond the parent’s experience or ability to cope. The crisis may last up to 6 weeks depending upon available support systems, the healthcare team, parent education, culture, and spiritual intervention. Parents will be more receptive to interventions as they begin to seek methods and new techniques to manage ­stress. 140. Correct answer: ­C Negative relationships may develop at any point during the stay in the intensive care unit. Although it is good that the parents visit, staring at the monitor indicates a failure to adapt to the intensive care environment. In addition, the parents have failed to establish contact or interaction with the infant visually or tactilely. They cannot begin to accept the alteration in circumstances if they cannot accept the infant’s birth. This is a form of ­avoidance. Blaming oneself, or the mother, for an event preceding the delivery is common as parents and family adjust to the fact the neonate has not been born at term. As the nurse, you should acknowledge concerns and fears about causing premature delivery, and then focus on the infant’s presence and progress. Regardless of the cause for premature delivery or anomalies, the parents can be redirected to the infant and the actions they can take to establish a strong and healthy bond with their child. Blessings, baptisms, and last rites are expected methods of preparation for the possible death of the critical neonate by acknowledging death while maintaining an element of ­hope. 141. Correct answer: ­C All parents should be encouraged and offered parenting classes if available. Each age group poses unique challenges to parenting. Do not assume that because of age, one set of parents is more or less prepared than another age group. The same principle should be applied to discharge teaching. All parents should receive complete discharge instruction despite their age or number of previous children as recommendations and practice may have changed or improved since their last child was ­born.

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142. Correct answer: ­B It is important to provide parents with information in lay terms that do not cause panic or increase anxieties. Telling the parents that “An echocardiogram was ordered for today to evaluate blood flow through Evan’s heart” provides the parents with more accurate information than guessing or assuming what the study will find. Parents may not initially understand that the patent ductus arteriosus does not mean a cardiac defect or permanent heart disease. Parents may perceive the worst possible scenario rather than a standard diagnostic ­procedure. Stating that “Evan was really sick this morning, but we took care of it before you got here” may lead the parents to believe that staff are hiding Evan’s condition or are withholding information if the parents believe they are not being notified when he is sick. The more therapeutic statement would be, “We treated Evan with another dose of medicine this morning to improve his ability to breathe and provide oxygen to his tissues.” This provides correct information simply as a standard treatment for premature ­neonates. “We are beginning trophic or enteral gavage feedings so that Evan can wean off TPN and IL within the week” provides too much technical information without explanation. It is best to avoid definitive timelines or statements as outcome is not absolutely known. Parents may focus on time and perceive they are being lied to if the neonate does not accomplish the goal or action in the time stated. Definitive statements may be positive or negative, but should be avoided unless the statement is absolutely ­correct. When speaking with parents, first establish their knowledge base, level of education, and preferred method of communication. This will provide staff with guidelines for providing information. Information should be accurate, timely, and not overly positive or negative. Ensure that parent understanding is correct by asking them to repeat what they heard in their own words. You should then provide corrections, clarification, and/or additional information as needed. Be aware that while under stress, parents may not hear or focus on every element of information and may need information repeated. Encourage parents to journal information and questions for future ­reference. 143. Correct answer: ­A Children under 2 years of age cannot grasp the concept of another baby. It is best to explain to the parents that their 2-­year-­old daughter will not understand that the baby is in the unit, but may have separation anxiety with you visiting for great lengths of time. For children who are 2–6 years old, it is best to keep information simple, to explain that it is nothing they did, and that the staff is helping the baby to get better without going into detail. Older children should be involved in care based on age and ability. Teach them proper ­hand-­washing technique and encourage interaction using ­age-­appropriate terms and ­actions. 144. Correct answer: ­A Staff may be hesitant to interrupt grieving parents, but it is important to tell them you are sorry for their loss and offer to listen or pray with them as appropriate. This acknowledges the death and allows parents the opportunity to talk. Loss is difficult for any parent, but multiple births pose another challenge when some die and others survive. If the surviving neonate(s) are in critical condition, parents may be hesitant to bond for fear of additional loss. Often staff and family members are unsure how to deal with grief and avoid even mentioning the baby’s name. This may give the impression

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to parents that you do not care, have no interest in their grief, are not available as a support, and that may delay the grieving process. Positive interventions include reaffirming that they are the parents of multiples, affirm that the child did exist, ask about emotions, and provide support as needed. You should involve social services, contact clergy if appropriate, and always be honest with your emotions. Encourage involvement with living sibling(s) to support healthy bonding and to affirm ­life. 145. Correct answer: ­B Risk management plays a role in the collection, analysis, and storage of records regarding potential and actual injuries in the hospital, as well as court filings. It also identifies situations or actions of potential liability and analyzes techniques to prevent future injuries, medication errors, and ­accidents. 146. Correct answer: ­A SBAR is an abbreviation for Situation, Background, Assessment, and Recommendation for use when reporting patient care to another healthcare professional. SBAR is used to organize information succinctly to provide rapid report and recommendations for actions to physicians, advanced practice personnel, another nurse, social services, and ancillary departments. Situation refers to what information is most important or critical to patient care at the time. Background provides the necessary history that has brought the patient into the unit. Assessment includes objective and subjective data relevant to patient situation. Recommendations refers to the course of action that the reporting staff member believes should occur to improve patient situation. For example, recommendations for a term neonate with a glucose of 25 mg/dl and emesis would be to provide intravenous glucose and start a maintenance intravenous infusion with hourly glucose until stable.

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Maternal Risk Factors and Delivery Issues R ­ eferences Anand, K. J. S. (2007). Pharmacological approaches to the management of pain in the neonatal intensive care unit. Journal of Perinatology, 27(S1), S4–S11. Anonymous. (2008). In infants, the pain associated with palivizumab injections is more effectively alleviated with lidocaine/prilocaine [EMLA] cream plus nitrous oxide/oxygen. (2008). Paediatrics Today, 19(116), ­4. Anonymous. (2009). EMLA and amethocaine equally successful in children’s cannulation. Nursing Standard, 23(50), 1 ­ 7. Armstrong, S., Wynn, G., & Sandson, N. (2009). Pharmacokinetic drug interactions of synthetic opiate analgesics. Psychosomatics, 50(2), 169–176. Axelin, A., & Salanterä, S. (2008). Ethics in neonatal pain research. Nursing Ethics, 15(4), 492–499. Baird, J., Spiegelman, J., Prianti, R., et al. (2009). Noninvasive ventilation during pediatric interhospital ground transport. Prehospital Emergency Care, 13(2), 198–202. Bak, M., Gökgöz, S., & Ünalp, A. (2009). Neurological manifestations of vitamin B12 deficiency in 15 infants. Journal of Pediatric Neurology, 7(3), 275–278. Bhasin, K., van Nas, A., Martin, L., et al. (2009). Maternal ­low-­protein diet or hypercholesterolemia reduces circulating essential amino acids and leads to intrauterine growth restriction. Diabetes, 58(3), 559–566. Biri, A., Bozkurt, N., Turp, A., et al. (2007). Role of oxidative stress in intrauterine growth restriction. Gynecologic and Obstetric Investigation, 64(4), 187–192. Bozeman, B., Slade, C., & Hirsch, P. (2009). Understanding bureaucracy in health science ethics: Toward a better institutional review board. American Journal of Public Health, 99(9), 1549– 1556. Brodsky, D., & Martin, C. (2003). Neonatology review. Philadelphia, PA: Hanley & ­Belfus. Burge, D. M. (2003). Operating and anaesthetic time required for inguinal hernia repair in the neonate. Pediatric Surgery International, 19(9–10), 646–648. Campbell, D. M., Shah, P. S., Shah, V., & Kelly, E. N. (2006). Nasal continuous positive airway pressure from high flow cannula versus infant flow for preterm infants. Journal of Perinatology, 26(9), 546–549. Chen, T., Jong, Y., Yang, R., & Yang, S. (2009). Massive cerebral air embolism in a preterm with fetal alcohol syndrome. Neurology India, 57(2), 227–228. Cloherty, J. P., Eichenwald, E. C., & Stark, A. R. (2004). Manual of neonatal care (5th ed.). Philadelphia, PA: ­Lippincott. Collier, R. (2009). Squabble over risks of probiotic infant formula. Canadian Medical Association Journal, 181(3/4), E46–E47. Colombini, N., Elias, R., Busuttil, M., et al. (2008). Hospital morphine preparation for abstinence syndrome in newborns exposed to buprenorphine or methadone. Pharmacy World & Science, 30(3), 227–334. Davies, B. W., Fraser, N., Najmaldin, A. S. et al. (2003). A prospective study of neonatal inguinal herniotomy: The problem of the postoperative hydrocele. Pediatric Surgery International, 19(1–2), 68–70. Davies, H., Sadler, R., Bielsa, S., et al. (2009). Clinical impact and reliability of pleural fluid mesothelin in undiagnosed pleural effusions. American Journal of Respiratory and Critical Care Medicine, 180(5), 437–444. de Barros Duarte, L., Dantas Moisés, E., Carvalho, et al. (2009). Distribution of fentanyl in the placental intervillous space and in the different maternal and fetal compartments in term pregnant women. European Journal of Clinical Pharmacology, 65(8), 803–808. De La Fuente, L., Campbell, D.E., Rios, A, et al. (2006). Frequency analysis of air and skin temperature in neonates in ­servo-­controlled incubators. Journal of Perinatology, 26(5), 301–305.

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68  y  Section 3 Mendez, R., Pensado, A., Tellado, M., et al. (2002). Management of massive air leak following intubation injury in a very low birth weight infant. British Journal of Anaesthesia, 88(5), 722–724. Merenstein, G. B., & Gardner, S. L. (2006). Handbook of neonatal intensive care (6th ed.). St. Louis, MO: ­Mosby. Miller, F., Hayeems, R., & Robert, J. (2009). Carrier detection and clinical uncertainty: The case of public health ethics. American Journal of Public Health, 99(8), 1349–1350. Mohamed, I. S. I., Wynn, R. J., Cominsky, K., et al. (2006). White blood cell left shift in a neonate: A case of mistaken identity. Journal of Perinatology, 26(6), 378–380. Murray, K. L., Miller, K. M., & Pearson, D. L. (2007). Neonatal withdrawal syndrome following in utero exposure to paroxetine, clonazepam and olanzapine. Journal of Perinatology, 27(8), 517–518. Philbrick, K. (2009). ­Evidence-­based medical ethics: Cases for ­practice-­based learning. Mayo Clinic Proceedings, 84(8), ­758. Polin, R. A., & Spritzer, A. R. (2007). Fetal & neonatal secrets (2nd ed.). Philadelphia, PA: ­Mosby. Prescribing Reference. (2009, Summer). NPPR: Nurse Practitioner’s Prescribing Reference, 16(2). Rangel, E. (2009). Clinical ethics and the dynamics of group ­decision-­making: Applying the psychological data to decisions made by ethics committees. HEC Forum, 21(2), 207–228. Rennie, J. M., & Roberton, N. R. C. (2002). A manual of neonatal intensive care (4th ed.). New York, NY: A ­ rnold. Roy, S., & Vivero, R. (2007). Bilateral choanal atresia in a newborn. Ear, Nose & Throat Journal, 86(10), 608–609. Saunamäki, T., Jehkonen, M., Huupponen, E., et al. (2009). Visual dysfunction and computational sleep depth changes in obstructive sleep apnea syndrome. Clinical EEG and Neuroscience, 40(3), 162–167. Schuckit, M. (2009). A ­ lcohol-­use disorders. The Lancet, 373(9662), 492–501. Taeusch, H. W., Ballard, R. A., & Gleason, C. A. (2005). Avery’s diseases of the newborn (8th ed.). Philadelphia, PA: ­Elsevier. Tanaka, T., Nakajima, K., Murashima, A., et al. (2009). Safety of neuraminidase inhibitors against novel influenza A (H1N1) in pregnant and breastfeeding women. Canadian Medical Association Journal, 181(1/2), 55–58. Tehrani, F., & Abbasi, S. (2009). Evaluation of a computerized system for mechanical ventilation of infants. Journal of Clinical Monitoring and Computing, 23(2), 93–104. Thompson, J. (2009). Breastfeeding and childhood asthma. Community Practitioner, 82(8), ­40. Toh, S., Mitchell, A., Louik, C., et al. (2009). Selective serotonin reuptake inhibitor use and risk of gestational hypertension. The American Journal of Psychiatry, 166(3), 320–328. van Lieshout, E., de Vos, R., Binnekade, J., et al. (2008). Decision making in interhospital transport of critically ill patients: National questionnaire survey among critical care physicians. Intensive Care Medicine, 34(7), 1269–1273. Verklan, M. T., & Walden, M. (Ed.). (2004). Certification and core review for neonatal intensive care nursing (3rd ed.). St. Louis, MO: E ­ lsevier. Verklan, M. T., & Walden, M. (Ed.). (2010). Certification and core review for neonatal intensive care nursing (4th ed.). St. Louis, MO: ­Elsevier. Vural, M., & Ogel, K. (2006). Dilated cardiomyopathy associated with toluene abuse. Cardiology, 105(3), 158–161. Webercarstens, S., Bercker, S., Hommel, M., et al. (2009). Hypercapnia in ­late-­phase ALI/ARDS: Providing spontaneous breathing using pumpless extracorporeal lung assist. Intensive Care Medicine, 35(6), 1100–1105. Wise, L., Titusernstoff, L., Newcomb, P., et al. (2009). Exposure to breast milk in infancy and risk of breast cancer. Cancer Causes & Control, 20(7), 1083–1090. Young, T. E., & Magnum, B. (2009). Neofax 2009. Montvale, NJ: Thomson ­Reuters.

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Section 4 Cardiovascular ­System

Questions 1. Stroke volume in a term newborn is relatively fixed a ­t A. 1.5 ­ml/kg B. 60 ­ml/min. C. 120–200 ­ml/kg/minute 2. In a premature infant, hypertension is defined ­as A. A systolic blood pressure greater than 90 mm Hg and a diastolic blood pressure greater than 60 mm Hg B. A systolic blood pressure greater than 60 mm Hg and a diastolic blood pressure greater than 40 mm Hg C. A systolic blood pressure greater than 80 mm Hg and a diastolic blood pressure greater than 50 mm Hg 3. You are caring for a term infant and note that the PR interval on the EKG is 0.14 second. This ­measurement A. Is normal for a term ­infant B. Indicates r­ ight-­sided heart ­failure C. Indicates a fi ­ rst-­degree heart b ­ lock 4. A medication utilized to close a patent ductus arteriosus (PDA) ­is A. ­Prostaglandin B. ­Indomethacin C. ­Lysine 5. A ­moderate-­sized ventricular septal defect results ­in A. A ­r ight-­to-­left s­ hunt B. A decrease in pulmonary vascular ­edema C. A l­eft-­to-­r ight ­shunt 6. An atrial septal defect results ­in A. No damage to the ­septum B. A ­left-­to-­r ight shunt with volume ­overload C. A diastolic murmur at the upper sternal ­border

y

y

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70  y  Section 4

7. Tetralogy of Fallot manifests itself by which of the following combinations of ­defects? A. VSD, overriding aorta, pulmonary stenosis, and right ventricular ­hypertrophy B. Aortic stenosis, atrial septal defect, coarctation of the aorta, and ­PDA C. ASD, mitral prolapse, PDA, and pulmonary ­stenosis 8. Tet spells may be treated with which of the following ­medications? A. ­Gentamycin B. ­Digoxin C. ­Morphine 9. The most common causes of CHF in neonates ­are A. Right ventricular ­hypertrophies B. Structural heart ­defects C. Tricuspid a ­ tresias 10. Your patient required placement of a left atrial pressure monitoring line. The pressure reads 14 mm Hg. This value might ­indicate A. Mitral valve ­dysfunction B. Pulmonic ­stenosis C. Tricuspid r­ egurgitation 11. Which of the following best describes the function of the ductus arteriosus in a ­fetus? A. The ductus shunts blood from the inferior vena cava to the pulmonary ­artery B. The ductus provides an opening for blood to be shunted from the pulmonary artery to the ­aorta C. The ductus allows for lower pulmonary pressures in the ­fetus 12. While preparing to give report at shift change, you note a 7-cm diameter pool of blood under your patient. The infant dislodged the umbilical catheter. The infant’s heart rate is 188, skin is pale, and capillary refill is about 3 seconds. A normal saline bolus is ordered for fluid replacement. Which of the following parameters indicates improvement after the saline bolus has ­infused? A. Heart rate stabilized at ­192 B. Warm ­extremities C. Capillary refill 5 ­seconds 13. While assessing the placement of a new umbilical line, you note the umbilical waveform is dampened. Which of the following actions would be most appropriate when troubleshooting this p ­ roblem? A. Flush the line with 0.5 ml of normal ­saline B. Check the tubing for kinks and loose ­connections C. Add one section of extension tubing to improve the ­waveform

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14. A chest radiograph of your preterm infant shows a ­“boot-­shaped” heart. This is indicative ­of A. Tetralogy of ­Fallot B. An atrial septal ­defect C. Trisomy ­13 15. Which of the following medications has some vasodilatory properties, increases the heart rate, and improves ­contractility? A. ­Dopamine B. ­Nitroprusside C. ­Milrinone 16. Which of the following statements about digoxin is ­true? A. Digoxin causes h ­ yperkalemia B. It must be discontinued when the heart rate is below ­150 C. Digoxin causes an increase in toxic effects in premature ­infants 17. The most common cardiac cause of death in the first week of life ­is A. Tetralogy of ­Fallot B. Total Anomalous Pulmonary Venous Return (TAPVR) C. Hypoplastic Left Heart ­Syndrome 18. In neonates with hypoplastic left heart syndrome, nitrogen therapy is often used. The purpose of using nitrogen is t­ o A. Decrease inspired oxygen to , 21% B. Prepare the patient for a Fontan ­procedure C. Keep the ductus arteriosus ­patent 19. Type II truncus arteriosus is characterized ­by A. A longer pulmonary artery arising from the base of a common ­trunk B. The right and left pulmonary arteries having separate ­origins C. The right and left pulmonary arteries are joined at the base of the common ­trunk 20. The most common cardiac cause of cyanosis in neonates ­is A. Transposition of the great ­vessels B. Tricuspid a ­ tresia C. Pulmonary ­stenosis 21. When assessing your s­ ix-­day-­old male infant, you note that the systolic blood pressure is 18 mm Hg higher in the upper extremities than in the lower extremities. As a NICU nurse, you suspect the infant ­has A. A ventricular septal ­defect B. An endocardial cushion ­defect C. A coarctation of the ­aorta

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22. Which of the following congenital heart defects will produce ­cyanosis? A. Atrial septal ­defect B. Tricuspid a ­ tresia C. Ventricular septal ­defect 23. It is possible to increase cardiac output in a neonate by ­increasing A. ­Contractility B. ­A fterload C. Heart ­rate 24. Neonates with cyanotic heart defects are at particular risk ­for A. Intrarenal kidney ­injury B. Congestive heart ­failure C. ­Polycythemia 25. Stroke volume is comprised of which of the following ­factors? A. Blood volume, viscosity, and ­impedance B. Cardiac output, heart rate, and ­compliance C. Contractility, preload, and ­afterload 26. What is the MAP (mean arterial pressure) for a neonate with a blood pressure of 70/45 and a heart rate of ­190? A. ­53 B. ­2.4 C. ­45 27. Normal values for pulmonary artery pressures in a term infant would b ­e A. RAP 7–10 mm Hg LAP 5–7 mm Hg PAOP 4 mm ­HG B. RAP 3 mm Hg LAP 8 mm Hg PAOP 6–10 mm Hg C. RAP 10–20 mm Hg LAP 12–14 mm Hg PAOP 10–14 mm Hg 28. The resistance against which the right ventricle must eject its volume is known ­as A. ­PAOP B. ­SVR C. ­PVR 29. The mean pressure difference in the systemic vascular bed divided by blood flow is known a ­s A. ­SVR B. ­L AP C. ­PVRI 30. Which of the heart valves is most commonly affected by infective ­endocarditis? A. ­Aortic B. ­Pulmonic C. ­Mitral

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31. Which of the following statements is true regarding epicardial pacing wires placed for postoperative cardiac ­management? A. The wires are placed in the right atrium and left ­ventricle B. The wires provide a temporary backup for 3 days ­postoperatively C. Dual chamber pacing is most important after surgical repairs near the conduction ­system 32. You are caring for a term neonate who just returned from surgery for an endo­ cardial cushion defect. The EKG shows changes in lead V5, and this change probably i­ ndicates A. ­High-­amplitude P ­waves B. Septal or lateral wall ­ischemia C. Anterior wall ­infarction 33. Alpha adrenergic effects of norepinephrine ­include A. Increased force of myocardial ­contraction B. Peripheral arteriolar ­vasoconstriction C. Increased AV conduction ­time 34. Fetal cardiac development occurs primarily ­from A. Day 18 to the 12th week of fetal l­ ife B. Day 24 to the 18th week of fetal ­life C. Day 42 to the 21st week of fetal l­ ife 35. Contractility of heart muscle in neonates is positively influenced by which of the following ­conditions? A. ­Hypoxia B. Electrolyte ­disturbances C. Exogenous catecholamine ­release 36. The first heart sound represents which of the following mechanical events in the ­heart? A. Opening of the aortic and tricuspid ­valves B. Closure of the mitral and tricuspid ­valves C. Increased blood flow across the AV ­valves 37. The third heart sound occurs as a result ­of A. Active atrial ­contraction B. Increased blood flow across the AV ­valves C. Closure of the aortic and pulmonic ­valves 38. Which of the following statements is true regarding the fourth heart sound (S4 )? A. S4 occurs just after the first heart ­sound B. The fourth heart sound occurs with ventricular ­contraction C. The fourth heart sound is always p ­ athologic

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39. Normal blood pressure for a healthy term infant ­is A. 56–77 mm Hg/33–50 mm Hg B. 62–86 mm Hg/50–68 mm Hg C. 42–60 mm Hg/38–44 mm Hg 40. To evaluate a possible aortic arch abnormality, blood pressures must be taken i­ n A. Both legs and one ­arm B. One arm and one leg on opposite ­sides C. Both arms and one ­leg 41. On an EKG, ­left-­sided heart failure results ­in A. Tall, peaked P ­waves B. Wide, notched P ­waves C. Changes in ST ­segments 42. Infants with CHF are at high risk during interventional procedures because ­of A. Balloon atrial ­septostomy B. Transcatheter defect ­occlusion C. Contrast ­dye 43. A complication of indomethacin use ­is A. Active ­bleeding B. Transient ­oliguria C. ­T hrombocytopenia 44. Indomethacin needs to be administered over 30–40 minutes ­to A. Minimize adverse effects such as hypoglycemia and platelet ­dysfunction B. Prevent metabolization in the ­liver C. Prevent problems with compatibility with all other ­medications 45. A 4-­day-­old infant has been diagnosed with CHF. Which of the following medications would you anticipate administering to this ­infant? A. ­Hydralazine B. ­Fentanyl C. ­Octreotide 46. Intravenous hydralazine is incompatible ­with A. ­Dobutamine B. ­Heparin C. ­Furosemide 47. Which of the following medications is specific to the treatment of sustained supraventricular ­tachycardia? A. ­Atropine B. ­Adenosine C. ­A miodarone

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48. Which of the following nursing actions is true regarding the administration of ­adenosine? A. Adenosine must be administered over 30 seconds if given ­intravenously B. Adenosine must be diluted with ­D5W C. Do not refrigerate ­adenosine 49. Dopamine is incompatible ­with A. Potassium ­chloride B. ­A miodarone C. ­Insulin 50. If a dopamine infusion infiltrates, the suggested treatment is to inject which of the following medications into the affected ­area? A. ­Atropine B. ­Phentolamine C. ­Lidocaine 51. Adverse effects of epinephrine could ­include A. ­Hyperkalemia B. Intracranial ­hemorrhage C. Increased ­SVR 52. Surgical management of hypoplastic left heart syndrome includes staged procedures. The second stage is a bidirectional Glenn procedure. Which of the ­following statements is true regarding this ­procedure? A. A single right ventricle supplies systemic circulation after the ­procedure B. The ductus is ligated and the pulmonary artery is ­divided C. The superior vena cava is anastomosed to the right pulmonary ­artery 53. A contraindication to the surgical correction for hypoplastic left heart syndrome would ­include A. A previous transcatheter balloon atrial septostomy to decompress the right ­atrium B. Use of inhaled O2 and n ­ itrogen C. Significant tricuspid valve ­dysplasia 54. Which of the following heart defects would be classified as a cyanotic heart ­defect? A. Pulmonary ­atresia B. Aortic valve ­stenosis C. Coarctation of the ­aorta 55. An example of an acyanotic congenital heart defect would ­be A. Tricuspid s­ tenosis B. Mitral ­stenosis C. Pulmonary valve ­stenosis

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56. Your patient is a n ­ ear-­term infant who exhibited worsening perioral and periorbital cyanosis after birth. The infant developed a profound generalized cyanosis after the ductus closed and has been progressively more tachypneic. A pansystolic murmur is auscultated at the left lower sternal border and is accompanied by a thrill and a diastolic rumble. These symptoms are suggestive ­of A. Aortic ­stenosis B. Tetralogy of ­Fallot C. Pulmonary atresia with an intact ventricular ­septum 57. You are caring for a 1-­day-­old term infant who has been diagnosed with pulmonary atresia with an intact ventricular septum. An inotropic medication often used for this condition ­is A. ­Furosemide B. ­Digoxin C. ­A miodarone 58. As the NICU nurse, it is important to educate the parents/caregivers of your patients about congenital heart defects. An important topic for this condition would ­be A. The cardiac surgeon’s office ­number B. Genetic counseling for future ­pregnancies C. Family history of cyanotic heart ­defects 59. The most important noncardiac problem in the differential diagnosis of neonates with truncus arteriosus ­is A. ­Sepsis B. ­A nemia C. Coarctation of the ­aorta 60. Which of the following statements is true about truncus ­arteriosus? A. Cyanosis is always ­present B. Systemic oxyhemoglobin saturation is usually below 60% C. Patients usually have decreased systemic arterial oxygen ­saturation 61. The type of heart murmur commonly heard in patients with tricuspid atresia ­is A. A diastolic ­murmur B. A systolic ­murmur C. A holosystolic ­murmur 62. A patient with tricuspid atresia may ­exhibit A. ­Polycythemia B. A smaller than normal ­heart C. Sinus t­ achycardia 63. Cardiac catheterization in infants is used primarily to ­determine A. The extent of a ventricular septal ­defect B. The source of pulmonary blood ­flow C. Mitral valve ­competency

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64. Ebstein’s anomaly has been associated with maternal use ­of A. ­T halidomide B. ­Lithium C. ­Cigarettes 65. ­Twelve-­lead EKG findings for a patient with Ebstein’s anomaly could ­include A. Sinus rhythm with paroxysmal ­SVT B. Wenckebach ­phenomenon C. Junctional ­tachycardia 66. Cardiac glycosides are often used in the treatment of Ebstein’s anomaly. A major effect of this classification of medications ­is A. ­Inotropism B. Positive ­chronotropism C. Its usefulness as a ventricular ­antiarrhythmic 67. A definitive diagnosis of myocarditis can be made ­via A. An endomyocardial ­biopsy B. Transesophageal ­ultrasound C. Transmural ­catheterization 68. Which of the heart valves is most rarely affected by infective ­endocarditis? A. ­Aortic B. ­Pulmonic C. ­Mitral 69. Which drug listed below has a high iodine ­content? A. ­A miodarone B. ­Lidocaine C. ­A minophylline 70. An infant was born at 35-weeks gestation via cesarean section because of persistent fetal tachycardia. At day two of life, severe tachypnea developed. Blood cultures were obtained and all labs were normal. That evening, the infant became apneic and bradycardic. Antibiotics were started. The next morning, the infant developed a fever, DIC, jaundice, slight hepatomegaly, and hepatitis. Labs showed high numbers of bands and an elevated platelet count. A diagnosis of myocarditis was made. A likely causative agent for this condition ­is A. Group B ­Streptococcus B. Escherichia c­ oli C. Coxsackie B1 ­virus 71. Right ventricular afterload may be reduced ­by A. Administration of ­epinephrine B. ­Hypoventilation C. Inhaled nitric ­oxide

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78  y  Section 4

72. Which of the following conditions is likely to predispose an infant to hypovolemic ­shock? A. ­Myelomeningocele B. Cardiac ­arrhythmias C. Congenital heart ­lesions 73. Which of the following statements is true regarding hypovolemic shock in the ­neonate? A. Tachycardia with activity is the first ­sign B. Blood pressure may be ­normal C. Mottling and weak, thready pulses are late ­signs 74. A possible cause of distributive shock in a newborn would ­be A. A cardiac ­lesion B. ­Endotoxins C. ­Hypoxemia 75. Mast cell degranulation with resultant histamine release and vasodilation would be an appropriate definition ­of A. Septic ­shock B. A pleural ­effusion C. ­A naphylaxis 76. An example of obstructive shock in the neonate would ­be A. Coarctation of the ­aorta B. A ­gram-­negative ­sepsis C. Decreased ­PVR 77. Systemic inflammatory response syndrome (SIRS) can best be defined ­as A. Sepsis with cardiovascular ­failure B. Sepsis with accompanying organ ­failure C. Tachycardia or tachypnea with a ­fever 78. A female infant weighing 907 g was delivered by emergency cesarean section at 28 weeks after complete placental abruption. At birth, there was absent heartbeat, and the infant required immediate intubation and cardiopulmonary resuscitation. Cord pH was 6.9, bicarbonate was 17.6, and base excess was 222. Intratracheal epinephrine was given, and the infant responded. Her Apgar scores were 1, 5, and 8 at 1, 5, and 10 minutes, respectively. The infant received surfactant and was placed on mechanical ventilation for respiratory distress syndrome. An umbilical venous catheter and an arterial catheter were placed. After resuscitation, the infant is likely to develop which of the following acid–base ­abnormalities? A. Respiratory ­alkalosis B. Metabolic ­acidosis C. Respiratory ­acidosis

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79. An infant of extremely low birth weight (ELBW) may require indwelling catheters for continuous monitoring, infusion of normal saline, blood products, and total parenteral nutrition. This infant is at risk of developing thrombi a ­ nd A. Pericardial ­tamponade B. Fluid ­overload C. ­Endocarditis 80. Lupus may cause cardiomyopathy ­and A. Congenital heart ­block B. Blisters on ­skin C. Weight ­gain 81. Which of the following medications should be monitored for cyanide toxicity and avoided in patients with renal ­problems? A. ­Naloxone B. Sodium ­nitroprusside C. ­Isoproterenol 82. Which of the following medications is incompatible with sodium nitroprusside at the terminal injection ­site? A. ­Insulin B. Potassium ­chloride C. ­A miodarone 83. Your patient has had recurrent episodes of supraventricular tachycardia, which are refractory to adenosine and vagal maneuvers. Another drug you might consider in the course of treatment for this infant would ­be A. ­A miodarone B. ­Digoxin C. ­Procainamide 84. Adverse effects of procainamide ­include A. Severe ­hypotension B. Junctional ­tachycardia C. ­A systole 85. Lidocaine may cause which of the following adverse ­effects? A. Premature ventricular ­complexes B. CNS t­ oxicity C. Ventricular ­tachycardia 86. Which of the following conditions might result if an infant suffers a severe deceleration during l­ abor? A. ­A nemia B. ­PPHN C. A myocardial ­infarction

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80  y  Section 4

87. The shunt that permits most of the blood from the placenta to bypass the liver and then enter the vena cava is ­the A. Foramen ­ovale B. Ductus ­venosus C. Umbilical ­vein 88. The opening in the septum between the atria that allows a portion of blood to flow from the right atrium directly to the left atrium is t­ he A. Foramen ­ovale B. Umbilical ­artery C. Ductus ­venosus 89. In fetal circulation, blood leaving the left atrium goes through the left ventricle, then travels to the head and neck ­via A. The ascending ­aorta B. The superior vena ­cava C. The carotid ­artery 90. In fetal circulation, oxygen diffuses from maternal uterine arteries in the placenta. From the placenta, the oxygenated blood flows through which of the following vessels to the ­fetus? A. The superior vena ­cava B. Ductus v ­ enosus C. The umbilical ­vein 91. Fetal circulation divides at the liver. About half the blood enters the liver and the other half of the blood bypasses the liver through ­the A. Portal ­vein B. Umbilical ­vein C. Ductus v ­ enosus 92. In fetal circulation, blood from which of the following structures enters the inferior vena ­cava? A. Ductus v ­ enosus B. Superior vena ­cava C. The right ­atrium 93. When a newborn takes the first breath and the umbilical cord is occluded, the newborn’s systemic vascular r­ esistance A. ­Lowers B. Becomes ­elevated C. Remains the ­same

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94. Immediately after birth, the right ventricular output flows through the lungs and results in higher pressures in the left atrium. This increased pressure, along with increased systemic vascular resistance, functionally closes ­the A. Access to the aortic ­arch B. Foramen ­ovale C. Umbilical ­vein 95. After a normal term birth, the ductus arteriosus closes ­after A. 1 ­hour B. 1 to 2 ­days C. 15 to 24 ­hours 96. Cessation of flow through a structure caused by changes in pressure is ­called A. ­Obliteration B. Functional ­closure C. A ­coarctation 97. After a normal birth, pulmonary artery pressure remains high for several hours. When the pulmonary vascular resistance lessens, the direction of blood flow through the ductus arteriosus reverses. The direction of the blood flow at this time would ­be A. Bi-­directional B. Right to ­left C. Left to ­r ight 98. Obliteration of a structure by growth or constriction is c ­ alled A. A ­coarctation B. An anatomic ­closure C. An o ­ cclusion 99. A condition that would cause transient opening of a fetal shunt after birth would ­include A. ­Hypoxia B. Persistent pulmonary hypertension of the ­newborn C. ­Hypovolemia 100. Which of the following statements is true about events that occur during a normal cardiac ­cycle? A. Diastole comprises about 30% of the cardiac ­c ycle B. Metabolism of the heart is decreased during ­diastole C. An increase in cardiac output increases ­diastole 101. The average newborn’s cardiac cycle is about second for diastole and second for ­systole. A. 0.20; 0.05; ­0.15 B. 0.08; 0.06; ­0.02 C. 0.4; 0.2; ­0.2

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second, with about

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82  y  Section 4

102. The mechanism by which a newborn can respond to increased volume ­is A. Baroreceptor ­stretch B. Increased heart r­ ate C. Decreased pulmonary vascular ­resistance 103. Stimulation of the right vagus nerve stimulates t­ he A. Sinoatrial (SA) ­node B. Chemoreceptors in the carotid ­arch C. Acetylcholine ­reabsorption 104. If a beta2 receptor in the heart is stimulated, it may ­cause A. A reflex ­bradycardia B. Increased ­SVR C. ­Bronchodilation 105. Early signs of left ventricular failure a ­ re A. Central cyanosis and paradoxical ­respirations B. Tachypnea and ­tachycardia C. Dyspnea and profuse s­ weating 106. Cyanosis of the skin, mucus membranes, and nail beds can occur when deoxygenated hemoglobin is present within the circulation. This cyanosis may be observed when which of the following levels of deoxygenated hemoglobin is ­reached? A. 15 g/250 ­ml B. 50 g/500 ­ml C. 5 g/100 ­ml 107. Infants must be observed constantly for color changes. Prolonged physiologic jaundice may be indicative ­of A. ­Polycythemia B. Congenital ­hypothyroidism C. ­Dehydration 108. The presence of sweating in the newborn is likely due ­to A. Chronic heart ­disease B. Bronchial ­constriction C. ­A nemia 109. During assessment of your infant, you palpate the precordium for the PMI (point of maximal impulse), thrills, and hyperactivity. You note precordial bulging. This finding is probably due t­ o A. Chronic cardiac ­enlargement B. Acute onset cardiac ­dysfunction C. Pectus ­excavatum

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110. During your assessment of a newborn, you note that the right brachial pulse is stronger than the left brachial pulse. This finding is most likely due ­to A. A cardiac ­tamponade B. A thoracic ­aneurysm C. Supravalvular aortic ­stenosis 111. A hyperactive precordium usually ­indicates A. The PMI has moved to the right sternal ­border B. A heart defect with increased ­volume C. A ­pneumothorax 112. Newborns usually demonstrate right ventricular dominance. The location of the PMI in this case would b ­e A. At the right lower sternal ­border B. At the left lower sternal ­border C. At the second right intercostal ­space 113. You note a thrill at the upper left sternal border. This location is useful in assessing problems in the area of t­ he A. Pulmonary ­artery B. Aortic ­valve C. Mitral ­valve 114. The S2 heart sound is created ­by A. Closure of the pulmonic and tricuspid ­valves B. Closure of the pulmonic and aortic ­valves C. Fluid ­overload 115. Regurgitation systolic murmurs are associated w ­ ith A. Aortic ­stenosis B. Pulmonic valve ­disease C. ­VSD 116. The murmur of aortic regurgitation is best heard a ­t A. The third left intercostal ­space B. The second intercostal space on the ­r ight C. The fourth intercostal space on the ­r ight 117. The most commonly heard continuous murmur in a newborn is the murmur ­from A. The closure of the pulmonic ­valve B. Closure of the tricuspid ­valve C. The patent ductus ­arteriosus 118. What type of murmur is commonly found in ­low-­birth-­weight i­ nfants? A. A ­mid-­diastolic ­murmur B. A pulmonary ­murmur C. A harsh holosystolic ­murmur

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119. Cyanosis in the extremities is known ­as A. Methemoglobin ­c yanosis B. Teratogenic ­c yanosis C. ­Acrocyanosis 120. In patients with normal cardiac anatomy, right atrial pressure e ­ quals A. Right ventricular ­end-­diastolic ­pressure B. Left atrial ­pressure C. Right ventricular systolic ­pressure 121. A pulmonary artery catheter may be placed in a neonate’s right ventricular outflow tract to ­evaluate A. ­Left-­to-­r ight ­shunts B. Pulmonary ­hypertension C. Oxygen ­delivery 122. Your patient has an arterial line and is being mechanically ventilated. You note very pronounced phasic variations in the arterial line and suspect ­that A. The ETT tube is becoming ­dislodged B. The patient has tricuspid ­regurgitation C. The patient is suffering from heart ­failure 123. In patients with normal cardiac anatomy, pulmonary artery wedge pressure ­equals A. Central venous ­pressure B. Aortic ­stenosis C. Pulmonary artery occlusion ­pressure 124. Your patient returned from cardiac surgery for a mitral valve dysfunction 2 hours ago. A pulmonary artery catheter was placed during surgery. Initial pulmonary artery wedge pressures have been holding at 8–12 mm Hg. Over the past 15 minutes, the PAOP acutely rose to 24–26 mm Hg. This rise in pressure i­ ndicates A. A tension ­pneumothorax B. ­Post-­pericardial ­effusions C. Impending ­r ight-­heart f­ailure 125. The chest radiograph of your patient shows an “egg on a string” appearance with mild cardiomegaly. These findings are indicative ­of A. Decreased pulmonary ­vascularity B. ­Mesocardia C. Transposition of the great ­vessels 126. The chest radiograph for a 4-day-­old infant shows enlargement of the right atrium, right ventricle, and pulmonary artery. The heart also exhibits a “snowman” appearance. These findings are indicative ­of A. Total anomalous pulmonary venous ­return B. Aspiration ­pneumonitis C. An atrial septal ­defect

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127. The type of total anomalous pulmonary venous return that exists when pulmonary veins attach directly to the coronary sinus and drain into the right atrium is known a ­s A. ­Supracardiac B. ­Cardiac C. ­Infracardiac 128. Total anomalous pulmonary venous return can be classified as nonobstructed or obstructed. A characteristic of the obstructed form ­is A. Respiratory distress that may not respond to mechanical ­ventilation B. A ­left-­to-­r ight shunt across the atrial ­septum C. Mild ­c yanosis 129. AV canal defects are also known ­as A. Endocardial cushion ­defects B. Ventricular septal ­defects C. Ebstein’s ­anomaly 130. A type of endocardial cushion defect in the atrial septum at the level of the tricuspid and mitral valves is known ­as A. Valvular ­atresia B. Ostium ­imperfecta C. An ostium ­primum 131. Pressures in the left side of the heart and pulmonary filling pressures are represented by t­ he A. ­CI B. ­PAD C. ­PAOP 132. Actions of beta blockers ­include A. Increased myocardial oxygen ­demand B. Increased heart r­ ate C. Increased diastolic filling ­time 133. Alpha adrenergic effects of norepinephrine ­include A. Increased force of myocardial ­contraction B. Increased SA node ­firing C. Peripheral arteriolar ­vasoconstriction 134. The mother of your patient visits at bath time. While she is watching you bathe her son, she asks you if there is any suggestion you may have about her weight control. When preparing to teach this mother about goals for her weight control, you suggest her BMI should be assessed. The patient’s mother states she is 30 years old. Her BMI should be ­between A. 12.6 and 1 ­ 5.0 B. 11.2 and ­15.8 C. 19.0 and ­24.9

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135. Which of the following statements is true about ­lidocaine? A. Lidocaine causes ­hypotension B. Lidocaine may cause a moderate gastrointestinal ­intolerance C. Lidocaine does not result in impairment of normal ­contractility 136. Calcium channel blockers act primarily ­on A. Reduction of cardiac ­output B. Arteries to ­arterioles C. Lung receptors ­only 137. The type of echocardiography that shows the quantity of flow across an obstruction is ­called A. ­Two-­dimensional ­echocardiography B. ­Continuous-­wave Doppler ­echocardiography C. ­M-­mode ­echocardiography 138. The type of echocardiography used to evaluate the motion of the cardiac valves and detect pericardial fluid is ­echocardiography. A. M-­mode B. Two-­dimensional C. ­Contrast 139. The type of echocardiography used to detect a PDA or ventricular dysfunction is known as ­echocardiography. A. Two-­dimensional B. ­Contrast C. ­Color-­flow ­Doppler 140. Which of the following statements is true regarding the use of ­color-­flow Doppler ­echocardiography? A. ­Color-­flow Doppler echocardiography does not detect ­shunts B. ­Color-­flow Doppler echocardiography is a replacement for cardiac ­catheterization C. ­Color-­flow Doppler echocardiography detects patterns of blood ­flow 141. A complication of h ­ igh-­contrast mediums used for cardiac catheterizations ­is A. ­Polycythemia B. High sodium ­content C. ­Hypoglycemia 142. A contraindication for the use of indomethacin to close a PDA in a premature infant would ­be A. Necrotizing ­enterocolitis B. A BUN , 30 ­mg/dl C. A serum creatinine clearance of . 1.8 ­mg/dl

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143. If aortic stenosis is severe in utero, blood flow through the ventricle is decreased. This will result ­in A. Left ventricular ­hypoplasia B. ­R ight-­sided heart ­syndrome C. A systolic ­thrill 144. A complete obstruction of the pulmonic valve resulting in a hypoplastic right ventricle and tricuspid valve is the definition ­of A. Persistent pulmonary ­hypertension B. Pulmonary ­atresia C. Pulmonary ­stenosis 145. CHF is often caused by premature ductus arteriosus closing. This premature closing may be caused ­by A. Endogenous ­epinephrine B. Maternal use of ­ibuprofen C. Human growth ­hormone 146. Diuretics are used in the treatment of CHF. A diuretic that reduces urinary calcium losses ­is A. ­Spironolactone B. ­Chlorothiazide C. ­Furosemide 147. An S2 ­fixed-­split heart murmur requires what level of m ­ onitoring? A. ­Normal B. ­A lert C. ­A larm 148. Early signs of left ventricular failure a ­ re A. Normal heart rate with ­fever B. Tachypnea and ­tachycardia C. Blood pressure . 50% of ­baseline 149. Indomethacin should be given to close a ­PDA A. If given to a newborn , 20 days of l­ ife B. At a dose rate of 0.3 mg/kg IV 12 hours 3 2 ­doses C. While closely monitoring BUN and creatinine levels due to ­nephrotoxicity 150. Which of the following is an end effect of ­CHF? A. Systemic venous ­engorgement B. Increased renal ­perfusion C. Pulmonary venous ­shrinkage

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151. Your patient has been diagnosed with an AV canal defect. Defects closely associated with this condition would ­be A. Ostium primum ASD, PDA, a cleft in the anterior mitral valve leaflet, and a VSD in the inlet portion of the ventricle ­septum B. PDA, a cleft in the anterior mitral valve leaflet, pulmonary stenosis, and a cleft in the septal leaflet of the tricuspid ­valve C. Ostium primum ASD, a cleft in the anterior mitral valve leaflet, a VSD in the inlet portion of the ventricle septum, and a cleft in the septal leaflet of the tricuspid ­valve 152. Hypoplastic left heart syndrome consists of a group of cardiac defects, ­including A. A small aorta, aortic and mitral valve stenosis or atresia, and a small left atrium and ­ventricle B. A large aorta, pulmonary valve stenosis, and a large right ­atrium C. A normal aorta, mitral valve stenosis, and a large left ­ventricle 153. You are attending a delivery of an infant with prolonged fetal supraventricular tachycardia unresponsive to maternal digitalis administration. You know the baby is at greatest risk for which of the following conditions because of the sustained ­SVT? A. Polyhydramnios and heart ­failure B. Polyhydramnios and ­PDA C. Heart failure and hydrops ­fetalis 154. Your patient has severe hypertension. The doctor has ordered labetalol 3 mg/kg IV bolus every 6 hours. You ­should A. Not give this drug because it is never given to a ­newborn B. Call the doctor and ask to change the frequency to every 8 ­hours C. Call the doctor and ask to change the dosage to 0.20–1 ­mg/kg 155. In fetal circulation, blood entering the right atrium from the inferior vena cava is shunted across the foramen ovale to the left a ­ trium A. To oxygenate the lower ­extremities B. To shunt deoxygenated blood to the placental ­circulation C. To provide oxygenated blood to the coronary, carotid, and subclavian ­arteries 156. After the infant successfully transitions from fetal to neonatal circulation, which chamber of the heart pumps against the highest ­pressure? A. Right ­ventricle B. Left ­atrium C. Left ­ventricle 157. Increased pulmonary blood flow is associated with which of the following cyanotic congenital heart ­defects? A. Pulmonary ­atresia B. Tetralogy of ­Fallot C. Transposition of the great ­vessels

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158. Decreased pulmonary blood flow in cyanotic congenital heart defects may be associated ­with A. Pulmonary ­edema B. ­Polycythemia C. ­Pneumonia 159. Dobutamine improves cardiac output primarily ­by A. Decreasing preload B. Increasing heart r­ ate C. Improving ­contractility 160. An increase in the fetal heart rate baseline is likely due ­to A. Intact amniotic ­membranes B. Maternal ­fever C. Terbutaline ­tocolysis 161. A nearly flat baseline on a fetal monitor might ­indicate A. Fetal ­compromise B. Heart rate ­acceleration C. Respiratory rate ­deceleration 162. An infant in atrial flutter has been receiving digoxin for 3 days and is now showing a first degree heart block. This is likely due t­ o A. ­Hypokalemia B. ­Hypocalcemia C. ­Hypermagnesemia 163. Your patient has been scheduled for a ­Blalock-­Taussig shunt. This procedure will ­connect A. The superior vena cava and pulmonary ­artery B. The subclavian and pulmonary ­arteries C. The aorta and pulmonary ­artery 164. Cyanosis usually occurs ­with A. Atrial septal ­defect B. Patent ductus ­arteriosus C. Tricuspid a ­ tresia 165. Your 1-­week-­old patient is showing a heart rate of 282 with narrow QRS complexes on her EKG. You would anticipate administration ­of A. ­A miodarone B. ­Adenosine C. ­Epinephrine

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166. R ­ ight-­sided congestive heart failure signs and symptoms ­include A. Respiratory distress and ­hepatomegaly B. Rapid capillary refill and absent jugular venous ­distention C. Weak femoral pulses and abdominal ­tenderness 167. If an umbilical line is positioned in the inferior vena cava, just above the diaphragm, it can ­measure A. Arterial ­pressure B. Central venous ­pressure C. Pulmonary artery ­pressure 168. After insertion of an umbilical catheter 45 minutes ago, you notice a bluish color on the toes of the patient’s right foot. You first action should be t­ o A. Do ­nothing B. Administer ­oxygen C. Apply a warm compress to the ­foot 169. Indications for use of extracorporeal membrane oxygenation ­include A. Transient tachypnea of the ­newborn B. Persistent pulmonary hypertension of the ­newborn C. Bronchopulmonary ­dysplasia 170. Exclusion criteria for the use of ECMO for respiratory failure include which of the ­following? A. ­Sepsis B. Gestational age less than 34-­weeks C. Birth weight greater than 2500 ­grams

This concludes the Cardiovascular System ­questions.

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Cardiovascular System ­Answers 1. Correct answer: ­A Stroke volume is the amount of blood ejected from the left ventricle with each heartbeat. Stroke volume is affected by preload, contractility, and afterload and is relatively fixed at 1.5 ml/g. Normal term heart rate is between 120 and 160. Cardiac output is the amount of blood ejected from the heart in 1 minute and is approximately ­120–200 ml/kg/minute for a normal term infant. CO 5 HR 3 ­SV. 2. Correct answer: ­C Answer C, a systolic blood pressure greater than 80 mm Hg and a diastolic blood pressure greater than 50 mm Hg, is the accepted definition for hypertension in a premature infant. Answer A is the accepted definition of hypertension in a term ­infant. 3. Correct answer: ­C The PR interval on the EKG is 0.14 second and indicates a ­first-­degree heart block. The PR interval for a normal term infant is 0.09 to 0.12 seconds. A ­first-­degree heart block in a term infant is usually benign. ­R ight-­sided heart failure usually results in tall, peaked P w ­ aves. 4. Correct answer: ­B Indomethacin is a medication utilized to close a patent ductus arteriosus (PDA). The ductus arteriosus is a normal connection between the pulmonary artery and aorta; it is necessary for proper fetal circulation. At birth, the rise in PaO2 and decline in prostaglandin concentration cause closure of the ductus arteriosus, usually within the first 10 to 15 hours of ­life. Patent ductus arteriosus (PDA) is a persistence of the fetal connection (ductus arteriosus) between the aorta and pulmonary artery after birth, resulting in a ­left-­to-­right shunt. Symptoms may include failure to thrive, poor feeding, tachycardia, and tachypnea. A continuous ­machine-­like murmur in the upper left sternal border is common. Diagnosis is by echocardiography. It is very important that you remember that the PDA results in a ­left-­to-­right shunt and a ­“machine-­like” murmur is heard. Administration of indomethacin (a prostaglandin inhibitor), with or without fluid restriction may be used in premature infants with a significant shunt but not in term infants with PDA. If the connection persists, surgical or ­catheter-­based correction is indicated. Endocarditis prophylaxis is recommended before and for 6 to 12 months following a ­correction. 5. Correct answer: ­C A moderate-­sized ventricular septal defect causes the PVR (pulmonary vascular resistance) to be less than the SVR (systemic vascular resistance). This will cause a ­left-­to-­r ight shunt. Too much blood can enter the lungs, increasing edema and possibly preventing or delaying development and maturation of arterioles. A pansystolic murmur can be heard over the left sternal ­border. 6. Correct answer: ­B An atrial septal defect results in a ­left-­to-­r ight shunt with volume overload. At birth, a very small ­r ight-­to-­left shunt may occur because right ventricular pressure exceeds

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left ventricular pressure, or there may be no shunt. However, as the PVR decreases soon after birth, a ­left-­to-­r ight shunt develops, and volume overload occurs in the right ventricle with resultant ­hypertrophy. 7. Correct answer: ­A Tetralogy of Fallot results in low oxygenation of blood due to the mixing of oxygenated and deoxygenated blood in the left ventricle via the VSD and mixing of blood from both ventricles through the aorta because of the obstruction to flow through the pulmonary valve. This causes a ­r ight-­to-­left shunt. The primary symptom is low blood oxygen saturation with or without cyanosis from birth or developing in the first year of life. If the baby is not cyanotic, then it is sometimes referred to as a “pink tet”. Other symptoms include a harsh grade II to IV systolic murmur with a thrill, difficulty in feeding, failure to gain weight, and retarded growth and physical development. Polycythemia may be present with dyspnea on exertion, and clubbing of the fingers and toes. Children with Tetralogy of Fallot may exhibit “tet spells.” The precise mechanism of these episodes is unknown, but may result from a transient increase in resistance to blood flow to the lungs with increased flow of desaturated blood to the body. Tet spells may be precipitated by activity and are characterized by paroxysms of hyperpnea, irritability, and prolonged crying, increasing cyanosis, and decreasing intensity of the heart murmur, and may result in hypoxic brain injury and death. Older children may squat during a tet spell, which cuts off circulation to the legs, raises intrathoracic pressure and systemic vascular resistance, and therefore improves blood flow to the brain and vital ­organs. 8. Correct answer: ­C Tet spells may be treated with morphine to promote venous dilation. The dose is usually 0.1 to 0.2 mg/kg IM. IV fluids are used for volume expansion with an increase in systemic BP. If this does not control the spell, systemic BP can be increased with phenylephrine or ketamine (this has the added benefit of being sedating). Propranolol may prevent or mitigate ­spells. 9. Correct answer: ­B The most common causes of CHF in neonates are structural heart defects. Some of these defects include an absent pulmonary valve, premature PDA closure, and AV malformations. Other causes may include arrhythmias, sepsis, volume overload, birth asphyxia, and severe ­anemia. 10. Correct answer: ­A The mitral valve dysfunction is often seen with a postendocardial cushion repair. The LAP is usually around 8 mm Hg, so 14 mm Hg is high. The right atrial pressures would be increased by pulmonic stenosis or tricuspid ­regurgitation. 11. Correct answer: ­B The ductus arteriosus is an opening between the aorta and the pulmonary artery. In a fetus, higher pressures exist in the pulmonary area, so blood flows from the pulmonary artery to the aorta via the ­ductus.

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12. Correct answer: ­B Improved circulation is evidenced by warm extremities. A faster heart rate and extended capillary refill are not indicators of ­improvement. 13. Correct answer: ­B If the umbilical waveform is dampened, check the tubing for kinks or loose connections. In addition, troubleshooting the line should include assessing for clots in the tubing and making certain the tip of the catheter is not against the vessel wall. The flush is not appropriate, and adding extension tubing only diminishes the waveform ­f urther. 14. Correct answer: ­A The ­“boot-­shaped” heart is a classic presentation on an ­X-­ray for Tetralogy of Fallot. The heart is usually normal in size, but the right ventricle is hypertrophied. In an anterior–posterior view of the heart, the apex of the heart is upturned slightly, thus giving the appearance of a ­boot. 15. Correct answer: ­C Milrinone has vasodilatory properties, increases the heart rate, and improves contractility. Milrinone also does not affect platelet function. Dopamine has a potential effect of increasing pulmonary vascular resistance. Nitroprusside is a venodilator and reduces ­afterload. 16. Correct answer: ­C Digoxin has a longer serum ­half-­life in preterm infants (between 61 and 170 hours). In the term infant, the ­half-­life is between 18 and 45 hours. Digoxin should be discontinued if the heart rate is , 100 bpm. Hypokalemia is a danger because the effects of digoxin are enhanced. Hyperkalemia is not caused by ­digoxin. 17. Correct answer: ­C The most common cardiac cause of death in the first week of life is hypoplastic left heart syndrome (HLHS). Hypoplastic left heart syndrome accounts for 1–4% of all cardiac defects. Neonates can appear stable at birth, but when the ductus closes, they deteriorate rapidly. CHF is usually the primary problem, and then, because of poor systemic perfusion, shock and acidosis ­develop. 18. Correct answer: ­A Most patients with hypoplastic left heart syndrome have mitral atresia, aortic atresia, or both. PGE1 is used to keep the ductus open initially. After a few days of life, the pulmonary resistance lowers, causing pulmonary overcirculation and an underperfusion systemically. Pulmonary vascular tone must be increased by either hypercapnic ventilation or inducing hypoxia. Nitrogen is used to keep inspired oxygen to , 21% and systemic perfusion increases along with an increase in pulmonary artery tone. If the O2 saturation is kept between 65% and 80%, the systemic perfusion should be ­adequate. 19. Correct answer: ­B Type II truncus arteriosus is characterized by the right and left pulmonary arteries having separate origins. A short pulmonary artery may arise from the base of the common trunk (type I), or the branch pulmonary arteries may arise separately but in close

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proximity from the posterior surface of the common trunk. The right and left pulmonary arteries may arise widely separate from the lateral aspects of the truncal artery (type III). Truncus arteriosus results in ­left-­to-­right shunting of blood and pulmonary overcirculation. Congestive heart failure and pulmonary artery hypertension are common. Pulmonary vascular obstructive disease will occur early if no intervention is ­made. 20. Correct answer: ­A The most common cardiac cause of cyanosis in neonates is transposition of the great vessels. This defect is more predominant in ­males—­about 2:1 compared to females and occurs in approximately 1 in 5000 ­births. 21. Correct answer: ­C A difference in systolic blood pressure in the upper extremities over the lower extremities is indicative of a coarctation of the aorta. The incidence of coarctation of the aorta is greater in males than in females. Many of the females also have Turner’s syndrome. The coarctation usually occurs in the thoracic section of the descending aorta, distal to the juncture of the left subclavian artery, near the location of the ­ductus. 22. Correct answer: ­B Tricuspid atresia will produce cyanosis. There is no tricuspid valve in tricuspid atresia. No blood can flow from the right atrium to the right ventricle. The only way for circulation to occur is an ­intra-­atrial shunt. This results in a ­r ight-­to-­left shunt mixing of oxygenated and unoxygenated blood, and results in ­c yanosis. An atrial septal defect and a ventricular septal defect result in a ­left-­to-­right ­shunt. 23. Correct answer: ­C It is possible to increase cardiac output in a neonate by increasing the heart rate. The formula for cardiac output is HR 3 SV, with SV being stroke volume. Preload, afterload, and contractility are components that affect cardiac output but do not necessarily increase ­it. 24. Correct answer: ­C Neonates with cyanotic heart defects are at particular risk for polycythemia. The neonate with a cyanotic defect is chronically hypoxemic. The kidneys produce erythropoietin to enhance production of red blood cells, and the increased number of red blood cells results in polycythemia. Also, hemoglobin ­oxygen-­carrying ability capacity ­increases. 25. Correct answer: ­C Stroke volume is comprised of contractility, preload, and afterload. Answer A represents the components of afterload. Answer B lists the components of cardiac output. Neonatal myocardium is very sensitive to changes, especially increased afterload. With only minute changes in afterload, the stroke volume can fall ­rapidly. 26. Correct answer: ­A The MAP is a mean pressure that takes into account that the diastolic phase of the cardiac cycle comprises ­t wo-­thirds of the cycle. The calculation for the MAP is

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MAP 5 2(DBP) 1 (SBP) 4 3. If you take the average of the two pressures, it would not account for the importance of the diastolic phase. The heart rate is not entered into this calculation. The MAP should be monitored along with peripheral perfusion and urine output. Low mean arterial pressures have been associated with cerebral hemorrhage and ischemic lesions, especially in extremely ­low-­birth-­weight ­infants. 27. Correct answer: ­B B represents the normal range of values for pulmonary artery pressures: RAP 3 mm Hg, LAP 8 mm Hg, and PAOP 6–10 mm Hg. 28. Correct answer: ­C PVR is pulmonary vascular resistance. This pressure represents a mean pressure in the pulmonary vasculature that is divided by the blood flow. Another way to think of it is the pressure against which the right ventricle must ­pump. 29. Correct answer: ­A The mean pressure difference in the systemic vascular bed divided by blood flow is known as systemic vascular resistance (SVR). SVR is systemic vascular resistance and is the resistance against which the left ventricle must ­pump. 30. Correct answer: ­C The mitral valve is the most common site for development of infective endocarditis. The aortic valve is the next most common valve affected. The valve least affected is the pulmonic valve. An infant’s tricuspid valve is often involved secondarily as a result of IV drug abuse by his or her ­mother. 31. Correct answer: ­C Epicardial pacing wires placed for postoperative cardiac management are placed because d ­ ual-­chamber pacing is most important after surgical repairs near the cardiac conduction system. The wires are placed in the right atrium and right ventricle. The wires may stay in place up to 10 ­days. 32. Correct answer: ­B Changes in lead V5 will show septal or lateral wall ­ischemia. 33. Correct answer: ­B Alpha-­adrenergic effects of norepinephrine include peripheral arteriolar vasoconstriction. A and C are the effects of ­beta-­adrenergic sympathetic ­stimulation. 34. Correct answer: ­A Between 18 and 19 days of fetal life, a heart tube forms. Each heart tube consists of an inner layer of endothelial tissue, a middle layer of cardiac jelly, and an outer layer of myocardial tissue. Each layer has distinct and specific functions, ranging from formation of ventricles, the bulbus cordis, and outflow tracts. The heart begins to beat. Circulation is established at days 26 and 27. The heartbeat is not a true pulsatile type pattern, and the blood flow is more of a swishing back and forth. While the rate is

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initially slow (60–70 bpm), it increases steadily throughout the mother’s pregnancy, reaching 130–150 bpm around the time of ­birth. 35. Correct answer: ­C Contractility of heart muscle in neonates is positively influenced by exogenous catecholamine release. Exogenous catecholamines, such as dopamine and dobutamine, will increase cardiac output and blood pressure. Contractility is decreased by hypoxia, electrolyte disturbances, hypoglycemia, and ­acidosis. 36. Correct answer: ­B The first heart sound S1, is made when the mitral and tricuspid valves close during the onset of ventricular ­systole. 37. Correct answer: ­B The third heart sound, S3, occurs when increased blood flow travels across the AV valves secondary to rapid passive ventricular filling from the atria. This is easy to remember if you associate the S3 sound with fluid. It will be prominent in CHF, mitral valve insufficiency, anemia, and ­left-­to-­r ight shunts like ASD, VSD, and ­PDA. 38. Correct answer: ­C The fourth heart sound, S4, is always pathologic after the first 24 hours of life and indicates a decreased ventricular compliance. The S 4 is produced when an atrial contraction fills up the ventricle. S 4 is rarely heard in the newborn and occurs just before the S1 heart sound. During the first 24 hours of life, the S4 may be heard just after the first heart sound and sounds like a clicking ­noise. 39. Correct answer: ­A Answer A, 56–77 mm Hg/33–50 mm Hg, corresponds to the blood pressure of a healthy term infant. A premature infant has a blood pressure that varies with size and gestational age. Blood pressure may also be affected by postnatal age, size of the cuff, body temperature, and the behavioral state of the ­infant. 40. Correct answer: ­C To evaluate a possible aortic arch abnormality, blood pressures must be taken in both arms, and it is only necessary in one leg. The legs have a common blood supply from the descending aorta, which is below the level of the defect. If the systolic pressure is 20 mm Hg greater in the upper extremities than in the lower extremities, coarctation should be suspected. A patent ductus arteriosus may mask the ­symptoms. 41. Correct answer: ­B On an EKG, l­eft-­sided heart failure results in wide, notched P waves. A tall, peaked P wave is indicative of ­r ight-­sided heart failure. Changes in ST segments (or T waves) usually indicate myocardial ­ischemia. 42. Correct answer: ­C Infants with CHF are at high risk during interventional procedures because of contrast dye. Contrast dye has high sodium content. Sodium contributes to myocardial

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depression and creates an osmotic effect which temporarily increases intravascular volume. Answers A and B are procedures, not ­r isks. 43. Correct answer: ­B Complications of the administration of indomethacin include transient oliguria and decreased renal blood flow, inhibition of platelet aggregation for 7–9 days (possibility of intracerebral hemorrhage), and GI bleeding. Contraindications are active bleeding, thrombocytopenia, suspected NEC, or ­sepsis. 44. Correct answer: ­A Indomethacin must be given slowly over 30–40 minutes to help prevent adverse events such as platelet dysfunctions, oliguria, GI bleeding, hypoglycemia, reduction in organ blood flow, hyponatremia, and hypokalemia. Indomethacin is excreted via the ­liver. 45. Correct answer: ­A Hydralazine reduces systemic vascular resistance and directly relaxes smooth muscles in arterioles. Cardiac output is increased along with blood flow to the cerebral, coronary, renal, and splanchnic areas. Adverse effects include diarrhea, emesis, and temporary ­agranulocytosis. 46. Correct answer: ­C Hydralazine is incompatible with furosemide, phenobarbital, aminophylline, and ampicillin. Hydralazine is compatible with heparin, dobutamine, hydrocortisone, ­ ex/AA. potassium chloride, prostaglandin E1, and D 47. Correct answer: ­B Adenosine is used for the suppression/elimination of sustained supraventricular tachycardia. It can also be used in diagnostic studies to establish the cause of the SVT. Adverse effects may include transient arrhythmias, flushing, dyspnea, and rarely, apnea. It is important to note that in approximately 30% of patients, SVT recurs. Caffeine and theophylline act by competitive antagonism to diminish the effect of ­adenosine. 48. Correct answer: ­C Adenosine must be given at room temperature, because if it has been refrigerated, it will crystallize. Also, the solution must be clear at the time it is administered. Adenosine should be diluted in normal saline. Adenosine should be given rapidly intravenously, followed immediately by a ­flush. 49. Correct answer: ­C Dopamine is incompatible with insulin, sodium bicarbonate, acyclovir, alteplase, amphotericin B, furosemide, and indomethacin. Dopamine is compatible with a large number of medications. Dopamine is also compatible with D5W, D5NS, D10W, LR, and ­NS. 50. Correct answer: ­B Phentolamine (Regitine) 1 mg/ml solution, should be injected in to the affected area if dopamine infiltrates. It may take as much as 5 ml to treat the ­area.

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51. Correct answer: ­B Because it is a powerful systemic vasoconstrictor (increases SVR), there is the danger of the patient developing severe hypertension and intracranial hemorrhage. The increase in SVR is normal, so it is not an adverse effect, but may precipitate adverse effects. Epinephrine is an alpha- and ­beta-­adrenergic stimulator. Epinephrine increases heart rate, myocardial oxygen consumption, contractility, automaticity, and conduction velocity. Epinephrine can cause ­hypokalemia. 52. Correct answer: ­C The bidirectional Glenn procedure is usually done prior to 6 months of age to specifically reduce volume overload to the right ventricle. Answer B is a description of the first stage, the Norwood procedure. Answer A describes the end result and disadvantage of the staged ­procedures. 53. Correct answer: ­C A contraindication to the surgical correction for hypoplastic left heart syndrome would be significant tricuspid valve dysplasia. If the patient has significant dysplasia of the tricuspid or pulmonic valves, especially if he or she also has a functional issue, the surgery has a poor chance of success. Answers A and B are a normal part of treatment for hypoplastic ­left-­heart s­ yndrome. 54. Correct answer: ­A All of the following heart defects would be classified as cyanotic heart ­defects: Tetralogy of Fallot, transposition of the great vessels, pulmonary atresia, total anomalous pulmonary venous return, truncus arteriosus, hypoplastic left heart syndrome, and tricuspid valve ­abnormalities. 55. Correct answer: ­C Acyanotic congenital heart defects include ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA), pulmonary valve stenosis, aortic valve stenosis, and coarctation of the ­aorta. 56. Correct answer: ­C Pulmonary atresia with an intact ventricular septum is also known as PAIVS. The most common finding upon physical examination is central (perioral and periorbital) cyanosis. Following ductal closure, profound generalized cyanosis is present and the apical left ventricular impulse may be pronounced. The first and second heart sounds are single. A pansystolic murmur is often heard at the lower left sternal border, ­consistent with tricuspid regurgitation. If severe, the murmur of tricuspid regurgitation may be associated with a thrill and a diastolic ­r umble. A systolic ejection murmur of the patent ductus arteriosus may be heard at the left second or third intercostal space, particularly after initiating prostaglandin ­infusion. 57. Correct answer: ­B Digoxin (Lanoxin) is a frequently used cardiac glycoside that inhibits sarcolemmal ­Na-­K adenosine triphosphatase, which leads to an increase in intracellular Ca concentration and increased myocardial contractility (the inotropic action). Digoxin has

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direct inotropic effects in addition to indirect effects on the cardiovascular system. Digoxin acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any increase in mean arterial ­pressure. Patients who are hypersensitive to digoxin may develop atrioventricular block, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, hypokalemia, or renal ­failure. 58. Correct answer: ­B The family should be informed about the necessity of genetic counseling for future pregnancies. It is important to consider and educate the parents/caregivers about obtaining cardiopulmonary resuscitation instruction to family members. As NICU nurses, we should educate caregivers about congenital heart disease and provide a list of resources and support groups if ­possible. 59. Correct answer: ­A Sepsis is probably the most important noncardiac problem in the differential diagnosis of neonates with truncus arteriosus, as well as other forms of complex congenital heart disease. Young infants with truncus arteriosus frequently present in shock because of high output heart failure with significant pulmonary overcirculation. This condition may resemble the presentation of neonatal sepsis, especially when the ratio of ­pulmonary-­to-­systemic blood flow is sufficiently high that the patient is not ­c yanotic. Truncus arteriosus usually presents with cyanosis and systemic ventricular volume overload. Outflow from both ventricles is directed into the common arterial trunk. Pulmonary blood flow is derived from this combined ventricular output, and its extent depends on the ratio of resistances to flow in the pulmonary and systemic vascular beds. Because of the mixing of left and right ventricular output that occurs primarily during systole and at the level of the common arterial trunk, subnormal systemic arterial oxygen saturation is common. Similarly, because the systemic and pulmonary circulations are essentially in parallel, pulmonary blood flow typically is at least threefold higher than systemic blood flow, with pulmonary overcirculation and increased myocardial work that results in increased resting oxygen demand and decreased metabolic ­reserve. 60. Correct answer: ­C Patients with truncus arteriosus often present with cyanosis and typically have decreased systemic arterial oxygen saturation. Cyanosis may not be evident, especially in very young neonates in whom pulmonary vascular resistance remains ­elevated. Even in slightly older neonates and young infants, pulmonary congestion and flow of left and right ventricular blood into the aorta and pulmonary arteries, respectively, may occasionally result in systemic oxyhemoglobin saturation well above 90%. Symptoms and signs of congestive heart failure are probably more common than cyanosis in patients presenting early in life. With progressively increasing pulmonary blood flow and, consequently, myocardial work, the initial symptoms of congestive heart failure become more evident as failure to thrive ensues. Symptoms of failure typically manifest as pulmonary vascular resistance falls and pulmonary overcirculation ­increases.

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61. Correct answer: ­C Cardiac murmurs are present in 80% of patients with tricuspid atresia. A holosystolic murmur that may have a crescendo and decrescendo quality is heard, suggestive of blood flow through the ventricular septal defect. A continuous murmur may be present. ­Systemic-­to-­pulmonary arterial collaterals or ­arterial-­to-­pulmonary arterial anastomoses surgically created to improve pulmonary blood flow may cause this finding. A murmur of mitral insufficiency may also be ­present. 62. Correct answer: ­A Because of hypoxia in patients with pulmonary atresia, polycythemia may be present. Prothrombin time and activated partial thromboplastin time may be abnormal secondary to the polycythemia. Cardiomegaly is usually present, with a prominent right heart border that reflects enlargement of the right atrium. Sinus rhythm is generally present, with tall P waves indicative of atrial enlargement. ­First-­degree atrioventricular block may be observed. Because of the origin of the left bundle branch from a common bundle, the frontal plane QRS axis may be leftward or ­superior. 63. Correct answer: ­B In infants, the primary use of cardiac catheterization is to determine the source and reliability of pulmonary blood flow and, in particular, to assess the status of the patent ductus arteriosus. If a restrictive atrial septal defect is present, then balloon septostomy can be ­performed. 64. Correct answer: ­B Environmental factors implicated in etiology of Ebstein’s anomaly include maternal ingestion of lithium in the first trimester of pregnancy. Some researchers report a teratogenic potential of high doses of lithium, and a 400-fold increase in the occurrence of Ebstein’s anomaly in association to its exposure in utero. In patients with bipolar disorder, the benefits of lithium may outweigh the small risk of Ebstein’s anomaly. Other possible causes of Ebstein’s anomaly include maternal rubella, maternal benzodiazepine use, maternal exposure to varnishing substances, and maternal history of previous fetal ­loss. 65. Correct answer: ­A Twelve-­lead EKG findings for a patient with Ebstein’s anomaly could include sinus rhythm with paroxysmal SVT. Usually, normal sinus rhythm with intermittent SVT, paroxysmal SVT, atrial flutter, atrial fibrillation, and ventricular tachycardia are ­present. The PR is usually prolonged with abnormal P waves consistent with right atrial enlargement. However, the PR interval may be normal or short in patients with WPW ­syndrome. 66. Correct answer: ­A Cardiac glycosides possess positive inotropic activity, which is mediated by inhibition of ­sodium-­potassium adenosine triphosphatase. Also, cardiac glycosides reduce conductivity in the heart, particularly through the atrioventricular node, and therefore have a negative chronotropic effect. The cardiac glycosides have very similar pharmacologic effects but differ considerably in their speed of onset and duration of action.

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They are used to slow the heart rate in supraventricular arrhythmias, especially atrial fibrillation, and also are used in patients with chronic heart ­failure. 67. Correct answer: ­A The only definitive way to diagnose myocarditis is via an endomyocardial ­biopsy. 68. Correct answer: ­B The pulmonic valve is least affected by infective endocarditis. The mitral valve is the most common site affected by infective endocarditis. The aortic valve is the next most common valve affected. The tricuspid valve is often involved secondarily as a result of IV drug ­abuse. 69. Correct answer: ­A The high iodine content of amiodarone can actually exert an effect on the thyroid and thus produce an antiarrhythmic ­action. 70. Correct answer: ­C Myocarditis is clinically defined as inflammation of the heart muscle. There are numerous infections, systemic diseases, drugs, and toxins that associate with the development of myocarditis. Viruses, bacteria, protozoa, and even worms have been implicated as infectious agents. Coxsackie B1 has been more prevalent as a frequent cause of myocarditis. The normal WBC count in neonates varies, but values of , ­4000­/­µL or . 25,000/µL are abnormal. The absolute band count is not sensitive enough to predict sepsis, but a ratio of immature:total polymorphonuclear leukocytes , 0.2 has a very high negative predictive value. A rapid fall in a known absolute eosinophil count and morphologic changes in neutrophils may indicate sepsis. The platelet count may fall hours to days before the onset of clinical sepsis but more often remains elevated until a day or so after the neonate becomes ­symptomatic. 71. Correct answer: ­C Right ventricular afterload may be reduced by inhaled nitric oxide. Epinephrine will increase systemic afterload due to vasoconstriction and promote increased PVR because of the increased left heart pressures. Hypoventilation and subsequent hypoxia will also increase right ventricular afterload. Use of inhaled nitric oxide, nitroglycerin, nitroprusside, PGE1, or hyperventilation will reduce right ventricular ­afterload. 72. Correct answer: ­A Hypovolemic shock may be caused by skin integrity losses such as gastroschisis and myelomeningocele. Congenital heart lesions and arrhythmias are causes of cardiogenic ­shock. 73. Correct answer: ­B Blood pressure may be normal with hypovolemic shock in the neonate. Shock is a physiologic state characterized by a reduction in tissue perfusion, resulting in decreased tissue oxygen delivery. Although the effects of inadequate tissue perfusion are initially reversible, prolonged oxygen deprivation leads to generalized cellular

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hypoxia and cell membrane ion pump failure, intracellular edema, capillary leakage, and metabolic ­acidosis. 74. Correct answer: ­B A possible cause of distributive shock in a newborn would be endotoxins. Endotoxins are toxins of ­Gram-­negative bacteria and occasionally ­Gram-­positive bacteria. These toxins may cause sepsis, a form of distributive shock. Sepsis can induce activity of the enzyme nitric oxide synthase, resulting in production of the potent direct vasodilator nitric oxide. The nitric oxide may lead to massive systemic ­vasodilation. 75. Correct answer: ­C Mast cell degranulation with resultant histamine release and vasodilation would be an appropriate definition of anaphylaxis. Anaphylaxis is a form of distributive shock. The histamine release may cause normal peripheral vascular tone to become inappropriately relaxed. Vasodilation results in increased venous capacitance, causing a relative hypovolemia even if the infant has not actually lost any net fluid. The common physiologic disturbance in all forms of distributive shock is a decrease in ­preload. 76. Correct answer: ­A Obstructive shock can be defined as a type of shock caused by an obstruction to cardiac output, such as an obstructive congenital heart disease. Examples would include coarctation of the aorta, interrupted aortic arch, and severe aortic valvular stenosis. In addition, acquired heart disease from diseases such as rheumatic fever or subacute bacterial endocarditis, as well as hypertrophic cardiomyopathy, can lead to direct obstruction of cardiac output. A partial surgical correction or a temporary measure in neonates may require maintaining patency of the ductus arteriosus in order to bypass the obstruction until more definitive surgery can be ­completed. 77. Correct answer: ­C The international consensus conference in 2002 standardized the definition of systemic inflammatory response syndrome (SIRS). SIRS is defined as tachycardia or tachypnea with fever or high leukocyte count. Sepsis is defined as SIRS in the presence of suspected or proven infection; and severe sepsis is defined as sepsis with accompanying organ dysfunction. When cardiovascular failure occurs in the setting of severe sepsis, then it is classified as septic ­shock. 78. Correct answer: ­B This infant is at risk for metabolic ­acidosis. 79. Correct answer: ­C If the catheters are positioned within the heart, damage to the endocardium may result. Consequently, intracardiac thrombi may develop. There is a high risk for these infants to develop an infection around the indwelling monitoring line. The development of infected intracardiac vegetations (infective endocarditis) increases the risk of a prolonged infection and possible dissemination of septic emboli. If the infected valve is destroyed, progressive cardiac failure may result. In many cases, it is impossible to eliminate the infection after removal of the infected line and antibiotic treatments.

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If the sepsis persists, surgical intervention may be necessary. In the ELBW infant, weight negates the use of cardiac bypass. Multisystem failure due to prolonged sepsis is associated with a high mortality. In addition, thrombocytopenia and disseminated intravascular coagulation may also develop, increasing the risk of intraventricular ­hemorrhage. 80. Correct answer: ­A Neonatal congenital heart block is thought to be caused by maternal antibodies passing through the placenta into fetal circulation. Some infants with congenital heart block are treated with corticosteroids and have limited mediation of symptoms. These symptoms may include thrombocytopenia, skin rash, and hepatitis. If these symptoms resolve, some research has shown that these infants often develop a variety of autoimmune disorders later in life. If the congenital complete heart block does not resolve, it will become permanent. In about ­t wo-­thirds of infants with complete heart block, a pacemaker is ­required. 81. Correct answer: ­B Patients receiving sodium nitroprusside should be monitored for cyanide toxicity, and sodium nitroprusside should be avoided in patients with renal problems. Cyanide toxicity may result in tachycardia and severe hypotension. Monitor venous O2 concentration and acid­–base balance. If nitroprusside extravasates from an IV, it will cause tissue sloughing and necrosis. The RBC cyanide level should be less than 50 ­mcg/ml. 82. Correct answer: ­C Amiodarone is incompatible with sodium nitroprusside at the terminal injection ­site. 83. Correct answer: ­C Procainamide is a class 1A antiarrhythmic that increases the effective refractory period of the atria and ventricles. It can also be used for acute treatment of ventricular tachycardia, which does not respond to adenosine or cardioversion. It is important to remember that amiodarone and cimetidine interact and potentiate the effects of ­procainamide. 84. Correct answer: ­A Procainamide’s adverse effects include severe hypotension (usually with a rapid infusion) and ­A-­V block. Procainamide may also widen the QRS complex due to slow impulse conduction through the Purkinje fibers and ventricular myocardium. If the QRS widens more that 35–50%, the drug should be discontinued. Adverse effects usually disappear when the drug is ­discontinued. 85. Correct answer: ­B Signs of CNS toxicity from lidocaine may include agitation, vomiting, drowsiness, and muscle twitching. Later signs may include loss of consciousness, seizures, respiratory depression, and apnea. Cardiac toxicity may develop and cause hypotension, bradycardia, heart block, and may lead to cardiovascular ­collapse.

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86. Correct answer: ­C Neonatal myocardial infarction may result in death or severe morbidity because of irreversible myocardial damage. New research is available to suggest that when fetal heart tones are absent, the fetus may actually be in asystole. A myocardial infarction usually occurs in association with congenital heart disease or coronary artery abnormalities such as a heart obstruction, obstructed pulmonary venous return, and anomalous origin of the left coronary artery. In neonates without these conditions, most of these infarctions are probably due to thromboemboli and perinatal asphyxia, but most are ­idiopathic. 87. Correct answer: ­B The ductus venosus is the shunt that permits most of the blood from the placenta to bypass the liver and then enter the vena ­cava. 88. Correct answer: ­A The foramen ovale is an opening in the septum between the atria that allows a portion of blood to flow from the right atrium directly to the left atrium. This helps reduce the flow of blood through the pulmonary ­system. 89. Correct answer: ­A In fetal circulation, blood leaving the left atrium goes through the left ventricle, then travels to the head and neck via the ascending aorta. This circuit ensures the fetal brain receives adequate blood and ­oxygen. 90. Correct answer: ­C From the placenta, the oxygenated blood flows through the umbilical vein to the ­fetus. 91. Correct answer: ­C In fetal circulation, about half the blood enters the liver and the other half of the blood bypasses the liver through the ductus ­venosus. 92. Correct answer: ­A In fetal circulation, blood from the ductus venosus enters the inferior vena cava. In the inferior vena cava, unoxygenated blood from the lower extremities, GI tract, and liver mixes with blood with a higher oxygen content from other parts of the body. This mixed blood then enters the right ­atrium. 93. Correct answer: ­B When a newborn takes the first breath and the umbilical cord is occluded, the newborn’s systemic vascular resistance is elevated. The amount of blood flow through the ductus is reduced. The cord occlusion causes a rapid increase in blood pressure, stimulating the baroreceptors and the sympathetic nervous system. The initial respirations cause lung expansion and decrease pulmonary vascular ­resistance.

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94. Correct answer: ­B Immediately after birth, the right ventricular output flows through the lungs and results in higher pressures in the left atrium. This increased pressure, along with increased systemic vascular resistance, functionally closes the foramen ­ovale. 95. Correct answer: ­C The ductus arteriosus normally closes after 15–24 hours. The ductus closes in response to increased oxygen ­content. 96. Correct answer: ­B The definition of a functional closure is the cessation of flow through a structure caused by changes in pressure. An example would be the closure of the foramen ­ovale. 97. Correct answer: ­C After birth, the direction of blood flow through the ductus arteriosus reverses. The direction of the blood flow at this time would be left to ­r ight. 98. Correct answer: ­B The definition of an anatomic closure is an obliteration of a structure by growth or constriction. An example would be the ductus ­venosus. 99. Correct answer: ­A Hypoxia will cause a transient opening of a constricted ductus arteriosus and may reestablish increased pulmonary vascular resistance and lead to persistent pulmonary hypertension of the newborn (PPHN). 100. Correct answer: ­B Metabolism of the heart is decreased during diastole, which comprises about ­one-­half of the cardiac cycle at birth. Shortly after birth, the diastolic phase lengthens to comprise ­t wo-­thirds of the cardiac cycle. An increase in cardiac output decreases ­diastole. 101. Correct answer: ­C The average newborn’s cardiac cycle is about 0.4 second, with about 0.2 second for diastole and 0.2 second for systole (based on a heart rate of 150 bpm). 102. Correct answer: ­B Usually, increased volume results in increased pressure and contractility is increased. This is due to stimulation of stretch receptors. In the newborn, there are fewer fibers and they cannot stretch enough to meet the increased volume. Therefore, increasing the heart rate is the only mechanism by which the newborn can respond to the increased ­volume. 103. Correct answer: ­A When the parasympathetic and sympathetic nervous systems stimulate the right vagus nerve, the SA node is affected and slows the heart rate. Acetylcholine is a ­neurotransmitter.

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104. Correct answer: ­C If a beta2 receptor in the heart is stimulated, it may cause vasodilation (lowered SVR), bronchodilation, and smooth muscle ­relaxation. 105. Correct answer: ­B Early signs of left ventricular failure are tachypnea and tachycardia. Severe left ventricular failure also causes dyspnea and ­retractions. 106. Correct answer: ­C Cyanosis may be observed when at least 5 g/100 ml of deoxygenated hemoglobin is present in the ­circulation. 107. Correct answer: ­B Prolonged physiologic jaundice may be indicative of CHF or congenital hypothyroidism associated with PDA and pulmonary stenosis. Pale skin is associated with CHF or shock, as well as anemia. A ruddy or plethoric color may be due to ­polycythemia. 108. Correct answer: ­A In the newborn, sweating is often indicative of chronic heart disease. If cardiac output is decreased, sweating is a compensatory mechanism (sympathetic overactivity). 109. Correct answer: ­A Precordial bulging is often a sign of chronic cardiac enlargement. If hyperactivity occurred without bulging, it would probably indicate an acute cardiac dysfunction. Pectus excavatum does not cause cardiac ­dysfunction. 110. Correct answer: ­C If the right brachial pulse is stronger than the left brachial pulse, this is most likely due to supravalvular stenosis or a coarctation located proximal to or very near the origin of the left subclavian ­artery. 111. Correct answer: ­B A hyperactive precordium usually indicates a heart defect with increased volume. An example would be a large ­left-­to-­r ight shunt or heart disease with valvular ­regurgitation. 112. Correct answer: ­B The location of the PMI in a newborn is usually located at the lower left sternal border. The apical impulse of a newborn is usually felt at the fourth intercostal space just to the left of the midclavicular ­line. 113. Correct answer: ­A A thrill at the upper left sternal border is useful in assessing problems with the pulmonic valve or pulmonary ­artery.

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114. Correct answer: ­B The S2 heart sound is created by closure of the pulmonic and aortic valves. S2 is best heard in the upper left sternal border or pulmonic ­area. 115. Correct answer: ­C Regurgitation systolic murmurs are associated with VSD, tricuspid regurgitation, and mitral valve regurgitation. Regurgitation systolic murmurs are caused by blood flow from an area of higher pressure throughout systole to an area of lower ­pressure. 116. Correct answer: ­A The murmur of aortic regurgitation is best heard at the third left intercostal space. For optimal sound transmission it is best to use the diaphragm of the stethoscope. Aortic regurgitation murmurs are early diastolic ­murmurs. 117. Correct answer: ­C The PDA is the most commonly heard continuous murmur in a newborn. It is louder during systole. The PDA murmur is loudest at the upper left sternal border or the left infraclavicular ­area. 118. Correct answer: ­B Infants with a pulmonary flow murmur have low birth weight and have hypoplastic pulmonary arteries at birth. After birth, blood flow and turbulence increases, causing the murmur. This murmur usually disappears about 3–6 months after ­birth. 119. Correct answer: ­C Cyanosis in the extremities is known as acrocyanosis. This is most probably due to reduced blood flow through small capillaries. Oxygen is removed from the hemoglobin by these capillaries and results in the blue appearance of the ­skin. 120. Correct answer: ­A In patients with normal cardiac anatomy, right atrial pressure equals right ventricular ­end-­diastolic pressure, which equals central venous ­pressure. 121. Correct answer: ­B A pulmonary artery catheter may be placed in a neonate’s right ventricular outflow tract to evaluate the risk for pulmonary hypertension. Pulmonary hypertension is anticipated in endocardial cushion repair, mitral stenosis, and ­TAPVR. 122. Correct answer: ­C Phasic variations in an arterial pressure waveform during mechanical ventilation usually indicate hypovolemia or heart ­failure. 123. Correct answer: ­C In patients with normal cardiac anatomy, pulmonary artery wedge pressure equals pulmonary artery occlusion pressure, which equals left ventricular ­end-­diastolic pressure, which equals left atrial ­pressure.

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124. Correct answer: ­C An acute rise in a pulmonary wedge pressure (pulmonary artery occlusion pressure) indicates a pulmonary artery hypertensive crisis and ­r ight-­sided heart ­failure. 125. Correct answer: ­C A chest radiograph that shows an “egg on a string” appearance with mild cardiomegaly is indicative of transposition of the great vessels. The cardiac silhouette is usually normal ­size. 126. Correct answer: ­A Enlargement of the right atrium, right ventricle, and pulmonary artery and a heart that exhibits a “snowman” appearance on a chest radiograph indicates a total anomalous pulmonary venous return. The snowman appearance is a widening of the superior mediastinum. The widening of the mediastinum is caused by connecting blood vessels and is a rare phenomenon. Cardiomegaly and increased pulmonary vascular markings are also seen with this ­condition. 127. Correct answer: ­B The type of total anomalous pulmonary venous return that exists when pulmonary veins attach directly to the coronary sinus and drain into the right atrium is known as cardiac or type II TAPVR. Supracardiac TAPVR exists when pulmonary veins attach above the diaphragm, most often to the superior vena cava. The infracardiac form of TAPVR occurs when pulmonary veins attach below the diaphragm into the portal venous system and drain into the inferior vena ­cava. 128. Correct answer: ­A A characteristic of the obstructed form of total anomalous pulmonary venous return is respiratory distress that may not respond to mechanical ventilation. A ­r ight-­to-­left shunt exists across the atrial septum. Pulmonary edema is present, cyanosis is profound, and the EKG shows right ventricular hypertrophy with a right axis ­deviation. 129. Correct answer: ­A AV canal defects are also known as endocardial cushion defects. The atrioventricular junction is flanked by two masses of endocardial cushions, a superior and an inferior cushion. The septum grows towards the atrioventricular endocardial cushion and fuses with it. The atrioventricular valves form during the 5th to 8th week of development. When the atria and inlet portions of the ventricle enlarge, the sulcus drops into the ventricular cavity, forming a hanging flap. The endocardial cushion tissue is located at the tip of this flap. Nonunion can cause an atrial septal defect and a ventricular septal defect to form. These defects will cause ­left-­to-­r ight shunting, leading to congestive heart failure. Patients with AV canal defect develop elevated pulmonary vascular resistance secondary to right ventricular hypertension and increased pulmonary blood flow. Upper airway obstruction occurs in patients with trisomy ­21. 130. Correct answer: ­C Ostium primum is a defect in the atrial septum at the level of the tricuspid and mitral valves. The defect occurs at the portion of the heart where the atrial septum meets the ventricular septum and the mitral valve meets the tricuspid ­valve.

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131. Correct Answer: ­C When the balloon of a pulmonary artery catheter is inflated, it eventually “wedges” in the pulmonary artery. The turbulence behind it is blocked, and it senses what is in front of it, which is the pulmonary vascular bed and left side of the heart. The pressure was formerly known as PAWP (Pulmonary Artery Wedge Pressure). It is now known as PAOP (Pulmonary Artery Occlusive Pressure). It is sometimes known as PCWP (Pulmonary Capillary Wedge Pressure). The normal value should be 5–12 mm Hg. 132. Correct answer: ­C Actions of beta blockers include increased diastolic filling ­time. 133. Correct answer: ­C Alpha-­adrenergic effects of norepinephrine include peripheral arteriolar vasoconstriction. A and B are the effects of ­beta-­adrenergic sympathetic ­stimulation. 134. Correct answer: ­C A body mass index over 30 indicates obesity. A BMI of about 25–30 means the person is overweight. In addition, a waist circumference of less than 36 inches is considered normal for females. To calculate the BMI, use this formula: BMI 5 weight in pounds 3 703 4 (height in inches)­2 135. Correct answer: ­C Lidocaine has no impairment of normal contractility. Answers A and B are effects of phenytoin, another class 1B drug. Lidocaine may shorten the QT interval and the side effects usually involve the CNS—­drowsiness, irritability, paresthesias, seizures, and ­convulsions. 136. Correct answer: ­B Calcium channel blockers act primarily on arteries to arterioles. Large lumen vessels in the arterial system are affected. The advantage of this action is that both systolic and diastolic pressures are reduced and the patient will not have a drop in blood pressure. The blood pressure may be lowered slightly and cause a reflex baroreceptor response to speed up the heart rate to maintain cardiac ­output. 137. Correct answer: ­B The type of echocardiography that shows the quantity of flow across an obstruction is known as ­continuous-­wave echocardiography. This type of echocardiography is used to detect direction of shunting, estimating cardiac output, and assessing ventricular diastolic function. This type of echocardiography provides a good estimate of pressure ­gradients. 138. Correct answer: ­A Motion of cardiac valves and detection of pericardial fluid is done with ­M-­mode echocardiography. ­M-­mode echocardiography enables evaluation of anatomic relationships and the relative sizes of ­each. 139. Correct answer: ­A A PDA or ventricular dysfunction in a premature infant is done using ­t wo-­dimensional ­echocardiography.

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140. Correct answer: ­C Color-­flow Doppler echocardiography detects patterns of blood flow such as ­r ight-­to-­left or ­left-­to-­r ight shunts. Regurgitation and location of restrictions are also detected using ­color-­flow Doppler ­echocardiography. 141. Correct answer: ­B The ­high-­contrast mediums used in cardiac catheterizations have a high sodium ­content. 142. Correct answer: ­C Contraindications for the use of indomethacin to close a PDA in a premature infant would be a serum creatinine clearance of . 1.8 mg/dl, a BUN . 30 mg/dl, and a urine output , 0.6 ml/Kg/hour. Necrotizing enterocolitis is not a ­contraindication. 143. Correct answer: ­A Severe aortic stenosis in utero decreases blood flow through the ventricle and results in left ventricular hypoplasia and ­left-­sided heart ­syndrome. 144. Correct answer: ­B Pulmonary atresia is complete obstruction of the pulmonic valve resulting in a hypoplastic right ventricle and tricuspid ­valve. 145. Correct answer: ­B Premature ductus arteriosus closure may be caused by maternal use of a prostaglandin inhibitor such as ibuprofen or ­aspirin. 146. Correct answer: ­B Chlorothiazide reduces urinary calcium losses and does not result in profound potassium l­osses. 147. Correct answer: ­C A fixed S2 split heart murmur indicates possible abnormalities of the pulmonic and/or aortic valves. Widening S2 sounds indicate atrial septal defect, total anomalous pulmonary venous return, Tetralogy of Fallot, pulmonary stenosis, or Ebstein’s ­anomaly. 148. Correct answer: ­B Left ventricular failure may first be noted by tachypnea and tachycardia as the infant attempts to compensate drop in forward blood flow and oxygenation to ­tissues. 149. Correct answer: ­C Indomethacin used in the treatment of PDA may lead to elevated BUN and creatinine levels as a result of nephrotoxicity. In addition, urine output should be monitored for ­oliguria.

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150. Correct answer: ­A Congestive heart failure results in a systemic venous engorgement as blood does not flow forward and is allowed to pool in organs and peripheral circulation. Tissues become hypoxic and further diminish function until complete failure and ­death. 151. Correct answer: ­C Atrioventricular canal is a defect that is often associated with an ostium primum ASD, a cleft in the anterior mitral valve leaflet, a VSD in the inlet portion of the ventricle septum, and a cleft in the septal leaflet of the tricuspid ­valve. 152. Correct answer: ­A Hypoplastic left heart syndrome consists of a group of cardiac defects, including a small aorta, aortic and mitral valve stenosis or atresia, and a small left atrium and ­ventricle. 153. Correct answer: ­C Prolonged fetal supraventricular tachycardia will lead to heart failure and nonimmunologic hydrops fetalis, and blood fails to flow forward through the heart due to diminished fill time. As cardiac output fails, blood pools in organs and peripheral circulation leading to capillary damage and third spacing. Treatment is to identify and treat the specific underlying cause quickly before significant tissue damage ­occurs. 154. Correct answer: ­C The order for labetalol is 3 times the normal dosage and must be corrected. As bedside nurses, we are an additional step of safety and cannot ethically or legally administer a medication that would harm our patient. Doctor–nurse relationships may make it difficult to address potential medication errors. It is important to verify drug dosages with a separate reliable source, for example Redbook or Neofax, prior to addressing the issue with a physician. If unable to clarify or correct the order, nurses must follow and document communication and results via chain of command until ­resolution. 155. Correct answer: ­C In fetal circulation, blood entering the right atrium from the inferior vena cava is shunted across the foramen ovale to the left atrium to provide oxygenated blood to the coronary, carotid, and subclavian ­arteries. 156. Correct answer: ­C After the infant successfully transitions from fetal to neonatal circulation, the left ventricle of the heart pumps against the highest pressure (SVR). 157. Correct answer: ­C Transposition of the great vessels results in an increased pulmonary blood flow as blood from the lungs is circulated through the left heart in a closed loop, and systemic blood flow circulates via the right side of the heart in a closed loop. Survivability is dependent on a patent ductus arteriosus and/or concurrent septal defects allowing mixing of blood in the heart, resulting in low oxygenated blood to circulate to the ­body.

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158. Correct answer: ­B Polycythemia results in an increased blood viscosity, causing decreased pulmonary blood flow in cyanotic congenital heart ­defects. 159. Correct answer: ­C Dobutamine improves cardiac output primarily by increasing cardiac contractility. Unlike dopamine, dobutamine does not increase heart rate and can be used in tachycardic ­patients. 160. Correct answer: ­B Fetal heart may be increased by maternal fever resulting in fetal hyperthermia and increased metabolic ­demands. 161. Correct answer: ­A A nearly flat baseline on a fetal monitor indicates fetal compromise. New research indicates that flat fetal heart rates may indicate fetal asystole with cardiac tissue death. In addition to cardiac tissue death, neurologic function and health may be impaired and at risk for cerebral palsy and ­hypoxic-­ischemic ­encephalopathy. 162. Correct answer: ­A Digoxin toxicity may cause hypokalemia and lead to atrial flutter or fibrillation. These cardiac rhythms place the neonate at risk for blood clots as blood does not completely leave the atrium. Monitor clotting factors as well as provide heparin ­therapy. 163. Correct answer: ­B The ­Blalock-­Taussig procedure will attach the subclavian and the pulmonary artery to redirect blood flow through the ­heart. 164. Correct answer: ­C Cyanosis usually occurs when tricuspid atresia is present. Blood flow is unable to proceed through to the right ventricle to the pulmonary circulation. Increased ­r ight-­sided pressures cause unoxygenated blood to shunt through the foramen ovale to the left side to systemic circulation. Temporary management is to maintain a patent ductus arteriosus to shunt some blood to pulmonary circulation and to be oxygenated. Surgical intervention is required for ­long-­term ­treatment. 165. Correct answer: ­B A heart rate of 282 with narrow QRS complexes indicates SVT. Adenosine is the most likely pharmacologic initial treatment for supraventricular tachycardia. If the patient is stable and the rhythm is not witnessed by a physician or advanced practitioner, attempt to obtain a 12-lead EKG prior to spontaneous or ­vagal-­induced conversion. Once the heart is converted to a normal rhythm, the infant may be started on an antiarrhythmic. Dosage recalculations should be done every few days as the patient’s weight ­increases. 166. Correct answer: ­A Right-­sided congestive heart failure signs and symptoms include respiratory distress and hepatomegaly. The respiratory distress is the result of blood’s failure to flow to the

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lungs causing hypoxemia. As blood backs up into the hepatic system, hepatomegaly and splenomegaly may ­result. 167. Correct answer: ­B If the umbilical venous catheter is placed in the inferior vena cava, central venous pressure can be monitored if the catheter is connected to a transducer. Hemodynamic monitoring may provide early indication of sepsis and hypovolemic ­shock. 168. Correct answer: ­C A warm compress to the foot and groin and application of nitropaste will cause vasodilation to increase blood flow to the toes after umbilical artery placement. If “cath toes” does not resolve, then the physician should be notified and the catheter should be ­removed. 169. Correct answer: ­B Extracorporeal membrane oxygenation (ECMO) may be indicated if the patient has persistent pulmonary hypertension to provide oxygenation. Transient tachypnea of the newborn is a temporary condition and is not an indicator for ­ECMO. 170. Correct answer: ­B Exclusion criteria for the use of ECMO for respiratory failure would include a gestational age less than 34 weeks due to premature and fragile ­tissues.

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Cardiovascular System ­References Adler, A., Litmanovitz, I., Bauer, S., & Dolfin, T. (2004, September/October). Aspirin treatment for neonatal infectious endocarditis. Pediatric Cardiology, 25(5), 562–564. Al Dhahri, K., Sandor, G., & Duncan, W. (2006). ­Intra-­atrial thrombus in a neonate with coarctation of the aorta. Cardiology in the Young, 16(4), 392–394. Alexander, C. P., Sood, B. G., Zilberman, M. Z., et al. (2006). Congenital hepatic arteriovenous malformation: An unusual cause of neonatal persistent pulmonary hypertension. Journal of Perinatology, 26(5), 316–318. Anatomy. (2009, March). Studies from H.G. Lim et al have provided new data on anatomy. Cardiovascular Week, 26–28. Bozza, F. A., Salluh, J. I., Japiassu, A. M., et al. (2007). Cytokine profiles as markers of disease severity in sepsis: A multiplex analysis. Critical Care, 11, ­R49. Brodsky, D., & Martin, C. (2003). Neonatology review. Philadelphia, PA: Hanley & ­Belfus. Brucato, A., Jonzon, A., Friedman, D., et al. (2003). Proposal for a new definition of congenital complete atrioventricular block. Lupus, 12(6), 427–435. Bueltmann, M., Kong, X., Mertens, M., et al. (2009). Inhaled milrinone attenuates experimental acute lung injury. Intensive Care Medicine, 35(1), 171–178. Caforio, A. L. P, Daliento, L., Angelini, A., et al. (2005). Autoimmune myocarditis and dilated cardiomyopathy: Focus on cardiac autoantibodies. Lupus, 14(9), 652–655. Capasso, L., Raimondi, F., Capasso, A., et al. (2003). Early cord clamping protects ­at-­r isk neonates from polycythemia. Biology of the Neonate, 83(3), 197–200. Carabello, B., & Paulus, W. (2009). Aortic stenosis. The Lancet, 373(9667), 956–966. Chaudhari, M., Hamilton, L., & Hasan, A. (2006). Correction of coronary arterial anomalies at surgical repair of common arterial trunk with ischemic left ventricular dysfunction. Cardiology in the Young, 16(2), 179–181. Cloherty, J. P., Eichenwald, E. C., & Stark, A. R. (2004). Manual of neonatal care (5th ed.). Philadelphia, PA: ­Lippincott. Costedoat-­Chalumeau, N., ­Georgin-­Lavialle, S., Amoura, A., & Piette, J. C. (2005). ­A nti-­SSA/Ro and ­anti-­SSB/La ­antibody-­mediated congenital heart block. Lupus, 14(9), 660–664. Dabir, T., Mccrossan, B., Sweeney, L., et al. (2008). Down syndrome, achondroplasia and tetralogy of Fallot. Neonatology, 94(1), 68–70. Dong, L., Zhang, F., Shu, X., et al. (2009). Left ventricular torsional deformation in patients undergoing transcatheter closure of secundum atrial septal defect. The International Journal of Cardiovascular Imaging, 25(5), 479–486. Hakuno, D., Kimura, N., Yoshioka, M., & Fukuda, K. (2009). Molecular mechanisms underlying the onset of degenerative aortic valve disease. Journal of Molecular Medicine, 87(1), 17–24. Halliday, H. L., McClure, B. G., & Reid, M. (2002). Handbook of neonatal intensive care (4th ed.). Philadelphia, PA: ­Saunders. Hay, W. W., Levin, M. J., et al. (2007). Current diagnosis and treatment in pediatrics (18th ed.). New York: McGraw-­Hill. Hetzel, P. G., Glanzmann, R., Günthard, J., et al. (2007). Failed detection of complex congenital heart disease (including double outlet right ventricle and total anomalous pulmonary venous return) by neonatal pulse oximetry screening. European Journal of Pediatrics, 166(6), 625–626. Huhta, J. (2004). Neonatal hemodynamics in patients with hypoplastic left heart syndrome. Cardiology in the Young, 14(S1), 22–26. Jones, K. L. (2006). Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: ­Elsevier. Kaestner, M., Handke, R., Photiadis, J., et al. (2008). Implantation of stents as an alternative to reoperation in neonates and infants with acute complications after surgical creation of a ­systemic-­to-­pulmonary arterial shunt. Cardiology in the Young, 18(2), 177–184.

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Cardiovascular ­System  y  115 Karlsen, K. (2006). The S.T.A.B.L.E. program (5th ed.). Park City, UT: American Academy of ­Pediatrics. Karlsen, K., & Tani, L. Y. (2003). S.T.A.B.L.E.—Cardiac module. Park City, UT: American Academy of ­Pediatrics. Kattwinkle, J. (Ed.). (2006). Neonatal resuscitation textbook (5th ed.). American Academy of Pediatrics and American Heart ­A ssociation. Kenner, C., Amlung, S. & Flandermeyr, A. (1998). Protocols in neonatal nursing. Philadelphia, PA: ­Saunders. Kenner, C., & Lott, J. (2004). Neonatal nursing handbook. St. Louis, MO: E ­ lsevier. Kenner, C., & Lott, J. (2007). Comprehensive neonatal care (4th ed.). St Louis, MO: ­Elsevier. Kenner, C., & McGrath, J. M. (Ed.). (2004). Developmental care of newborns & infants: A guide for health professionals. St. Louis, MO: M ­ osby. Koschel, M. J. (2006, August). Management of the ­c yanide-­poisoned patient. Journal of Emergency Nursing, 32(4, suppl.), S19–S28. Kumar, A., Kumar, A., Paladugu, B., et al. (2007). Transforming growth ­factor-­beta1 blocks in vitro cardiac myocyte depression induced by tumor necrosis ­factor-­alpha, interleukin-1 beta, and human septic shock serum. Critical Care Medicine, 35, 358–364. Kutsal, A., Yavuz, T., & Ulusan, V. (2006). Right atrial and tricuspid hypoplasia. Journal of Cardiovascular Surgery, 47(3), 353–354. Larmay, H. J., & Strasburger, J. F. (2004). Differential diagnosis and management of the fetus and newborn with an irregular or abnormal heart rate. Pediatrics Clinics of North America, 51, 1033–1050. Lee, L. (2009). The clinical spectrum of neonatal lupus. Archives of Dermatological Research, 301(1), 107–110. Lennestål, R., Otterblad Olausson, P., & Källén, B. (2009). Maternal use of antihypertensive drugs in early pregnancy and delivery outcome, notably the presence of congenital heart defects in the infants. European Journal of Clinical Pharmacology, 65(6), 615–625. MacDonald, M. G., & Ramasethu, J. (2007). Atlas of procedures in neonatology (4th ed.). Philadelphia, PA: Lippincott ­Williams. Markiewicz, M., & Abrahamson, E. (1999). Diagnosis in color: Neonatology. Philadelphia, PA: ­Mosby. Marks, K. A., Zucker, N., Kapelushnik, J., et al. (2002, January). Infective endocarditis successfully treated in extremely low birth weight infants with recombinant tissue plasminogen activator. Pediatrics 109(1), 1 ­ 53. Melegh, Z., Patel, Y., & Ramani, P. (2008). Solitary pulmonary infantile hemangioma in an infant with atrial septal defect. Pediatric and Developmental Pathology, 11(6), 465–468. Merenstein, G. B., & Gardner, S. L. (2006). Handbook of neonatal intensive care (6th ed.). St. Louis, MO: ­Mosby. Miller, S., McQuillen, P., Hamrick, S., et al. (2007). Abnormal brain development in newborns with congenital heart disease. The New England Journal of Medicine, 357(19), 1928–1938. Mongiovi, M., & Pipitone, S. (2008). Supraventricular tachycardia in fetus: How can we treat? Current Pharmaceutical Design, 14(8), 736–742. Montenegro, N., Levi, S., Matias, A., et al. (2005). Placental blood flow mapping in the first trimester of human pregnancy. Ultrasound Review of Obstetrics and Gynecology, 5(1), 23–28. Morgan, T. (2007). Turner syndrome: Diagnosis and management. American Family Physician, 76(3), 405–410. Morgan, P., Madgula, R., Gilvarry, E., & Findlay, M. (2009). Substance misuse during pregnancy: Its effects and treatment. Fetal and Maternal Medicine Review, 20(1), 1–16. Olmsted, K., Oluola, O., Parthiban, A., & Raghuveer, T. (2007). Can inhaled prostacyclin stimulate surfactant in ELBW infants? Journal of Perinatology, 27(11), 724–726.

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116  y  Section 4 Padalino, M., Castellani, C., Toffoli, S., et al. (2008). Pathological changes and myocardial remodelling related to the mode of shunting following surgical palliation for hypoplastic left heart syndrome. Cardiology in the Young, 18(4), 415–422. Perles, Z., Gavri, S., Azaria, J. J. T., & Rein, A. J. (2006). Tachyarrhythmias in the fetus: State of the art diagnosis and treatment. Progress in Pediatric Cardiology, 22, 95–107. Polin, R. A. & Spritzer, A. R. (2007) Fetal & neonatal secrets (2nd ed.). Philadelphia, PA: ­Mosby. Prescribing Reference. (2009, Summer). NPPR: Nurse practitioner’s prescribing reference, 16(2). Rennie, J. M., & Roberton, N. R. C. (2002). A manual of neonatal intensive care (4th ed.). New York: ­A rnold. Sakata, M., Utsu, M., & Maeda, K. (2006). Fetal circulation and placental blood flow in monochorionic twins. Ultrasound Review of Obstetrics and Gynecology, 6(3/4), 135–140. Sekar, K., Clyman, R., & Seri, I. (2006). Discussion 1: Focus on neonatal hemodynamics. Journal of Perinatology, 26(S1), S22–S23. Shehata, B. M., & Abramowsky, C. R. (2005). Alveolar capillary dysplasia in an infant with trisomy 21. Pediatric and Developmental Pathology, 8(6), 696–700. Siles, A., & Lapierre, C. (2008). Infracardiac total anomalous pulmonary venous return (TAPVR). Pediatric Radiology, 38(12), 1 ­ 354. Stoll, B. J., Hansen, N., Fanaroff, A. A., et al. (2002). ­Late-­onset sepsis in very low birth weight neonates: The experience of the NICHD neonatal research network. Pediatrics, 110, 285–291. Taeusch, H. W., Ballard, R. A., & Gleason, C. A. (2005). Avery’s diseases of the newborn (8th ed.). Philadelphia, PA: ­Elsevier. Takeuchi, M., Suzuki, T., Nakayama, M., et al. (2006). Neonatal myocardial infarction due to thrombotic occlusion. Journal of Maternal, Fetal & Neonatal Medicine, 19(2), 121–123. University of California. (2008, July). Atrioventricular canal defects: Reports outline atrioventricular canal defects research from University of California. Cardiovascular Week, ­33. Verklan, M. T., & Walden, M. (Ed.). (2004). Certification and core review for neonatal intensive care nursing (3rd ed.). St. Louis, MO: E ­ lsevier. Verklan, M. T. & Walden, M. (Ed.). (2010). Certification and core review for neonatal intensive care nursing (4th ed.). St. Louis, MO: ­Elsevier. Yildirim, S., Tokel, K., Saygili, B., & Varan, B. (2008). The incidence and risk factors of arrhythmias in the early period after cardiac surgery in pediatric patients. The Turkish Journal of Pediatrics, 50(6), 549–553. Young, T. E., & Magnum, B. (2009). Neofax 2009. Montvale, NJ: Thomson ­Reuters. Zhao, H., Strasburger, J. F., Cuneo, B. F., & Wakai, R. T. (2006). Fetal cardiac repolarization abnormalities. American Journal of Cardiology, 98, 491–496.

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Section 5 Pulmonary ­System

Questions 1. Type II pneumocytes ­produce A. ­Macrocytes B. ­Phagocytes C. ­Surfactant 2. If you hear faint breath sounds on the left side of the chest and normal sounds on the right side immediately after your patient has been intubated, most ­likely A. The right mainstem has been ­intubated B. The physician has intubated the ­esophagus C. The ETT is at the c­ arina 3. Your term infant has required some supplemental oxygen. She is placed on 2 ­L­/­min via NC and ABGs have been drawn. Blood gas results show: pH 7.54, PaO2 98 mm Hg, PaCO2 26 mm Hg, and HCO3 24 mEq/L. These blood gas results ­show A. Uncompensated respiratory ­alkalosis B. Compensated metabolic ­alkalosis C. Compensated metabolic ­acidosis 4. Your patient is septic and you have just drawn an ABG. The respiratory therapist asks if the patient has a fever. The possibility of fever will have what effect on the ­sample? A. The HCO3 will be ­elevated B. The pH will ­r ise C. Fever has no ­effect 5. Your patient has been hypoxic since birth 3 days ago. What potential imbalance would be expected with this ­condition? A. ­Hypokalemia B. ­Hypochloremia C. Decreased bicarbonate ­levels 6. Hypoxemia is best defined ­as A. A decrease in oxygen at the cellular ­level B. A decrease in oxygen levels in arterial ­blood C. A decrease in oxygen levels in venous ­blood

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y

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7. Baby M. has respiratory distress syndrome (RDS) and has been on mechanical ventilation for 4 days as a result of meconium aspiration. During your assessment, you note a temperature of 100° F, heart rate of 200, respiratory rate of 76, increased cough, and decreased breath sounds on the right side without tracheal deviation. You suspect her symptoms are the result ­of A. Pulmonary ­edema B. ­Atelectasis C. ­Pneumothorax 8. The cells that are responsible for forming a barrier for alveoli ­are A. ­Macrophages B. Type II alveolar epithelial ­cells C. Type I alveolar epithelial ­cells 9. Anatomic dead space is defined ­as A. Minute ­ventilation B. Wasted ­ventilation C. A conducting ­airway 10. The oxyhemoglobin dissociation curve i­ s A. A relation between dissolved oxygen and the affinity for oxygen by the hemoglobin ­molecule B. A measure of ­methemoglobin C. A way to calculate gas transport across the ­alveoli 11. If the oxyhemoglobin curve shifts to the right, one of the factors that will affect this shift ­is A. A decrease in ­CO2 B. A decrease in ­pH C. A decrease in ­temperature 12. If the oxyhemoglobin dissociation curve shifts to the left, which of the following conditions would precipitate this ­change? A. Increased ­temperature B. An increased ­pH C. Increased 2,3 D ­ PG 13. Chronic hypoxia usually results in which of the following electrolyte ­imbalances? A. Decreased ­chloride B. Decreased ­potassium C. Decreased ­calcium 14. Infants with immature lungs are at risk for respiratory distress syndrome. Which of the following characteristics would be present in most cases of respiratory distress s­ yndrome? A. Pulmonary ­hyperperfusion B. Surfactant present at ­birth C. ­Prematurity

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15. Retractions are an indication of respiratory distress in infants. Retractions can best be s­ een A. At the 5th right intercostal space in the midclavicular ­line B. At the xiphoid and intercostal markings of the infant’s ­chest C. At the 2nd left intercostal space, midclavicular ­line 16. In an infant, respiratory distress may lead t­ o A. ­Hypoglycemia B. ­Bradycardia C. ­Left-­to-­r ight intrapulmonary ­shunting 17. Which of the following signs would be present in an infant with respiratory distress s­ yndrome? A. ­Hematocrit B. Pulse ­oximetry C. ­Grunting 18. Which of the following statements is true about transient tachypnea of the ­newborn? A. TTN only affects infants who are born after 35 weeks ­gestation B. Epinephrine may be useful in the treatment of ­T TN C. Sodium transport is decreased after ­birth 19. A risk factor for transient tachypnea of the newborn would ­include A. Maternal ­diabetes B. Vaginal ­delivery C. Female ­gender 20. Which of the following statements about meconium aspiration in newborns is ­true? A. Meconium aspiration will always require use of mechanical ­ventilation B. Meconium is a medium for bacterial ­growth C. Only ­post-­term infants have meconium ­aspiration 21. Many neonates with RDS and hypoxemia are managed by increasing the inflating pressure and application of positive ­end-­expiratory pressure (PEEP). PEEP is useful in RDS b ­ ecause A. PEEP decreases cardiac ­output B. PEEP prevents ­barotraumas C. PEEP can open collapsed ­alveoli 22. Falsely low readings on a pulse oximeter may be due ­to A. ­Fever B. Vascular ­dyes C. ­Phototherapy

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23. Your patient’s physician tells you that your patient needs the left upper lobes drained if possible. The correct position to help this patient ­is A. Semi-­reclining B. Flat with hips ­elevated C. ­Supine 24. PEEP may cause an increase ­in A. ­SVR B. ­PVR C. ­PAOP 25. Increased PEEP may ­cause A. ­Barotrauma B. A decrease in left atrial ­pressure C. ­Hemothorax 26. Which of the following drugs is a ­methylxanthine? A. ­Prednisone B. ­T heophylline C. ­Atropine 27. The oxyhemoglobin dissociation curve may be shifted to the right ­by A. Alkalosis, hyperthermia, and ­hypercapnia B. Acidosis, hypercarbia, and ­hyperthermia C. Acidosis, hypocarbia, and ­hypothermia 28. On a ventilator, a h ­ igh-­pressure limit alarm may sound i­ f A. The tubing is ­disconnected B. A leak in a chest tube ­occurs C. A pneumothorax may have ­occurred 29. A factor that increases pulmonary vascular resistance ­is A. Prostaglandin ­therapy B. ­Sepsis C. ­Hypoxia 30. The control variable on a ventilator refers t­ o A. The variable manipulated to cause ­inspiration B. The ventilatory ­patterns C. A preset maximum ­value 31. A methylxanthine used to treat apnea of prematurity ­is A. ­A lbuterol B. Solu-­Medrol C. ­Caffeine

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32. A distinguishing feature of transient tachypnea versus respiratory distress syndrome is ­that A. TTN requires mechanical ­ventilation B. TTN patients have virtually clear X-­rays C. Infants with TTN tend to have a normal or low ­pCO2 33. Infants at highest risk for retaining fetal lung fluid ­include A. Female ­infants B. Maternal ­sedation C. Those infants who were severely ­premature 34. Persistent pulmonary hypertension of the newborn (PPHN) is caused b ­y A. Low PVR and pulmonary artery ­pressures B. ­R ight-­to-­left shunting through fetal ­channels C. Increased SVR 35. The most common precipitating factor for developing PPHN ­is A. ­Hypoxia/asphyxia B. Bacterial ­sepsis C. Pulmonary parenchymal ­disease 36. Which of the following statements is true regarding ­early-­onset p ­ neumonia? A. Infants with ­early-­onset pneumonia will have a clear chest X-­ray B. A pleural effusion may ­occur C. ­Early-­onset pneumonia occurs after birth and prior to 3 days of ­life 37. Which of the following drugs is a ­lusitropic? A. ­Milrinone B. ­Dobutamine C. ­T heophylline 38. Which of the following pulmonary dilators is used for the treatment of pulmonary ­hypertension? A. ­Epinephrine B. ­Dopamine C. Inhaled nitric ­oxide 39. Infants can develop pulmonary air leaks while on mechanical ventilation. A risk factor for the development of an air leak would ­be A. PEEP set too l­ow B. ­Asynchrony C. Use of ­SIMV 40. Bronchopulmonary dysplasia primarily occurs in i­ nfants A. Only born at or near ­term B. Who have a preexisting lung ­disease C. Who are ­premature

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41. Congenital diaphragmatic hernia (CDH) ­is A. A condition that manifests itself about 5 hours after ­birth B. A condition that may lead to hypoxia and ­hypercarbia C. A condition that results in a ­left-­to-­r ight ­shunt 42. Mothers who receive MgSO4 have been identified as a risk factor for their infants. This is ­because A. Labor begins ­prematurely B. Respiratory depression in the infant can ­occur C. Fetal heart tones lose ­amplitude 43. Apnea of prematurity may be classified ­as A. ­Obstructive B. Medullary-­controlled C. ­Tangential 44. Potential treatment for apnea of prematurity ­includes A. Caffeine and ­theophylline B. ­Opioids C. ­Norepinephrine 45. The occurrence of pulmonary interstitial emphysema (PIE) is closely associated ­with A. ­A ntibiotics B. Maternal ­age C. ­Barotrauma 46. Herniation of abdominal contents into the chest cavity early in gestation accompanied by ipsilateral pulmonary hypoplasia is known a ­s A. Gunner’s ­syndrome B. Congenital diaphragmatic ­hernia C. Potter’s ­syndrome 47. Your newborn patient has been diagnosed with a congenital diaphragmatic hernia. Which of the following actions should be avoided when caring for this ­infant? A. Dopamine ­infusions B. Pulmonary ­vasodilators C. ­Bag-­mask ­ventilation 48. At delivery, you note that your patient has a small amount of discharge from the left nares and some mild cyanosis. The baby pinked up immediately when crying. You were unable to pass a catheter through the left nares while suctioning. It is probable this baby h ­ as A. Choanal ­atresia B. A cleft ­palate C. A small ­oropharynx

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49. Your patient just arrived from the delivery room. On admission, you note hyperresonant but severely decreased breath sounds in the right middle lobe. You are unable to visualize the area with the transilluminator. A subsequent ultrasound showed an overexpansion of the right middle lobe. These findings are suggestive ­of A. Bronchial ­dysplasia B. A tension ­pneumothorax C. Congenital lobar ­emphysema 50. Congenital lobar emphysema predominately affects which lung ­field? A. Right upper ­lobe B. Right middle ­lobe C. Left upper ­lobe 51. An intrinsic factor that may contribute to the pathogenesis of congenital lobar emphysema ­is A. An ­adenoma B. Pulmonary artery ­dilation C. Lymph node ­compression 52. You have just assisted with the delivery of a ­full-­term infant. The airway was cleared and the infant placed on a radiant warmer. The infant has no spontaneous respirations and the Apgar score is 3 at 1 minute. Your next nursing action should be ­to A. Administer ­naloxone B. Provide b ­ ag-­mask ­ventilation C. Begin chest ­compressions 53. A potential cause of neonatal asphyxia ­is A. Maternal age . 25 years ­old B. Compression of the umbilical cord during ­birth C. Respiratory ­alkalosis 54. You are caring for a 32-week gestation infant in acute respiratory failure. The patient is receiving O2 therapy and has ABGs scheduled every 4 hours. As a NICU nurse, you know that the arterial oxygen tension should be maintained ­between A. 40–60 mm ­Hg B. 50–80 mm ­Hg C. 70–90 mm ­Hg 55. The infant you are caring for will require oxygen therapy at home, so it is important the parents are t­ aught A. To secure oxygen tanks in a vertical ­position B. To have the child wear synthetic clothing to reduce the risk of static ­electricity C. To use alcohol based swabs to reduce oral ­dryness

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56. A male infant was born at 33 weeks gestation via cesarean section. Immediately after birth, he developed respiratory distress, requiring supplemental oxygen and mechanical ventilation. The chest ­X-­ray shows a diffuse “ground glass pattern.” The most likely cause of this infant’s RDS ­is A. Persistent pulmonary hypertension of the ­newborn B. Bronchopulmonary ­dysplasia C. Deficient ­surfactant 57. Which of the following statements is true regarding the administration of ­CPAP? A. CPAP may be administered via nasal ­prongs B. CPAP allows for a decrease in functional residual ­capacity C. CPAP provides decreased pressure to the posterior ­pharynx 58. An adverse effect of excessive CPAP ­is A. A sudden change in cerebral blood ­flow B. A rise in intrathoracic ­pressure C. Intraventricular ­hemorrhage 59. Which of the following statements is true regarding pressure ­ventilators? A. Pressure ventilators are only rarely used in ­NICUs B. Pressure ventilators deliver a constant tidal volume with each ­breath C. Pressure ventilators are ­pressure-­limited, constant flow, and time-­c ycled 60. One means of decreasing pCO2 when using a pressure ventilator is t­ o A. Add P ­ EEP B. Increase the ventilator ­rate C. Decrease the tidal ­volume 61. A disadvantage of using a pressure ventilator would b ­e A. ­Overdistention B. PEEP cannot be ­adjusted C. Changes in pressure are done on a timed ­c ycle 62. In a ­volume-­limited ventilator, an increase in ventilation may be achieved b ­y A. Decreasing the ventilator ­rate B. Increasing the delivered tidal ­volume C. Decreasing the ­PEEP 63. A disadvantage of using a volume ventilator would b ­e A. Very high positive inspiratory pressure may be ­necessary B. Tidal volumes must be set ­manually C. Hypotension is ­common 64. An action of nitric oxide ­is A. Vascular smooth muscle ­relaxation B. To increase pulmonary vascular ­resistance C. To release ­macrophages

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65. Your preterm patient has become increasingly restless. You note that the infant’s chest has become more ­barrel-­shaped and ­overdistended. Breath sounds have become distant on the left side. These findings are indicative ­of A. A tension ­pneumothorax B. A ­pneumomediastinum C. Pulmonary interstitial ­emphysema 66. A drug given to lessen the effects of respiratory syncytial virus ­is A. ­Palivizumab B. ­Curosurf C. ­Attenovir 67. The risk of pulmonary hemorrhage in an infant is increased if the infant is ­receiving A. ­Digoxin B. ­Adenosine C. ­Surfactant 68. If an infant is receiving lipids, pleural fluid may appear milky in color and be confused ­with A. A cardiac ­effusion B. ­Chylothorax C. Infectious e­ xudate 69. An uncommon cause of pulmonary hemorrhage in infants ­is A. ­Caffeine B. A bronchogenic ­c yst C. Sickle cell ­disease 70. A genetic disorder with cystic hygroma as a presenting disease ­is A. Fetal alcohol ­syndrome B. A ­parvovirus C. Turner ­syndrome 71. One of the nurses in your NICU is 16 weeks pregnant and tells you her fetus was diagnosed with a cystic hygroma. A cystic hygroma ­is A. A ­fluid-­filled ­sac B. A ­t umor C. A ­virus-­filled ­nodule 72. Your patient was diagnosed with Pierre Robin sequence. As a NICU nurse, you k ­ now A. The infant’s condition is often ­fatal B. This is a thoracic ­c yst C. The infant must be fed in the prone ­position

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73. If positive inspiratory pressure (PIP) is increased on a mechanical ventilator, what effect should this have on your patient’s blood ­gases? A. It will raise the PaCO2 and decrease the ­PaO2 B. It will lower the PaCO2 and lower the ­PaO2 C. It will decrease the PaCO2 and increase the ­PaO2 74. Retinopathy of prematurity (ROP) is considered to be the result ­of A. A complication of Trisomy ­13 B. Displaced sequence on chromosome ­15 C. High oxygen ­saturations 75. When utilizing transcutaneous PO2 measurements, some conditions may make the results unreliable. Underestimation of oxygenation ­may A. Occur when there is an air bubble between the electrode and the ­skin B. Occur when the alignment is ­improper C. Occur when skin is ­hypoperfused 76. Which of the following statements is true about ­high-­frequency jet ­ventilators? A. ­High-­f requency jet ventilators deliver ­high-­flow, ­short-­duration ­pulses B. No conventional breaths can be delivered during ­HFJV C. Exhalation is active and ­forced 77. An advantage of h ­ igh-­frequency ventilators over conventional ventilators i­ s A. Mean airway pressures do not have to be m ­ onitored B. HF ventilators can transport CO2 out of the lungs utilizing smaller ­pressures C. HF ventilators should not cause barotrauma because of lower ­pressures 78. Which of the following conditions may lead to inaccurate oxygen saturation values on a pulse ­oximeter? A. Cyanotic heart ­disease B. ­Hyperthermia C. Decreased peripheral ­perfusion 79. Your patient has undergone a diaphragmatic hernia repair. While preparing the infant for discharge, it is necessary to teach the parents about the possibility of long-­term A. Necrotizing ­enterocolitis B. Gastroesophageal ­reflux C. Spontaneous ­pneumothoraces 80. Which of the following statements about extracorporeal membrane oxygenation (ECMO) is ­true? A. ECMO is used for treatment of pulmonary ­hypoplasia B. ECMO replaces nitric oxide as the therapy of choice for ­PPHN C. ECMO should not be used in cases of intravascular ­hemorrhage

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Questions 81–93 require interpretation of arterial blood gas results. On occasion, exam writers will attempt to provide additional, unnecessary information in the stem of a question. The goal is simply to identify the acid–base ­abnormality. 81. Analyze the following arterial blood gas ­values pH ­7.38 CO2 ­27 HCO3 ­16 A. Compensated metabolic ­acidosis B. Compensated respiratory ­acidosis C. Uncompensated metabolic ­acidosis 82. Analyze the following arterial blood gas results from a 32-week gestation infant on 2L O2 via a nasal c ­ annula pH ­7.46 CO2 ­34 HCO3 ­24 A. ­Normal B. Compensated respiratory ­acidosis C. Uncompensated respiratory ­alkalosis 83. Analyze the following arterial blood gas from a 36-week gestation infant with Tetralogy of ­Fallot pH ­7.18 CO2 ­40 HCO3 ­15 A. ­Normal B. Compensated respiratory ­acidosis C. Uncompensated metabolic ­acidosis 84. Analyze the following arterial blood gas results from an infant with a ventricular septal ­defect pH ­7.56 CO2 ­25 HCO3 ­34 A. Uncompensated (mixed) respiratory/metabolic ­alkalosis B. Compensated respiratory ­acidosis C. Compensated metabolic ­acidosis 85. Analyze the following arterial blood gas ­values pH ­7.42 CO2 ­36 HCO3 ­23 A. Compensated respiratory ­acidosis B. ­Normal C. Compensated metabolic ­acidosis

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86. Analyze the following arterial blood gas ­values pH ­7.49 CO2 ­30 HCO3 ­22 A. Uncompensated respiratory ­alkalosis B. Compensated respiratory ­acidosis C. Compensated metabolic ­acidosis 87. Analyze the following arterial blood gas ­values pH ­7.37 CO2 ­68 HCO3 ­38 A. Uncompensated metabolic ­alkalosis B. Compensated respiratory ­acidosis C. Compensated metabolic ­acidosis 88. Analyze the following arterial blood gas ­values pH ­7.11 CO2 ­65 HCO3 ­17 A. Uncompensated (mixed) respiratory/metabolic ­acidosis B. Uncompensated metabolic ­alkalosis C. Compensated metabolic ­acidosis 89. Analyze the following arterial blood gas ­values pH ­7.43 CO2 ­31 HCO3 ­20 A. Uncompensated metabolic ­alkalosis B. Uncompensated respiratory ­alkalosis C. Compensated respiratory ­alkalosis 90. Analyze the following arterial blood gas ­values pH ­7.51 CO2 ­40 HCO3 ­35 A. Uncompensated metabolic ­alkalosis B. Compensated metabolic ­acidosis C. Uncompensated respiratory ­alkalosis 91. Analyze the following arterial blood gas from a term infant on room ­air pH ­7.17 CO2 ­55 HCO3 ­20 A. Uncompensated metabolic ­alkalosis B. Uncompensated (mixed) respiratory/metabolic ­acidosis C. Compensated metabolic ­acidosis

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92. Analyze the following arterial blood gas ­values pH ­7.38 CO2 ­38 HCO3 ­22 A. ­Normal B. Compensated respiratory ­acidosis C. Compensated metabolic ­acidosis 93. Analyze the following arterial blood gas ­values pH ­7.30 CO2 ­61 HCO3 ­25 A. Uncompensated metabolic ­alkalosis B. Uncompensated respiratory ­acidosis C. Compensated metabolic ­acidosis 94. A female infant was born at 30-weeks gestation and was diagnosed with surfactant deficiency syndrome. The infant has required 2 months of support via a mechanical ventilator and oxygen therapy. The infant will be discharged tomorrow on 0.25 L/min of oxygen. Which of the following statements is true regarding this infant’s lung ­function? A. The infant’s lung function will deteriorate over ­time B. This infant has an obstructive lung ­disease C. This infant is at high risk for ­choking 95. You are caring for a 24-week gestation female delivered vaginally yesterday. She was intubated after 6 attempts with a 2.0 ETT at 6.25 cm at lip. There was vocal cord and oral mucosal damage with bleeding. Arterial blood gases show worsening respiratory acidosis with RDS on chest ­X-­ray. Despite surfactant therapy, conventional ventilation is ineffective. Which mode of ventilation would be contraindicated for this ­patient? A. ­High-­f requency jet ­ventilation B. ­High-­f requency, ­flow-­interrupted ­ventilation C. ­High-­f requency oscillator ­ventilation 96. With which of the following ­high-­frequency ventilation modalities is exhalation ­active? A. ­High-­f requency, ­flow-­interrupted ­ventilation B. ­High-­f requency jet ­ventilation C. ­High-­f requency oscillator ­ventilation

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97. Baby Ester is a 25-week gestation neonate who is 3 days old. She was placed on ­high-­frequency jet ventilation (HFJV) for severe respiratory acidosis unresponsive to surfactant replacement and conventional ventilation. During your assessment, you note that the chest is not vibrating and there are secretions in the ETT. Which of the following statements is true regarding suctioning a patient on H ­ FJV? A. Suctioning may only be done when ventilation is in ­use B. Suction must be applied while inserting and withdrawing the ­catheter C. Suctioning is not possible due to the size of the ­ETT 98. You are verifying the settings on the ­high-­frequency jet ventilator used by your 25-week gestation patient. The servo control pressure is lower than reported. This ­means A. Your patient is improving and is ready to be changed to a conventional ­ventilator B. Your patient’s condition is deteriorating or the tubing is ­kinked C. This is irrelevant and means the humidity needs to be ­adjusted 99. Eric is a 28-week gestation infant placed on ­high-­frequency, ­flow-­interrupted ventilation 2 hours ago. His arterial blood gas shows a pH of 7.02, a CO2 of 76, a PaO2 of 50, a HCO3 of 24, and base excess of 20.4. You report the results to the neonatologist. Which of the following orders would you expect to receive as a result of these lab ­values? A. Increase positive inspiratory pressure (PIP) by 2 and repeat the arterial blood gas in 2 ­hours B. Decrease the positive ­end-­expiratory pressure (PEEP) by 2 and repeat the arterial blood gas in 4 ­hours C. Administer sodium bicarbonate 2 mEq/kg IV over 20 minutes and repeat arterial blood gas in 8 ­hours 100. The parents of your 23-week gestation patient are asking why the baby’s chest is vibrating while on h ­ igh-­frequency oscillator ventilation. You should tell the ­parents A. “The ventilator uses a ­microprocessor-­controlled pneumatic valve to create pulsation of airflow.” B. “The ventilator injects air into the ETT in short, rapid pulses that make the baby’s chest wobble.” C. “The ventilator uses a piston to move small amounts of air to and from the baby at a rapid rate, causing the chest to vibrate.” 101. The daily chest ­X-­ray taken of your patient on h ­ igh-­frequency oscillator ventilation indicates overinflation and pulmonary interstitial emphysema. You would anticipate which of the following changes to your present ventilator ­settings? A. Decrease the amplitude by 2 and draw a blood gas in 2 ­hours B. Increase mean airway pressure (MAP) by 1 and draw a blood gas in 2 ­hours C. Increase Hertz by 2 and draw a blood gas in 4 ­hours

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102. Aaron is a 29-week gestation neonate placed on ­high-­frequency ventilation for deteriorating respiratory status despite conventional ventilation. Aaron’s father asks you about the “wobble” and how fast his baby is breathing. He does not understand the relationship between Hertz and the number of breaths per minute. You would ­explain A. “One hertz represents 30 breaths per minute.” B. “One hertz represents 60 breaths per minute.” C. “One hertz represents 100 breaths per minute.” 103. Liquid ventilation using perfluorocarbons continues to be researched and evaluated for the use of rescue ventilation for extremely l­ ow-­birth-­weight neonates and neonates with pulmonary hypoplasia. Perfluorocarbons work b ­y A. Increasing alveolar surface ­tension B. Allowing for greater pressures to improve ­ventilation C. Recruiting ­alveoli 104. Which of the following interventions may have the greatest impact in reducing ­ventilator-­associated pneumonia (VAP) in ­neonates? A. ­Education B. Daily ventilator circuit ­changes C. Patient ­positioning 105. H ­ igh-­frequency ventilators have a “sigh” setting. This setting is used ­to A. Deliver respirations to fully exchange gases within the ­lungs B. Decrease microatelectasis and recruit ­alveoli C. Assess lung sounds apart from ventilator ­sounds 106. Which nursing intervention is critical to maintaining patency and proper functioning of ­high-­frequency jet ­ventilation? A. Irrigate the jet port with 0.5 ml of normal saline every 3–4 ­hours B. Kink the tubing when turning the infant to prevent a drop in ­pressures C. Monitor respiratory rate every ­hour 107. Antenatal corticosteroids affect lung maturation and help prevent respiratory distress syndrome. Steroids work ­by A. Thickening the intraalveolar ­septa B. Accelerating the rate of glycogen ­depletion C. Reducing the number of lamellar bodies inside the ­cells 108. Which of the following statements is true about infant ­stress? A. Steroids must be administered to all premature infants born at 35 weeks or ­less B. Glucocorticoids should be routinely used for cases of infant ­stress C. Chronic stress in utero usually produces an infant small for gestational ­age

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109. Retractions may best be described ­as A. The chest wall caving inward with a large increase in pleural ­pressure B. The chest wall caving inward with a small decrease in pleural ­pressure C. The chest wall caving inward with a moderate decrease in pleural ­pressure 110. When caring for an infant with an expiratory grunt, the i­ nfant A. Needs to be intubated ­immediately B. Requires monitoring of vital signs every ­hour C. Is exhaling against a closed ­glottis 111. Which of the following actions should be done routinely when caring for an infant with a pulse ­oximeter? A. Eliminate all arterial blood gas draws to prevent unnecessary pain for the ­infant B. Use a disposable ­cuff C. Check the disposable sensor q 24 ­hours 112. Baby F. is a 37-week gestation infant at 5 days of life. He has been receiving antibiotics for a respiratory infection for the past 4 days. When you change his diaper, you note some small, bright red, raised areas on his buttocks. In the buccal area in his mouth, you note white plaques and you suspect that Baby F. is suffering ­from A. ­T hrush B. Strep ­throat C. Herpes ­virus 113. An important consideration when teaching a parent about the care of an infant with thrush ­is A. Use a ­cotton-­tipped applicator to swab the ­plaques B. Swab skin lesions with gentian ­violet C. Swab oral lesions with gentamicin ­ointment 114. Which of the following considerations should the nurse know prior to the administration of v ­ ancomycin? A. Vancomycin is ­nephrotoxic B. Vancomycin is specific for Staphylococcus ­aureus C. Vancomycin should always be given with an ­aminoglycoside 115. Baby Luke has respiratory distress syndrome. He has been grunting and having retractions, and now he has a respiratory rate of 701 and is exhibiting “seesaw” respirations. Which of the following statements explains the ­phenomenon? A. Seesaw respirations are an ­up-­and-­down motion of the chest ­wall B. Seesaw respirations are a sign of improvement in the baby’s ­condition C. Terminal airways are kept open by forcing the expansion of the ­lungs

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116. You are part of the patient safety team for your NICU and are responsible for evaluating compliance with ­hospital-­acquired infection procedures. To prevent ­ventilator-­associated pneumonia (VAP) in the NICU, the Centers for Disease Control and Prevention recommends that respiratory equipment that comes in direct contact with patient mucosal membranes ­be A. ­Wet-­heat pasteurized at temperatures . 158° F for 30 minutes and packaged sterile for next ­use B. Washed with sterile normal saline at 90° F for 5 minutes prior to ­use C. Steam sterilized or autoclaved by the facility regardless if the item is multiuse or single ­use 117. Precautions used when handling respiratory equipment or providing respiratory patient care i­ nclude A. Using an ­alcohol-­based antiseptic agent, if your hands are visibly soiled prior to, during, and after patient ­care B. Using an antimicrobial soap and water prior to and after providing patient ­care C. Only decontaminating your hands after providing care if gloves are ­worn 118. Virginia is a 23-week gestation neonate at 80 days of life with cerebral palsy. She is ventilator dependent. You are preparing to provide tracheostomy care. Which of the following statements is true regarding ­ventilator-­associated pneumonia (VAP) precautions during tracheostomy ­care? A. Tracheostomy care must be performed ­sterilely B. Tracheostomy care requires gloves and gown with aseptic ­technique C. Suctioning is never required as the tube is being ­changed 119. ­Ventilator-­associated pneumonia (VAP) precautions recommend that patients receiving enteral feedings while intubated on mechanical ventilation ­should A. Be fed using the smallest feeding tube available to prevent ­aspiration B. Always receive continuous feedings to prevent gastric distention and ­aspiration C. Be positioned at a 15–45° angle if not medically ­contraindicated 120. You have just received the report and are evaluating your patient’s environment. Which of the following findings supports current VAP prevention ­guidelines? A. The ­single-­patient-­use resuscitation bag is in the patient’s ­isolette B. The oral suction catheter is in a sealed bag at the foot of the ­bed C. The suction canisters for both the endotracheal line and the oral suction line are clean and ­labeled 121. Gentamicin is useful in the treatment ­of A. ­Gram-­positive ­rods B.  Pseudomonas ­aeruginosa C.  Bacillus

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122. You are told that the mother of your 31-week gestational age patient received steroids prior to giving birth. You know that this medication was probably prescribed because of the need ­to A. Decrease fetal tissue and alveolar ­surfactant B. Increase infant glucose ­levels C. Decrease fetal mortality ­rate 123. The mitral and tricuspid valves open ­during A. ­Diastole B. ­Systole C. ­A systole 124. Your 28-week gestation patient is intubated and being maintained using a ventilator at IMV 20, PIP 12, PEEP 4, at 40% FiO2 with a respiratory rate of 22. You just drew an ABG with the following results: pH 7.25, PaCO2 70, HCO3 27, Base Excess 2, and a PaO2 45 with saturation of 90%. Based on this arterial blood gas result, you would anticipate which of the following ventilator ­changes? A. Decrease PIP and increase P ­ EEP B. Decrease PEEP and increase IMV ­rate C. Increase PIP and increase IMV ­rate 125. You are caring for a 26-week gestational age patient on ­high-­frequency ventilation with transcutaneous CO2 monitoring (TCOM). You notice a sudden increase in PaCO2 levels with a sudden decrease in PaO2 levels. You s­ uspect A. Tension ­pneumothorax B. ­PDA C. ­VSD 126. The mother of a 900-gram, 27-week gestation infant asks when the baby will have her first ROP exam. You tell her the examination will be ­scheduled A. In 2 ­weeks B. When the baby is at 30 weeks gestation or 4 weeks of life, whichever is ­sooner C. At 32 weeks gestational age or 5–6 weeks of life, whichever is ­greater 127. A complication of persistent pulmonary hypertension of the newborn i­ s A. Renal ­failure B. Systemic ­hypertension C. ­Apnea 128. When managing a patient with persistent pulmonary hypertension, the pH should be maintained between while the PaCO2 should be between . A. 7.35 and 7.45; 40 and ­50 B. 7.45 and 7.6; 20 and ­30 C. 7.30 and 7.40; 50 and ­70

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129. You are preparing to discharge your patient to his home. If an infant requires oxygen at home, the parents should be taught ­to A. Secure oxygen tanks in a horizontal ­position B. Have the child wear synthetic clothing to reduce static ­electricity C. Use ­lemon-­glycerin swabs to reduce oral ­dryness 130. Pulmonary interstitial emphysema (PIE) may be treated w ­ ith A. ­Cefotaxime B. ­Clonidine C. ­Dexamethasone 131. A male infant was born at 32-weeks gestation via cesarean section to a mother in active labor with complete placenta previa. Soon after birth, he developed respiratory distress requiring supplemental oxygen and mechanical ventilation. The chest ­X-­ray shows decreased lung volumes and a diffuse “ground glass pattern” with air bronchograms. Which of the following is the most likely cause of respiratory distress syndrome (RDS)? A. Persistent pulmonary hypertension of the newborn (PPHN) B. Deficient ­surfactant C. Fluid retention in the ­lungs 132. You are evaluating a 3-­day-­old infant with significant respiratory distress. He was delivered by emergency cesarean section at 42-weeks gestation because of fetal distress. You note that he has an oxygen saturation of 76% in room air that increases to 95% with administration of 100% oxygen. Which of the following statements is most accurate regarding persistent pulmonary hypertension of the newborn (PPHN)? A. PPHN occurs most frequently in premature infants but may occur in ­post-­term ­infants B. PPHN usually resolves ­spontaneously C. Adequate oxygenation is the best preventive measure and ­treatment 133. Appropriate treatment for a bronchospasm would ­include A. ­Furosemide B. ­Digoxin C. ­A lbuterol 134. An infant who is receiving corticosteroids should be observed for development ­of A. Respiratory ­alkalosis B. Pulmonary ­edema C. ­Hypertension 135. A complication of nitric oxide therapy ­is A. Metabolic ­alkalosis B. ­Methemoglobinemia C. ­Apnea

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136. Although surfactant levels may be low, at what gestational age is the fetal lung first capable of supporting extrauterine ­life? A. 18–23 ­weeks B. 26–28 ­weeks C. 24–25 ­weeks 137. An early sign(s) of respiratory distress in a neonate may ­include A. ­Hypotension B. Grunting, ­retractions C. Respiratory rate of 30–45 ­breaths/minute 138. Apnea that is refractory to methylxanthine therapy may be treated successfully ­with A. ­A minophylline B. ­Doxapram C. ­T heophylline 139. Leah was in a car accident when she was 16 weeks pregnant. She suffered neurologic injuries and was paralyzed for 2 weeks while intubated. Her baby is now at 35 weeks gestation and Leah is in labor. The neonate maybe at risk ­for A. Pulmonary ­hypoplasia B. Necrotizing ­enterocolitis C. Renal ­agenesis

This concludes the Pulmonary System ­questions.

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Pulmonary System ­Answers 1. Correct answer: ­C Type II pneumocytes produce surfactant. Surfactant is a lipoprotein and functions by increasing surface tension of alveoli and allows alveoli to expand and contract. We should have some residual pressure in the alveoli at the end of respiration to keep the alveoli open (physiologic PEEP). If surfactant production is impaired, the alveoli’s ability to exchange O2 is compromised. Type I cells line the outside of the ­alveoli. 2. Correct answer: ­A If you hear faint breath sounds on the left side of the chest and normal sounds on the right side immediately after your patient has been intubated, most likely the right mainstem bronchus has been intubated. The right mainstem bronchus is somewhat wider and has less of an angle off the mainstem bronchus, so it is much more readily ­intubated. 3. Correct answer: ­A The pH is outside the normal range, so it is uncompensated. The pH is elevated, indicating alkalosis. The HCO3 is normal, the PaCO2 is decreased, and that indicates respiratory ­alkalosis. 4. Correct answer: ­B Most ABG machines are calibrated to 37° C. If the patient has a fever, the oxyhemoglobin curve will be shifted to the right. More oxygen will be given off to the tissues, so the machine has to be calibrated to account for the ­temperature. 5. Correct answer: ­B Chronic hypoxia leads to chronic respiratory acidosis. The kidneys then retain bicarbonate in the form of sodium bicarbonate. The bicarbonate is exchanged for sodium chloride. Ammonia is an acid, and excess amounts must be removed from the body. This is done by releasing ammonium chloride. In chronic hypoxia, there is an increase in bicarbonate levels and a decrease in chloride levels. Other causes of hypochloremia are NG suction, vomiting, and ­diarrhea. 6. Correct answer: ­B Hypoxemia is a decreased oxygen level in the arterial blood or a PaO2 of , 80 mm Hg. Option A defines ­hypoxia—­a decreased oxygen level at the cellular ­level. 7. Correct answer: ­B In addition to RDS and MAS, four days of high FiO2 has resulted in a nitrogen washout, and atelectasis. Nitrogen’s high partial pressure is necessary to maintain alveolar inflation. It is important to titrate FiO2 to maintain saturations within a prescribed range when oxygen therapy is utilized. Pulmonary edema would result in coarse breath sounds. With a unilateral pneumothorax, there would probably be tracheal ­deviation.

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8. Correct answer: ­C The cells that are responsible for forming a barrier for alveoli are type I epithelial ­cells. Type I cells line the outside of the alveoli and are easily inflamed by inhaled toxins or heated air. In addition, type I cells maintain the blood–gas interface. Type II cells produce ­surfactant. 9. Correct answer: ­C. Anatomic dead space is defined as a conducting airway. Conducting airways (e.g., ­trachea, bronchi) are ventilated, but perfusion (gas exchange) does not take place. Wasted ventilation (option B) is the amount of ventilation that does not participate in gas e­ xchange. 10. Correct answer: ­A The oxyhemoglobin dissociation curve is a curve that reflects physiologic circumstances and their effect on the hemoglobin’s affinity for ­oxygen. 11. Correct answer: ­B A shift to the right caused by a decreased pH level means hemoglobin has less affinity for oxygen. In other words, O2 will be given off more readily to the ­tissues. 12. Correct answer: ­B When the patient is alkalotic, hemoglobin holds onto the ­oxygen. 13. Correct answer: ­A Chronic hypoxia usually results in decreased chloride levels. The kidneys try to correct the imbalance by retaining bicarbonate. Chronic hypoxia results in increased CO2 , creating a chronic respiratory acidosis. The bicarbonate exchanges for the chloride to maintain a ­balance. 14. Correct answer: ­C Prematurity is present in almost all cases of respiratory distress syndrome. Surfactant production is usually diminished, and this leads to atelectasis and pulmonary edema. The patient worsens, an alteration in the ventilation perfusion ratio occurs, and functional residual capacity is diminished. Serum protein leaks into the alveoli and inhibits surfactant function. The immature lung lacks the ability to clear this fluid, and becomes compromised because of decreased surface area for gas exchange. The collapsed alveoli fill with a ­protein-­r ich fluid that forms hyaline ­membranes. Spasm of the pulmonary arteries results in blood being shunted away from the lungs via the foramen ovale and ductus arteriosus. These abnormalities all result in respiratory failure with poor oxygenation of the ­blood. 15. Correct answer: ­B Retractions can best be seen at the xiphoid and intercostal markings of the infant’s chest. Pleural pressure normally decreases during inspiration. When a disease that affects the parenchyma exists, the chest wall produces more negative pressure. The chest wall is very compliant, so it caves inward in the presence of a moderate decrease in pleural pressure, which results in ­retractions.

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16. Correct answer: ­A In an infant, respiratory distress may lead to hypoglycemia. Hypoglycemia results from increased glucose consumption secondary to the increased work of ­breathing. 17. Correct answer: ­C Answers A and B are not signs; they are tools for diagnostics. Grunting is frequently seen in infants with RDS because the infants are trying to prevent alveolar collapse. The grunting sound is produced from the closure of the glottis in the attempt to maintain normal functional residual capacity. Other signs might include tachypnea (above 60 per minute), retractions, flaring of nares, open mouth (decreases resistance), cyanosis on room air; PaO2 below 60 mm Hg (torr) in FIO2 . 0.4, reduced air entry, stridor, and ­apnea. 18. Correct answer: ­B Epinephrine may be useful in the treatment of TTN. Endogenous catecholamines assist the lung epithelium to transition, in the first few minutes after birth, from a secretory to an absorptive mode by inducing active sodium transport across the pulmonary epithelium. Catecholamines aid in enhancing absorption of fetal lung fluid, so it is plausible that a relative catecholamine deficiency plays a role in the etiology of TTN, and that administration of exogenous catecholamines may be an effective treatment. Delivery by cesarean section, especially prior to the onset of labor, is associated with an increased risk of TTN. Levels of circulating catecholamines are higher in newborns delivered vaginally than in newborns delivered by cesarean section, possibly due to the stimulation of catecholamine release from the stress of ­labor. 19. Correct answer: ­A Risk factors for transient tachypnea of the newborn (TTN) include maternal diabetes, maternal asthma, male gender, and cesarean delivery. TTN is the most common cause of neonatal respiratory distress. TTN occurs when residual pulmonary fluid remains in fetal lung tissue after delivery. Prostaglandins released after delivery dilate lymphatic vessels to remove lung fluid as pulmonary circulation increases with the first breath. When fluid remains and is not eliminated, TTN can ­occur. 20. Correct answer: ­B Although sterile, meconium is locally irritating, can obstruct the air passages, and is a medium for bacterial growth. Meconium passage indicates fetal distress and hypoxia in utero. Meconium aspiration syndrome causes significant respiratory distress immediately after delivery. ­Meconium-­stained amniotic fluid occurs in approximately 15% of deliveries, causing meconium aspiration syndrome in the infant, usually in term and ­post-­term infants. Meconium is composed of desquamated cells, secretions, lanugo, water, bile pigments, pancreatic enzymes, and amniotic ­fluid. 21. Correct answer: ­C PEEP is useful in RDS because it can help open collapsed alveoli. Lowered cardiac output is a complication of PEEP. PEEP can cause barotrauma. PEEP must be regulated so as not to cause barotraumas, but still keep alveoli from collapsing during ­expiration.

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22. Correct answer: ­C Falsely low readings on a pulse oximeter may be due to phototherapy. If the infant is hypothermic or has decreased peripheral perfusion, the results will be inaccurate. Position of the probe and patient motion can also cause inaccurate readings on a pulse ­oximeter. 23. Correct answer: ­A The left upper lobes can be drained initially utilizing gravity by placing the patient in a s­ emi-­reclining ­position. 24. Correct Answer: ­B Because PEEP raises intrathoracic pressure and PVR, blood backs up and can cause hepatic congestion. The increased intrathoracic pressure also can compress blood vessels, cause or exacerbate hypovolemia, and cause low cardiac ­output. 25. Correct Answer: ­A Any pressure in the thorax decreases preload, cardiac output, and blood pressure. Forward blood flow is impeded by increased pressure in the pulmonary vasculature. A pulmonary air leak could occur from the increased pressure caused by ­PEEP. 26. Correct answer: ­B Methylxanthines are an important classification of drugs. In addition to theophylline, caffeine and theobromine are also methylxanthines. They can be found in coffee, tea, and cocoa. Low doses of drugs in this classification can stimulate cortical arousal and in higher doses cause insomnia. They can cause tachycardias and increase production of gastric acid and digestive enzymes. Methylxanthines inhibit histamine ­release. 27. Correct answer: ­B Acidosis, hypercarbia, and hyperthermia will all lead to a right shift in the oxyhemoglobin dissociation curve. Hemoglobin in this instance has a decreased affinity for oxygen and enhances tissue uptake of ­oxygen. 28. Correct answer: ­C On a ventilator, a ­high-­pressure-­limit alarm may sound if a pneumothorax may have ­occurred. Due to increases and changes in thoracic pressure in the presence of a pneumo­ thorax, a ­high-­pressure alarm will sound to alert staff. In addition, alarms for saturation and possibly heart rate may alarm on the cardio respiratory monitor. A ­low-­limit alarm may sound if tubing is disconnected or a leak in the chest tube ­occurs. 29. Correct answer: ­B Sepsis may result in lung tissue injury and resultant increased pulmonary vascular resistance (PVR). Prostaglandin and oxygen therapies result in pulmonary vasodilatation and decrease ­PVR. 30. Correct answer: ­A Examples of control variables include pressure, volume, and flow. This value does not change with changes in patient lung compliance or ­resistance.

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31. Correct answer: ­C Methylxanthines have become part of the routine clinical management of apnea of prematurity, with caffeine the treatment of choice because of its long ­half-­life in the ­neonate. Other physiologic consequences of caffeine, such as increased catecholamine levels leading to increased cardiac output and oxygenation, might explain the beneficial effects of caffeine in premature ­infants. 32. Correct answer: ­C In an infant with TTN, the pCO2 tends to be lower than in an infant with RDS. TTN may require ­positive-­pressure support of short ­duration. 33. Correct answer B ­ Infants at highest risk for retaining fetal lung fluid include those whose mothers were ­sedated. Maternal sedation is a risk factor for TTN, along with cesarean delivery without labor, breech delivery, emergent delivery, second twin, macrosomia, maternal asthma, and delayed cord clamping. The delay in clamping the cord results in a transfusion of blood to the infant that will elevate the CVP. Pulmonary artery pressures may elevate as well. If hypoxemia and tachypnea continue, the infant may develop persistent pulmonary hypertension of the newborn. Males tend to develop TTN more than females. TTN affects infants at or near ­term. 34. Correct answer: ­B PPHN is the result of ­r ight-­to-­left shunting through the fetal shunts at the atrial and ductal levels. The PVR and pulmonary artery pressures are also elevated. The high PVR and pulmonary hypertension restrict pulmonary blood flow, and this, in turn, promotes hypoxemia, acidemia, and lactic acidosis. Most newborns are diagnosed within the first 24 hours of ­life. 35. Correct answer: ­A The most common precipitating factor for developing PPHN is ­hypoxia/asphyxia. Hypoxia and asphyxia are correlated with remodeling small pulmonary arteries and abnormal muscularization. Persistent pulmonary vasospasm may be caused by asphyxia. Although pulmonary parenchymal diseases (pneumonia, meconium aspiration, RDS, and aspiration syndromes) may also cause pulmonary vasospasm, it is not the most common cause of PPHN. Bacterial sepsis will rarely lead to ­PPHN. 36. Correct answer: ­B Early-­onset pneumonia is acquired transplacentally or during labor or delivery. Ascending infection is particularly likely if there is prolonged rupture of the membranes, causing pleural effusions. Infants with transplacentally acquired infection present at birth, and those infected with organisms acquired from the birth canal present within the first 48 hours after birth. Infants with ­early-­onset pneumonia have respiratory distress with nonspecific signs, such as poor feeding and irritability, and they may be febrile or ­hypothermic.

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37. Correct answer: ­A Milrinone is a class III phosphodiesterase inhibitor with inotropic, lusitropic (myocardial relaxation), and vasodilator ­properties. 38. Correct answer: ­C Inhaled nitric oxide (iNO) is a selective pulmonary vasodilator to treat pulmonary hypertension. In infants born at or near term, iNO reduces death or the need for extracorporeal membrane oxygenation (ECMO), but the positive effect is in reducing the ECMO requirement. Do not use iNO on patients who are dependent on a ­right-­to-­left ­shunt. 39. Correct answer: ­B Many NICUs use conventional mechanical ventilators that deliver intermittent ­positive-­pressure inflations and positive ­end-­expiratory pressure at a preset rate, which may be out of synch with the infant’s respiratory efforts. Asynchrony has been shown to be a risk factor for the development of air leaks. The resultant barotrauma might increase bronchopulmonary dysplasia (BPD). 40. Correct answer: ­C BPD tends to affect infants with a predisposition to premature birth, family history, and/or a genetic susceptibility. BPD can occur in infants with mild or no initial lung disease in the first week after birth, but occurs more commonly in infants with severe lung disease complicated by air leak, PDA, and/or infection. These problems expose infants to the potential problems of volutrauma and oxygen toxicity. At a consensus conference of the National Institutes of Health, it was agreed that babies should be diagnosed as having BPD if they remained oxygen dependent for at least 28 days and then classified as suffering from mild, moderate, or severe BPD according to their respiratory support requirement at a later ­date. 41. Correct answer: ­B Congenital diaphragmatic hernia is a herniation of the abdominal viscera through the diaphragmatic defect during fetal life. The herniation impairs lung development, causing pulmonary hypoplasia. This affects the number of bronchial divisions and alveoli, the pulmonary vasculature, and both lungs are affected. The contralateral (opposite) lung is affected to a lesser degree. The decreased alveolar surface and reduced available vascular area lead to hypoxia, hypercarbia, and pulmonary hypertension after birth. Not only is the pulmonary vascular area reduced, but there is also increased thickness of the peripheral pulmonary arteries, into intraacinar arteries. This results in increased vulnerability to pulmonary vasoconstriction that, in turn, results in pulmonary hypertension and ­r ight-­to-­left shunting through the foramen ovale and the ductus ­arteriosus. 42. Correct answer: ­B In utero exposure to MgSO4, especially in cumulative doses higher than 10 g, is an independent risk factor for PIE in ELBW infants. Infants with PIE had lower Apgar scores (which suggested depression) and hypotonia in the immediate postnatal period. Both of these conditions could be related to the toxicity of ­MgSO4.

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43. Correct answer: ­A Apnea of prematurity is defined as cessation of breathing by a premature infant that lasts for more than 20 seconds and/or is accompanied by hypoxia or bradycardia. Apnea is traditionally classified as obstructive, central, or mixed. Obstructive apnea may occur when the infant’s neck is hyperflexed or hyperextended. Some of the causes of apnea include infection, anemia, metabolic conditions, hypoxemia, drugs, and neural ­immaturity. 44. Correct answer: ­A Caffeine and theophylline are methylxanthines. Xanthines may have a central stimulatory effect on brainstem respiratory neurons. Xanthines may decrease the severity and frequency of apneic episodes. Xanthines can be toxic, so some of the symptoms may include tachycardia, diuresis, vomiting, or CNS ­effects. 45. Correct answer: ­C Positive-­pressure ventilation and reduced lung compliance are significant predisposing factors for the development of PIE. Infants are often mechanically ventilated for other conditions such as respiratory distress syndrome (RDS), meconium aspiration syndrome (MAS), amniotic fluid aspiration, and infection. However, in extremely premature infants, pulmonary interstitial emphysema can occur at low mean airway pressure and probably reflects increased sensitivity of the stretch in an underdeveloped lung. PIE has been rarely reported in the absence of mechanical ventilation or continuous positive airway pressure. PIE is a collection of gases inside the connective tissue surrounding the lung s­ tructures. 46. Correct answer: ­B In congenital diaphragmatic hernia, there is also some contralateral hypoplasia of the lung. The abdomen is usually scaphoid, and breath sounds are often absent on the side of the defect. In about 90% of cases, the defect is on the left side. Bowel sounds may be heard in the chest. Heart sounds may be heard on the right side because the herniated abdominal contents shift the mediastinum to the ­r ight. 47. Correct answer: ­C Bag-­mask ventilation should be avoided with congenital diaphragmatic hernia. The herniated bowel can fill with gas and compress the lungs, making ventilation more difficult. ECMO and pulmonary vasodilators have limited effect. Dopamine may be necessary for the treatment of ­hypotension. 48. Correct answer: ­A Choanal atresia is a curable condition. If both nares were blocked, the baby would have to be emergently intubated. Babies can learn to mouth breathe, and reconstructive surgery may be postponed while the baby is learning to ­breathe. 49. Correct answer: ­C Congenital lobar emphysema is caused by bronchial narrowing. Weakened or absent bronchial cartilage is present, so that there is inspiratory air entry but collapse of the narrow bronchial lumen during expiration. This bronchial defect results in lobar

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air trapping. Surgery is indicated in most cases, but rarely the condition may resolve with ventilatory therapy. Emergency lobectomy may be required if the lobe cannot be decompressed by intubation of the opposite side or by placing the infant in the decubitus position with the involved lobe ­dependent. 50. Correct answer: ­C In congenital lobar emphysema, the left upper lobe is affected 41% of the time, the right middle lobe 34% of the time, and the right upper lobe 21% of the time. There are rare cases of both lungs affected. Congenital lobar emphysema has two forms: hypoalveolar (affecting less than the expected number of alveoli) and polyalveolar (affecting greater than the expected number of alveoli). 51. Correct answer: ­A An intrinsic factor that may contribute to the pathogenesis of congenital lobar emphysema is an adenoma. Answers B and C are extrinsic factors to the development of congenital lobar emphysema. Other intrinsic factors are infection, tuberculosis, retained secretions, absence of cartilage, stenosis, and ­hypoplasia. 52. Correct answer: ­B Airway management is your priority as this infant had no spontaneous ­respirations. 53. Correct answer: ­B According to the World Health Organization, asphyxia neonatorum is one of the leading causes of newborn deaths in developing countries, in which 4–9 million cases of newborn asphyxia occur each year, accounting for about 20% of the infant mortality rate. There are certain ­high-­r isk pregnancies that can contribute to possible asphyxia and they include maternal sedation, maternal age , 16 years old or . 40 years old, maternal alcohol abuse or smoking, and maternal illness such as diabetes. Additional factors include maternal hypertension, cord compression, anemia, and a history of previous abortions or stillbirths. Preterm labor is also a potential cause as are socioeconomic status and prenatal ­care. 54. Correct answer: ­B In preterm infants, the arterial oxygen tension should be maintained between 50 mm Hg and 80 mm ­Hg. 55. Correct answer: ­A Oxygen tanks should be stored in the upright position. Synthetic materials often cause static electricity. Only ­lemon-­glycerin swabs and non­–­alcohol-­based swabs should be ­used. 56. Correct answer: ­C RDS in this patient is most likely caused by a deficiency of surfactant leading to the “ground glass” pattern seen on ­X-­ray. ­Ground-­glass opacity is a nonspecific, but highly significant finding because 60–80% of patients with ­ground-­glass opacity on ­X-­ray have an active and potentially treatable lung disease. In the other 20–40% of the cases, the lung disease is not treatable and the ­ground-­glass pattern is the result of ­fibrosis.

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57. Correct answer: ­A Continuous positive airway pressure (CPAP) may be delivered by nasal prongs, nasopharyngeal tubes, or the endotracheal tube. CPAP provides increased pressure to the posterior pharynx and increases transpulmonary pressure. CPAP can prevent alveolar collapse and helps prevent obstructive ­apnea. 58. Correct answer: ­B Excessive CPAP may increase intrathoracic pressure to the point of compressing the right atrium and vena cava. The preload will be decreased and cardiac output will be ­reduced. 59. Correct answer: ­C Neonates can only generate very small inspiratory efforts. Pressure ventilators provide a constant flow of gas through an endotracheal tube. Breaths are provided at fixed intervals, and a peak inspiratory pressure (PIP) is maintained throughout ­inspiration. 60. Correct answer: ­B If a patient is retaining pCO2 , it is possible to help wash it out by increasing the respiratory rate (the ventilator rate in this case) or increase the tidal ­volume. 61. Correct answer: ­A A disadvantage of using a pressure ventilator would be possible overdistention. Lung compliance can change fairly quickly. Compliance can be likened to the elasticity of the lung. If it is less compliant, it can be described as stiffer. If it is more compliant, it can be said to be easier to inflate and deflate. If a pressure ventilator is used and the lung becomes more compliant, the alveoli and lung may overdistend. If the lung is less compliant, less tidal volume will be delivered because the ventilator only delivers to a preset ­volume. 62. Correct answer: ­B In a v ­ olume-­limited ventilator, it is possible to increase ventilation by increasing the delivered tidal volume or increasing the ventilator rate. Oxygenation can be increased by increasing the PEEP, FiO2 , or tidal ­volume. 63. Correct answer: ­A If a lung changes compliance, the volume ventilator will continue to deliver a fixed volume. This could lead to use of very high ­PIPs. 64. Correct answer: ­A An action of nitric oxide is causing vascular smooth muscle relaxation. Nitric oxide (NO) is the molecule released from the endothelium that enables smooth muscle relaxation. NO inhibits platelet aggregation and adherence and is thought to alter vascular permeability. NO also may participate in nonspecific immunity because it is generated when macrophages are ­activated.

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65. Correct answer: ­B This infant probably had some degree of PIE initially. However, the air has migrated from the ruptured alveoli and has tracked to the mediastinum, causing a pneumomediastinum. If a tension pneumothorax existed, the infant would desaturate, become hypotensive and bradycardic, and be ­c yanotic. 66. Correct answer: ­A Two medications are recommended for babies at high risk for RSV to protect them against the serious complications of the illness. During the peak of RSV season, either respiratory syncytial virus immune globulin intravenous ­(RSV-­IGIV) or palivizumab (an antibody against RSV) is usually given monthly. These medications are not vaccines and do not prevent the virus. But they do lessen the severity of the illness and may help shorten the hospital stay. There are no medications used to treat the virus itself. Care of a baby with RSV involves treating the effects of the virus on the respiratory system. Because a virus causes the illness, antibiotics are not ­useful. 67. Correct answer: ­C The risk of pulmonary hemorrhage is increased about 5% if the infant is receiving surfactant. Suction can cause bleeding because the respiratory endothelium is damaged. Pulmonary hemorrhage is rarely isolated and often accompanies other conditions such as RDS, aspiration of gastric contents or maternal blood, asphyxia, or ­DIC. 68. Correct answer: ­B Chylothorax is an accumulation of lymphatic fluid. Chylothorax may be congenital or acquired. If it is acquired, it may be secondary to a perforated or obstructed thoracic duct. It also may be a surgical complication due to repair of a congenital heart defect, diaphragmatic hernia, or a tracheoesophageal fistula. If the chylothorax is congenital, it may impede ventilatory efforts in a ­newborn. 69. Correct answer: ­B Bronchogenic cysts, especially when they are infected, can be the source of a pulmonary hemorrhage. Thoracic gastroenteric cysts have gastric mucosa and secrete acid. The cyst wall can be eroded and the erosion can extend to adjacent bronchial walls, vessels, or lung parenchyma, resulting in massive bleeding. Lung abscesses, fungal infections, allergic bronchopulmonary aspergillosis, and lung parasitic infections can all produce massive pulmonary bleeding from erosion of airway and vessel walls. Bleeding from sickle cell disease usually occurs in ­adolescents. 70. Correct answer: ­C The majority of prenatally diagnosed cystic hygromas are associated with Turner syndrome. Turner syndrome is a chromosome abnormality in which a female has only one X chromosome instead of two. Cystic hygroma is also associated with chromosome abnormalities such as trisomies 13, 18, and 21, as well as Noonan syndrome. The pattern of inheritance for these syndromes varies depending upon the specific syndrome. Rare cases of cystic hygroma can be inherited as an autosomal recessive disorder for which parents are carriers. A cystic hygroma can also be idiopathic. Parvovirus and fetal alcohol syndrome are potential causes of cystic ­hygromas.

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71. Correct answer: ­A Cystic hygromas are ­fluid-­filled sacs that result from a blockage in the lymphatic system. Cystic hygromas are single or multiple cysts found mostly in the neck region. A cystic hygroma in a developing baby can progress to hydrops and eventually fetal death. Some cases of congenital cystic hygromas resolve, leading to webbed neck, edema, and lymphangiomas. Sometimes, the hygroma can progress in size to become larger than the ­fetus. 72. Correct answer: ­C Pierre Robin sequence is a condition present at birth in which an infant has a very small lower jaw, a tongue that tends to fall back and downward, and a soft cleft palate. The infant may choke, so feeding in the prone position may be necessary. Also, the infant should not be placed on its back. The mandible may grow and resolve when the infant reaches 6–12 months of ­age. 73. Correct answer: ­C If positive inspiratory pressure (PIP) is increased on a mechanical ventilator, the result will be a decrease in the PaCO2 and an increase in the ­PaO2 . 74. Correct answer: ­C High levels of oxygen have been associated with ROP, but lower levels of oxygen may lead to more respiratory complications (e.g., hypoxia) and death in premature infants. Better oxygen level monitoring has led to better control of oxygen administered to premature infants. Today, however, there is an increase in ROP due to the fact that neonatal care advances mean more ­low-­weight premature infants are ­surviving. 75. Correct answer: ­C When utilizing transcutaneous PO2 measurements, underestimation of oxygenation may occur when the skin is hypoperfused or the calibration is improper. Overestimation of oxygenation may occur if there is an air bubble or leak between the electrode and the ­skin. 76. Correct answer: ­A High-­frequency jet ventilators (HFJV) deliver ­high-­flow, ­short-­duration pulses of pressurized gas directly into the upper airway through a specially made endotracheal lumen. The pulses are delivered to the upper airway and are superimposed on gas flow from a conventional ventilator that provides positive ­end-­expiratory pressure (PEEP). In addition, conventional breaths may be delivered in conjunction with the jet ventilation. The systems operate at rates of 150–600 breaths per minute and exhalation is ­passive. 77. Correct answer: ­B High-­f requency ventilators have the advantage of transporting CO2 out of the lungs with smaller pressure and volume fluctuations than are required during conventional mechanical ventilation. Originally it was thought that adequate gas exchange could be obtained at relatively low mean airway pressures, but now it is known that recruiting lung volume is essential in not only optimizing oxygenation but also in minimizing lung injury. Studies have shown that if both types of ventilators had matched

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mean airway pressures, PaO2 was significantly greater during ­high-­f requency oscillation than during conventional mechanical ­ventilation. 78. Correct answer: ­C Adequate perfusion must exist for the probe on a pulse oximeter to register an accurate evaluation of hemoglobin saturation. If the infant is hypothermic or has decreased peripheral perfusion, the results will be inaccurate. Phototherapy, position of the probe, and patient motion can also cause inaccurate readings on a pulse ­oximeter. 79. Correct answer: ­B In addition to teaching the parents about gastroesophageal reflux, it is important to remember that the patient could also suffer other potential side effects such as growth restrictions, recurrent diaphragmatic hernias, and delayed neurological ­development. 80. Correct answer: ­C ECMO should not be used for intravascular hemorrhage because systemic heparinization is required. ECMO also increases the risk for intravascular hemorrhage in premature infants, especially those less than 35 weeks ­gestation. 81. Correct answer: ­A This result is compensated metabolic acidosis. The pH is between 7.35 and 7.45, so the value is compensated, but since it is closer to 7.35, the value is considered acidotic. To determine whether the acidosis is respiratory or metabolic, find the value that repre­ Eq/L. sents acidosis. This would be the HCO3 at , 22 m 82. Correct answer: ­C This result is uncompensated respiratory alkalosis. The pH is greater than 7.45 so the value is an uncompensated alkalosis. To determine whether the alkalosis is respiratory or metabolic, find the value that represents alkalosis. This would be the CO2 at , 35 mm ­Hg. 83. Correct answer: ­C The result of this blood gas analysis is an uncompensated metabolic acidosis. The pH is less than 7.35 so the value is uncompensated acidosis. To determine whether the acidosis is respiratory or metabolic, find the value that represents acidosis. This would be the HCO3 at , 22 m ­ Eq/L. 84. Correct answer: ­A This is an uncompensated (mixed) respiratory/metabolic alkalosis. The pH is greater than 7.45 so the value is uncompensated. To determine whether the acidosis is respiratory or metabolic, find the value that represents alkalosis. This would be both the HCO3 at . 26 mEq/L and the CO2 , 35 mm Hg, meaning the cause of the alkalosis is both respiratory and metabolic in ­nature. 85. Correct answer: ­B The pH is between 7.35 and 7.45; the CO2 is between 35 and 45 mm Hg; and the HCO3 is between 22 and 26 mEq/L). All the results are within normal ranges, so this ABG is considered ­normal.

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86. Correct answer: ­A This result signifies an uncompensated respiratory alkalosis. The pH is greater than 7.45, so the value is uncompensated alkalosis. To determine whether the alkalosis is respiratory or metabolic, find the value that represents alkalosis. This would be the CO2 at , 35 mm ­Hg. 87. Correct answer: ­B This ABG result is a compensated respiratory acidosis. The pH is between 7.35 and 7.45, so the value is compensated, but since it is closer to 7.35, the value is considered acidotic. To determine whether the acidosis is respiratory or metabolic, find the value that represents acidosis. This would be the CO2 at . 45 mm ­Hg. 88. Correct answer: ­A This is an uncompensated (mixed) respiratory/metabolic acidosis. The pH is less than 7.35, so the value is uncompensated acidosis. To determine whether the acidosis is respiratory or metabolic, find the value that represents acidosis. This would be both the HCO3 at , 22 mEq/L and the CO2 . 45 mm Hg, meaning the cause of the acidosis is both respiratory and metabolic in ­nature. 89. Correct answer: ­C This result demonstrates a compensated respiratory alkalosis. The pH is between 7.35 and 7.45, so the value is compensated, but since it is closer to 7.45, the value is considered alkalotic. To determine whether the alkalosis is respiratory or metabolic, find the value that represents alkalosis. This would be the CO2 at , 35 mm ­Hg. 90. Correct answer: ­A This result is an uncompensated metabolic alkalosis. The pH is greater than 7.45, so the value is uncompensated. To determine whether the alkalosis is respiratory or metabolic, find the value that represents alkalosis. This would be the HCO3 at . 26 ­mEq/L. 91. Correct answer: ­B This result is an uncompensated (mixed) respiratory/metabolic acidosis. The pH is less than 7.35, so the value is uncompensated acidosis. To determine whether the acidosis is respiratory or metabolic, find the value that represents acidosis. This would be both the HCO3 at , 22 mEq/L and the CO2 . 45 mm Hg, meaning the cause of the acidosis is both respiratory and metabolic in ­nature. 92. Correct answer: ­A The pH (between 7.35 and 7.45); the CO2 (between 35 and 45 mm Hg) and the HCO3 (22–26 mEq/L) are within normal ranges, so the ABG is considered ­normal. 93. Correct answer: ­B This result is an uncompensated respiratory acidosis. The pH is less than 7.35—the value is uncompensated. To determine whether the acidosis is respiratory or metabolic, find the value that represents acidosis. This would be the CO2 at . 45 mm ­Hg.

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94. Correct answer: ­B This infant has endured prolonged mechanical ventilation and has developed bronchopulmonary dysplasia. The prematurity of the infant and the probability of barotrauma increased this infant’s risk for BPD or lung injury. Obstructive and restrictive lung diseases are often the result of lung ­injury. 95. Correct answer: ­A High-­f requency jet ventilation would be contraindicated in this patient because it requires reintubation with a ­triple-­lumen ETT. This patient had 6 intubation attempts with a 2.0 ETT with airway trauma. The risk of extubation and failed reintubation is high, and an adequate airway may not be obtained. If available, ­flow-­interrupted or oscillator ventilation is ­preferred. 96. Correct answer: ­C During ­high-­f requency oscillator ventilation, the exhalation phase is active with the oscillator controlling inspiration and expiration. Frequency or rate of breaths per minute is expressed as ­Hertz. 97. Correct answer: ­B To prevent alveolar rupture, suction should be applied during both insertion and withdrawal of the suction catheter when using ­high-­f requency jet ventilation. If suction is not applied while inserting the catheter, the circuit becomes overpressurized due to the occlusion, and the alveoli will rupture. Suctioning may be done when the ventilator is in use or on standby. Suctioning while in standby mode will prevent airway damage from opposing pressures from the ventilator and suction ­catheter. 98. Correct answer: ­B The servocontrol pressure on a ­high-­f requency jet ventilator is the internal adjustment of pressure in response to lung compliance and pressure changes. A drop in this measurement indicates worsening lung disease, an obstruction, pneumothorax, or that the tubing is kinked. As the airway resistance increases, the servocontrol pressure decreases. In reverse, as the airway resistance decreases, the servocontrol pressure must increase. This effect is seen when the patient’s condition improves with greater lung compliance or if there is a leak in the system or ­circuit. 99. Correct answer: ­A The ability of the ­high-­f requency, ­flow-­interrupted ventilator to decrease CO2 is accomplished by changing the difference between PIP and PEEP. The greater the difference, the greater the ability to clear CO2 or improve ventilation. Caution should be exercised to prevent air trapping with higher pressures. PEEP is used to impact oxygenation. The arterial blood gas indicates respiratory acidosis, with acceptable HCO3 and base excess. Sodium bicarbonate administration is not indicated at this ­time. 100. Correct answer: ­C The h ­ igh-­f requency oscillator uses a rapidly moving piston to deliver gases to and from the baby, causing the chest to vibrate or wobble. This chest movement indicates that the ETT is in correct position as breath sounds and respiratory rate cannot be

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assessed while the ventilator is in use. Prolonged standby mode or disconnection of the ventilator for assessment or repositioning may cause the airway to collapse and increase air ­trapping. 101. Correct answer: ­A Amplitude is used to manipulate ventilation or volume when using an oscillator for ventilatory support. The chest ­X-­ray indicates overinflation. To decrease volume delivered, the amplitude should be decreased. The mean airway pressure is manipulated to adjust oxygenation. The term Hertz refers to breaths per ­minute. 102. Correct answer: ­B One Hertz represents 60 breaths per minute or 1 breath per second. So if the Hertz is 10, then the respiratory rate is 600. Hertz indicates the rate at which fresh gases are delivered to the ­lungs. 103. Correct answer: ­C Liquid ventilation uses perfluorocarbons to decrease alveolar surface tension to recruit collapsed alveoli. This allows for the use of lower pressures to maintain oxygenation. Perfluorocarbons allow the rapid transport of oxygen and carbon dioxide to newly opened alveoli to improve ­oxygenation. 104. Correct answer: ­A Ventilator-­associated pneumonia (VAP) education for both parents and staff can significantly reduce the incidence of VAP in neonates. An awareness and training change practice and improve patient safety by decreasing the risk of infection. Daily ventilator circuit changes are not recommended as this involves a break in a closed system and risks contamination of the airway. Circuits should only be changed when visibly soiled or when one is changing ventilation support modes (e.g., changing from conventional to oscillator modes). There is no specific evidence that supports or refutes turning a patient to prevent VAP. It is recommended that oral suctioning be performed prior to position changes to prevent aspiration of oral ­secretions. 105. Correct answer: ­B The sigh setting is a backup rate that provides a set number of ventilations per minute that recruit additional alveoli and minimize microatelectasis while ­high-­f requency ventilation is ­utilized. 106. Correct answer: ­A The jet port of the endotracheal tube may become occluded with secretions, so a critical nursing intervention would include flushing the jet port every 3–4 hours with 0.5 ml of normal saline. The tubing should never be kinked when turning, repositioning, or moving the patient as it may cause a collapse of the alveoli. Respiratory rate cannot be directly monitored when ­high-­f requency ventilation is used. Instead, the chest should be monitored for continuous wobble or vibration to indicate patent airway and correct endotracheal tube ­placement.

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107. Correct answer: ­B Antenatal corticosteroids affect lung maturation and help prevent respiratory distress syndrome. Steroids work by accelerating the rate of glycogen depletion and glycerophospholipid biosynthesis. This process results in thinning the ­intra-­alveolar septa and increases the size of the ­alveoli. 108. Correct answer: ­C Chronic stress in utero usually produces an infant small for gestational age but who has more mature lungs. These infants also have a small thymus and large adrenal glands. Many researchers believe this is because the infant may have high glucocorticoid levels while in ­utero. 109. Correct answer: ­C Retractions occur when parenchymal disease is present and the chest wall produces a greater negative pressure. The chest wall is very compliant and caves inward with a moderate decrease in pleural pressure. Retractions are best seen at the xiphoid and intercostal markings of the ­chest. 110. Correct answer: ­C An infant with an expiratory grunt is performing a Valsalva maneuver in which the infant exhales against a closed glottis, thus producing a sound similar to a moan. Transpulmonary pressure is increased, which decreases or prevents atelectasis. Oxygenation and alveolar ventilation are improved. Intubation should not be tried unless the infant’s condition is rapidly deteriorating, because the ETT prevents the Valsalva maneuver and the alveoli will ­collapse. 111. Correct answer: ­B When caring for an infant with a pulse oximeter, it is important to use a disposable cuff. A reusable cuff will wear down and allow ambient light to get in and invalidate the readings. The disposable sensor should be checked at least every 8 hours. The pulse oximeter is useful for trending, but arterial blood gases must be drawn to correlate with the pulse ­oximeter. 112. Correct answer: ­A Small, raised, bright red areas around the diaper line and white plaques in the buccal area, cheeks, on the tongue, or on the gums usually indicates thrush. This infant has been on antibiotic therapy, and there is probable overgrowth of ­bacteria. 113. Correct answer: ­A When swabbing the mouth of an infant with thrush, it is very important to use a ­cotton-­tipped applicator. It is necessary to distinguish between thrush and milk curds. If the white areas are thrush, they will bleed and be very raw after removal. Administer gentian violet (1–2%) swabbed on oral lesions. Do not get the gentian violet on normal mucosa because it burns the tissue. Gentian violet will also stain the mouth and clothing. After 1 hour (or after feeding), nystatin can be applied to the baby’s oral mucosa. Swab skin lesions with topical nystatin and instruct the mother to treat her nipples with nystatin if she is ­breastfeeding.

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114. Correct answer: ­A Prior to the administration of vancomycin, the nurse should know that vancomycin is nephrotoxic, especially if used in combination with aminoglycosides. Vancomycin is used in the treatment of ­methicillin-­resistant strains (Staphylococcus epidermidis) and must be administered by slow intravenous ­drip. 115. Correct answer: ­C Baby Luke has been gradually getting worse and has developed retractions. The compliance of his lungs decreased, the respiratory pattern became less efficient, and he cannot compensate for the respiratory insufficiency by closing his glottis. The seesaw respirations reflect the diminished lung compliance, which forces the protrusion of the diaphragm into the abdominal cavity and causes the bulging of the abdomen with each inspiration. Accessory thoracic respiratory muscles are used to keep the terminal airways open by forcing the expansion of lungs via negative intrathoracic pressure. This creates the paradoxical motion of the seesaw when the anterior ribs and sternum collapse because of the negative thoracic ­pressure. 116. Correct answer: ­A The 2003 guidelines from the Centers for Disease Control and Prevention (CDC) recommend that any equipment that comes in direct contact with patient mucosal membranes undergo ­wet-­heat pasteurization at . 158° F for at least 30 minutes and be packaged in sterile wrapping for the next use. An autoclave may also be used for reusable equipment. Any ­single-­use equipment is recommended for resterilization per FDA guidelines by a ­third-­party agency prior to use with another patient. If equipment must be washed after sterilization, the CDC recommends the use of sterile water, not normal saline. Although many units may direct respiratory therapy to maintain and sterilize all respiratory equipment, as a patient advocate, the bedside nurse should be aware of and ensure equipment is properly sterilized prior to use on his or her ­patient. 117. Correct answer: ­B Antimicrobial soap and water may be used to decontaminate hands prior to, during, and after patient care regardless of glove use, visible soiling, or no apparent soiling. Hands should always be decontaminated using soap and water, waterless soap, or ­alcohol-­based antiseptic agent prior to and after patient care to prevent the spread of organisms from patient to patient or from surfaces. ­A lcohol-­based antiseptic agents should only be used if no visible soiling is ­observed. 118. Correct answer: ­B According to current VAP precautions, tracheostomy care should be provided using both gloves and gown and using aseptic technique. Currently, studies do not support the use of ­sterile-­only technique while providing tracheostomy care compared to aseptic technique. Suctioning prior to or during respiratory care is recommended if secretions are visible to prevent migration into the airways during tube changes. Sterile fluid and a sterile, ­single-­use suction catheter should be utilized when suctioning when using an open ­system.

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119. Correct answer: ­C Premature neonates may begin enteral feedings prior to extubation due to medical status. To prevent VAP, the patient should be positioned at a 15–45° angle if it is not medically contraindicated. In addition, feeding tube placement, regardless of insertion location (nasal, oral, gastric, or jejunumal), should be checked frequently to identify tip migration or dislocation leading to increased risk for aspiration. Conclusive evidence is not available to support or refute the use of continuous feedings versus bolus feedings or to recommend specific feeding tube s­ izes. 120. Correct answer: ­C Appropriate VAP prevention guidelines recommend that there are separate suction canisters for endotracheal and oral suctioning to prevent ­cross-­contamination. Suction canisters should be changed when soiled and labeled. Oral suction catheters should be stored in open plastic containers and changed daily to prevent bacterial colonization in the tubing or catheter. Caution should be used when storing any oral or respiratory equipment at the foot of a bed due to the proximity to the anus and urethra. Contamination may occur during diaper changes and positioning. Resuscitation bags should also be stored outside the bed or isolette to prevent contamination from excretions or oral ­secretions/emesis. 121. Correct answer: ­B Gentamicin is effective against ­gram-­negative rods and ­penicillin-­resistant staphylococci, Escherichia coli strains, and Pseudomonas aeruginosa. Gentamicin may cause ototoxicity and nephrotoxicity. The nurse needs to follow serum levels (peak and trough). Gentamicin must never be given as IV push—­only as a drip over at least 30–60 minutes. If the infant has oliguria or anuria, the dose must be decreased or ­discontinued. 122. Correct answer: ­C Antenatal steroids are prescribed to mothers in preterm labor to decrease fetal mortality by decreasing the risk of respiratory distress, necrotizing enterocolitis, and intracranial ­hemorrhage. 123. Correct answer: ­B Mitral and tricuspid valves open during systole. The first heart sound or S1 represents closure of these valves at the end of ventricular ­systole. 124. Correct answer: ­C An ABG with a pH of 7.25, PaCO2 70, HCO3 27, Base Excess of 2, and a PaO2 of 45 with saturation of 90% should be managed by increasing PIP and increasing IMV rate to treat the hypercapnia and improve ­oxygenation. 125. Correct answer: ­A Any intubated patient who suddenly presents with a rapid increase in PaCO2 and decrease in PaO2 should be immediately evaluated for a tension pneumothorax related to ventilator pressures. Delayed treatment could result in hypoxia, hypoxemia, and death. Rapid assessment and treatment with needle decompression is necessary to prevent tissue ­injury.

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126. Correct answer: ­C The first retinopathy of prematurity exam should be scheduled when the neonate is at 32 weeks gestational age or 5–6 weeks of life, whichever is greater. Normal vascular genesis begins at about week 16 of gestation and proceeds from the optic nerve to the retina. At around 32 weeks gestation, vascular growth occurs toward the nasal bridge. Injury or an altered growth pattern becomes visible and pronounced with rapid, irregular, and torturous vessel development between established and avascular regions. If spontaneous recovery does not occur, laser surgery is used to halt torturous growth to decrease the risk of retinal ­detachment. 127. Correct answer: ­A Persistent pulmonary hypertension of the newborn may lead to renal failure due to an increase in systemic pressures and renal hypoxemia due to v/q ­mismatch. 128. Correct answer: ­B When managing a patient with persistent pulmonary hypertension, the pH should be maintained between 7.45 and 7.6, while the PaCO2 should be between 20 and ­30. 129. Correct answer: ­C Home oxygen therapy can be very drying to the mucosal membranes, so parents should be taught to use ­lemon-­glycerin swabs to reduce oral dryness. Oxygen tanks should be stored vertically in special containers and prevented from ­falling. 130. Correct answer: ­C Pulmonary interstitial emphysema (PIE) may be treated with dexamethasone to decrease tissue inflammation. Complications of dexamethasone administration place the infant at greater risk of infection, hypertension, hyperlipidemia, and ­hyperglycemia. 131. Correct answer: ­B The mostly likely cause of respiratory distress syndrome is deficient surfactant due to prematurity. Replacement surfactant allows for greater alveolar expansion and ­oxygenation. 132. Correct answer: ­C Adequate oxygenation is the best preventive measure and treatment for persistent pulmonary hypertension of the newborn (PPHN). 133. Correct answer: ­C Bronchospasms should be treated with albuterol to dilate airways and improve ­oxygenation. 134. Correct answer: ­C Corticosteroids may cause hypertension in neonates. It is important to evaluate blood pressures at least every 12 hours if the infant is ­stable.

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135. Correct answer: ­B Nitric oxide therapy may cause methemoglobinemia as hemoglobin is altered to a state that will not carry ­oxygen. 136. Correct answer: ­B At 26–28 weeks gestation, the fetal lung is first capable of supporting extrauterine life independently. Supplemental oxygen and minor respiratory support may still be required depending upon the degree of disease and ­development. 137. Correct answer: ­B Early signs of respiratory distress in the neonate include grunting and retractions as the infant attempts to compensate for impaired lung function. Retractions indicate the degree of negative pressure exerted by the diaphragm to pull air into the lungs, and grunting provides ­end-­expiratory pressure to maintain alveolar expansion and increase functional residual ­capacity. 138. Correct answer: ­B Doxapram is a respiratory stimulant that increases tidal volume and respiratory ­rate. 139. Correct answer: ­A This neonate is at risk for pulmonary hypoplasia due to maternal neuromuscular depression during her hospitalization. Neuromuscular depression causes central nervous system depression in the fetus that decreases pulmonary growth during the critical growth period of 16–27 weeks gestation. If pulmonary hypoplasia is suspected, respiratory support should be provided immediately with expectations of poor compliance and cautious pressure management to limit risk of ­pneumothorax.

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Pulmonary System ­References Abman, S. (2007). Recent advances in the pathogenesis and treatment of persistent pulmonary hypertension of the newborn. Neonatology, 91(4), 283–290. Abman, S. (2008). The dysmorphic pulmonary circulation in bronchopulmonary dysplasia: A growing story. American Journal of Respiratory and Critical Care Medicine, 178(2), 114–115. Apisarnthanarak, A., H ­ olzmann-­Pazgal, G., Hamvas, A., et al. (2003, December). ­Ventilator­associated pneumonia in extremely preterm neonates in a neonatal intensive care unit: Characteristics, risk factors, and outcomes. Pediatrics, 112; 1283–1289. Arioni, C., Bellini, C., Scopesi, F., et al. (2006). Pulmonary interstitial emphysema in preterm twins on continuous positive airway pressure. Journal of Maternal–Fetal & Neonatal Medicine, 19(10), 671–673. Bachman, T., Marks, N., & Rimensberger, P. (2008). Factors effecting adoption of new neonatal and pediatric respiratory technologies. Intensive Care Medicine, 34(1), 174–178. Baird, T. M., et al. (2002). Clinical associations, treatment, and outcome of apnea of prematurity. NeoReviews, 3(4), e66–e70. Baraldi, E., & Filippone, M. (2007). Chronic lung disease after premature birth. New England Journal of Medicine, 357, 1946–1955. Bhandari, V., ­Choo-­Wing, R., Lee, C., et al. (2008). Developmental regulation of ­NO-­mediated ­V EGF-­induced effects in the lung. American Journal of Respiratory Cell and Molecular Biology, 39(4), 420–430. Bhuta, T., & ­Henderson-­Smart, D. J. (2005). Elective high frequency jet ventilation versus conventional ventilation for respiratory distress syndrome in preterm infants. Cochrane Database of Systematic Reviews, (2), ­CD000328. Blackburn, et al. (2007). Maternal, fetal & neonatal physiology: A clinical perspective. Philadelphia, PA: W. B. ­Saunders. Bohlin, K., Jonsson, B., Gustafsson, A., & Blennow, M. (2008). Continuous positive airway pressure and surfactant. Neonatology, 93(4), 309–315. Boussemart, T., Nsota, J., ­Martin-­Coignard, D., & Champion, G. (2009). Nephrogenic diabetes insipidus: Treat with caution. Pediatric Nephrology, 24(9), 1761–1763. Brodsky, D., & Martin, C. (2003). Neonatology review. Philadelphia, PA: Hanley & ­Belfus. Brown, C. M., Redd, S. C., & Damon, S. A. (2004, March). Acute idiopathic pulmonary hemorrhage among infants. Recommendations from the Working Group for Investigation and Surveillance. MMWR Recommendations and Reports, 12(53, RR-2), 1–12. CDC. (2002). Guideline for hand hygiene in ­health-­care settings. Recommendations of the Healthcare Infection Control Practices Advisory Committee and the HICPAC/SHEA/APIC/ IDSA Hand Hygiene task force [Excerpt]. MMWR, 51(RR16), 1–44. CDC. (2004). Guidelines for preventing ­health-­care associated pneumonia. 2003 recommendations of the CDC and the Healthcare Infection Control Practices advisory committee [Excerpt]. MMWR, 53(RR03), 1–36. Cloherty, J. P., Eichenwald, E. C., & Stark, A. R. (2004). Manual of neonatal care (5th ed.). Philadelphia, PA: ­Lippincott. Dabir, T., Mccrossan, B., Sweeney, L., et al. (2008). Down syndrome, achondroplasia and tetralogy of Fallot. Neonatology, 94(1), 68–70. Dani, C., Bertini, G., Pezzati, M., et al. (2006). Effects of pressure support ventilation plus volume guarantee vs. h ­ igh-­f requency oscillatory ventilation on lung inflammation in preterm infants. Pediatric Pulmonology, 41, 242–249. Dellamonica, J., Louis, B., Lyazidi, A., et al. (2008). Intrapulmonary percussive ventilation superimposed on conventional ventilation: Bench study of humidity and ventilator behaviour. Intensive Care Medicine, 34(11), 2035–2043. Delsing, B., Lopriore, E., Blom, N., et al. (2007). Risk of persistent pulmonary hypertension of the neonate in ­t win-­to-­t win transfusion syndrome. Neonatology, 92(2), 134–138.

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158  y  Section 5 Donn, S. M., & Malden, M. A. (Eds). (2002). Birth asphyxia and the brain: Basic science and clinical implications. Armonk, NY: Futura Publishing ­Co. Foglia, E., Meier, M. D., & Elward, A. (2007). ­Ventilator-­associated pneumonia in neonatal and pediatric intensive care unit patients. Clinical Microbiology Reviews, 20(3), 409–425. Gien, J., Seedorf, G., Balasubramaniam, V., et al. (2007). Intrauterine pulmonary hypertension impairs angiogenesis in vitro: Role of vascular endothelial growth ­factor-­nitric oxide signaling. American Journal of Respiratory and Critical Care Medicine, 176(11), 1146–1153. Goissen, C., Ghyselen, L., Tourneux, P., et al. (2008). Persistent pulmonary hypertension of the newborn with transposition of the great arteries: Successful treatment with bosentan. European Journal of Pediatrics, 167(4), 437–440. Greenough, A., & Murthy, V. (2008). Respiratory distress syndrome. Fetal and Maternal Medicine Review, 19(3), 203–225. Halliday, H. (2008). Surfactants: Past, present and future. Journal of Perinatology, 28(S1), S47–S56. Halliday, H. L., McClure, B. G., & Reid, M. (2002). Handbook of neonatal intensive care (4th ed.). Philadelphia, PA: ­Saunders. Hay, W. W., Levin, M. J., et al. (2007). Current diagnosis and treatment in pediatrics (18th ed.). New York, NY: McGraw-­Hill. Hermansen, C., & Lorah, K. (2007). Respiratory distress in the newborn. American Family Physician, 76(7), 987–994. Hoshino, T., Kato, S., Oka, N., et al. (2007). Pulmonary inflammation and emphysema: Role of the cytokines IL-18 and IL-13. American Journal of Respiratory and Critical Care Medicine, 176(1), 49–62. Hummler, H. D., Engelmann, A., Pohlandt, F., & Franz, A. R. (2006). ­Volume-­controlled intermittent mandatory ventilation in preterm infants with hypoxemic episodes. Intensive Care Medicine, 32, 577–584. Iyer, V., Joshi, A., & Ryu, J. (2009). Spontaneous pneumomediastinum: Analysis of 62 consecutive adult patients. Mayo Clinic Proceedings, 84(5), 417–421. Jones, K. L. (2006). Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: ­Elsevier. Kao, B., De Ramirez, S., Belfort, M., & Hansen, A. (2008). Inhaled epinephrine for the treatment of transient tachypnea of the newborn. Journal of Perinatology, 28(3), 205–210. Karlsen, K. (2006). The S.T.A.B.L.E. program (5th ed.). Park City, UT: American Academy of ­Pediatrics. Karlsen, K., & Tani, L. Y. (2003). S.T.A.B.L.E.—Cardiac module. Park City, UT: American Academy of ­Pediatrics. Kattwinkle, J. (Ed.). (2006). Neonatal resuscitation textbook (5th ed.). Elk Grove Village, IL: American Academy of Pediatrics and American Heart ­A ssociation. Kenner, C. and Lott, J. W. (2007). Comprehensive neonatal nursing. Philadelphia, PA: W.B. ­Saunders. Kenner, C., Amlung, S., & Flandermeyr, A. (1998). Protocols in neonatal nursing. Philadelphia, PA: ­Saunders. Kenner, C., & Lott, J. (2004). Neonatal nursing handbook. St. Louis, MO: E ­ lsevier. Kenner, C., & Lott, J. (2007). Comprehensive neonatal care (4th ed.). St. Louis, MO: ­Elsevier. Kenner, C., & McGrath, J. M. (Ed.). (2004). Developmental care of newborns & infants: A guide for health professionals. St. Louis, MO: M ­ osby. Laberge, J. M., & Flageole, H. (2007). Fetal tracheal occlusion for the treatment of congenital diaphragmatic hernia. World Journal of Surgery, 31(8), 1577–1586. Lee, B. H., Stoll, B. J., McDonald, S. A., & Higgins, R. D. (2006). Adverse neonatal outcomes associated with antenatal dexamethasone versus antenatal betamethasone. Pediatrics, 117, 1503–1510. Ly, L., Hawes, J., Whyte, H., et al. (2007). The hemodynamically significant ductus arteriosus in critically ill ­f ull-­term neonates: Two case reports? Neonatology, 91(4), 260–265. MacDonald, M. G., & Ramasethu, J. (2007). Atlas of procedures in neonatology (4th ed.). Philadelphia, PA: Lippincott ­Williams.

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Pulmonary ­System  y  159 Mainali, E. S., Greene, C., Rozycki, H. J., & Gutcher, G. R. (2007). Safety and efficacy of ­high­f requency jet ventilation in neonatal transport. Journal of Perinatology, 27(10), 609–613. Marjut Varpula, M., Minna, M., Saukkonen, K., et al. (2005). Hemodynamic variables related to outcome in septic shock. Intensive Care Medicine, 31(8), 1066–1071. Markiewicz, M., & Abrahamson, E. (1999). Diagnosis in color: Neonatology. Philadelphia, PA: ­Mosby. Martin, R. J., ­Abu-­Shaweesh, J. M., & Baird, T. M. (2002). Pathophysiologic mechanisms underlying apnea of prematurity. NeoReviews, 3(4), e59–e65. Martin, R. J. & Fanaroff. A. A. (2006). Neonatal–perinatal medicine: Diseases of the fetus and infant. Philadelphia, PA: Elsevier ­Mosby. McCurnin, D., Pierce, R., Willis, B., et al. (2009). Postnatal estradiol ­up-­regulates lung nitric oxide synthases and improves lung function in bronchopulmonary dysplasia. American Journal of Respiratory and Critical Care Medicine, 179(6), 492–500. Merenstein, G. B., & Gardner, S. L. (2006). Handbook of neonatal intensive care (6th ed.). St. Louis, MO: ­Mosby. Nanan, R., Liu, A., Poulton, A., et al. (2008). Nasal CPAP for very preterm infants. The New England Journal of Medicine, 358(23), ­2521. Ng, G., Derry, C., Marston, L., et al. (2008). Reduction in ­ventilator-­induced lung injury improves outcome in congenital diaphragmatic hernia? Pediatric Surgery International, 24(2), 145–150. Northwestern University. (2009, January). Pulmonary hypertension: Research from Northwestern University, Department of Pediatrics provides new data about pulmonary hypertension. Proteomics Weekly, ­837. Polin, R. A., & Spritzer, A. R. (2007). Fetal & neonatal secrets (2nd ed.). Philadelphia, PA: ­Mosby. Prescribing Reference. (2009, Summer). NPPR: Nurse Practitioner’s Prescribing Reference, 16(2). Pursnani, S. K., Amodio, J. B., Guo, H., et al. (2006). Localized persistent interstitial pulmonary emphysema presenting as a spontaneous tension pneumothorax in a full term infant. Pediatric Surgery International, 22(7), 613–616. Ramanathan, R. (2008). Optimal ventilatory strategies and surfactant to protect the preterm lungs. Neonatology, 93(4), 302–308. Rennie, J. M., & Roberton, N. R. C. (2002). A manual of neonatal intensive care (4th ed.). New York, NY: A ­ rnold. Rojas, M. A., Lozano, J. M., Rojas, M. X., et al. (2005). Randomized, multicenter trial of conventional ventilation versus ­high-­f requency oscillatory ventilation for the early management of respiratory failure in term or ­near-­term infants in Colombia. Journal of Perinatology, 25(11), 720–724. Schmidt, B., Roberts, R. S., Davis, P., et al. (2007). ­Long-­term effects of caffeine therapy for apnea of prematurity. The New England Journal of Medicine, 357(19), 1893–1902. Short, E., Kirchner, H., Asaad, G., et al. (2008). ­Long-­term sequelae of postnatal surfactant and corticosteroid therapies for BPD. Journal of Perinatology, 28(7), 498–504. Stevenson, D. (2007). On the caffeination of prematurity. The New England Journal of Medicine, 357(19), 1967–1968. Sweet, D., Bevilacqua, G., Carnielli, V., et al. (2007). European consensus guidelines on the management of neonatal respiratory distress syndrome. Journal Perinatal Medicine, 35, 175–186. Taeusch, H. W., Ballard, R. A., & Gleason, C. A. (2005). Avery’s diseases of the newborn (8th ed.). Philadelphia, PA: ­Elsevier. Tasker, R. (2008). CPAP and HFOV: Different guises of the same underlying intensive care strategy for supporting RSV bronchiolitis. Intensive Care Medicine, 34(9), 1560–1561. Thome, U., Carlo, W., & Pohlandt. (2005). Ventilation strategies and outcome in randomized trials of high frequency ventilation. Archives of Disease in Childhood Fetal and Neonatal Edition, 90F: 466–473. Tripathi, S., & Saili, A. (2007). The effect of steroids on the clinical course and outcome of neonates with meconium aspiration syndrome. Journal of Tropical Pediatrics, 53(1), 8–12.

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160  y  Section 5 Verklan, M. T., & Walden, M. (Eds.). (2004). Certification and core review for neonatal intensive care nursing (3rd ed.). St. Louis, MO: E ­ lsevier. Verklan, M. T., & Walden, M. (Eds.). (2010). Certification and core review for neonatal intensive care nursing (4th ed.). St. Louis, MO: ­Elsevier. Verma, R. P., Chandra, S., Niwas, R., & Komaroff, E. (2006). Risk factors and clinical outcomes of pulmonary interstitial emphysema in extremely low birth weight infants. Journal of Perinatology, 26(3), 197–200. Yiming, M., Lederer, D., Sun, L., et al. (2008). Platelets enhance endothelial adhesiveness in high tidal volume ventilation. American Journal of Respiratory Cell and Molecular Biology, 39(5), 569–575. Young, T. E., & Magnum, B. (2009). Neofax 2009. Montvale, NJ: Thomson ­Reuters.

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Section 6 Gastrointestinal ­System

Questions 1. The 36-week gestation female born 2 hours ago who you are feeding begins coughing and choking. She has large amounts of oral secretions as well. Her color is poor and she is in respiratory distress. What is the most likely cause of these ­symptoms? A. Esophageal ­atresia B. Status a ­ sthmaticus C. Cystic ­fibrosis 2. Which of the following is the most common cause of intestinal obstruction in the ­newborn? A. ­Intussusception B. Meconium i­ leus C. Intestinal ­atresia 3. The infant you are caring for had meconium plug syndrome at birth. The mother reports the baby has a salty taste when kissed. What could be wrong with this ­infant? A. Hirschsprung’s ­disease B. Cystic ­fibrosis C. Malrotation with ­volvulus 4. The parents of a ­full-­term female infant are concerned because Hirschsprung’s disease runs in the family. An appropriate, therapeutic explanation would ­be A. “Hirschsprung’s disease never occurs in females.” B. “It is caused by prenatal alcohol consumption.” C. “Hirschsprung’s is a congenital bowel disease.” 5. At what stage of fetal development does the GI tract begin to d ­ evelop? A. 2–4 weeks gestational a ­ ge B. 4–8 weeks gestational ­age C. 6–10 weeks gestational ­age 6. How do an omphalocele and gastroschisis ­differ? A. Omphalocele is the herniation of some or all the abdominal contents into a sac at the ­umbilicus B. Gastroschisis is the herniation of all the abdominal contents into a sac at the ­umbilicus C. Omphalocele is the herniation of the small bowel into a sac outside the ­abdomen

y

y

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7. The parents of an infant born with gastroschisis are concerned about postoperative care. What important fact will you teach them about the correction of this ­problem? A. It is a minor surgical procedure done at the ­bedside B. It will take many days for their baby to ­recover C. Their baby will be monitored for fluid status, signs of infection, and nasogastric ­suction 8. You are in attendance at the cesarean section of a 32-week gestational age infant. The infant emerges in severe respiratory distress with a probable diaphragmatic hernia. What medical therapies do you ­anticipate? A. Intubation, nasogastric tube, IVs, chest ­X-­ray, and ­surgery B. None, this will resolve ­spontaneously C. Intubation, chest tubes, and a ­Sengstaken-­Blakemore ­t ube 9. While resuscitating an infant with a diaphragmatic hernia, which oxygen delivery method is ­best? A. ­Bag-­mask ­device B. Intubation and mechanical ­device C. Simple oxygen mask or ­blow-­by ­oxygen 10. Infants born with diaphragmatic hernia are described to have a scaphoid abdomen on assessment. What does scaphoid ­mean? A. A sunken or concave ­appearance B. A sunken or convex ­appearance C. A concave or protruding ­appearance 11. VATER association presents with which of the following ­anomalies? A. Vertebral anomalies, anal anomalies, tracheoesophageal fistula, esophageal atresia, and renal ­anomalies B. Vertebral anomalies, anal anomalies, tracheoesophageal fistula, ear anomalies, and renal ­anomalies C. Vertebral anomalies, anal anomalies, thoracic anomalies, esophageal atresia, and rectal ­anomalies 12. A 3-­day-­old, 36-week gestational age male has suddenly started having bilious vomitus without abdominal distention. What could be the cause of his ­distress? A. Duodenal ­atresia B. Meconium p ­ lug C. Malrotation of the midgut and ­volvulus 13. For an infant with malrotation of the midgut and volvulus, what treatments in addition to surgery would you e ­ xpect? A. Feeding tube with basic soy ­formula B. Nasogastric tube, antibiotics, and NPO ­status C. Nasogastric tube ­only

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14. A ­full-­term baby with Down syndrome has started vomiting. You notice his abdomen is becoming distended. Which of the following conditions may be the cause of this ­problem? A. High meconium ­plug B. Malrotation and ­volvulus C. Duodenal ­atresia 15. Ductal atresia is often seen with Trisomy 13. What other conditions are associated with Trisomy ­13? A. Congenital heart defects, malrotation of the gut, and tracheoesophageal ­abnormalities B. Congenital heart disease, limb abnormalities, and tracheoesophageal ­abnormalities C. Congenital heart disease, anorectal defects, and neural tube ­defects 16. The infant you are caring for is suspected of having Hirschsprung’s disease. What diagnostic procedures do you anticipate the infant will have to undergo for treatment of this ­disease? A. Plain abdominal films; blood work, including amylase and ­lipase B. Rectal biopsy, plain abdominal ­X-­rays, and barium ­enema C. Rectal biopsy and ­MRI 17. The parents of the infant you are caring for are very concerned about their baby’s ability to have a normal life with Hirschsprung’s disease. What can you tell t­ hem? A. Nothing; all information has to come from the ­physician B. There will be no residual ­problems C. Surgery may help, but their child may still have some ­long-­term ­issues 18. Which of the following problems is often associated with meconium plug and Hirschsprung’s d ­ isease? A. Down ­syndrome B. Cystic ­fibrosis C. VATER a ­ ssociation 19. A 26-week gestational age infant has a diaper full of bloody stool. What are some common causes of lower gastrointestinal ­bleeding? A. Infection, meconium plug, maternal drug ­abuse B. Maternal infection, lactose intolerance, necrotizing ­enterocolitis C. Hirschsprung’s ­disease 20. Which of the following preventive actions can reduce the risk of an infant developing necrotizing e ­ nterocolitis? A. Breastfeeding is the only prevention ­necessary B. Adequate fluids, reduced stress, and careful ­feedings C. Nasogastric tube and surgery to repair the ­perforation

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21. Which infant mentioned below is at the greatest risk of developing necrotizing ­enterocolitis? A. A 38-week gestational age, breastfed ­baby B. A 30-week gestational age baby whose mother had Salmonella food poisoning just prior to giving ­birth C. A 26-week gestational age baby who is currently ­stable 22. Aside from lower gastrointestinal bleeding, what are some additional symptoms seen with necrotizing ­enterocolitis? A. Lethargy, tachycardia, ­jitteriness B. Lethargy, apnea, bradycardia, abdominal ­distention C. Lethargy, tachypnea, ­fever 23. What could cause the 31-week gestational age infant you are feeding to suddenly gag and become a ­ pneic? A. Tracheoesophageal ­fistula B. Duodenal ­atresia C. Gastroesophageal ­reflux 24. Short bowel syndrome puts neonates at increased risk for which of the following ­deficiencies? A. Vitamins A, D, E, and K ­deficiencies B. Vitamin B complex and vitamin K ­deficiencies C. Vitamin C, B complex, and vitamin D ­deficiencies 25. Which of the following conditions is treated with cholestyramine in short bowel ­syndrome? A. ­Fat-­soluble vitamin ­malabsorption B. Cholestyramine is never used in short bowel ­syndrome C. ­Steatorrhea 26. What is the optimal postfeeding position for an infant with gastroesophageal ­reflux? A. Supine or right lateral position with the head of the crib at 30° B. Right lateral position with the head of the crib at 20° C. Prone or left lateral position with the head of the crib at 30° 27. What is the drug of choice for treating gastroesophageal ­reflux? A. ­Metoclopramide B. ­Questran C. ­Regitine 28. Prolonged TPN administration can lead to which of the following ­conditions? A. ­Hypoglycemia B. ­Cholestasis C. Short bowel ­syndrome

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29. Hyperbilirubinemia is the elevation of which of the following lab ­values? A. Total serum ­bilirubin B. Alanine ­transferase C. ­Polycythemia 30. The 48-­hour-­old, ­full-­term infant you are caring for has developed hyperbilirubinemia with a total serum bilirubin (TSB) of 16 mg/dl. You know this result indicates which of the ­following? A. This is a ­low-­r isk ­score B. This is a ­high-­r isk ­score C. This is a low internal risk ­score 31. A 48-­hour-­old infant has a total serum bilirubin of 14 mg/dl and is showing no sign of jaundice. How much phototherapy should this infant ­receive? A. 10–12 h ­ ours B. 5–7 h ­ ours C. No phototherapy is ­indicated 32. Polyhydramnios is associated with which of the following disorders in ­newborns? A. Chromosomal and anatomic ­disorders B. Neural tube ­defects C. ­CMV 33. The 34-week gestational age male infant you are caring for has failed to pass his meconium plug. The parents are upset at the thought of their newborn having a rectal biopsy. How would you counsel ­them? A. By saying, “It does not have to be done. He should pass the meconium in the next day or two.” B. By saying, “The biopsy involves taking a small bit of tissue from the rectum to verify disease.” C. After assessing their knowledge about the procedure, you fill in or clarify ­information 34. Upon initial assessment of a 28-week gestational age male, it is discovered that he has a high, imperforate anus. This diagnosis is highly significant for this infant ­because A. He needs surgery right away for a ­colostomy B. He will be fine after the bowel is ­reanastomosed C. He will have normal bowel function after ­surgery 35. What is the most common presentation of gastroesophageal reflux (GER)? A. Sandifer ­syndrome B. ­Flatulence C. ­Emesis

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36. After 3 weeks in the NICU, a 28-week gestational age male with Trisomy 18 begins having projectile vomiting of formula immediately after feeding. He is irritable but hungry. Which of the following is the most likely cause of his ­problem? A. Short bowel ­syndrome B. Hypertrophic pyloric ­stenosis C. ­Prader-­Willi ­syndrome 37. The mother of a 36-week gestational age infant is concerned about the frequency of the baby’s bowel movements. She states, “You always seem to be changing diapers when I’m here.” Which of the following statements is the most ­therapeutic? A. “This is nothing; you should see the baby in the next bassinet.” B. “He is having a lot of stools. I will call the physician.” C. “It is normal for a newborn to have stools 4–5 times a day.” 38. Which of the following conditions can cause early upper gastrointestinal bleeding in the ­neonate? A. Swallowed maternal ­blood B. Necrotic ­enterocolitis C. Infectious c­ olitis 39. Lower gastrointestinal bleeding is often caused by which of the ­following? A. Necrotizing ­enterocolitis B. Gastroesophageal reflux ­disease C. Intestinal ­polyps 40. The 32-week gestational age male you are caring for has developed idiopathic neonatal hepatitis. The parents are worried about their infant. You reassure them by ­explaining A. “This is a ­Crigler-­Najjar Type I syndrome. It is an autosomal recessive defect.” B. “This is a ­self-­limiting problem that resolves without complications in 80% of those affected.” C. “This is a serious condition that may require a liver transplant.” 41. What symptoms would you expect in a child with neonatal ­hepatitis? A. Jaundice, fever, tarry ­stools B. Jaundice, hepatomegaly, ­lethargy C. Jaundice, petechiae, ­hyperactivity 42. What treatments would you expect for an infant with neonatal ­hepatitis? A. No treatment is necessary as this is a ­self-­limiting ­disorder B. Breastmilk, corticosteroids, ­broad-­spectrum a ­ ntibiotics C. Adequate nutrition, fluids, ­fat-­soluble ­vitamins

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43. Jason is a 1-­day-­old, 37-week gestational age infant who has developed jaundice. He has been diagnosed with a1-antitrypsin deficiency liver disease. What lab findings would you expect for ­Jason? A. Elevated ­gamma-­glutamyl transpeptidase, ­clay-­colored ­stools B. Jaundice, decreased ­GGTP C. Increased LDH, ­jaundice 44. Common side effects of cimetidine ­include A. Elevated BUN and ­creatinine B. Rash, nausea, and a ­ gitation C. Decreased liver enzymes and ­anorexia 45. Cimetidine, famotidine, and ranitidine belong to what class of ­medications? A. Proton pump inhibitors (PPI) B. ­A ntacids C. H2 ­blockers 46. How do proton pump inhibitors (PPIs) ­work? A. They inhibit the release of hydrochloric acid by gastric parietal ­cells B. They antagonize the H2 histamine ­receptors C. They increase gastric emptying, reducing the acid ­production 47. Paul is a 36-week gestation, 22-­day-­old infant who has been receiving metoclopramide for 2 weeks. You notice abnormal movements of his limbs. As a NICU nurse, you suspect Paul is suffering ­from A. Restlessness, with no underlying ­condition B. Extrapyramidal symptoms from the ­drug C. Focal seizures; the physician must be notified at ­once 48. Matthew is a ­full-­term infant who was just delivered. He has Pierre Robin sequence. As Matthew’s NICU nurse, you should perform which of the following nursing a ­ ctions? A. Insertion of a nasogastric tube, lateral or prone positioning, airway ­monitoring B. Insertion of a nasogastric tube or begin TPN, supine ­positioning C. Insertion of a nasogastric tube and administration of oxygen via simple ­mask 49. Palate fusion is complete at A. ­4 B. ­8 C. ­12

 weeks ­gestation.

50. You are transporting an infant with severe abdominal distention and tenderness to a facility with a higher level of care. Which of the following consents should the team ensure is signed prior to ­departure? A. Consent for morphine ­administration B. Consent for blood ­transfusion C. Consent for ­pictures

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51. A baby with Pierre Robin sequence has similar feeding needs ­as A. A baby with a cleft ­lip/palate B. A baby with tracheal esophageal ­fistula C. A baby with diaphragmatic ­hernia 52. Your 26-week gestation patient is now 2 weeks old and receiving gavage feeds. Upon your next assessment, you aspirate ­t wo-­thirds of the previous feeding’s volume. You also note temperature instability, apneic and bradycardic episodes, lethargy, abdominal distention, and bloody stools with absent bowel tones. There is also grimacing when the abdomen is touched. You suspect which of the following staging criteria for N ­ EC? A. I ­A—­suspected N ­ EC B. I B—­suspected N ­ EC C. II A definite NEC—­mildly ­ill 53. Joan delivered a 28-week gestation neonate 5 days ago. She was taken to the ICU for hypovolemic shock, postuterine rupture, and resulting hysterectomy. She still wants to breastfeed and has begun pumping. What effect will the delay have on her total breastmilk s­ upply? A. Joan will produce exactly the same total amount of breastmilk regardless of her ­complications B. Joan will likely produce less breastmilk than the expected total if she had not had ­complications C. Joan will likely produce more breastmilk than the expected total if she had not had ­complications 54. You are at the delivery of a 38-week gestation infant. On assessment you note abdominal distention, wrinkled abdominal skin, and a lack of abdominal muscle tone. You ­suspect A. Potter’s ­syndrome B. ­Prune-­Belly ­syndrome C. Polycystic kidney ­disease 55. Soon after birth, a term newborn infant presents with increased oral secretions and mild respiratory distress. Which of the following is the most likely ­problem? A. Persistent pulmonary hypertension of the ­newborn B. ­Pneumonia C. Esophageal ­atresia 56. You are called to the delivery room to evaluate a newborn infant born at 37-weeks gestation who has an abdominal wall defect noted on delivery. Based on your initial physical examination, you diagnose an omphalocele. Which of the following statements is consistent with this clinical ­diagnosis? A. To rule out gastroschisis definitively, an abdominal computed tomographic scan is ­necessary B. Compared with gastroschisis, omphalocele is more frequently associated with other congenital ­malformations C. This abdominal wall defect is just lateral to the ­umbilicus

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57. A female infant born at 30-weeks gestation develops abdominal distention, abdominal tenderness, and bloody stools on the third day of life. Which of the following statements regarding the most likely diagnosis of necrotizing enterocolitis is ­correct? A. The diagnosis is supported by a double bubble sign on abdominal ­radiographs B. The diagnosis is supported by thickened bowel walls and pneumatosis intestinalis on abdominal ­radiographs C. The diagnosis is supported by a ­soap-­bubble appearance on abdominal ­radiographs 58. A healthy, ­full-­term infant requires how many kcal/kg/day for adequate growth and ­development? A. 70–90 B. 100–120 C. 150–180 59. Vitamin E is considered to ­be A. A treatment for ­anemia B. An effective o ­ xidant C. Absorbed readily when given in ­formula 60. Similac PM 60/40 is frequently given ­for A. ­Fatty-­acid metabolism ­defect B. Renal ­insufficiency C. Neurologic ­disease 61. S ­ oy-­based formulas are indicated for which of the following ­conditions? A. Low birth ­weight B. Bloody ­stools C. Lactase ­deficiency 62. An infant can be expected to coordinate swallowing, sucking, and breathing ­by A. 29–31 weeks g ­ estation B. 32–34 weeks g ­ estation C. 35–37 weeks g ­ estation 63. Portagen is frequently used for infants with which of the following ­conditions? A. Hepatic ­disorder B. ­Fatty-­acid ­metabolism C. Feeding i­ ntolerance 64. For the first 2 months of life, preterm infants require iron supplements if they are also ­receiving A. ­Erythropoietin B. Enteral ­feedings C. Parental nutrition with ­lipids

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65. Medications that are bound to protein may have which of the following ­effects? A. Increased ­availability B. Rapid ­distribution C. Less available drug for the desired ­effect 66. Which disease process may present similarly to a congenital diaphragmatic ­hernia? A. Pulmonary interstitial ­emphysema B. Congenital ­asthma C. Cystic adenomatoid ­malformation 67. A ­long-­term complication of congenital diaphragmatic repair ­is A. Gastroesophageal ­reflux B. ­Chylothorax C. Cor ­pulmonale 68. Pregestimil is indicated as a treatment ­for A. ­Sepsis B. Small gastric ­capacity C. ­Malabsorption

This concludes the Gastrointestinal System q ­ uestions.

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Gastrointestinal System A ­ nswers 1. Correct answer: ­A This collection of symptoms is classic for esophageal atresia and often occurs with a tracheoesophageal fistula. This condition occurs in 1 out of every 25,000 infants. Esophageal atresia is a failure of the esophagus to complete the connection between the throat and stomach, forming a blind pouch. There is often a fistula between the esophagus and the trachea called a tracheoesophageal fistula. Assessment of these infants includes insertion of a nasogastric tube into the blind pouch, then taking a chest ­X-­ray. Depending on the severity of the atresia, it is possible to close the fistula. It is important to close the fistula as soon as possible to reduce the risk of aspiration pneumonia. A ­G -­t ube may be inserted for feedings, and the nasogastric tube placed on low suction. Nursing care includes elevating the head of the bassinet, IV fluids, providing oxygen, and maintaining the nasogastric tube. Approximately half the infants born with esophageal atresia have other congenital problems such as VATER association, other gastrointenstinal abnormalities of the anus and rectum, renal malformations, cardiac abnormalities, and skeletal ­deformities. 2. Correct answer: ­C Intestinal atresia is the most common cause of intestinal obstruction in the newborn. ­Bile-­colored vomitus is the hallmark of intestinal atresia. The site of the obstruction may occur anywhere in the small or large bowel. Symptoms depend on the obstruction’s location. Duodenal atresia is often seen with Down syndrome. Malrotation with volvulus usually occurs at the duodenal–jejunal junction and is a surgical emergency. Jejunal atresia, often seen with meconium ileus, presents with bile vomitus at birth. Meconium plug syndrome is often seen with Hirschsprung’s disease and cystic fibrosis and causes diffuse distention of the ­bowel. 3. Correct answer: ­B Cystic fibrosis is often seen with meconium plug syndrome. Symptoms include abdominal distention, a salty taste, no meconium plug passage, and vomiting. ­X-­rays of the abdomen will show distention with fine bubbles in the bowel. Most states require infant testing for cystic fibrosis as a part of the national newborn screening requirements. Gastrointestinal abnormalities that occur with cystic fibrosis in addition to meconium plug syndrome include rectal prolapses, pancreatic disorders, chronic liver disease, failure to thrive, low protein levels with edema, ­fat-­soluble vitamin deficiencies, sodium depletion, electrolyte imbalances, and ­alkalosis. 4. Correct answer: ­C Hirschsprung’s disease is a congenital abnormality seen with meconium plug. It is a neural tube defect in which the ganglia that enervates the distal bowel and rectum do not migrate caudally (toward the feet). There is often a strong family history of Hirschsprung’s disease, especially in males, who are affected 5:1 over females. Symptoms include vomiting (sometimes bilious), constipation, and abdominal obstruction. A rectal biopsy shows a lacks of ganglionic cells in the rectum. Surgical resection of the affected area may ­help.

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5. Correct answer: ­B The organs of the gastrointestinal system begin developing at 4 weeks gestation and are generally well defined by 8 weeks. At 10 weeks, the midgut moves to the abdomen. It is during this time that a problem may occur with herniation of some or all of the gastrointestinal contents. At 12 weeks, the gastrointestinal tract begins ­peristalsis. 6. Correct answer: ­A An omphalocele is the herniation at the umbilicus of abdominal contents, contained in a sac that may include the large and small bowel, liver, pancreas, and spleen. An omphalocele is often seen with other congenital abnormalities such as other gastrointestinal disorders, congenital cardiac anomalies, and chromosomal disorders. Gastroschisis is the herniation of the intestines only through an abdominal wall defect. There is no sac covering the intestines in ­gastroschsis. 7. Correct answer: ­C Gastroschisis is the herniation of the intestines through a defect in the abdominal wall and is usually to the right of the umbilicus. Unlike omphalocele, gastroschisis does not involve other gastrointestinal organs, nor are the intestines contained in a sac. The surgery can be difficult as the bowel may be friable, and there is a chance of perforation. Infants with gastroschisis rarely have any other congenital ­anomalies. 8. Correct answer: ­A A diaphragmatic hernia is a medical emergency. If there is a defect in the development of the diaphragm, the abdominal contents can herniate into the thorax, preventing lung development. Because of this, it is often difficult to tell if the lungs are developed enough to sustain life. An infant with a diaphragmatic hernia is at great risk for pneumothorax. Pulmonary hypertension is a postoperative risk that may require special ventilation. ­One-­third of infants with a diaphragmatic hernia die, even with ­treatment. 9. Correct answer: ­B Immediate intubation and mechanical ventilation with 100% oxygen is the only approved oxygenation method for this infant. A ­bag-­mask device can cause distention of the bowel that is already impeding breathing. The simple oxygen mask and ­blow-­by are inappropriate in this ­case. 10. Correct answer: ­A Scaphoid means “hollowed out” or “boat shaped.” The abdomen of an infant with a diaphragmatic hernia is sunken or concave in shape because the abdominal contents are in the thoracic ­cavity. 11. Correct answer: ­A VATER also include VATERR, VACTRL, and VACTERL associations. VATER includes vertebral anomalies, anal anomalies, tracheoesophageal fistula, esophageal atresia, and renal anomalies. The other forms of this condition may include cardiac defects, radial dysplasia, limb anomalies, and single umbilical artery. The cause of these anomalies is unknown, but it is believed the defects begin as early as 4–6 weeks of gestational age. VATER association may require numerous surgeries to correct the anomalies as the child ­grows.

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12. Correct answer: ­C The bilious vomitus is the hallmark of malrotation and volvulus. This is a surgical emergency to prevent necrotic bowel. Duodenal atresia may also have bilious vomitus, but a dilated abdomen and duodenum are seen on abdominal ­X-­rays. Meconium plug and Hirschsprung’s disease have abdominal distention and delayed stooling as their ­hallmark. 13. Correct answer: ­B It is important to decompress the stomach and decrease the risk of aspiration. Most infants return to normal bowel function within 10 days. The NPO status is maintained until bowel motility has resumed. Feedings are then slowly resumed. The infant may be maintained on TPN to allow for gut rest. Antibiotics are routinely given in these ­cases. 14. Correct answer: ­C Duodenal atresia is seen in about 30% of infants born with Down syndrome. On ­X-­ray, a double bubble of gas is often seen. Treatment will include a nasogastric tube, NPO status, and IV fluids. The infant may also receive ­antibiotics. 15. Correct answer: ­A Approximately 30% of infants with ductal atresia (biliary atresia) also have congenital heart defects, malrotation of the gut, tracheoesophageal abnormalities, and anorectal defects. Generally, ductal atresia has an excellent ­prognosis. 16. Correct answer: ­B After plain films, a barium enema may be performed. The rectal biopsy is the best diagnostic tool for Hirschsprung’s disease. The biopsy shows a lack of parasympathetic ganglions in the distal bowel and rectum. This problem usually develops around the 12th week of gestation. Hirschsprung’s is seen in males four to five times more often than in females, and it may also be seen in ­families. 17. Correct answer: ­C With Hirschsprung’s disease, surgery may help, but their child may still have some ­long-­term issues. Surgery is done to move the innervated bowel to the anus. There may be some delay in bowel training. In some cases, a child with Hirschprung’s disease has fecal incontinence or chronic ­constipation. 18. Correct answer: ­B Cystic fibrosis is associated with meconium plug and Hirschsprung’s disease in about 10% of cases. All 50 states require chloride sweat testing on all newborns to test for cystic ­fibrosis. 19. Correct answer: ­B Lower gastrointestinal bleeding in the neonate is often due to maternal infection such as Salmonella or lactose intolerance. It is also common in infants under stress, such as in prematurity. The stress of prematurity can lead to necrotizing ­enterocolitis.

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20. Correct answer: ­B Prevention of necrotizing enterocolitis can be maximized by keeping an infant’s stress to a minimum, maintaining adequate fluids, and starting feedings slowly. Prebiotics and probiotics such as Bifidobacterium species have been administered in some hospitals, but there is no ­long-­term study demonstrating either safety or ­efficacy. 21. Correct answer: ­B A maternal infection such as Salmonella can lead to necrotizing enterocolitis in an infant. This infant should be carefully monitored for abdominal distention, hypotension, lethargy, and bloody ­stools. 22. Correct answer: ­B In addition to abdominal distention, symptoms seen with necrotizing enterocolitis include increased gastric residuals, lethargy, apnea, bradycardia, temperature instability, decreased urine output, hypotension, and bilious ­vomitus. 23. Correct answer: ­C Gastroesophageal reflux is considered a common physiologic condition that occurs in most people. In infants, the condition may spontaneously resolve within 12–18 months. It becomes a problem when they develop failure to thrive, apnea, aspiration, or stridor. Treatment involves using a thickening agent, feeding smaller volumes with more frequency, increasing the head of the crib to 45–60% after feeding, and keeping the baby still during ­feedings. 24. Correct answer: ­A Vitamins A, D, E, and K are ­fat-­soluble vitamins that are absorbed in the ileum. The ileum is responsible for the absorption of the majority of the nutrition consumed, B12 , and bile salts, in addition to the ­fat-­soluble vitamins. In the neonate, the two most common causes of short bowel syndrome are volvulus and necrotizing ­enterocolitis. 25. Correct answer: ­C Cholestyramine is used to treat steatorrhea, which is a fatty stool that can occur with short bowel syndrome where absorption is ­interrupted. 26. Correct answer: ­C The optimal postfeeding position for an infant with gastroesophageal reflux is the prone or left lateral position to facilitate gastric emptying. Keeping the head of the crib elevated to 30° helps reduce ­reflux. 27. Correct answer: ­A Metoclopramide increases gastric emptying and motility, thereby reducing reflux symptoms. The infant should be monitored for diarrhea and neurologic signs such as tremors or muscle ­t witching. 28. Correct answer: ­B Cholestasis is the impaired flow of bile and may be seen with other conditions such as cystic fibrosis, biliary atresia, and infection. Cholestasis can also occur as a result of ­cholelithiasis.

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29. Correct answer: ­A Total serum bilirubin (TSB) is the measure of hyperbilirubinemia. The condition may be mild to severe. A patient with hyperbilirubinemia presents with jaundice in a cephalocaudal, or ­head-­to-­toe, pattern and resolves in the reverse order. If hyperbilirubinemia occurs acutely and if it is severe enough, it can cause severe neurologic damage and ­death. 30. Correct answer: ­B A TSB of 16 mg/dl in a 48-­hour-­old infant is a ­high-­r isk score. This infant may develop kernicterus or brain damage from the bilirubin entering the brain tissue. If the bilirubin is deposited in the basal ganglia, it may cause irreversible brain damage. The baby may exhibit lethargy, hypotonia, or poor sucking. The process may progress to hypertonia, opisthotonus, and a hallmark ­high-­pitched cry. When the TSB is 20 mg/dl or more, the most likely cause is Rh factor ­incompatibility. 31. Correct answer: ­C An infant who has a TSB of 14 mg/dl and who is showing no sign of jaundice should receive no phototherapy. The key to this question is that the infant is not jaundiced. This puts him in a ­low-­r isk category. When a ­f ull-­term infant develops hyperbilirubinemia and is otherwise stable, he can be monitored for a day or two. If the TSB rises, then phototherapy could be ­started. 32. Correct answer: ­A Polyhydramnios is defined as greater than 2 liters of amniotic fluid. The exact cause for the majority of cases is not known, but it is seen with chromosomal and anatomic disorders in the baby and gestational diabetes in the mother. Chromosomal disorders such as Down and Edwards’ syndromes produce polyhydramnios. Anatomic disorders that affect swallowing include tracheoesophageal fistula, duodenal atresia, intestinal obstruction, and annular ­pancreas. 33. Correct answer: ­C After assessing their knowledge about the rectal biopsy, you fill in or clarify information. It is always prudent to ascertain the knowledge level and comprehension of the parents before they agree to a procedure. If the physician has already obtained consent, the physician should be notified of their concerns prior to the ­procedure. 34. Correct answer: ­A A high, imperforate anus is a surgical emergency that often requires a colostomy. The bowel will be reanastomosed to the rectum eventually, but the child may have malabsorption problems and fecal incontinence in the ­f uture. 35. Correct answer: ­C Emesis is the most common symptom of gastroesophogeal reflux, and it may be dramatic. Infants with gastroesophageal reflux are sometimes called “happy spitters.” More than 50% of infants have episodes of spitting up that are not pathologic, and for most infants, this condition resolves in the first year of ­life.

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36. Correct answer: ­B Hypertrophic pyloric stenosis is seen 2–3 weeks after birth with nonbilious vomitus that is usually formula. This condition is often seen in infants with Trisomy 18, tracheoesophageal fistula, and duodenal atresia. These children are at high risk for fluid and electrolyte imbalances. Hypertrophic pyloric stenosis may be corrected with a ­pyloroplasty. 37. Correct answer: ­C Newborns typically stool 4–5 times a day. This number decreases to 1–2 per day by age 1 ­year. 38. Correct answer: ­A Early upper gastrointestinal bleeding in the neonate may be caused by swallowing maternal blood during birth. Blood from fissured nipples may also be swallowed and later ­vomited. 39. Correct answer: ­A Necrotizing enterocolitis is the primary cause of lower gastrointestinal bleeding in neonates. Other causes include swallowed maternal blood, allergic colitis, Hirschsprung’s disease, and volvulus. Gastroesophageal reflux disease, intestinal polyps, and ­Mallory-­Weiss tear are seen in older ­children. 40. Correct answer: ­B Idiopathic neonatal hepatitis is an inflammation of the liver, and often the cause is unknown. About 80% of the infants recover fully. The nonidiopathic causes of neonatal hepatitis include metabolic, genetic, or infectious ­origins. 41. Correct answer: ­B Infants with neonatal hepatitis may present with jaundice, hepatomegaly, vomiting, poor sucking, anorexia, lethargy, fever, and petechiae. The causes of neonatal hepatitis can range from idiopathic to inborn metabolic errors and ­infection. 42. Correct answer: ­C Treatments you would expect for an infant with neonatal hepatitis include adequate nutrition, fluids, and ­fat-­soluble vitamins. The infant may not be able to tolerate PO nutrition and may need IV fluids or TPN and ­fat-­soluble vitamins. Corticosteroids would be contraindicated in this case. Antibiotics or antivirals should only be given if the diagnosis warrants their ­use. 43. Correct answer: ­A An infant with a1-antitrypsin (A1-AT) deficiency liver disease has increased gamma­glutamyl transpeptidase (GGTP) levels, jaundice, ­clay-­colored stools, and malabsorption. Small gestational age, hepatosplenomegaly, and a family history of emphysema or cirrhosis will also be seen with this condition. Jason will probably need a liver transplant in the future. He is susceptible also to developing emphysema or cirrhosis at a young a ­ ge.

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44. Correct answer: ­B Adverse reactions to cimetidine can include rash, nausea, agitation, vomiting, diarrhea, flushing, neutropenia, elevated liver enzymes, and drowsiness, to name a ­few. 45. Correct answer: ­C Cimetidine, famotidine, and ranitidine are H2 blockers. They inhibit gastric acid production by antagonism of the histamine H2 receptors. They are used for prevention of ulcers and in the treatment of gastrointestinal ­bleeding. 46. Correct answer: ­A Proton pump inhibitors work by inhibiting an enzyme that triggers the gastric parietal cells to release hydrochloric acid. This reduction in acid production can reduce the effectiveness of many oral medications such as antifungals. It may increase the absorption of drugs such as digoxin and ­f urosemide. 47. Correct answer: ­B The infant is having extrapyramidal symptoms due to the metoclopramide. Metoclopramide has antidopaminergic properties that affect the nervous system both centrally and peripherally. The drug also facilitates the release of acetylcholine—­a neurostimulator—­and has a prokinetic ­effect. 48. Correct answer: ­A Pierre Robin sequence is a congenital defect that occurs at about 7–10 weeks gestation. The condition is characterized by a small mandible (micrognathia), retraction of the tongue, and a ­U-­shaped cleft palate. Infants with Pierre Robin sequence need special teats or a nasogastric tube for feeding. They need to be positioned laterally or prone as the tongue will otherwise occlude the airway. Airway management is imperative and may require intubation or a tracheostomy. Children with this condition often have recurrent otitis media. They should be fed in an upright ­position. 49. Correct answer: ­C Palate fusion is complete at 12 weeks ­gestation. 50. Correct answer: ­B During transportation of a critically ill infant, it is important to obtain consent for blood and blood products in addition to consent for transport and treatment. The patient presents with severe gastrointestinal distress; if the cause is related to perforation or megacolon, sepsis and hypovolemic shock may result, threatening life. Pro­ active staff will anticipate potential administration of blood and blood products and prevent delay in treatment if the patient becomes ­critical. 51. Correct answer: ­A Patients with Pierre Robin sequence often have cleft palates, resulting in additional respiratory and nutritional challenges. It is important to establish a multidisciplinary approach to the care of such a patient by consulting speech therapists, craniofacial surgeons, and lactation consultants in addition to routine multidisciplinary team ­involvement.

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52. Correct answer: ­C You should suspect II A definite NEC if the neonate presents with temperature instability, apnea, bradycardia, lethargy, abdominal distention with high residual volumes and/or bilious return, abdominal tenderness, and bloody stools with absent bowel tones. Respiratory support as well as ­anti-­inflammatory drugs should be started immediately. Diagnosis should rule out ­perforation. 53. Correct answer: ­B Joan will likely produce less breastmilk than the expected total if she had not had complications of hypovolemic shock with delayed ­pumping. 54. Correct answer: ­B You would suspect P ­ rune-­Belly syndrome if the neonate presented with abdominal distention, wrinkled abdominal skin, and a lack of abdominal muscle ­tone. 55. Correct answer: ­C If a term newborn infant presents with increased oral secretions and mild respiratory distress, you should anticipate esophageal atresia. Do not feed the infant; attempt to pass an orogastric tube and obtain a chest ­X-­ray. Esophageal atresia will be noted at the end of the orogastric tube, which will be curled in the esophageal ­pouch. 56. Correct answer: ­B Compared with gastroschisis, omphalocele is more frequently associated with other congenital malformations. This patient will need to have genetic testing completed and possible genetic counseling, depending upon the number and type of additional congenital anomalies ­identified. 57. Correct answer: ­B The diagnosis of necrotizing enterocolitis is supported by thickened bowel walls and pneumatosis intestinalis on abdominal radiographs. Clinical presentation includes abdominal distention, abdominal tenderness, and bloody ­stools. 58. Correct answer: ­C A healthy, ­f ull-­term infant requires 150–180 kcal/kg/day for adequate growth and development. Premature infants require significantly more; this varies based on degree of prematurity and concurrent disease process(es). 59. Correct answer: ­A Vitamin E is a ­fat-­soluble vitamin that is used concurrently with iron and erythropoietin to treat anemia. Vitamin E is also an antioxidant and has been used in the healing of damaged ­tissues. 60. Correct answer: ­B Similac PM 60/40 is frequently given to neonates with renal insufficiency. This formula has a low mineral content, low iron content, and electrolyte ­balance.

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61. Correct answer: ­C Soy-­based formulas are indicated for lactase deficiency as the carbohydrate base is plant and not lactose based. Lactase is the enzyme that breaks down ­lactose. 62. Correct answer: ­B An infant can be expected to coordinate swallowing, sucking, and breathing by 32–34 weeks g ­ estation. 63. Correct answer: ­B Portagen is frequently used for infants with ­fatty-­acid metabolism deficiency. These patients are unable to digest and utilize the ­long-­chain fatty acids from ­foods. 64. Correct answer: ­A For the first 2 months of life, a preterm infant requires iron supplements if also receiving erythropoietin. The purpose of erythropoietin is to build red blood cells. Hemoglobin is a crucial element in ­oxygen-­carrying capacity and requires sufficient iron stores to create the h ­ emoglobin. 65. Correct answer: ­C Medications that are bound to protein may have less available drug for the desired ­effect. 66. Correct answer: ­C Cystic adenomatoid malformation may present similarly to a congenital diaphragmatic hernia. Cystic adenomatoid malformation is a nonfunctional cystic mass that replaces a portion of lung. If the mass grows beyond the size of the normal lung tissue it replaces, the mass will impede lung growth and function of surrounding tissue. Presentation is similar to diaphragmatic hernia, but without the bowel tones in the thoracic cavity and the scaphoid abdomen. Cystic adenomatoid is typically found and monitored via fetal ultrasound prior to birth. Neonatal surgical intervention with removal of the cyst has a positive prognosis. Respiratory support is provided, depending upon the severity and size of the cyst until surgery is ­completed. 67. Correct answer: ­A Gastroesophageal reflux is a ­long-­term complication of congenital diaphragmatic repair. Parent teaching should include management and prevention of reflux and aspiration with resulting respiratory ­distress. 68. Correct answer: ­C Pregestimil is indicated for fat malabsorption treatment. Conditions that are associated with fat malabsorption include cystic fibrosis, short bowel syndrome, and protein malnutrition. Pregestimil protein is from ­medium-­chain triglycerides (MCT) ­oil.

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Gastrointestinal System R ­ eferences Anonymous. (2003, August). Cystic fibrosis: Neonatal intestinal obstruction is an indicator for the need to screen for CF. Gastroenterology Week, ­12. Applegate, K. (2009). ­Evidence-­based diagnosis of malrotation and volvulus. Pediatric Radiology, 39(Suppl.), 161–163. Birmingham Children’s Hospital. (2008, December). ­Pierre-­Robin syndrome: Research from Birmingham Children’s Hospital NHS Trust in the area of ­Pierre-­Robin syndrome in children published. Health & Medicine Week, ­2903. Columbus Children’s Hospital. (2009, July). Pediatric surgery: Research from Columbus Children’s Hospital yields new data on pediatric surgery. Pediatrics Week, 1 ­ 15. Dartmouth Hitchcock Medical Center. (2009). Polyhydramnios. Retrieved from http://www.dhmc .org/webpage.cfm?site_id=2&org_id=828&morg_id=0&sec_id=0&gsec_id=47098&­item_id =47108 Eghbalian, F., Monsef, A., & ­Mousavi-­Bahar, S. (2009). Urinary tract and other associated anomalies in newborns with esophageal atresia. Urology Journal, 6(2), 123–126. George Washington University. (2009, August). Pediatrics: Findings in pediatrics reported from George Washington University. Pediatrics Week, ­199. Gomes Ferreira, C., Reinberg, O., Becmeur, F., et al. (2009). Neonatal minimally invasive surgery for congenital diaphragmatic hernias: A multicenter study using thoracoscopy or laparoscopy. Surgical Endoscopy, 23(7), 1650–1659. Harvard University. (2008, September). Pediatric gastroenterology and nutrition: New pediatric gastroenterology and nutrition research from Harvard University discussed. Pediatrics Week, ­25. Jiang, D., Xu, C., Wu, B., et al. (2009). Effects of botulinum toxin injection on anal achalasia after ­pull-­through operations for Hirschsprung’s disease: A 1-year ­follow-­up study. International Journal of Colorectal Disease, 24(5), 597–598. Lillehei, C., & Hansen, A. (2009). SIX: Respiratory disorders: Part 1: Esophageal atresia and tracheoesophageal fistula (pp. 159–168). People’s Medical Publishing House USA Ltd (PMPH). Linder, C. E.,. (2009, June). Perinatal and neonatal nursing: New perinatal and neonatal nursing research from C. E. Linder and colleagues discussed. Health & Medicine Week, ­2410. The National Craniofacial Association. (2006). Pierre Robin sequence. Retrieved from http://www. faces-­cranio.org/Disord/PierreRobin.htm Nicholson, P. (2006). Multidisciplinary approach benefits neonatal short bowel syndrome. Medical Post, 42(32), 1 ­ 6. Oestreicher-­Kedem, Y., DeRowe, A., Nagar, H., et al. (2008). Vocal fold paralysis in infants with tracheoesophageal fistula. The Annals of Otology, Rhinology, and Laryngology, 117(12), 896–901. Olgun, H. (2009, May). Gastroenterology: New gastroenterology study findings recently were reported by H. Olgun and c­ o-­researchers. Cardiovascular Week, ­166. Root, E. (2009). The ecology of birth defects: ­Socio-­economic and environmental determinants of gastroschisis in North Carolina (Doctoral dissertation, The University of North Carolina at Chapel Hill, United States—­North Carolina.) Retrieved from Dissertations & Theses: Full ­text. Sanjay Gandhi Postgraduate Institute of Medical Sciences. (2009, February). Biliary atresia epidemiology. Studies from Sanjay Gandhi Postgraduate Institute of Medical Sciences, Department of Pathology in the area of biliary atresia epidemiology described. Hepatitis Weekly, 3 ­ 1. Shew, S. (2009). Surgical concerns in malrotation and midgut volvulus. Pediatric Radiology, 39 (Suppl.), 167–171. Stanford School of Medicine. (2009). Bilirubin screening and management of hyperbilirubinemia. Retrieved from http://newborns.stanford.edu/BiliSummary.html#­PhototxGuide. University of Heidelberg. (2009, February). Diaphragmatic hernia: Research from University of Heidelberg provide new insights into diaphragmatic hernia. Gastroenterology Week, ­196.

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Gastrointestinal ­System  y  181 University of Washington. (2009, August). Hirschsprung disease: Research in the area of Hirschsprung disease reported from University of Washington. Gastroenterology Week, ­105. Wu, Y., Yan, Z., Hong, L., et al. (2009). Thoracoscopic repair of congenital esophageal atresia in infants. Journal of Laparoendoscopic & Advanced Surgical Techniques, 19(3), 461–463. Yale University Medical Department. (2008, November). Cleft lip: Studies from Yale University, medical department further understanding of cleft lip. Genomics & Genetics Weekly, ­52.

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Section 7 Genitourinary ­System

Questions 1. In utero, which of the following is responsible for maintaining fluid regulation and electrolyte balance in the ­fetus? A. The fetal ­kidneys B. The p ­ lacenta C. The fetal intestinal ­tract 2. Fetal urination is most pronounced during which ­trimester? A. First ­trimester B. Second ­trimester C. Third ­trimester 3. Urine formation occurs in which part of the neonate’s ­kidneys? A. The Bowman’s c­ apsule B. The n ­ ephron C. The loop of ­Henle 4. Nephrogenesis begins ­during A. The 2nd week of g ­ estation B. The 2nd month of ­gestation C. The 2nd t­ rimester 5. Ultrafiltration in the kidneys of a neonate is driven ­by A. ­Pressure B. ­Chemicals C. ­Osmosis 6. The blood urea nitrogen and creatinine levels are elevated on your 28-week gestation patient at day 2 of life. These results ­are A. Normal, and you would notify the physician when ­available B. Abnormal, and you should call the physician STAT for a dopamine order at 5 ­mcg/kg/min C. Abnormal, and you should call the physician STAT for a saline bolus at 20 ml/kg. 7. A significant drop in arterial blood pressure may result in the activation o ­f A. The parasympathetic nervous ­system B. The ­renin-­angiotensin-­aldosterone ­system C. The renal prostaglandin ­system

y

y

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8. Normal urine output in a neonate is considered to ­be A. 0.5–1.5 ­ml/kg/day B. 1–3 ­ml/kg/day C. 2–5 ­ml/kg/day 9. You are caring for a 24-week gestation neonate at 3 days of life. The neonate is intubated on conventional ventilation support at 30% FiO2 . A d ­ ouble-­walled isolette at 65% humidity and a ­K-­pad are in place. Double phototherapy was started for hyperbilirubinemia. Which of the following treatments would contribute most to insensible water l­ oss? A. Conventional ­ventilation B. 30% ­FiO2 C. ­Phototherapy 10. Joey is a 25-week gestation neonate at day 3 of life. He is currently on conventional ventilation at 60% FiO2 . He has a right chest tube ­post ­pneumothorax. He is receiving a fentanyl drip in addition to TPN and intralipids. He has generalized 12 edema, crackles in all lung fields, and a night weight gain of 100 grams. Labs show blood Na 120 mEq/L, K 3.2, and Cl 91, and urine analysis shows elevated Na levels and osmolality. Urine output is 0.75 ml/kg/day for the last 24 hours. Upon reporting your findings to the physician, you would anticipate an order t­ o A. Decrease sterile water feedings, Lasix administration, and NaCl ­supplementation B. Increase sterile water feedings, Dyazide administration, and K+ ­supplementation C. Continue same sterile water feedings and Lasix ­administration 11. You are caring for Angel, a 34-week gestation neonate who is the recipient of a ­t win-­to-­t win transfusion on day 2 of life. There are multiple bruises on her torso and extremities. Morning labs reveal a Hgb of 17.9  g/dl and a Hct of 66%. Mean arterial blood pressure is 45. She is on 1 L/m nasal cannula at 25% FiO2 with saturation at 92%. Intravenous therapy includes TPN of D10 at 90 ml/kg/d via peripheral line. Urine output for the last 24 hours is 1.3 ml/kg/d. The low urine output is probably due to ­the A. Hgb and ­Hct B. Potential ­hyperbilirubinemia C. Low oxygen ­saturation 12. You just drew an arterial blood gas from your patient’s umbilical arterial line. The pH is 7.24. What is the expected response from the kidneys to compensate for this acid–base ­deficit? A. Increased secretion of hydrogen ions in the distal ­t ubule B. Reabsorption of bicarbonate in the distal ­t ubules C. Excretion of ­ammonia 13. Prerenal acute renal failure is best described ­as A. Relative hypoperfusion of a normal ­kidney B. Renal cellular damage compromising ­f unction C. Obstruction of flow distal from the ­kidneys

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14. You are receiving report on a 37-week gestation neonate delivered via emergency cesarean section due to the mother’s uterine hemorrhage. The mother was involved in a motor vehicle accident yesterday. Apgar scores were 1 at 1 minute, 3 at 5 minutes, 4 at 10 minutes, and 7 at 15 minutes. The neonate was intubated immediately for poor chest rise via ­positive-­pressure ventilation with b ­ ag-­mask support. At delivery, umbilical lines were placed with difficulty. Intravenous epinephrine and volume expanders were administered. Urine output is 0.8 ml/kg/h. You would suspect the baby is in acute renal failure. Lab results usually seen with acute renal failure i­ nclude A. Urine osmolarity 0.2000 ­mMol/kg B. Azotemia with blood urea nitrogen greater than 10 mg/dl and rising 5 ­mg/dl/day C. Serum creatinine greater than 1.5 mg/dl and rising more than 0.2 mg/dl per ­day 15. The urine analysis for your patient indicates the presence of casts, tubular cells, and proteinuria. This result ­indicates A. Prerenal ­failure B. Intrinsic renal ­failure C. Postrenal ­failure 16. Your patient has been diagnosed as having acute renal failure. Which of the following electrolyte imbalances would be ­expected? A. ­Hyperkalemia B. ­Hypernatremia C. ­Hypophosphatemia 17. Which of the following statements is true regarding a treatment for renal ­failure? A. A fluid challenge is used to rule out postrenal causes of renal ­failure B. A urine output of less than 2 ml/kg/h after furosemide administration indicates intrinsic or postrenal causes of renal ­failure C. Rapid and sustained diuresis within 1–2 hours of fluid bolus indicates postrenal causes for renal ­failure 18. Your term gestation patient was diagnosed as having acute pyelonephritis. Which of the following statements is true regarding fluid management for this ­patient? A. The patient should have a 20–30 g/d weight ­gain B. Fluids should not be ­restricted C. Fluid management should be based on 30 ml/kg/d plus urinary fluid ­output 19. High blood pressure in a neonate is defined ­as A. A blood pressure is consistently higher than the 50th ­percentile B. A blood pressure is consistently higher than the 75th ­percentile C. A blood pressure is consistently higher than the 95th ­percentile

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20. Your patient is a term neonate who was exposed to cocaine and heroin during pregnancy. What effect would this have on neonatal blood ­pressure? A. Blood pressure would be lower than ­normal B. Blood pressure would be higher than ­normal C. Blood pressure would not be ­affected 21. Baby Lee is a 24-week gestation neonate at day 30 of life. He is intubated on conventional ventilation with a chest tube p ­ ost pneumothorax. TPN, intralipids, and fentanyl are infusing via a right arm PICC line. You note mild general edema, strong and bounding peripheral pulses, and capillary refill is , 3 seconds. Baby Lee’s heart rate is 180, respiratory rate is 76, and the O2 saturation is 92% on 30% FiO2 . Cuff blood pressure is 96/67 (MAP77). Your first action should be t­ o A. Call the physician for a hydralazine ­order B. Verify that the cuff size you are using is half the arm ­diameter C. Check the intravenous line site to verify patency 22. A term infant would be considered as being hypertensive if the blood pressure ­was A. ­98/69 B. ­85/52 C. ­77/34 23. You have just recorded a blood pressure of 90/55 for your 33-week gestation patient. You receive an order for antihypertensive medication. Which of the following orders would you ­question? A. Hydralazine 0.5 mg/kg/dose IV every 8 ­hours B. Propranolol 1 mg/kg/dose IV ­BID C. Enalapril 0.1 mg/kg/day PO in two divided ­doses 24. Which of the following statements is true regarding Oligohydramnios ­syndrome? A. Oligohydramnios syndrome is also known as Potter’s ­syndrome B. Oligohydramnios syndrome is more common in females than ­males C. Death is rare after Oligohydramnios syndrome is ­diagnosed 25. You are called to a vaginal delivery for a woman who received no prenatal care and has no amniotic fluid. Which of the following assessment criteria for her baby would support a diagnosis of Potter’s ­syndrome? A. Blunted nose with protruding chin, ­low-­set ears, and narrowly spaced ­eyes B. Small for gestation, bowed legs, clubbed feet, and severe respiratory ­distress C. ­Barrel-­shaped chest, large ­claw-­shaped hands, and dry ­skin 26. Which of the following actions should be taken when caring for an infant diagnosed with Potter’s ­syndrome? A. Show the parents how to take a baby’s ­temperature B. Inform the parents that the baby must be kept in a ­side-­lying p ­ osition C. Assist with the arrangement of a baptism at family ­request

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27. Bilateral multicystic dysplastic kidneys a ­ re A. Functional ­kidneys B. More common than a unilateral multicystic dysplastic ­kidney C. Not compatible with ­life 28. Which of the following statements is true regarding the difference between autosomal recessive polycystic kidney disease (ARPKD) and autosomal dominant polycystic kidney disease (ADPKD)? A. ARPKD cysts are large and look like a “snowstorm” on ultrasound, while ADPKD cysts are variable in ­size B. ARPKD is more common than ­ADPKD C. Liver cysts are found only with ­ARPKD 29. Hydronephrosis is best defined ­as A. Dilation of the pelvis and calyces of one or both kidneys due to ­obstruction B. Dysplasia of one or both ­kidneys C. Hypoperfusion of one or both kidneys resulting in poor urine ­output 30. Baby Steve was diagnosed as having ­Prune-­Belly syndrome. The parents are asking you what assessment criteria were used to make the diagnosis. You should ­explain A. “Steve has hyperplasia of the abdominal wall muscles.” B. “Steve has unilateral cryptorchidism.” C. “Steve has urinary tract abnormalities on ultrasound.” 31. Your 32-week gestation patient was diagnosed with mild unilateral hydronephrosis. Urine output is 1.5 ml/kg/h. The parents ask you if surgery will be necessary. You should ­answer A. “Surgery is always indicated and will occur at 3 months of life.” B. “Immediate surgery is indicated despite normal levels of amniotic fluid at birth.” C. “The need for surgery is dependent upon the cause and severity of the hydronephrosis.” 32. During your first assessment you note that your 29-week gestation patient on day 3 of life has a right flank mass with frank blood noted in the diaper. You do not see any open wounds and the anus appears normal. Your patient is NPO and receiving nutrition via TPN and intralipids via a UVC. Arterial blood pressure is monitored via a UAC and indicates hypertension. Phototherapy was started yesterday for a bilirubin level of 6.4 mg/dl. Morning labs show a Hgb of 12.9 ­g­/­dl, Hct of 45%, and PLT count of 36,000. You notify the physician immediately because you ­suspect A. Renal vein ­thrombosis B. Renal artery ­thrombosis C. A Wilms’ ­t umor

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33. You just attended a cesarean section for a term infant of a mother with severe ­pregnancy-­induced hypertension, Upon delivery, you note bladder exstrophy on the infant. The parents ask you when this condition occurred. Your appropriate reply should ­be A. “Bladder exstrophy occurs during the 4th and 5th weeks of fetal development.” B. “Bladder exstrophy occurs during the 6th and 8th weeks of fetal development.” C. “Bladder exstrophy occurs during the 9th and 10th weeks of fetal development.” 34. You are at the delivery of a 35-week gestation infant. During assessment you note a scrotal hypospadia, a bifid scrotum, and nonpalpable gonads. You are asked the sex of the infant for documentation and admission paperwork. You should ­say A. “The infant is a boy.” B. “The infant is a girl.” C. “The genitalia are ambiguous.” 35. You are caring for a term baby being treated for a urinary tract infection. Ampicillin and gentamicin were initiated. The parents ask you how long the baby will remain in the NICU on antibiotic therapy. An appropriate answer at this time would ­be A. “Antibiotic therapy will be stopped after 3 days.” B. “Antibiotic therapy will continue for 7 days.” C. “Antibiotic therapy will continue for 14 days and then an additional 3 days if a repeat culture is negative.” 36. Your term patient is noted to have an enlarged scrotal sac without a palpable mass. Rapid assessment is used to differentiate between hydrocele and an incarcerated inguinal hernia. Which of the following procedures would be utilized to differentiate these ­conditions? A. Aspiration of fluid by the ­physician B. Transillumination of the scrotal ­sac C. Insertion of urinary ­catheter 37. You are caring for a 38-week gestation patient who underwent surgery yesterday for testicular torsion. Nursing actions i­ nclude A. Measuring the abdominal girth every 8 ­hours B. Inserting a nasogastric tube for continuous ­suction C. Positioning the patient supine or ­side-­lying ­only 38. You are caring for a neonate with Wilms’ tumor. Which of the following statements is ­true? A. Wilms’ tumor is also known as a ­neuroblastoma B. The tumor should never be ­palpated C. The tumor is usually a rough and uneven flank ­mass

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39. You are providing discharge teaching to parents of a term infant following a circumcision with PlastiBell. Discharge instructions should i­ nclude A. That it is normal for the penis to bleed for 2 ­hours B. Pulling the PlastiBell off after 48 ­hours C. Applying petroleum gauze to the penis for 24 ­hours 40. Chlorothiazide was just ordered for your patient. When is the optimal time to schedule ­administration? A. With f­ eedings B. Every ­morning C. Every ­evening 41. Baby N. has congestive heart failure and is not responding to furosemide therapy. The physician orders bumetanide 0.05 g/kg/dose every 6 hours slow IVP. Which electrolyte imbalance would indicate an adverse effect of bumetanide ­administration? A. ­Hypernatremia B. ­Hyperkalemia C. ­Hypochloremia 42. Your patient with bronchopulmonary dysplasia was started on Diuril 2 days ago for pulmonary edema. While drawing a capillary blood gas, you perform a bedside glucose check. The serum glucose is 180 mg/dl. The most likely cause for the hyperglycemia in this patient ­is A. The patient is under ­stress B. Stimulation of the adrenal ­glands C. Suppression of the pancreatic release of ­insulin 43. Which of the following statements is true regarding furosemide (Lasix) ­administration? A. Oral Lasix should be administered with ­food B. Intravenous Lasix should be administered ­rapidly C. Lasix solutions, oral or intravenous, should be protected from ­light 44. You are changing baby Andrew’s diaper and note that urine appears to be leaking from the umbilicus. You notify the physician immediately because you ­suspect A. A ­hernia B. A patent ­urachus C. Extrophy of the ­bladder 45. Posterior urethral valves are diagnosed initially ­via A. X-­ray B. Magnetic resonance ­imaging C. ­Ultrasound

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46. You are completing the admission assessment of a term female infant with anuria. You note a cystic bulge within the labia. You s­ uspect A. Ectopic ­ureters B. An ­ureterocele C. Posterior urethral ­valves 47. You are preparing to administer spironolactone to your patient. The bottle reads 4 mg/ml, it expires in 30 days, and is stored in the patient’s drawer. You ­should A. Administer the medication as ­ordered B. Return the bottle to the pharmacy because the concentration is ­wrong C. Return the bottle to the pharmacy because the solution is ­unstable 48. Aaron was diagnosed as having bilateral cryptorchidism. His parents are concerned about future complications. The parents researched the condition on the Internet and have many comments. Which of the following statements by the parents is true? A. “If the testes do not descend by age 6 months, it is too late for surgical intervention.” B. “Aaron’s risk for testicular cancer is higher than it is for the average population.” C. “Aaron cannot have children.” 49. You are administering indomethacin to your 26-week gestation patient with a large patent ductus arteriosus. You are monitoring urinary output closely ­because A. The nephrotoxic effects of indomethacin are ­permanent B. The indomethacin causes renal vasodilation and sodium ­retention C. The indomethacin inhibits prostaglandin ­production 50. You are caring for a 30-week gestation infant in chronic renal failure awaiting renal transplant. Peritoneal dialysis is being started. Which of the following statements is true regarding nursing management during peritoneal d ­ ialysis? A. Maintain clean gauze dressings around the catheter insertion ­site B. If turbidity is noted, request a complete blood count with differential, Gram stain, and culture ­fluid C. The best position for the infant is flat, supine, or ­prone 51. In preterm infants, water comprises what percentage of total body ­weight? A. 65% B. 80% C. 95% 52. In term infants, water makes up 75% of their total body weight. What factor accounts for the differences in ­water-­to-­total body weight between term and preterm i­ nfants? A. Body m ­ ass B. ­Fat C. Completed development of ­organs

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53. Which hormone is released by the pituitary when a hyperosmolar state is ­present? A. ­Renin B. ­Prostaglandin C. Antidiuretic ­hormone 54. The physician orders high humidity and a d ­ ouble-­wall isolette for a newly born 23-week gestation neonate. This is ­because A. The insulated walls provide a sound ­barrier B. The humidity protects the skin from ­infection C. Fluid regulation is impaired in the extremely ­low-­birth-­weight ­patient 55. You are caring for a new 37-week gestation infant with transient tachypnea of the newborn. The infant’s weight is 3 kg. The doctor orders D10W with 250 mg/dl of calcium gluconate to run at 18 ml/h. You ­should A. Administer as ­ordered B. Contact the physician to clarify the intravenous ­rate C. Contact the physician to clarity the calcium gluconate ­dosage 56. Your 1260-gram patient has an order for intravenous fluids to run at 80 ­ml­/­kg­/­day. Which of the following intravenous rates is ­correct? A. 4.2 ­ml/h B. 5.6 ­ml/h C. 8.4 m ­ l/h 57. The normal specific gravity of urine at 1.002–1.012 reflects the normal value of urine osmolality. Normal urine osmolarity ­is A. 50–100 m ­ Osm/L B. 100–300 m ­ Osm/L C. 200–500 ­mOsm/L 58. You are preparing to admit a 3-­day-­old, term infant from home for dehydration. The infant has been vomiting and has had diarrhea for the past 2 days. Which of the following lab results would support the diagnosis of ­dehydration? A. Metabolic acidosis obtained via capillary blood gas ­analysis B. Urine output of 2.2 ­ml/kg/h C. Low blood urea nitrogen and creatinine ­levels 59. Your patient is to receive volume replacement for dehydration. There are no signs or symptoms of trauma. Which of the following is the most appropriate initial choice for immediate fluid ­replacement? A. Normal saline or lactated ringer’s ­solution B. Packed red blood ­cells C. ­D12.5W

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60. Which of the following statements regarding sodium is ­true? A. Sodium is a major intracellular ­cation B. Hyponatremia results in intravascular ­hypotonicity C. Hypernatremia causes a fluid shift from the extracellular space to the intracellular ­space 61. Your 31-week gestation patient is at 10 days of life. Feedings are by gavage every 3 hours with expressed breastmilk. Cafcit was started PO yesterday for periodic apnea and bradycardia. A glycerin suppository is ordered every 24 hours if the infant has not passed stool. Morning labs indicate hyponatremia. This is mostly likely due ­to A. Breastmilk ­nutrition B. ­Cafcit C. The s­ uppository 62. Baby Jane is a 34-week gestation infant with transient tachypnea of the newborn. Morning labs reveal a serum Na of 120 mEq/L and serum osmolality at 250 mOsm/L. What additional diagnostic testing should be done to differentiate between hemodilution and excessive sodium ­loss? A. Urine ­osmolality B. Renal ­ultrasound C. Sputum ­sodium 63. You are called to the nursery to evaluate a 2-­day-­old, term infant for suspected seizure activity. You note that this term infant has a ­high-­pitched cry, irritability, dry mucus membranes, and sunken fontanels. The mother reports that she is breastfeeding exclusively, but the baby “always seems to be sleepy and hard to wake up.” While obtaining vital signs, you note a period of apnea and seizure activity. You bring the infant to the NICU and notify the physician. Which order should be carried out ­first? A. Draw a complete blood count, chemistry panel, and blood ­glucose B. Obtain a chest X-­ray C. Ensure newborn and hearing screening are ­done 64. Potassium comprises what percentage of the intracellular fluid in a ­neonate? A. 65% B. 78% C. 90% 65. Your 26-week gestation patient’s morning arterial blood gas indicates metabolic alkalosis. What is the expected effect on serum ­potassium? A. Serum potassium will not be ­affected B. Serum potassium will be higher than ­normal C. Serum potassium will be lower than ­normal

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66. You are caring for Baby Flo, a 29-week gestation infant, 4 days post–abdominal surgery for necrotizing enterocolitis. She has a nasogastric tube to low wall intermittent suction and is NPO. You note abdominal distention, absent bowel tones, and hypotonia. Electrocardiogram shows sinus rhythm at 150 bpm, flattened T waves, and ST depression. Before calling the physician, you s­ hould A. Check the electrocardiogram leads and turn up the gain on the ­monitor B. Switch the nasogastric tube to continuous ­suction C. Check the results of the last chemistry ­panel 67. A laboratory technician just called to report a critical value for your patient: a potassium level of 6.9 mEq/L. The chemistry panel was drawn via heel stick. Specimen hemolysis is 13. The patient is undergoing phototherapy. The skin is bruised and a cephalohematoma is resolving. The electrocardiogram shows sinus rhythm at 140 bpm with peaked T waves and a widened QRS. You report the lab result and your assessment to the physician. Which order would you expect to ­receive? A. Draw a chemistry panel in the ­morning B. Redraw a chemistry panel via an arterial line or venous ­stick C. Redraw a chemistry panel now using the opposite ­foot 68. In addition to insulin and glucose infusion ordered for your patient with a potassium level of 8 mEq/L, the physician has ordered Kayexalate. Which of the following labs results indicate a side effect of Kayexalate ­administration? A. ­Hypercalcemia B. ­Hypernatremia C. ­Hypermagnesemia 69. Which of the following hormones directly increases serum ­calcium? A. Parathyroid ­hormone B. ­Calcitonin C. Antidiuretic ­hormone 70. What is the effect of acidosis on free ionized calcium levels in the ­serum? A. There is no relationship between blood pH and free ionized calcium ­levels B. Acidosis results in higher free ionized calcium ­levels C. Acidosis results in lower free ionized calcium ­levels 71. You are admitting a term, ­large-­for-­gestation infant delivered via emergency cesarean section 3 hours ago because of a prolapsed cord and fetal bradycardia. Apgars were 4 at 1 minute and 7 at 5 minutes. The patient’s mother is an ­insulin-­controlled diabetic. The patient has stridor with a ­high-­pitched cry. The infant was fed in the nursery with 10 ml formula for jitteriness and twitching. Bedside blood glucose is 62 and blood was drawn for an electrolyte panel. You would expect which lab result to be ­abnormal? A. ­Sodium B. ­Potassium C. ­Calcium

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72. You are assessing an infant in the nursery for hypotonia, weak suck, poor muscle movement, diminished grasp, and plantar reflexes. The infant chokes during feeding and is irritable. The mother was untreated during pregnancy for hypoparathyroidism. You ­suspect A. The infant is ­hypercalcemic B. The infant is ­hyperkalemic C. The infant is ­hypomagnesemic 73. You are present for the delivery of a 36-week gestation infant born to a mother with severe ­pregnancy-­induced hypertension. The mother is receiving a magnesium infusion at 3 g/h. At delivery, you note that the infant is apneic and floppy. Priority treatment of this infant is t­ o A. Administer Narcan at 0.1 mg/k intramuscular ­immediately B. Intubate ­immediately C. Establish ­ventilation 74. Acid–base regulation in the body is via the lungs and the kidneys. Which of the following statements is true regarding acid–base ­regulation? A. Acids are hydrogen (H1) ­receptors B. HCO32 is a buffer that binds with free hydrogen to form carbonic ­acid C. Respiratory imbalances are determined by assessing HCO32, and metabolic imbalances are determined by assessing ­PCO2 75. You are caring for baby Sarah, a 23-week gestation infant at 5 days of life with respiratory distress syndrome and hypoxic ischemic encephalopathy. The morning weight is 675 g and an arterial blood gas reveals a pH of 7.19, PCO2 44 mmHg, HCO32 13 mEq/L, and base excess of 28. The physician orders sodium bicarbonate 2 mEq/kg to run over 30 minutes. What volume would you administer if the concentration is (4.2%) 0.5 ­mEq/ml? A. 1.35 ­ml B. 2.0 ­ml C. 2.7 ­ml 76. You have just completed the shift change report when you review your patient orders. You note that there is an unsigned order for NaHCO3 4 mEq IV to be given over 30 minutes. You check the medication administration record and cannot find documentation of administration. You ­should A. Administer the medication as ­ordered B. Call the pharmacy to see if the order was received and ­dispensed C. Call the previous shift nurse to verify ­administration

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77. You are orienting a new graduate to intravenous therapy in the neonatal population. Which of the following statements is ­true? A. The intravenous solution amount should be recorded every hour and the site checked every 2 ­hours B. Surgical intervention may be necessary for severe calcium ­burns C. Infiltration of hypertonic solutions to extremities should be treated with hot ­compresses 78. You are caring for 5-­day-­old Adam, a 26-week gestation neonate with feeding intolerance. The umbilical venous catheter is noted to be at 3 cm. You notify the physician about this measurement and anticipate an order t­ o A. Switch the TPN and intralipids to the umbilical artery ­line B. Set up for new umbilical venous line insertion using a 5 Fr ­t ube C. Set up for a peripherally inserted central catheter line ­insertion 79. The physician is preparing to insert umbilical lines. A nursing student assigned to observe procedures in your unit makes several comments to another student. Which of the following statements would you ­clarify? A. “There are usually 2 arteries and 1 vein in the umbilicus.” B. “The venous line has a leg loop on ­X-­ray.” C. “Vasospasms cause ‘Cath Toes.’ ” 80. You are securing the umbilical artery and venous catheter lines that were just placed by the physician and note that the buttocks are blanching. You ­should A. Remove the arterial line ­immediately B. Place warm compresses over the ­groin C. Insert the catheter 1 ­cm 81. The physician has ordered the removal of the peripherally inserted central catheter (PICC) line for your patient on full feeds and oral medications. Which of the following statements is ­true? A. Direct pressure should be held on the site for 30 seconds after removal of the PICC ­line B. The PICC line length should be documented upon ­removal C. The PICC line should be pulled forcefully if any resistance is ­met 82. An expected/normal evaporative water loss for a neonate weighing less than 1250 g i­ s A. 21–30 m ­ l/kg/day B. 40–70 m ­ l/kg/day C. 60–120 ml/kg/day 83. A fluid challenge is ­considered A. Anytime there is decreased urine ­output B. Before an obstruction is ruled ­out C. After an obstruction is ruled ­out

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84. A normal range for serum potassium in the neonate would ­be A. 3.5–4.2 ­mEq/L B. 3.8–4.8 m ­ Eq/L C. 4.5–6.8 m ­ Eq/L 85. An abdominal mass is detected on examination of a 2-­day-­old infant while in the newborn nursery. Which of the following is the most likely cause of this abdominal ­mass? A. ­Hydronephrosis B. Wilms’ ­t umor C. Multicystic ­kidney 86. If a baby is noted to be small for gestational age at birth, which of the following conditions might ­exist? A. ­R h-­positive blood ­group B. Gestational ­diabetes C. Renal ­disease 87. A frequent cause of hypertension in neonates ­is A. Congestive heart ­failure B. ­Left-­to-­r ight ­shunting C. Renal vein ­thrombosis

This concludes the Genitourinary System ­questions.

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Genitourinary System ­Answers 1. Correct answer: ­B In utero, the placenta is responsible for maintaining the majority of fluid regulation, electrolyte balance, and acid–base homeostasis. The fetal kidneys, like the lungs, are not functionally required until birth. As a result, kidney pathologies may not be diagnosed until after ­birth. 2. Correct answer: ­C Fetal urination is most pronounced during the third trimester. During the third trimester, the fetal kidneys are critical to the regulation of amniotic fluid present in the amniotic sac. A reduction in fetal urine will lead to significant oligohydramnios. Postdelivery management should include close monitoring of input and output with renal ultrasound if continued deficiencies are ­noted. 3. Correct answer: ­B The nephron is the functional part of the kidneys that forms urine. The glomerulus and the renal tubule create the nephron. The Bowman’s capsule and the glomerular capillaries are part of the glomerulus. The proximal convoluted tubule, the loop of Henle, and the distal convoluted tubule make up the renal ­t ubule. 4. Correct answer: ­B Nephrogenesis begins during the 2nd month of gestation and completes around the 35th week of gestation. Anatomic kidney development is unaffected by gestation at delivery while functional development may be hindered by premature delivery and ­anoxia. 5. Correct answer: ­A Ultrafiltration by the kidneys is pressure driven. Without sufficient blood pressure, the kidneys are unable to maintain ultrafiltration through the glomerulus. In the premature neonate, a drop in blood pressure results in decreased ultrafiltration. Elevated renal vascular resistance is normal in the fetus as minimal renal filtration is required for amniotic fluid ­production. 6. Correct answer: ­A Blood urea nitrogen and creatinine levels would be elevated during the first 7–10 days of life for neonates because the kidney glomerular filtration rate (GFR) is limited by prematurity and elevated renal vascular resistance. Creatinine may also be elevated due to maternal creatinine crossing the placenta prior to delivery. Nephrogenesis is not complete until around 35-weeks gestation regardless of gestation at delivery. Elevated levels would be expected to decrease over time. Any increases in creatinine levels should be monitored closely as they could reflect significant decrease in GFR and kidney ­impairment. 7. Correct answer: ­B A significant drop in arterial blood pressure may result in the activation of the ­renin-­angiotensin-­aldosterone system (RAS). The RAS is activated when arterial blood pressure falls in the afferent arteriole, dropping glomerular filtration rate. This may

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be due to blood loss, dehydration, infection, and/or stimulation of the sympathetic nervous system. Renin is released by the afferent arterioles and leads to the release of aldosterone. Aldosterone release results in the reabsorption of sodium and water. Additional reabsorption increases extracellular fluid volume and results in increased renal perfusion. Prostaglandin release balances the RAS and leads to vasodilation and diuresis by blocking angiotensin ­II. 8. Correct Answer: ­B Normal urine output in a neonate is 1–3 ml/kg/day. In addition to urine output, serial weights, electrolytes, and physiologic and environmental factors should be used to monitor water balance in ­neonates. 9. Correct answer: ­C Infants lose approximately ­one-­third of their insensible water via the respiratory tract, but ­t wo-­thirds through the skin. In this scenario, phototherapy contributes most to insensible water loss. Additional factors that increase water loss include the patient’s gestational age, skin breakdown, use of radiant warmers, elevated body and environmental temperatures, and congenital skin defects. Use of the ­double-­walled isolette and humidity are also utilized to counter insensible water ­loss. 10. Correct answer: ­A Joey is exhibiting symptoms of syndrome of inappropriate ADH secretion (SIADH). The ­positive-­pressure ventilation, pain, opiate administration, and pneumothorax are contributing factors. Presentation of an infant with SIADH includes rapid weight gain, edema, hemodilution, and elevated urine osmolality. Treatment includes free water restriction, Lasix administration, and Na supplementation if serum Na levels are , 120 mEq/L. Answer B is the treatment for nephrogenic diabetes ­insipidus. 11. Correct answer: ­A An elevated hematocrit decreases the glomerular filtration rate, decreasing urine production. Other factors include decreased blood pressure, renal vasoconstriction, decreased glomerular capillary area, and prematurity of , 34 weeks g ­ estation. 12. Correct answer: ­A The renal response to acidosis is to increase secretion of hydrogen ions in the distal tubule. Reabsorption of bicarbonate takes place in the proximal tubule. Production of ammonia occurs to buffer the acid in the ­blood. 13. Correct answer: ­A Prerenal acute renal failure is best described as a relative hypoperfusion of normal kidneys. Intrinsic acute renal failure occurs with renal cellular damage of any renal structure that compromises renal function. Postrenal acute renal failure is the obstruction of urine flow distal to the ­kidney. 14. Correct answer: ­C This baby is exhibiting signs and symptoms of acute renal failure (ARF) due to delivery complications. A serum creatinine greater than 1.5 mg/dl and rising more than

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0.2 mg/dl/d as well as a blood urea nitrogen of greater than 20 mg/dl and rising more than 10 mg/dl/d would be expected with urine output of less than 1 ml/kg/h. The blood loss and asphyxia contributed to prerenal ARF. Prolonged asphyxia also contributes to intrinsic ARF due to tubular necrosis. Although there was difficulty placing the umbilical venous catheter, no other indication for renal thrombosis is indicated. Further laboratory testing and renal ultrasound are indicated to differentiate between prerenal, intrinsic, and postrenal ­causes. 15. Correct answer: ­B The presence of casts, tubular cells, and proteinuria in urine indicates intrinsic renal failure. Intrinsic renal failure may be caused by congenital anomalies, thromboembolic disease, infection, inflammatory disease, or acute tubular ­necrosis. 16. Correct answer: ­A In acute renal failure, the kidneys are unable to function normally and excretion and reabsorption of electrolytes are impaired. Lab results would indicate hyperkalemia, hyponatremia, hyperphosphatemia, and ­hypocalcemia. 17. Correct answer: ­B Urine output of less than 2 ml/kg/h after furosemide administration indicates intrinsic or postrenal causes of renal failure. Fluid boluses of 10–20 ml/kg are used to differentiate between intrinsic, prerenal, and postrenal causes of renal failure. If the cause for renal failure is prerenal, then response to fluid challenges is a rapid and sustained diuresis that occurs within 1–2 hours after the fluid ­challenge. 18. Correct answer: ­C Acute pyelonephritis can cause acute intrinsic renal failure. Fluid management should focus on fluid restriction to prevent fluid overload. Urine output of 30 ­ml­/­kg/d or more for term infants and up to 70 ml/kg/day plus urinary output for preterm infants is the goal of fluid management. With a fluid restriction, the patient should have a 20–30 g/d weight loss. Once normal kidney function is established, strict fluid monitoring should include daily or twice daily weights. Nutritional support at 100 cal/kg with fat and carbohydrates and 1–2 g/kg/d of protein is necessary. Blood pressure should be closely monitored to provide early indication of congestive heart failure and renal ­damage. 19. Correct answer: ­C Blood pressure is considered high if the blood pressure is higher than the 95th percentile. Factors that may affect blood pressure include cuff size, gestation, body weight, and quality of equipment ­used. 20. Correct answer: ­B Maternal use of cocaine and heroin will cause neonatal blood pressure to be higher than normal and may lead to hypertension. Neonatal exposure to dexamethasone, caffeine, and phenylephrine will also contribute to ­hypertension.

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21. Correct answer: ­C Blood pressure may be elevated due to several factors. The first action should be to check intravenous patency of the fentanyl drip. Vital signs are elevated and may indicate pain related to the chest tube and intubation. Baby Lee also has generalized edema that may mask a peripherally inserted central catheter line infiltration. Treatment of potential causes of hypertension, including pain, hypervolemia, and titrating of inotropic medications, should be initiated prior to starting antihypertensive medications. Manual cuff size should be ­t wo-­thirds the extremity diameter. Since the baby is now 30 days old, the cuff size should be increased for growth. Moving the cuff site does not identify probable causes for the hypertension and may delay appropriate ­treatment. 22. Correct answer: ­A Term infants are considered hypertensive if the blood pressure is greater than 90/60. Preterm infants are considered hypertensive if the blood pressure is greater than ­80/50. 23. Correct answer: ­C You should question the order for enalapril 0.1 mg/kg/day PO in two divided doses. The correct dosage should be mg/kg/dose not per day. Normal dosing for enalapril is ­0.1–0.3 mg/kg/dose intravenously every 12–24 hours. Hydralazine is ordered ­0.10–0.5 mg/kg/dose IV bolus every 8–10 hours. Propranolol is ordered ­ ­0.025–­1 ­mg/kg/dose IV ­BID. 24. Correct answer: ­A Oligohydramnios syndrome is also known as Potter’s syndrome. There is a higher prevalence in male infants than female infants. Approximately 40% of births are stillborn and live births result in death within hours to days after ­delivery. 25. Correct answer: ­B A baby with Potter’s syndrome is usually small for gestation, has bowed legs and clubbed feet, severe respiratory distress, a ­bell-­shaped chest, large ­claw-­shaped hands, and dry skin. Assessment criteria that would support a diagnosis of Potter’s syndrome include a blunted nose with receding chin, low-set ears, and widely spaced eyes. Other criteria include renal agenesis on renal ultrasound, anuria, and pulmonary hypoplasty. Genital abnormalities and/or gastrointestinal malformations may also be ­noted. 26. Correct answer: ­C Prognosis for patients with Potter’s syndrome is very poor. Nursing actions should focus on palliative care and assisting parents in the grieving process. Infant baptism, parent counseling, grief support, and encouraging parent discussion are all important in assisting parents in the grief process. Patient positioning should be as comfortable as possible and diminish respiratory ­distress. 27. Correct answer: ­C Bilateral multicystic dysplastic kidney disease is not compatible with life because the kidneys are not functional. Unilateral multicystic dysplastic kidney disease is more common and is the most frequently palpated abdominal ­mass.

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28. Correct answer: ­A Autosomal recessive polycystic kidney disease (ARPKD) cysts are large and look like a “snowstorm” on ultrasound, while autosomal dominant polycystic kidney disease (ADPKD) cysts are variable in size. ARPKD is less common than ADPKD, with ADPKD usually presenting after the neonatal period. Liver cysts are found with both diseases, but ADPKD also has cysts on the pancreas and the spleen. Hypertension is a common complication of both ARPKD and ADPKD and should be treated ­aggressively. 29. Correct answer: ­A Hydronephrosis is the dilation of the pelvis and calyces of one or both kidneys due to obstruction. Obstruction of urine from the kidney causes retrograde flow into the pelvis resulting in dilation. This is the most common renal abnormality found during prenatal ultrasound, and the cause is not always known. Patients present with poor urinary output, a possible abdominal mass, and urinary tract ­infection. 30. Correct answer: ­C Prune-­Belly syndrome or ­Eagle-­Barrett syndrome has a triad of assessment criteria. These criteria include absence or hypoplasia of the abdominal wall, bilateral cryptorchidism, and urinary tract abnormalities on ultrasound. Visual inspection of the abdomen reveals a shriveled appearance from lack of abdominal muscular structure and poor urine output. Ultrasound also reveals hydronephrosis. Responses for A and B utilize medical terminology and not more simple terms the family can ­understand. 31. Correct answer: ­C The need for corrective surgery is dependent upon the cause and severity of the hydronephrosis. This patient has unilateral hydronephrosis with moderately low urine output. Further testing and time will provide support for or against surgery. The need for surgery is dependent upon the level of kidney function, presence of an obstruction, whether hypertension is present, and/or whether the hydronephrosis ­worsens. 32. Correct answer: ­A Clinical presentation for this patient is consistent with renal vein thrombosis. This includes right flank mass, hematuria, hypertension, anemia, and thrombocytopenia. Management includes review of the history and physical for maternal diabetes, fetal asphyxia, maternal thiazide therapy, polycythemia, sepsis, RDS, or congenital heart disease. Treatment is focused on correcting the underlying illness, fluid therapy, possible dialysis, and close continuous ­monitoring. 33. Correct answer: ­B Bladder exstrophy occurs during the 6th and 8th weeks of fetal development. The infant may also have anomalies of the lower abdominal wall, anterior bony pelvis, and external genitalia. Bladder exstrophy occurs due to failure of the abdominal wall to close during fetal development and results in protrusion of the posterior bladder wall through the lower abdominal ­wall. Surgical intervention should occur within the first 48 hours of life to improve ­long-­term urine continence. Epispadias is commonly present with exstrophy and should be surgically corrected at the same time. Upper urinary tract anomalies are not common with this ­population.

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34. Correct answer: ­C The genitalia of this patient may initially indicate an undervirilized male, but they could also indicate a female. It is important to send chromosomes for genetic testing to determine sex. Pelvic ultrasound, genitourethrogram, and magnetic resonance imaging are also useful in determining urinary structures, gonadal structures, and diagnosing additional congenital ­anomalies. 35. Correct answer: ­C Urinary tract infections should be treated with antibiotics for 14 days. During anti­ biotic therapy, the patient should be tested for urinary reflux. After antibiotics have been discontinued, the patient’s urine should be cultured. Results typically take 3 days to return, so it is best to continue the antibiotics while awaiting results of the ­culture. 36. Correct answer: ­B Transillumination provides rapid assessment to differentiate fluid in the scrotal sac from an inguinal hernia. Aspiration of fluid may be done to relieve fluid pressure once an inguinal hernia has been ruled out. Immediate surgery is required if the inguinal hernia presents with a palpable mass that does not reduce, or vomiting, abdominal distention, and ­irritability. 37. Correct answer: ­C Patients who have undergone surgery to correct testicular torsion should be positioned supine or ­side-­lying only to prevent excessive pressure to the abdominal and scrotal areas. Girth should be measured every 4 hours to monitor for distention. A nasogastric tube may be used if the patient is vomiting. Intermittent ­low-­wall suctioning should be used and the tube irrigated every 2–4 hours to ensure ­patency. 38. Correct answer: ­B It is essential that a Wilms’ tumor never be palpated, as this may cause the tumor to seed to other areas of the body. Wilms’ tumor is also referred to as a nephroblastoma and is usually a round abdominal or flank mass. Pyelograms and renal ultrasound are used to differentiate diagnoses while ­X-­rays are used to determine if the tumor seeds to other ­areas. 39. Correct answer: ­C Discharge teaching about circumcision should include the application of petroleum gauze to the penis for 24 hours and petroleum to the penis until healed. It is necessary to leave the PlastiBell in place until falls off on its own. The parents should be taught to alert the physician if the baby has not voided within 2–4 hours, if bleeding, pus, or redness is noted, if the baby develops a temperature, or the penis tip becomes blackened. In addition, diapers should be changed every 2–3 hours or when ­wet. 40. Correct answer: ­A Chlorothiazide is administered twice daily and is best administered with feedings to increase absorption. Normal dosing is 10 to 20 mg/kg per dose PO every 12 hours. Take caution when transcribing so as not to confuse this medication with ­hydrochlorothiazide.

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41. Correct answer: ­C Adverse effects of bumetanide include hypochloremic alkalosis, hyponatremia, and hypokalemia. Bumetanide works by inhibiting chloride reabsorption and tubular sodium transport resulting in hyponatremia and hypochloremia. In addition, there is urinary loss of potassium, calcium, and ­bicarbonate. 42. Correct answer: ­C Diuril inhibits pancreatic insulin release resulting in hyperglycemia. Bedside glucose monitoring should be done at least daily to monitor for hyperglycemia. Additional electrolyte monitoring should include sodium, potassium, magnesium, chloride, bicarbonate, and phosphorous due to increased urinary ­loss. 43. Correct answer: ­C Lasix solutions should be protected from light because light will cause degradation. Oral Lasix should be administered on an empty stomach to improve absorption and intraveous Lasix should be given slowly to prevent muscle ­spasms. 44. Correct answer: ­B Patent urachus presents with urine leaking from the umbilicus. This is due to a communication between the umbilicus and the bladder when the urachal tube fails to close. The incidence of patent urachus is more common in males than in females. Common complications include urinary tract infections, excoriation, and infection. The duct may close spontaneously or require surgical ­intervention. 45. Correct Answer: ­C Diagnosis of posterior urethral valves (PUV) begins first with ultrasound of the bladder and kidneys. Since the ureters carry urine from the kidneys to the bladder, posterior positioning of the ureters results in functional obstruction and will lead to hydronephrosis, dilation of the ureters, and bladder hypertrophy. Final diagnosis is made with fluoroscopic voiding cystourethrogram (VCUG) and will indicate the structural placement, severity, and functional impairment. PUV is more common in males than in females. If PUV began early in development, then dysplastic changes may be seen with severe renal ­damage. 46. Correct answer: ­B A cystic bulge on the labia is most commonly associated with an ureterocele that prolapses into the urethra. The ureterocele leads to obstruction, resulting in impaired urine output and ­hydronephrosis. 47. Correct answer: ­C Spironolactone should be prepared as a 4 mg/ml solution that will expire within 30 days or less and must be kept in the refrigerator. The medication is not stable if it is kept at room ­temperature. 48. Correct answer: ­B Bilateral cryptorchidism places the patient at higher risk than the average population. Although testicular cancer is rare, early observation and detection is critical. Surgical intervention is necessary if his testes have not descended by age 6 months, and his ability to have children decreases as he ages if his condition is not ­corrected.

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49. Correct answer: ­C Indomethacin administration results in transient nephrotoxic effects that lead to poor urinary output, hyponatremia, and elevated creatinine levels. These toxic effects lead to renal vasoconstriction and inhibit local prostaglandin ­production. 50. Correct answer: ­B If the return fluid is noted to be turbid (cloudy with sediment), then the nurse should inform the physician and request orders for complete blood count with differential and to send the fluid for Gram stain and culture. Peritonitis is a common and potentially deadly complication of peritoneal dialysis. If infection is suspected, prepare to administer antibiotics. Catheter sites should be sterile with an occlusive dressing. To minimize respiratory distress from increased abdominal pressure on the diaphragm, position the patient supine or ­side-­lying with the head of the bed elevated. You must also ensure the tubing is kink free. Accurate monitoring of dialysate flow provides early detection of fluid absorption, dehydration, and potential obstructions in the ­system. 51. Correct answer: ­C Water makes up 95% of total body weight in early gestation infants. The majority of this water is in the extracellular space. This is important when monitoring for fluid shifts between the intracellular spaces and the intravascular and interstitial ­spaces. 52. Correct answer: ­B Body fat accounts for the differences in ­water-­to-­total body weight between the term and preterm infant. Organ development has no bearing on water concentration. By term, more water has shifted into the intracellular space as organs have reached greater developmental milestones than they have in the preterm ­infant. 53. Correct answer: ­C Antidiuretic hormone (also known as ADH or vasopressin) is secreted by the pituitary gland when hyperosmolality and/or hypotension occur. Renin and prostaglandin are secreted by the kidneys. ADH results in water reabsorption by making distal tubules permeable to ­water. 54. Correct answer: ­C High humidity and the ­double-­wall isolette aid in fluid regulation of the extremely ­low-­birth-­weight patient by minimizing transepidermal water loss (TEWL). Due to poor or underdeveloped dermal and epidermal layers in the skin, TEWL increases as gestational age decreases. Water loss can be attributed to poor keratinization, high water content, limited subcutaneous fat, large surface area, and skin vascularity of the premature neonate. TEWL is closely related to ambient humidity until a fully functional stratum corneum can develop, which may take several weeks. Failure to calculate and replace TEWL fluid losses will lead to dehydration and hypotension. Although the ­double-­wall isolette does inhibit some sound, it is not the primary reason for use in this patient. The double wall assists in maintaining humidity and thermoregulation. High humidity places the infant at risk for infection, and reverse isolation techniques should be ­utilized.

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55. Correct answer: ­B Normal first day hydration is calculated at 60­–100 ml/kg/day and adjusted for the patient’s condition and gestation. If administered, this patient would be receiving 144 ml/kg/day (18 ml/h 3 24 h 4 3 kg). The initial fluid rate for a new infant should be sufficient to permit ongoing physiology and adapt to fluid and electrolyte changes, but still prevent overhydration. The calcium order is appropriate and could range from 200–300 mg/dl added to base ­fluid. 56. Correct answer: ­A The correct intravenous rate for a patient weighing 1260 grams at 80 ml/kg/d is 4.2 ml/h. This is calculated by changing the grams to kilograms (1260 4 1000 5 1.260) and multiplying by 80 ml/kg and dividing the total by 24 h/d. Thus, 1.260 3 80 4 24 5 4.2 ­ml/h. 57. Correct answer: ­B Normal specific gravity of urine should reflect a normal value of urine osmolality at 100–300 mOsm/L. This value is accurate if the patient does not have blood, glucose, and/or protein in the ­urine. 58. Correct answer: ­A A capillary blood gas analysis will indicate metabolic acidosis when dehydration is present. Urine output should be , 1 ml/kg/h and blood urea nitrogen and creatinine should be normal or ­elevated. 59. Correct answer: ­A Normal saline and lactated ringers are the most appropriate choice for fluid replacement in a dehydrated infant. Both solutions are isotonic crystalloids. As there is no indication of blood loss, packed red blood cells should not be used as the patient may become polycythemic. Although a patient may be hypoglycemic on admission related to poor intake or excessive diarrhea or vomiting, glucose solutions should not be used for fluid replacement, but for fluid ­maintenance. 60. Correct answer: ­B Hyponatremia results in intravascular hypotonicity and fluid shifts into the cells, causing cellular edema and intravascular dehydration. Sodium is a major extracellular cation and movement across the cellular membrane is via the sodium/potassium pump. Hypernatremia causes intravascular hypertonicity and a fluid shift into the extracellular space, causing cellular dehydration and intravascular ­overload. 61. Correct answer: ­A If an infant is rapidly growing, the content of sodium in breastmilk may not be sufficient and may lead to delayed onset of hyponatremia. Most often this occurs during the first week of life. Although Cafcit may lead to sodium loss due to diuretic effects, it is unlikely in this case because this patient was just started on this ­medication.

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62. Correct answer: ­A To differentiate between hemodilution and excessive sodium loss, a urine osmolality and sodium level should be done. Renal ultrasound may be used to clarify renal structural development. Sputum sodium testing is used to help diagnose cystic ­fibrosis. 63. Correct answer: ­A Upon admission it is important to obtain a complete blood count, chemistry panel, and blood glucose immediately to rule out fluid and electrolyte imbalances. A ­high-­pitched cry, irritability, lethargy, and seizure activity may indicate hypernatremia. If the mother is exclusively breastfeeding, milk production may be insufficient. Hypocalcemia and hypoglycemia both may cause seizures if not managed rapidly. Lab draws may be completed while awaiting radiology staff to perform the chest X-­ray. 64. Correct answer: ­C Potassium is the major cation in cells and comprises 90% of the intracellular fluid. Excretion via the kidneys is based on glomerular filtration rate, urine flow, and aldosterone ­sensitivity. 65. Correct answer: ­C If the pH is high, potassium is driven into the cell, causing serum potassium levels to drop. Insulin will also force potassium back into the cell by stimulating the sodium/ potassium ­pump. 66. Correct answer: ­C Baby Flo is exhibiting signs and symptoms of hypokalemia (abdominal distention, ileus, hypotonia, flattened T waves, and ST depression). Abdominal surgery with nasogastric suctioning places the infant at high risk for potassium depletion. The physician will need to know the last potassium level prior to ordering potassium ­replacement. 67. Correct answer: ­B The elevated potassium level may be explained by the lab draw via heel stick, hemolysis, and cellular injury related to the bruising and resolving cephalohematoma, but the patient is also exhibiting signs of hyperkalemia: peaked T waves and widened QRS. To obtain a more accurate potassium level, the blood for the chemistry lab should be drawn via venous stick or arterial line (without TPN infusion) if available. If TPN is infusing via an umbilical arterial catheter, make sure to draw a sufficient waste amount to clear the line to obtain an accurate ­specimen. 68. Correct answer: ­B Administration of sodium polystyrene sulfonate (Kayexalate) may cause hypocalcemia, hypernatremia, and hypomagnesemia. For every mEq of potassium absorbed, the Kayexalate releases 1 mEq of sodium into the patient’s system. If the Kayexalate and possible insulin/glucose infusion are not sufficient to decrease serum potassium, then the physician may perform an exchange transfusion with fresh blood that is , 3 days ­old.

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69. Correct answer: ­A Parathyroid hormone (PTH) increases serum calcium by increasing the breakdown of bone, increasing intestinal absorption, and inhibiting renal excretion. Calcitonin is secreted by the thyroid C cells to lower serum calcium by counteracting PTH. Anti­ diuretic hormone controls water excretion and retention in the kidneys and may indirectly impact calcium through hemoconcentration or ­hemodilution. 70. Correct answer: ­B Acidosis results in an increase of free ionized calcium in the blood. Acidosis inhibits calcium’s binding ability with albumin, therefore increasing serum ­calcium. 71. Correct answer: ­C Multiple factors contributed to hypocalcemia in this patient. Maternal diabetes may result in delayed neonatal parathyroid production. The cord prolapse may have inhibited oxygen delivery to the fetus, resulting in possible perinatal asphyxia and stress. Asphyxia results in calcitonin release, which prohibits parathyroid action. Tissue damage releases phosphorous into the blood, which further inhibits calcium uptake. Patient assessment criteria that support hypocalcemia are stridor with ­high-­pitched cry, jitteriness and twitching not related to hypoglycemia, and possible seizure ­activity. 72. Correct answer: ­A Untreated maternal hypoparathyroidism may result in overproduction of parathyroid hormone in the neonate, resulting in hypercalcemia. History and clinical presentation are consistent with hypercalcemia. Hypomagnesemia presents with hyperreflexia and irritability. Hyperkalemia would present with cardiac ­irritability. 73. Correct answer: ­C The best treatment is to establish ventilation via stimulation or ­positive-­pressure ventilation to restore normal heart rate and color. Hypermagnesemia causes smooth muscle relaxation, resulting in respiratory, neuromuscular, and gastrointestinal depression. The patient may require intubation and temporary mechanical ventilation support until the kidneys are able to excrete sufficient quantities of urine. Meconium presence in a patient who is not vigorous at birth is managed with immediate intubation and tracheal suctioning. There is no history of narcotic administration that may be linked to fetal respiratory depression. Narcan administration would be ­pointless. 74. Correct answer: ­B HCO32 is a buffer that binds with free hydrogen to form carbonic acid. Carbonic acid then breaks down into water and CO2 for removal via the lungs. Additional buffers include plasma proteins and hemoglobin. Acids are hydrogen donors and bases are hydrogen receptors. When evaluating the primary cause of acid–base imbalances, respiratory imbalances are determined by evaluating PCO2 , and metabolic imbalances are determined by evaluating HCO32. 75. Correct answer: ­C The correct calculation of sodium bicarbonate for this patient ­is: 2 mEq/kg 3 0.675 kg 4 0.5 mEq/ml 5 2.7 ­ml

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76. Correct answer: ­C Because no documentation is available, the first step is to call the previous nurse to determine if the drug was administered. Many units may have access to certain medications that bypass pharmacy checks. If computer charting is used to document administration, the system may have been down or not available during administration time. A charge nurse may not be specifically aware of administration, but may provide assistance in locating the nurse or calling pharmacy. Due to the high risk for metabolic alkalosis related to ­over-­administration of NaHCO3, it is best to wait until the administration can be verified prior to a second administration. If the nurse cannot be found, contact the physician. A repeat blood gas may be used to determine if NaHCO3 is still ­needed. 77. Correct answer: ­B Infiltration of hypertonic solutions containing calcium may cause severe sloughing and extravasations leading to tissue necrosis. Initial treatment is aimed at restoring the skin barrier and preventing local and systemic infection. Depending upon the location of the infiltration and the severity, ­long-­term management may require cosmetic or reconstructive surgery to improve patient outcome. The best and most effective treatment for infiltration is prevention and early treatment. For this reason, intravenous fluid amounts and site appearance should be observed and documented at least once per hour. Compresses, cold or warm/hot, should be avoided as they may cause additional tissue damage. The physician should be notified prior to starting any infiltration ­therapy. 78. Correct answer: ­C The nurse would expect the physician to order a peripherally inserted central catheter (PICC) line to replace the umbilical venous catheter. This line provides central access for fluids and medications while still permitting free movement of the extremity. PICC lines also limit the excessive number of peripheral intravenous lines required when caring for premature neonates needing ­long-­term intravenous therapy. Although TPN may be used in umbilical arterial lines, it is not the preferred route, and intralipids should not be administered via UAC at all. The patient is only 26.5 weeks gestation, so a 5 Fr line is not recommended. A 3.5 Fr would be more ­appropriate. 79. Correct answer: ­B Arterial lines, not venous lines, have a leg loop on ­X-­ray where the catheter follows the vessel toward the legs before arching superiorly toward the aorta. The correct tip location is either below the renal arteries and above the aortic bifurcation (L3–L4) or above the diaphragm and below the left subclavian artery (T7–T9). Vasospasms will cause “Cath Toes” or catheter toes and do not require removal of the line. Treatment with warm compresses or nitroglycerin paste will cause vasodilatation. Normally there are two arteries and one vein located at 4 o’clock, 8 o’clock, and 12 o’clock respectively. If one artery is present, further evaluation should be conducted to identify potential genetic ­anomalies. 80. Correct answer: ­A Although the physician must be notified immediately, the catheter should be removed. An embolus or severe arterial spasm has inhibited blood flow to the affected area. Without immediate correction, tissue necrosis may result. If emboli formation is suspected,

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antithrombolytic therapy may be considered. Warm compresses are indicated for bluish coloration to the toes and represent vasospasm. The site is no longer sterile and the catheter should not be advanced due to potential introduction of bacteria into ­circulation. 81. Correct answer: ­B The length of the removed PICC line should be compared to the length reported on insertion. Any discrepancy should be reported to the physician immediately and represents a surgical emergency. Any remaining catheter within the vessel could cause infarction and tissue necrosis by blocking blood flow. During removal, if the PICC line does not move easily or any resistance is met, do not pull forcefully as this may tear the catheter or vessel. Stop the removal immediately and notify the physician. It is possible for lines to spontaneously knot or kink in vessels. An ­X-­ray will confirm placement and identify obstructions to removal. Once the PICC line is removed, the nurse should apply 5 minutes of direct pressure to the site to stop bleeding and prevent ­hematoma. 82. Correct answer: ­C Expected or normal evaporative water loss for a neonate weighing less than 1250 g is 60–120 ml/kg/day. Volume replacement and humidity are used to counter the rapid water loss and pursuing electrolyte ­imbalances. 83. Correct answer: ­C A fluid challenge is used when oliguria is present after an obstruction is ruled out. If oliguria is present due to an obstruction, the additional fluid with the challenge may place additional stress on the kidneys, leading to worsening hydronephrosis. Renal ultrasound may be used to determine structural causes of ­oliguria. 84. Correct answer: ­C A normal range for serum potassium in the neonate would be 4.5–6.8 ­mEq/L. 85. Correct answer: ­A The most common cause of an abdominal mass in the newborn is hydronephrosis. If it is unilateral, urine output may not be significantly reduced and the identification of the abdominal mass may be the first indicator of ­disease. 86. Correct answer: ­C Small for gestation has a multitude of potential causes. If renal deficiency is noted also, then the infant should be assessed for renal ­disease. 87. Correct answer: ­C Renal vein thrombosis is a frequent cause of hypertension in neonates due to feedback loops indicating false ­hypovolemia.

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Genitourinary System ­References Anonymous. (2007). Fetal kidney volume and its association with growth and blood flow in fetal life: The Generation R study. Kidney International, 72(8), ­1034. Boutros, M., Vicanek, C., Rozen, R., & Goodyer, P. (2005). Transient neonatal cystinuria. Kidney International, 67(2), 443–448. Brodsky, D., & Martin, C. (2003). Neonatology review. Philadelphia, PA: Hanley & ­Belfus. Chen, C. P., Wang, W., & Lin, S. P. (1998). Perinatal findings in a male fetus with congenital megacystis and anorectal malformations. Fetal Diagnosis and Therapy, 13(6), 348–351. Cloherty, J. P., Eichenwald, E. C., & Stark, A. R. (2004). Manual of neonatal care (5th ed.). Philadelphia, PA: ­Lippincott. Gloor, J. M., Breckle, R. J., Gehrking, W. C., et al. (1997). Fetal renal growth evaluated by prenatal ultrasound examination. Mayo Clinic Proceedings, 72(2), 124–129. Halliday, H. L., McClure, B. G., & Reid, M. (2002). Handbook of neonatal intensive care (4th ed.). Philadelphia, PA: ­Saunders. Hay, W. W., Levin, M. J., et al. (2007). Current diagnosis and treatment in pediatrics (18th ed.). New York, NY: McGraw-­Hill. Hosseini, S., Gooran, S., Alizadeh, F., & Dadgari, S. (2009). Polycystic horseshoe kidney anomaly. Applied Radiology, 38(9), 38–39. Jain, S., Suarez, A. A., McGuire, J., & Liapis, H. (2007). Expression profiles of congenital renal dysplasia reveal new insights into renal development and disease. Pediatric Nephrology, 22(7), 962–974. Jones, K. L. (2006). Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: ­Elsevier. Karlsen, K. (2006). The S.T.A.B.L.E. program (5th ed.). Park City, UT: American Academy of ­Pediatrics. Karlsen, K., & Tani, L. Y. (2003). S.T.A.B.L.E.—Cardiac module. Park City, UT: American Academy of ­Pediatrics. Kattwinkle, J. (Ed.). (2006). Neonatal resuscitation textbook (5th ed.). Elk Grove Village, IL: American Academy of Pediatrics and American Heart ­A ssociation. Kenner, C., Amlung, S., & Flandermeyr, A. (1998). Protocols in neonatal nursing. Philadelphia, PA: ­Saunders. Kenner, C., & Lott, J. (2004). Neonatal nursing handbook. St. Louis, MO: E ­ lsevier. Kenner, C., & Lott, J. (2007). Comprehensive neonatal care (4th ed.). St. Louis, MO: ­Elsevier. Kenner, C., & McGrath, J. M. (Ed.). (2004). Developmental care of newborns & infants: A guide for health professionals. St. Louis, MO: M ­ osby. MacDonald, M. G., & Ramasethu, J. (2007). Atlas of procedures in neonatology (4th ed.). Philadelphia, PA: Lippincott ­Williams. Markiewicz, M., & Abrahamson, E. (1999). Diagnosis in color: Neonatology. Philadelphia, PA: ­Mosby. Merenstein, G. B., & Gardner, S. L. (2006). Handbook of neonatal intensive care (6th ed.). St. Louis, MO: ­Mosby. Mitra, S. C., Seshan, S. V., Salcedo, J. R., & Gil, J. (2000). Maternal cocaine abuse and fetal renal arteries: A morphometric study. Pediatric Nephrology, 14(4), 315–318. Morris, S., Akima, S., Dahlstrom, J. E. et al. (2004). Renal tubular dysgenesis and neonatal hemochromatosis without pulmonary hypoplasia. Pediatric Nephrology, 19(3), 341–344. Patrakka, J., Martin, P., Salonen, R., et al. (2002). Proteinuria and prenatal diagnosis of congenital nephrosis in fetal carriers of nephron gene mutations. The Lancet, 359(9317), 1575–1577. Persutte, W. H., & Lenke, R. R. (1995). Failure of a ­ mniotic-­fluid-­cell growth: Is it related to fetal aneuploidy? The Lancet, 345(8942), 96–97.

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Genitourinary ­System  y  211 Polin, R. A., & Spritzer, A. R. (2007). Fetal & neonatal secrets (2nd ed.). Philadelphia, PA: ­Mosby. Prenatal hydronephrosis: Researchers from McGill University report recent findings in prenatal hydronephrosis. (2009, August). Gastroenterology Week, ­345. Prescribing Reference. (2009, Summer). NPPR: Nurse practitioner’s prescribing reference, 16(2). Quintero, R. A., Homsy, Y., Bornick, P. W. et al. (2001). ­In-­utero treatment of fetal ­bladder-­outlet obstruction by a ureterocele. The Lancet, 357(9272), 1947–1948. Rennie, J. M., & Roberton, N. R. C. (2002). A manual of neonatal intensive care (4th ed.). New York, NY: A ­ rnold. Taeusch, H. W., Ballard, R. A., & Gleason, C. A. (2005). Avery’s diseases of the newborn (8th ed.). Philadelphia, PA: ­Elsevier. Tiniakos, D., Anagnostou, V., Stavrakis, S., et al. (2004). Ontogeny of intrinsic innervation in the human kidney. Anatomy and Embryology, 209(1), 41–47. Vanpée, M., Herin, P., Lagercrantz, H., & Aperia, A. (1997). Effect of extreme prematurity on renal dopamine and norepinephrine excretion during the neonatal period. Pediatric Nephrology, 11(1), 46–48. Verklan, M. T., & Walden, M. (Eds.). (2004). Certification and core review for neonatal intensive care nursing (3rd ed.). St. Louis, MO: E ­ lsevier. Verklan, M. T., & Walden, M. (Eds.). (2010). Certification and core review for neonatal intensive care nursing (4th ed.). St. Louis, MO: ­Elsevier. Young, T. E., & Magnum, B. (2009). Neofax 2009. Montvale, NJ: Thomson ­Reuters.

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Section 8 Hematology/Immunology

Questions 1. The most common cause of DIC ­is A. ­Carcinoma B. ­Surgery C. ­Sepsis 2. Baby C. is a term neonate who has been increasingly restless and now has petechiae on the trunk, arms, and legs. The abdomen is slightly distended and the baby demonstrates increased respiratory effort. She is now obtunded. During your assessment, you note ­pink-­tinged urine in the diaper, generalized ecchymosis, and oozing from the umbilical line insertion point. You suspect baby C. may ­have A. ­DIC B. Pulmonary ­emboli C. A fat ­embolism 3. As a critical care nurse, you know that baby C.’s abrupt onset of DIC may be due to a previously undiagnosed cause. Some of these causes could ­include A. Viral ­infections B. ­Gram-­negative i­ nfections C. ­Trauma 4. In the NICU, neonates with DIC are at a high risk of ­developing A. Deficiencies in vitamin K and ­folate B. Increased fibrinogen ­levels C. A decreased ­D -­dimer (less than 300 mcg/l) 5. Factor VIII deficiency is also known ­as A. Von Willebrand’s ­disease B. Sickle cell ­anemia C. Aplastic ­anemia 6. The blood component that contains factor VIII ­is A. ­PRBCs B. ­Cryoprecipitate C. Salt poor ­albumin

y

y

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7. Following ­heart-­lung transplants, prostaglandin (PGE1 ) is used ­to A. Provide inotropic ­support B. Augment the heart r­ ate C. Promote pulmonary ­vasodilation 8. Hemoglobin is phagocytized primarily in ­the A. ­Liver B. ­Lungs C. ­Kidneys 9. Your patient is a term infant who has been diagnosed with thalassemia and is about to be discharged home. An important aspect of parent teaching for a neonate with thalassemia would ­include A. Using cool water for skin ­cleansing B. Avoiding oral stimulants like ­pacifiers C. Feeding fewer, larger, ­high-­protein ­meals 10. The synthesis of hemoglobin is regulated ­by A. ­T hrombopoietin B. A myeloid stem ­cell C. ­Erythropoietin 11. At birth, what percentage of fetal hemoglobin (HbF) is contained in red blood ­cells? A. 20–30% B. 70–90% C. 40–60% 12. When does hemoglobin synthesis begin during embryonic ­life? A. At approximately 14 days of embryonic ­life B. At 2 months of embryonic ­life C. At 45 days of embryonic ­life 13. During fetal life, erythropoietin is produced primarily by ­the A. ­Kidneys B. ­Spleen C. Submandibular ­glands 14. Baby M. is a 34.5-week gestation female infant in the 4th day of life. Today her total serum bilirubin (TSB) is 24 mg/dl. She is placed under blue fluorescent phototherapy ­bulbs. As a NICU nurse, you know this infant must be closely monitored ­for A. ­Diarrhea B. ­Burns C. ­Dehydration

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15. Your patient is a 35-week gestation infant who has developed respiratory distress and sepsis. The infant is being mechanically ventilated and has had a varying pH (from 7.22 to 7.30) and a base deficit that has also been fluctuating from 25 to 26 over the last 10 hours. The infant has an indirect bilirubin of 22.9. This infant will be receiving a d ­ ouble-­volume exchange transfusion. Which of this infant’s problems must be addressed prior to the start of the ­transfusion? A. The bilirubin ­level B. The a ­ cid-­base ­imbalance C. Extubate the ­infant 16. There are four types of bilirubin. The type of bilirubin found in cord blood ­is A. Unconjugated b ­ ilirubin B. Free b ­ ilirubin C. Conjugated ­bilirubin 17. If albumin binding sites are filled, increased amounts of free bilirubin are passed into the central nervous system and may result ­in A. Cerebral ­hemorrhage B. ­PPHN C. ­Kernicterus 18. Infants with kernicterus will have certain brain cells that stain a bright yellow color. The areas of the brain most readily affected by this staining ­are A. The cortex, pons, and temporal ­regions B. The cerebellum, dentate nucleus, and ­hypothalamus C. Broca’s area, the pontine area, and the ophthalmic ­tract 19. Hemolysis in the fetus caused by Rh incompatibility results in which of the following d ­ iseases? A. ABO i­ ncompatibility B. ­Polycythemia C. Erythroblastosis ­fetalis 20. Hydrops fetalis is characterized ­by A. Generalized ­edema B. Gonadal ­agenesis C. ­Blindness 21. Hemorrhage in an infant with hydrops fetalis is usually the result ­of A. Altered hepatic ­synthesis B. ­Hyperglycemia C. ­Hypoxia 22. All the clotting factors in blood are synthesized in the liver ­except A. VIII and ­XII B. V and I­ X C. IX and I­ II

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23. Cellular humoral immunity is mediated ­by A. T ­lymphocytes B. ­Eosinophils C. B l­ymphocytes 24. Which of the following types of cell would be considered a nongranular ­leukocyte? A. ­Eosinophil B. ­Monocyte C. ­Basophil 25. The most prevalent type of anemia ­is A. Chronic ­anemia B. ­Iron-­deficiency ­anemia C. Pernicious ­anemia 26. What is the most common cause of a fatal transfusion ­reaction? A. ­Immune-­compromised ­recipient B. Mismatched ­blood C. Volume ­overload 27. Rejection of a transplanted organ usually occurs as a result o ­f A. Cellular ­immunity B. Humoral ­immunity C. Delayed hypersensitivity ­reaction 28. A transfusion reaction that usually occurs within 5–30 minutes of the start of the transfusion is known ­as A. An acute intravascular hemolytic ­reaction B. An extravascular transfusion ­reaction C. An allergic transfusion ­reaction 29. Pernicious anemia results from a lack ­of A. Vitamin ­B 6 B. Vitamin ­A C. Vitamin ­B12 30. A function of a red blood cell would ­be A. Carbonic acid ­dissociation B. To function as a ­macrophage C. To initiate ­hemostasis 31. The physician has ordered an MCH test for your patient. This test ­evaluates A. The average concentration of hemoglobin per single ­RBC B. The average amount (by weight) of hemoglobin in each ­RBC C. The average size and volume of a single ­RBC

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32. The normal range for platelets in the term and premature infant i­ s A. 100,000 to 250,000/mm3 B. 200,000 to 350,000/mm3 C. 150,000 to 400,000/mm3 33. Transient deficiencies in newborn clotting mechanisms ­include A. Hyperactivity of ­enzymes B. Excess clotting factors at ­birth C. A deficiency of vitamin K at ­birth 34. Which of the following statements regarding ABO incompatibilities is ­true? A. ABO incompatibility does not protect against fetal Rh ­disease B. ABO incompatibility is more frequent in mothers with type O ­blood C. ABO incompatibility is more severe than Rh ­incompatibility 35. A positive result from a direct Coombs test will ­indicate A. The blood type of the ­newborn B. That antibodies are present in the maternal ­serum C. Maternal IgG antibodies are present on the surface of the infant’s red ­cells 36. Vitamin K deficiency is also known as hemorrhagic disease of the newborn. A factor that will negatively influence the production of vitamin K ­is A. A lack of sufficient intestinal ­flora B. Overproduction of coagulation factors II and ­X C. Excess circulating extracellular ­calcium 37. The most common coagulation disorder in neonates ­is A. ­Polycythemia B. ­T hrombocytopenia C. ­Hemophilia 38. Which of the following medications may cause thrombocytopenia in a ­neonate? A. ­Gentamycin B. ­Furosemide C. ­Heparin 39. Which of the following conditions can cause a neonate’s body to destroy platelets more quickly than they are ­produced? A. Lupus e­ rythematosus B. Chlamydia ­infection C. Hepatitis ­B

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40. Your term patient is at 1 hour of life. His bilirubin level is 7 mg/dl, and his hemoglobin level is 7.6 g/dl. This infant requires an exchange transfusion. Which of the following statements about exchange transfusions is ­true? A. Rebound bilirubin levels in 1 hour will be 75% of the original ­value B. A ­double-­volume is not as effective as a ­single-­volume exchange when removing ­bilirubin C. A s­ ingle-­volume exchange removes 85–90% of ­bilirubin 41. A major side effect of an exchange transfusion ­is A. Necrotizing ­enterocolitis B. ­Hematoma C. ­Urticaria 42. The ­Kleihauer-­Betke test ­identifies A. Common blood group ­antigens B. Fetal hemoglobin in maternal ­blood C. Bone marrow lymphocyte ­precursors 43. A decrease in the available number of erythrocytes caused by bone marrow production failure is known ­as A. Chronic ­anemia B. Aplastic ­anemia C. Hemolytic ­anemia 44. Your patient’s mother carries the sickle cell trait. Which statement about sickle cell anemia is ­true? A. Sickle cell anemia always results in death by age ­40 B. Sickle cell anemia causes a loss of protein intrinsic ­factor C. High blood viscosity and low oxygen tension may cause a ­crisis 45. The diagnosis of immune thrombocytopenic purpura may be made ­by A. Thrombin ­time B. Platelet antibody ­screen C. ­PTT 46. A partial thromboplastin time (PTT) is used to ­assess A. The number of ­platelets B. The number of fibrin degradation products ­present C. Intrinsic and common portions of the coagulation ­cascade 47. Your patient is a term infant just admitted to the NICU with mild respiratory distress. On assessment, you note a ruddy skin color and the infant appears lethargic. About 10 minutes after admission, the infant has a 20-second seizure. You suspect the infant ­has A. Hyperviscosity ­syndrome B. Physiologic anemia of the ­newborn C. An ABO i­ ncompatibility

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48. A nursing action appropriate when administering amphotericin B intravenously i­ s A. Monitoring daily serum ­levels B. Not mixing with normal ­saline C. Decreasing the infant’s sodium ­intake 49. When administered intravenously, amphotericin B is compatible with which of the following ­medications? A. Magnesium ­sulfate B. ­Gentamycin C. ­Heparin 50. Your patient is a term neonate who has been diagnosed with a urinary tract infection and is receiving cefazolin. Cefazolin is effective against which of the following ­organisms? A. ­Penicillin-­resistant Staphylococcus ­aureus B.  Escherichia c­ oli C. ­Protozoa 51. A mother’s breastmilk contains the immunoglobulin known ­as A. ­IgE B. ­IgA C. ­IgM 52. The major immunoglobulin of serum and interstitial fluid ­is A. ­IgG B. ­IgM C. ­IgE 53. Drug therapy in the extremely ­low-­birth-­weight (ELBW) ­infant A. May more readily pass through the blood–brain ­barrier B. Is an exact ­science C. Is i­ neffective 54. Which of the following statements about human breastmilk is ­true? A. Human breastmilk provides absolute protection against bronchopulmonary ­dysplasia B. Human breastmilk is always the last choice for an ­infant C. Human breastmilk is high in ­c ytokines 55. Genitourinary infection in the two first trimesters of pregnancy is associated ­with A. Intraventricular ­hemorrhage B. Cerebral ­palsy C. Cytomegalovirus ­infection

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56. Pertussis in infants less than 1 month old who received erythromycin are at increased risk ­for A. Infantile hypertrophic pyloric ­stenosis B. ­Toxoplasmosis C. ­Rubella 57. Obstetric tetanus is defined ­as A. Contamination of the umbilical ­stump B. Tetanus during pregnancy or within 6 weeks after termination of a ­pregnancy C. A ­gram-­positive ­sepsis 58. Which of the following statements about respiratory diptheria is ­true? A. Protection against respiratory diphtheria is predominantly from immunoglobulin G ­antibody B. Maternal antitoxin is not transferred to the ­fetus C. The mortality rate is approximately 90% when ­untreated 59. Erythropoietin levels will decrease i­ n A. Down ­syndrome B. Intrauterine growth ­restriction C. ­Hypertransfusion 60. A disease that can lead to destruction of red blood cells, decreased platelets, and impairment of kidney function ­is A. Hemolytic uremic ­syndrome B. Idiopathic thrombocytopenic ­purpura C. Thrombotic thrombocytopenic ­purpura 61. The normal volume of blood in a term infant ­is A. 40 to 60 ­ml/kg B. 80 to 100 m ­ l/kg C. 100 to 120 m ­ l/kg 62. The normal volume of blood in a preterm infant ­is A. 70 to 80 m ­ l/kg B. 90 to 105 m ­ l/kg C. 110 to 125 m ­ l/kg 63. The extrinsic system of the coagulation cascade is initiated ­by A. Factor X ­activation B. Vascular endothelial ­injury C. Exposure of cell membrane tissue ­factor 64. Which of the following tests is used to assess the extrinsic and common pathways of the coagulation c ­ ascade? A. Split fibrin ­product B. Partial thromboplastin ­time C. Prothrombin time

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65. ­Twin-­to-­t win anemia at birth may be caused ­by A. Traumatic a ­ mniocentesis B. Umbilical cord ­r upture C. A monozygotic ­placenta 66. Rhesus (Rh) hemolytic disease of the newborn is a severe, often fatal disease caused ­by A. An ­Rh-­negative mother alloimmunized during the first Rh incompatible ­pregnancy B. An R ­ h-­positive mother with an ­R h-­negative f­ etus C. An allergy to rhesus ­serum 67. If the mother of your neonatal patient received an incompatible transfusion of ­Rh-­positive platelets following a placental rupture, it would be appropriate to ­administer A. ­CuroSurf B. ­Neostigmine C. ­R hoGAM 68. Venous hemoglobin values of less than in infants born at 34–35 weeks gestation or more are considered a ­ bnormal A. 13 ­g/dl B. 19 g ­ /dl C. 15 ­g/dl 69. A pharmacologic antagonist to vitamin K ­is A. ­Phenobarbital B. ­Digoxin C. ­A miodarone 70. Coagulation factors are 30–40% lower in neonates ­with A. Congenital heart ­defects B. ­Polycythemia C. ­Hypoxia 71. The blood component that carries factor VIII, factor XIII, and fibrinogen ­is A. ­FFP B. ­PRBC C. ­Cryoprecipitate 72. Your patient is receiving INH (isoniazid) for treatment of TB. The normal dosage should be calculated ­at A. 3–5 ­mg/kg/day B. 5–10 m ­ g/kg/day C. 10–20 ­mg/kg/day

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73. You receive the H&H results on a term infant. The H&H reveals Hgb 19.5; Hct 64; MCV 122; reticulocytes 33; and platelets 300,000/ml. You ­should A. Place the results in the ­chart B. Report to the doctor the increased Hgb ­level C. Report to the doctor the decreased platelet ­level 74. Neonates experience both acute (hemorrhage or line displacement) and chronic blood loss (intraventricular hemorrhage and blood sampling) during treatment in a NICU. Which statement best describes the level of treatment for chronic and acute blood l­ oss? A. A baby with chronic blood loss requires less intervention than a baby with acute blood ­loss B. A baby with chronic blood loss requires more intervention than a baby with acute blood ­loss C. Chronic and acute blood loss are managed exactly the same ­way 75. Contraindications for use of an intraosseous needle ­include A. Infection and Osteogenesis ­imperfecta B. Infection and hepatic vein ­thrombosis C. Hepatic vein thrombosis and renal vein ­thrombosis 76. Which of the following medications should be protected from ­light? A. ­A mikacin B. ­A mphotericin C. ­Atropine 77. You are preparing to assist in the delivery of an infant with a prenatal diagnosis of hydrops fetalis. You should be prepared for which of the following ­complications? A. ­Seizures B. ­Kernicterus C. Severe respiratory ­distress 78. Your patient has been exposed to Group B Streptococcus. You should notify the physician if the baby e ­ xhibits A. Hypothermia and poor ­feeding B. Anemia and ­pallor C. Heart murmur and ­hypercalcemia 79. What is the most common cause of hydrops ­fetalis? A. Rh i­ mmunization B. Maternal substance ­abuse C. Fetal cardiac ­disorder

This concludes the Hematology/Immunology ­questions.

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Hematology/Immunology ­Answers 1. Correct answer: ­C Sepsis is the most common cause of DIC because endotoxins stimulate production of tissue factor and the extrinsic pathway is activated. If the process continues, thrombi in capillaries can lead to hypoperfusion and metabolic acidosis, causing free radical formation and damage to tissues. Tissue factor is released, resulting in DIC. Answers A and B are also causes of DIC, but neither is the most common ­cause. 2. Correct answer: ­A Disseminated intravascular coagulation is an overstimulation of the clotting cascade. Both the intrinsic and extrinsic pathways are activated at the same time. This causes an acceleration of the clotting process. When the clots lyse, the fibrin split products are anticoagulants. Eventually, all the clotting factors are used up, and no further clots can form. Neonates are at increased risk for DIC secondary to inappropriate levels of anticoagulants and fibrinolytics, along with decreased levels of antithrombin and protein. One of the treatments for DIC is antithrombin III, which sometimes attenuates organ failure and reverse ­coagulopathy. 3. Correct answer: ­B Progressive ­gram-­negative bacterial infections and malignancies may cause DIC. Trauma may result in a bacterial ­infection. 4. Correct answer: ­A Neonates with DIC are at increased risk of developing deficiencies in vitamin K and folate. Neonates are also at risk for developing intraventricular and parenchymal hemorrhage, renal and other organ failure, and microvascular ­thrombosis. 5. Correct answer: ­A Factor VIII deficiency is also known as von Willebrand’s ­disease. 6. Correct answer: ­B Cryoprecipitate means it is quick frozen. It contains large amounts of factor VIII, factor XIII, and some factor I (fibrinogen). Cryoprecipitate does have disadvantages. It is expensive, and there are newer recombinant factor VIII products available. There is the risk of transmission of hepatitis A, hepatitis C, hepatitis G, and ­HIV. 7. Correct answer: ­C Prostaglandin relaxes the smooth muscles within the pulmonary airways and ­promotes vasodilation within arteries. Inotropic support is usually accomplished with ­epinephrine. 8. Correct answer: ­A Hemoglobin is comprised of two parts. The first part is “heme” that causes the reddish color and contains iron and porphyrin. The second part is a protein called “globin” that hemoglobin combines with oxygen to form oxyhemoglobin. Hemoglobin also binds with CO2 and carries it to alveoli to be expired. When the hemoglobin is

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phagocytized, it breaks down into the heme and globin. The iron in the hemoglobin is processed and reused to manufacture new hemoglobin. The porphyrin converts to bilirubin and is excreted in urine and ­feces. 9. Correct answer: ­A In this type of anemia, the erythrocytes are very thin and fragile. They are known as “target cells.” The serum bilirubin is very elevated, so you should caution the parent against scratching. Cool water and lotion may be used for skin care. Oral stimulants could lead to vasoconstriction. The integrity of the skin is weakened, so careful positioning is paramount. The patient may require ongoing transfusions. Multiple transfusions may actually lead to too much iron that might have to be chelated ­out. 10. Correct answer: ­C Erythropoietin is a hormone responsible for the synthesis of hemoglobin. Erythropoietin levels increase in response to anemia and low oxygen states and levels decrease in response to hypertransfusion. Levels are also increased in infants with Down syndrome and intrauterine growth restriction. Erythropoietin may also be elevated in infants born to mothers with ­pregnancy-­induced hypertension or ­diabetes. 11. Correct answer: ­B Transition from fetal hemoglobin to adult hemoglobin (HgA) begins at the end of fetal life. Red blood cells contain about 70–90% fetal hemoglobin (HgF) at ­birth. 12. Correct answer: ­A Hemoglobin synthesis begins at approximately 14 days of embryonic ­life. 13. Correct answer: ­C During fetal life, erythropoietin is produced primarily by the submandibular glands and the liver. After birth, the kidneys produce the majority of ­erythropoietin. 14. Correct answer: ­C Baby M. had a total serum bilirubin of 24 mg/dl, indicating mild dehydration and hyperbilirubinemia. The baby was placed under phototherapy lights to help resolve the jaundice by breaking down the bilirubin. Phototherapy will cause insensible water loss. If this infant has diarrhea, the loose stools will help clear the bilirubin, but could add to dehydration. It is incumbent on the nurse to closely monitor for further signs of dehydration. Infants usually do not become hyperthermic or suffer burns during ­phototherapy. 15. Correct answer: ­B This patient is in metabolic acidosis. The acidosis increases the risk of bilirubin toxicity, so it must be corrected prior to the transfusion. Increased hydrogen ions and an acidotic state impede bilirubin binding. At a serum pH of 7.1, bilirubin binding drops to half its potential for binding at albumin binding sites. During acidosis, free fatty acids are formed and compete with the albumin binding ­sites.

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16. Correct answer: ­A All cord blood found in a fetus is unconjugated and is metabolized, conjugated, and excreted by the maternal gallbladder and liver. If an infant is unaffected by hemolytic disease, the mean cord blood bilirubin concentration is 1.8 mg/dl. This value is independent from and unaffected by the infant’s gestational age or ­weight. 17. Correct answer: ­C If albumin binding sites are filled, increased amounts of free bilirubin are passed into the central nervous system and may result in kernicterus. Free bilirubin crosses the blood–brain barrier and lodge in the brain cells. The patient is usually affected in the first 5 days of life. Symptoms include lethargy, irritability, hypotonia, a ­high-­pitched cry, and poor eating. The infant may also demonstrate spasticity and opisthotonic posturing. If bilirubin levels rise slowly, phototherapy might be able to reduce the bilirubin level and prevent kernicterus. If the bilirubin level rises quickly, exchange transfusion may be ­required. 18. Correct answer: ­B Infants with kernicterus will have certain brain cells that stain a bright yellow color. The areas most affected by this staining are the cerebellum, the dentate nucleus, and the hypothalamus. Resultant neurologic damage cannot be directly correlated with the amount of staining present. Some damage occurs, and kernicterus has been associated with cerebral palsy, deafness, and tooth enamel ­degradation. 19. Correct answer: ­C Erythroblastosis fetalis is the result of Rh incompatibility in the fetus. This condition usually results in anemia and hyperbilirubinemia. Too many circulating immature red blood cells (erythroblasts) are forced into the fetal circulation to compensate for rapid destruction of fetal blood cells. Anemia and hyperbilirubinemia are the result. Jaundice is secondary to the rapid rise in bilirubin and the infant may have hepatosplenomegaly. Hydrops fetalis may also be ­present. 20. Correct answer: ­A Hydrops fetalis is a severe, total body edema and occasionally is accompanied by pleural effusions and ascites. Most research concludes that this condition is the result of congestive heart failure and intrauterine hypoxia from severe anemia. The patient also has low serum albumin levels. Most infants with hydrops have portal and umbilical venous hypertension secondary to hepatic ­hematopoiesis. 21. Correct answer: ­A Hemorrhage in an infant with hydrops fetalis is usually the result of altered hepatic synthesis. Production of vitamin K and vitamin ­K-­dependent clotting factors may lead to hemorrhage. Initial signs of bleeding in an infant with hydrops fetalis may be oozing from cord and blood sampling sites and generalized petechiae. Hypoglycemia may also be ­present.

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22. Correct answer: ­A All factors except VIII and XII are synthesized in the liver, so that is why liver injuries can bleed so much and are so dangerous. While we were researching and confirming this question, we found out that the factors in the clotting cascades were numbered by order of discovery, not in the order of use, which explains why the numbers are out of order. Just FYI! 23. Correct answer: ­C The B lymphocytes originate in bone marrow and mature there. The B lymphocytes form antibodies (immunoglobulins) that formulate a response to a specific antigen that binds itself to the B cell’s receptor sites. The B cell then forms a specific antibody for that particular antigen. Five different types of immunoglobulins are available: IgG, IgA, IgM, IgE, and IgD. After the antibodies are synthesized, the specific antibody can attach to its antigen and set off the reaction to allow for phagocytosis. The cells retain a “memory” for the specific antigen, and if another exposure occurs, the response will be quicker and ­stronger. 24. Correct answer: ­B Monocytes are essentially immature macrophages. Monocytes and lymphocytes are classified as agranulocytes or nongranular leukocytes. The monocyte is the largest leukocyte, but it comprises a small amount of the total cell count for WBCs. The monocytes mature and become a tissue macrophage and work as phagocytes. When a phagocyte lives in the liver, it is called a Kupffer cell. When it is in the lungs, it is called an alveolar macrophage, and when it is in the connective tissues, it is a ­histiocyte. Macrophages contain lysosomal enzymes, and chemicals that can destroy bacteria. If the macrophage is activated by an antigen, it will secrete monokines, which control communication among all the cells involved in an immune response. The monocyte is responsible for clearing out old blood cells, opsonized bacteria, cellular debris, ­antigen-­antibody complexes, and remaining activated clotting factors from the ­circulation. 25. Correct answer: ­B Iron-­deficiency anemia is the most frequently occurring anemia seen around the world. It can be caused by an ­iron-­poor diet or an excessive loss of iron. Trauma is a primary cause of acute loss and can result from bleeding. Idiopathic blood losses, repeated blood draws, GI bleeding, malabsorption syndromes, and excessive diarrhea are all potential causes of this type of anemia. If you can view the erythrocytes, they look kind of “puny”. They are pale from lack of hemoglobin and tend to be smaller than a normal ­cell. 26. Correct Answer: ­B Mismatched blood causes a hemolytic reaction resulting in systemic cellular lysis. The overwhelming destruction of cells cannot be corrected rapidly enough by the bone ­marrow.

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27. Correct answer: ­A Thymus-­derived (T cells) lymphocytes initiate a response to delayed hypersensitivity reactions and ­graft-­versus-­host disease. The function of T cells is cellular immunity. These cells recognize the transplanted organ cells as foreign, resulting in a mounted attack leading to rejection. Immunosuppressive drugs suppress this normal ­response. 28. Correct answer: ­A An acute intravascular hemolytic reaction may cause chills, fever, tachycardia, hypotension, hematuria, back pain, and the infant may exhibit additional signs of shock. The extravascular hemolytic reaction may manifest as fever, an elevated bilirubin, and a low H and H, even after ­transfusion. 29. Correct answer: ­C Pernicious anemia results from lack of protein intrinsic factor in the stomach that helps the body absorb vitamin B12 . This stress on the heart from resultant hypoxia can cause heart murmurs, tachycardias, arrhythmias, hypertrophy, and heart failure. A lack of vitamin B12 raises the homocysteine level. High levels of homocysteine add to the buildup of fatty deposits. A lack of vitamin B12 can damage nerve cells and cause problems such as paresthesias in the hands and feet and problems with ambulation and balance. Memory loss, visual disturbances, and confusion may develop. The condition was named “pernicious” because it was often fatal before the cause was discovered to be a lack of vitamin ­B12 . 30. Correct answer: ­A A red blood cell has multiple functions, including carbonic acid dissociation to form bicarbonate ions. The RBC provides oxygen transport via hemoglobin and carbon dioxide transport via carboxyhemoglobin. The RBC buffers protons by binding with hemoglobin to form acid ­hemoglobin. 31. Correct answer: ­B A mean corpuscular hemoglobin (MCH) test measures the average amount (by weight) of hemoglobin in each RBC. A mean corpuscular hemoglobin concentration (MCHC) test measures the average concentration of hemoglobin per single RBC. A mean corpuscular volume (MCV) test measures the average size and volume of a single ­RBC. 32. Correct answer: ­C The normal range for platelets in the term and premature infant is 150,000 to 400,000/mm3. Infants who are small for gestational age generally have platelet counts 20–25% lower. Neonatal platelets are hypoactive during the first few days after birth. This hypoactivity may predispose the infant to an increased risk of coagulopathy and bleeding. However, the low platelet count protects the infant against ­thrombosis. 33. Correct answer: ­C Transient deficiencies in newborn clotting mechanisms include a deficiency of vitamin K at birth. The vitamin K is necessary for the synthesis of factors II, VII, IX, X, and XII. Diminished platelet function and a transient deficiency of clotting factors II, VII, IX, X,

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XI, and XII exist. The clotting factors are at approximately 50% of adult levels at birth and remain at that level for several weeks. Immaturity of hepatic enzymes, lack of sufficient vitamin K, and the low factor concentrations result in transient deficiencies of newborn clotting ­mechanisms. 34. Correct answer: ­B ABO incompatibility is most often seen in mothers with type O blood, which has an absence of antigens. If the mother is exposed to A and B antigens from the fetus, an ABO incompatibility will exist. The mother can also be exposed to A and B antigens carried in food, bacteria, and pollen. This can make the first pregnancy perilous. ABO incompatibility is less severe than Rh incompatibility and occurs more frequently. ABO incompatibility protects against fetal Rh disease because of rapid destruction of A and B cells. This rapid destruction prevents Rh antigen exposure and resultant maternal antibody ­production. 35. Correct answer: ­C A positive result from a direct Coombs’ test will indicate presence of maternal IgG antibodies on the surface of the infant’s red blood cells. Cord blood that is contaminated by Wharton’s jelly may produce unreliable results. Cold agglutinins may cause ­false-­positive results. Heparin sodium and heparin calcium may cause ­false-­negative results. Many diseases and drugs (quinidine, methyldopa, and procainamide) can lead to production of these ­antibodies. A positive, indirect Coombs’ test indicates that antibodies against the infant’s red cells are present in the mother’s ­serum. 36. Correct answer: ­A A more appropriate term for hemorrhagic disease of the newborn is vitamin K deficiency bleeding (VKDB) disease. Vitamin K production depends on sufficient intestinal flora. Vitamin K is necessary for the production of calcium and various coagulation ­factors. Newborns are relatively deficient in vitamin K. Contributing factors to this deficiency include low vitamin K stores at birth, poor placental transfer of vitamin K, low levels of vitamin K in breastmilk, and sterility of the gut. Basic commercial infant formulas contain supplemental vitamin K, so VKDB is almost exclusively a condition resulting from breastfed infants. Infants with inadequate intake are at higher risk. The most common sites of bleeding are the umbilicus, mucous membranes, GI tract (melena), circumcision, and venipuncture sites. The infant may also present with a distended abdomen, jaundice, and signs of increased intracranial pressure. Hematomas, such as large cephalohematomas, along with bruising, are also common findings. Intracranial hemorrhage (ICH) is responsible for nearly all deaths from ­V KDB. 37. Correct answer: ­B The most common coagulation disorder in neonates is thrombocytopenia. This is an acquired disease, which results in a decrease in the platelet count to , 150,000/mm3. There are four significant causes of thrombocytopenia: a decrease in production of platelets by the bone marrow, platelet loss through hemorrhage, increased destruction of circulating platelets, and increased trapping of platelets by the spleen. The most

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common cause of thrombocytopenia is a decrease in the production of platelets by the bone marrow. When abnormalities such as an infection occurs in the bone marrow, whether it is bacterial or congenital, the platelets may be consumed or not develop in sufficient ­numbers. 38. Correct answer: ­C Heparin is a medication that can cause a thrombocytopenic reaction by confusing the immune system and causing it to destroy platelets. Other medications with a thrombocytopenic action are quinidine, quinine, ­sulfa-­containing antibiotics, some oral diabetes drugs, gold salts, and ­r ifampin. 39. Correct answer: ­A Examples of maternal conditions that will cause a neonate’s body to use or destroy platelets more quickly than they are produced are lupus erythematosus, rheumatoid arthritis, and ­pregnancy. 40. Correct answer: ­B A ­double-­volume exchange is not as effective as a ­single-­volume exchange when removing bilirubin. During a ­double-­volume exchange, only about 25% of bilirubin is removed. This is probably because the majority of the bilirubin would be in the extravascular space, which is not affected by blood exchange. Rebound bilirubin levels in a s­ ingle-­volume exchange reach about 55% of the original level within 1 hour of the transfusion. A ­double-­volume exchange removes about 85–90% of the infant’s RBCs. Exchange transfusions also have the advantage of removing maternal antibodies and Rh positive fetal RBCs and can help normalize a ­hematocrit. 41. Correct answer: ­A Some serious side effects of exchange transfusions are necrotizing enterocolitis, air embolism, volume and pressure changes, thromboembolism, bradycardia, bacterial contamination, hypothermia, volume overload, ­transfusion-­mediated lung injury, and ­death. 42. Correct answer: ­B The K ­ leihauer-­Betke test identifies fetal hemoglobin in maternal blood. The results of this test allow for calculations to determine the amount of ­fetal-­maternal hemorrhage and the amount of immune globulin (RhoGAM) necessary to prevent sensitization. If a patient has a positive test, ­follow-­up testing at a postpartum ­check-­up should be done to rule out the possibility of a false positive result. For example, a ­false-­positive result in the mother could be caused by sickle cell trait, which causes persistent elevation of fetal ­hemoglobin. 43. Correct answer: ­B A decrease in the available number of erythrocytes caused by bone marrow production failure is known as aplastic anemia. About 50% of all the cases of aplastic anemia are caused by toxins. The other 50% have an unknown cause. Some of the causes are radiation ­(X-­rays, radioactive isotopes, radium), benzene, streptomycin, carbon tetrachloride, DDT and other pesticides, chloramphenicol, and ­sulfonamides.

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44. Correct answer: ­C Sickle cell anemia occurs primarily in the African American population. People with this condition are homozygous for HgS and have more HgS than HgA. This causes some of the cells to form a “sickle” shape—curved with rough edges. A crisis can occur when the low oxygen tension (postulated) causes a proliferation of these cells. The sharp edges of these cells travel through the microcirculation and damage capillaries. A simple thing like cold weather can precipitate massive sickling. Other identified factors include dehydration, vomiting, diarrhea, high altitude, excessive exercise, and stress. When these cells break apart, they occlude the microcirculation and lower oxygen tension, which initiates more sickling. This is a very painful time for the patient, and oxygen, pain management, and fluids are very ­important. 45. Correct answer: ­B The PT, PTT, and thrombin time are normal in a patient with immune thrombocytopenic purpura (ITP) because these tests only measure nonplatelet factors in the coagulation cascade. The platelet antibody screen measures the presence of IgG and IgM antiplatelet ­antibodies. 46. Correct answer: ­C A partial thromboplastin time (PTT) is used to assess intrinsic and common portions of the coagulation cascade. The results may be affected by an absence of clotting factors, anticoagulants, low levels of clotting factors, inhibitors, and an increased use of clotting ­factors. A high PTT may be the result of hemophilia A or B, factor VIII or factor IX deficiency, liver disease, or ­DIC. 47. Correct answer: ­A Hyperviscosity syndrome occurs when the percentage of red blood cells in the infant’s blood is greater than 65%. Various conditions that develop before birth may cause this condition. For example, birth defects, congenital diseases, hypoxia, and delayed cord clamping may cause hyperviscosity syndrome. Classic symptoms include respiratory distress, a ruddy appearance, lethargy or sleepiness, and ­seizures. 48. Correct answer: ­B A nursing action appropriate when administering amphotericin B intravenously is not mixing the drug with normal saline. Mixing in normal saline will cause the solution to precipitate. It is also necessary to protect this drug from light. Amphotericin B injectable is compatible with D5W, D10W, D15W, and D20W. Monitoring serum levels daily is not routinely necessary. When the infant is receiving amphotericin B, it is necessary to augment the sodium intake to at least . 4 mEq/Kg per day to help prevent ­nephrotoxicity. 49. Correct answer: ­C When administered intravenously, amphotericin B is compatible with heparin, amiodarone, hydrocortisone, sodium bicarbonate, and ­zidovudine.

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50. Correct answer: ­A Cefazolin is effective against ­penicillin-­resistant Staphylococcus aureus, Proteus and Klebsiella. Cefazolin is a ­first-­generation cephalosporin and is used against multiple ­gram-­positive organisms and a few ­gram-­negative organisms. It is important to note that cefazolin is inactivated by ­B -­lactamase producing organisms. The ­half-­life of this drug in neonates is 3 to 5 h ­ ours. 51. Correct answer: ­B A mother’s breastmilk contains the immunoglobulin known as immunoglobulin A (IgA). This immunoglobulin is also secreted in human colostrum. IgA does not cross the placental barrier and is the most common immunoglobulin in the gastrointestinal and respiratory ­tracts. During pregnancy and lactation, because of hormonal stimuli, IgA B lymphocytes colonize mammary glands and produce a specific secretory IgA that may bind to a pathogen and prevent infection. The antimicrobial effects of IgA antibodies are related both to immune exclusion, interference, or an inhibited ability to adhere to the epithelial cell wall help provide protection to the neonate. Agglutination, neutralization, and immune elimination by phagocytosis and cytotoxicity may enhance the antimicrobial effects as well. ­HIV-­infected mothers do not evidence protection. IgA antibodies may enhance transmission of HIV ­infection. 52. Correct answer: ­A The major immunoglobulin of serum and interstitial fluid is immunoglobulin G (IgG). This immunoglobulin provides immunity against viral and bacterial pathogens. The IgG transfers to the fetus either actively or passively and increases somewhat gradually until 40 weeks of gestation. In postmature infants, the levels of IgG are decreased as they are with premature infant. However, in the preterm infant, the level is proportionate to the gestational ­age. 53. Correct answer: ­A Prematurity may influence pharmacology through penetration of drug into sites that are usually shielded, such as the blood–brain barrier. Drug therapy of the ELBW newborn is often extrapolated from older newborns, children, or even adults. Drug therapy in this extremely immature group may not produce similar effects to those in the older population for a variety of reasons. The concentration of the drug at the site of action and the mechanism of action may be very different in extremely ­low-­birth-­weight infants. This discrepancy may be due to the volume of the medication, protein binding, metabolism, and excretion. These factors are difficult to predict from experience or studies in more mature infants. Even if you administer similar concentrations at the site of action, the effects of those concentrations may also differ. Receptor number, binding, affinity, and coupling of those receptors may be unique to each ELBW ­infant. 54. Correct answer: ­C Human breastmilk is high in cytokines. Cytokines are soluble proteins that help stimulate the chemotaxis of neutrophils and help in epithelial cell propagation. Of all the factors associated with ­immunity—­immunological, hormonal, and enzymatic—­ cytokines are believed to play a significant role in the immune modulation and

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immune protection of breastmilk. Most of the cytokines that are known to be deficient in the neonate, particularly in preterm infants, have been found in significant amounts in ­breastmilk. 55. Correct answer: ­B Genitourinary infection in the first two trimesters of pregnancy is associated with increased risk of cerebral palsy. The association is very strong in infants born before 37-weeks gestation and weighing less than 2500 g. A genitourinary infection is not significant in term or ­normal-­birth-­weight infants. In one study, specific infections, such as chlamydia, trichomoniasis, and urinary tract infection appeared to be associated with cerebral palsy. Gonorrhea and candidiasis were not associated with a significant risk of cerebral palsy. Chorioamnionitis (symptomatic intrauterine infection) is also a risk factor for cerebral palsy. The study further showed that in term infants born to women with histologic chorioamnionitis, contributed to the incidence of cerebral palsy. It was thought that the inflammatory response to intrauterine infection (cytokines) including interleukin and tumor necrosis factor alpha may cause periventricular ­leukomalacia. 56. Correct answer: ­A Infants younger than 1 month who receive erythromycin are at increased risk of infantile hypertrophic pyloric stenosis. For this reason, and because azithromycin is associated with fewer adverse effects than erythromycin, azithromycin is the preferred antimicrobial for prophylaxis of newborns exposed to pertussis. A macrolide antibiotic (erythromycin, azithromycin, or clarithromycin) is preferred for postexposure prophylaxis and treatment of pertussis. Antimicrobials generally do not affect the severity or course of illness after paroxysmal cough has begun, but they can eliminate Bordetella pertussis and stop transmission to newborns. A macrolide should be given to women with pertussis that is acquired late in pregnancy, to their household contacts, and to their infants. Early recognition of pertussis is necessary to ensure the effectiveness of this ­approach. 57. Correct answer: ­B Obstetric tetanus is defined as tetanus that occurs during pregnancy or within 6 weeks after termination of a pregnancy. It occurs after contamination of wounds or abrasions, or after nonsterile deliveries or abortions. Obstetric tetanus has the highest mortality rate when the incubation period is short and respiratory complications are present. Cases can be complicated by ­gram-­negative sepsis. Neonatal tetanus is associated with contamination of the umbilical stump. Onset occurs during the first month of life, and symptoms (e.g., increasing irritability, difficulty feeding) commonly begin at 3 to 14 days of life. Pregnant women who receive a booster dose of tetanus toxoid have an immune response within 5 days. Placental transport of antitoxin is excellent and cord blood levels are similar to maternal levels. After the neonatal period, infants are at little risk of tetanus until they become ­mobile. 58. Correct answer: ­A Protection against respiratory diphtheria is predominantly from immunoglobulin G antibody. Maternal antitoxin is transferred to the fetus. Transplacental maternal ­antitoxin provides newborns with protection against diphtheria if the mother is

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immune. Respiratory diphtheria can occur during any trimester of pregnancy, at term, or in the postpartum period. The mortality rate of obstetric respiratory diphtheria is estimated at 50% when no antitoxin is ­administered. 59. Correct answer: ­C Erythropoietin levels will decrease in hypertransfusion. Levels will increase in Down syndrome, intrauterine growth restriction, hypoxia, and infants born to mothers with ­pregnancy-­induced hypertension or ­diabetes. 60. Correct answer: ­A Hemolytic uremic syndrome can cause a decrease in platelets, destruction of red blood cells, and impairment of kidney function. Sometimes, this can occur in association with a bacterial Escherichia coli (E. coli) infection. Idiopathic thrombocytopenic purpura (ITP) is a condition in which the immune system mistakenly identifies platelets as a threat and forms antibodies that attack them. Thrombotic thrombocytopenic purpura (TTP) is a l­ ife-­threatening condition that occurs when small blood clots suddenly form throughout the body, using up large numbers of platelets. TTP sometimes happens as a result of a genetic deficiency, but more often the cause is unknown. In some cases, it may be associated with infection or a chronic illness. Blood poisoning is very rare in a neonate. However, severe bacterial infections may cause destruction of ­platelets. 61. Correct answer: ­B The normal volume of blood in a term infant is 80 to 100 ­ml/kg. 62. Correct answer: ­B The normal volume of blood in a preterm infant is 90 to 105 ­ml/kg. 63. Correct answer: ­C The extrinsic system of the coagulation cascade is initiated by exposure of cell membrane tissue factor from tissue injury. The intrinsic system is initiated by vascular endothelial ­injury. 64. Correct answer: ­C The prothrombin time (PT), is used to assess the extrinsic and common pathways of the coagulation cascade. The test used to assess the intrinsic and common pathways of the coagulation cascade is the partial thromboplastin time (PTT). 65. Correct answer: ­C Twin-­to-­t win anemia at birth may be caused by a monozygotic, monochorionic (single) placenta. A hemoglobin difference between twins greater than 5 g/dl is also a cause of ­t win-­to-­t win anemia. ­Fetal-­maternal anemia can be caused spontaneously or from a traumatic ­amniocentesis. 66. Correct answer: ­A Rhesus (Rh) hemolytic disease of the newborn (HDN) is a severe, often fatal disease caused by incompatibility between an ­Rh-­negative mother and her ­Rh-­positive fetus. The mother becomes alloimmunized to the D antigen present on fetal red blood cells

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(RBCs) during the first ­Rh-­incompatible pregnancy. The first pregnancy is rarely affected because the number of Rh antibodies produced by the mother is low and the antibodies are usually IgM. When the mother is exposed to ­D -­positive fetal RBCs during a subsequent ­Rh-­incompatible pregnancy, the mother develops a secondary immune response. A large number of IgG Rh antibodies are produced that cross the placenta and make fetal red cells susceptible to attack by antibodies. The mother may also be alloimmunized from ­fetal-­maternal hemorrhage, bleeding that occurs during normal delivery, ectopic pregnancies, spontaneous or induced abortions, and abdominal ­trauma. 67. Correct answer: ­C If the mother of your neonatal patient received an incompatible transfusion of ­R h-­positive platelets following a placental rupture, it would be appropriate to administer RhoGAM. This medication may be used for prevention of Rh immunization in any ­R h-­negative person after transfusion of ­R h-­positive blood or blood products such as platelets or red blood ­cells. 68. Correct answer: ­A A venous hemoglobin value of 13 g/dl for an infant born at 34- to 35-weeks or more gestation in the first week of life is considered ­abnormal. 69. Correct answer: ­A Pharmacologic antagonists to vitamin K are phenobarbital and hydantoin (anticonvulsants) and heparin and warfarin (anticoagulants). These drugs induce hepatic enzymes and increase vitamin K degradation. Vitamin K transport across the placenta is inhibited, and vitamin K dependent clotting factors are ­depressed. 70. Correct answer: ­A Coagulation factors are 30–40% lower in neonates with congenital heart defects compared to neonates who have no congenital heart ­defects. 71. Correct answer: ­C Cryoprecipitate carries factor VII, factor XII, and fibrinogen. There is a risk of disease transmission and transfusion ­reactions. 72. Correct answer: ­C The normal dosage for INH (isoniazid) for treatment of TB is 10 to 20 ­mg/kg/day. 73. Correct answer: ­A An Hgb of 19.5, an Hct of 64, an MCV of 122, reticulocytes of 33, and platelets of 300,000/ml is considered normal. Continue to observe and monitor for ­changes. 74. Correct answer: ­A A baby with chronic blood loss requires less intervention than a baby with acute blood loss. Chronic blood loss may be indicative of disease or profound injury and may be occult in the early stages. An intraventricular bleed that does not resolve or slowly worsens overtime is an example of a chronic blood loss, but the cause for the intraventricular bleed may be genetic or pathologic in nature, such as undiagnosed hemophilia or other clotting disorder. Acute loss of blood has specific and easily identified causes that can be readily treated with volume ­expanders.

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75. Correct answer: ­A Infection and ostegenesis imperfecta are contraindications for use of an intraosseous needle for emergency fluid resuscitation in infants. The preferred route for fluid resuscitation in a neonate is via umbilical line ­catheterization. 76. Correct answer: ­B Amphotericin should be protected from light during administration with a dark bag or foil to prevent the drug from degenerating. This includes tubing, and not just the bag or syringe during ­administration. 77. Correct answer: ­C Infants with hydrops fetalis often have severe respiratory distress at delivery either from initial disease process or complications from congestive heart failure or pulmonary hypoplasia. Respiratory support should be ready and available for intubation and ventilator ­management. 78. Correct answer: ­A Hypothermia and poor feeding are often the first signs and symptoms of Group B streptococcal infection. Worsening respiratory distress may be noted after 2 to 4 hours of life. The infant should cultured and antibiotic therapy started if infection is ­suspected. 79. Correct answer: ­C The most common cause of hydrops fetalis is related to a fetal cardiac disorder resulting in fetal congenital congestive heart failure that was undiagnosed or untreated during ­pregnancy.

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Hematology/Immunology ­References Ahrens, T. (2006). Critical care nursing certification. Columbus, OH: McGraw-­Hill. American Association of Critical-Care Nurses. (2006). Core curriculum for critical care nursing (6th ed.). Philadelphia, PA: ­Saunders. American Association of ­Critical-­Care Nurses. (2007). AACN certification and core review for high acuity and critical care (6th ed.). Philadelphia, PA: ­Saunders. Armstrong, C. (2009). ACIP releases guideline on prevention of pertussis, tetanus, and diphtheria in pregnant women and newborns. American Family Physician, 79(6), 513–514. Aster, R. H., & Bougie, D. W. (2007). ­Drug-­induced immune thrombocytopenia: Current concepts: The New England Journal of Medicine, 357(6), 580–587. Bhullar, I. S., Braman, R., & Block, E. F. J. (2007). Recombinant factor VII as an adjunct to control of hemorrhage from chest trauma in a Jehovah’s Witness. The American Surgeon, 73(8), 818–819. Biagini, E., Spirito, P., Leone, O., et al. (2008). Heart transplantation in hypertrophic cardiomyopathy. The American Journal of Cardiology, 101(3), ­387. Brodsky, D., & Martin, C. (2003). Neonatology review. Philadelphia, PA: Hanley & ­Belfus. Burns, S. M. (Ed.). (2007). American Association of ­Critical-­Care Nurses (AACN): AACN protocols for practice: Healing environments (2nd ed.). Sudbury, MA: Jones and ­Bartlett. Caliskan, M., Erdogan, D., Gullu, H., et al. (2007). Low serum bilirubin concentrations are associated with impaired aortic elastic properties, but not impaired left ventricular diastolic function. International Journal of Clinical Practice, 61(2), 218–224. Chierakul, W., Tientadakul, P., Suputtamongkol, Y., et al. (2008). Activation of the coagulation cascade in patients with leptospirosis. Clinical Infectious Diseases, 46(2), 2 ­ 54. Chirico, G. (2005). Development of the immune system in neonates. Journal of Arab Neonatal Forum, 2, 5–11. Clark, N., Witt, D., Delate, T., et al. (2008). The clinical consequence of subtherapeutic anticoagulation: The low INR study (LINeRS). Journal of Thrombosis and Thrombolysis, 25(1), 127–128. Cloherty, J. P., Eichenwald, E. C., & Stark, A. R. (2004). Manual of neonatal care (5th ed.). Philadelphia, PA: ­Lippincott. Curley, M. A. Q. (1998). Patient nurse synergy: Optimizing patients’ outcomes. American Journal of Critical Care, 7, 64–72. Danes, A. F., Cuenca, L. G., Rodriguez Bueno, S., et al. (2008). Efficacy and tolerability of human fibrinogen concentrate administration to patients with acquired fibrinogen deficiency and active or in ­high-­r isk severe bleeding. Vox Sanguinis, 94(3), 221–226. Dhaliwal, G., Cornett, P. A., & Tierney, L. M. (2004). Hemolytic anemia. American Family Physician, 69(11), 2599–2606. Dingguo Z., Fumin Z., Yuqing Z., et al. (2007). Erythropoietin enhances the angiogenic potency of autologous bone marrow stromal cells in a rat model of myocardial infarction. Cardiology, 108(4), 228–236. Domen, R. E., & Hoeltge, G. A. (2003). Allergic transfusion reactions: An evaluation of 273 consecutive reactions. Archives of Pathology & Laboratory Medicine, 127(3), 316–320. Dossey, B. M., Keegan, L., & Guzzetta, C. (2003). Holistic nursing: A handbook for practice (3rd ed.). Sudbury, MA: Jones and ­Bartlett. Doyle, P., Sajid, M., O’Brien, T., et al. (2008). Drugs targeting parasite lysosomes. Current Pharmaceutical Design, 14(9), 889–900. Dvorak, C. C., & Cowan, M. J. (2008). Hematopoietic stem cell transplantation for primary immunodeficiency disease. Bone Marrow Transplantation, 41(2), 119–126. Edwards, D. F. (1999). The Synergy Model: Linking patient needs to nurse competencies. Critical Care Nurse, 19(1), 88–98.

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Hematology/Immunology  y  237 Emergency Nurses Association & Newberry, L. (2003). Sheehy’s emergency nursing: Principles and practice (5th ed.). St. Louis, MO: M ­ osby/Elsevier. Finch, R. (2009). Antimicrobials: Past, present and uncertain future. Clinical Medicine, 9(3), 257–258. Finkelmeier, B. A. (2000). Cardiothoracic surgical nursing (2nd ed.). Philadelphia, PA: Lippincott Williams & ­Wilkins. Goldstein, G., Toren, A., & Nagler, A. (2007). Transplantation and other uses of human umbilical cord blood and stem cells. Current Pharmaceutical Design, 13(13), 1363–1373. Gurm, H. S., & Eagle, K. A. (2008). Use of anticoagulants in ­ST-­segment elevation myocardial infarction patients: A focus on ­low-­molecular-­weight heparin. Cardiovascular Drugs and Therapy, 22(1), 59–69. Halliday, H. L., McClure, B. G., & Reid, M. (2002). Handbook of neonatal intensive care (4th ed.). Philadelphia, PA: ­Saunders. Hardin, S. R., & Kaplow, R. (Eds.). (2004). Synergy for clinical excellence: The AACN Synergy Model for patient care. Sudbury, MA: Jones and ­Bartlett. Hay, W. W., Levin, M. J., et al. (2007). Current diagnosis and treatment in pediatrics (18th ed.). New York, NY: McGraw-­Hill. Hedner, U., & Brun, N. C. (2007). Recombinant factor VIIa (rFVIIa): Its potential role as a hemostatic agent. Neuroradiology, 49(10), 789–793. Heparin-­induced thrombocytopenia: A quick review of recent studies. (2007). The Journal of Respiratory Diseases, 28(9), 3 ­ 96. Hirohata, A., Nakamura, M., Waseda, K., Honda, Y., et al. (2007). Changes in coronary anatomy and physiology after heart transplantation. The American Journal of Cardiology, 99(11), ­1603. Isaacs, C. E. (2005). Human milk inactivates pathogens individually, additively, and synergistically. Journal of Nutrition, 135, 1286–1288. James, A. H. (2007). Prevention and management of venous thromboembolism in pregnancy. The American Journal of Medicine: Management of Venous Thromboembolism, 120(10B), S­ 26 Johnsen, P., Townsend, J., Bøhn, T., et al. (2009). Factors affecting the reversal of ­antimicrobial­drug resistance. The Lancet Infectious Diseases, 9(6), 357–364. Jones, K. L. (2006). Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: ­Elsevier. Kamoun, M., Grossman, R. A., ­Baid-­Agrawal, S., et al. (2008). ­K idney-­t ransplant rejection and ­anti-­MICA antibodies. The New England Journal of Medicine, 358(2), 196; author reply 1 ­ 96. Karlsen, K. (2006). The S.T.A.B.L.E. Program (5th ed.). Park City, UT: American Academy of ­Pediatrics. Karlsen, K., & Tani, L. Y. (2003). S.T.A.B.L.E.—Cardiac module. Park City, UT: American Academy of ­Pediatrics. Katiyar, R., Kriplani, A., Agarwal, N., et al. (2007). Detection of fetomaternal hemorrhage following chorionic villus sampling by Kleihauer Betke test and rise in maternal serum alpha feto protein. Prenatal Diagnosis, 27(2), 139–142. Kattwinkle, J. (Ed.). (2006). Neonatal resuscitation textbook (5th ed.). Elk Grove Village, IL: American Academy of Pediatrics and American Heart ­A ssociation. Kenner, C., & Lott, J. (2004). Neonatal nursing handbook. St. Louis, MO: ­Elsevier. Kenner, C., & Lott, J. (2007). Comprehensive neonatal care (4th ed.). St. Louis, MO: ­Elsevier. Kenner, C., Amlung, S., & Flandermeyr, A. (1998). Protocols in neonatal nursing. Philadelphia, PA: ­Saunders. Kenner, C., & McGrath, J. M. (Ed.). (2004). Developmental care of newborns & infants: A guide for health professionals. St. Louis, MO: M ­ osby. Kramer, B. (2008). Antenatal inflammation and lung injury: Prenatal origin of neonatal disease. Journal of Perinatology, 28(S1), S21–S27.

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238  y  Section 8 Krishnamoorthy, P., Alyaarubi, S., Abish, S., et al. (2006). Primary hyperparathyroidism mimicking ­vaso-­occlusive crises in sickle cell disease. Pediatrics, 118(2), 786–787. Labbé, E., Herbert, D., & Haynes, J. (2005). Physicians’ attitude and practices in sickle cell disease pain management. Journal of Palliative Care, 21(4), 246–251. Lawrence, R. M., & Pane, C. A. (2007). Human breast milk: Current concepts of immunology and infectious diseases. Current Problems in Pediatric and Adolescent Health Care, 37, 7–36. Levi, M., & Cate, H. T. (1999). Disseminated intravascular coagulation. The New England Journal of Medicine, 341(8), 586–592. Lewis, D. B., & Wilson, C. B. (2006). Developmental immunology and role of host defenses in fetal and neonatal susceptibility to infection. In J. S. Remington, J. O. Klein (Eds.), Infectious diseases of the fetus and newborn infant (6th ed., pp. 87–210). Philadelphia, PA: Elsevier Saunders ­Company. Lipson, J. G., Dibble, S. L., & Minarik, P.A. (Eds.). (1996). Culture and nursing care: A pocket guide. San Francisco, CA: UCSF Nursing ­Press. Lisman, T., & Leebeek, F. W. G. (2007). Hemostatic alterations in liver disease: A review on pathophysiology, clinical consequences, and treatment. Digestive Surgery, 24(4), 250–258. MacDonald, M. G., & Ramasethu, J. (2007). Atlas of procedures in neonatology (4th ed.). Philadelphia, PA: Lippincott ­Williams. Mann, J., McDermott, S., Bao, H., & Bersabe, A. (2009). Maternal genitourinary infection and risk of cerebral palsy. Developmental medicine and child neurology, 51(4), 282–288. Markiewicz, M., & Abrahamson, E. (1999). Diagnosis in color: Neonatology. Philadelphia, PA: ­Mosby. Martinelli, P., Agangi, A., & Maruotti, G. M. (2007). Screening for toxoplasmosis in pregnancy. The Lancet, 369(9564), 823–824. McNally, P. (2001). GI/liver secrets (2nd ed.). Philadelphia, PA: Hanley & ­Belfus/Elsevier. Medina, J., & Puntillo, K. (2006). AACN protocols for practice: Palliative care and ­end-­of-­life issues in critical care. Sudbury, MA: Jones and ­Bartlett. Merenstein, G. B., & Gardner, S. L. (2006). Handbook of neonatal intensive care (6th ed.). St. Louis, MO: ­Mosby. Mitka, M. (2007). Dual antithrombotic therapy’s increased risks not always offset by benefit. Journal of the American Medical Association, 298(13), ­1504. Mongardon, N., Bruneel, F., ­Henry-­Lagarrigue, et al. (2007). Shock during ­heparin-­induced thrombocytopenia: Look for adrenal insufficiency! Intensive Care Medicine, 33(3), 547–548. Norris, W. E. (2004). Acute hepatic sequestration in sickle cell disease. Journal of the National Medical Association, 96(9), 1235–1239. Olson, J. D., Brandt, J. T., Chandler, W. L., et al. (2007). Laboratory reporting of the international normalized ratio: Progress and problems. Archives of Pathology & Laboratory Medicine, 131(11), 1641–1647. Pagana, K. D., & Pagana, J. (2005). Mosby’s manual of diagnostic and laboratory tests (3rd ed.). St. Louis, MO: M ­ osby/Elsevier. Polin, R. A., & Spritzer, A. R. (2007). Fetal & neonatal secrets (2nd ed.). Philadelphia, PA: ­Mosby. Prasad, V. K., & Kurtzberg, J. (2008). Emerging trends in transplantation of inherited metabolic diseases. Bone marrow transplantation, 41(2), 99–108. Prescribing Reference. (2009, Summer). NPPR: Nurse Practitioner’s Prescribing Reference, 16(2). Rennie, J. M., & Roberton, N. R. C. (2002). A manual of neonatal intensive care (4th ed.). New York, NY: A ­ rnold. Shorr, A. K., Helman, D. L., Davies, D. B., & Nathan, S. D. (2004). Sarcoidosis, race, and ­short­term outcomes following lung transplantation. Chest, 125(3), 990–996. Skidmore-­Roth, L. (2004). Mosby’s 2004 nursing drug reference. St. Louis, MO: ­Mosby/Elsevier. Swanson, K., Dwyre, D. M., Krochmal, J. & Raife, T. J. (2006). ­Transfusion-­related acute lung injury (TRALI): Current clinical and pathophysiologic considerations. Lung, 184(3), 177–185.

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Hematology/Immunology  y  239 Taeusch, H. W., Ballard, R. A., & Gleason, C. A. (2005). Avery’s diseases of the newborn (8th ed.). Philadelphia, PA: ­Elsevier. Unkle, D. W. (2007). ­Heparin-­induced thrombocytopenia. Orthopaedic Nursing, 26(6), 383–387. Urden, L. D., Stacy, K. M., & Lough, M. E. (2007). Thelan’s critical care nursing: Diagnosis and management (5th ed.). St. Louis, MO: M ­ osby. Verklan, M. T., & Walden, M. (Ed.). (2004). Certification and core review for neonatal intensive care nursing (3rd ed.). St. Louis, MO: E ­ lsevier. Verklan, M. T,. & Walden, M. (Ed.). (2010). Certification and core review for neonatal intensive care nursing (4th ed.). St. Louis, MO: ­Elsevier. von ­Köckritz-­Blickwede, M., & Nizet, V. (2009). Innate immunity turned ­inside-­out: antimicrobial defense by phagocyte extracellular traps. Journal of Molecular Medicine, 87(8), 775–783. Ward, R. M., Lane, R. H., & Albertine, K. H. (2006). Basic and translational research in neonatal pharmacology. Journal of Perinatology, 26(S2), S8–S12. Young, T. E., & Magnum, B. (2009). Neofax 2009. Montvale, NJ: Thomson ­Reuters. Zhang, D., Zhang, F., Zhang, Y., et al. (2007). Erythropoietin enhances the angiogenic potency of autologous bone marrow stromal cells in a rat model of myocardial infarction. Cardiology, 108(4), 228–236.

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Section 9 Neurology

Questions 1. At what week of fetal development does the neural plate become the neural ­tube? A. 8th ­week B. 4th ­week C. 6th w ­ eek 2. The neural tube further develops into which ­structures? A. Peripheral nervous ­system B. Choroid plexus and cerebrospinal fluid ­system C. Brain, cerebellum, brain stem, spinal cord, and ­ventricles 3. Alpha fetal protein (AFP) screening of the mother’s serum potentially detects what percentage of neural tube ­defects? A. 80–90% B. 75–85% C. 60–75% 4. Which of the following best describes hydrocephalus in the ­neonate? A. It is a benign ­macrocrania B. Buildup of CSF secondary to overproduction, blockage, or absorption ­problems C. Multiple bulging ­fontanelles 5. Where is cerebrospinal fluid ­formed? A. In the lateral ­ventricles B. In the third ­ventricle C. In the fourth v ­ entricle 6. What is the most common congenital cause of hydrocephalus in the ­neonate? A. ­Dandy-­Walker ­syndrome B. Arteriovenous ­malformation C. Obstruction of the aqueduct of ­Sylvius 7. You are caring for a 28-week gestation infant at day 20 of life with hydrocephalus. The baby has had a ventriculoperitoneal (VP) shunt placed. Over the past few hours, you have noticed the baby has become increasingly lethargic. As the NICU nurse you know ­that A. The shunt may be ­occluded B. It is not unusual for babies with hydrocephalus to fatigue ­easily C. The shunt is functioning ­well

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8. Which of the following is the most common cause of acquired ­hydrocephalus? A. HIV ­infection B. Maternal rubella ­infection C. Bacterial ­meningitis 9. Which of the following are some of the common symptoms of hydrocephalus in the ­neonate? A. Macewen’s sign, fused ­fontanelles B. Macrocrania, rapid skull growth, failure to ­thrive C. Flat scalp veins, split ­sutures 10. A 32-week gestational age male infant with hydrocephalus received a ventriculoperitoneal shunt 2 days ago. On your initial assessment you find him febrile, tachycardic, and lethargic. You should anticipate orders for this patient to ­include A. CBC with differential, CRP, sed rate, ceftazidime, and cooling ­measures B. CSF cultures, acetaminophen, and ­azithromycin C. CT scan, CSF cultures, and ­ciprofloxacin 11. Where is the most common location for ­craniosynostosis? A. Coronal ­suture B. Lambdoidal ­suture C. Saggital ­suture 12. What is the significance of measuring an infant’s head ­circumference? A. There is no ­significance B. It is an indicator of brain growth and gives clues to problems such as ­hydrocephalus C. It allows for close examination of the skull and ­eyes 13. What is the most common form of seizure seen in preterm ­infants? A. ­Subtle B. ­Myoclonic C. ­Tonic 14. Which of the following etiologies of seizures in the neonate has the best ­long-­term ­prognosis? A. ­Hypoglycemia B. Congenital ­malformations C. ­A sphyxia 15. What is the most significant factor in reducing the occurrence of spina ­bifida? A. Prenatal folic ­acid B. Prenatal ­diet C. Prenatal ­multivitamins

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16. You are caring for a 36-week gestational age neonate with spina bifida with a meningocele. With this condition you would expect to ­see A. Dura mater and arachnoid tissue visible through the ­defect B. A visible defect of the vertebral ­arches C. A visible defect with protrusion of the meninges and spinal cord contained in a ­cyst 17. What other neural tube defects can occur with a folic acid deficiency in addition to spina ­bifida? A. Anencephaly and ­encephalocele B. ­Septo-­optic ­dysplasia C. Schizencephaly and Down ­syndrome 18. Syringomyelia is most often seen in conjunction with what other neurologic ­problem? A. It occurs as a unique ­entity B. Myoclonic ­seizures C. Chiari II ­malformation 19. Your 28-week gestational age male patient has craniosynostosis. What would you teach the parents to realistically expect for their ­child? A. Closed sutures can lead to increased ICP and a lower ­IQ B. The skull will grow to accommodate the ­brain C. This condition never affects the ­IQ 20. Your patient has just returned from endoscopic surgery to correct his saggital suture craniosynostosis. How should you position ­him? A. ­Supine B. ­Prone C. Right ­lateral 21. What basic assessments/actions should be done for an infant with hydrocephalus after a ventriculoperitoneal shunt ­placement? A. Frequent neuro checks, HOB elevated at 30 degrees, and head ­midline B. VS Q 15 minutes, HOB flat, and positioned on his or her left ­side C. Neuro checks hourly, HOB elevated at 30 degrees, and positioned on his or her right ­side 22. What nursing diagnoses should be addressed for a neonate after a ventriculoperitoneal shunt is p ­ laced? A. Increased ICP, potential for inadequate oxygenation, potential for infection, and ­pain B. Decreased ICP, potential for inadequate oxygenation, potential for infection, and ­pain C. Potential for infection, pain management, and potential for ­dehydration

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23. You are caring for a 27-week gestational age female with subtle seizures. What medications do you anticipate using for this ­patient? A. ­Carbamazepine B. ­Diazepam C. ­Lorazepam 24. Correct positioning is crucial when treating a newborn with a myelomeningocele. Which of the following positions would be appropriate for an infant with a ­myelomeningocele? A. Prone ­position B. Supine position to reduce the ­c yst C. Prone positioning, cover with normal ­saline-­soaked gauze, avoid fecal ­contamination 25. Which of the following actions may reduce jitteriness in a ­neonate? A. Medicate the ­baby B. Reduce the ­stimulation C. Play music or sing to the ­baby 26. What neonatal movements can mimic seizure ­activity? A. Apnea and myoclonic ­jerking B. Benign nocturnal myoclonus, jitteriness, and nonconvulsive ­apnea C. Tonic deviation of the eyes and paroxysmal ­laughing 27. Sudden, jerking movements of the limbs during sleep is the hallmark of which of the following types of neuromuscular ­actions? A. Benign nocturnal ­myoclonus B. Myoclonic ­seizures C. Focal clonic ­seizures 28. What is the most common cause of hearing loss in the United ­States? A. ­Syphilis B. Loud ­music C. Cytomegalovirus ­infection 29. How is cytomegalovirus spread to the ­neonate? A. By the genital tract contact during vaginal ­birth B. From a placental blood ­transfusion C. By the genital contact and ­breastmilk 30. You are caring for a 30-week gestational age infant who was born 5 hours ago. The infant has developed petechiae over his chest and arms. However, this infant is not thrombocytopenic. What is a possible cause of this ­condition? A. ­Hyperbilirubinemia B. CMV ­infection C. Hemolytic disease, early ­stage

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31. What are some of the common manifestations of CMV in the ­neonate? A. Microcephaly, hydrocephalus, and ­hepatosplenomegaly B. Heart defects, bone lesions, and ­jaundice C. Petechiae, glaucoma, and ­lymphadenopathy 32. In which socioeconomic strata are you most likely to find a mother with ­cytomegalovirus? A. A ­well-­educated, ­middle-­class, working professional ­primipara B. Lower socioeconomic female with a toddler in ­preschool C. A lower socioeconomic ­primipara 33. What is the drug of choice to treat active ­cytomegalovirus? A. ­Ganciclovir B. ­Valacyclovir C. ­Famciclovir 34. What is the expected outcome for an infant born with an acute, severe CMV ­infection? A. Severe liver disease, severe neurologic impairment, and possible ­death B. CHF, pneumonia, and hearing ­loss C. Mental retardation, CHF, and bone ­lesions 35. What does the acronym TORCH ­represent? A. Tularemia, other infection, retrovirus, chlamydia, and ­herpes B. Tuberculosis, other infection, rhinovirus, chicken pox, and herpes ­zoster C. Toxoplasmosis, other infection, rubella, CMV, and herpes ­simplex 36. The mother of a 36-week gestational age infant has tested positive for toxoplasmosis. What symptoms would you expect to see during your assessment of the ­baby? A. Hydrocephalus, CHF, and ­flaccidity B. Microcephaly, seizures, and ­hydrocephalus C. Seizures, GI mobility problems, and ­CHF 37. The infant you are caring for has no obvious symptoms of toxoplasmosis at birth. How would you teach the parents about the possibility of problems in this infant’s ­future? A. The baby will be fine since he was born ­asymptomatic B. 80% of infants infected with toxoplasmosis develop hearing or vision changes later in l­ ife C. Routine well baby checks are ­sufficient 38. What risk factors may increase the incidence of bacterial meningitis in the ­neonate? A. Prolonged rupture of the membranes, maternal chorioamnionitis, low socioeconomic ­status B. Male gender, mother’s age greater than 40 years, high birth ­weight C. Low birth weight, female gender, very young ­mother

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39. Which of the following signs is utilized to help diagnose ­hydrocephalus? A. Battle’s s­ ign B. Macewen’s ­sign C. Lhermitte’s ­sign 40. You are caring for a 35-week gestational age infant who has a poor traction when tested. As the NICU nurse, you s­ hould A. Do nothing as this is a normal response for this gestational ­age B. Tell the charge ­nurse C. Notify the neonatologist right ­away 41. Moro’s reflex is also known ­as A. The Stroop ­test B. Babinski ­reflex C. The startle ­reflex 42. The rooting reflex tests which of the cranial ­nerves? A. III—­Oculomotor B. IV—­Trochlear C. V—­Trigeminal 43. What is the expected response from an infant after eliciting the rooting ­reflex? A. Opens mouth, turns head away from stimulus, and grasps ­nipple B. Turn head toward the stimulus, opens mouth, and grasps ­nipple C. Opens mouth, keeps head midline, and grasps ­nipple 44. How can you elicit the sucking reflex for a 30-week gestational age ­infant? A. The infant eagerly takes the bottle every time it is ­offered B. Gently pinch the nose closed, and as the mouth opens, the tip of the tongue will rise to ­midline C. This reflex cannot be assessed in an infant less than 38 weeks ­gestation 45. Neural tube defects in the closure of the anterior portion of the neural tube results in what congenital ­problems? A. ­A nencephaly B. ­Myelocele C. ­Myelomeningocele 46. How do you elicit a palmar ­grasp? A. Touch the palmar surface of the infant’s hand from the radial side of the ­hand B. Touch the palmar surface of the infant’s hand from the ulnar side of the ­hand C. Tickle the palm along the lateral ulnar side of the ­hand 47. The plantar grasp is elicited by what m ­ ethod? A. Touch the plantar surface of the foot at the ­arch B. Touch the plantar surface of the foot at the ­heel C. Touch the plantar surface of the foot at the base of the ­toes

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48. Which three tests should be included when checking postural or truncal tone in an i­ nfant? A. Traction response, vertical suspension, and phasic ­tone B. Traction response, vertical suspension, and horizontal ­suspension C. Traction response, tonic neck reflex, and horizontal ­suspension 49. Which of the following conditions puts a 26-week gestational age infant at risk for periventricular ­leukomalacia? A. Apnea, patent ductus arteriosus, and ­infection B. Pneumothorax, apnea, and CMV ­infection C. Respiratory distress syndrome, pneumothorax, patent ductus arteriosus, and ­apnea 50. Which of the following is a commonly seen manifestation of periventricular leukomalacia in the premature i­ nfant? A. Abnormal tone power in the ­legs B. No manifestations are seen until the 3rd month of ­life C. Normal CT ­scans 51. How does periventricular hemorrhagic infarction differ from periventricular ­leukomalacia? A. Periventricular hemorrhagic infarction is a diffuse arterial bleed, whereas periventricular leukomalacia is a venous ­bleed B. Periventricular hemorrhagic infarction is a unilateral venous bleed, whereas periventricular leukomalacia is a more diffuse arterial ­bleed C. They are both seen with ­f ull-­term ­infants 52. In comparing periventricular hemorrhagic infarction with periventricular leukomalacia, which condition is associated more closely with gestational age and ­weight? A. Both conditions are equally associated with gestational age and ­weight B. Periventricular ­leukomalacia C. Periventricular hemorrhagic ­infarction 53. How soon after birth can a neonate born to an H ­ IV-­infected mother test positive for ­HIV? A. 1 ­month B. 2 d ­ ays C. 3 w ­ eeks 54. You have been caring for an 18 d ­ ay-­old term infant who has been feeding poorly all day. His mother had active herpes simplex at birth. Now the baby has developed a fever and bulging fontanelles. He begins having tremors, which progress to seizure activity. What could be the cause of the infant’s worsening ­condition? A. Febrile ­seizures B. HSV ­encephalitis C. HSV ­outbreak

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55. A 26-week gestational age female was born 4 hours ago of a mother with a compressed placenta. The infant was hypoxic for 7 minutes. What neurologic problems are to be expected with ­hypoxic-­ischemic ­encephalopathy? A. Decreased level of consciousness, apnea, and ­hypotonia B. Agitation, periods of apnea, and ­hyperreflexia C. Decreased level of consciousness, jitteriness, and ­hyperreflexia 56. An infant was hypoxic for 6 minutes immediately after birth. He had a 5-minute Apgar score of 2. The neonatal neurologist has rated the infant’s condition as severe ­hypoxic-­ischemic encephalopathy (HIE). What other neurologic injuries would you anticipate in this i­ nfant? A. Periventricular leukomalacia, and sterile ­meningitis B. No other neurologic conditions would be ­expected C. Periventricular hemorrhagic infarction and periventricular ­leukomalacia 57. A ­full-­term, 8-pound, 6-ounce infant who was delivered by traction has a flaccid left arm. What is the possible cause of this ­problem? A. Cervical spine ­injury B. Brachial plexus ­injury C. Epiphyseal fracture of the ­humerus 58. The ­full-­term infant you are caring for endured a difficult, prolonged delivery requiring repeated vacuum extraction attempts. You notice an expanding, soft, pliable mass on the top of the infant’s head. What is the probable cause of this ­phenomenon? A. Subgaleal ­hemorrhage B. Subdural ­hematoma C. Subarachnoid ­hemorrhage 59. Which of the following situations can lead to subgaleal ­hemorrhage? A. Forceps delivery and skull ­f racture B. Forceps delivery and coagulopathy at ­birth C. Forceps delivery and abrupt ­delivery 60. A ­full-­term infant born breech has extensive molding of the head. What type of neurovascular hemorrhage would you expect in this ­infant? A. Periventricular hemorrhagic ­infarction B. Infratentorial subdural ­hematoma C. Subarachnoid ­hemorrhage 61. What method is used for intracranial pressure (ICP) monitoring in the ­neonate? A. Intraventricular ­catheter B. Subdural ­bolt C. No direct intracranial monitoring is done on ­neonates

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62. How does caput succedaneum differ from ­cephalhematoma? A. Caput succedaneum is edema that crosses suture ­lines B. Cephalhematoma is bleeding over suture lines along the presenting ­part C. Caput succedaneum is bleeding over the parietal ­sutures 63. Which of the following birth injuries has a favorable ­outcome? A. Fracture of the distal epiphysis of the ­humerus B. Caput ­succedaneum C. Bilateral spinal cord ­injury 64. Which of the following conditions may lead to a birth ­injury? A. Breech presentation and C-­section B. A 7-pound, f­ ull-­term baby and prolonged ­labor C. Prematurity and cephalopelvic ­disproportion 65. A sympathetic response to a stimuli results ­in A. Heightened awareness, increased blood pressure, bronchial dilation, and increased ­glycogenosis B. Dilated pupils, bronchial relaxation, increased gastric motility, and normal urine ­output C. Vasodilatation, increased blood pressure, decreased gastric secretions, and pupils at 3 m ­ m 66. When should a lumbar puncture be done on a patient with increased intracranial ­pressure? A. Once the patient has had a CT or MRI ­scan B. Always, to check intracranial ­pressure C. ­Never 67. Which of the following decreases intracranial ­pressure? A. CO2 ­retention B. PaO2 less than 50 ­mmHg C. Increased cerebrospinal fluid ­absorption 68. Communicating hydrocephalus is caused by what ­mechanism? A. Closed head ­injury B. Subarachnoid ­hemorrhage C. Epidural ­bleed 69. Spinal reflexes ­indicate A. Functional upper motor ­neurons B. Nonfunctional lower motor ­neurons C. Functional lower motor ­neurons

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70. What does the pediatric Glasgow coma scale ­measure? A. Verbal response, orientation, and ­activity B. Eye opening, motor response, and verbal ­response C. Eye opening, orientation, and motor ­response 71. What is the most common bacteria causing meningococcal ­meningitis? A.  Streptococcus ­pneumoniae B.  Neisseria ­meningitidis C.  Staphylococcus ­aureus 72. Bilateral ascending muscle weakness is suggestive of which of the following ­illnesses? A. ­Guillain-­Barré ­syndrome B. Chronic fatigue ­syndrome C. ­Tetanus 73. Cerebrospinal fluid is formed in what ­location? A. Lateral ­ventricles B. Choroid ­plexus C. Subarachnoid ­space 74. Chris is a term infant admitted to the NICU for seizure activity after an uneventful delivery. As a NICU nurse, you should anticipate orders for which of the following laboratory t­ ests? A. CBC, lipid panel, and toxicology ­screen B. CBC, toxicology screen, and L ­ FTs C. CBC, CMP, sedimentation rate, CRP, RPR, and toxicology ­screen 75. Susan is very depressed. She is visiting her son, who is a 34-week gestation infant born with extreme hydrocephalus. The physician has just informed Susan that her son will probably die within the next few hours. Susan states, “I don’t want him to die alone in this place like my brother did.” Susan is exhibiting behavior appropriate for which ­Kubler-­Ross stage of g ­ rief? A. ­Denial B. ­Bargaining C. ­A nger 76. What is the frequency that acetaminophen can be safely administered to a 28-week gestational aged ­infant? A. Every 4 ­hours B. Every 8 ­hours C. Every 12 ­hours

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77. Mary, a 26-week gestational age female, has been given too much acetaminophen. Her serum acetaminophen level is 175 mcg/ml. What orders do you ­anticipate? A. ­N-­acetylcysteine ­infusion B. Intravenous fl ­ uids C. Chelating ­agents 78. You are to administer a single dose of chloral hydrate to a 30-week gestational age infant for sedation. What is the most common side effect of this drug in premature i­ nfants? A. CNS d ­ epression B. ­Bradycardia C. ­Seizures 79. What is the antagonist for f­ entanyl? A. Calcium ­chloride B. ­Midazolam C. ­Naloxone 80. What is the usual dose of fentanyl for ­sedation? A. 50–100 mg/kg intravenously every 2–4 ­hours B. 0.5–4 mcg/kg intravenously every 2–4 ­hours C. 5–10 mcg/kg intravenously every 2–4 ­hours 81. What are two common side effects of fentanyl ­administration? A. Excitability and ­ileus B. CNS and cardiac ­depression C. Cardiac and respiratory ­depression 82. Baby Laura is a 25-week gestational age female who had prenatal benzodiazepine exposure. She is lethargic with poor respiratory effort and poor muscle tone. You anticipate which of the following medications to be administered to this ­infant? A. ­Flumazenil B. ­Naloxone C. N-­acetylcysteine 83. What is the intravenous dosing for the administration of flumazenil for benzodiazepine ­overdose? A. 5–10 mcg/kg intravenously over 15 ­seconds B. 5–10 mg/kg intravenously in 20 cc normal saline over 3 ­hours C. 5–10 mg/kg intravenously in 100 cc normal saline over 24 ­hours 84. What is the primary indication for fosphenytoin ­administration? A. ­Hyperactivity B. ­Seizure C. ­Sedation

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85. What is the usual loading dose of fosphenytoin in a neonate with ­seizures? A. 1–2 m ­ g/kg B. 5–10 m ­ g/kg C. 15–20 ­mg/kg 86. Brad, a 32-week gestational age male with status epilepticus, is receiving fosphenytoin. In addition to monitoring this infant for further seizure activity, what other parameter should you closely ­monitor? A. Blood ­pressure B. Heart ­rate C. Respiratory ­rate 87. The 26-week gestational age female you are caring for has refractory seizures to phenobarbital and lorazepam. What other medication do you anticipate ­administering? A. ­Phenytoin B. ­Fosphenytoin C. ­Levetiracetam 88. What is the usual dose for levetiracetam in neonates with refractory ­seizures? A. 10 mg/kg every 24 hours orally or ­intravenously B. 12 mg/kg every 12 hours orally or ­intravenously C. 5 mcg/kg every 24 hours orally or ­intravenously 89. Grace, a ­full-­term infant with severe recurrent seizures, has not responded to any of the ­first-­line medications. The neurologist orders lidocaine. What is the loading dose for seizures for this ­infant? A. 5 mg/kg intravenously over 30 ­minutes B. 2 mg/kg intravenously over 10 ­minutes C. 2 mg/kg intravenously over 1 ­hour 90. The infant in the previous question is receiving lidocaine for severe recurrent seizures. The physician orders phenytoin infusion to run with the lidocaine. You know t­ hat A. They are compatible and may be infused ­simultaneously B. The combination is contraindicated due to cardiac ­effects C. It is acceptable to give via separate intravenous ­lines 91. You have been ordered to give 0.5 mg/Kg of lorazepam intravenously to a one day old infant. Your first response should be which of the ­following? A. Refuse to give the medication as it is an incorrect ­dose B. Give the lorazepam as ­ordered C. Tell the physician he must give the ­dose

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92. Baby Gene, a 27-week gestational age male born to a mother addicted to heroin, is now going through withdrawal symptoms. Which of the following medications do you anticipate a ­ dministering? A. ­Lorazepam B. ­Naloxone C. ­Methadone 93. What monitoring, if any, should be done for an infant receiving ­methadone? A. Respiratory, cardiac, and gastrointestinal ­monitoring B. Respiratory, cardiac, and CNS ­monitoring C. Respiratory, cardiac, and genitourinary ­monitoring 94. What is the recommended dose range for ­midazolam? A. 0.2–0.5 mg/kg given IV ­push B. 0.05–0.15 mg/kg IV over 5 ­minutes C. 1–2 mcg/kg given IV ­push 95. You are caring for a 34-week gestational age infant born to a mother with myasthenia gravis. The infant has been diagnosed with neonatal myasthenia gravis. Considering the diagnosis, what medication do you anticipate ­giving? A. ­Neostigmine B. ­Naloxone C. ­Phenobarbital 96. What is the usual dose of neostigmine for an infant with neonatal myasthenia ­gravis? A. 0.1 mg IM 30 minutes before ­feeding B. 0.1 mcg/kg IM 30 minutes before f­ eeding C. 0.5 mg IM 15 minutes before ­feeding 97. Pentobarbital belongs to what class of ­medications? A. ­Benzodiazepines B. Narcotic ­antagonists C. ­Short-­acting ­barbiturates 98. Which of the following conditions are common side effects of ­pentobarbital? A. Respiratory depression, ­Stevens-­Johnson syndrome, and ­agitation B. Respirator depression, bradycardia, and ­hypertension C. Respiratory depression, tachycardia, and ­hypertension 99. Baby Doe, estimated gestational age of 28-weeks, was born by emergency ­C-­section after his mother died in a motor vehicle accident. He was severely hypoxic at birth. The neurologist orders phenobarbital to be given immediately. ­Why? A. To sedate the baby and improve cardiac ­output B. To prevent seizures and improve overall ­outcome C. For the prevention of seizures ­only

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100. What is the usual loading dose of phenobarbital for seizure activity in a ­neonate? A. 10 mg/kg IV over 30 ­minutes B. 20 mcg/kg IV ­push C. 20 mg/kg IV given over 10–15 ­minutes 101. An infant with an H ­ IV-­positive mother has been receiving phenobarbital for his seizures. His last dose of phenobarbital was given 12 hours ago. During your initial assessment, you note areas of erythema and what look like blisters on the baby’s skin. What is the probable cause of his ­condition? A. ­Stevens-­Johnson ­syndrome B. ­Urticaria C. ­Second-­degree burns from the bili ­light 102. What is the ­first-­line drug given for ­seizures? A. ­Pentobarbital B. ­Phenytoin C. ­Phenobarbital 103. What is the loading dose for phenytoin for ­seizures? A. 15–20 mg/kg given IV over 30–45 ­minutes B. 15–20 mcg/kg given IV over 10 ­hours C. 4–8 mg/kg given IV ­push 104. The physician is planning a bedside surgical procedure for Baby Alice. He orders rocuronium to be given prior to the procedure. Which of the following is the best ­response? A. Refuse to give the d ­ rug B. Ask what analgesic he would like given ­first C. Give the drug as ­ordered 105. What is the correct dose range for ­rocuronium? A. 0.3–0.6 mg/kg given IV over 5–10 ­seconds B. 3–6 mg/kg given IV over 5–10 ­seconds C. 5–10 mcg/kg given IV over 1 ­minute 106. The type of paralysis that involves swelling in the shoulder and a possible fractured clavicle i­ s A. Duchenne’s ­paralysis B. Erb’s ­palsy C. Klumpke’s ­paralysis 107. Klumpke’s paralysis is a type of brachial nerve paralysis. Which cranial nerves would be affected with this ­disorder? A. Cervical nerve VI to thoracic nerve ­I B. Cervical nerve IV to cervical nerve ­VI C. Thoracic nerve II to thoracic nerve ­IV

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108. Skull fractures in neonates may be due ­to A. Extreme ­molding B. Fragile suture ­lines C. Depressed ­fontanelles 109. Linear fractures in ­neonates A. Are very ­rare B. Result in separation of adjacent ­sutures C. Are a ­ symptomatic 110. A depressed skull fracture in a ­neonate A. Crosses suture ­lines B. Always causes separation of adjacent ­sutures C. Often occurs in the right parietal ­bone 111. Which of the following statements is true regarding phrenic nerve ­paralysis? A. Phrenic nerve paralysis always occurs with brachial plexus ­injury B. Phrenic nerve palsy is diaphragmatic ­paralysis C. Phrenic nerve palsy results from stretched nerve ­sheaths 112. An elevated hemidiaphragm and paradoxical movement of the affected side with breathing are cardinal signs o ­f A. Flail ­chest B. Hiatal ­hernia C. Phrenic nerve ­paralysis 113. A characteristic of traumatic facial nerve palsy ­is A. The defects are ­permanent B. Disruption of the nerve ­fiber C. Hemorrhage and edema into the nerve ­sheath 114. Maximal vasodilation of the cerebral vasculature occurs as a response t­ o A. ­Acidemia B. ­Hypercalcemia C. ­Acidosis 115. An increase in calcium ions will c ­ ause A. A decreased cerebral blood ­flow B. An increased ­SVR C. A reciprocal change in blood glucose ­levels 116. Baby S. suffered a cephalohematoma during birth. Which of the following statements is true regarding c ­ ephalohematomas? A. The injury is usually ­bilateral B. The injury site feels s­ oft C. The cephalohematoma does not ­transilluminate

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117. A birth injury in which the affected arm is abducted and internally rotated is known a ­s A. A palmar ­rotation B. Erb’s ­palsy C. McEllen’s ­palsy 118. A primary subarachnoid hemorrhage ­is A. Bleeding into the ­CSF-­filled space between the arachnoid and pial ­membranes B. Bleeding secondary to an extension of a subdural ­hemorrhage C. Bleeding from an arterial ­origin 119. Seizures in the newborn may be caused by a metabolic encephalopathy such ­as A. ­Hypercalcemia B. Decreased adenosine triphosphate p ­ roduction C. ­Hypoammonemia 120. Why is it important to stop seizure activity in status e ­ pilepticus? A. Oxygen depletion due to an impeded ­airway B. Increased risk of cerebrovascular ­accident C. Continued seizure activity causes lactic acidosis and cerebral ­edema 121. An infant with severe brain damage from hypoxic ­ischemic-­encephalopathy (HIE) may present with which of the following anatomic ­findings? A. Cerebral ­edema B. Cord ­compression C. Subgaleal ­hemorrhage 122. An infant who was born at 27-weeks gestation has hypertonic lower extremities with increased deep tendon reflexes. Facial and upper extremity movement is normal. This infant probably ­has A. ­Diplegia B. ­Hemiplegia C. ­Athetosis 123. Polyhydramnios may be associated with what other condition in an ­infant? A. Hypoplastic ­lungs B. ­A nencephaly C. ­Hypovolemia 124. Café au lait spots may be associated ­with A. ­Neurofibromatosis B. Chromosome ­abnormalities C. Neural tube ­defects

This concludes the Neurology ­questions.

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Neurology ­Answers 1. Correct answer: ­B The neural tube development begins with the neural plate developing in the 3rd week. The plate then folds on itself and fuses to become the neural tube at about the 4th gestational ­week. 2. Correct answer: ­C The neural tube develops ­rostro-­caudally (head to tail) to form the brain, cerebellum, brainstem, spinal cord, and ­ventricles. 3. Correct answer: ­A AFP screening done at 15–22 weeks into the pregnancy can detect up to 80-90% of all neural tube defects. This test can detect 80% of spina bifida aperta and 90% of ­anencephaly. 4. Correct answer: ­B Hydrocephalus can be the result of any of the three problems of overproduction, blockage, or malabsorption as a single entity or in combination. The symptoms include dilated ventricles on CT or MRI. An infant with benign macrocrania may have hydrocephalus in addition to other symptoms; it is not diagnostic of hydrocephalus. Bulging fontanelles and widening sutures are symptoms of hydrocephalus and not the ­definition. 5. Correct answer: ­A Cerebrospinal fluid (CSF) is primarily produced by the choroid plexus of the lateral ventricles. The CSF flows through the foramen of Monro, into the third ventricle, through the aqueduct of Sylvius into the fourth ventricle. The fluid then travels through the foramina of Luschka and Magendie, where it bathes the spinal cord and brain before returning to the ventricular cisterns and subarachnoid space before being reabsorbed by the arachnoid ­villi. 6. Correct answer: ­C An obstruction of the aqueduct of Sylvius is the primary cause of congenital hydrocephalus in the neonate. Chiari II malformation and tumor can lead to the obstruction of the aqueduct of Sylvius but are not the primary cause. Arteriovenous malformation (AVM) causes intracerebral bleeds and is the leading cause of stroke in ­children. 7. Correct answer: ­A This is a medical emergency. The neurosurgeon needs to be notified immediately. If any infant with a ventriculoperitoneal shunt becomes lethargic, and this means the shunt is not working for some reason, and there is an increase in the ­ICP. 8. Correct answer: ­C Bacterial meningitis of many types can damage the CSF system, leading to an acquired hydrocephalus. While HIV infection can be spread to the neonate, it is not as common as the bacterial meningitis. Idiopathic causes of hydrocephalus are ­congenital in nature. Maternal rubella infections cause deafness, cataracts, and cardiac ­diseases.

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9. Correct answer: ­B There are many symptoms associated with hydrocephalus. The most common include rapid skull growth ( . 2 cm/week), macrocrania, bulging fontanelles, split cranial sutures, apnea, and bradycardia. In addition, the infant may have distended scalp veins, failure to thrive, vomiting, drowsiness, Macewen’s sign, head bobbing, and transcranial ­illumination. 10. Correct answer: ­A Following the shunt procedure, the infant appears to have an infection. A CBC with differential, CRP, sed rate, ceftazidime, and cooling measures are appropriate orders for this ­patient. Establishing that there is an infection is important. Antibiotics such as ceftazidime are used due to their ­broad-­spectrum coverage. Cooling measures and acetaminophen are important to reduce the fever and therefore reduce the risk of febrile seizures. The other two options suggest fluoroquinolone antibiotics, which have a narrow antibacterial spectrum and are not appropriate for this ­infant. 11. Correct answer: ­C Approximately 50% of craniosynostosis occurs along the saggital suture. Craniosynostosis, the premature closure of one or more cranial sutures, occurs at a much lower rate along the other sutures with the lambdoidal suture being a rare ­occurrence. 12. Correct answer: ­B Measuring head circumference allows for a quick assessment of brain growth. It is also an indicator of problems such as hydrocephalus and ­craniosynostosis. 13. Correct answer: ­C Tonic seizures are the most common in preterm infants and are generally due to trauma at birth or metabolic disorders. About 25% of infants with tonic seizures will have ­long-­term sequelae. Myoclonic seizures may be mistaken for jitteriness in the ­infant. 14. Correct answer: ­A A seizure caused by hypoglycemia has the best prognosis especially if diagnosed early. It still has significant risk for complications and even death if not treated early. CNS infections have a 70% risk of complications or death. Infants with seizures due to asphyxia have a 50% chance of a poor ­outcome. 15. Correct answer: ­A The most significant factor in reducing the occurrence of spina bifida is prenatal folic ­acid. The RDA for folic acid is 400 mcg daily for those 14 years and older, but increases to 600–800 mcg daily for pregnancy. For women who have had any family history of neural tube defects, it is recommended they take up to 4000 mcg daily. Folic acid should be started at least 1 month prior to pregnancy and continue through the first trimester. Folic acid is now found in a lot of baked goods but is also found in leafy vegetables, legumes, fruits, and ­seeds.

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16. Correct answer: ­A With a meningocele, the dura mater and arachnoid are visible through the defect. Answer A could be spina bifida occulta. Answer B is spina bifida aperta. Answer C is a myelomeningocele, the most severe form of spina ­bifida. 17. Correct answer: ­A Anencephaly is a lack of normal brain tissue in the skull. An encephalocele is the protrusion of brain tissue through the skull. Both of these conditions are neural tube defects related to folic acid ­deficiency. 18. Correct answer: ­C Syringomyelia is most often seen with Chiari II malformation. It creates cysts called syrinx that grow to produce long cavities within the spinal cord. These cavities damage the afferent spinal cord, causing interruption of nerve conduction from the brain to the extremities resulting in weakness and pain. It is best identified on an ­MRI. 19. Correct answer: ­A There are many problems associated with craniosynostosis, the premature closure of the cranial sutures. Examples of these problems include increased ICP, developmental delays, seizures, blindness, and lower IQ. Causes can be genetic or metabolic, such as hyperthyroid or rickets. Craniosynostosis may also be seen with microcephaly or ­hydrocephalus. 20. Correct answer: ­A For the correction of a saggital suture craniosynostosis, supine or ­back-­lying is the position of choice until a helmet is available. The other positions could interfere with the surgical site and may cause the infant ­pain. 21. Correct answer: ­A Frequent neuro checks are strongly recommended. The HOB elevated at 30 degrees promotes drainage of CSF, and the head midline maintains optimal CSF drainage. It is imperative to notify the neurosurgeon at once if there is a change in the neuro signs, especially if the baby becomes ­lethargic. 22. Correct answer: ­A The four nursing diagnoses in option A, increased ICP, potential for inadequate oxygenation, potential for infection, and pain are the most appropriate for a baby with a new ventriculoperitoneal shunt. There is at least one inappropriate option in the other ­answers. 23. Correct answer: ­C Lorazepam is the drug of choice for neonatal subtle seizures. Subtle seizures can mimic normal movements of the infant. Some of the common movements seen with this condition include apnea, tonic deviation of the eyes, repetitive eyelid fluttering, drooling, chewing, sucking, swimming arm movements, pedaling of legs, and paroxysmal laughing. Dilantin and phenobarbital may also be used to treat subtle seizures in this infant. Carbamazepine is not indicated for subtle seizures. Diazepam is used for status ­epilepticus.

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24. Correct answer: ­C Myelomeningocele is the most severe form of spina bifida. The defects occur in the lumbar sacral area and develop in the first month of gestation. The clinical features of a myelomeningocele include a protrusion of meninges and spinal cord and cerebrospinal fluid contained in a sac. Hydrocephalus and paralysis, including loss of bowel and bladder function, are common manifestations of myelomeningocele. It is imperative to keep pressure off this area, keep it moist, and avoid infection. Other nursing actions include daily measuring of head circumference, careful I&O, and providing emotional support of the ­family. 25. Correct answer: ­B Reducing as much stimulation as possible should reduce the jitteriness in a neonate. If reducing stimulation does not reduce the jitteriness, seizure activity should be considered. This may not hold true for a baby born to a mother with an addiction to drugs such as marijuana, cocaine, or methamphetamines. Jitteriness is an excessive response to minimal stimulus and can be seen with a vigorous Moro’s reflex. Jitteriness may also occur ­spontaneously. 26. Correct answer: ­B Neonatal movements that can mimic seizure activity are benign nocturnal myoclonus, jitteriness, and nonconvulsive apnea. All these movements are generally considered benign. All the other movements are indicators of seizures in the ­neonate. 27. Correct answer: ­A Sudden, jerking movements of the limbs during sleep is the hallmark of benign nocturnal myoclonus. This is a benign condition easily confused with seizures. There is no treatment indicated, and antiseizure medications may increase the frequency of the benign nocturnal movements. The EEG for this infant will be ­normal. 28. Correct answer: ­C Cytomegalovirus (CMV) is the leading cause of hearing loss in the United States. CMV is a beta herpes virus that can cause a myriad of neurologic problems for the neonate. CMV is the most common congenital infection in humans and it has the most serious ­sequelae. 29. Correct answer: ­C CMV is spread to the infant via the genital tract and breastmilk. Transmission rates are the greatest if this is the mother’s initial infection or she is having an active ­recurrence. 30. Correct answer: ­B CMV depresses the bone marrow leading to the petechiae or purpura. This condition usually appears within the first few hours after birth and lasts for a few days. It may or may not be associated with ­thrombocytopenia. 31. Correct answer: ­A CMV has many manifestations including hepatosplenomegaly, hydrocephalus, petechiae, purpura, organomegaly, jaundice, and microcephaly. Additional defects include ocular defects, intrauterine growth retardation, dental defects, and hearing ­loss.

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32. Correct answer: ­B CMV has a high rate of transmission among mothers with children in preschool/daycare, especially those in lower socioeconomic status. Although no one is immune to CMV, good hygiene is the best way to decrease its ­spread. 33. Correct answer: ­A Ganciclovir is the optimal antiviral for active CMV. The patient must be monitored for severe neutropenia and hepatorenal toxicity. This medication helps reduce hearing loss associated with CMV, but brain damage may already be considerable at ­birth. 34. Correct answer: ­A An acute, severe CMV infection has a 30% death rate. Even treatment with ganciclovir, there may be severe neurologic defects as the damage may be complete prior to ­birth. 35. Correct answer: ­C TORCH is a collection of similar clinical conditions seen with HIV infections. Toxoplasmosis is a protozoan infection passed from the mother to the infant. Other infections include hepatitis B, syphilis, ­varicella-­zoster virus, HIV, and parvovirus B19. The complex may also be written, “TORCHES,” and includes syphilis. Blood, urine, and CFS are tested, and the infant is considered positive if the IgM for one or more of the aforementioned infections are present in a symptomatic baby. Symptoms include chorioretinitis, microcephaly, hydrocephalus, small for gestational age, petechial rash, jaundice, hepatosplenomegaly, mental retardation, seizures, autism, and hearing ­loss. 36. Correct answer: ­B There are several neurologic symptoms associated with toxoplasmosis including chorioretinitis, microcephaly, hydrocephalus, intracranial calcifications, abnormal spinal fluid, seizures, hearing loss, visual impairment, spasticities, palsy, and mental ­retardation. 37. Correct answer: ­B Unless the mother was treated early in her pregnancy, the baby is at great risk for hearing and visual changes. There is also a 40% chance of neurologic abnormalities such as seizures or palsy. The most common problem is chorioretinitis, and the baby will need eye exams every 3–4 ­months. 38. Correct answer: ­A Risk factors that may increase the incidence of meningitis include low birth weight, prolonged rupture of membranes ( . 18 hours), and maternal chorioamnionitis. Other factors include low socioeconomic status, male gender, invasive monitoring, and invasive ­resuscitation. 39. Correct answer: ­B Macewen’s sign is diagnostic for hydrocephalus. It can be elicited by tapping the junction of the frontal, parietal, and temporal lobes. A strong resonant sound is produced with hydrocephalus and is also seen in brain abscesses. Battle’s sign is ecchymosis behind the ear over the mastoid area seen with a basilar skull fracture. Lhermitte’s sign is a sudden neck pain produced with flexing the neck.

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40. Correct answer: ­C The neonatologist needs to know this finding immediately as it could indicate a spinal cord lesion. A normal traction test result is when the baby is gently lifted by the wrists, the baby lifts the head parallel to the body and all the joints ­flex. 41. Correct answer: ­C Moro’s reflex is also known as the startle reflex. This reflex can be elicited by giving the baby the sensation of falling. The normal response causes extension then flexion of the arms and opening of the hands. Then the arms are adducted and flex over the body and the fists close. There may be some extension of the legs. This response is seen in infants of greater than 30 weeks gestation. The Stroop test is a neuropsychologic exam that measures mental acuity. A Babinski reflex test, also called the plantar reflex test, is a neurologic exam testing the brain and spinal cord for ­lesions. 42. Correct answer: ­C Cranial nerve V, the trigeminal nerve, is responsible for the rooting reflex. This reflex is elicited by touching the perioral area; the baby will turn the head toward the stimulus, open the mouth and grasp onto the object, stimulating the reflex. It can be seen as early as 28 weeks gestation but is weak and slow. Cranial nerves III, IV, and VI (oculomotor, trochlear, and abducens) deal with the movement of the ­eye. 43. Correct answer: ­B The expected response from an infant after eliciting the rooting reflex is the infant should turn his or her head toward the stimulus, open his or her mouth, and grasp the nipple in the mouth. It can be seen as early as 28 weeks gestation but is weak and slow. It is normally strong by 36 weeks ­gestation. 44. Correct answer: ­B The sucking reflex shows that the infant can coordinate sucking and swallowing to prevent aspiration. In the expected response, the infant will open the mouth, then the tip of the tongue rises to ­midline. 45. Correct answer: ­A The anterior portion of the neural tube deals with the formation of the brain, cerebellum, and brain stem. Closure defects results in anencephaly. The posterior portion of the neural tube becomes the spinal cord. Defects in the closure of the posterior neural tube lead to spina ­bifida. 46. Correct answer: ­B The infant’s head should be midline then you touch the palmar surface of his hand from the ulnar side (opposite the thumb) of the hand. There should be a strong grasp that tightens as you attempt to withdraw your ­finger. 47. Correct answer: ­C The plantar grasp is a primitive reflex seen in infants up to 8 months of age. The toes turn downward when the foot is touched at the base of the toes. This is not the same as the plantar reflex also known as the Babinski ­reflex.

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48. Correct answer: ­B This is a ­three-­section exam done on infants greater than 33 weeks gestation. The traction test is the most sensitive of the three tests. To evoke the grasp reflex, grasp the infant’s wrists and gently pull her to a sitting position. There will be a slight head lag. The vertical suspension test is done by placing the hands under the axilla, grasping the thorax, and lifting the infant straight up while she is facing the examiner. The head should be erect and midline, the limbs will flex, and the eyes will open. For the horizontal suspension test, the infant is lifted parallel with the bed with the hands around the thorax. The head should rise intermittently and the limbs ­flex. 49. Correct answer: ­C Four cardiorespiratory problems are most commonly associated with periventricular leukomalacia (apnea, PDA, pneumothorax, RDS). The ­hypoxic-­ischemic injury caused by these problems is seen in the vulnerable areas of the white matter around the lateral ventricles and the foramen of Monro. Because the white matter in myelinated nerve tissue carries messages to the body, there can be severe motor problems, delayed mental development, hearing and vision deficits, and coordination problems. There is no treatment for PVL, only supportive care. The prognosis depends on the severity of the brain ­damage. 50. Correct answer: ­A Abnormal tone power to the legs is common with periventricular leukomalacia. This occurs because the motor areas are the closest to the heavily damaged regions of the brain and they rely on myelinated nerves to carry impulses to the muscles. Hence, the muscle tone will be ­affected. 51. Correct answer: ­B Periventricular hemorrhagic infarction (PHI) is a unilateral venous bleed seen in infants weighing less than 700 grams. PHI is distinguishable from periventricular leukomalacia (PVL) on CT or MRI scan because of the unilateral nature if this hemorrhage. The PVL is not limited to one hemisphere or side of the ­brain. 52. Correct answer: ­C Periventricular hemorrhagic infarction (PHI) is seen more often with the earlier gestational age and lower birth weight, especially in those infants weighing less than 700 grams. The PHI infant is also more likely to develop ­hydrocephalus. 53. Correct answer: ­B Infants born to ­HIV-­infected mothers can convert within 48 hours. Tests must be repeated in about 1 month. Neonates infected with HIV can have a myriad of neurologic problems such as microcephaly, HIV encephalopathy, and motor deficits. Children who are treated early with the latest antiviral medications have the best ­prognosis. 54. Correct answer: ­B Herpes simplex (HSV) encephalitis is characterized by poor feeding, fever or temperature instability, seizures, lethargy, tremor, bulging fontanelles, and motor tract signs. Most of the ­HSV-­infected infants will have herpetic vesicles on inspection. Mortality for HSV

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encephalopathy can be as high as 50%. Of those who survive, many may have learning disabilities, porencephalic cysts, movement disorders, and blindness. Porencephalic cysts are cysts or cavities that develop after a brain injury, in this case ­encephalitis. 55. Correct answer: ­A There are many sequelae to asphyxia at birth. The early signs occur for about the first 12 hours and include poor muscle tone, periodic apnea, and seizures. The severity and prognosis of the ­hypoxic-­ischemic encephalopathy depends on the severity of the ­hypoxic-­ischemic event. The electroencephalogram (EEG) will be very abnormal. CT scans may show tissue ischemic changes when done sequentially. This condition greatly increases the risk of cerebral palsy and possible mental ­retardation. 56. Correct answer: ­C Periventricular hemorrhagic infarction (PHI) and periventricular leukomalacia (PVL) can be seen with ­hypoxic-­ischemic encephalopathy, especially in preterm infants. An infant with severe HIE and either PVL or PHI has an extremely poor ­prognosis. 57. Correct answer: ­B This is a classic injury of the brachial plexus. There are many injuries that can occur during the birthing process. Injury to the cervical spine at the C5–6 nerve roots is the most common cause of a brachial plexus injury. Symptoms include adduction of the limb, internal rotation, extension and pronation of the elbow, and flexion of the wrist. If the C7–8 nerve roots are injured, the hand may be flaccid. Brachial plexus injuries generally resolve spontaneously in a few weeks up to 3 months. Splinting and physical therapy are used to maintain function of the ­arm. 58. Correct answer: ­A The mechanism of this injury is often seen with subgaleal hemorrhage, especially with the prolonged labor and vacuum extraction attempts. Subgaleal hemorrhage occurs in the potential space between the aponeurosis and the skull. Whenever there is a prolonged labor, vacuum extraction, forceps use, or a coagulopathy, a subgaleal hemorrhage can occur. This is a medical emergency requiring blood transfusion, frequent coagulation lab tests, and vitamin K administration. The infant may develop hemorrhagic shock. The hemorrhage generally resolves spontaneously in a few ­weeks. 59. Correct answer: ­B Several situations can lead to subgaleal hemorrhage, including forceps delivery, prolonged labor, vacuum delivery, and coagulopathies. These traumatic events cause bleeding by the veins between the aponeurosis and the skull. What appears to be a small bump on the scalp can hold as much as 250 ml of blood. The subgaleal hemorrhage is a soft mass with the blood moving in a wavelike manner. Expect to monitor the infant for hypotensive shock, anemia, and coagulopathies. Whole blood may be administered. The hemorrhage usually resolves over a few weeks. In addition to anemia, hypotensive shock, and coagulopathies, a subgaleal hemorrhage can lead to the infant’s death. Skull fractures rarely occur with this ­condition.

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60. Correct answer: ­B A ­f ull-­term infant born breech has extensive molding of the head. Infratentorial subdural hematoma can occur in this patient due to the shearing forces associated with a breech presentation. The symptoms might not be seen for as long as 12 hours. A change in the rate and depth of respirations occurs first. Later the baby’s cry becomes high pitched or hoarse followed by decreased feeding and sucking. The fontanelles become firm and bulging due to the increased intracranial pressure (ICP). 61. Correct answer: ­C There is no generally used monitoring of intracranial pressure in the neonate. Most neurologists utilize the fontanelles and neurologic assessment as a guide of the intracranial ­pressure. 62. Correct answer: ­A Caput succedaneum is edema under the scalp as it crosses the suture lines and is seen with prolonged labor or early rupture of the membranes. Cephalhematoma is bleeding under the aponeurosis and may occur with a skull fracture. Both conditions are considered benign and resolve spontaneously over days to weeks without ­treatment. 63. Correct answer: ­B All the options are birth injuries; however, caput succedaneum is a ­self-­limiting injury that resolves spontaneously over a few days and leaves no residual. A fracture of the distal epiphysis of the humerus, or any other long bone, can lead to deformity of that limb. Bilateral spinal cord injury has a very poor prognosis for the infant as up to 50% die of complications. A subgaleal hemorrhage can cause shock and even death if not treated ­quickly. 64. Correct answer: ­C Some of the most common causes of birth injuries are weight over 8 pounds, 13 ounces; less than 37 weeks gestational age; cephalopelvic disproportion; difficult or prolonged labor; and abnormal presentation. While a birth injury can occur with a ­C-­section, it is a much less common ­occurrence. 65. Correct answer: ­A There are many responses to sympathetic stimuli as the body prepares for flight, fright, or fight. Responses include heightened awareness, bronchial dilation, increased glycogenosis, dilated pupils for increased visual acuity, increased heart rate, increased myocardial contractility, increased blood pressure, increased respiratory rate, decreased gastric motility, decreased gastric secretion, decreased urine output, decreased insulin production, and decreased renal blood ­flow. 66. Correct answer: ­C Lumbar punctures in a patient with increased intracranial pressure can lead to herniation of the tentorium or brainstem, so they should not be ­performed. 67. Correct answer: ­C Cerebrospinal fluid absorbed at an increased rate decreases intracranial pressure. The other answers contribute to increased intracranial ­pressure.

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68. Correct answer: ­B Communicating hydrocephalus is caused when the blood cells from a subarachnoid hemorrhage block the arachnoid villi from reabsorbing the cerebrospinal fluid. This condition may be transient. If the condition is permanent, a ventriculoperitoneal (VP) shunt will be ­placed. 69. Correct answer: ­C The knee jerk, also known as the patellar reflex, is an example of a functional lower motor neuron. These reflexes are also known as deep tendon reflexes. The nerve impulse makes an arc from the tendon to the sensory portion of the spinal cord to the motor root and back to the patella causing extension of the lower ­leg. 70. Correct Answer: ­B The pediatric Glasgow coma scale measures eye opening, motor response, and verbal response. It rates each with a total scale from 3 to 15 with 15 being a fully responsive patient. A score of 6–7 is comatose level. The longer that the patient remains in the lower score ranges, the worse the projected ­outcome. 71. Correct answer: ­B Neisseria meningitidis is the causative agent for meningococcal meningitis. Streptococcus pneumoniae causes pneumococcal meningitis. Haemophilus influenzae causes Haemophilus meningitis. Staphylococcus aureus is not likely to cause meningitis but can cause infection in the brain with a communicating ­opening. 72. Correct answer: ­A Guillain-­Barré syndrome is a demyelinating autoimmune process affecting the spinal and cranial nerves. It is seen after viral infections, is usually ascending, and is extremely rare in ­neonates. 73. Correct answer: ­B Cerebrospinal fluid is formed by the choroid plexus of the third ventricle. The arachnoid villi, projections from the subarachnoid space, reabsorb the cerebrospinal ­fluid. 74. Correct answer: ­C CBC, CMP, sedimentation rate, CRP, RPR, and toxicology screen are the tests that need to be performed to establish the cause of Chris’ seizures. Seizures can be caused by illness, infection, maternal overdose on drugs or alcohol, tertiary syphilis, dehydration, electrolyte imbalance, and cardiac ­arrhythmias. 75. Correct answer: ­C Susan is angry at her son’s diagnosis and afraid he will suffer the same fate as her brother. The five stages of grief identified by ­Kubler-­Ross in 1969 are denial, anger, bargaining, depression, and acceptance. A person can move through the stages in any order and can revisit a stage at any time. As the nurse, you should try to encourage Susan to express her feelings. The physician and chaplain should also be notified, and Susan should receive grief counseling. An unknown factor here is the history of Susan’s brother and whether he died in a NICU or some other area of the ­hospital.

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76. Correct answer: ­C The safe frequency for administration of acetaminophen to a 28-week gestational age infant is every 12 hours. More frequent dosing leads to toxicity, a potentially lethal ­condition. 77. Correct answer: ­A N-­acetylcysteine (Mucomyst) is given at a dose of 150 mg/kg in D5W or 0.45% normal saline intravenously over 60 minutes. The next dose is 50 mg/kg ­N-­acetylcysteine in the same solutions over 4 hours. The third dose is 100 mg ­N-­acetylcysteine over 16 hours. The infusions are continued until the hepatic function improves and acetaminophen levels are not ­detectable. 78. Correct answer: ­B Bradycardic episodes may occur for up to 24 hours after administration of chloral hydrate to a premature infant. Other potential side effects include agitation, gastrointestinal effects, plus depression of the cardiac, CNS, and respiratory systems may occur with repeat ­doses. 79. Correct answer: ­C Naloxone (Narcan) is an effective narcotic antagonist used to reverse the CNS depression caused by ­narcotics. 80. Correct answer: ­B 0.5–4 mg/kg is administered slowly intravenously and may be repeated every 24 hours when used for sedation. Monitor closely for CNS and respiratory ­depression. 81. Correct answer: ­C Cardiac and respiratory depression are two of the most common side effects of fentanyl administration. Also monitor for abdominal distention, muscle rigidity, anaphylaxis, seizures, and urinary ­retention. 82. Correct answer: ­A Flumazenil is the reversal agent for benzodiazepines. Monitor the infant closely for seizure ­activity. 83. Correct answer: ­A In a benzodiazepine overdose, the correct intravenous push dose would be 5–10 ­mcg­/­kg given over 15 seconds. The dose may be repeated every 45 seconds until the patient awakens. The patient must be closely monitored for seizure activity. A patent IV is essential to prevent local pain and irritation after ­administration. 84. Correct answer: ­B Fosphenytoin is chemically equivalent to phenytoin (Dilantin) at a 1:1 ratio. Fosphenytoin is used for status epilepticus and seizure disorders especially in phenobarbital ­resistance.

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85. Correct answer: ­C Give 15–20 mg/kg once in 24 hours as a loading dose of fosphenytoin. Lorazepam may be added for synergistic ­effect. 86. Correct answer: ­A Blood pressure needs to be closely monitored for hypotension during the infusion. Phenytoin (not fosphenytoin) levels should be monitored as fosphenytoin is the prodrug of choice and is rapidly converted to ­phenytoin. 87. Correct answer: ­C Levetiracetam is a ­second-­line drug for seizures that are resistant to phenobarbital, lorazepam, and other anticonvulsants. This patient should be weaned off the drug slowly to keep the threshold for seizure activity ­up. 88. Correct answer: ­A The dose for neonates is 10 mg/kg every 24 hours and may be given either intravenously or orally. While monitoring levetiracetam levels is not typically done, the therapeutic range for this drug is 10–40 ­mcg/ml. 89. Correct answer: ­B Lidocaine, when used as an anticonvulsant, is recommended to be given at 2 mg/kg over 10 minutes. It should be immediately followed by a 6-mg/kg infusion for 6 hours, then a 4-mg/kg infusion for 12 hours, and finally 2 mg/kg for 12 hours. Lidocaine should only be given to ­f ull-­term, normothermic infants. Premature infants and those infants who are hypothermic absorb lidocaine erratically and are more likely to develop toxic levels ­quickly. 90. Correct answer: ­B Lidocaine and phenytoin should never be given at the same time due to the potential for cardiac arrhythmias. Infants receiving infusions of lidocaine should be monitored to include EKG, vital signs, and seizure activity. Lidocaine levels are not usually checked unless toxicity is ­suspected. 91. Correct answer: ­A The dose of lorazepam is incorrect; 0.5 mg is an adult dose of lorazepam. The dosing for an infant is 0.05 mg/kg to 0.1 mg/kg given slow IV push. Monitor respiratory status and IV site for ­infiltration. 92. Correct answer: ­C Methadone is the drug of choice for this baby. It can help the infant overcome withdrawal symptoms from birth to 6 weeks of age. The weaning schedule is adjusted for the intensity of ­symptoms. 93. Correct answer: ­A Respiratory, cardiac, and gastrointestinal systems monitoring is essential for the infant receiving methadone. Respiratory depression is more likely with larger doses of methadone. Cardiac monitoring for either symptomatic bradycardia or tachycardia

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is necessary. The gastrointestinal effects include large gastric residuals, abdominal distention, and loss of bowel ­sounds. 94. Correct answer: ­B The midazolam dose range is 0.05–0.15 mg/kg given via intravenous push over 5 minutes. The dose may be repeated every 2–4 hours. Lower doses are used if the infant is also receiving narcotics. The route of administration can be intranasally, IM, sublingually, orally, or via continuous IV ­infusion. 95. Correct answer: ­A Neostigmine is indicated for neonatal myasthenia gravis. This condition is seen in infants born to mothers with myasthenia gravis. The mother’s acetylcholine receptor antibodies are transferred across the placenta to the baby. The infants display poor feeding and hypotonia. These symptoms usually resolve in the first month of life, and the infants are treated ­symptomatically. 96. Correct answer: ­A The usual dose of neostigmine for neonatal myasthenia gravis is 0.1 mg IM 30 minutes before feeding or 1 mg 2 hours before feedings. The neostigmine inhibits anticholinesterase at the neuromuscular junction, thus improving muscle tone and preventing aspiration of ­feedings. 97. Correct answer: ­C Pentobarbital is a ­short-­acting barbiturate. It is without analgesic properties and is used for the brief hypnotic effect it produces. It may be used in conjunction with antiepileptic ­drugs. 98. Correct answer: ­A Barbiturates in general can cause respiratory depression. ­Stevens-­Johnson syndrome (SJS) is a serious, ­life-­threatening dermal reaction to a medication. SJS causes bullae and skin sloughing similar to a second- or ­third-­degree burn. Patients with more severe forms of SJS are often treated in burn units. Agitation is an idiosyncratic response to ­pentobarbital. 99. Correct answer: ­B Phenobarbital is given early in a case like baby Doe’s. The drug improves outcome and increases the threshold for seizure activity. It is given as a 40-mg/kg infusion over 1 ­hour. 100. Correct answer: ­C Phenobarbital remains one of the few useful barbiturates and is most often used as an anticonvulsant. The loading dose of phenobarbital in seizures is 20 mg/kg given IV over 10–15 minutes. The maintenance dose is 3–4 mg daily that is started 12–24 hours after the loading ­dose. 101. Correct answer: ­A Stevens-­Johnson syndrome (SJS) is a mucocutaneous, ­drug-­induced skin reaction. SJS is a rare, but serious skin reaction that is potentially life threatening as it can lead to

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multisystem failure. It is seen in immunocompromised patients such as those with HIV. Patients with this condition are treated like burn patients. Intravenous fluids and electrolytes are an important part of the therapy. Corticosteroids may be given, but their use is somewhat ­controversial. 102. Correct answer: ­C Phenobarbital is the drug of choice for seizure activity. Phenytoin is used as a ­second-­line medication. Pentobarbital is not indicated for seizure activity. Other medications used for seizures include lorazepam and fosphenytoin. Treating the cause of the seizure, if possible, is the most important ­action. 103. Correct answer: ­A The loading dose of phenytoin is 15–20 mg/kg given IV over 30–45 minutes. It is then followed every 24 hours by 4–8 mg/kg for a maintenance dose. It is important to assess the patency of the IV, then flush with normal saline before and after administering phenytoin. Extravasation of phenytoin can cause tissue ­necrosis. 104. Correct answer: ­B Rocuronium or any neuromuscular blocking agent should only be given after an analgesic. A patient receiving these drugs without pain medications first is fully awake and aware but cannot respond to the trauma. To give this medication without anesthesia may be construed as negligence and battery, making you liable for legal ­action. 105. Correct answer: ­A The correct dosing for rocuronium is 0.3–0.6 mg/kg given IV over 5–10 seconds and should only be given after an analgesic has been ­administered. 106. Correct answer: ­C Klumpke’s paralysis may present as shoulder swelling, a fractured clavicle, a ­claw-­hand deformity, and no grasp in the affected ­hand. 107. Correct answer: ­A The cranial nerves affected in a patient with Klumpke’s paralysis are cervical nerve VI to thoracic nerve ­I. 108. Correct answer: ­A Skull fractures in neonates are usually caused by extreme molding or excessive force used in a forceps ­delivery. 109. Correct answer: ­C Linear fractures in neonates are asymptomatic and quite common. Linear fractures do not separate adjacent sutures and usually completely heal within 3 ­months. 110. Correct answer: ­C Depressed skull fractures in neonates often occur in the right parietal bone and do not cross suture lines. They only occasionally cause separation of adjacent ­sutures.

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111. Correct answer: ­B Phrenic nerve palsy is diaphragmatic paralysis as a result of overstretching cervical nerves III, IV, and V. Nerve sheaths are torn, and about 80–90% of these injuries are associated with brachial plexus ­injury. 112. Correct answer: ­C An elevated hemidiaphragm and paradoxical movement of the affected side with breathing are cardinal signs of phrenic nerve ­paralysis. 113. Correct answer: ­C A characteristic of facial nerve palsy is edema and hemorrhage into the nerve sheath. Symptoms usually resolve within a few ­months. 114. Correct answer: ­C Maximal vasodilation of the cerebral vasculature occurs as a response to acidosis, hypercapnia, and ­hypoxemia. 115. Correct answer: ­A An increase in calcium ions will cause a decrease in cerebral blood ­flow. 116. Correct answer: ­C The cephalohematoma does not transilluminate and generally feels firm to touch. The cephalohematoma will enlarge the first few days after birth, is usually unilateral, and does not extend across cranial suture ­lines. 117. Correct answer: ­B A birth injury in which the affected arm is abducted and internally rotated is known as Erb’s palsy. The elbow is extended, the arm pronated, and the wrist flexed. There is also an absent Moro’s ­reflex. 118. Correct answer: ­A A primary subarachnoid hemorrhage is bleeding into the CSF filled space between the arachnoid and pial membranes on the surface of the brain. Bleeding is venous in origin and not secondary to an extension of a subdural ­hemorrhage. 119. Correct answer: ­B Seizures in the newborn may be caused by a metabolic encephalopathy such as decreased adenosine triphosphate production, hypoglycemia, hypoxemia, and ­ischemia. 120. Correct answer: ­C It is important to stop seizure activity in status epilepticus because continued seizure activity causes lactic acidosis and cerebral ­edema. 121. Correct answer: ­A An infant with severe brain damage from hypoxic ­ischemic-­encephalopathy (HIE) may present with cerebral ­edema.

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122. Correct answer: ­A Diplegia presents as hypertonic lower extremities with increased deep tendon reflexes while facial and upper extremity movement is normal. Diplegia may be associated with neurologic damage such as seen with cerebral ­palsy. 123. Correct answer: ­B Polyhydramnios may be associated with anencephaly due to the inability of the fetus to swallow amniotic ­fluid. 124. Correct answer: ­A Six or more café au lait spots of sizes . 5 mm may be associated with ­neurofibromatosis.

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Neurology ­References Ali, M., Khan, A., Khan, H., & Khwanzada, K. (2009). ­Short-­term complications of ventriculoperitoneal shunt in children suffering from hydrocephalus. Journal of Pediatric Neurology, 7(2), 165–169. Alspach, J. G. (2006). American association of ­critical-­care nurses: Core curriculum for critical care nursing (6th ed.). Philadelphia, PA: W. B. ­Saunders. Alwan, S., Reefhuis, J., Rasmussen, S. A., et al. (2007). Use of selective ­serotonin-­reuptake inhibitors in pregnancy and the risk of birth defects. The New England Journal of Medicine, 356(26), 2684–2692. Arts, M. P., & de Jong, T. H. R. (2004). Thoracic meningocele, meningomyelocele or myelocystocele? Diagnostic difficulties, consequent implications and treatment. Pediatric Neurosurgery, 40(2), 75–79. Bader, M. K., Littlejohns, L. R. (2004). AANN core curriculum for neuroscience nursing (4th ed.). St. Louis, MO: S­ aunders. Barker, E. (Ed.) (2002). Neuroscience nursing: A spectrum of care (2nd ed.). St. Louis, MO: ­Mosby. Bickley, L. S., & Szilagyi, P. G. (2003). Bates’ guide to physical examination and history taking (8th ed.). Philadelphia, PA: Lippincott, Williams & ­Wilkins. Blom, H. J., Shaw, J. M., den Heijer, M., & Finnell, R. H. (2006). Neural tube defects and folate: Case far from closed. Nature reviews. Neuroscience, 7(9), 724–731. Braunwald, E., Fauci, A. S., Kasper, D. L., Hauser, S. L., Longo, D. L., Jameson, J. L. (Eds.). (2008). Harrison’s principles of internal medicine (17th ed.). New York, NY: McGraw-­Hill. Brodsky, D., & Martin, C. (2003). Neonatology review. Philadelphia, PA: Hanley & ­Belfus. Chan, E., Ong, C., Hsu, L., et al. (2008). Corticosteroids for bacterial meningitis. The New England Journal of Medicine, 358(13), ­1399. Chernecky, C. C., & Berger, B. J. (2001). Laboratory tests and diagnostic procedures (3rd ed.). Philadelphia, PA: W. B. ­Saunders. Cloherty, J. P., Eichenwald, E. C., & Stark, A. R. (2004). Manual of neonatal care (5th ed.). Philadelphia, PA: ­Lippincott. Crocoli, A., Madafferi, S., Jenkner, A., et al. (2008). Elevated serum ­alpha-­fetoprotein in Wilms tumor may follow the same pattern of other fetal neoplasms after treatment: Evidence from three cases. Pediatric Surgery International, 24(4), 499–502. Dekker, C., & Arvin, A. (2009). One step closer to a CMV vaccine. The New England Journal of Medicine, 360(12), 1250–1252. Eide, P. K., Egge, A., ­Due-­Tennesses, B. J., & Helseth, E. (2007). Is intracranial pressure waveform analysis useful in the management of pediatric neurosurgical patients? Pediatric Neurosurgery, 43, 472–481. Retrieved from June 28, 2009 from Pro Quest ­database. Emory School of Medicine. (2008). Cystic fibrosis. Retrieved ­f rom http://genetics.emory.edu/pdf/ Emory_Human_Genetics_Cystic_Fibrosis_Carrier.PDF Emory University School of Medicine. (2008). Cystic hygroma. Retrieved from ­w ww.genetics .emory.edu/pdf/Emory_Human_Genetics_Cystic_Hygroma.PDF Fenichel, G. M. (2007). Neonatal neurology (4th ed.). Philadelphia, PA: Churchill ­Livingstone. Fichter, M., Dornseifer, U., Henke, J., et al. (2008). Fetal spina bifida ­repair—­Current trends and prospects of intrauterine neurosurgery. Fetal Diagnosis and Therapy, 23(4), 271–286. Geerdink, N., Pasman, J., Rotteveel, J., et al. (2008). Compound muscle action potentials in newborn infants with spina bifida. Developmental Medicine and Child Neurology, 50(9), 706–711. Gilman, S., & Newman, S. W. (2003). Manter and Gatz’s essentials of clinical neuroanatomy and neurophysiology (10th ed.). Philadelphia, PA: F. A. ­Davis. Glass, H., & Wu, Y. (2009). Epidemiology of neonatal seizures. Journal of Pediatric Neurology, 7(1), 13–17.

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274  y  Section 9 Halliday, H. L., McClure, B. G., & Reid, M. (2002). Handbook of neonatal intensive care (4th ed.). Philadelphia, PA: ­Saunders. Hassanein, S., Moharram, H., & Monib, A., et al. (2008). Perinatal ventriculomegaly. Journal of Pediatric Neurology, 6(4), 293–307. Hay, W. W., Levin, M. J., Sondheimer, J. M., & Deterding, R. R. (2007). Current pediatric diagnosis and treatment (18th ed.). New York, NY: McGraw-­Hill. Hickey, J. V. (2002). The clinical practice of neurological and neurosurgical nursing (5th ed.). Philadelphia, PA: ­Lippincott. Jones, K. L. (2006). Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: ­Elsevier. Karlsen, K. (2006). The S.T.A.B.L.E. program (5th ed.). Park City, UT: American Academy of ­Pediatrics. Karlsen, K., & Tani, L. Y. (2003). S.T.A.B.L.E.—Cardiac module. Park City, UT: American Academy of ­Pediatrics. Kattwinkle, J. (Ed.). (2006). Neonatal resuscitation textbook (5th ed.). Elk Grove Village, IL: American Academy of Pediatrics and American Heart ­A ssociation. Kenner, C., & Lott, J. (2004). Neonatal nursing handbook. St. Louis, MO: ­Elsevier. Kenner, C., & Lott, J. (2007). Comprehensive neonatal care (4th ed.). St. Louis, MO: ­Elsevier. Kenner, C., Amlung, S., & Flandermeyr, A. (1998). Protocols in neonatal nursing. Philadelphia, PA: ­Saunders. Kenner, C., & McGrath, J. M. (Ed.). (2004). Developmental care of newborns & infants: A guide for health professionals. St. Louis, MO: M ­ osby. Kuriyama, N., Tokuda, T., Kondo, M., et al. (2008). Evaluation of autonomic malfunction in idiopathic normal pressure hydrocephalus. Clinical Autonomic Research, 18(4), 213–220. Laroia, N. (2008). Birth trauma. Retrieved from http://emedicine.medscape.com/article /980112-­overview MacDonald, M. G., & Ramasethu, J. (2007). Atlas of procedures in neonatology (4th ed.). Philadelphia, PA: Lippincott ­Williams. Markiewicz, M., & Abrahamson, E. (1999). Diagnosis in color: Neonatology. Philadelphia, PA: ­Mosby. Mc Cance, K. L., & Huether, S. E. (2002). Pathophysiology: The biologic basis for disease in adults and children (4th ed.). St. Louis, MO: ­Mosby. Merck Manuals Online Library. (2008). Intracranial and spinal tumors. Retrieved from ­http://www.merck.com/mmpe/print/sec16/ch225/ch225a.html Merenstein, G. B., & Gardner, S. L. (2006). Handbook of neonatal intensive care (6th ed.). St. Louis, MO: ­Mosby. Montañana, A. P., Palacios, I. E., & Cariñena, L. S. (2008). Dexamethasone therapy for bacterial meningitis in children and newborns. Acta Pediatrica Espanola, 66(8), 390–395. Montefiore Medical Center. (2009). Birth injuries. Retrieved from ­http://www.montefiore.org /healthlibrary/centers/pregnancy/birthinj/ Pearl, P. (2009). New treatment paradigms in neonatal metabolic epilepsies. Journal of Inherited Metabolic Disease, 32(2), 204–213. Pearse, R. V., & Tabin, J. T. (2006). Development: Twists of fate in the brain. Nature, 439(7075), 404–405. Polin, R. A., & Spritzer, A. R. (2007). Fetal & neonatal secrets (2nd ed.). Philadelphia, PA: ­Mosby. Prescribing Reference. (2009, Summer). NPPR: Nurse Practitioner’s Prescribing Reference, 16(2). Rangel-­Castillo, L., Gopinath, S., & Robertson, C. S. (2008, May). Management of intracranial hypertension. Neurologic Clinics, 26(2), 521–541. Retrieved from http://www.neurologic .theclinics.com/article/S0733-8619(08)00021-­2/abstract Rennie, J. M., & Roberton, N. R. C. (2002). A manual of neonatal intensive care (4th ed.). New York, NY: A ­ rnold.

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Neurology  y  275 Riffaud, L., Moughty, C., Henaux, P., et al. (2008). Acquired Chiari I malformation and syringomyelia after valveless lumboperitoneal shunt in infancy. Pediatric Neurosurgery, 44(3), 229–233. Robertson, J., & Sholkofski, N. (2005). The Harriet Lane handbook (17th ed.). Philadelphia, PA: Elsevier-­Mosby. Rogerson, S., Malenga, G., & Molyneux, E. M. (2004, June). Integrated pathways: A tool to improve infant monitoring in a neonatal unit. Annals of Tropical Paediatrics, 24(2), 171–174. Retrieved from Pro Quest ­database. Siedel, H. M., Ball, J. W., Dains, J. E., & Benedict, G. W. (2003). Mosby’s physical examination handbook (3rd ed.). St. Louis, MO: M ­ osby. Siegel, A., & Siegel, H. (2002). Neuroscience: Pretest ­self-­assessment and review (5th ed.). New York, NY: McGraw-­Hill. Stemp-­Morlock, G. (2007). Pesticides and anencephaly. Environmental Health Perspectives, 115(2), ­A78. Taeusch, H. W., Ballard, R. A., & Gleason, C. A. (2005). Avery’s diseases of the newborn (8th ed.). Philadelphia, PA: ­Elsevier. University of Maryland Medical Center. (2007). Increased intracranial pressure—­treatment. Retrieved from­http://www.umm.edu/ency/article/000793trt.htm Van Rooij, L. G. M., Toet, M. C., Rademaker, K. M. A, et al. (2004). Cardiac arrhythmias in neonates receiving lidocaine as anticonvulsive treatment. European Journal of Pediatrics, 163(11), 637–641. Verklan, M. T., & Walden, M. (Eds.). (2004). Certification and core review for neonatal intensive care nursing (3rd ed.). St. Louis, MO: E ­ lsevier. Verklan, M. T., & Walden, M. (Eds.). (2010). Certification and core review for neonatal intensive care nursing (4th ed.). St. Louis, MO: ­Elsevier. Woodhouse, C. (2008). Myelomeningocele: Neglected aspects. Pediatric Nephrology, 23(8), 1223–1231. Yadav, Y., Jaiswal, S., Adam, N., et al. (2006). Endoscopic third ventriculostomy in infants. Neurology India, 54(2), 161–163. Young, T. E., & Magnum, B. (2009). Neofax 2009. Montvale, NJ: Thomson ­Reuters. Zoair, A., ­El-­A ziz, S., Awny, M., et al. (2008). Neonatal cerebrospinal fluid plasminogen and plasminogen activator inhibitor-1 assay as predictors of posthemorrhagic hydrocephalus. Journal of Pediatric Neurology, 6(3), 237–242.

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Section 10 Endocrinology

Questions 1. Baby C. was born yesterday at 38 weeks gestation. She is edematous, hyponatremic, has a serum osmolality of 272 mOsm/L, and is maintaining a normal blood pressure for her age. As a NICU nurse, you suspect this infant is suffering f­ rom A. A Wilms’ ­t umor B. ­SIADH C. Adrenal ­hyperplasia 2. Baby M. was born at 37 weeks gestation and is now at day 4 of life. This baby has poor skin turgor, depressed anterior fontanelle, mottled skin, weak pulses, low blood pressure, and is lethargic. Lab results indicate a urine specific gravity of 1.005, an elevated serum osmolality and a low urine osmolality. Urine output the past 24 hours has been 6.5 ml/kg/hr. Baby M. probably ­has A. Congenital ­hypopituitarism B. Thyroid ­dysgenesis C. Diabetes ­insipidus 3. The infant you are caring for has been diagnosed with central diabetes insipidus. The anticipated treatment for this condition ­is A. Thyroid stimulating ­hormone B. ­A ldosterone C. ­DDAVP 4. When administering desmopressin for diabetes insipidus, it is important ­to A. Avoid ­hyperglycemia B. Promote ­diuresis C. Monitor cortisol ­levels 5. During shift report, you are informed that the baby you will be caring for has a “musty” odor that emanates from the infant’s urine. This odor is usually characteristic ­of A. Maple syrup ­disease B. ­Phenylketonuria C. Potter’s ­disease 6. Sally is a 38-week gestational age infant at 2 days of life. She was LGA at birth and has small left colon syndrome. This condition is specific ­to A. Esophageal ­atresia B. Infants of diabetic ­mothers C. Infants of ­drug-­abusing m ­ others

y

y

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7. Maternal hyperglycemia ultimately c ­ auses A. Decelerated ­lipogenesis B. ­Microsomia C. Increased hepatic glucose ­uptake 8. Which of the following statements is true regarding excess fetal ­insulin? A. Excess fetal insulin may be the cause of delayed maturation of type II alveolar ­cells B. Excess fetal insulin promotes the development of pulmonary ­surfactant C. Excess fetal insulin causes idiosyncratic ­hyperglycemia 9. Infants who are insulin deficient often h ­ ave A. Mental ­retardation B. Intrauterine growth ­restriction C. ­Polycythemia 10. Which of the following statements is true about transient neonatal diabetes ­mellitus (TNDM)? A. TNDM is a common form of diabetes that disappears within 2 weeks of ­life B. TNDM will completely resolve within 24 months of ­birth C. TNDM infants have hyperglycemia and low insulin ­levels 11. Which of the following conditions would inhibit the release of ­thyroxine? A. ­Hypokalemia B. ­Hyperthermia C. ­Hypernatremia 12. Hyperthyroidism is also known ­as A. Grave’s d ­ isease B. Huntington’s ­disease C. Paget’s ­disease 13. Stimulation of the anterior pituitary gland is from the hypothalamus by way of t­ he A. Sympathetic nervous ­system B. Parasympathetic nervous ­system C. Vascular ­system 14. Calcitonin is released by t­ he A. Anterior pituitary ­gland B. Thyroid ­gland C. Adrenal ­medulla 15. An increase in serum calcium, glucagon, or magnesium will stimulate the release o ­f A. Parathyroid ­hormone B. Endogenous ­epinephrine C. ­Calcitonin

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16. Which of the following statements is true regarding ­glucagon? A. Glucagon is released from the alpha cells in the ­pancreas B. Glucagon enables glucose to move into the ­cells C. Glucagon helps the body store ­glycogen 17. An increase in catecholamines and amino acids with a decrease in serum glucose will stimulate the release o ­f A. ­Glucagon B. ­Insulin C. ­A ldosterone 18. Which of the following statements is true regarding ­insulin? A. Insulin enables glucose to move out of the ­cell B. Insulin decreases protein ­synthesis C. Insulin inhibits ­lipolysis 19. A high serum magnesium or phosphate level, low serum calcium, with catecholamine release results in secretion ­of A. ­A ldosterone B. Parathyroid ­hormone C. ­Glyceride 20. A medication that blocks the effect of antidiuretic hormone on the renal tubule would ­be A. ­Lithium B. ­Acetaminophen C. ­Nicotine 21. The type of diabetes characterized by beta cell destruction that usually leads to absolute insulin deficiency is known a ­s A. Type 1 d ­ iabetes B. Type 2 d ­ iabetes C. ­HHNK 22. The type of diabetes characterized by insulin resistance and a relative insulin deficiency is known ­as A. ­HHNK B. ­Adult-­onset ­diabetes C. A hyperglycemic ­crisis 23. Patients with hyperosmolar, hyperglycemic, nonketotic syndrome (HHNK or HHNS) ­exhibit A. Increased potassium, increased ­calcium B. Decreased phosphorus, decreased ­magnesium C. Decreased sodium, decreased B ­ UN

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24. The most important nursing consideration in the neonatal patient with HHNS ­is A. The administration of ­insulin B. To monitor ­electrolytes C. To prevent ­aspiration 25. The most common cause of hypothyroidism ­is A. Decreased cortisol ­levels B. ­Acromegaly C. Hashimoto’s ­disease 26. Congenital hypothyroidism may lead ­to A. Increased organ ­weight B. Mental ­retardation C. ­SIADH 27. The mother of your patient tells you she cannot breastfeed her infant because she has a disorder that prevents her from producing breastmilk. She further states that during her first pregnancy, she had copious bleeding at delivery and could not breastfeed adequately following birth of that child. You suspect the mother may ­have A. ­A nemia B. Sheehan’s s­ yndrome C. ­Hyperpituitarism 28. Vitamin D deficiency in the neonate may manifest itself ­as A. ­R ickets B. ­Hypercalcemia C. ­Prune-­Belly ­syndrome 29. Michael is a 36-week gestation neonate at day 3 of life. He was admitted for a PDA that was ligated. During his assessment, you note that his wrist appears wider than normal. His chest appears wider than normal as well. His serum calcium level is also low. As a NICU nurse, you believe Michael is probably suffering ­from A. Vitamin A ­deficiency B. Vitamin D ­deficiency C. Vitamin B2 ­deficiency 30. Rickets is likely to develop in neonates ­who A. Consume large amounts of fortified ­milk B. Are lactose ­tolerant C. Are breastfed and not exposed to ­sunlight 31. Which of the following is a primary effect of ­aldosterone? A. Aldosterone increases renal potassium ­excretion B. Aldosterone increases serum sodium ­levels C. Aldosterone decreases potassium ­excretion

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32. Aldosterone is secreted by ­the A. Zona reticularis of the adrenal ­cortex B. Zona glomerulus of the adrenal ­cortex C. Adrenal ­medulla 33. Aldosterone secretion is regulated ­by A. Angiotensin ­II B. Potassium ions in intracellular ­fluid C. ­Hypercalcemia 34. A factor that suppresses aldosterone production ­is A. ­Hypocalcemia B. ­Hyperkalemia C. Atrial natriuretic ­hormone 35. Cortisol is a glucocorticoid. A major effect of cortisol in the body is ­to A. Increase the loss of sodium from small ­intestines B. Lower blood pressure in times of ­stress C. Raise the level of free amino acids in the serum 36. Baby R. is a 35-week gestation infant at day 8 of life. Baby R. has respiratory distress syndrome and has difficulty weaning from the ventilator. Instead of raising ventilator pressures, prednisone was started to assist with the weaning process. An important nursing consideration when a patient is receiving prednisone is ­to A. Obtain blood pressure measurements every 2 ­hours B. Place an orogastric tube for administration of ­prednisone C. Monitor blood glucose ­levels 37. Baby D. has been vomiting with diarrhea for the past 2 days. The infant has failure to thrive and hepatomegaly. Cataracts are noted and have an “oil droplet” appearance. Baby D. is probably symptomatic for which inborn error of ­metabolism? A. ­Galactosemia B. Wilson’s ­disease C. ­Tay-­Sachs ­disease 38. Galactosemia is a congenital disease transmitted as ­an A. ­X-­linked recessive ­disease B. Autosomal recessive ­disorder C. ­X-­linked dominant ­trait 39. A mild form of galactosemia that can be treated and tends to disappear after 1 year of life is known ­as A. Harper’s ­galactosemia B. Parker’s ­galactosemia C. Duarte ­galactosemia

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40. A disorder caused by a defect in copper excretion is known ­as A. Meikle’s ­disease B. Cooper’s ­disease C. Wilson’s ­disease 41. An ­X-­linked recessive disorder caused by abnormal copper transport is known ­as A. Menkes kinky hair ­disease B. ­Porphyrias C. Pompe’s d ­ isease 42. The lysosomal storage disease caused by glucocerebrosidase deficiency is known ­as A. ­Tay-­Sachs ­disease B. Gaucher’s ­disease C. Pompe’s d ­ isease 43. Hexosaminidase A deficiency is known ­as A. Pompe’s d ­ isease B. Seither’s ­disease C. ­Tay-­Sachs ­disease 44. An autosomal recessive disorder that is classified as a membrane transport defect that may lead to renal stones ­is A. ­Cystinurea B. ­Galactosemia C. Hartnup ­disease 45. A disorder that is caused by a defect in transport of neutral amino acids is ­called A. Piaget’s ­disease B. Hartnup ­disease C. Potter’s ­disease 46. Which of the following disorders would be classified as a urea cycle ­defect? A. Piaget’s ­disease B. Cooper’s ­disease C. Ornithine translocase ­deficiency 47. Isabel is a d ­ ay-­old, 37-week gestation infant who appeared to be doing well at home until she began having seizures. On initial assessment, you note Isabel is lethargic and has poor muscle tone. She appears to be having muscle spasms. Isabel will not feed and has a “burned sugar” smell to her urine. You believe Isabel is suffering ­from A. ­R habdomyolysis B. ­Tyrosinemia C. Maple syrup urine ­disease

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48. The effects of hypothyroxinemia on prematurity can be mitigated by the nurse in which of the following ­ways? A. Discontinuing the administration of ­digoxin B. Using Betadine sparingly on the infant’s ­skin C. Administering ­propylthiouracil 49. Congenital hypothyroidism is manifested ­by A. An elevated TSH and a decreased ­T4 B. A decreased TSH and a decreased ­T4 C. A decreased TSH and an elevated ­T4 50. Maternal thyroid disease can have a substantial influence on fetal and neonatal thyroid function ­because A. Immunoglobulin G (IgG) autoantibodies can cross the ­placenta B. Thioamides can stimulate thyroid ­growth C. Radiation will stimulate thyroid ­f unction 51. Physical findings of congenital hypothyroidism ­include A. Hyperthermia and poor ­feeding B. Diarrhea and ­irritability C. Macroglossia and large ­fontanelles 52. Physical findings in an infant with hyperthyroidism ­include A. Hypothermia and depressed ­fontanelles B. Hypertension and ­vomiting C. Constipation and cool, dry ­skin 53. An infant with a large goiter should be ­positioned A. Supine with the head slightly elevated and ­extended B. Side-­lying C. Prone with a small towel under the c­ heek 54. Symptoms of Graves’ disease ­include A. An atrial septal ­defect B. ­Microglossia C. Pretibial ­myxedema 55. Congenital adrenal hyperplasia is the most common cause ­of A. Neonatal ­asphyxia B. Ambiguous ­genitalia C. Fatty acid ­defects 56. Nonketotic hyperglycinemia manifests itself in the newborn ­via A. Lethargy that may progress to ­coma B. Hyperactivity and ­agitation C. ­Hypertonia

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57. Signs of vitamin D toxicity ­include A. ­Hypocalcemia B. Increased susceptibility to respiratory ­diseases C. ­A zotemia 58. Frank B. has a crush injury to his right thigh from being trapped between his mother’s mattress and bed rail when she fell asleep. Why does this put Frank at risk for ­hyperkalemia? A. There is more risk for ­hypokalemia B. He is at no risk for ­hyperkalemia C. Cellular destruction leads to increased circulating potassium ­levels 59. Functions of the thyroid gland, adrenal gland, and male and female reproductive glands are regulated by t­ he A. Pineal gland of the ­brain B. Thyroid ­gland C. Hypothalamic–pituitary ­axis 60. Where is the pituitary gland ­located? A. Inferior to the hypothalamus and sits in the sella turcica of the ­skull B. Superior to the hypothalamus gland near the optic ­chiasm C. Between the thalamus and hypothalamus in the ­midbrain 61. Which of the following hormones are produced by the anterior pituitary ­gland? A. GRF, TSH, and Substance ­P B. ADH, TSH, and ­FSH C. FSH, LH, TSH, and ­ACTH 62. Where is the thyroid gland ­located? A. Just below the hyoid ­bone B. In the throat on both sides of the ­trachea C. Above the ­larynx 63. Your patient’s TSH (thyroid stimulating hormone) level is 0.001. What condition does this value ­indicate? A. Hypoactive anterior pituitary ­f unction B. Hyperactive anterior pituitary ­f unction C. ­Hyperthyroidism 64. Treatment for a thyrotoxic crisis i­ ncludes A. Administration of PTH and symptomatic ­care B. Symptomatic care and wait for symptoms to ­subside C. Synthroid administration and supportive ­care 65. What feature is unique to the ­pancreas? A. There are no unique ­features B. It is both an endocrine and exocrine ­gland C. It is the largest gland in the ­body

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66. Where are the parathyroid glands ­located? A. Anterior to the ­thyroid B. Posterior to the thyroid ­gland C. Atop the thyroid ­gland 67. Why are hyperglycemia and hyperlipidemia seen concurrently in diabetes ­mellitus? A. VLDL (very low density lipoproteins) production increases in response to increased insulin ­production B. Insulin resistance promotes VLDL (very low density lipoproteins) ­production C. Lipid breakdown is hindered by ­hyperinsulinemia 68. How is insulin secretion r­ egulated? A. Hormonal, insulin, and neuronal ­controls B. Chemical, hormonal, and neuronal ­controls C. Chemical, glucagon, and insulin ­controls 69. Where is renin s­ tored? A. Renal ­t ubule B. Loop of ­Henle C. Juxtaglomerular cells of the ­nephron 70. What can trigger the ­renin-­angiotensin m ­ echanism? A. Aldosterone and ­diuretics B. Diuretics and decreased renal blood ­flow C. Diuretics and adrenergic ­blockers 71. What is the correct order of the ­renin-­angiotensin mechanism when there is a decrease in blood flow to the ­kidneys? A. Renin, ACTH, and angiotensin ­I B. Increased ADH, renin, angiotensin I, and angiotensin ­II C. Renin, angiotensinogen, angiotensin I, angiotensin ­I-­converting enzyme, ­angiotensin II, and ­aldosterone 72. The anterior pituitary gland controls which of the following ­glands? A. Parathyroid, adrenal medulla, and ­gonads B. Thyroid, adrenal medulla, and ­gonads C. Thyroid, adrenal cortex, and ­gonads 73. What inhibits ADH (antidiuretic hormone) ­production? A. Pituitary ­t umors B. Water ­intoxication C. Increased serum ­osmolality 74. Which of the following is responsible for the symptoms of ­hypothyroidism? A. Low levels of T4 (thyroxine) B. Low levels of ­T3 C. Decreased ­thyrocalcitonin

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75. Which glands regulate thyroid f­ unction? A. Posterior pituitary and ­hypothalamus B. Anterior pituitary and ­thalamus C. Anterior pituitary and ­hypothalamus 76. Which of the following can cause a thyroid storm in a patient with ­hyperthyroidism? A. Overdose of PTU (propylthiouracil) B. Increased iodine ­intake C. Trauma or ­infection 77. What symptoms are to be expected with thyrotoxic ­crisis? A. Hypotension, ­bradycardia B. Hyperthermia, ­bradycardia C. Hypertension, ­hyperthermia 78. At birth, an infant should have a blood glucose level equal to what percentage of the mother’s blood glucose ­level? A. 50% B. 60% C. 80% 79. Infants with DiGeorge syndrome may have which of the following electrolyte imbalances because of absent parathyroid ­glands? A. ­Hypocalcemia B. ­Hypercalcemia C. ­Hypokalemia 80. Which of the following conditions will result in a hyperinsulinemic ­state? A. Carnitine ­deficiency B. ­A lpha-­cell ­adenoma C. Leucine ­resistance 81. You are admitting a term patient with hyperbilirubinemia. The last level was 30 at 3 days of life. Based on these results you anticipate further treatment. You ­should A. Set up one phototherapy light and an eye ­shield B. Prepare to start a peripheral IV and give dextrose 15% C. Prepare for umbilical line insertion and an exchange ­transfusion 82. The parents of a term infant diagnosed with physiologic jaundice are very concerned that their child is at risk for brain damage. Which of the following statements regarding hyperbilirubinemia is most ­accurate? A. Breastfeeding, as compared with formula feeding, is associated with higher peak serum bilirubin ­levels B. Serum conjugated bilirubin concentration is the best predictor of bilirubin ­encephalopathy C. Bilirubin encephalopathy does not occur in healthy, term ­infants

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83. If an infant is large for gestational age, the nurse should assess for which of the following ­conditions? A. ­Hypercalcemia B. ­Hypoglycemia C. ­Hypothermia 84. If a newborn is less than 24 hours old, treatment for hypoglycemia should begin for which of the following serum glucose ­levels? A. 35 m ­ g/dl B. 50 ­mg/dl C. 60 m ­ g/dl

This concludes the Endocrinology ­questions.

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Endocrinology ­Answers 1. Correct answer: ­B Baby C. is probably suffering from the syndrome of inappropriate antidiuretic hormone (SIADH). This condition is a disorder of the posterior pituitary gland. Free water clearance is impaired by an increased secretion of vasopressin (ADH). ADH levels may become elevated in infants with fetal distress, respiratory diseases, surgery, central nervous system infections, injury (e.g., birth asphyxia), and pain. Prematurity, intracranial hemorrhage, and meningitis may also be ­factors. Infants with SIADH will exhibit weight gain, dilutional hyponatremia, edema, and a serum osmolality of , 275 mOsm/L. Urine osmolality will be high because the urine will be concentrated. Blood pressure can be normal as can adrenal, thyroid, and cardiac function. 2. Correct answer: ­C Baby M. has diabetes insipidus. This baby has poor skin turgor, depressed anterior fontanelle, mottled skin, weak pulses, low blood pressure, and is lethargic. Lab results indicate a urine specific gravity of 1.005, an elevated serum osmolality and a low urine osmolality. Urine output the past 24 hours has been 6.5 ­ml/kg/hr. 3. Correct answer: ­C The traditional treatment of central diabetes insipidus involves the replacement of vasopressin or use of an analogue, the most effective available being desmopressin (DDAVP). 4. Correct answer: ­A When administering desmopressin for diabetes insipidus, it is important to avoid hyperglycemia because it can lead to glycosuria, which will increase diuresis even more. It is also necessary to avoid rapid fluid shifts and rapid shifts in serum sodium levels. Seizures may occur in infants with severe hypernatremia, so neurologic status must be monitored ­closely. 5. Correct answer: ­B A “musty” or “mousy” odor is often characteristic of phenylketonuria (PKU). PKU is an autosomal recessive disease and results in an inability to metabolize phenylalanine. PKU is considered a defect in amino acid metabolism. This condition may result in mental retardation, developmental delay, eczema, and heart defects. PKU is completely preventable and can be controlled by a diet low in ­phenylalanine. 6. Correct answer: ­B Babies born with small left colon syndrome are infants of diabetic mothers. The abdomen is usually distended and there is a failure to pass ­meconium. 7. Correct answer: ­C Maternal hyperglycemia ultimately causes increased hepatic glucose uptake secondary to fetal hyperinsulinemia and hyperglycemia. Increased glycogen synthesis, accelerated lipogenesis, and macrosomia will ­result.

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8. Correct answer: ­A Excess fetal insulin may be the cause of delayed maturation of type II alveolar cells and pulmonary surfactant deficiency. The excess insulin may cause congenital cardiac anomalies such as cardiomyopathy and intraventricular septal ­hypertrophy. 9. Correct answer: ­B Infants who are insulin deficient often have intrauterine growth restriction. These infants often present with hyperglycemia, osmotic polyuria, glycosuria, dehydration, and ­ketoacidosis. 10. Correct answer: ­C Transient (TNDM) and permanent (PNDM) neonatal diabetes mellitus are rare conditions. The infant with TNDM has hyperglycemia and low levels of insulin. TNDM infants develop diabetes in the first few weeks of life but go into remission in a few months, with possible relapse to a permanent diabetic state usually around adolescence. Patients with TNDM are more likely to have intrauterine growth retardation and are less likely to develop ketoacidosis than those with ­PNDM. 11. Correct answer: ­B Hyperthermia would inhibit the release of ­thyroxine. 12. Correct answer: ­A Hyperthyroidism is also known as Graves’ ­disease. 13. Correct answer: ­C Stimulation of the anterior pituitary gland is from the hypothalamus by way of the vascular ­system. 14. Correct answer: ­B Calcitonin is released by the thyroid ­gland. 15. Correct answer: ­C An increase in serum calcium, glucagon, or magnesium will stimulate the release of calcitonin. Calcitonin will reduce serum calcium levels. Calcitonin targets bone and kidney cells to inhibit bone cell lysis, and it decreases calcium reabsorption by the ­kidney. 16. Correct answer: ­A Glucagon is released from the alpha cells in the pancreas. Glucagon inhibits glycolysis, increases lipolysis, and increases blood glucose by stimulating glycogenolysis and ­gluconeogenesis. 17. Correct answer: ­A An increase in catecholamines and amino acids with a decrease in serum glucose will stimulate the release of glucagon. If one becomes low on serum glucose from exercise, for example, glucagon is released to help produce an increase in blood ­glucose.

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18. Correct answer: ­C Insulin inhibits lipolysis and increases protein synthesis. Insulin is produced by the beta cells of the pancreas and enables glucose to move into a cell where it helps tissue and muscle oxidation. Insulin also aids in the storage of ­glycogen. 19. Correct answer: ­B A high serum magnesium or phosphate level, low serum calcium, with catecholamine release results in secretion of parathyroid hormone. Bone breakdown is accelerated, releasing calcium into the blood. Calcium reabsorption is increased in the intestine, and kidney tubule reabsorption is decreased. Phosphate loss is increased in the urine, which decreases serum phosphate. Parahormone increases reabsorption of magnesium by renal tubules. Renal calculi may ­develop. 20. Correct answer: ­A A medication that blocks the effect of antidiuretic hormone on the renal tubule would be lithium. Additional drugs that would block the effect of ADH and precipitate diabetes insipidus are amphotericin B, caffeine, Dilantin, ethanol, norepinephrine, and some ­tetracyclines. 21. Correct answer: ­A The type of diabetes characterized by beta cell destruction that usually leads to absolute insulin deficiency is known as type 1 diabetes. This type of diabetes is also known as ­insulin-­dependent diabetes mellitus or ­juvenile-­onset d ­ iabetes. 22. Correct answer: ­B The type of diabetes characterized by insulin resistance and a relative insulin deficiency is known as ­adult-­onset diabetes, type 2 or ­noninsulin-­dependent diabetes ­mellitus. 23. Correct answer: ­B Patients with hyperosmolar, hyperglycemic, nonketotic syndrome (HHNK or HHNS) exhibit normal or elevated sodium, decreased phosphorus, decreased magnesium, elevated serum osmolality, normal or slightly elevated ketones, and blood glucose between 600 and ­2200. 24. Correct answer: ­C The most important nursing consideration in the neonatal patient with HHNS is to prevent aspiration. The severe hypokalemia is the result of no i­ ntracellular-­to-­extracellular shift occurring because the patient is not usually acidotic. When the patient is hypokalemic, hypocalcemia and hypomagnesemia also occur. Muscle activity is compromised and the patient is at high risk for aspiration because of a paralytic ­ileus. 25. Correct answer: ­C Hashimoto’s disease, or chronic lymphocytic thyroiditis, is the most common cause of hypothyroidism. Hashimoto’s disease is an autoimmune disorder. In Hashimoto’s disease, the immune system makes antibodies against the thyroid and interferes with production of thyroid ­hormone.

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26. Correct answer: ­B Congenital hypothyroidism may lead to mental retardation and growth failure. Some babies are born with a thyroid that is not fully developed or is dysfunctional. Early treatment can prevent future problems. Hypothyroidism is treated with synthetic thyroxine, which is identical to the T4 made by the thyroid. Within a few months of giving birth, a small percentage of women develop postpartum thyroiditis, which is believed to be an autoimmune condition. In some cases, the thyroid does not heal and their hypothyroidism is ­permanent. 27. Correct answer: ­B This mother probably has Sheehan’s syndrome. Symptoms of Sheehan’s syndrome may develop years later, including inability to breastfeed, fatigue, low blood pressure, loss of pubic and axillary hair, and absence of menses. The mother must take progesterone hormone replacement therapy and thyroid and adrenal hormones for the rest of her ­life. 28. Correct answer: ­A Vitamin D deficiency in the neonate may manifest itself as rickets. The neonate will have muscle weakness (rickety myopathy). This condition is also known as “floppy baby syndrome” or “slinky baby” because of lack of muscle tone. Babies with rickets are at high risk for ­f ractures. 29. Correct answer: ­B Neonates with rickets often have a wider than normal wrist, hypocalcemia, craniotubes, and costochondrial swelling. Muscle tetany may also be ­present. 30. Correct answer: ­C Rickets is likely to develop in neonates who are breastfed and not exposed to sunlight. Breastfed neonates whose mothers are not exposed to sunlight are at risk for developing rickets as well. If a baby is lactose intolerant or does not consume fortified milk, he or she is at risk for rickets. It is interesting to note that research has shown that infants with red hair have a decreased risk for rickets, probably because of their greater production of vitamin D in sunlight. Congenital rickets occurs if a mother has low vitamin D levels during ­pregnancy. 31. Correct answer: ­A The primary effects of aldosterone are to increase reabsorption of water and sodium, which results in an increased extracellular fluid volume. Renal excretion of potassium is i­ ncreased. 32. Correct answer: ­B Aldosterone is secreted by the zona glomerulus of the adrenal ­cortex. 33. Correct answer: ­A Aldosterone secretion is regulated by angiotensin II. When renal blood flow is decreased, angiotensin is released, and it stimulates aldosterone secretion. An increase in potassium ions in extracellular fluid will also stimulate the release of aldosterone. Adreno­ corticotropic hormone will stimulate aldosterone production for short periods of ­time.

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34. Correct answer: ­C A factor that suppresses aldosterone production is atrial natriuretic hormone. Hypercalcemia and hypokalemia also suppress aldosterone ­production. 35. Correct answer: ­C Cortisol raises the free amino acids in the serum by inhibiting collagen formation and protein synthesis. Cortisol stimulates gastric acid secretion, inhibits loss of sodium from the small intestines, acts as an antidiuretic hormone, and works with epinephrine and norepinephrine to increase blood ­pressure. 36. Correct answer: ­C An important nursing consideration when a patient is receiving prednisone is to monitor blood glucose levels. Prednisone has a similar action to cortisol and increases gluconeogenesis and promotes breakdown of lipids and proteins. This leads to increased circulating glucose concentrations in the blood. There is also an increased glycogen breakdown in the liver. Prolonged cortisol secretion causes hyperglycemia, so the glucose levels must be monitored ­diligently. 37. Correct answer: ­A The infant has failure to thrive and hepatomegaly, along with vomiting and diarrhea. Cataracts are noted and have an “oil droplet” appearance. These findings are highly suggestive for galactosemia. The baby will be hypoglycemic and have renal tubular acidosis. Galactose is present in all milk sources and must be metabolized to glucose for absorption from the intestine. If the condition is caught early, the baby can be managed on a ­galactose-­f ree diet. A neonate is especially susceptible to Escherichia coli septicemia, liver dysfunction, and bleeding ­tendencies. 38. Correct answer: ­B Galactosemia is a congenital disease transmitted as an autosomal recessive disorder. As an autosomal recessive disorder, the parents of a child with galactosemia are unaffected, healthy carriers of the condition and have one normal gene and one abnormal ­gene. 39. Correct answer: ­C A mild form of galactosemia that can be treated and tends to disappear after 1 year of life is known as Duarte galactosemia. An infant with Duarte galactosemia has one Duarte allele and one galactosemia gene (Dg). Most children with Duarte galactosemia develop normally and do not have medical complications, despite occasionally having some of the symptoms of classic galactosemia. After about a year of treatment (on average), once the child’s diet diversifies and the liver matures, treatment is ­discontinued. 40. Correct answer: ­C A disorder caused by a defect in copper excretion is known as Wilson’s disease. Copper is deposited in the liver, brain, heart, and eyes. Hepatic dysfunction is common, along with elevated serum and urine copper. An infant with Wilson’s disease may have seizures, dystonia, dysarthria, and tremors. Chelation therapy with oral penicillin and zinc salts may be prescribed. On occasion, a liver transplant may be ­necessary.

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41. Correct answer: ­A An ­X-­linked recessive disorder caused by abnormal copper transport is known as Menkes kinky hair disease. Usually, this disorder will develop in the first few months of life. Symptoms include pale, kinky hair, optic nerve atrophy, progressive neurologic degeneration, seizures, and death. Serum studies show low copper and ­ceruloplasmin. 42. Correct answer: ­B The lysosomal storage disease caused by glucocerebrosidase deficiency is known as Gaucher’s disease. This disorder is autosomal recessive and may be managed by enzyme replacement therapy. Symptoms include thrombocytopenia and hepatosplenomegaly. On X ­ -­ray, a classic “Erlenmeyer ­flask-­shape” of the distal femur is ­seen. 43. Correct answer: ­C Hexosaminidase A deficiency is known as ­Tay-­Sachs disease, an autosomal recessive disorder. The incidence of the disease is higher among Ashkenazi Jews. An infant with ­Tay-­Sachs disease often presents with macrocephaly, a ­cherry-­red macula, hypotonia, increased startle reflex, and motor weakness. Infantile ­Tay-­Sachs disease is untreatable, and death usually occurs by 4 years of ­age. 44. Correct answer: ­A An autosomal recessive disorder that is classified as a membrane transport defect that may lead to renal stones is cystinurea. This disorder is a defect in the renal reabsorption of cystine, arginine, lysine, and ornithine, and it causes renal ­stones. 45. Correct answer: ­B A disorder that is caused by a defect in transport of neutral amino acids is called Hartnup ­disease. An infant with Hartnup disease cannot absorb amino acids from the intestine and cannot reabsorb them properly from tubules in the kidneys. Excessive amounts of amino acids, especially tryptophan, are excreted in the urine. These symptoms tend to be more pronounced in times of stress. When a deficit of tryptophan occurs, the body is unable to make adequate amounts of the ­B -­complex vitamin niacinamide (niacin). 46. Correct answer: ­C Ornithine translocase deficiency is an ­X-­linked recessive deficiency and is the most common urea cycle defect. There are actually six enzymes in the urea cycle. These enzymes are ornithine transcarbamylase (OTC), argininosuccinase acid lyase (ALD), carbamyl phosphate synthetase (CPS), argininosuccinic acid synthetase (ASD), arginase (ARG), and ­N-­acetylglutamate synthetase (NAGS). These enzymes are found in the liver, where they process ­nitrogen-­containing waste products (such as ammonia) into urea. When the ammonia levels become too high, neurologic deficits and permanent damage will occur. An infant with ornithine translocase deficiency may exhibit lethargy, vomiting, seizures, coma, and death. Diagnosis is made from elevated urine orotic acid levels and decreased serum citrulline with increased ornithine ­levels.

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47. Correct answer: ­C Isabel is probably suffering from maple syrup urine disease. Her urine has the characteristic “burned sugar” or “sweet maple syrup” smell. Other symptoms include lethargy, poor feeding, muscle weakness, spasms, seizures, and poor weight gain. During acute episodes, the infant will exhibit hyperglycemia and profound ­acidosis. 48. Correct answer: ­B The effects of hypothyroxinemia in prematurity can be mitigated by using Betadine solutions sparingly on the skin of the infant. The solutions and ointments contain iodine and can easily be absorbed transdermally and affect thyroid ­f unction. 49. Correct answer: ­A Congenital hypothyroidism is manifested by an elevated TSH and a decreased T4. This disease is usually autosomal recessive, but some cases are autosomal dominant. Congenital hypothyroidism occurs when the thyroid gland fails to develop or function properly. The thyroid gland may be absent, hypoplastic, or displaced. In a few cases, the gland is enlarged, but the production of thyroid hormones is deficient or ­absent. 50. Correct answer: ­A Maternal thyroid disease can have a substantial influence on fetal and neonatal thyroid function because immunoglobulin G (IgG) autoantibodies can cross the placenta and inhibit thyroid function. Thioamides used to treat maternal hyperthyroidism can obstruct fetal thyroid hormone synthesis. Most of these effects are transient. Radioactive iodine administered to a pregnant woman can destroy the fetus’s thyroid ­gland. 51. Correct answer: ­C Physical findings of congenital hypothyroidism include macroglossia, pallor, enlarged fontanelles, cool, dry skin, umbilical hernias, and coarse facial features. Not all of the infants with congenital hypothyroidism display these characteristics. In addition, an infant with this condition may have an atrial or ventricular septal defect. Developmental delay is ­common. 52. Correct answer: ­B Physical findings in an infant with hyperthyroidism include hyperthermia, poor feeding, weight loss, sweating, tachycardia, and hypertension. In addition, the infant will exhibit congestive heart failure, craniosynostosis, vomiting, diarrhea, dehydration, and ­goiter. 53. Correct answer: ­A An infant with a large goiter should be positioned supine with the head slightly elevated and extended. Maintaining a patent airway is a nursing priority. The goiter may impinge on the trachea, so correct positioning is ­essential. 54. Correct answer: ­C Symptoms of Graves’ disease include pretibial myxedema (a reddish color thickening or lump on the shin), sweating, diarrhea, weight loss, and tremors. Additional signs may include exophthalmos and goiter. Increased eye tearing, muscle weakness, brittle hair and nails, and tachypnea are also signs of Graves’ ­disease.

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55. Correct answer: ­B Congenital adrenal hyperplasia (as a result of 21 hydroxylase hyperplasia) is the most common cause of ambiguous ­genitalia. 56. Correct answer: ­A Nonketotic hyperglycinemia (NKH) is an autosomal recessive disorder of glycine metabolism caused by a defect in the glycine cleavage enzyme complex (GCS). This disease is also known as glycine ­encephalopathy—­a disorder that affects neuron transmission of glycinergic receptors. Symptoms include hypotonia, lethargy that progresses to coma, seizures, and hiccups. An infant with this condition may develop apnea during the first 2 weeks of life and require ventilator support. Treatment is with sodium ­benzoate. 57. Correct answer: ­C Signs of vitamin D toxicity include azotemia, hypercalcemia, vomiting, and nephrocalcinosis. Vitamin D deficiency will result in hypocalcemia, increased susceptibility to respiratory diseases, lethargy, and ­seizures. 58. Correct Answer: ­C Crush injuries cause a massive release of potassium into the bloodstream. There is approximately 135–145 mEq of potassium in each ­cell. 59. Correct Answer: ­C The hypothalamic–pituitary axis releases a number of hormones that inhibit or release several hormones that affect body ­f unctions. 60. Correct Answer: ­A The pituitary gland is inferior to the hypothalamus and sits in the sella turcica of the sphenoid bone. The other locations are incorrect. The correct order descending from the cortex is the thalamus, hypothalamus, infundibulum, and pituitary ­gland. 61. Correct Answer: ­C The anterior pituitary gland produces numerous hormones that including TSH, LH, FSH, ACTH, and ­melanocyte-­stimulating hormone. Substance P is a hormone released by the hypothalamus. Vasopressin and oxytocin are released by the posterior pituitary ­gland. 62. Correct Answer: ­B The thyroid gland is located below the larynx on both sides of the ­trachea. 63. Correct answer: ­C This lab value indicates hyperthyroidism or thyrotoxicosis. Causes can include goiter, Graves’ disease, thyroid carcinoma, or ­TSH-­secreting pituitary adenoma. Hyperthyroidism without toxicosis is most often related to excessive intake of thyroid ­hormones. 64. Correct answer: ­A Treatment of thyrotoxic crisis includes PTH to decrease ­thyroid-­stimulating hormone and thyroid hormones plus symptomatic ­care.

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65. Correct Answer: ­B The pancreas is both an endocrine gland that secretes glucagon and insulin and an exocrine gland that secretes amylase and ­lipase. 66. Correct Answer: ­B The parathyroid glands are located posterior to the thyroid gland and may consist of 4–6 small glands. The parathyroid glands produce PTH (parathyroid hormone) that regulates serum calcium, magnesium, and phosphorus levels. PTH also stimulates the kidney to produce bioavailable vitamin ­D. 67. Correct answer: ­C The pancreas releases insulin in response to glucose. With diabetes, if the tissues are insulin resistant, insulin leaves remain high due to hyperglycemia. Insulin prohibits lipid breakdown and results in higher blood lipid concentration as tissues are unable to uptake glucose or fat cells able to store lipids. 68. Correct Answer: ­B Insulin secretion is controlled by chemicals such as glucose and amino acids; hormones such as GI hormones, prostaglandin; neuronal such as sympathetic response to increased ­glucose. 69. Correct answer: ­C Renin is stored in a crystalline form in the juxtaglomerular cells of the kidneys. When the kidneys perceive a decreased blood flow, the sympathetic response triggers the release of renin, which converts angiotensinogen to angiotensin I, a mild vasoconstrictor. If the problem is not resolved, the lungs release angiotensin I converting enzyme to create angiotensin II, a powerful vasoconstrictor. Angiotensin II triggers the release of aldosterone from the adrenal glands, which causes sodium and water retention, thereby increasing the blood ­pressure. 70. Correct answer: ­B Anything that causes a perceived drop in renal blood flow triggers the renin–angiotensin mechanism. Renin release can also be triggered by sodium and volume depletion such as seen with diuretic ­use. 71. Correct answer: ­C The kidneys perceive a drop in blood flow leading to the release of renin from the juxtaglomerular cells of the nephron. This stimulates the release of angiotensinogen, which stimulates the release of angiotensin I. If the blood pressure is not corrected, the lungs release angiotensin ­I-­converting enzyme; that, in turn, causes the secretion of angiotensin II. Angiotensin II causes the release of aldosterone from the adrenal glands, causing the retention of sodium and water and increased blood ­pressure. 72. Correct answer: ­C The anterior pituitary or adenohypophysis controls the function of the thyroid gland with TSH (thyroid stimulating hormone), the adrenal cortex with ADH (antidiuretic hormone), and the gonads with LH (luteinizing) or ICSH (interstitial ­cell-­stimulating hormone).

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73. Correct answer: ­B ADH (antidiuretic hormone) is controlled by a negative feedback system. Production is decreased when the osmoreceptors of the hypothalamus recognize hemodilution or hemoconcentration and adjust ADH production ­accordingly. 74. Correct Answer: ­A The decreased T4 leads to hypothyroid symptoms of cold, dry skin, hair loss, periorbital edema, and possible thyroid ­enlargement. 75. Correct answer: ­C The anterior pituitary gland secretes TSH ­(thyroid-­stimulating hormone), and the hypothalamus regulates the anterior pituitary gland with ­thyrotropin-­releasing hormone (TRH). 76. Correct answer: ­C Injury or infection as well as manipulation of the thyroid gland can trigger a thyroid storm and ­thyrotoxicosis. 77. Correct Answer: ­C Thyrotoxic crisis symptoms include hypertension, hyperthermia, flushing, tachycardia (especially atrial tachyarrhythmia), ­high-­output heart failure, nausea and vomiting, psychosis, and delirium. Treatment includes supportive care and medications to block catecholamine ­effects. 78. Correct answer: ­C At birth, an infant should have a serum blood glucose level equal to 80% of the mother’s blood glucose level. This is important in determining and preparing for potential glucose instability. If maternal glucose prior to delivery is only 50 mg/dl, then the neonate’s glucose level should be 40 mg/dl and should be monitored ­closely. 79. Correct answer: ­A Infants with DiGeorge syndrome may have hypocalcemia because of absent parathyroid ­glands. 80. Correct answer: ­A Carnitine deficiency may result in a hyperinsulinemia state. Carnitine is needed to break down triglycerides into energy. In the absence of fat as a source of fuel, the body continually burns glycogen, resulting in hyperinsulinemia and hypoglycemia. Some premature infants are unable to efficiently produce sufficient carnitine, and supplementation with ­l-­carnitine may be ­necessary. 81. Correct answer: ­C An infant presenting with a bilirubin level of 30 at 3 days of life should be prepared for umbilical line insertion and exchange transfusion to prevent or limit kernicterus. Phototherapy and fluids are also recommended after an exchange ­transfusion.

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82. Correct answer: ­A Breastfeeding, as compared with formula feeding, is associated with higher peak serum bilirubin levels due to volume ­intake. 83. Correct answer: ­B If an infant is large for gestational age, the nurse should assess for hypoglycemia. It is likely that the mother is diabetic (type 1, type 2, or gestational), which resulted in a hyperglycemic state in the infant. Fetal response to hyperglycemia is hyperinsulinemia. At birth, the high glucose level is no longer present, but pancreatic response is delayed with continued hyperinsulinemia and resulting ­hypoglycemia. 84. Correct answer: ­A If a newborn is less than 24 hours old, treatment for hypoglycemia should begin when the serum glucose level is at 35 mg/dl. Initial hypoglycemia is expected due to the rapid glucose metabolism immediately postdelivery. Oral nutrition and compensatory mechanisms in healthy, term infants should prevent the glucose level from dropping below 35 m ­ g/dl.

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Endocrinology ­References Adam, A. C., ­Rubio-­Texeira, M., & Polaina, J. (2004). Lactose: The milk sugar from a biotechnological perspective. Critical Reviews in Food Science and Nutrition, 44(7/8), 553–557. Ala, A., Walker, A. P., Ashkan, K., et al. (2007). Wilson’s disease. The Lancet, 369(9559), 397–408. Andresen, C., Moalli, M., Turner, C., et al. (2008). Bone parameters are improved with intermittent dosing of vitamin D3 and calcitonin. Calcified Tissue International, 83(6), 393–403. Balasubramanian, S., & Ganesh, R. (2008). Vitamin D deficiency in exclusively ­breast-­fed infants. Indian Journal of Medical Research, 127(3), 250–255. Bosch, A. M. (2006). Classical galactosaemia revisited. Journal of Inherited Metabolic Disease, 29(4), 516–525. Bosch, A. M., Ijlst, L., Oostheim, W., et al. (2005). Identification of novel mutations in classical galactosemia. Human Mutation, 25(5), 5 ­ 02. Brodsky, D., & Martin, C. (2003). Neonatology review. Philadelphia, PA: Hanley & ­Belfus. Calonge, N., Teutsch, S., & Botkin, J. (2008). Expanding newborn screening: Process, policy, and priorities. The Hastings Center Report, 38(3), 32–39. Chachkin, C. (2007). What potent blood: ­Non-­invasive prenatal genetic diagnosis and the transformation of modern prenatal care. American Journal of Law and Medicine, 33(1), 9–53. Cheung, M., & Glorieux, F. (2008). Osteogenesis imperfecta: Update on presentation and management. Reviews in Endocrine & Metabolic Disorders, 9(2), 153–160. Chiruvolu, A., Engle, W., Sendelbach, D., et al. (2008). Serum calcium values in term and ­late­preterm neonates receiving gentamicin. Pediatric Nephrology, 23(4), 569–574. Cloherty, J. P., Eichenwald, E. C., & Stark, A. R. (2004). Manual of neonatal care (5th ed.). Philadelphia, PA: ­Lippincott. Crenner, C. (2008). The troubled dream of genetic medicine: Ethnicity and innovation in ­Tay-­Sachs, cystic fibrosis, and sickle cell disease. Journal of the History of Medicine and Allied Sciences, 63(1), 124–126. Cunningham, F. G., Leveno, K. L., Bloom, S. L., et al. (2005). Obstetrical hemorrhage. In F. G. Cunningham, K. L. Leveno, S. L. Bloom, et al. (Eds.), Williams obstetrics. (22nd ed.). New York, NY: McGraw-­Hill. Ensenauer, R. E., Michels, V. V., & Reinke, S. S. (2005). Genetic testing: Practical, ethical, and counseling considerations. Mayo Clinic Proceedings, 80(1), 63–73. Erdeve, M., Atasay, B., Arsan, S., et al. (2007). Hypocalcemic seizure due to congenital rickets in the first day of life. The Turkish Journal of Pediatrics, 49(3), 301–303. Figueiredo, B., & Costa, R. (2009). Mother’s stress, mood and emotional involvement with the infant: 3 months before and 3 months after childbirth. Archives of Women’s Mental Health, 12(3), 143–153. Fingerhut, R., Simon, E., Maier, E., et al. (2008). Maple syrup urine disease: Newborn screening fails to discriminate between classic and variant forms. Clinical Chemistry, 54(10), 1739–1741. Grabowski, G. (2008). Lysosomal storage disease 1: Phenotype, diagnosis, and treatment of Gaucher’s disease. The Lancet, 372(9645), 1263–1271. Halliday, H. L., McClure, B. G., & Reid, M. (2002). Handbook of neonatal intensive care (4th ed.). Philadelphia, PA: ­Saunders. Hay, W. W., Levin, M. J., et al. (2007). Current diagnosis and treatment in pediatrics (18th ed.). New York: McGraw-­Hill. Hernan, A., Lopez, M., Debes, J. D., & Dickstein, G. (2007). Wilson’s disease: What lies beneath. Digestive Diseases and Sciences, 52(4), 941–942. Jones, K. L. (2006). Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: ­Elsevier.

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300  y  Section 10 Kalelioglu, I., Uzum, A. K., Yildirim, A., et al. (2007). Transient gestational diabetes insipidus diagnosed in successive pregnancies: Review of pathophysiology, diagnosis, treatment, and management of delivery. Pituitary, 10(1), 87–93. Karlsen, K. (2006). The S.T.A.B.L.E. program (5th ed.). Park City, UT: American Academy of ­Pediatrics. Karlsen, K., & Tani, L. Y. (2003). S.T.A.B.L.E.—Cardiac module. Park City, UT: American Academy of ­Pediatrics. Kattwinkle, J. (Ed.). (2006). Neonatal resuscitation textbook (5th ed.). Elk Grove Village, IL: American Academy of Pediatrics and American Heart ­A ssociation. Kenner, C., Amlung, S., & Flandermeyr, A. (1998). Protocols in neonatal nursing. Philadelphia, PA: ­Saunders. Kenner, C., & Lott, J. (2004). Neonatal nursing handbook. St. Louis, MO: ­Elsevier. Kenner, C., & Lott, J. (2007). Comprehensive neonatal care (4th ed.). St. Louis, MO: ­Elsevier. Kenner, C., & McGrath, J. M. (Eds.). (2004). Developmental care of newborns & infants: A guide for health professionals. St. Louis, MO: M ­ osby. Knoers, N. (2009). Inherited forms of renal hypomagnesemia: An update. Pediatric Nephrology, 24(4), 697–705. Lambert, C., & Boneh, A. (2004). The impact of galactosaemia on quality of life—­A pilot study. Journal of Inherited Metabolic Disease, 27(5), 601–608. Lewis, A., Courtney, C., & Atkinson, A. (2009). All patients with ‘idiopathic’ hypopituitarism should be screened for hemochromatosis. Pituitary, 12(3), 273–275. MacDonald, M. G., & Ramasethu, J. (2007). Atlas of procedures in neonatology (4th ed.). Philadelphia, PA: Lippincott ­Williams. Mak, C., Lam, C., & Tam, S. (2008). Diagnostic accuracy of serum ceruloplasmin in Wilson disease: Determination of sensitivity and specificity by ROC curve analysis among ­ATP7B­genotyped subjects. Clinical Chemistry, 54(8), 1356–1362. Malee, M. P. (2007). Pituitary and adrenal disorders in pregnancy. In S. G. Gabbe, J. R. Niebyl, & J. L. Simpson (Eds.), Obstetrics—­Normal and problem pregnancies (5th ed.). Philadelphia, PA: Elsevier Churchill ­Livingstone. Markiewicz, M., & Abrahamson, E. (1999). Diagnosis in color: Neonatology. Philadelphia, PA: ­Mosby. Merenstein, G. B., & Gardner, S. L. (2006). Handbook of neonatal intensive care (6th ed.). St. Louis, MO: ­Mosby. Minniti, G., Gilbert, D., & Brada, M. (2009). Modern techniques for pituitary radiotherapy. Reviews in Endocrine & Metabolic Disorders, 10(2), 135–144. Ng, P., Lee, C., Lam, C., et al. (2004, March). Transient adrenocortical insufficiency of prematurity and systemic hypotension in very low birthweight infants. Archives of Disease in Childhood—Fetal & Neonatal Edition, 89(2), F119–126. Packman, W., Henderson, S., Mehta, I., et al. (2007). Psychosocial issues in families affected by maple syrup urine disease. Journal of Genetic Counseling, 16(6), 799–809. Polin, R. A., & Spritzer, A. R. (2007). Fetal & neonatal secrets (2nd ed.). Philadelphia, PA: ­Mosby. Poomthavorn, P., Lertbunrian, R., Preutthipan, A., et al. (2009). Serum free cortisol index, free cortisol, and total cortisol in critically ill children. Intensive Care Medicine, 35(7), 1281–1285. Potter, S. J., Lu, A., Wilcken, B., et al. (2002). Hartnup disorder: Polymorphisms identified in the neutral amino acid transporter SLC1A5. Journal of Inherited Metabolic Disease, 25(6), 437–448. Prescribing Reference. (2009, Summer). NPPR: Nurse Practitioner’s Prescribing Reference, 16(2). Rennie, J. M., & Roberton, N. R. C. (2002). A manual of neonatal intensive care (4th ed.). New York, NY: A ­ rnold. Sanchez, C. (2008). Mineral metabolism and bone abnormalities in children with chronic renal failure. Reviews in Endocrine & Metabolic Disorders, 9(2), 131–137.

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Endocrinology  y  301 Sarkar, S., Hagstrom, N. J., Ingardia, C. J., et al. (2005). Prothrombotic risk factors in infants of diabetic mothers. Journal of Perinatology, 25(2), 134–138. Sarkissian, C., Gámez, A., & Scriver, C. (2009). What we know that could influence future treatment of phenylketonuria. Journal of Inherited Metabolic Disease, 32(1), 3–9. Simon, E., Fingerhut, R., Baumkötter, J., et al. (2006). Maple syrup urine disease: Favourable effect of early diagnosis by newborn screening on the neonatal course of the disease. Journal of Inherited Metabolic Disease, 29(4), 532–537. Summar, M. L., Dobbelaere, D., Brusilow, S., & Lee, B. (2008). Diagnosis, symptoms, frequency and mortality of 260 patients with urea cycle disorders from a 21-year, multicentre study of acute hyperammonaemic episodes. Acta Paediatrica, 97, ­1420. Suvarna, J., & Hajela, S. (2008). C ­ herry-­red spot. Journal of Postgraduate Medicine, 54(1), 54–57. Taeusch, H. W., Ballard, R. A., & Gleason, C. A. (2005). Avery’s diseases of the newborn (8th ed.). Philadelphia, PA: ­Elsevier. Tezer, H., Siklar, Z., Dallar, Y., & Dogankoç, S. (2009). Early and severe presentation of vitamin D deficiency and nutritional rickets among hospitalized infants and the effective factors. The Turkish Journal of Pediatrics, 51(2), 110–115. Transient adrenocortical insufficiency of prematurity and systemic hypotension in very low birthweight infants. Archives of Disease in Childhood—­Fetal and Neonatal Edition, 89(2), F119–F126. Vandergheynst, F. & Decaux, G. (2008). Lack of elevation of urinary albumin excretion among patients with chronic syndromes of inappropriate antidiuresis. Nephrology, Dialysis, Transplantation, 23(7), 2399–2401. Verklan, M. T., & Walden, M. (Eds.). (2004). Certification and core review for neonatal intensive care nursing (3rd ed.). St. Louis, MO: E ­ lsevier. Verklan, M. T., & Walden, M. (Eds.). (2010). Certification and core review for neonatal intensive care nursing (4th ed.). St. Louis, MO: ­Elsevier. Young, T. E., & Magnum, B. (2009). Neofax 2009. Montvale, NJ: Thomson ­Reuters. Zaritzky, M., Ben, R., Zylberg, G., & Yampolsky, B. (2009). Magnetic compression anastomosis as a nonsurgical treatment for esophageal atresia. Pediatric Radiology, 39(9), 945–949.

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Section 11 Genetics

Questions 1. Which of the following statements is true regarding ­X-­linked recessive ­disorders? A. Fathers cannot transmit the disease to ­sons B. ­X-­linked recessive disorders are usually evident in ­females C. Fragile X syndrome is an ­X-­linked recessive ­disorder 2. Conditions that lead to an absent or weakened suck ­include A. Choanal atresia and ­dysautonomia B. Micrognathia and cleft ­palate C. Maternal anesthesia and ­hypothyroidism 3. Down syndrome is also known ­as A. Edward ­syndrome B. Trisomy ­21 C. Patau s­ yndrome 4. The most common ­single-­gene disorder (autosomal recessive) in Caucasian populations i­ s A. Adult polycystic kidney ­disease B. Cystic ­fibrosis C. ­Retinoblastoma 5. An example of an autosomal dominant disorder would ­be A. Sickle cell ­disease B. Fragile X ­syndrome C. Adult polycystic kidney ­disease 6. Which of the following statements is true regarding the use of karyotyping in genetic ­screening? A. Obtaining results from karyotyping will take only 3–4 ­days B. Karyotyping is done in ­utero C. Karyotyping will provide a complete genetic ­map 7. Genetic disorders associated with advanced maternal age ­include A. Marfan syndrome, achondroplasia, Apert’s ­syndrome B. Osteogenesis imperfecta, Treacher Collins syndrome, and Waardenburg’s ­syndrome C. Trisomy 21, trisomy 13, Klinefelter’s ­syndrome

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8. Cleft lip and/or palate is usually associated w ­ ith A. DiGeorge syndrome, Pierre Robin sequence, Trisomy ­13 B. Trisomy 21, ­Prader-­Willi syndrome, ­Russell-­Silver ­syndrome C. Carpenter syndrome, Klinefelter’s syndrome, ­Holt-­Oram ­syndrome 9. Syndactyly may be defined ­as A. A gap or malformation of eye ­tissue B. A fusion of ­digits C. A malformation of the ­tongue 10. Your patient is a 34-week gestation infant who is thought to have a trisomy 18 defect. Which of the following characteristics would you expect this infant to ­present? A. Cleft lip and palate and grand mal ­seizures B. ­Low-­set ears and low birth ­weight C. Malformed ears and ­cataracts 11. VATER is an acronym for which of the following ­anomalies? A. Tracheoesophageal, anal, vertebral ­anomalies B. Ventricular septal defect, abdominal, tricuspid ­anomalies C. Vertebral, auditory, tricuspid ­anomalies 12. Your male patient has a genetic disorder and the mother is a carrier. If the father is not a carrier and the mother’s two brothers do not have the disorder, this disorder must be ­an A. Autosomal dominant ­disorder B. Autosomal recessive ­disorder C. ­X-­linked recessive ­disorder 13. A major goal of genetic counseling ­is A. Reinforcement of information previously ­presented B. Emphasis on ­communication C. ­Diagnosis 14. Different causes of genetic disorders that may produce similar characteristics are ­called A. ­A ssociations B. ­Malformations C. Genetic ­heterogeneity 15. Your patient has blue eyes and brown hair. These visible characteristics are known a ­s A. Mendelian ­traits B. ­Phenotypes C. ­Genotypes

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16. Information that has been coded in genetic material and is inherited by an individual is ­a A. ­Genotype B. ­Locus C. ­Phenotype 17. If one individual carries two different cell lines, this is known ­as A. ­Polyploidy B. ­Mosaicism C. ­Polygenic

Questions 18–22 are all related to genetic screening ­tests. 18. Which of the following substances is produced by the ­placenta? A. Unconjugated estriol (uE3) B. ­A lpha-­fetoprotein (AFP) C. Human chorionic gonadotropin (hCG) 19. A neighbor comes to you because she knows you work in a NICU at a local hospital. She is very anxious and says she has to have screening tests for her fetus because of birth ­defects. Your best response would be to tell her ­that A. A screening test is not a diagnostic ­test. B. Her baby must have a 1:10 chance of an ­abnormality C. The odds are with her that she will have a healthy ­baby 20. Low levels of unconjugated estriol and hCG may ­indicate A. Trisomy ­13 B. Trisomy ­18 C. Trisomy ­21 21. If the mother has higher than normal levels of AFP present during prenatal testing, this result could ­indicate A. Paget’s ­disease B. Trisomy ­21 C. An open neural tube ­defect 22. If hCG and inhibin A levels are high, this might be an indication of which of the following genetic ­defects? A. Marfan ­syndrome B. Trisomy ­21 C. Gaucher ­disease

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23. Type I methemoglobinemia is an example of ­an A. ­X-­linked recessive ­disorder B. Autosomal dominant ­disorder C. Autosomal recessive ­disorder 24. Symptoms of type II methemoglobinemia (generalized reductase deficiency) ­include: A. Developmental delay and failure to ­thrive B. ­PPHN C. Severely low arterial oxygen ­levels 25. Loss of a chromosomal segment is known ­as A. ­Mosaicism B. ­Disjunction C. ­Deletion 26. Which of the following conditions is an autosomal recessive ­disorder? A. ­Phenylketonuria B. Cystic ­fibrosis C. Adult polycystic kidney ­disease 27. Which of the following disorders could have its origins in ­mosaicism? A. Turner ­syndrome B. Potter’s ­syndrome C. Kyle’s ­syndrome 28. Which of the following syndromes is also known as the 47, XXY ­syndrome? A. Turner ­syndrome B. Klinefelter’s ­syndrome C. Down s­ yndrome 29. A 37-week gestation female infant is admitted to your NICU. Your initial assessment reveals a webbed neck, swollen hands and feet, arms that turn out at the elbow, and l­ ow-­set ears and a receding lower jaw. Based on this initial assessment, which chromosomal defect do you suspect this infant may ­have? A. Down ­syndrome B. Turner ­syndrome C. Cystic ­fibrosis 30. The phenomenon by which a single mutant allele can have widespread effects or expression is known ­as A. ­Penetrance B. ­Heterogeneity C. ­Pleiotropy

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31. The proportion of individuals with a particular genotype that express the same phenotype is known ­as A. ­Homogeneity B. ­Penetrance C. A profound hereditary ­pattern 32. The variability in degree (severity) of phenotypic expression seen in individuals with the same mutant genotype is known ­as A. ­Segregation B. ­Homogeneity C. ­Expressivity 33. A number of different genetic mutations may produce phenotypes that are similar or identical and result in a broad terminology for a diagnosis. This is ­called A. Genetic ­heterogeneity B. ­Homozygous C. ­Segregation 34. A phenotype causing different mutations at a single gene level is ­called A. Allelic ­heterogeneity B. Independent ­assortment C. ­Heterozygous 35. When gene pairs are separated during gamete formation, the process is known ­as A. Autosomal dominant ­separation B. ­Dominance C. ­Segregation 36. When the segregation of different alleles occurs independently, this is known ­as A. ­Heterozygosity B. Independent ­assortment C. Recombinant ­processing 37. A technique that labels a known chromosome sequence with DNA probes attached to fluorescent dyes is known ­as A. ­FISH B. ­CSFSD C. ­GOAL 38. The study of genetics at the chromosome level is known ­as A. ­Tropism B. ­Genomics C. ­Cytogenetics

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39. If more than one phenotype is caused by different allelic mutations at a single site (locus), the condition is ­called A. Phenotypic ­heterogeneity B. Locus h ­ eterogeneity C. Mendelian ­placement 40. Birth defects that result from altered genetic or developmental causes are referred to a ­s A. Dysmorphic ­defects B. ­Sequences C. ­Malformations 41. Cri du Chat syndrome is characterized ­by A. Lethargy and failure to ­thrive B. A ­cat-­like cry and ­microcephaly C. Hypercalcemia and ­hypospadia 42. Micrognathia, a cleft lip and palate, along with a large, protruding tongue, are classic characteristics ­of A. Turner ­syndrome B. Trisomy ­13 C. Pierre Robin ­syndrome 43. A small, triangular face and a normal head circumference with short stature is characteristic ­of A. ­Russell-­Silver ­syndrome B. Trisomy ­18 C. Williams ­syndrome 44. Your patient is a term infant who appears to be short statured, yet has no discernible skeletal abnormalities. This infant also has a single eyebrow. You suspect this infant h ­ as A. Williams ­syndrome B. ­Ehlers-­Danlos ­syndrome C. Cornelia de Lange ­syndrome 45. A clinical presentation of “elfin facies”, a flat nasal bridge and round cheeks, are all characteristics ­of A. Down ­syndrome B. Williams ­syndrome C. CHARGE a ­ ssociation 46. Severe oligohydramnios that causes lung hypoplasia and fetal compression with limb abnormalities are classic congenital abnormalities manifested in infants w ­ ith A. Amniotic band ­syndrome B. Potter’s ­syndrome C. Klinefelter’s ­syndrome

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47. Characteristics of an infant with fetal alcohol syndrome would ­include A. Microcephaly and ventricular septal ­defect B. Hypocalcemia and ­hypokalemia C. Lethargy and increased ­appetite 48. A medial bone abnormality such as a short ulna or tibia is c ­ alled A. Skeletal ­hypoformatting B. ­Acromelia C. ­Mesomelia 49. Rhizomelia is characterized ­by A. A distal abnormality such as small hands and ­feet B. A proximal long bone abnormality such as a short humerus and ­femur C. Midline ­defects 50. Your patient is a 37-week gestation male at 4 days of life. He has had vomiting and diarrhea for the past 2 days and just had a generalized seizure. A stat serum glucose reveals a blood sugar of 30. The edge of his liver is now palpated at the pelvis. A blood culture reveals the presence of Escherichia coli. This infant most likely is suffering ­from A. Noonan ­syndrome B. Gaucher’s ­disease C. ­Galactosemia 51. Your term patient was admitted an hour ago to the NICU with aortic regurgitation. Additional clinical findings are elongated extremities and fingers, kyphosis, a decreased ­upper-­to-­lower segment ratio, and retinal detachment. This infant probably h ­ as A. Gaucher’s ­disease B. DiGeorge s­ yndrome C. Marfan ­syndrome

This concludes the Genetics questions.

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Genetics ­Answers 1. Correct answer: ­A Females have two copies of the X chromosome; males have only one. The ­X-­linked recessive diseases are usually present in males. A male can only pass one X chromosome to a son. Therefore, the father cannot pass the disease to the son because the son must have XY chromosomes. Fragile X syndrome is an ­X-­linked dominant ­disorder. 2. Correct answer: ­C Additional causes of a weakened or absent suck may include maternal anesthesia, hypothyroidism, hypoxia, congenital syndromes, neuromuscular disorders, sepsis, and prematurity. Answers A and B are conditions of disordered ­swallowing. 3. Correct answer: ­B There is a risk of Down syndrome with advanced maternal age, but about 75% of children with trisomy 21 are born to mothers less than 35 years ­old. 4. Correct answer: ­B The most common ­single-­gene disorder (autosomal recessive) in Caucasian populations is cystic fibrosis. Autosomal recessive inheritance means that two copies of an altered gene located on one of the autosomes (that is, not the X or Y chromosomes) must be present for an individual to be affected with the trait. The parents would both have the gene pattern ­R r—­this is a ­heterozygote. An affected individual would get an rr pattern—­this is a ­homozygote. The risk of two heterozygotes, or carriers, having an affected child is 25% (1 in 4), for each child who they have. There is a 3 in 4 chance that each child will not be ­affected. Males and females are at equal risk for being ­affected. Two affected individuals usually produce children who are all ­affected. 5. Correct answer: ­C An example of an autosomal dominant disorder would be adult polycystic kidney ­disease. Autosomal dominant inheritance means that the gene carrying a mutation is located on one of the autosomes. This means that males and females are equally likely to inherit the mutation. Dominant means that having a mutation in just one of the two copies of a particular gene is all it takes for a person to have a trait. So, if the infant is Dd, the trait will be expressed. The trait will not be expressed if the infant is ­dd. An important characteristic of dominant gene mutations is that they can have variable expression. This means that some people have milder or more severe symptoms than others. In addition, which systems of the body the mutation affects can vary as can the age at which the disease starts, even in the same ­family. Dominant gene mutations can have reduced penetrance. This means that sometimes a person can have a dominant mutation but not show any signs of disease. The concept of reduced penetrance is particularly important in the case of autosomal dominant cancer susceptibility genes. Sometimes, breast cancer occurs eventually in all or most generations in a family. This has led to genetic testing and to some women having mastectomies even if they do not presently exhibit the ­cancer.

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6. Correct answer: ­C Karyotyping is a physical type of analysis. Cells are cultured and allowed to grow until the point that chromosomes are most clearly visible. The growth of the cells is then arrested, and staining and photographing the chromosomes is done. The chromosomes are then arranged in corresponding pairs according to function and size. With a complete map of genetic material, it is possible to determine whether a fetus’ chromosomes exist in the correct number, shape, and size. An abnormal number of certain chromosomes will indicate a genetic disorder. Karyotyping typically takes 10–12 days but may take up to 3 w ­ eeks. 7. Correct answer: ­C Genetic disorders associated with advanced maternal age include trisomy 21, trisomy 13, and Klinefelter syndrome. All the possible selections in answers A and B are associated with advanced paternal age. These disorders also carry an increased risk of new ­mutations. 8. Correct answer: ­A Cleft lip and/or palate is usually associated with DiGeorge syndrome, Pierre Robin sequence and trisomy 13. All the answers for B and C are associated with clinodactyly, a curving or deviation of a ­finger. 9. Correct answer: ­B Syndactyly may be defined as a fusion of digits. The fusion of the digits may or may not involve the bones. Answer A is the definition of coloboma. Answer C is a definition that could include ­macroglossia. 10. Correct answer: ­B Trisomy 18 severely affects all organ systems. The physical appearance of the infant at birth will suggest the diagnosis of trisomy 18. However, most babies are diagnosed before birth by amniocentesis. Ultrasounds of the heart and abdomen can detect abnormalities, as can ­X-­rays may detect deformities of the ­skeleton. The following areas may be ­affected: Nervous system and brain—­mental retardation and delayed development, high muscle tone, and physical malformations such as brain ­defects Head and face—­small head, small eyes, ­wide-­set eyes, small lower jaw, ­low-­set e­ ars Heart—­ventricular septal defect and patent ductus ­arteriosus Bones—­severe growth retardation, clenched hands with 2nd and 5th fingers on top of the others, unfolded ­thumb Digestive tract—­malformations Urinary tract—­malformations Genitals—­malformations 11. Correct answer: ­A VATER association refers to birth defects that show a related relationship between three physical areas. The letter V represents the vertebrae and abnormalities of the spinal column. The A represents anal abnormalities, such as atresia. The TE represents an abnormal connection between the esophagus and the trachea or a missing section of those areas. The R refers to renal, or kidney anomalies or abnormalities of the radius.

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No one is certain when these abnormalities develop. However, it is thought abnormalities takes place in the 3rd week of ­pregnancy. VACTERL association is another acronym for these birth ­defects. V stands for vertebrae, A stands for imperforate anus or anal atresia, or an anus that does not open to the outside of the body. C is added to the acronym to denote cardiac ­anomalies. TE stands for tracheoesophageal fistula, R stands for renal or kidney anomalies. The L is for limb ­abnormalities. 12. Correct answer: ­C The patient is male and has the disorder. The mother has two copies of the X chromosome. She is a carrier because the disorder is recessive and is on both her ­chromosomes. 13. Correct answer: ­C Answers A and B are principles of genetic counseling. Additional principles are nondirective communication and are grounded in diagnosis and pattern of inheritance. Actual goals of genetic counseling include diagnosis, determining the role of heredity, assessing recurrence risks and options, possible responses, and communication with the primary care ­physician. 14. Correct answer: ­C Different causes of genetic disorders that may produce similar characteristics are called genetic heterogeneity. Examples of genetic heterogeneity are cleft lip, cleft palate, and hydrocephalus. Association is a nonrandom occurrence that does not represent a sequence and occurs in two or more individuals. Malformations are problems within developing ­structures. 15. Correct answer: ­B A phenotype is what you can observe about your patient (or any organism). The phenotype is comprised of the atoms, molecules, macromolecules, cells, structures, metabolism, energy utilization, tissues, organs, reflexes, and behaviors—­anything that is part of the observable structure, function, or behavior of your ­patient. 16. Correct answer: ­A Information that has been coded in genetic material and is inherited by an individual is a genotype. A genotype is an organism’s genetic makeup, even if it is not ­expressed. 17. Correct answer: ­B If one individual carries two different cell lines, it is known as mosaicism. Mosaicism occurs during mitosis (after fertilization) and results in two different cell lines in the same person. Selected disorders include hemophilia A and B, Marfan syndrome, Duchenne muscular dystrophy, neurofibromatosis type I and type II, and ­pseudoachondroplasia.

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18. Correct answer: ­C Human chorionic gonadotropin is a hormone produced by the placenta. Levels usually rise in the first trimester, then fall to less than 10% during the rest of the pregnancy. Inhibin A is also produced by the placenta. ­A lpha-­fetoprotein is a protein produced by fetal tissue. Unconjugated estriol is a form of estrogen produced by the ­fetus. 19. Correct answer: ­A A screening test is not a diagnostic test. Even if there is an abnormal result, it will not necessarily mean an abnormality exists. However, it might mean that a more specific diagnostic test might be necessary to rule out abnormalities. Answers B and C are not therapeutic and will add to the mother’s ­anxiety. 20. Correct answer: ­B If low levels of hCG and unconjugated estriol are detected along with variable AFP levels, this is an indication for the presence of trisomy ­18. 21. Correct answer: ­C Higher than normal levels of AFP are usually due to an open neural tube defect. An opening may exist in the head, spine, or abdominal wall, and this leak allows AFP to pass into the mother’s ­circulation. 22. Correct answer: ­B If the hCG and inhibin A levels are high and AFP and unconjugated estriol levels are low, this would indicate a possibility of trisomy 21. Please remember that all these tests are based on an accurate estimate of gestational age. If the gestational age is not accurate, all results may be falsely elevated or too ­low. 23. Correct answer: ­C Type I methemoglobinemia is an autosomal recessive disorder. This means it is passed on by both parents who do not have the condition themselves but carry the gene for the condition. This is due to a problem with an enzyme called cytochrome b5 reductase. There are two types of autosomal recessive methemoglobinemia. Type 1, also called erythrocyte reductase deficiency, occurs when red blood cells lack the enzyme. Type 2, also called generalized reductase deficiency, occurs when the enzyme doesn’t function anywhere in the ­body. 24. Correct answer: ­A Mental retardation, seizures, failure to thrive, and developmental delay are all symptoms of type II methemoglobinemia. Type II methemoglobinemia is much more serious and usually causes death within the first few years of life. Arterial blood gases and pulse oximetry results will be ­normal. 25. Correct answer: ­C A deletion, by definition, is loss of a chromosomal ­segment.

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26. Correct answer: ­A Phenylketonuria is an autosomal recessive disorder in which mutations result in a defect of the enzyme that catalyzes the first step in the metabolism of phenylalanine. This results in accumulation of phenylalanine and a disease whose characteristics include severe ­retardation. 27. Correct answer: ­A Mosaicism, such as is seen with Turner syndrome, is a condition in which cells within the same person have a different genetic makeup. This condition can affect skin cells, egg and sperm cells, and blood cells. Other examples of mosaicism include mosaic and Klinefelter’s ­mosaic. 28. Correct answer: ­B As babies, 47, XXY males with Klinefelter’s syndrome tend to be quiet and undemanding. They may have long limbs, fifth finger clinodactyly, hypogonadism, gynecomastia, cryptorchidism, and hypospadia. Advanced maternal age plays a small part in this condition, but advanced paternal age may lead to production of more XY ­sperm. 29. Correct answer: ­B The infant with Turner syndrome will have monosomy—­the loss of all or part of one copy of the X chromosome. Often, the paternal X chromosome is missing. Turner’s syndrome is a condition associated with small stature, narrow palate, ­low-­set ears, hearing loss, ptosis, and a broad nasal bridge. An infant with Turner syndrome may also have a broad chest with ­wide-­spaced nipples, pectus excavatum, lymphadema of the extremities, and insufficient circulating concentrations of female sex steroids. The infant may also be infertile and have normal intelligence. Diagnosis is based on phenotype combined with a genotype that includes partial or complete ­X-­chromosome monosomy (45,X). 30. Correct answer: ­C The phenomenon by which a single mutant allele can have widespread effects or expression is known as pleiotropy. This expression may involve different tissues or organ systems. The allele may produce more than one effect on the phenotype. When the fibrillin gene contains a single mutation, Marfan syndrome can result, and the allele produces effects on the multiple ­systems. 31. Correct answer: ­B The proportion of individuals with a particular genotype that expresses the same phenotype is known as penetrance. If 100% of the individuals in a population expresses the phenotype, the penetrance is complete. If some of the mutant individuals do not express the phenotype, the penetrance is incomplete or ­reduced. 32. Correct answer: ­C The variability in degree (severity) of phenotypic expression seen in individuals with the same mutant genotype is known as expressivity. Expression can vary widely, even within the same ­family.

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33. Correct answer: ­A A number of different genetic mutations may produce phenotypes that are similar or identical and result in a broad terminology for a diagnosis. This is called genetic heterogeneity. An example would be ­anemia. 34. Correct answer: ­A A phenotype causing different mutations at a single gene level is called allelic heterogeneity. For example, cystic fibrosis may be caused by several different genetic changes. If a person is homozygous for two or more mutations, it is called compound ­heterozygosity. 35. Correct answer: ­C When gene pairs are separated during gamete formation, this process is known as segregation. Each gamete should receive one copy of each gene (allele). 36. Correct answer: ­B When the segregation of different alleles occurs independently, the segregation is known as independent ­assortment. 37. Correct answer: ­A A technique that labels a known chromosome sequence with DNA probes attached to fluorescent dyes is known as fluorescent in situ hybridization (FISH). This technique allows visualization of specific regions on chromosomes under fluorescent microscopy. FISH also allows lymphocytes and amniocytes to be screened for numerical abnomalities such as trisomy 13, 18, 21, and sex chromosome ­anomalies. 38. Correct answer: ­C The study of genetics at the chromosome level is known as cytogenetics. Studies have concluded that chromosomal anomalies occur in about 0.4% of all live births. Chromosomal anomalies are the most prevalent cause of congenital anomalies and mental retardation. The percentage of chromosomal anomalies is very high for stillbirths and spontaneous a ­ bortions. 39. Correct answer: ­A If more than one phenotype is caused by different allelic mutations at a single site (locus), the condition is called phenotypic heterogeneity. Examples of this would be the different craniosynostosis disorders such as Aspen syndrome, ­Jackson-­Weiss syndrome, and Pfeiffer ­syndrome. 40. Correct answer: ­C Birth defects that result from altered genetic or developmental causes are referred to as malformations. Malformations that occur more frequently than would be expected by chance alone are called associations. If the order of the maldevelopment is known, it is called a sequence (e.g., ­Pierre-­Robin sequence).

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41. Correct answer: ­B Cri du Chat syndrome is characterized by a ­cat-­like cry, microcephaly, growth retardation, ­down-­slanting palpebral fissures, major organ anomalies, and developmental delay. This syndrome is caused by a partial deletion of the short arm of chromosome ­5. 42. Correct answer: ­C Micrognathia, a cleft lip and palate, along with a large, protruding tongue, are classic characteristics of Pierre Robin syndrome. Upper airway obstruction is common and feeding can be quite ­difficult. 43. Correct answer: ­A A small, triangular face and a normal head circumference with short stature is characteristic of ­Russell-­Silver syndrome. Other characteristics include excessive sweating, café au lait spots on the skin, and limb ­asymmetry. 44. Correct answer: ­C Infants with Cornelia de Lange syndrome have the distinguishing feature of one eyebrow. They are small for gestational age, have long, curly eyelashes, microcephaly, and thin, ­down-­t urned upper lips. Cardiac defects may also be present. Infants with this syndrome will have small hands and feet and generally have no facial ­expression. 45. Correct answer: ­B A clinical presentation of “elfin facies”, a flat nasal bridge and round cheeks are all characteristics of Williams syndrome. An infant with Williams syndrome will have idiopathic hypercalcemia, supravalvular aortic stenosis, a hoarse voice, hernias, and other connective tissue ­abnormalities. This syndrome is caused by a deletion on chromosome ­7. 46. Correct answer: ­B Severe oligohydramnios that causes lung hypoplasia and fetal compression with limb abnormalities are classic congenital abnormalities manifested in infants with Potter’s syndrome. The cause of this syndrome may be a chronic amniotic fluid leak or intrauterine renal failure. The renal failure may be caused by renal agenesis, an obstruction, or polycystic ­kidneys. 47. Correct answer: ­A Characteristics of an infant with fetal alcohol syndrome would include microcephaly, cardiac defects such as a ventricular septal defect, small for gestational age, small eye openings, hyperactive behavior, and sleep and sucking disorders. Additional defects may include a sunken nasal bridge, shortened nose, flat cheekbones, smoothing and elongation of the ridged area (the philtrum) between the nose and lips, and a smooth, thin upper ­lip. 48. Correct answer: ­C A medial bone abnormality such as a short ulna and tibia is called mesomelia. It is a type of skeletal ­dysplasia.

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49. Correct answer: ­B Rhizomelia is characterized by a proximal long bone abnormality such as a short humerus and ­femur. 50. Correct answer: ­C Galactosemia is characterized by hepatomegaly and hypoglycemia. Vomiting and diarrhea occur from either breastmilk or formula as both contain galactose. Soy milk does not contain ­galactose. 51. Correct answer: ­C Marfan syndrome is characterized by elongated extremities and fingers, kyphosis, a decreased ­upper-­to-­lower segment ratio, and retinal detachment. There is usually aortic root dilatation that may have an associated aortic dissection, mitral valve prolapse, and/or aortic ­regurgitation.

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Genetics ­References Allen, E., Freeman, S., Druschel, C., et al. (2009). Maternal age and risk for trisomy 21 assessed by the origin of chromosome nondisjunction: A report from the Atlanta and national Down syndrome projects. Human Genetics, 125(1), 41–52. Altug-­Teber, O., Bonin, M., Walter, M., et al. (2008). Specific transcriptional changes in human fetuses with autosomal trisomies. Cytogenetic and Genome Research, 119(3–4), 171–184. Benachi, A., & Costa, J. M. (2007). ­Non-­invasive prenatal diagnosis of fetal aneuploidies. The Lancet, 369(9560), 440–442. Bondy, C. A. (2007). Care of girls and women with Turner syndrome: A guideline of the Turner syndrome study group. Journal of Clinical Endocrinology and Metabolism, 92: 10–25. Brodsky, D., & Martin, C. (2003). Neonatology Review. Philadelphia, PA: Hanley & ­Belfus. Chial, H. (2008). Somatic mosaicism and chromosomal disorders. Retrieved March 9, 2010, from http://www.nature.com/scitable/topicpage­/Somatic-­Mosaicism-­and-­Chromosomal -­Disorders-­867 Cloherty, J. P., Eichenwald, E. C., & Stark, A. R. (2004). Manual of neonatal care (5th ed.). Philadelphia, PA: ­Lippincott. DeBaun, M. R., & Vichinsky, E. (2007). Hemoglobinopathies. In R. M. Kliegman, R. E. Behrman, H. B. Jenson, & B. F. Stanton (Eds.), Nelson textbook of pediatrics (18th ed., pp. Chapter 462). Philadelphia, PA: Saunders ­Elsevier. Dierssen, M., ­Ortiz-­Abalia, J., Arqué, G. et al. (2006). Pitfalls and hopes in Down syndrome therapeutic approaches: In the search for ­evidence-­based treatments. Behavior Genetics, 36(3), 454–468. Gravholt, C. (2008). Epidemiology of Turner syndrome. Lancet Oncology, 9(3), 193–195. Gregg, X. T., & Prchal, J. T. (2005). Red blood cell enzymopathies. In R. Hoffman, E. J. Benz Jr., S. J. Shattil, B. Furie, & H. J. Cohen (Eds.), Hematology:Basic principles and practice (4th ed., Chapter 39). Philadelphia, PA: Churchill Livingston ­Elsevier. Hall, J. G. (2007). ­Single-­gene and chromosomal disorders. In L. Goldman, D. Ausiello, (Eds.), Cecil Medicine (23rd ed.). Philadelphia, PA: Saunders ­Elsevier. Halliday, H. L., McClure, B. G., & Reid, M. (2002). Handbook of neonatal intensive care (4th ed.). Philadelphia, PA: ­Saunders. Hay, W. W., Levin, M. J., et al. (2007). Current diagnosis and treatment in pediatrics (18th ed.). New York, NY: McGraw-­Hill. Hjerrild, B. E., Mortensen, K. H., & Gravholt, C. H. (2008). Turner syndrome and clinical treatment. British Medical Bulletin, 86(1), 77–93. Jones, K. L. (2006). Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: ­Elsevier. Karlsen, K. (2006). The S.T.A.B.L.E. program (5th ed.). Park City, UT: American Academy of ­Pediatrics. Karlsen, K., & Tani, L. Y. (2003). S.T.A.B.L.E.—Cardiac module. Park City, UT: American Academy of P ­ ediatrics. Kattwinkle, J. (Ed.). (2006). Neonatal resuscitation textbook (5th ed.). Elk Grove Village, IL: American Academy of Pediatrics and American Heart ­A ssociation. Kenner, C., Amlung, S., & Flandermeyr, A. (1998). Protocols in neonatal nursing. Philadelphia, PA: ­Saunders. Kenner, C., & Lott, J. (2004). Neonatal nursing handbook. St. Louis, MO: ­Elsevier. Kenner, C., & Lott, J. (2007). Comprehensive neonatal care (4th ed.). St. Louis, MO: ­Elsevier. Kenner, C., & McGrath, J. M. (Eds.). (2004). Developmental care of newborns & infants: A guide for health professionals. St. Louis, MO: M ­ osby. Lanie, A. D., Jayaratne, T. E., Sheldon, J. P. et al. (2004). Exploring the public understanding of basic genetic concepts. Journal of Genetic Counseling, 13(4), ­305.

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Genetics  y  319 MacDonald, M. G., & Ramasethu, J. (2007). Atlas of procedures in neonatology (4th ed.). Philadelphia, PA: Lippincott ­Williams. Markiewicz, M., & Abrahamson, E. (1999). Diagnosis in color: Neonatology. Philadelphia, PA: ­Mosby. Merenstein, G. B., & Gardner, S. L. (2006). Handbook of neonatal intensive care (6th ed.). St. Louis, MO: ­Mosby. Merhar, S. L., & ­Manning-­Courtney, P. (2007). Two boys with 47, XXY and autism. Journal of Autism and Developmental Disorders, 37(5), 840–846. Polin, R. A., & Spritzer, A. R. (2007) Fetal & neonatal secrets (2nd ed.). Philadelphia, PA: ­Mosby. Prescribing Reference. (2009, Summer). NPPR: Nurse Practitioner’s Prescribing Reference, 16(2). Rennie, J. M., & Roberton, N. R. C. (2002). A manual of neonatal intensive care (4th ed.). New York, NY: A ­ rnold. Taeusch, H. W., Ballard, R. A., & Gleason, C. A. (2005). Avery’s diseases of the newborn (8th ed.). Philadelphia, PA: ­Elsevier. Verklan, M. T., & Walden, M. (Ed.). (2004). Certification and core review for neonatal intensive care nursing (3rd ed.). St. Louis, MO: E ­ lsevier. Young, T. E., & Magnum, B. (2009). Neofax 2009. Montvale, NJ: Thomson ­Reuters.

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Section 12 RNC Practice ­Examination

The RNC practice examination consists of 175 questions with three possible responses. Please allow 3 hours for ­testing. The questions on the online exam are from the same pool of questions found on this written test. However, the mix of questions may vary. Please enter your answers on the forms at the end of this ­section.

Questions 1. Your patient is a term infant who has required some supplemental oxygen. She is placed on 2 L/min via NC and ABGs have been drawn. Blood gas results show pH 7.54, PaO2 98 mmHg, PaCO2 26 mmHg, and HCO3 24 mEq/L. These blood gas results ­show A. Uncompensated respiratory ­alkalosis B. Compensated metabolic ­alkalosis C. Compensated metabolic ­acidosis 2. To provide consistent and complete transfer of care from one healthcare professional to another, regulating bodies recommend standardized ­report-­off or ­hand-­off documentation or reports. An example of this ­is A. ­SBAR B. ­DBAR C. ­SOAR 3. Your patient is septic and you have just drawn an ABG. The respiratory therapist asks if the patient has a fever. The possibility of fever will have what effect on the ­sample? A. The HCO3 will be ­elevated B. The pH will ­r ise C. Fever has no ­effect 4. The 36-week gestational aged female born 2 hours ago whom you are feeding begins coughing and choking. She has large amounts of oral secretions as well. Her color is poor and she is in respiratory distress. What is the most likely cause of these ­symptoms? A. Esophageal ­atresia B. Status a ­ sthmaticus C. Cystic ­fibrosis

y

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5. You are caring for a 24-week gestation neonate at 3 days of life. The neonate is intubated on conventional ventilation support at 30% FiO2 . A d ­ ouble-­walled isolette at 65% humidity and a ­K-­pad are in place. Double phototherapy was started for hyperbilirubinemia. Which of the following treatments would contribute most to insensible water l­ oss? A. Conventional ­ventilation B. 30% ­FiO2 C. ­Phototherapy 6. You are admitting a term male infant from home at 9 days of life with severe dehydration, hyperkalemia, hypoglycemia, and hyponatremia, with a history of poor feeding, vomiting, diarrhea, and possible seizure activity. The mother reports receiving a strange phone call from screening people yesterday, but was unable to report why. You would anticipate the physician ordering which t­ ests? A. 21-OHD l­evel B. 17-OHP l­evel C. RSH s­ tudies 7. The infant you are caring for has been pharmacologically weaned from opiates. Weaning is usually deemed successful if the ­neonate A. Can be easily ­consoled B. Maintains weight for 1 ­week C. Scores a 10 or less on the neonatal abstinence scoring ­system 8. Baby M. was born at 37 weeks gestation and is now at day 4 of life. This baby has poor skin turgor, depressed anterior fontanelle, mottled skin, weak pulses, low blood pressure, and is lethargic. Lab results indicate a urine specific gravity of 1.005, an elevated serum osmolality, and a low urine osmolality. Urine output the past 24 hours has been 6.5 ml/kg/hr. Baby M. probably ­has A. Congenital ­hypopituitarism B. Thyroid ­dysgenesis C. Diabetes ­insipidus 9. Alpha fetal protein (AFP) screening of the mother’s serum potentially detects what percentage of neural tube ­defects? A. 80–90% B. 75–85% C. 60–75% 10. Baby Sam is about to undergo a circumcision. A topical anesthetic that may be used for this procedure would ­be A. A Lidoderm ­patch B. EMLA ­cream C. ­Duragesic

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11. The most common ­single-­gene disorder (autosomal recessive) in Caucasian populations i­ s A. Adult polycystic kidney ­disease B. Cystic ­fibrosis C. ­Retinoblastoma 12. Tetralogy of Fallot manifests itself by which of the following combinations of ­defects? A. VSD, overriding aorta, pulmonary stenosis, and right ventricular ­hypertrophy B. Aortic stenosis, atrial septal defect, coarctation of the aorta, and ­PDA C. ASD, mitral prolapse, PDA, and pulmonary ­stenosis 13. A gravida 1 mother with no prenatal care and severe ­pregnancy-­induced hypertension treated with magnesium just gave birth to a baby girl. How will the mother’s medical treatment for PIH alter the gestational ­scoring? A. Maternal medical management has no effect on gestational ­scoring B. The neurological assessment portion will be ­altered C. The physical assessment portion will be ­altered 14. AV canal defects are also known ­as A. Endocardial cushion ­defects B. Ventricular septal ­defects C. Ebstein’s ­anomaly 15. Anatomic dead space is defined ­as A. Minute ­ventilation B. Wasted ­ventilation C. A conducting ­airway 16. Fetal urination is most pronounced during which ­trimester? A. First ­trimester B. Second ­trimester C. Third ­trimester 17. How do an omphalocele and gastroschisis ­differ? A. Omphalocele is the herniation of some or all the abdominal contents into a sac at the u ­ mbilicus B. Gastroschisis is the herniation of all the abdominal contents into a sac at the ­umbilicus C. Omphalocele is the herniation of the small bowel into a sac outside the ­abdomen

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18. Baby C. is a term neonate who has been increasingly restless and now has petechiae on the trunk, arms, and legs. The abdomen is slightly distended and the baby demonstrates increased respiratory effort. She is now obtunded. During your assessment, you note ­pink-­tinged urine in the diaper, generalized ecchymosis, and oozing from the umbilical line insertion point. You suspect Baby C. ­has A. ­DIC B. Pulmonary ­emboli C. A fat e­ mbolism 19. Where is cerebrospinal fluid ­formed? A. In the lateral ­ventricles B. In the third ­ventricle C. In the fourth v ­ entricle 20. While preparing to give report at shift change, you note a 7 cm diameter pool of blood under your patient. The infant dislodged the umbilical catheter. The infant’s heart rate is 188, skin is pale, and capillary refill is about 3 seconds. A normal saline bolus is ordered for fluid replacement. Which of the following parameters indicates improvement after the saline bolus has ­infused? A. Heart rate stabilized at ­192 B. Warm ­extremities C. Capillary refill 5 ­seconds 21. Your team confirms umbilical line placement on chest ­X-­ray prior to transporting your term patient. The umbilical artery catheter is located at T4 and the umbilical venous catheter tip is just above the diaphragm. Based on the chest ­X-­ray results, which of the following choices would be an appropriate ­action? A. Nothing; the lines are properly placed and the infant is ready for ­transport B. Withdraw the arterial line to T11–T12 and the venous line to 1 cm below the diaphragm before securing the ­lines C. Confirm with the neonatologist and prepare to withdraw the arterial line back to T6–T9 and the secure the venous ­line 22. Infants with immature lungs are at risk for respiratory distress syndrome. Which of the following characteristics would be present in most cases of respiratory distress s­ yndrome? A. Pulmonary ­hyperperfusion B. Surfactant present at ­birth C. ­Prematurity

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23. You are called to a primary cesarean section for a term infant’s failure to progress. Over the last few minutes of labor, the baby experienced three 4-minute decelerations with a fetal heart rate of 68. Upon receipt of the baby, you note that the infant is apneic with no tone. After drying the baby and removing blankets, the initial assessment indicates a heart rate 70, apnea despite stimulation, and the baby remains limp and cyanotic. This baby i­ s A. In primary ­apnea B. In secondary ­apnea C. In tertiary ­apnea 24. To achieve the most accurate gestation score, the neonate should be ­assessed A. Once within 6 ­hours B. Once within 12 ­hours C. Twice within 12 ­hours 25. You are in attendance at the ­C-­section of a 32-week gestational age infant. The infant emerges in severe respiratory distress with a probable diaphragmatic hernia. What medical therapies do you ­anticipate? A. Intubation, NG tube, IV’s, chest ­X-­ray, and ­surgery B. None; this will resolve ­spontaneously C. Intubation, chest tubes, and ­Sengstaken-­Blakemore ­t ube 26. Factor VIII deficiency is also known ­as A. Von Willebrand’s ­disease B. Sickle cell ­anemia C. Aplastic ­anemia 27. You are caring for a 28-week gestation infant at day 20 of life with hydrocephalus. The baby has had a ventriculoperitoneal (VP) shunt placed. Over the past few hours, you have noticed the baby has become increasingly lethargic. As the NICU nurse you know ­that A. The shunt may be ­occluded B. It is not unusual for babies with hydrocephalus to fatigue ­easily C. The shunt is functioning ­well 28. Which of the following statements is true regarding excess fetal ­insulin? A. Excess fetal insulin may be the cause of delayed maturation of type II alveolar ­cells B. Excess fetal insulin promotes the development of pulmonary ­surfactant C. Excess fetal insulin causes idiosyncratic ­hyperglycemia 29. A newborn’s visual acuity is estimated to be in the range o ­f A. ­20/400 B. ­20/200 C. ­20/100

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30. Type II truncus arteriosus is characterized ­by A. A longer pulmonary artery arising from the base of a common ­trunk B. The right and left pulmonary arteries having separate ­origins C. The right and left pulmonary arteries are joined at the base of the common ­trunk 31. The physician orders high humidity and a d ­ ouble-­walled isolette for your newly born 23-week gestation neonate. This is b ­ ecause A. The insulated walls provide a sound ­barrier B. The humidity protects the skin from ­infection C. Fluid regulation is impaired in the extremely ­low-­birth-­weight ­patient 32. You are mentoring a nursing student and discussing the importance of obtaining a maternal history prior to delivery. Maternal health during the pregnancy is extremely important in the development of the fetus and directly affects the postdelivery management of the neonate. Which are the most important maternal factors you should discuss with the nursing ­student? A. Placental function and inherent maternal ­resources B. Placental location and overall maternal ­health C. Maternal socioeconomic and physiologic ­status 33. Short bowel syndrome puts the neonate at increased risk for which of the following d ­ eficiencies? A. Vitamins A, D, E, and ­K B. Vitamin B complex and vitamin ­K C. Vitamin C, B complex, and vitamin ­D 34. Which of the following statements is true about transient tachypnea of the ­newborn? A. TTN only affects infants who are born after 35 weeks ­gestation B. Epinephrine may be useful in the treatment of ­T TN C. Sodium transport is decreased after ­birth 35. You are anticipating the delivery of a 24-week gestation neonate. Which of the following assessment findings would be consistent with a 24-week gestation ­neonate? A. A popliteal angle of 160° B. Thinning ­lanugo C. Flat areola with no ­bud 36. One of the differences between the symmetric and asymmetric intrauterine growth restriction (IUGR) ­is A. Symmetrical IUGR has normal number of cells with reduced cell ­size B. Asymmetrical IUGR has a lower weight and shorter length compared to head ­circumference C. Asymmetrical IUGR is associated with congenital ­malformation

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37. VATER association presents with which of the following ­anomalies? A. Vertebral anomalies, anal anomalies, tracheoesophageal fistula, esophageal atresia, and renal ­anomalies B. Vertebral anomalies, anal anomalies, tracheoesophageal fistula, ear anomalies, and renal ­anomalies C. Vertebral anomalies, anal anomalies, thoracic anomalies, esophageal atresia, and rectal ­anomalies 38. A term infant has been diagnosed with thalassemia and is about to be discharged home. An important aspect of parent teaching for a neonate with thalassemia would ­include A. Using cool water for skin ­cleansing B. Avoiding oral stimulants like ­pacifiers C. Feeding fewer, larger, ­high-­protein ­meals 39. Stimulation of the anterior pituitary gland is from the hypothalamus by way of t­ he A. Sympathetic nervous ­system B. Parasympathetic nervous ­system C. Vascular ­system 40. Which of the following statements is true regarding epicardial pacing wires placed for postoperative cardiac ­management? A. The wires are placed in the right atrium and left ­ventricle B. The wires provide a temporary backup for 3 days ­postoperatively C. ­Dual-­chamber pacing is most important after surgical repairs near the conduction ­system 41. After 3 weeks in the NICU, a 28-week gestational age male with Trisomy 18 begins having projectile vomiting of formula immediately after feeding. He is irritable but hungry. Which of the following is the most likely cause of his ­problem? A. Short bowel ­syndrome B. Hypertrophic pyloric ­stenosis C. ­Prader-­Willi ­syndrome 42. You are participating in the delivery and resuscitation of a 28-week gestation neonate. The heart rate is 140 with a respiratory rate of 80 with moderate retractions and circumoral cyanosis. Your next step is ­to A. Provide positive pressure ventilation with 100% ­FiO2 B. Provide CPAP using a s­ elf-­inflating ­bag-­mask ­valve C. Provide CPAP using a T ­ -­piece ­resuscitator

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43. Your transport team arrives at a hospital emergency room that does not have maternal child services to transport a term infant who was surrendered to staff. The infant’s first temperature was 35.4° C. You find the infant wrapped in heated blankets under a radiant warmer. During your assessment, you find the blood glucose to be 17 mg/dl. The ER physician orders dextrose 25% at 2 ml/kg IV stat. You ­should A. Administer the dextrose as ­ordered B. Refuse the order and hang a D10W continuous drip at 150 ­mg/kg/day C. Politely refuse the order, notify your transport neonatologist, and follow standard stabilization protocol of using dextrose 10% at 2 ml/kg ­IV 44. A factor that increases pulmonary vascular resistance ­is A. Prostaglandin ­therapy B. ­Sepsis C. ­Hypoxia 45. A 30-week gestation neonate was vaginally delivered with vacuum assist 20 ­minutes ago. The weight was 2000 grams, the length was 45 cm, and the head circumference was 31 cm. You would classify this neonate ­as A. Preterm small for gestational age (SGA) B. Preterm appropriate for gestational age (AGA) C. Preterm large for gestational age (LGA) 46. You are preparing for the delivery of a 38-week gestation neonate via emergency primary cesarean section to a 29 ­year-­old, gravida 1 mother with uncontrolled type 2 diabetes. The mother has been pushing for 15 hours without significant cervical changes or fetal drop. Maternal vital signs at delivery are a heart rate of 120, respiratory rate 20, blood pressure 135/85, and saturation in room air 92%. Fetal weight on last ultrasound was 4.2 kg. Which postdelivery fetal complication would you ­anticipate? A. ­A nemia B. ­Hypotension C. Respiratory distress ­syndrome 47. Persistent pulmonary hypertension of the newborn (PPHN) is caused b ­y A. Low PVR and pulmonary artery ­pressures B. ­R ight-­to-­left shunting through fetal ­channels C. Increased ­SVR 48. A ­full-­term Down syndrome baby has started vomiting. You notice his abdomen is becoming distended. Which of the following conditions may be the cause of this ­problem? A. High meconium ­plug B. Malrotation and ­volvulus C. Duodenal ­atresia

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49. Your patient is a 35-week gestation infant who has developed respiratory distress and sepsis. The infant is being mechanically ventilated and has had a varying pH (from 7.22 to 7.30) and a base deficit that has also been fluctuating from 25 to 26 over the last 10 hours. The infant has an indirect bilirubin of 22.9. This infant will be receiving a d ­ ouble-­volume exchange transfusion. Which of this infant’s problems must be addressed prior to the start of the ­transfusion? A. The bilirubin ­level B. The a ­ cid-­base ­imbalance C. Extubate the ­infant 50. You are caring for Angel, a 34-week gestation neonate who is the recipient of a ­t win-­to-­t win transfusion on day 2 of life. There are multiple bruises on her torso and extremities. Morning labs reveal a Hgb of 17.9g/dl and a Hct of 66%. Mean arterial blood pressure is 45. She is on 1 L/m nasal cannula at 25% FiO2 with saturation at 92%. IV therapy includes TPN of D10 at 90 ml/kg/d via peripheral IV. Urine output for the last 24 hours is 1.3 ml/kg/d. The low urine output is probably due to ­the A. Hgb and ­Hct B. Potential ­hyperbilirubinemia C. Low oxygen ­saturation 51. The mother of a 30-week gestation neonate is sitting at bedside when you let her know that you will be transferring the baby to an isolette. She states, “I don’t want him to go into that box thing. I’ll be afraid to touch him. Can’t he stay in this warmer thing?” What is the best response to her ­request? A. “Sure, we can leave him in the warmer for another week.” B. “No, he must be changed over to an isolette per our policy.” C. “We will assist you in touching and holding your baby as he gets better, and the isolette allows for better temperature control and reduces stress to the baby.” 52. You are asked to visit the parents of a premature neonate who will deliver imminently. The labor and delivery nurse tells you that they have multiple questions regarding prognosis and expected plan of care. You s­ hould A. Go speak with the parents ­immediately B. Ask the neonatologist to speak with the ­parents C. Wait until after the delivery to speak with the ­parents 53. Your 1260 gram patient has an order for intravenous fluids to run at 80 ­ml­/­kg­/­day. Which of the following IV rates is ­correct? A. 4.2 ­ml/h B. 5.6 ­ml/h C. 8.4 m ­ l/h 54. On an EKG, ­left-­sided heart failure results ­in A. Tall, peaked P ­waves B. Wide, notched P ­waves C. Changes in ST ­segments

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55. The occurrence of pulmonary interstitial emphysema (PIE) is closely associated w ­ ith A. ­A ntibiotics B. Maternal ­age C. ­Barotrauma 56. A contraindication to the surgical correction for hypoplastic left heart syndrome would ­include A. A previous transcatheter balloon atrial septostomy to decompress the right ­atrium B. Use of inhaled O2 and n ­ itrogen C. Significant tricuspid valve ­dysplasia 57. Your patient with bronchopulmonary dysplasia (BPD) was started on Diuril 2 days ago for pulmonary edema. While drawing blood for a capillary blood gas (CBG), you perform a bedside glucose check. The serum glucose is 180 mg/dl. The most likely cause for the hyperglycemia in this patient ­is A. ­Stress B. Stimulation of the adrenal ­glands C. Suppression of the pancreatic release of ­insulin 58. Hemorrhage in an infant with hydrops fetalis is usually the result ­of A. Altered hepatic ­synthesis B. ­Hyperglycemia C. ­Hypoxia 59. Where is the most common location for ­craniosynostosis? A. Coronal ­suture B. Lambdoidal ­suture C. Saggital ­suture 60. Your patient is a n ­ ear-­term infant who exhibited worsening perioral and periorbital cyanosis after birth. The infant developed a profound generalized cyanosis after the ductus closed and has been progressively more tachypneic. A pansystolic murmur is auscultated at the left lower sternal border and is accompanied by a thrill and a diastolic rumble. These symptoms are suggestive ­of A. Aortic ­stenosis B. Tetralogy of ­Fallot C. Pulmonary atresia with an intact ventricular ­septum 61. You are assisting in a neonatology consultation for a 17 y ­ ear-­old in preterm labor at 25-weeks gestation who is addicted to energy drinks. What potential complications in the neonate must you ­anticipate? A. Complications similar to fetal alcohol ­syndrome B. Complications similar to Trisomy ­13 C. Complications similar to heroin ­addiction

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62. You are caring for a 27-week gestational age female with subtle seizures. What medications do you anticipate using for this ­patient? A. ­Carbamazepine B. ­Diazepam C. ­Lorazepam 63. What is the major factor that causes parental stress while their infant is in the neonatal intensive care u ­ nit? A. Illness of the ­infant B. Deviation from expected ­events C. Change in family ­dynamics 64. A 47 y ­ ear-­old gravida 10, para 5 mother is in active labor. She received late prenatal care, seeing the obstetrician only once. Estimated gestation is 21 weeks and 450 grams on ultrasound. The mother believes she is at 23 weeks. After a family conference with the obstetrician, neonatologist, and social service, the parents decide to hold resuscitation efforts until gestation can be assessed. Upon delivery, the infant is dried, positioned, and placed on the warmer. Weight is estimated to be 350 grams. The skin is friable and sticky, the eyelids are tightly fused, and the heel–toe length is 45 mm. These findings ­suggest A. The parents should be allowed to choose what resuscitation measures should be taken or ­continued B. The infant is viable and all resuscitation measures should be ­initiated C. The infant is not viable and resuscitation efforts should ­held 65. Similac PM 60/40 is frequently given ­for A. Fatty acid metabolism ­defect B. Renal ­insufficiency C. Neurologic ­disease 66. Many neonates with RDS and hypoxemia are managed by increasing the inflating pressure and application of positive ­end-­expiratory pressure (PEEP). PEEP is useful in RDS b ­ ecause A. PEEP decreases cardiac ­output B. PEEP prevents ­barotraumas C. PEEP can open collapsed ­alveoli 67. The parents of 24-week gestation triplets ask you if they have opened their eyes. You would explain t­ hat A. The eyelids are fused at this time but will open at 25-weeks ­gestation B. The eyelids are fused at this time but will open between the 26th and 30th ­weeks C. You can separate the eyelids in the morning with forceful ­pressure

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68. A transfusion reaction that usually occurs within 5–30 minutes of the start of the transfusion is known a ­s A. An acute intravascular hemolytic ­reaction B. An extravascular transfusion ­reaction C. An allergic transfusion ­reaction 69. Your 31-week gestation patient is at 10 days of life. Feedings are by gavage every 3 hours with expressed breastmilk. Cafcit was started PO yesterday for periodic apnea and bradycardia. A glycerin suppository is ordered every 24 hours if the infant has not stooled. Morning labs indicate hyponatremia. This is mostly likely due ­to A. Breastmilk ­nutrition B. ­Cafcit C. The s­ uppository 70. Herniation of abdominal contents into the chest cavity early in gestation accompanied by ipsilateral pulmonary hypoplasia is known a ­s A. Gunner’s ­syndrome B. Congenital diaphragmatic ­hernia C. Potter’s ­syndrome 71. You are attending the vaginal delivery of a term infant with meconium. Which of the following infant presentations would you classify as ­vigorous? A. The baby is crying and kicking, with a heart rate . 100 ­bpm B. The baby is weakly crying, grabbing at the stethoscope, and the heart rate is . 80 b ­ pm C. The baby is apneic and floppy, with a heart rate . 100 ­bpm 72. You are caring for a 32-week gestation infant in acute respiratory failure. The patient is receiving O2 therapy and has ABGs scheduled every four hours. As a NICU nurse, you know that the arterial oxygen tension should be maintained ­between A. 40 and 60 m ­ mHg B. 50 and 80 m ­ mHg C. 70 and 90 m ­ mHg 73. You are explaining to a student nurse the correct method to assess arm recoil during the gestational assessment. Which of the following statements is ­correct? A. Hold the infant prone in your arms to assess the flexion in the ­arms B. Place the patient supine, stretch the arms straight out, then release the arms and measure the a ­ ngle C. Place the neonate supine, hold the arms flexed for 5 seconds, then straighten and release the arms to measure the ­angle

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74. The baby you are resuscitating has persistent cyanosis despite strong respiratory efforts and a heart rate . 100 bpm. What is the next nursing action that should be performed to assist this infant’s respiratory ­status? A. Provide supplemental oxygen via continuous positive airway pressure (CPAP) using a T-­piece B. Provide ­f ree-­flow supplemental oxygen via a ­self-­inflating b ­ ag-­valve-­mask C. Provide ­blow-­by supplemental oxygen via a ­flow-­inflating b ­ ag-­valve-­mask 75. As a transport nurse, you have been trained to use the new ­T-­piece resuscitation device for preterm deliveries. Which of the following statements is true regarding the use of a ­T-­piece for preterm ­infants? A. The PIP and PEEP are preset on the machine ­f ront B. The ­pop-­off valve is fixed at 30 ­mmHg C. The rate and inspiratory time are controlled by the staff member manipulating the T-­piece 76. The neonatal resuscitation team is called to the vaginal delivery of a 38-week ­gestation infant of a mother having seizures. She is reported as having headaches, hypertension, nausea, and vomiting prior to delivery. The preeclampsia workup shows normal platelet count, normal AST and ALT, and no proteinuria. What condition is the infant at risk for during d ­ elivery? A. ­Hyperglycemia B. ­Hypervolemia C. Cerebrovascular ­accident 77. What is the drug of choice to treat active ­cytomegalovirus? A. ­Ganciclovir B. ­Valacyclovir C. ­Famciclovir 78. An intrinsic factor that may contribute to the pathogenesis of congenital lobar emphysema ­is A. An ­adenoma B. Pulmonary artery ­dilation C. Lymph node ­compression 79. Which of the following statements is true regarding the use of karyotyping in genetic ­screening? A. It will take only 3–4 days to determine results of ­karyotyping B. Karyotyping is done in ­utero C. Karyotyping will provide a complete genetic ­map

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80. Your preterm patient has become increasingly restless. You note that the infant’s chest has become more ­barrel-­shaped and overdistended. Breath sounds have become distant on the left side. These findings are indicative ­of A. A tension ­pneumothorax B. A ­pneumomediastinum C. Pulmonary interstitial ­emphysema 81. Heather is a 22 ­year-­old admitted in labor at 30-weeks gestation. She denies any drug or alcohol use throughout the pregnancy. When questioned further during the neonatology consult, she admits to drinking a “few beers” weekly while partying at college. Knowing that most patients will underestimate drug and alcohol consumption, which fetal complications could you anticipate finding at ­delivery? A. Large for gestation, short long ­bones B. Small for gestation, ­microencephaly C. Hypotonicity, lengthened long ­bones 82. You are called to the nursery to evaluate a 2-day-­old term infant for suspected seizure activity. You note that this term infant has a ­high-­pitched cry, irritability, dry mucus membranes, and sunken fontanels. The mother reports that she is breastfeeding exclusively, but the baby “always seems to be sleepy and hard to wake up.” While obtaining vital signs, you note a period of apnea and seizure activity. You bring the infant to the NICU and notify the physician. Which order should be carried out ­first? A. Draw a CBC, chemistry panel, and blood ­glucose B. Obtain a chest X-­ray C. Ensure newborn and hearing screening are ­done 83. You are preparing to update Anna and Steven about their son, Evan, a 23-week gestation neonate. He is orally intubated and was treated effectively this morning with a second course of surfactant for increasing respiratory distress and mechanical support. An echocardiogram was ordered for today to evaluate a murmur and fluctuating oxygen requirements. Trophic feedings were started using breastmilk provided by Anna. Which of the following comments or statements regarding Evan’s condition is the most therapeutic and ­correct? A. “Evan was really sick this morning, but we took care of it before you got here.” B. “An echocardiogram was ordered for today to evaluate blood flow through Evan’s heart.” C. “We are beginning trophic or enteral gavage feedings so that Evan can wean off TPN and IL within the week.” 84. One means of decreasing pCO2 when using a pressure ventilator is t­ o A. Add P ­ EEP B. Increase the ventilator ­rate C. Decrease the tidal ­volume

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85. During feeding time, you have the opportunity to teach a new mother and father about taking their infant’s temperature. Which of the following statements about this process is ­true? A. Environmental factors do not impact the infant’s ability to ­self-­regulate ­temperature B. Brown fat does not need oxygen to b ­ urn C. A normal temperature for an infant is between 36.5 and 37.5° C (97.7–99.5° F) 86. What is the frequency that acetaminophen can be safely administered to a 28-week gestational aged ­infant? A. Every 4 ­hours B. Every 8 ­hours C. Every 12 ­hours 87. Developmental care is based on which of the following standards of critical ­care? A. All infants receive the same care with slight ­differences B. Parents play a critical role in infant care once the infant is ­stable C. Parent and infant interaction is continuously assessed and interactions ­supported 88. A high serum magnesium or phosphate level, low serum calcium, with catecholamine release results in secretion ­of A. ­A ldosterone B. Parathyroid ­hormone C. ­Glyceride 89. An uncommon cause of pulmonary hemorrhage in infants ­is A. ­Caffeine B. A bronchogenic ­c yst C. Sickle cell ­disease 90. Cleft lip and/or palate is usually associated w ­ ith A. DiGeorge syndrome, Pierre Robin sequence, Trisomy ­13 B. Trisomy 21, ­Prader-­Willi syndrome, ­Russell-­Silver ­syndrome C. Carpenter syndrome, Klinefelter syndrome, ­Holt-­Oram ­syndrome 91. When utilizing transcutaneous PO2 measurements, some conditions may make the results unreliable. Underestimation of oxygenation ­may A. Occur when there is an air bubble between the electrode and the ­skin B. Occur when the alignment is ­improper C. Occur when skin is ­hypoperfused 92. You are called to the vaginal delivery of a 44-week gestation infant with meconium. You anticipate the skin to ­be A. Leathery and cracked with meconium stained ­nails B. Rough with peeling and few v ­ eins C. Smooth with rare ­veins

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93. If positive inspiratory pressure (PIP) is increased on a mechanical ventilator, what effect should this have on your patient’s blood ­gases? A. Raise the PaCO2 and decrease the ­PaO2 B. Lower the PaCO2 and lower the ­PaO2 C. Decrease the PaCO2 and increase the P ­ aO2 94. What is the usual dose of fentanyl for ­sedation? A. 50–100 mg/kg intravenously every 2–4 ­hours B. 0.5–4 mcg/kg intravenously every 2–4 ­hours C. 5–10 mcg/kg intravenously every 2–4 ­hours 95. Analyze the following arterial blood gas from a 36-week gestation infant with Tetralogy of ­Fallot pH ­7.18 CO2 ­40 HCO3 ­15 A. ­Normal B. Compensated respiratory ­acidosis C. Uncompensated metabolic acidosis 96. Portagen is frequently used for infants with which of the following ­conditions? A. Hepatic ­disorder B. Fatty acid metabolism ­deficiency C. Feeding ­intolerance 97. Your male patient has a genetic disorder and the mother is a carrier. If the father is not a carrier and the mother’s two brothers do not have the disorder, this disorder must be ­an A. Autosomal dominant ­disorder B. Autosomal recessive ­disorder C. ­X-­linked recessive ­disorder 98. Michael is a 36-week gestation neonate at day 3 of life. He was admitted for a PDA that was ligated. During his assessment you note that his wrist appears wider than normal. His chest appears wider than normal as well. His serum calcium level is also low. As a NICU nurse, you believe Michael is probably suffering ­from A. Vitamin A ­deficiency B. Vitamin D ­deficiency C. Vitamin B2 ­deficiency 99. Cardiac glycosides are often used in the treatment of Ebstein’s anomaly. A major effect of this classification of medications ­is A. ­Inotropism B. Positive ­chronotropism C. Its usefulness as a ventricular ­antiarrhythmic

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100. You are caring for a 36-week gestational age neonate with spina bifida with a meningocele. With this condition you would expect to ­see A. Dura mater and arachnoid tissue visible through the ­defect B. A visible defect of the vertebral ­arches C. A visible defect with protrusion of the meninges and spinal cord contained in a ­cyst 101. The delivery team has been called to the vaginal delivery of a female infant to a mother with late prenatal care. The infant is placed on the warmer. The arms are extended straight with slight flexion of the legs. The elbow crosses the sternum, but not past the trunk. You note that the skin is smooth with visible veins and abundant lanugo. When you assess the soles, you note faint red marks without creases. The pinna are slightly curved and soft with slow recoil. The clitoris is prominent with an enlarged minora, and the nipples have no bud with flat areola. The findings are consistent with a neonate of which gestational ­age? A. 24 w ­ eeks B. 29 w ­ eeks C. 33 w ­ eeks 102. Analyze the following arterial blood gas ­values pH ­7.49 CO2 ­30 HCO3 ­22 A. Uncompensated respiratory ­alkalosis B. Compensated respiratory ­acidosis C. Compensated metabolic ­acidosis 103. Baby D. has been vomiting with diarrhea for the past 2 days. The infant has failure to thrive and hepatomegaly. Cataracts are noted and have an “oil droplet” appearance. Baby D is probably symptomatic for which inborn error of ­metabolism? A. ­Galactosemia B. Wilson’s ­disease C. ­Tay-­Sachs ­disease 104. Information that has been coded in genetic material and is inherited by an individual is ­a A. ­Genotype B. ­Locus C. ­Phenotype 105. After assisting with the delivery of a term infant who is apneic, you provided 30 seconds of positive pressure ventilation. The infant remains apneic. What should you do ­next? A. Continue positive pressure ventilation if the heart rate is . 100 bpm and spontaneous breathing is ­established B. Insert an orogastric ­t ube C. Begin chest compressions if the heart rate is 80 ­bpm

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106. Retinopathy of prematurity (ROP) is considered to be the result ­of A. A complication of trisomy ­13 B. Displaced sequence on chromosome ­15 C. High oxygen ­saturations 107. Infants are at great risk for heat loss at a rate of 3–4 times that of an adult ­because A. The infant has a greater ­surface-­to-­body r­ atio B. Infants cannot ­shiver C. Infants cannot regulate body ­temperature 108. Loss of a chromosomal segment is known ­as A. ­Mosaicism B. ­Disjunction C. ­Deletion 109. As parents of premature and/or sick neonates cope with stress, they must complete several psychological tasks in order to build a healthy relationship with their child. During the neonate’s course of hospitalization, nurses must continually monitor and intervene to support healthy bonding. Which of the following parental actions would indicate the beginning of a negative parental–neonate ­relationship? A. The father states that he caused the mother go into labor because he let the mother move heavy boxes the day of ­delivery B. The parents insist that clergy performs a blessing and ­baptism C. The parents visit for only 10 minutes at a time and stare at the ­monitor 110. Baby F. is a 37-week gestation infant at 5 days of life. He has been receiving antibiotics for a respiratory infection for the past 4 days. When you change his diaper, you note some small, raised, bright red areas on his buttocks. In the buccal area of his mouth, you note white plaques, and you suspect that baby F. is suffering ­from A. ­T hrush B. Strep ­throat C. Herpes ­virus 111. Mara is a 16 ­year-­old who was brought into the emergency room after collapsing at a party. She is 25-weeks pregnant and having mild contractions. Mara’s cardiac and neurologic workup is negative. Her urine drug screen is negative. If Mara delivers in the next few hours, what immediate infant complication could you ­anticipate? A. Respiratory ­depression B. ­Irritability C. Projectile ­vomiting 112. Over the last 2 weeks you have been teaching Jan and Eric about behavioral cues and interventions when caring for their 26-week gestation neonate. You would document effective learning and application ­if A. They call you to the bedside when the baby ­cries B. They provide hand containment when they see arms and legs ­failing C. They adjust the neonate with extended extremities while providing kangaroo ­care

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113. Your 26-week gestation patient’s morning arterial blood gas indicates metabolic alkalosis. What is the expected effect on serum ­potassium? A. Serum potassium will not be ­affected B. Serum potassium will be higher than ­normal C. Serum potassium will be lower than ­normal 114. Today you are performing the duties of the charge nurse and are attending an emergency cesarean section. The infant is a 26-week neonate with an 18 ­year-­old gravida 1 mother with placenta previa. The infant was delivered pale, floppy, and apneic with a heart rate of 50 bpm. The neonatal nurse practitioner intubated the neonate immediately and positive pressure ventilation was provided with effective chest rise. The heart rate increased to 80 bpm with minimal spontaneous effort. The patient is pale and hypotonic. What is the next step to be performed by your resuscitation t­ eam? A. Provide chest ­compressions B. Prepare to give epinephrine via the endotracheal ­t ube C. Insertion of an umbilical venous line and administration of normal ­saline 115. Baby Alia is a 28-week gestation infant born 24 hours ago to a 14 ­year-­old mother with no prenatal care. After reviewing the chart, you note that the gestational assessment was not completed. Which of the following assessment criteria is no longer accurate if done ­now? A. The plantar ­creases B. The ­lanugo C. Square ­window 116. Pregestimil is indicated as a treatment ­for A. ­Sepsis B. Small gastric ­capacity C. ­Malabsorption 117. Your term gestation patient was diagnosed as having acute pyelonephritis. Which of the following statements is true regarding fluid management for this ­patient? A. The patient should have a 20–30 g/d weight ­gain B. Fluids should not be ­restricted C. Fluid management should be based on 30 ml/kg/d plus urinary fluid ­output 118. Which of the following conditions can cause a neonate’s body to destroy platelets more quickly than they are ­produced? A. Lupus e­ rythematosus B.  Chlamydia ­infection C. Hepatitis ­B

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119. The mother of a 900 gram, 27-week gestational infant asks when the baby will have her first ROP exam. You tell her the examination will be ­scheduled A. In 2 weeks regardless of ­age B. When the baby is at 30 weeks gestation or 4 weeks of life, whichever is ­sooner C. At 32 weeks gestational age or 5–6 weeks of life, whichever is ­greater 120. Which of the following conditions is likely to predispose an infant to hypovolemic ­shock? A. ­Myelomeningocele B. Cardiac ­arrhythmias C. Congenital heart ­lesions 121. The 26-week gestational age female you are caring for has refractory seizures to phenobarbital and lorazepam. What other medication do you anticipate ­administering? A. ­Phenytoin B. ­Fosphenytoin C. ­Levetiracetam 122. While you are shopping at a local department store, a fellow shopper’s amniotic sac ruptures. The mother tells you she has had three previous children and this child should be due this week. You realize delivery of a single, term infant appears imminent. Emergency medical services (EMS) have been initiated and are 5 minutes away. Before EMS can arrive, a female infant is delivered. What is the first action you should ­take? A. Dry and stimulate the infant using available clothing ­articles B. Leave the cord attached and focus on establishing ­ventilation C. Place the infant on the floor and pile clothing on ­top 123. During resuscitation in an operating suite, a term infant is placed on a radiant warmer that was just turned on. The front of the baby is dried, but the wet linens are not removed. Oxygen is provided via free flow. By which mechanism is the infant losing ­heat? A. Conduction from being placed on a cool ­surface B. Convection from wet ­linens C. Evaporative via ­f ree-­flow oxygen delivery 124. The variability in degree (severity) of phenotypic expression seen in individuals with the same mutant genotype is known ­as A. ­Segregation B. ­Homogeneity C. ­Expressivity 125. Cortical perception of pain is complete before A. ­16 B. ­20 C. ­24

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126. Sammy is a 35-week gestation infant just born and diagnosed with fetal alcohol syndrome. His mother reports drinking “heavily” while pregnant and does not want any interaction with the baby. Due to the baby’s presentation and history, you would anticipate withdrawal symptoms to ­occur A. Within the first 12 hours of ­life B. Between 24 and 48 hours of ­life C. Between 48 and 72 hours of ­life 127. Systemic inflammatory response syndrome (SIRS) can best be defined ­as A. Sepsis with cardiovascular ­failure B. Sepsis with accompanying organ ­failure C. Tachycardia or tachypnea with a ­fever 128. Which of the following statements about extracorporeal membrane oxygenation (ECMO) is ­true? A. ECMO is used for treatment of pulmonary ­hypoplasia B. ECMO replaces nitric oxide as the therapy of choice for ­PPHN C. ECMO should not be used in cases of intravascular ­hemorrhage 129. The infant you are caring for is suspected of having Hirschsprung’s disease. What diagnostic procedures do you anticipate the infant to undergo prior to treatment of this ­disease? A. Plain abdominal films and blood work including amylase and ­lipase B. Rectal biopsy, plain abdominal ­X-­rays, and barium ­enema C. Rectal biopsy and ­MRI 130. You are caring for a 24-week gestation female delivered vaginally yesterday. She was intubated after six attempts with a 2.0 ETT at 6.25 cm at lip. There was vocal cord and oral mucosal damage with bleeding. Arterial blood gases show worsening respiratory acidosis with RDS on chest ­X-­ray. Despite surfactant therapy, conventional ventilation is ineffective. Which mode of ventilation would be contraindicated for this ­patient? A. ­High-­f requency jet ­ventilation B. ­High-­f requency ­flow-­interrupted ­ventilation C. ­High-­f requency oscillator ­ventilation 131. Damian is a 32-week gestation neonate who is now 34-weeks adjusted gestational age. You are beginning trial oral feedings because of noted increase in quiet alert state, vigorous sucking on the pacifier without desaturation, and rooting during kangaroo care. Successful transition to oral feeding with breast and/or bottle ­is A. Dependent upon nursing skill in feeding with a bottle and/or assisting with ­breastfeeding B. With assistance of speech or occupational therapy in creating a feeding ­plan C. The infant’s ability to maintain physiologic stability, a flexed posture, and a quiet alert ­state

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132. You are completing discharge teaching to a foster parent for a term baby treated for cocaine withdrawal. The baby still has moments of stiffness and rigidity with arching. What would you suggest to the foster parent regarding ­positioning? A. Always wrap tightly and position him supine when he is ­arching B. Use forceful hand containment to obtain a flexed ­position C. Use a hammock or sling bed for ­sleeping 133. Prolonged TPN administration can lead to which of the following ­conditions? A. ­Hypoglycemia B. ­Cholestasis C. Short bowel ­syndrome 134. A partial thromboplastin time (PTT) is used to ­assess A. The number of ­platelets B. The number of fibrin degradation products ­present C. Intrinsic and common portions of the coagulation ­cascade 135. Vernix should exist in skin folds at which level of ­gestation? A. 34–35 w ­ eeks B. 38–40 w ­ eeks C. 40–41 w ­ eeks 136. Which of the following drugs is a ­methylxanthine? A. ­Prednisone B. ­T heophylline C. ­Atropine 137. What neonatal movements can mimic seizure ­activity? A. Apnea and myoclonic ­jerking B. Benign nocturnal myoclonus, jitteriness, and nonconvulsive ­apnea C. Tonic deviation of the eyes and paroxysmal ­laughing 138. A mother’s breastmilk contains the immunoglobulin known ­as A. ­IgE B. ­IgA C. ­IgM

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139. A female infant weighing 907 g was delivered by emergency cesarean section at 28 weeks after complete placental abruption. At birth, there was absent heartbeat and the infant required immediate intubation and cardiopulmonary resuscitation. Cord pH was 6.9, bicarbonate was 17.6, and base excess was 222. Intratracheal epinephrine was given and the infant responded. Her Apgar scores were 1, 5, and 8 at 1, 5, and 10 minutes, respectively. The infant received surfactant and was placed on mechanical ventilation for respiratory distress syndrome. Umbilical venous (UVC) and arterial (UAC) catheters were placed. After resuscitation, the infant is likely to develop which of the following ­acid-­base ­abnormalities? A. Respiratory ­alkalosis B. Metabolic ­acidosis C. Respiratory ­acidosis 140. Temperature regulation is controlled by the hypothalamus with the release of which ­hormone? A. ­Epinephrine B. ­Norepinephrine C. ­Cortisol 141. Analyze the following arterial blood gas ­values pH ­7.51 CO2 ­40 HCO3 ­35 A. Uncompensated metabolic ­alkalosis B. Compensated metabolic ­acidosis C. Uncompensated respiratory ­alkalosis 142. Jose and Nita are parents of 24-week ­triplets—­Juanita, Sergio, and Tierra, born 5 days ago. Tierra was diagnosed with a grade IV intraventricular hemorrhage and cardiogenic shock and was removed from life support yesterday. Jose and Nita are crying at the bedside while visiting Juanita and Sergio today. What should you ­do? A. Tell them you are sorry for their loss and offer to listen or pray with ­them B. Do not say anything about Tierra, just update them about Juanita and ­Sergio C. Pull the curtain and give them ­privacy 143. Paul is a 36-week gestation and 22 ­day-­old infant who has been receiving metoclopramide for 2 weeks. You notice abnormal movements of his limbs. As a NICU nurse, you suspect Paul is suffering f­ rom A. Restlessness, with no underlying ­condition B. Extrapyramidal symptoms from the ­drug C. Focal seizures, and the physician must be notified at ­once

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144. While Nora is participating in kangaroo holding of her 29-week gestation son, Dalton, it is time for cares. At which point would you perform cares to lessen stress on the b ­ aby? A. When Dalton is asleep without ­movement B. When Dalton is asleep with movements of the chest and ­head C. When Dalton is ­crying 145. Which of the following drugs is a ­lusitropic? A. ­Milrinone B. ­Dobutamine C. ­T heophylline 146. You are assisting with the neonatal consultation for a 32-week gestation infant of a gravida 3, para 2, 18 ­year-­old mother named Brenna. Brenna appears euphoric and confused. While looking around the room you note a c ­ hewed-­up pacifier in the bed with her. Why would the pacifier be ­significant? A. Brenna’s other children were in the room just before you ­arrived B. Brenna may have been using the pacifier prior to your entering the ­room C. The pacifier was caught in the railing and ­torn 147. Leah was in a car accident when she was 16-weeks pregnant. She suffered neurologic injuries and was paralyzed for 2 weeks while intubated. She is now 35 weeks gestation and in labor. The neonate maybe at risk ­for A. Pulmonary ­hypoplasia B. Necrotizing ­enterocolitis C. Renal ­agenesis 148. Baby Lee is a 24-week gestation neonate at day 30 of life. He is intubated on conventional ventilation with a chest tube post pneumothorax. TPN, intralipids, and fentanyl are infusing via a r­ ight-­arm PICC line. You note mild general edema strong and bounding peripheral pulses, and capillary refill is , 3 seconds. Baby Lee’s heart rate is 180, respiratory rate is 76, and the O2 saturation is 92% on 30% FiO2 . Cuff blood pressure is 96/67 (MAP77). Your first action should be to A. Call the physician for a hydralazine ­order B. Verify that the cuff size you are using is half the arm ­diameter C. Check the IV site to verify ­patency 149. How does periventricular hemorrhagic infarction differ from periventricular ­leukomalacia? A. Periventricular hemorrhagic infarction is a diffuse arterial bleed, whereas periventricular leukomalacia is a venous ­bleed B. Periventricular hemorrhagic infarction is a unilateral venous bleed, whereas periventricular leukomalacia is a more diffuse arterial ­bleed C. They are both seen with ­f ull-­term ­infants

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150. You are assessing an infant in the nursery for hypotonia, weak suck, poor muscle movement, diminished grasp, and plantar reflexes. The infant chokes during feeding and is irritable. The mother was untreated during pregnancy for hypoparathyroidism. You ­suspect A. The infant is ­hypercalcemic B. The infant is ­hyperkalemic C. The infant is ­hypomagnesemic 151. You are receiving report for a 26-week gestation infant with respiratory distress and feeding intolerance at day 15 of life. The neonate is developmentally positioned in a “Snuggly” in a ­double-­walled isolette on servo control with the skin probe on the abdomen. The nurse reports a wide fluctuation in isolette temperature and that the skin probe reading does not always match actual temperatures taken. Which statement best explains these ­findings? A. The baby was lying on the temperature probe during some of the ­day B. The baby was wrapped too warmly in the developmental positioning ­device C. The temperature probe only records skin temperature at any given time, not core ­temperature 152. A father notes several 1 mm papules on the baby’s chin and cheeks. He asks you what would cause this. You should offer which of the following ­explanations? A. He should consult the doctor about birth ­defects B. The spots are normal and will disappear within a few ­weeks C. They are precancerous ­lesions 153. Jill is a 26 ­year-­old gravida 12, para 4 presenting in preterm labor at estimated 28-weeks gestation. She has had no prenatal care and refuses to provide health history. Her admitting weight is 100 pounds. A urine drug screen is positive for opioids, oxycodone, marijuana, and cocaine. What additional testing should be ­considered? A. Gonorrhea, Hepatitis D, and ­Candida B. Syphilis and Hepatitis B and ­C C. Gonorrhea, syphilis, and ­Candida 154. You are caring for a 26-week gestational patient on ­high-­frequency ventilation with transcutaneous CO2 monitoring (TCOM). You notice a sudden increase in PaCO2 levels with a sudden decrease in PaO2 levels. You s­ uspect A. Tension ­pneumothorax B. ­PDA C. ­VSD 155. The purpose of risk management is ­to A. Identify healthcare staff who are ­negligent B. Collect and store records regarding potential and actual injuries in the ­hospital C. Determine scope of practice within the hospital based on financial ­liability

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156. Sarah and Graham are the parents of a 30-week gestation neonate admitted to the intensive care unit for respiratory distress syndrome, possible sepsis, and prematurity. Sarah and Graham ask about what to tell the three older siblings about the baby being in the unit. Which of the following statements would be ­appropriate? A. “Your 2 y ­ ear-­old daughter will not understand that the baby is in the unit, but she may have separation anxiety with you visiting for great lengths of time.” B. “Tell your 5 ­year-­old daughter that the baby is sick and we are giving him shots to make him feel better, and she may visit when he is better.” C. “Tell your 11 ­year-­old son that since he is in school, that he cannot visit because of risk of infection.” 157. The parents of your 31-week gestation neonate, Zeke, are at the hospital to participate in kangaroo holding. Prior to placement skin to skin, Zeke’s temperature is taken and is 38° C. Which parent is the better choice for performing kangaroo care at this ­time? A. Neither; Zeke is too ­unstable B. Zeke’s f­ather C. Zeke’s m ­ other 158. Developmental positioning is crucial to prevent both skeletal and muscular deformities. Common deformities ­include A. Hip abduction and internal ­rotation B. Shoulder flexion and scapular ­abduction C. Neck extension and head ­molding 159. You are called to a vaginal delivery for a woman who received no prenatal care and has no amniotic fluid. Which of the following assessment criteria for her baby would support a diagnosis of Potter’s ­syndrome? A. Blunted nose with protruding chin, ­low-­set ears, and narrowly spaced ­eyes B. Small for gestation, bowed legs and clubbed feet, and severe respiratory ­distress C. Barrel shaped chest, large claw-shaped hands, and dry ­skin 160. Your patient has had recurrent episodes of supraventricular tachycardia, which are refractory to adenosine and vagal maneuvers. Another drug you might consider in the course of treatment for this infant would ­be A. ­A miodarone B. ­Digoxin C. ­Procainamide 161. A complication of persistent pulmonary hypertension of the newborn ­is A. Renal ­failure B. Systemic ­hypertension C. ­Apnea

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162. Marilyn and Frank are the new parents of a 23-week gestation neonate who delivered via emergency cesarean section due to uterine rupture. Initially they say little while visiting and have a glazed expression while at bedside. The nurse recognizes this ­as A. The initial response to a ­crisis B. The initial steps to ­coping C. Adaptation to the intensive care ­environment 163. A ­ cid-­base regulation in the body is via the lungs and the kidneys. Which of the following statements is true regarding ­acid-­base ­regulation? A. Acids are hydrogen (H1 ) ­receptors B. HCO32 is a buffer that binds with free hydrogen to form carbonic ­acid C. Respiratory imbalances are determined by assessing HCO32, and metabolic imbalances are determined by assessing ­pCO2 164. Your term patient was admitted an hour ago to the NICU with aortic regurgitation. Additional clinical findings are elongated extremities and fingers, kyphosis, a decreased ­upper-­to-­lower segment ratio, and retinal detachment. This infant probably h ­ as A. Gaucher ­disease B. DiGeorge ­syndrome C. Marfan ­syndrome 165. An infant is determined to be healthy if he demonstrates which of the ­following? A. A flexed ­posture B. A relaxed ­posture C. ­Hypertonicity 166. Your newborn’s breasts appear enlarged and have a milky discharge. This finding might i­ ndicate A. ­Infection B. Maternal ­estrogen C. Trisomy ­13 167. In fetal circulation, oxygen diffuses from maternal uterine arteries in the placenta. From the placenta, the oxygenated blood flows through which of the following vessels to the ­fetus? A. The superior vena ­cava B. Ductus v ­ enosus C. The umbilical ­vein

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168. A female infant born at 30-weeks gestation develops abdominal distention, abdominal tenderness, and bloody stools on the 3rd day of life. Which of the following statements regarding the most likely diagnosis of necrotizing enterocolitis is ­correct? A. The diagnosis is supported by a double bubble sign on abdominal ­radiographs B. The diagnosis is supported by thickened bowel walls and pneumatosis intestinalis on abdominal ­radiographs C. The diagnosis is supported by a ­soap-­bubble appearance on abdominal ­radiographs 169. During your first assessment you note that your 29-week gestation patient on day 3 of life has a right flank mass with frank blood noted in the diaper. You do not see any open wounds and the anus appears normal. Your patient is NPO and receiving nutrition via TPN and intralipids via a UVC. Arterial blood pressure is monitored via a UAC and indicates hypertension. Phototherapy was started yesterday for a bilirubin level of 6.4 mg/dl. Morning labs show a Hgb of 12.9 gm/dl, Hct of 45%, and PLT count of 36,000. You notify the physician immediately because you ­suspect A. Renal vein ­thrombosis B. Renal artery ­thrombosis C. A Wilms’ tumor 170. A ­full-­term infant born breech has extensive molding of the head. What type of neurovascular hemorrhage would you expect in this ­infant? A. Periventricular hemorrhagic ­infarction B. Infratentorial subdural ­hematoma C. Subarachnoid ­hemorrhage 171. The primary reason to determine gestational age is ­to A. Provide statistical ­data B. Prevent ­complications C. Anticipate problems with ­development 172. If an infant requires oxygen at home, the parents should be taught ­to A. Secure oxygen tanks in a horizontal ­position B. Have the child wear synthetic clothing to reduce static ­electricity C. Use ­lemon-­glycerin swabs to reduce oral ­dryness 173. An action of nitric oxide ­is A. Vascular smooth muscle ­relaxation B. To increase pulmonary vascular ­resistance C. To release ­macrophages

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174. What type of murmur is commonly found in ­low-­birth-­weight i­ nfants? A. A ­mid-­diastolic ­murmur B. A pulmonary ­murmur C. A harsh holosystolic ­murmur 175. You are evaluating a 3-­day-­old infant with significant respiratory distress. He was delivered by emergency cesarean section at 42 weeks gestation because of fetal distress. You note that he has an oxygen saturation of 76% in room air that increases to 95% with administration of 100% oxygen. Which of the following statements is most accurate regarding persistent pulmonary hypertension of the newborn (PPHN)? A. PPHN occurs most frequently in premature infants, but may occur in ­post-­term ­infants B. PPHN usually resolves ­spontaneously C. Adequate oxygenation is the best preventive measure and ­treatment

This concludes the RNC Practice ­Examination.

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RNC Practice Examination A ­ nswers 1. Correct answer: ­A The pH is outside the normal range, so it is uncompensated. The pH is elevated, indicating alkalosis. The HCO3 is normal and the PaCO2 is decreased, and that indicates respiratory ­alkalosis. 2. Correct answer: ­A SBAR is an acronym for situation, background, assessment, and recommendations when reporting patient care to another healthcare professional. SBAR is used to organize information succinctly to provide rapid report and recommendations for actions to physicians, advanced practice personnel, another nurse, social services, and ancillary departments. Situation refers to what information is most important or critical to patient care at this time. Background provides the necessary history that has brought the patient into the unit. Assessment includes objective and subjective data relevant to patient situation. Recommendations refers to the course of action that the reporting staff member believes should occur to improve patient situation. For example, recommendations for a term neonate with a glucose of 25 mg/dL and emesis would be to provide intravenous glucose and start a maintenance intravenous infusion with hourly glucose until the neonate is ­stable. 3. Correct answer: ­B Most ABG machines are calibrated to 37° Centigrade. If the patient has a fever, the oxyhemoglobin curve will be shifted to the right. More oxygen will be given off to the tissues, so the machine has to be calibrated to account for the ­temperature. 4. Correct answer: ­A This collection of symptoms is classic for esophageal atresia and often occurs with a tracheoesophageal fistula. This condition occurs in 1 in 25,000 infants. Esophageal atresia is a failure of the esophagus to complete the connection between the throat and stomach, forming a blind pouch. There is often a fistula between the esophagus and the trachea called a tracheoesophageal fistula. Assessment of infants with this condition includes insertion of a nasogastric tube into the blind pouch, then taking a chest ­X-­ray. Depending on the severity of the atresia it is possible to close the fistula. It is important to close the fistula as soon as possible to reduce the risk of aspiration pneumonia. A ­G -­t ube may be inserted for feedings and the nasogastric tube placed on low suction. Approximately half the infants born with esophageal atresia have other congenital problems such as VATER association, other GI abnormalities of the anus and rectum, renal malformations, cardiac abnormalities, and skeletal deformities. Nursing care includes elevating the head of the bassinet, providing IV fluids, oxygen, and maintaining the NG ­t ube. 5. Correct answer: ­C Infants lose approximately ­one-­third of their insensible water via the respiratory tract, but ­t wo-­thirds through the skin. In this scenario, phototherapy contributes most to insensible water loss. Additional factors that increase water loss include the patient’s gestational age, skin breakdown, use of radiant warmers, elevated body and

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environmental temperatures, and congenital skin defects. Use of the ­double-­walled isolette and humidity are also utilized to counter insensible water ­loss. 6. Correct answer: ­B The patient is exhibiting possible symptoms of congenital adrenal hyperplasia (CAH). The most common form of CAH is 21-hydroxylase deficiency (21-OHD). Without this enzyme, progesterone cannot be broken down into cortisol or aldosterone. Absent cortisol causes an overproduction of adrenocorticotropic hormone (ACTH) by the adrenal cortex, resulting in higher levels of progesterone and resulting in pronounced virilization. Serum studies for 17-hydroxyprogesterone (17-OHP) will be elevated if the diagnosis is correct. Without the ability to break down 17-OHP into cortisol and aldosterone, marked salt and water wasting occurs with resulting hypovolemia. Presentation may initially be diagnosed as renal insufficiency. CAH is screened for by over 32 states within the newborn screening blood ­tests. 7. Correct answer: ­A Weaning is usually deemed successful if the neonate can be consoled, scores an 8 or less on the neonatal abstinence scoring system, maintains a rhythmic sleep and feeding cycle, and has steady weight ­gain. 8. Correct answer: ­C Baby M. has diabetes insipidus. This baby has poor skin turgor, depressed anterior fontanelle, mottled skin, weak pulses, low blood pressure and is lethargic. Lab results indicate a urine specific gravity of 1.005, an elevated serum osmolality and a low urine osmolality. Urine output the past 24 hours has been 6.5 ­ml/kg/hr. 9. Correct answer: ­A AFP screening done at 15–22 weeks into the pregnancy can detect up to 80–90% of all neural tube defects. This test can detect 80% of spina bifida aperta and 90% of ­anencephaly. 10. Correct answer: ­B EMLA cream is frequently used for topical anesthesia for procedures such as circumcisions, arterial punctures, venipunctures, and PICC line placement. EMLA contains lidocaine 2.5% and prilocaine 2.5%. EMLA works by releasing lidocaine and prilocaine from the cream into the epidermal and dermal layers of the skin and by the accumulation of lidocaine and prilocaine in the area of dermal pain receptors and nerve ­endings. 11. Correct answer: ­B The most common ­single-­gene disorder (autosomal recessive) in Caucasian populations is cystic fibrosis. Autosomal recessive inheritance means that two copies of an altered gene located on one of the autosomes (that is, not the X or Y chromosomes) must be present for an individual to be affected with the trait. The parents would both have the gene pattern Rr—­this is a ­heterozygote. An affected individual would get an rr pattern—­this is a ­homozygote.

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The risk of two heterozygotes, or carriers, having an affected child is 25% (1 in 4) for each child whom they have. There is a 3 in 4 chance that each child will not be ­affected. Males and females are at equal risk for being ­affected. Two affected individuals usually produce children all of whom are ­affected. 12. Correct answer: ­A Tetralogy of Fallot results in low oxygenation of blood due to the mixing of oxygenated and deoxygenated blood in the left ventricle via the VSD and mixing of blood from both ventricles through the aorta due to the obstruction of blood flow through the pulmonary valve. This causes a ­r ight-­to-­left shunt. The primary symptom is low blood oxygen saturation with or without cyanosis from birth or developing in the first year of life. If the baby is not cyanotic, then it is sometimes referred to as a “pink tet.” Other symptoms include a harsh grade II to IV systolic murmur with a thrill, difficulty in feeding, failure to gain weight, and retarded growth and physical development. Polycythemia may be present with dyspnea on exertion, and clubbing of the fingers and toes is also seen. Children with Tetralogy of Fallot may exhibit “tet spells”. The precise mechanism of these episodes is unknown, but may result from a transient increase in resistance to blood flow to the lungs with increased flow of desaturated blood to the body. Tet spells may be precipitated by activity and are characterized by paroxysms of hyperpnea, irritability and prolonged crying, increasing cyanosis, and decreasing intensity of the heart murmur, and it may result in hypoxic brain injury and death. Older children may squat during a tet spell, which cuts off circulation to the legs, raises intrathoracic pressure and systemic vascular resistance, and therefore improves blood flow to the brain and vital ­organs. 13. Correct answer: ­B The magnesium sulfate used to treat the mother’s p ­ regnancy-­induced hypertension will cause the neurologic portion of the assessment to be underscored. Other treatments that cause sedation, muscle relaxation, or neurologic depression may have similar affects on gestation assessment. Neonatal factors that may alter scoring include neonatal neurologic disorders, breech presentation, deformities, and ­asphyxia. 14. Correct answer: ­A AV canal defects are also known as endocardial cushion defects. The atrioventricular junction is flanked by two masses of endocardial cushions—­a superior and an inferior cushion. The septum grows towards the atrioventricular endocardial cushion and fuses with it. The atrioventricular valves form during the 5th to 8th week of development. When the atria and inlet portion of the ventricle enlarge, the sulcus drops into the ventricular cavity, forming a hanging flap. The endocardial cushion tissue is located at the tip of this flap. Nonunion can cause an atrial septal defect and a ventricular septal defect to form. These defects will cause ­left-­to-­r ight shunting, leading to congestive heart failure. Patients with AV canal defect develop elevated pulmonary vascular resistance secondary to right ventricular hypertension and increased pulmonary blood flow. Upper airway obstruction occurs in patients with Trisomy ­21.

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15. Correct answer: ­C Anatomic dead space is defined as a conducting airway. Conducting airways (e.g., trachea, bronchi) are ventilated, but perfusion (gas exchange) does not take place. Wasted ventilation is the amount of ventilation that does not participate in gas ­exchange. 16. Correct answer: ­C Fetal urination is most pronounced during the third trimester. During the third trimester, the fetal kidneys are critical to the regulation of amniotic fluid present in the amniotic sac. A reduction in fetal urine will lead to significant oligohydramnios. Postdelivery management should include close monitoring of input and output with renal ultrasound if continued deficiencies are ­noted. 17. Correct answer: ­A An omphalocele is the herniation at the umbilicus of abdominal contents, contained in a sac that may include the large and small bowel, liver, pancreas and spleen. Omphalocele is often seen with other congenital abnormalities such as other GI disorders, congenital cardiac anomalies, and chromosomal disorders. Gastroschisis is the herniation of the intestines only through an abdominal wall defect. There is no sac covering the intestines in ­gastroschisis. 18. Correct answer: ­A Disseminated intravascular coagulation is an overstimulation of the clotting cascade. Both the intrinsic and extrinsic pathways are activated at the same time. This causes an acceleration of the clotting process. When the clots lyse, the fibrin split products are anticoagulants. Eventually, all the clotting factors are used up, and no further clots can form. Neonates are at increased risk for DIC secondary to inappropriate levels of anticoagulants and fibrinolytics, along with decreased levels of antithrombin and protein. One of the treatments for DIC is Antithrombin III, which sometimes attenuates organ failure and reverse ­coagulopathy. 19. Correct answer: ­A Cerebrospinal fluid (CSF) is primarily produced by the choroid plexus of the lateral ventricles. The CSF flows through the foramen of Monro, into the third ventricle, through the aqueduct of Sylvius into the fourth ventricle. The fluid then travels through the foramina of Luschka and Magendie, where it bathes the spinal cord and brain before returning to the ventricular cisterns and subarachnoid space before being reabsorbed by the arachnoid ­villi. 20. Correct answer: ­B Improved circulation is evidenced by warm extremities. A faster heart rate and extended capillary refill are not indicators of ­improvement. 21. Correct answer: ­C Prior to starting any medications or fluids, confirm line placement with the physician or radiologist to avoid injury to the patient. The umbilical artery catheter tip should be located within T6–T9 for a high line and between L3 and L4 for a low line. The umbilical

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venous catheter tip should be located just above the diaphragm or only 2–5 cm in, depending on gestation and weight. Never advance a catheter if the sterility has been compromised. This patient should have the arterial line withdrawn to T6–T9. Repeat the chest ­X-­ray to confirm the new tip placement. Originating from the aortic arch, the coronary and subclavian arteries (above T5), celiac and superior mesenteric arteries (T11–T12), renal artery (L1), the inferior mesenteric arteries (L2), and the iliac arteries (L4–L5) may be damaged by fluids and medications administered via a misplaced umbilical artery catheter. A venous catheter tip in the liver may cause irreversible damage to the ­liver. 22. Correct answer: ­C Prematurity is present in almost all cases of respiratory distress syndrome. Surfactant production is usually diminished, and this leads to atelectasis and pulmonary edema. The patient worsens and an alteration in the ventilation perfusion ratio occurs, and functional residual capacity is diminished. Serum protein leaks into the alveoli and inhibits surfactant function. The immature lung lacks the ability to clear this fluid and the neonate’s condition is more compromised because of decreased surface area for gas exchange. The collapsed alveoli fill with a ­protein-­r ich fluid that forms hyaline membranes, giving the condition its former ­name. Spasm of the pulmonary arteries results in blood being shunted away from the lungs via the foramen ovale and ductus arteriosus. These abnormalities all result in respiratory failure with poor oxygenation of the ­blood. 23. Correct answer: ­B This baby is in secondary apnea as the baby did not respond to previous stimulation while drying and suctioning. Immediate action should include providing positive pressure ventilation with 100% oxygen for 30 seconds. As research becomes available, the concentration of FiO2 recommended currently at 100% may change. Please refer to the most current NRP provider manual from the American Academy of Pediatrics for any changes since publication of this ­text. 24. Correct answer: ­C To achieve the most accurate gestation score, the neonate should be assessed twice within 12 hours. To limit preconceived expectations of a neonate’s gestation from altering the score, the assessment should be done by two different evaluators. Before conducting the assessment, be cognizant of physical and neurologic factors that may influence ­scoring. 25. Correct answer: ­A A diaphragmatic hernia is a medical emergency. If there is a defect in the development of the diaphragm, the abdominal contents can herniate into the thorax, preventing lung development. Because of this, it is often difficult to tell if the lungs are developed enough to sustain life. These infants are at great risk for pneumothorax. Pulmonary hypertension is a postoperative risk that may require special ventilation. One third of infants with pulmonary hypertension die even with ­treatment. 26. Correct answer: ­A Factor VIII deficiency is also known as von Willebrand’s ­disease.

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27. Correct answer: ­A This is a medical emergency. The neurosurgeon needs to be notified immediately. If an infant with a ventriculoperitoneal shunt becomes lethargic, this means the shunt is not working for some reason, and there is an increase in the ­ICP. 28. Correct answer: ­A Excess fetal insulin may be the cause of delayed maturation of type II alveolar cells and pulmonary surfactant deficiency. The excess insulin may cause congenital cardiac anomalies such as cardiomyopathy and intraventricular septal ­hypertrophy. 29. Correct answer: ­A Neonatal visual acuity is estimated at 20/400 for term infants. It is important to teach parents that due to vision at 20/400, the infant best sees or focuses in on objects that are placed 6–10 inches from the ­face. 30. Correct answer: ­B Type II truncus arteriosus is characterized by the right and left pulmonary arteries having separate origins. A short pulmonary artery may arise from the base of the common trunk (type I), or the branch pulmonary arteries may arise separately but in close proximity from the posterior surface of the common trunk. The right and left pulmonary arteries may arise widely separate from the lateral aspects of the truncal artery (type III). Truncus arteriosus results in ­left-­to-­r ight shunting of blood and pulmonary overcirculation. Congestive heart failure and pulmonary artery hypertension are common. Pulmonary vascular obstructive disease will occur early if no intervention is ­made. 31. Correct answer: ­C High humidity and the ­double-­walled isolette aid in fluid regulation of the extremely ­low-­birth-­weight patient by minimizing transepidermal water loss (TEWL). Due to poor or underdeveloped dermal and epidermal layers in the skin, TEWL increases as gestational age decreases. Water loss can be attributed to poor keratinization, high water content, limited subcutaneous fat, large surface area, and skin vascularity on the premature neonate. TEWL is closely related to ambient humidity until a fully functional stratum corneum can develop, which may take several weeks. Failure to calculate and replace TEWL fluid losses will lead to dehydration and hypotension. Although the ­double-­walled isolette does inhibit some sound, it is not the primary reason for use in this patient. The double wall assists in maintaining humidity and thermoregulation. High humidity places the infant at risk for infection and reverse isolation techniques should be ­utilized. 32. Correct answer: ­A Placental function and inherent maternal resources are critical maternal factors to consider when planning care of the neonate prior to and after delivery. Placental function determines the rate and flow of nutrients and oxygen to the fetus as well as cellular waste products flowing away from the fetus. The placenta also plays a critical role in the physiologic changes within the mother that increase glucose and essential elements to the fetus during development. Maternal resources comprise the mother’s

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ability to obtain and maintain adequate and appropriate nutrition, cardiovascular and respiratory function in order to transport oxygen, carbohydrates, fats, vitamins, minerals, amino acids, and more to the fetus. If maternal resources are compromised, then fetal health becomes compromised. Additional complications to adequate maternal resources include preexisting medical and mental health conditions, drug ingestion, and ­exercise. 33. Correct answer: ­A Vitamins A, D, E, and K are ­fat-­soluble vitamins that are absorbed in the ileum. The ileum is responsible for the absorption of the majority of the nutrition consumed, B12 , and bile salts in addition to the ­fat-­soluble vitamins. In the neonate, the two most common causes of short bowel syndrome are volvulus and necrotizing ­enterocolitis. 34. Correct answer: ­B Epinephrine may be useful in the treatment of TTN. Endogenous catecholamines assist the lung epithelium to transition, in the first few minutes after birth, from a secretory to an absorptive mode by inducing active sodium transport across the pulmonary epithelium. Catecholamines aid in enhancing absorption of fetal lung fluid, so it is plausible that a relative catecholamine deficiency may play a role in the etiology of TTN, and that administration of exogenous catecholamines may be an effective treatment. Delivery by cesarean section, especially prior to the onset of labor, is associated with an increased risk of TTN. Levels of circulating catecholamines are higher in newborns delivered vaginally than in newborns delivered by cesarean section, possibly due to the stimulation of catecholamine release from the stress of ­labor. 35. Correct answer: ­A A popliteal angle of 160° would be consistent with a gestational age of 24 weeks. Thinning lanugo and flat areola with no bud are associated with gestations between 28 and 30 ­weeks. 36. Correct answer: ­B Asymmetrical IUGR has a lower weight and shorter length compared to head circumference while symmetrical IUGR has a proportional decrease in growth. With symmetrical IUGR, cells have a decreased growth potential or there are fewer cells. Asymmetrical IUGR results in a normal number of cells, but with reduced size. Symmetrical IUGR is associated with congenital infections, malformations, and errors in chromosomes. Asymmetrical IUGR is associated with external factors such as placental function and maternal ­malnutrition. 37. Correct answer: ­A VATER also includes VATERR, VACTRL, and VACTERL associations. VATER includes vertebral anomalies, anal anomalies, tracheoesophageal fistula, esophageal atresia, and renal anomalies. The other forms of this condition may include cardiac defects, radial dysplasia, limb anomalies, and single umbilical artery. The cause of these anomalies is unknown, but it is believed the defects begin as early as 4–6 weeks of gestational age. VATER association may require numerous surgeries to correct the anomalies as a child with the condition ­grows.

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38. Correct answer: ­A In this type of anemia, the erythrocytes are very thin and fragile. They are known as target cells. The serum bilirubin is very elevated, so you should caution the parent against scratching. Cool water and lotion may be used for skin care. Oral stimulants could lead to vasoconstriction. The integrity of the skin is weakened, so careful positioning is paramount. The patient may require ongoing transfusions. Multiple transfusions may actually lead to too much iron, which might have to be chelated ­out. 39. Correct answer: ­C Stimulation of the anterior pituitary gland is from the hypothalamus by way of the vascular ­system. 40. Correct answer: ­C Epicardial pacing wires placed for postoperative cardiac management are placed because ­dual-­chamber pacing is most important after surgical repairs near the cardiac conduction system. The wires are placed in the right atrium and right ventricle. The wires may stay in place up to 10 ­days. 41. Correct answer: ­B Hypertrophic pyloric stenosis is seen 2–3 weeks after birth with nonbilious vomitus that is usually formula. This condition is often seen in infants with Trisomy 18, tracheo­esophageal fistula, and duodenal atresia. These children are at high risk for fluid and electrolyte imbalances. Hypertrophic pyloric stenosis may be corrected with a ­pyloroplasty. 42. Correct answer: ­C A ­T-­piece resuscitator or ­flow-­inflating ­bag-­mask-­valve would be appropriate methods for providing continuous positive airway pressure (CPAP). A ­self-­inflating ­bag-­mask-­valve requires the user to squeeze the bag to provide oxygen. Current NRP guidelines recommend that the initial oxygen begin at less than 100% FiO2 during resuscitation of a premature neonate and that oxygen be titrated for saturation via pulse ­oximetry. 43. Correct answer: ­C It is the responsibility of the transport team to function and care for the neonate within the standards of care for stabilization. The ER physician may not be aware of or current to the standards of care in newborn stabilization. It would be appropriate to politely decline the order and contact the accepting neonatologist for orders while following standard stabilization practice. Dextrose concentrations of 25% and 50% provide a bolus of glucose that would cause profound hyperglycemia followed by rebound hypoglycemia, as well as severe damage to the ­vessels. 44. Correct answer: ­B Sepsis may result in lung tissue injury and resultant increased pulmonary vascular resistance (PVR). Prostaglandin and oxygen therapies result in pulmonary vasodilatation and decreases ­PVR.

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45. Correct answer: ­C At 30-weeks gestation, this neonate is preterm. The weight, length, and head circumference are all above the 90th percentile and would classify this neonate as preterm LGA (large for gestational age). 46. Correct answer: ­C Based on the maternal and fetal history and vital signs provided, the resuscitation team should anticipate respiratory distress syndrome. Elevated maternal glucose levels inhibit the normal production of surfactant in the fetus, resulting in respiratory distress syndrome. Additional postdelivery complications of a macrosomic infant could include pronounced hypoglycemia due to a fetal hyperinsulinemia, hypocalcemia, hypomagnesemia, and polycythemia resulting in hyperbilirubinemia and ­hypertension. 47. Correct answer: ­B PPHN is the result of ­r ight-­to-­left shunting through the fetal shunts at the atrial and ductal levels. The PVR and pulmonary artery pressures are also elevated. The high PVR and pulmonary hypertension restrict pulmonary blood flow, and this, in turn, promotes hypoxemia, acidemia, and lactic acidosis. Most newborns are diagnosed within the first 24 hours of ­life. 48. Correct answer: ­C Duodenal atresia is seen in about 30% of infants born with Down syndrome. On ­X-­ray, a double bubble of gas is often seen. Treatment will include an NG tube, NPO status, and IV fluids. The infant may also receive ­antibiotics. 49. Correct answer: ­B This patient is in metabolic acidosis. The acidosis increases the risk of bilirubin toxicity, so it must be corrected prior to the transfusion. Increased hydrogen ions and an acidotic state impede bilirubin binding. At a serum pH of 7.1, bilirubin binding drops to half its potential for binding at albumin binding sites. During acidosis, free fatty acids are formed and compete with the albumin binding ­sites. 50. Correct answer: ­A An elevated hematocrit decreases the glomerular filtration rate (GFR), decreasing urine production. Other factors include decreased blood pressure, renal vasoconstriction, decreased glomerular capillary area, and prematurity of , 34 weeks gestation. 51. Correct answer: ­C The best approach to parent concerns is to address them directly then provide the reason for your actions. Isolettes may be seen as a clear but physical barrier to the parents as compared to an open access radiant warmer. To encourage parent bonding, support open communication, and provide teaching, the nurse’s best response is, “We will assist you in touching and holding your baby as he gets better, and the isolette allows for better temperature control and reduces stress to the baby.” Leaving an infant in the warmer may meet the mother’s needs, but it does not address the infant’s need for a stable, neutral thermal environment. Stating policy and ignoring parent concerns closes communication and removes parent involvement in patient care and further compounds barriers to ­bonding.

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52. Correct answer: ­B The best staff member to speak with the parents is the neonatologist or nurse practitioner with or without the obstetrician present to answer questions regarding prognosis. The nurse can only speak to nursing plan of care and should not be discussing medical plan of care with the parents. It is important to establish effective communication and trust with the parents prior to delivery if time is available to improve overall understanding and patient ­outcomes. 53. Correct answer: ­A The correct IV rate for a patient weighing 1260 g at 80 ml/kg/d is 4.2 ml/h. This is calculated by changing the grams to kilograms (1260/1000 5 1.260) and multiplying by 80 ml/kg and dividing the total by 24 h/d. 1.260 3 80 4 24 5 4.2 ­ml/h. 54. Correct answer: ­B On an EKG, l­eft-­sided heart failure results in wide, notched P waves. A tall, peaked P wave is indicative of ­r ight-­sided heart failure. Changes in ST segments (or T waves) usually indicate myocardial ­ischemia. 55. Correct answer: ­C Positive pressure ventilation and reduced lung compliance are significant predisposing factors for the development of PIE and barotrauma. Infants are often mechanically ventilated for other conditions such as respiratory distress syndrome (RDS), meconium aspiration syndrome (MAS), amniotic fluid aspiration, and infection. However, in extremely premature infants, pulmonary interstitial emphysema can occur at low mean airway pressure and probably reflects increased sensitivity of the stretch in an underdeveloped lung. Pulmonary interstitial emphysema has been rarely reported in the absence of mechanical ventilation or continuous positive airway pressure. PIE is a collection of gases inside the connective tissue surrounding the lung ­structures. 56. Correct answer: ­C A contraindication to the surgical correction for hypoplastic left heart syndrome would be significant tricuspid valve dysplasia. Answers A and B are a normal part of treatment for hypoplastic left heart syndrome. If the patient has significant dysplasia of the tricuspid or pulmonic valves, especially if they also have a functional issue, the surgery has a poor chance of ­success. 57. Correct answer: ­C Diuril inhibits pancreatic insulin release, resulting in hyperglycemia. Bedside glucose monitoring should be done at least daily to monitor for hyperglycemia. Additional electrolyte monitoring should include sodium, potassium, magnesium, chloride, bicarbonate, and phosphorous due to increased urinary ­loss. 58. Correct answer: ­A Hemorrhage in an infant with hydrops fetalis is usually the result of altered hepatic synthesis. Production of vitamin K and vitamin ­K-­dependent clotting factors may lead to hemorrhage. Initial signs of bleeding in an infant with hydrops fetalis may be oozing from cord and blood sampling sites and generalized petechiae. Hypoglycemia may also be ­present.

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59. Correct answer: ­C Approximately 50% of craniosynostosis occurs along the saggital suture. Cranio­ synostosis, the premature closure of one or more cranial sutures, occurs at a much lower rate along the other sutures with the lambdoidal suture being a rare ­occurrence. 60. Correct answer: ­C Pulmonary atresia with an intact ventricular septum is also known as PAIVS. The most common finding upon physical examination is central (perioral and periorbital) cyanosis. Following ductal closure, profound generalized cyanosis is present, and the apical left ventricular impulse may be pronounced. The first and second heart sounds are single. A pansystolic murmur is often heard at the left lower sternal border, consistent with tricuspid regurgitation. If severe, the murmur of tricuspid regurgitation may be associated with a thrill and a diastolic ­r umble. A systolic ejection murmur of the patent ductus arteriosus may be heard at the left second or third intercostal space, particularly after initiating prostaglandin ­infusion. 61. Correct answer: ­A Energy drinks contain massive amounts of caffeine and a variety of ingredients that will break down into methamphetamines and amphetamines with effects on the fetus similar to fetal alcohol syndrome. Immediate presentation includes small for gestation, dehydration, poor coordination for suck and swallow, hyperactive behavior, and sleep disturbances. ­Long-­term effects include learning disabilities, speech and language delays, poor judgment, and low intelligence ­quotient. 62. Correct answer: ­C Lorazepam is the drug of choice for neonatal subtle seizures. Subtle seizures can mimic normal movements of the infant. Some of the common movements seen with this condition include apnea, tonic deviation of the eyes, repetitive eyelid fluttering, drooling, chewing, sucking, swimming arm movements, pedaling of legs, and paroxysmal laughing. Dilantin and phenobarbital may also be used to treat subtle seizures in this infant. Carbamazepine is not indicated for subtle seizures. Diazepam is used for status ­epilepticus. 63. Correct answer: ­B The unpredicted situation, or deviation from expected events, that occurs with delivery of a premature neonate or a neonate with congenital anomalies is the major negative factor in parental stress. Preconceived expectations allow parents to adjust to changes in lifestyle and family dynamics. When anticipated events do not occur, parents are often left without sufficient coping mechanisms and are rapidly overwhelmed by circumstances, creating a crisis. The birth of a new family member and the resulting change in family dynamics are positive stress factors for a new ­parent. 64. Correct answer: ­C The infant assessment indicates a neonate who is less than 23-weeks gestation weighing under 400 grams. Per NRP guidelines, this infant is not viable, and resuscitation measures should be withheld. Estimated gestational dates provided by the mother may be inaccurate by 6 2 weeks. The findings are more consistent with the ultrasound assessment of 21 weeks. The family conference before delivery provided the

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opportunity for the practitioners to discuss viability and outcomes with the parents to prepare them for the potential outcomes. Care should now be focused on family grieving and emotional ­support. 65. Correct answer: ­B Similac PM 60/40 is frequently given to neonates with renal insufficiency. This formula has a low mineral content, low iron content, and balanced ­electrolytes. 66. Correct answer: ­C PEEP is useful in RDS because it can help open collapsed alveoli. Lowered cardiac output is a complication of PEEP. PEEP can cause barotrauma. PEEP must be regulated so as not to cause barotraumas, but still keep alveoli from collapsing during ­expiration. 67. Correct answer: ­B The eyelids will open normally between 26 and 30 weeks gestation. Each day the eyelids should be assessed visually for the development of a crease between the upper and lower lids. To determine degree of fusion, use slight or gentle traction to the upper and lower lids. The lids should not be forced open because this causes injury to the ­eyes. 68. Correct answer: ­A An acute intravascular hemolytic reaction may cause chills, fever, tachycardia, hypotension, hematuria, back pain, and the infant may exhibit additional signs of shock. The extravascular hemolytic reaction may manifest as fever, an elevated bilirubin, and a low H and H, even after ­transfusion. 69. Correct answer: ­A If an infant is rapidly growing, the content of sodium in breastmilk may not be sufficient and may lead to delayed onset of hyponatremia. Most often this occurs during the first week of life. Although Cafcit may lead to sodium loss due to diuretic effects, it is unlikely in this case because this patient was just started on this ­medication. 70. Correct answer: ­B In congenital diaphragmatic hernia, there is also some contralateral hypoplasia of the lung. The abdomen is usually scaphoid, and breath sounds are often absent on the side of the defect. In about 90% of cases, the defect is on the left side. Bowel sounds may be heard in the chest. Heart sounds may be heard on the right side because the herniated abdominal contents shift the mediastinum to the ­r ight. 71. Correct answer: ­A The baby who has strong respiratory effort and has strong tone with a heart rate . 100 bpm is considered vigorous. The baby in option B has a weak cry, heart rate . 80 bpm, and is grabbing the stethoscope. This baby should be provided stimulation and positive pressure ventilation (PPV) for 30 seconds. The baby in option C should be tracheally intubated and suctioned for meconium prior to stimulation. PPV may be needed for the baby in option C if apnea persists despite ­stimulation.

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72. Correct answer: ­B In preterm infants, the arterial oxygen tension should be maintained between 50 mmHg and 80 ­mmHg. 73. Correct answer: ­C To measure arm recoil during gestational assessment, the neonate should be supine. Hold the arms flexed for 5 seconds, then straighten the arms down and quickly release. The angle the arm flexes indicates maturity. The more mature the neonate, the less degree of angle between the forearm and the upper ­arm. 74. Correct answer: ­C For persistent cyanosis despite stable heart rate and strong respiratory effort, provide ­f ree-­flow ­( blow-­by) supplemental oxygen. Oxygen delivery may be by means of a ­flow-­inflating b ­ ag-­mask-­valve, cupped oxygen tubing, ­T-­piece, or neonatal simple oxygen mask for 30 ­seconds. 75. Correct answer: ­C The staff member using the ­T-­piece controls the ventilation rate and inspiratory time. The PIP or positive inspiratory pressure and ­pop-­off valve should be preset on the ­T-­piece machine front. The PEEP or positive end-expiratory pressure is set at the ­T-­piece circuit near the patient. Changes can be made with each breath to assist in preventing pneumothorax and overventilation/underventilation during resuscitation. The system allows the user to provide ­ventilator-­like support using either a mask or endotracheal ­tube. 76. Correct answer: ­C This baby has likely been exposed to cocaine during the pregnancy and prior to delivery. Cocaine increases the risk of fetal cerebral vascular accident and intraventricular hemorrhage due to the stress of the vaginal delivery. Cocaine use should be considered with any mother presenting with eclampsia without proteinuria. Maternal urine should be tested for benzoylecgonine, which is a metabolite only seen with ­cocaine. 77. Correct answer: ­A Ganciclovir is the optimal antiviral for active CMV. An infant with CMV must be monitored for severe neutropenia and hepatorenal toxicity. This medication helps reduce hearing loss associated with CMV, but brain damage may already be considerable at ­birth. 78. Correct answer: ­A An intrinsic factor that may contribute to the pathogenesis of congenital lobar emphysema is an adenoma. Answers B and C are extrinsic factors to the development of congenital lobar emphysema. Other intrinsic factors are infection, TB, retained secretions, absence of cartilage, stenosis, and ­hypoplasia. 79. Correct answer: ­C Karyotyping is a physical type of analysis. Cells are cultured and allowed to grow until the point that chromosomes are most clearly visible. The growth of the cells is then arrested and the chromosomes are stained and photographed. The chromosomes

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are then arranged in corresponding pairs according to function and size. With a complete map of genetic material, it is possible to determine whether the fetus’s chromosomes exist in the correct number, shape, and size. An abnormal number of certain chromosomes will indicate a genetic disorder. Karyotyping typically takes 10–12 days, but it may take up to 3 ­weeks. 80. Correct answer: ­B This infant probably had some degree of PIE initially. However, the air has migrated from the ruptured alveoli and has tracked to the mediastinum, causing a pneumomediastinum. If a tension pneumothorax existed, the infant would desaturate, become hypotensive and bradycardic, and be ­c yanotic. 81. Correct answer: ­B This infant is at risk for fetal alcohol syndrome, and the delivery staff should anticipate an infant who is small for gestation with possible microencephaly, facial anomalies with broad nasal bridge, cardiac defects, kidney defects, hearing defects, and growth restriction with short long bones. Common facial features include short palpebral fissures, maxillary hypoplasia, long and smooth philtrum (the space between the nose and upper lip), and a thin upper lip. Common cardiac defects are ventricular septal defect and atrial septal defect. The most profound effects are ­long-­term damage to the central nervous system with learning difficulties, memory and judgment failure, irritability, and hyperactivity during infancy and early ­childhood. 82. Correct answer: ­A Upon the infant’s admission, it is important to obtain a CBC, chemistry panel, and blood glucose immediately to rule out fluid and electrolyte imbalances. A ­high-­pitched cry, irritability, lethargy, and seizure activity may indicate hypernatremia. If the mother is exclusively breastfeeding, milk production is insufficient. Hypocalcemia and hypoglycemia both may cause seizures if they are not managed rapidly. Lab draws may be completed while awaiting radiology staff to perform the chest X-­ray. 83. Correct answer: ­B It is important to provide parents with information in lay terms that do not cause panic or increase anxieties. Telling the parents that “An echocardiogram was ordered for today to evaluate blood flow through Evan’s heart,” provides the parents with more accurate information than guessing or assuming what the study will find. Parents may not initially understand that the patent ductus arteriosus does not mean a cardiac defect or permanent heart disease. Parents may perceive the worst possible scenario rather than a standard diagnostic ­procedure. Stating that “Evan was really sick this morning, but we took care of it before you got here,” may lead the parents to believe that staff are hiding Evan’s condition, or are withholding information if the parents believe they are not being notified when he is sick. The more therapeutic statement would be, “We treated Evan with another dose of medicine this morning to improve his ability to breathe and provide oxygen to his tissues.” This provides correct information simply as a standard treatment for premature ­neonates. “We are beginning trophic or enteral gavage feedings so that Evan can wean off TPN and IL within the week” provides too much technical information without

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explanation. It is best to avoid definitive timelines or statements as outcome is not absolutely known. Parents may focus on time and perceive they are being lied to if the neonate does not accomplish the goal or action in the time stated. Definitive statements may be positive or negative, but they should be avoided unless the statement is absolutely ­correct. When speaking with parents, first establish their knowledge base, level of education, and preferred method of communication. This will provide staff with guidelines for providing information. Information should be accurate, timely, and not overly positive or negative. Ensure that parent understanding is correct by asking the parents to repeat what they heard in their own words. You should then provide corrections, clarification, and/or additional information as needed. Be aware that while under stress, parents may not hear or focus on every element of information and may need information repeated. Encourage parents to journal information and questions for future r­ eference. 84. Correct answer: ­B If a patient is retaining pCO2 , it is possible to help wash it out by increasing the respiratory rate (the ventilator rate in this case) or increase the tidal ­volume. 85. Correct answer: ­C Normal temperature for an infant is between 36.5 and 37.5° C (97.7–99.5° F). Mild hypothermia begins when neonatal temperature falls below 36.4° C or 97.6° F. Moderate hypothermia begins at temperatures below 35.9° C or 96.6° F. Severe hypothermia and death result when the temperature drops below 32° C or 89.6° F. Premature infants are at greater risk of temperature instability at considered normal ranges if there is concurrent sepsis, cardiac anomalies, or respiratory distress. Brown fat deposits are incomplete in the premature infant as the majority of brown fat deposits occur during the third trimester. Similar to a match, brown fat burns fast and hot and requires oxygen to be metabolized. If the neonate is hypoxic, then metabolism of brown fat is further restricted. Environmental factors greatly impact the neonate’s ability to conserve heat and ­self-­regulate temperature. Bed type, humidity, moisture, ambient temperature, air drafts, and the temperature of items touching the skin (equipment, fluids, and clothing) all influence neonatal ­temperature. 86. Correct answer: ­C The safe frequency for administration of acetaminophen to a 28-week gestational age infant is every 12 hours. More frequent dosing leads to toxicity, a potentially lethal ­condition. 87. Correct answer: ­C Standards of critical care should focus on both infant and parent response and interaction. Interventions of care should be individualized for each infant–parent group. Interventions are continually adjusted based on ongoing assessment of infant cues and response as well as parental involvement and interaction. Parental support and bonding should be encouraged and facilitated at every ­opportunity.

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88. Correct answer: ­B A high serum magnesium or phosphate level, low serum calcium, with catecholamine release results in secretion of parathyroid hormone. Bone breakdown is accelerated, releasing calcium into the blood. Calcium reabsorption is increased in the intestine and kidney tubule reabsorption is decreased. Phosphate loss is increased in the urine, which decreases serum phosphate. Parahormone increases reabsorption of magnesium by renal tubules. Renal calculi may ­develop. 89. Correct answer: ­B Bronchogenic cysts, especially when they are infected, can be the source of a pulmonary hemorrhage. Thoracic gastroenteric cysts have gastric mucosa and secrete acid. The cyst wall can be eroded and the erosion can extend to adjacent bronchial walls, vessels or lung parenchyma, resulting in massive bleeding. Lung abscesses, fungal infections, allergic bronchopulmonary aspergillosis, and lung parasitic infections can all produce massive pulmonary bleeding from erosion of airway and vessel walls. Bleeding from sickle cell disease usually occurs in ­adolescents. 90. Correct answer: ­A Cleft lip and/or palate is usually associated with DiGeorge syndrome, Pierre Robin sequence, and trisomy 13. All the answers for B and C are associated with clinodactyly, a curving or deviation of a ­finger. 91. Correct answer: ­C When utilizing transcutaneous PO2 measurements, underestimation of oxygenation may occur when the skin is hypoperfused or the calibration is improper. Overestimation of oxygenation may occur if there is an air bubble or leak between the electrode and the ­skin. 92. Correct answer: ­A A 44-week gestation infant would be expected to have leathery and cracked skin. If meconium is present, then the nail beds may be stained. Smooth skin would have visible veins and would be expected of a 29-week gestation infant, and rough skin with few veins would be expected in a 34-week gestation ­infant. 93. Correct answer: ­C If positive inspiratory pressure (PIP) is increased on a mechanical ventilator, the result will be to decrease the PaCO2 and increase the P ­ aO2 . 94. Correct answer: ­B The usual dose of fentanyl is 0.5–4 mg/kg administered slowly intravenously, and it may be repeated every 24 hours when used for sedation. Monitor closely for CNS and respiratory ­depression. 95. Correct answer: ­C The result of this blood gas analysis is an uncompensated metabolic acidosis. The pH is less than 7.35 so the value is uncompensated acidosis. To determine whether the

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acidosis is respiratory or metabolic, find the value that represents acidosis. This would be the HCO3 at , 22 mEq/L. 96. Correct answer: ­B Portagen is frequently used for infants with fatty acid metabolism deficiency. These patients are unable to digest and utilize the ­long-­chain fatty acids from ­foods. 97. Correct answer: ­C The patient is male and has the disorder. The mother has two copies of the X chromosome. She is a carrier because the disorder is recessive and is on both her ­chromosomes. 98. Correct answer: ­B Neonates with rickets often have a wider than normal wrist, hypocalcemia, craniotubes, and costochondrial swelling. Muscle tetany may also be ­present. 99. Correct answer: ­A Cardiac glycosides possess positive inotropic activity, which is mediated by inhibition of ­sodium-­potassium adenosine triphosphatase. Also, cardiac glycosides reduce conductivity in the heart, particularly through the atrioventricular node, and therefore have a negative chronotropic effect. The cardiac glycosides have very similar pharmacologic effects but differ considerably in their speed of onset and duration of action. They are used to slow the heart rate in supraventricular arrhythmias, especially atrial fibrillation, and also are used in chronic heart ­failure. 100. Correct answer: ­A With a meningocele, the dura mater and arachnoid are visible through the defect. Answer A could be spina bifida occulta. Answer B is spina bifida aperta. Answer C is a myelomeningocele, the most severe form of spina ­bifida. 101. Correct answer: ­B A neonate at 29-weeks will have poor resting tone with only slight flexion of the knees and ankles. Additional findings include smooth skin with visible veins and abundant lanugo, the soles will be smooth with only faint red marks. The pinna will be slightly curved and soft with slow recoil. The nipples will not have buds and the areolas will be flat. Females of this gestation will have prominent clitorises and enlarged minora, the males will have rare rugae on the scrotum and the testes are noted in the upper ­canal. 102. Correct answer: ­A This result signifies an uncompensated respiratory alkalosis. The pH is greater than 7.45 so the value is uncompensated alkalosis. To determine whether the alkalosis is respiratory or metabolic, find the value that represents alkalosis. This would be the CO2 at , 35 mmHg. 103. Correct answer: ­A The infant has failure to thrive and hepatomegaly, along with failure to thrive, vomiting, and diarrhea. Cataracts are noted and have an oil droplet appearance. These findings are highly suggestive for galactosemia. The baby will be hypoglycemic and

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have renal tubular acidosis. Galactose is present in all milk sources and must be metabolized to glucose for absorption from the intestine. If the condition is caught early, the baby can be managed on a ­galactose-­f ree diet. A neonate is especially susceptible to Escherichia coli septicemia, liver dysfunction, and bleeding ­tendencies. 104. Correct answer: ­A Information that has been coded in genetic material and is inherited by an individual is a genotype. A genotype is an organism’s genetic makeup, even if it is not ­expressed. 105. Correct answer: ­B After providing positive pressure ventilation (PPV) for 30 seconds, the next step is to insert an orogastric tube. If spontaneous respiratory effort returns and the heart rate is . 100 bpm, then you may halt PPV. If the heart rate is 60–100 bpm, then PPV must continue for an additional 30 ­seconds. 106. Correct answer: ­C High levels of oxygen have been associated with ROP, but lower levels of oxygen may lead to more respiratory complications (e.g., hypoxia), and death in premature infants. Better oxygen level monitoring has led to better control of oxygen administered to premature infants. Today, however, there is an increase in ROP due to the fact that neonatal care advances mean more ­low-­weight, premature infants are ­surviving. 107. Correct answer: ­A Due to the greater ­surface-­to-­body ratio of infants, heat loss is 3–4 times greater or faster for infants than ­adults. 108. Correct answer: ­C A deletion, by definition, is loss of a chromosomal ­segment. 109. Correct answer: ­C Negative relationships may develop at any point during the stay in the intensive care unit. Although it is good that the parents visit, staring at the monitor indicates a failure to adapt to the intensive care environment. In addition, the parents have failed to establish contact or interaction with the infant visually or tactilely. They cannot begin to accept the alteration in circumstances if they cannot accept the infant’s birth. This is a form of ­avoidance. Blaming oneself or the mother for an event preceding the delivery is common as parents and family adjust to the fact the neonate has not been born at term. As the nurse, you should acknowledge concerns and fears about causing premature delivery and then focus on infant presence and progress. Regardless of the cause for premature delivery or anomalies, the parents can be redirected to the infant and the actions they can take to establish a strong and healthy bond with their child. Blessings, baptisms, and last rites are expected methods of preparation for the possible death of the critical neonate by acknowledging death while maintaining an element of ­hope.

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110. Correct answer: ­A Small, raised, bright red areas around the diaper line and white plaques in the buccal area, cheeks, on the tongue or gums, usually indicates thrush. This infant has been on antibiotic therapy and there is probable overgrowth of ­bacteria. 111. Correct answer: ­A The infant should be treated for respiratory depression and the airway supported with positive pressure ventilation and intubation if needed. Time will resolve the respiratory depression. The sudden collapse of a young woman at a party without explanation is suspicious for Gamma hydroxybutyrate (GHB) or ketamine ingestion. These are known date or party rape drugs. Due to the anesthetic and amnesic effects of the drug, an affected female may be unable to describe events leading up to the collapse. To determine exposure, the drug screen should be repeated looking specifically for Gamma hydroxybutyrate (GHB) and/or ­ketamine. 112. Correct answer: ­B After teaching, parents should have the knowledge and confidence to identify and intervene when negative cues are present. Additional teaching is needed if the parents only call the nurse’s attention to the behaviors, but fail to become involved. Teaching during kangaroo holding should also include positioning in the flexed position with ­skin-­to-­skin touching from chest to umbilicus to maximize ­benefits. 113. Correct answer: ­C If the pH is high, potassium is driven into the cell, causing serum potassium levels to drop. Insulin will also force potassium back into the cell by stimulating the sodium/ potassium ­pump. 114. Correct answer: ­C The history indicates hypovolemia related to maternal blood loss and poor neonatal response to resuscitation. Since the airway has been secured with a moderate heart rate increase, volume expanders are now indicated. Initial resuscitation with 10 ml/kg of normal saline is appropriate if packed red blood cells are not immediately ­available. 115. Correct answer: ­A Due to the drying effects of the environment, the soles begin to dry, changing the appearance of creases inconsistent with the gestational age. Gestational assessments should be completed within the first 12 hours after birth to increase ­accuracy. 116. Correct answer: ­C Pregestimil is indicated for fat malabsorption treatment. Conditions that are associated with fat malabsorption include cystic fibrosis, short bowel syndrome, and protein malnutrition. Pregestimil protein is from medium chain triglycerides (MCT) ­oil. 117. Correct answer: ­C Acute pyelonephritis can cause acute intrinsic renal failure. Fluid management should focus on fluid restriction to prevent fluid overload. Urine output of 30 ml/kg/d plus for term infants and up to 70 ml/kg/day plus urinary output for preterm infants is the goal

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of fluid management. With a fluid restriction, the patient should have a 20–30 g/d weight loss. Once normal kidney function is established, strict fluid monitoring should include daily or twice daily weights. Nutritional support at 100 cal/kg with fat and carbohydrates and 1 to 2 g/kg/d of protein is necessary. Blood pressure should be closely monitored to provide early indication of congestive heart failure and renal ­damage. 118. Correct answer: ­A Examples of maternal conditions that will cause a neonate’s body to use or destroy platelets more quickly than they are produced are lupus erythematosus, rheumatoid arthritis, and ­pregnancy. 119. Correct answer: ­C The first retinopathy of prematurity (ROP) exam should be scheduled when the neonate is at 32-weeks gestational age or 5–6 weeks of life, whichever is greater. Normal vascular genesis begins about week 16 of gestation and proceeds from the optic nerve to the retina. At around 32-weeks gestation, vascular growth occurs toward the nasal bridge. Injury or an altered growth pattern becomes visible and pronounced with rapid irregular and torturous vessel development between established and avascular regions. If spontaneous recovery does not occur, laser surgery is used to halt torturous growth to decrease the risk of retinal ­detachment. 120. Correct answer: ­A Hypovolemic shock may be caused by skin integrity losses such as gastroschisis and myelomeningocele. Congenital heart lesions and arrhythmias are causes of cardiogenic ­shock. 121. Correct answer: ­C Levetiracetam is a ­second-­line drug for seizures that are resistant to phenobarbital, lorazepam, and other anticonvulsants. This drug should be weaned slowly to keep the threshold for seizure activity ­up. 122. Correct answer: ­A The steps of resuscitation are the same regardless of location. Upon delivery of the infant, begin drying, stimulating, and clearing the airway using available articles of clothing. The cord should be clamped using a tie, shoelace, or other material that clamps, but does not cut the cord. Do not cut the cord as this may provide an entry of bacteria into neonatal circulation. Wait for EMS personnel to cut the cord using sterile equipment. Clamping the cord is instrumental in transitioning neonatal circulation. The infant should be placed skin to skin and face up on the mother’s chest, then covered to maintain temperature. The floor is cold and the infant will lose heat via ­conduction. 123. Correct answer: ­A This baby is losing heat multiple ways. Heat loss is by conduction from being placed on a cold surface. The radiant warmer was not on long enough to warm the bed prior to the infant being placed on the surface. Conduction heat loss may also occur if the staff’s hands or stethoscope are cold when touching the infant. Heat loss via convection occurred when the unheated oxygen was delivered and by the cold temperature

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of the room. Convective heat loss is accelerated if a draft blows on or near the infant. Evaporative heat loss occurs when there is loss of heat through moisture, such as skin left wet in a cold room. Radiant heat loss is the final mechanism not mentioned in the scenario. This is a loss of heat between solid objects/surfaces not in direct contact with the infant, such as windows, cold walls, and a ­single-­walled ­isolette. 124. Correct answer: ­C The variability in degree (severity) of phenotypic expression seen in individuals with the same mutant genotype is known as expressivity. Expression can vary widely, even within the same ­family. 125. Correct answer: ­C Research reveals that cortical perception of pain is complete before 24-weeks ­gestation and should be considered and treated for in the ­neonate. 126. Correct answer: ­A Alcohol withdrawal symptoms may occur within the first 12 hours of life. Presentation includes hypertonia, tremors, feeding intolerance with poor coordination and weak suck, and opisthotonos (severe arching of the back and neck). Comfort measures should include decreased external stimulation with a darkened and quiet room, containment, and grouped ­cares. 127. Correct answer: ­C The international consensus conference in 2002 standardized the definition of systemic inflammatory response syndrome (SIRS). SIRS is defined as tachycardia or tachypnea with fever or high leukocyte count. Sepsis is defined as SIRS in the presence of suspected or proven infection; and severe sepsis is defined as sepsis with accompanying organ dysfunction. When cardiovascular failure occurs in the setting of severe sepsis, it is classified as septic ­shock. 128. Correct answer: ­C ECMO should not be used for intravascular hemorrhage because systemic heparinization is required. ECMO also increases the risk for intravascular hemorrhage in premature infants, especially those less than 35 weeks ­gestation. 129. Correct answer: ­B After plain films, a barium enema may be performed. The rectal biopsy is the best diagnostic tool for Hirschsprung’s disease. The biopsy shows a lack of parasympathetic ganglions in the distal bowel and rectum. This problem usually develops around the 12th week of gestation. Hirschsprung’s is seen in males four times more often than females and may also be seen in ­families. 130. Correct answer: ­A High-­f requency jet ventilation would be contraindicated in this patient because it requires reintubation with a ­triple-­lumen ETT. This patient had six intubation attempts with a 2.0 ETT with airway trauma. The risk of extubation and failed reintubation is high, and an adequate airway may not be obtained. If available, ­flow-­interrupted or oscillator ventilation is ­preferred.

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131. Correct answer: ­C The greatest indicators of successful transition to oral feedings via breast and/or bottle are determined by the infant’s physiologic stability, ability to maintain a flexed posture during feeding, and the presence of a quiet alert state. Although we might wish it was all nursing or ancillary support that results in successful oral feedings, the major component is the neonate. Staff involvement in feedings comes from our ability to recognize instability, support flexed position, and identify quiet alert states that impact feeding tolerance. Feeding plans, techniques, and advancement should first evaluate infant cues and then incorporate parent involvement to increase ­long-­term feeding ­success. 132. Correct answer: ­C The best position for an infant who is arching is to use a sling or hammock style bed to promote flexion. Although swaddling may decrease arching, it is best to avoid placing the infant supine while arching to avoid the head pressing against the mattress and the scapula from tightening. Additional methods for this parent should include hydrotherapy and massage. As arching may occur until age 1 year, it is recommended that this infant not be placed in a walker, as this promotes extension and not ­flexion. 133. Correct answer: ­B Cholestasis is the impaired flow of bile and may be seen with other conditions such as cystic fibrosis, biliary atresia, and infection. Cholestasis can also occur as a result of ­cholelithiasis. 134. Correct answer: ­C A partial thromboplastin time (PTT) is used to assess intrinsic and common portions of the coagulation cascade. The results may be affected by an absence of clotting factors, anticoagulants, low levels of clotting factors, inhibitors, and an increased use of clotting ­factors. A high PTT may be the result of hemophilia A or B, factor VIII or factor IX deficiency, liver disease, or ­DIC. 135. Correct answer: ­C Vernix, or the waxy coating on baby’s skin will be present in the skin folds at 40 to 41-weeks gestation. Vernix begins to be secreted by the sebaceous glands during the 20th week of ­gestation. 136. Correct answer: ­B Methylxanthines are an important classification of drugs. Caffeine and theobromine are also methylxanthines. They can be found in coffee, tea, and cocoa. This classification of drugs in low doses can stimulate cortical arousal and in higher doses cause insomnia. They can cause tachycardias and increase production of gastric acid and digestive enzymes. Methylxanthines inhibit histamine ­release. 137. Correct answer: ­B Neonatal movements that can mimic seizure activity are benign nocturnal myoclonus, jitteriness, and nonconvulsive apnea. All these movements are generally considered benign. All the other movements are indicators of seizures in the ­neonate.

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138. Correct answer: ­B A mother’s breastmilk contains the immunoglobulin known as immunoglobulin A (IgA). This immunoglobulin is also secreted in human colostrum. IgA does not cross the placental barrier and is the most common immunoglobulin in the gastrointestinal and respiratory ­tracts. During pregnancy and lactation, because of hormonal stimuli, IgA B lymphocytes colonize mammary glands and produce a specific secretory IgA that may bind to a pathogen and prevent infection. The antimicrobial effects of IgA antibodies are related to immune exclusion, interference, and an inhibited ability to adhere to the epithelial cell wall. Agglutination, neutralization, and immune elimination by phagocytosis and cytotoxicity may enhance the antimicrobial effects as well. ­HIV-­infected mothers do not evidence protection. IgA antibodies may enhance transmission of HIV ­infection. 139. Correct answer: ­B This infant is at risk for metabolic ­acidosis. 140. Correct answer: ­B Norepinephrine is released by the hypothalamus in response to chemical and temperature receptors in the skin, face and along the spinal column. Norepinephrine triggers a cascade of actions within the body to retain or create heat in the core. The efficiency of this cascade is impaired by gestation, weight, disease process, and respiratory function. Peripheral vasoconstriction shunts blood flow to internal organs to control heat loss through the massive surface area of the infant. Unfortunately, in attempting to raise temperature, the metabolic rate is also increased, resulting in a significant increase and consumption of oxygen and glycogen to burn brown fat or cause movement and flexion of extremities to generate heat. Norepinephrine also causes pulmonary vasoconstriction that inhibits normal blood flow through the lungs and inhibiting blood oxygenation. In premature and sick neonates, hypoglycemia and hypoxia may be compounded by a lack of brown fat, muscle tone, or sympathetic nervous system control. The best treatment for hypothermia or cold stress is appropriate and timely ­prevention. 141. Correct answer: ­A This result is an uncompensated metabolic alkalosis. The pH is greater than 7.45 so the value is uncompensated. To determine whether the alkalosis is respiratory or metabolic, find the value that represents alkalosis. This would be the HCO3 at . 26 ­mEq/L. 142. Correct answer: ­A Staff may be hesitant to interrupt grieving parents, but it is important to tell them you are sorry for their loss and offer to listen or pray with them as appropriate. This acknowledges the death, and allows parents the opportunity to talk. Loss is difficult for any parent, but multiple births pose another challenge when one or more die and at least one baby survives. If the other neonate(s) are critical, parents may be hesitant to bond for fear of additional loss. Often staff and family members are unsure how to deal with grief and avoid even mentioning the baby’s name. This is may give the impression to parents that you do not care, have no interest in their grief, or are not available as a support, and that may delay the grieving process. Positive interventions

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include reaffirming that they are the parents of multiples, affirm that the child did exist, ask about emotions, and provide support as needed. You involve social services, contact clergy if appropriate, and always be honest with your emotions. Encourage involvement with living sibling(s) to support healthy bonding and to affirm ­life. 143. Correct answer: ­B The infant is having extrapyramidal symptoms to the metoclopramide. Meto­ clopramide has antidopaminergic properties that affect the nervous system both centrally and peripherally. The drug also facilitates the release of acetylcholine— ­a neurostimulator that has a prokinetic ­effect. 144. Correct answer: ­B It is best to perform cares when Dalton is asleep with core movement of the head and chest. This indicates wakefulness and awareness of his surroundings without stress. The patient is quiet and ready for stimulation without negative effects. Crying indicates profound stress and cares would only increase the amount and severity of the negative stress as seen with increased cortisol levels. When an infant is in deep sleep without movement, brain maturation is occurring. This baby should be left alone until spontaneous movement is noted. Brain maturation—the development and myelination of neurons—during kangaroo holding may last 5 to 45 ­minutes. 145. Correct answer: ­A Milrinone is a class III phosphodiesterase inhibitor with inotropic, lusitropic (myocardial relaxation), and vasodilator ­properties. 146. Correct answer: ­B A c­ hewed-­up pacifier and maternal presentation of euphoria and confusion should lead the staff to consider ecstasy addiction and abuse. Ecstasy or methylenedioxymethamphetamine (MDMA) causes trismus, or teeth grinding. Users may chew on pacifiers to preserve teeth and hide the grinding from others. Most children using pacifiers will not chew, but suck on ­pacifiers. 147. Correct answer: ­A This neonate is at risk for pulmonary hypoplasia due to maternal neuromuscular depression during her hospitalization. Neuromuscular depression causes central nervous system depression in the fetus that decreases pulmonary growth during the critical growth period of 16 to 27 weeks gestation. If suspected, respiratory support should be provided immediately with expectations of poor compliance and cautious pressure management to limit risk of ­pneumothorax. 148. Correct answer: ­C Blood pressure may be elevated due to several factors. The first action should be to check IV patency of the fentanyl drip. Vital signs are elevated and may indicate pain related to the chest tube and intubation. Baby Lee also has generalized edema that may mask a PICC line infiltration. Treatment of potential causes of hypertension, including pain, hypervolemia, and titrating of inotropic medications, should be initiated prior to starting antihypertensive medications. Manual cuff size should be

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t­ wo-­thirds the extremity diameter. Since the baby is now 30 days old, the cuff size should be increased for growth. Moving the cuff site does not identify probable causes for the hypertension and may delay appropriate ­treatment. 149. Correct answer: ­B Periventricular hemorrhagic infarction (PHI) is a unilateral venous bleed seen in infants weighing less than 700 grams. PHI is distinguishable from periventricular leukomalacia (PVL) on CT or MRI scan because of the unilateral nature if this hemorrhage. The PVL is not limited to one hemisphere or side of the ­brain. 150. Correct answer: ­A Untreated maternal hypoparathyroidism may result in overproduction of parathyroid hormone in the neonate, resulting in hypercalcemia. History and clinical presentation are consistent with hypercalcemia. Hypomagnesemia presents with hyperreflexia and irritability. Hyperkalemia would present with cardiac ­irritability. 151. Correct answer: ­C Although answers A and B are probably true, they do not explain the reason that the temperature probe used on servo controlled beds and warmers only reads the skin temperature and not the core temperature. When using temperature probes, it is important to continue to monitor core temperatures to determine the effectiveness of the bed or warmer in maintaining a neutral thermal environment. Infants who are lying on temperature probes, bundled tightly, or dressed in clothing may artificially read too warm, and the isolette or radiant warmer may decrease the temperature in response, leading the air surrounding the infant to cool. Any portion of the body still exposed to the air, such as the head and extremities, may cause a hypothermic cascade to be initiated. To counter this issue, move the temperature probe location with position changes to prevent the infant from lying on the ­probe. 152. Correct answer: ­B Papules about 1 mm in diameter around the chin and cheeks may concern parents, but are ­self-­resolving milia found in approximately 40% of term births. The milia are epidermal inclusion cysts of keratin. It is important to teach the parents not to pop or break these cysts, as doing so increases the risk of ­infection. 153. Correct answer: ­B Jill is presenting as polydrug user without providing any medical history. Polydrug users are at very high risk for sexually transmitted diseases such as syphilis, hepatitis B and C, gonorrhea, and Chlamydia. It is recommended that rapid screening be done as soon as possible and prophylactic treatments begin prior to delivery. Fetal monitoring is necessary as risk for spontaneous abortion is elevated in this ­high-­r isk group. Should the infant deliver, complications might include intrauterine growth restriction, low birth weight, hypoxia, withdrawal symptoms, congenital infections, and m ­ icroencephaly. 154. Correct answer: ­A Any intubated patient who suddenly presents with a rapid increase in PaCO2 and decrease in PaO2 should be immediately evaluated for a tension pneumothorax related

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to ventilator pressures. Delayed treatment could result in hypoxia, hypoxemia, and death. Rapid assessment and treatment with needle decompression is necessary to prevent tissue ­injury. 155. Correct answer: ­B Risk management plays a role in the collection, analysis, and storage of records regarding potential and actual injuries in the hospital; court filings; identifies situations or actions of potential liability; and analyzes techniques to prevent future injuries, medication errors, and ­accidents. 156. Correct answer: ­A Children under 2 years of age cannot grasp the concept of another baby. It is best to explain to the parents that the 2 ­year-­old daughter will not understand that the baby is in the unit, but may have separation anxiety with you visiting for great lengths of time. For children who are 2–6 years old, it is best to keep information simple, to explain that it is nothing they did and that the staff is helping the baby to get better without going into detail. Older children should be involved in care based on age and ability. Teach proper ­hand-­washing technique and encourage interaction using ­age-­appropriate terms and ­actions. 157. Correct answer: ­C The best choice for kangaroo care for a hyperthermic neonate is the mother if she is available. Maternal breast tissue can thermoregulate by both increasing and decreasing skin temperature. Paternal breast tissue can only increase temperature and would not be preferred when providing kangaroo care for a hyperthermic neonate. Current research supports kangaroo holding whenever available to increase and sustain stability faster and more efficiently than isolette or radiant warmer ­use. 158. Correct answer: ­C Due to neonatal instability and/or lack of developmental positioning, common neonatal positioning deformities include neck extension and head molding to maintain and maximize the airway patency. Without positioning devices, gravity exerts resistance greater than muscle strength, causing hips to adduct with external rotation and shoulder extension with scapular adduction. This presents as the classic “frog” appearance. Boundaries should allow some resistance to active extension of limbs, yet provides passive flexion when at ­rest. 159. Correct answer: ­B A baby with Potter’s syndrome is usually small for gestation, has bowed legs and clubbed feet, severe respiratory distress, a bell shaped chest, large, claw shaped hands and dry skin. Assessment criteria that would support a diagnosis of Potter’s syndrome include a blunted nose with receding chin, low-set ears and widely spaced eyes. Other criteria include renal agenesis on renal ultrasound, anuria, and pulmonary hypoplasty. Genital abnormalities and/or gastrointestinal malformations may also be ­noted. 160. Correct answer: ­C Procainamide is a class 1A antiarrhythmic that increases the effective refractory period of the atria and ventricles. It can also be used for acute treatment of ventricular

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tachycardia, which does not respond to adenosine or cardioversion. It is important to remember that amiodarone and cimetidine interact and potentiate the effects of ­procainamide. 161. Correct answer: ­A Persistent pulmonary hypertension of the newborn may lead to renal failure due to an increase in systemic pressures and renal hypoxemia due to v/q ­mismatch. 162. Correct answer: ­A With the early and traumatic delivery of a sick and premature neonate, these parents are experiencing the initial response to a crisis. The situation is sudden, unexpected, and involves an environment and circumstances beyond the parents’ experience or ability to cope. This response to the crisis may last up to 6 weeks depending upon available support systems, the healthcare team, parent education, culture, and spiritual intervention. Parents will be more receptive to interventions as they begin to seek methods and new techniques to manage ­stress. 163. Correct answer: ­B HCO32 is a buffer that binds with free hydrogen to form carbonic acid. Carbonic acid then breaks down into water and CO2 for removal via the lungs. Additional buffers include plasma proteins and hemoglobin. Acids are hydrogen donors, and bases are hydrogen receptors. When evaluating the primary cause of ­acid-­base imbalances, respiratory imbalances are determined by evaluating pCO2 , and metabolic imbalances are determined by evaluating HCO32. 164. Correct answer: ­C Marfan syndrome is characterized by elongated extremities and fingers, kyphosis, a decreased ­upper-­to-­lower segment ratio, and retinal detachment. In patients with Marfan syndrome, there is usually aortic root dilitation that may have an associated aortic dissection, mitral valve prolapse, and/or aortic ­regurgitation. 165. Correct answer: ­A A flexed posture indicates a healthy, term infant with sufficient oxygen delivery to all organs, including the muscles. A floppy or hypotonic posture may indicate central nervous system depression and/or oxygen ­deprivation. 166. Correct answer: ­B Elevated maternal estrogen levels may result in breast discharge in the infant, both female and male. This condition is ­self-­limiting as estrogen levels fall over ­time. 167. Correct answer: ­C From the placenta, the oxygenated blood flows through the umbilical vein to the ­fetus. 168. Correct answer: ­B The diagnosis of necrotizing enterocolitis is supported by thickened bowel walls and pneumatosis intestinalis on abdominal radiographs. Clinical presentation includes abdominal distention, abdominal tenderness, and bloody ­stools.

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169. Correct answer: ­A Clinical presentation for this patient is consistent with renal vein thrombosis. This includes right flank mass, hematuria, hypertension, anemia, and thrombocytopenia. Management includes review of the history and physical for maternal diabetes, fetal asphyxia, maternal thiazide therapy, polycythemia, sepsis, RDS, or congenital heart disease. Treatment is focused on correcting the underlying illness, fluid therapy, possible dialysis, and close continuous ­monitoring. 170. Correct answer: ­B A ­f ull-­term infant born breech has extensive molding of the head. Infratentorial subdural hematoma can occur in this patient due to the shearing forces associated with a breech presentation. The symptoms may not be seen for as long as 12 hours. A change in the rate and depth of respirations occurs first. Later, the baby’s cry becomes high pitched or hoarse, followed by decreased feeding and sucking. The fontanelles become firm and bulging due to the increased intracranial pressure (ICP). 171. Correct answer: ­C Gestational age assessment is primarily utilized to anticipate problems related to gestation and development. Multiple evaluation systems exist, but each is based on both physical presentation and neurologic ­development. 172. Correct answer: ­C Home oxygen therapy can be very drying to the mucosal membranes; parents should be taught to use ­lemon-­glycerin swabs to reduce oral dryness. Oxygen tanks should be stored vertically in special containers and prevented from ­falling. 173. Correct answer: ­A An action of nitric oxide is vascular smooth muscle relaxation. Nitric oxide is the molecule released from the endothelium that enables smooth muscle relaxation. NO inhibits platelet aggregation and adherence and is thought to alter vascular permeability. NO may also participate in nonspecific immunity because it is generated when macrophages are a ­ ctivated. 174. Correct answer: ­B Infants with a pulmonary flow murmur are low birth weight and have hypoplastic pulmonary arteries at birth. After birth, blood flow and turbulence increases, causing the murmur. This murmur usually disappears about 3–6 months after ­birth. 175. Correct answer: ­C Adequate oxygenation is the best preventive measure and treatment for persistent pulmonary hypertension of the newborn (PPHN).

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RNC Practice Examination Answer Sheet Use this sheet to test yourself with the practice examination.

1. A  B  C

26. A  B  C

51. A  B  C

76. A  B  C

2. A  B  C

27. A  B  C

52. A  B  C

77. A  B  C

3. A  B  C

28. A  B  C

53. A  B  C

78. A  B  C

4. A  B  C

29. A  B  C

54. A  B  C

79. A  B  C

5. A  B  C

30. A  B  C

55. A  B  C

80. A  B  C

6. A  B  C

31. A  B  C

56. A  B  C

81. A  B  C

7. A  B  C

32. A  B  C

57. A  B  C

82. A  B  C

8. A  B  C

33. A  B  C

58. A  B  C

83. A  B  C

9. A  B  C

34. A  B  C

59. A  B  C

84. A  B  C

10. A  B  C

35. A  B  C

60. A  B  C

85. A  B  C

11. A  B  C

36. A  B  C

61. A  B  C

86. A  B  C

12. A  B  C

37. A  B  C

62. A  B  C

87. A  B  C

13. A  B  C

38. A  B  C

63. A  B  C

88. A  B  C

14. A  B  C

39. A  B  C

64. A  B  C

89. A  B  C

15. A  B  C

40. A  B  C

65. A  B  C

90. A  B  C

16. A  B  C

41. A  B  C

66. A  B  C

91. A  B  C

17. A  B  C

42. A  B  C

67. A  B  C

92. A  B  C

18. A  B  C

43. A  B  C

68. A  B  C

93. A  B  C

19. A  B  C

44. A  B  C

69. A  B  C

94. A  B  C

20. A  B  C

45. A  B  C

70. A  B  C

95. A  B  C

21. A  B  C

46. A  B  C

71. A  B  C

96. A  B  C

22. A  B  C

47. A  B  C

72. A  B  C

97. A  B  C

23. A  B  C

48. A  B  C

73. A  B  C

98. A  B  C

24. A  B  C

49. A  B  C

74. A  B  C

99. A  B  C

25. A  B  C

50. A  B  C

75. A  B  C

100. A  B  C

Turn the page to answer questions 101–175.

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101. A  B  C

126. A  B  C

151. A  B  C

102. A  B  C

127. A  B  C

152. A  B  C

103. A  B  C

128. A  B  C

153. A  B  C

104. A  B  C

129. A  B  C

154. A  B  C

105. A  B  C

130. A  B  C

155. A  B  C

106. A  B  C

131. A  B  C

156. A  B  C

107. A  B  C

132. A  B  C

157. A  B  C

108. A  B  C

133. A  B  C

158. A  B  C

109. A  B  C

134. A  B  C

159. A  B  C

110. A  B  C

135. A  B  C

160. A  B  C

111. A  B  C

136. A  B  C

161. A  B  C

112. A  B  C

137. A  B  C

162. A  B  C

113. A  B  C

138. A  B  C

163. A  B  C

114. A  B  C

139. A  B  C

164. A  B  C

115. A  B  C

140. A  B  C

165. A  B  C

116. A  B  C

141. A  B  C

166. A  B  C

117. A  B  C

142. A  B  C

167. A  B  C

118. A  B  C

143. A  B  C

168. A  B  C

119. A  B  C

144. A  B  C

169. A  B  C

120. A  B  C

145. A  B  C

170. A  B  C

121. A  B  C

146. A  B  C

171. A  B  C

122. A  B  C

147. A  B  C

172. A  B  C

123. A  B  C

148. A  B  C

173. A  B  C

124. A  B  C

149. A  B  C

174. A  B  C

125. A  B  C

150. A  B  C

175. A  B  C

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Section 13 Neonatal CCRN Practice ­Examination

The CCRN practice examination consists of 150 questions with four possible responses. Please allow 2.5 hours for testing. The questions are the same on the online exam. However, the online exam may allow questions to be in random order. Please enter your answers on the forms at the end of this ­section.

Questions 1. Your 32-week gestation patient was diagnosed with mild unilateral hydronephrosis. Urine output is 1.5 ml/kg/h. The parents ask you if surgery will be necessary. You should ­answer A. “Surgery is always indicated and will occur at 3 months of life.” B. “Immediate surgery is indicated despite normal levels of amniotic fluid at birth.” C. “The need for surgery is dependent upon the cause and severity of the hydronephrosis.” D. “The physician must answer all questions.” 2. Brian is a 26-week gestation neonate at day 3 of life. He is currently on conventional ventilation at 60% FiO2 . He has a right chest tube post pneumothorax. He is receiving a fentanyl drip in addition to TPN and intralipids. He has generalized 12 edema, crackles in all lung fields, and a night weight gain of 100 g. Labs show blood Na 120 mEq/L, K 3.1, Cl 91, and urine analysis shows elevated Na levels and osmolality. Urine output is 0.75 ml/kg/day for the last 24 hours. Upon reporting your findings to the physician, you would anticipate an order t­ o A. Decrease sterile water feedings, administer Lasix, and supplement ­NaCl B. Increase sterile water feedings, administer Diazide administration, and supplement K1 C. Continue same sterile water feedings and administer ­Lasix D. Increase sterile water feedings, weigh twice daily and decrease sodium ­intake 3. A nursing student is feeding a 36-week gestational aged female born 2 hours ago. During the feeding, the infant begins coughing and choking. The student carries the infant to you and you note the baby has large amounts of oral secretions. The infant’s color is poor and she is in respiratory distress. What is the most likely cause of these ­symptoms? A. Overaggressive ­feeding B. Status a ­ sthmaticus C. Cystic ­fibrosis D. Esophageal ­atresia

y

y

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4. What does the acronym TORCH ­represent? A. Tularemia, other infection, retrovirus, Chlamydia, ­herpes B. Tuberculosis, other infection, rhinovirus, chicken pox, herpes ­zoster C. Toxoplasmosis, other infection, rubella, CMV, herpes ­simplex D. Toxoplasmosis, otalgia, rubella, chills, heat ­rash 5. Your patient is a n ­ ear-­term infant who exhibited worsening perioral and periorbital cyanosis after birth. The infant developed a profound generalized cyanosis after the ductus closed and has been progressively more tachypneic. A pansystolic murmur is auscultated at the left lower sternal border and is accompanied by a thrill and a diastolic rumble. These symptoms are suggestive ­of A. Aortic ­stenosis B. Tetralogy of ­Fallot C. Pulmonary atresia with an intact ventricular ­septum D. Mitral ­stenosis 6. Which of the following conditions may lead to inaccurate oxygen saturation values on a pulse ­oximeter? A. Cyanotic heart ­disease B. ­Hyperthermia C. Decreased peripheral ­perfusion D. ­Prematurity 7. You are mentoring a nursing student and discussing the importance of obtaining a maternal history prior to delivery. Maternal health during the pregnancy is extremely important in the development of the fetus and directly affects the postdelivery management of the neonate. Which are the most important maternal factors you should discuss with the nursing ­student? A. Maternal health and financial ­resources B. Placental location and overall maternal ­health C. Maternal socioeconomic and physiologic ­status D. Placental function and inherent maternal ­resources 8. Your patient has a platelet count of 95,000 mm3. Which of the following conditions can cause a neonate’s body to destroy platelets more quickly than they are ­produced? A. Lupus e­ rythematosus B.  Chlamydia ­infection C. Hepatitis ­B D. Viral ­infections 9. An increase in serum calcium, glucagon, or magnesium will stimulate the release o ­f A. Parathyroid ­hormone B. Endogenous ­epinephrine C. ­Sodium D. ­Calcitonin

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10. Normal values for pulmonary artery pressures in a term infant would b ­e A. RAP 7–10 mmHg LAP 5–7 mmHg PAOP 4 m ­ mHg B. RAP 3 mmHg LAP 8 mmHg PAOP 6–10 ­mmHg C. RAP 10–20 mmHg LAP 12–14 mmHg PAOP 10–14 m ­ mHg D. RAP 4–8 mmHg LAP 10–14 mmHg PAOP 18–22 m ­ mHg 11. Your patient has been diagnosed with bronchopulmonary dysplasia (BPD) and is being treated with furosemide. A side effect of furosemide ­is A. ­Nephrocalcinosis B. ­Hyperkalemia C. ­Hypercalcemia D. ­Hyperchloremia 12. You are assisting with a delivery of a 36-week gestation infant. The mother is a gravida 5, para 2. The last two pregnancies resulted in spontaneous abortions. The mother’s blood type is O negative. At birth, the infant has multiple petechiae all over the body and pronounced ascites. An ultrasound reveals pleural effusions. This infant is probably suffering ­from A. ­Kernicterus B. Congenital ­chylothorax C. ­A rnold-­Chiari ­syndrome D. Hydrops ­fetalis 13. The term infant in your care has been diagnosed with thalassemia and is about to be discharged home. An important aspect of parent teaching for a neonate with thalassemia would ­include A. Using cool water for skin ­cleansing B. Avoiding oral stimulants like ­pacifiers C. Feeding fewer, larger, ­high-­protein ­meals D. Applying occlusive dressings over infected ­areas 14. Your male patient’s mother carries the sickle cell trait. Which statement about sickle cell anemia is ­true? A. Sickle cell anemia always results in death by age ­40 B. Sickle cell anemia causes a loss of protein intrinsic ­factor C. Only males exhibit the ­disease D. High blood viscosity and low oxygen tension may cause a ­crisis 15. You are in attendance at the ­C-­section delivery of a 34-week gestational age infant. The infant emerges in severe respiratory distress with a probable diaphragmatic hernia. What medical orders and therapies do you ­anticipate? A. Intubation, NG tube, IV’s, chest ­X-­ray, and ­surgery B. ­None—­this will resolve ­spontaneously C. Intubation, chest tubes, and placement of a ­Sengstaken-­Blakemore ­t ube D. An umbilical line and chest tube ­placement

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16. Baby C. was born yesterday at 38 weeks gestation. She is edematous, hyponatremic, has a serum osmolality of 272 mOsm/L, and is maintaining a normal blood pressure for her age. As a NICU nurse, you suspect this infant is suffering f­ rom A. A Wilms’ ­t umor B. Diabetes ­insipidus C. Adrenal ­hyperplasia D. ­SIADH 17. Your patient has undergone a diaphragmatic hernia repair. While you are preparing the infant for discharge, it is necessary to teach the parents about the possibility of long-­term A. Necrotizing ­enterocolitis B. Gastroesophageal ­reflux C. Spontaneous ­pneumothoraces D. Respiratory ­distress 18. You are asked to visit the parents of a premature neonate who will deliver imminently. The labor and delivery nurse tells you that the parents have multiple questions regarding prognosis and expected plan of care. You ­should A. Go speak with the parents ­immediately B. Ask the charge nurse in labor and delivery to speak with the ­parents C. Wait until after the delivery to speak with the ­parents D. Ask the neonatologist to speak with the ­parents 19. Your resuscitation team is called to an emergency cesarean section of a 38-week gestation infant with absent fetal movement and heart rate for 5 minutes. An apneic, flaccid, and cyanotic infant is handed to you. After drying, suctioning and positioning, the infant remains apneic without heartbeat and is gray and mottled. Over the next few minutes, the team intubates the infant, provides effective positive pressure ventilation, and begins ­well-­coordinated chest compressions. An umbilical venous line is placed for epinephrine and volume replacement. Throughout the resuscitation, the team has never been able to establish a heartbeat in the infant. At what point may the team stop ­resuscitation? A. After 5 minutes of ­resuscitation B. After 10 minutes of ­resuscitation C. After 15 minutes of ­resuscitation D. When the parents have been presented all ­alternatives 20. You are caring for a 29-week gestational age female with subtle seizures. What medication do you anticipate using for this ­patient? A. ­Carbamazepine B. ­Diazepam C. ­Lorazepam D. ­Propofol

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21. An infant can be predisposed to bronchopulmonary dysplasia ­by A. ­Hypercalcemia B. Mechanical ­ventilation C. Transient tachypnea of the ­newborn D. ­Bronchospasm 22. Correct positioning is crucial when treating a newborn with a myelomeningocele. Which of the following positions would be appropriate for an infant with a ­myelomeningocele? A. Prone ­position B. Supine position to reduce the ­c yst C. Prone positioning, cover with normal ­saline-­soaked gauze, and avoid fecal ­contamination D. Supine position with a small pad to reduce ­pressure 23. Which of the following statements about extracorporeal membrane oxygenation (ECMO) is ­true? A. ECMO is used for treatment of pulmonary ­hypoplasia B. ECMO replaces nitric oxide as the therapy of choice for ­PPHN C. ECMO should not be used in cases of intravascular ­hemorrhage D. ECMO is indicated for treatment of bronchopulmonary ­dysplasia 24. Contractility of heart muscle in neonates is positively influenced by which of the following ­conditions? A. ­Hypoxia B. ­Hypooglycemia C. Exogenous catecholamine ­release D. ­Acidosis 25. You are transporting a preterm infant in respiratory distress to a facility for a higher level of care. The parents question why their baby will not be eating immediately. Your best response should ­be A. “We do not want the baby to eat because he has to have more tests.” B. “The baby will receive IV nutrition so it’s no big deal.” C. “The baby has high residual glucose from you [indicating the mother], and stress releases more glucose so that feeding at this time would cause hyperglycemia.” D. “Respiratory distress leads to an increased risk for aspiration and hypoxia that may inhibit normal intestinal function.” 26. The parents of a ­full-­term, female infant are concerned because Hirschsprung’s disease runs in the family. An appropriate, therapeutic explanation would ­be A. “Hirschsprung’s disease never occurs in females.” B. “It is caused by prenatal alcohol consumption.” C. “Hirschsprung’s is not passed from generation to generation.” D. “Hirschsprung’s disease is a congenital bowel disease.”

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27. Your patient is a 37-week gestation infant with tricuspid atresia. The infant is receiving prostaglandin E1. Which of the following parameters would indicate the PGE was e ­ ffective? A. Prolonged capillary refill ­time B. An increased heart ­rate C. Increased ­SpO2 D. Increased ­PVR 28. Your patient was diagnosed with a congenital chylothorax and required a thoracentesis. A thoracotomy tube was placed for drainage. As a NICU nurse, you know this patient must be continuously observed ­for A. ­Infection B. An air ­leak C. Respiratory ­acidosis D. Lung ­hyperinflation 29. Your patient is a 36-week gestation infant with a patent ductus arteriosus. The infant is exhibiting tachypnea with hypercarbia, some tissue mottling with delayed capillary refill, and oliguria. All these symptoms are the result ­of A. A ­r ight-­to-­left ­shunt B. Pulmonary ­congestion C. A side effect of prostaglandin ­E1 D. Decreased ­PVR 30. A patient with tricuspid atresia may ­exhibit A. ­Polycythemia B. A smaller than normal ­heart C. Sinus t­ achycardia D. Flattened, notched P ­waves 31. As a NICU nurse, you know that asphyxia is characterized by ­progressive A. Hypoxemia, hypercarbia, and ­alkalosis B. Hypoxia, hypocarbia, and ­alkalosis C. Hypoxemia, hypocarbia, and ­acidosis D. Hypoxia, hypercarbia, and ­acidosis 32. Your transport team arrives at a hospital emergency room that does not have maternal child services to transport a term infant who was surrendered to staff by a teenage mother. The infant’s first temperature was 35.4° C. The infant is wrapped in heated blankets under a radiant warmer. During your assessment, you find the blood glucose to be 17 mg/dl. The ER physician orders dextrose 25% at 2 ml/kg IV stat. You ­should A. Administer the dextrose as ­ordered B. Refuse the order and hang a D10W continuous drip at 150 ­mg/kg/day C. Immediately intubate the baby for ­transport D. Politely refuse the order, notify your transport neonatologist, and follow standard stabilization protocol of using dextrose 10% at 2 ml/kg ­IV

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33. A nursing action appropriate when administering amphotericin B intravenously i­ s A. Monitor daily serum ­levels B. To not mix with normal ­saline C. Decrease the infant’s sodium ­intake D. Buffer the mixture with sodium ­bicarbonate 34. You are caring for a 33-week gestational age infant who was born 8 hours ago. The infant has developed petechiae over his chest and arms. However, this infant is not thrombocytopenic. What is a possible cause of this ­condition? A. ­Hyperbilirubinemia B. CMV ­infection C. Hemolytic disease, early ­stage D. ­Jaundice 35. Your patient is a 38-week gestation infant whose delivery required excessive obstetric manipulations. The infant’s mother had chorioamnionitis, premature rupture of membranes, and a fever. This infant is at risk ­for A. Respiratory distress syndrome (RDS) B. Transient tachypnea of the newborn (TTN) C. Congenital ­pneumonia D. Meconium aspiration syndrome (MAS) 36. The mother of a preterm infant with acute respiratory distress syndrome is very anxious to hold her baby. It is important to explain to this mother the importance of which of the following actions to promote the recovery of her ­baby? A. To hold the baby upright so as to minimize ­secretions B. Maintaining strict I and O while in the ­unit C. To maintain a normothermic environment as much as ­possible D. Keeping the baby in isolation and ­untouched 37. The synthesis of hemoglobin is regulated ­by A. ­T hrombopoietin B. A myeloid stem ­cell C. ­Karyocytes D. ­Erythropoietin 38. The neonatal intensive care unit is seeing an increase in the number of teen mothers. Which of the following factors in the management of an infant of a teen mother should you consider when performing discharge ­teaching? A. Teaching the mother is the only ­priority B. Technical jargon is appropriate when explaining medical conditions and ­management C. The father must be identified and participate in the ­teaching D. Discharge teaching should include birth ­control

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39. Baby M. was born with green eyes and blond hair. These visible characteristics are known a ­s A. Mendelian ­traits B. ­Phenotypes C. ­Genotypes D. Autosomal ­traits 40. Cardiac glycosides are often used in the treatment of Ebstein anomaly. A major effect of this classification of medications ­is A. ­Inotropism B. Positive ­chronotropism C. Its usefulness as a ventricular ­antiarrhythmic D. ­Hyponatremia 41. Respiratory depression or apnea is a possible side effect ­of A. ­Methylxanthines B. Prostaglandin ­E1 C. ­Epinephrine D. ­Doxapram 42. Your patient has A ­ rnold-­Chiari syndrome. This defect may predispose the infant ­to A. Vocal cord ­paralysis B. Transient tachypnea of the newborn (TTN) C. A cleft ­palate D. ­Pneumonia 43. Fran is a 34-week gestation infant at 6 hours of life. She has been very unstable since birth and had meconium aspiration, which required intubation. Fran has severe respiratory distress syndrome requiring high pressures and 100% FiO2 by conventional ventilation to maintain saturations. There is an umbilical venous catheter (UVC) infusing D10W at 80 ml/kg/day. During your assessment you note pink, frothy secretions in the endotracheal tube, multiple premature atrial contractions, and tachycardia. You s­ hould A. Increase the PIP on the v ­ entilator B. Suction and provide morphine ­sulfate C. Remove the endotracheal tube and ­reintubate D. Check the UVC and endotracheal tube placement via chest X-­ray

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44. A rural hospital has called your transport team to transfer a term baby with moderate respiratory distress to your facility. You are able to place an umbilical line and your nurse practitioner confirms umbilical line placement on chest ­X-­ray prior to transporting your patient. The umbilical artery catheter is located at T4 and the umbilical venous catheter tip is just above the diaphragm. Based on the chest ­X-­ray results, which of the following choices would be an appropriate ­action? A. Nothing; the lines are properly placed and the infant is ready for ­transport B. Withdraw the arterial line to T11–T12 and the venous line to 1 cm below the diaphragm before securing the ­lines C. Confirm with the neonatologist and prepare to withdraw the arterial line back to T6–T9 and the secure the venous ­line D. Remove the venous line and leave the arterial line in ­place 45. A ­short-­term complication of ECMO therapy would ­be A. Renal ­insufficiency B. Intraventricular ­hemorrhage C. Respiratory distress ­syndrome D. Cerebral vascular ­accident 46. Which of the following would be a side effect of the administration of ­methylxanthines? A. Doxapram ­toxicity B. A cholinergic ­crisis C. Gastroesophageal ­reflux D. ­Bradycardia 47. The charge nurse informs you that your new patient has glossoptosis, micrognathia, and a cleft palate. These signs are characteristic ­of A. CHARGE a ­ ssociation B. Pierre Robin ­sequence C. Choanal ­atresia D. Potter’s ­syndrome 48. You are unable to pass a 6-Fr. catheter through the nares of your patient. In addition, the infant exhibits another characteristic suggestive of choanal atresia: when the infant cries, there is immediate improvement in cyanosis. Which of the following tests would you expect as part of a diagnostic ­workup? A. An e­ chocardiogram B. A head CT ­scan C. A V/Q ­scan D. X-­ray

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49. Your patient has respiratory distress syndrome and his blood gas report indicates respiratory alkalosis. Which of the following arterial blood gases matches this ­result? A. pH 7.23, PaCO2 46, HCO3 22, PaO2 ­80 B. pH 7.35, PaCO2 40, HCO3 24, PaO2 ­96 C. pH 7.49, PaCO2 32, HCO3 21, PaO2 ­78 D. pH 7.47, PaCO2 48, HCO3 37, PaO2 ­75 50. You are caring for a 1 day old, term infant who has been diagnosed with pulmonary atresia with an intact ventricular septum. An inotropic medication often used for this condition ­is A. ­Furosemide B. ­Digoxin C. ­Isuprel D. ­A miodarone 51. During feeding time, you have the opportunity to teach a new mother and father about taking their infant’s temperature. Which of the following statements about this process is ­true? A. Environmental factors do not impact the infant’s ability to ­self-­regulate ­temperature B. Brown fat does not need oxygen to b ­ urn C. All temperatures for the first month must be done ­rectally D. A normal temperature for an infant is between 36.5 and 37.5° C (97.7–99.5° F) 52. A term infant in your care has required some supplemental oxygen. She is placed on 2 L/min via NC and ABGs have been drawn. Blood gas results show pH 7.54, PaO2 98 mmHg, PaCO2 26 mmHg, and HCO3 24 mEq/L. These blood gas results ­show A. Uncompensated respiratory ­alkalosis B. Compensated metabolic ­alkalosis C. Compensated metabolic ­acidosis D. Uncompensated metabolic ­alkalosis 53. J.C. is a 3-day-old, 36-week gestational age male. This morning he has suddenly started having bilious vomitus without abdominal distention. What could be the cause of his ­distress? A. Duodenal ­atresia B. Meconium ­plug C. Esophageal ­atresia D. Malrotation of the midgut and ­volvulus 54. Your patient has been hypoxic since birth 3 days ago. What potential imbalance would be expected with this ­condition? A. ­Hypokalemia B. ­Hypochloremia C. Decreased bicarbonate ­levels D. A pH of . ­7.50

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55. Your patient was born at 34 weeks gestation via cesarean section because of persistent fetal tachycardia and rare decelerations. At day 4 of life, severe tachypnea developed. Blood cultures were obtained and all labs were normal. That evening, the infant became apneic and bradycardic. Antibiotics were started. The next morning, the infant developed a fever, DIC, jaundice, slight hepatomegaly, and hepatitis. Labs showed high numbers of bands and an elevated platelet count. A diagnosis of myocarditis was made. The most prevalent causative agent for this condition ­is A.  Escherichia c­ oli B.  ­Serratia C.  Staphylococcus ­epidermidis D.  Coxsackie B1 ­virus 56. If one individual carries two different cell lines, it is known ­as A. ­Polyploidy B. ­Mosaicism C. ­Polygenic D. A ­genotype 57. While orienting a new nurse, you are discussing methods to stabilize and maintain temperature of a newborn. Which of the following statements is ­true? A. A premature neonate may be placed in a ­single-­walled isolette if a ­double-­walled isolette is not ­available B. Premature infants should be placed in radiant heat warmers under a thin, heated ­blanket C. In the absence of radiant warmer or heat lamp, blankets warmed in the microwave may be ­used D. A radiant warmer and a heat lamp should not be utilized at the same ­time 58. A 37-week gestation female infant is admitted to your NICU. Your initial assessment reveals a webbed neck, swollen hands and feet, arms that turn out at the elbow, and l­ ow-­set ears and a receding lower jaw. Based on this initial assessment, which chromosomal defect do you suspect this infant may ­have? A. Down ­syndrome B. Turner ­syndrome C. Cystic ­fibrosis D. Potter’s ­syndrome 59. Your term gestation patient has been restless, tachycardic, and has a ­low-­grade fever. She was diagnosed as having acute pyelonephritis. Which of the following statements is true regarding fluid management for this ­patient? A. The patient should have a 20–30 g/d weight ­gain. B. Fluid management should be based on 30 ml/kg/d plus urinary fluid ­output. C. Urine output has no bearing on ­outcome D. Fluids should not be ­restricted

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60. Darsey is a f­ ull-­term Down syndrome baby who is 1 day old. She has just started vomiting without preamble. You notice her abdomen is becoming distended. Which of the following conditions may be the cause of this ­problem? A. High meconium ­plug B. Malrotation and ­volvulus C. Probable ­overfeeding D. Duodenal ­atresia 61. Which of the following conditions puts a 28-week gestational age infant at risk for periventricular ­leukomalacia? A. Apnea, patent ductus arteriosus, and ­infection B. Pneumothorax, apnea, and CMV ­infection C. Respiratory distress syndrome, pneumothorax, patent ductus arteriosus, and ­apnea D. Respiratory distress syndrome, apnea, and HIV ­infection 62. Hemolysis in the fetus caused by Rh incompatibility results in which of the following d ­ iseases? A. ABO i­ ncompatibility B. ­A nemia C. Erythroblastosis ­fetalis D. ­T halassemia 63. The mother of a 30-week gestation neonate is sitting at bedside when you let her know that you will be transferring the baby to an isolette. She states, “I don’t want him to go into that box thing. I’ll be afraid to touch him. Can’t he stay in this warmer thing?” What is the best response to her ­request? A. “Sure, we can leave him in the warmer for another week.” B. “No, he must be changed over to an isolette per our policy.” C. “The NICU is short on warmers and we have sicker babies here.” D. “We will assist you in touching and holding your baby as he gets better, and the isolette allows for better temperature control and reduces stress to the baby.” 64. A possible adverse effect with the use of fentanyl ­is A. ­Hyperthyroidism B. Chest wall ­r igidity C. ­Neutropenia D. ­Tachypnea 65. The infant you are caring for is suspected of having Hirschsprung’s disease. What diagnostic procedures do you anticipate the infant to undergo prior to treatment of this ­disease? A. Plain abdominal films and blood work including amylase and ­lipase B. Rectal biopsy, plain abdominal ­X-­rays, and barium ­enema C. Rectal biopsy and ­MRI D. Serum lipase and CT ­scan

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66. Hypoxemia is best defined ­as A. A decrease in oxygen at the cellular ­level B. A decrease in oxygen levels in arterial ­blood C. A decrease in oxygen levels in venous ­blood D. A decrease in oxygen levels from the ­brain 67. Baby T. has RDS and has been on mechanical ventilation for 4 days as a result of meconium aspiration. During your assessment, you note a temperature of 100.3° F, heart rate of 208, respiratory rate of 46, increased cough, and decreased breath sounds on the right side without tracheal deviation. You suspect her symptoms are the result ­of A. Pulmonary ­edema B. ­Atelectasis C. ­Pneumothorax D. Unilateral ­pneumonia 68. Your patient is a 36-week gestation infant who is thought to have a trisomy 18 defect. Which of the following characteristics would you expect this infant to ­present? A. Cleft lip and palate and grand mal ­seizures B. ­Narrow-­set eyes and patent foramen ­ovale C. Malformed ears and ­cataracts D. ­Low-­set ears and low birth ­weight 69. Characteristics of an infant with fetal alcohol syndrome would ­include A. Microcephaly and ventricular septal ­defect B. Hypocalcemia and ­hypokalemia C. Lethargy and increased ­appetite D. Large for gestational age and prominent ­cheekbones 70. If the oxyhemoglobin curve shifts to the right, one of the factors that will affect this shift ­is A. A decrease in ­CO2 B. A decrease in ­pH C. A decrease in ­temperature D. A decrease in 2,3 D ­ PG 71. Which of the following statements is true regarding excess fetal ­insulin? A. Excess fetal insulin may be the cause of delayed maturation of type I alveolar ­cells B. Excess fetal insulin promotes the development of pulmonary ­surfactant C. Excess fetal insulin causes idiosyncratic ­hyperglycemia D. Excess fetal insulin may be the cause of delayed maturation of type II alveolar ­cells

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72. Infants with immature lungs are at risk for respiratory distress syndrome. Which of the following characteristics would be present in most cases of respiratory distress s­ yndrome? A. Pulmonary ­hyperperfusion B. Surfactant present at ­birth C. ­Prematurity D. An increased functional residual ­capacity 73. You are assisting in a neonatology consultation for a 17-­year-­old in preterm labor at 25-week gestation who is addicted to energy drinks. What potential complications in the neonate must you ­anticipate? A. Complications similar to marijuana ­addiction B. Complications similar to Trisomy ­13 C. Complications similar to heroin ­addiction D. Complications similar to fetal alcohol ­syndrome 74. Which of the following signs would be present in an infant with respiratory distress s­ yndrome? A. ­Hematocrit B. Pulse ­oximetry C. ­Grunting D. ­Capnography 75. Which of the following drugs is a ­lusitropic? A. ­Milrinone B. ­Dobutamine C. ­T heophylline D. ­Digoxin 76. A distinguishing feature of transient tachypnea versus respiratory distress syndrome is ­that A. TTN requires mechanical ­ventilation B. TTN patients have virtually clear X-­rays C. Infants with TTN tend to have a normal or low ­pCO2 D. Infants with TTN have a significantly higher ­pCO2 77. Congenital diaphragmatic hernia (CDH) ­is A. A condition that manifests itself about 5 hours after ­birth B. A condition that may lead to hypoxia and ­hypercarbia C. A condition that results in a ­left-­to-­r ight ­shunt D. A condition that manifests itself in a “barrel” shaped abdomen 78. The occurrence of pulmonary interstitial emphysema (PIE) is closely associated w ­ ith A. ­A ntibiotics B. Maternal ­age C. ­Barotrauma D. Maternal alcohol ­consumption

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79. Your patient just arrived from the delivery room. On admission, you note hyperresonant but severely decreased breath sounds in the right middle lobe. You are unable to visualize the area with the transilluminator. A subsequent ultrasound showed an overexpansion of the right middle lobe. These findings are suggestive ­of A. Bronchial ­dysplasia B. A tension ­pneumothorax C. Congenital lobar ­emphysema D. A ­hemothorax 80. Calcium channel blockers act primarily ­on A. Reduction of cardiac ­output B. Arteries to ­arterioles C. Lung receptors ­only D. Venules to ­veins 81. Your patient is a male of 32 weeks gestation. His skin is very friable. The physician orders frequent arterial blood gases, but obtaining blood draws is very difficult. Your care team is reluctant to use transcutaneous oxygen and carbon dioxide monitoring. A good alternative form of monitoring in this case would ­be A. Use a pulse oximeter and end tidal carbon dioxide ­monitor B. To obtain capillary blood gases ­only C. There is no alternative form of ­monitoring D. To continue with ABGs but draw them only every 8 ­hours 82. The neonatal resuscitation team is called to the vaginal delivery of a 38-week gestation infant of a mother having seizures. She is reported as having headaches, hypertension, nausea, and vomiting prior to delivery. The preeclampsia workup shows normal platelet count, normal AST and ALT, and no proteinuria. What condition is the infant at risk for during ­delivery? A. ­Hyperglycemia B. ­Hypervolemia C. ­PPHN D. Cerebral vascular ­accident 83. Joy is a 17 ­year-­old who was brought into the emergency room after collapsing at a graduation party. She is 25-weeks pregnant and having mild contractions. Joy’s cardiac and neurologic workup is negative. Her urine drug screen is negative. If Joy delivers in the next few hours, what immediate infant complication could you ­anticipate? A. Respiratory ­depression B. ­Irritability C. Projectile ­vomiting D. ­Seizures

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84. A high serum magnesium or phosphate level, low serum calcium, with catecholamine release results in secretion ­of A. ­A ldosterone B. Thyroid ­hormone C. ­Glyceride D. Parathyroid ­hormone 85. Which of the following statements is true regarding ­high-­frequency oscillatory ­ventilation? A. Oxygenation is controlled by the ­PIP B. The rate can pulsate up to 200 times a ­minute C. Oscillatory amplitude controls CO2 ­elimination D. Only unilateral ventilation is ­possible 86. Congenital lobar emphysema predominately affects which lung ­field? A. Right upper ­lobe B. Right middle ­lobe C. Left upper ­lobe D. Left lower ­lobe 87. Cellular humoral immunity is mediated ­by A. T ­lymphocytes B. ­Eosinophils C. ­Macrocytes D. B ­lymphocytes 88. If a mother has a history of abusing solvents, what is the most likely effect on the ­fetus? A. Cardiac ­anomalies B. Limb ­deformities C. Large for gestational ­age D. Intrauterine growth ­restriction 89. You have just assisted with the delivery of a ­full-­term infant. The airway was cleared and the infant placed on a radiant warmer. The infant has no spontaneous respirations and the Apgar score is 3 at 1 minute. Your next nursing action should be ­to A. Administer ­naloxone B. Administer ­epinephrine C. Begin chest ­compressions D. Provide b ­ ag-­mask ­ventilation 90. The type of echocardiography that shows the quantity of flow across an obstruction is ­called A. ­Two-­dimensional ­echocardiography B. ­Continuous-­wave Doppler ­echocardiography C. ­M-­mode ­echocardiography D. Contrast ­echocardiography

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91. The most important nursing consideration in the neonatal patient with HHNS ­is A. The administration of ­insulin B. To monitor ­electrolytes C. Administer D12.5W for fluid management D. To prevent ­aspiration 92. The ­full-­term infant you are caring for endured a difficult, prolonged delivery requiring repeated vacuum extraction attempts. You notice an expanding, soft, pliable mass on the top of the infant’s head. What is the probable cause of this ­phenomenon? A. Epidural ­hemorrhage B. Subdural ­hematoma C. Subarachnoid ­hemorrhage D. Subgaleal ­hemorrhage 93. You are caring for a 36-week gestation infant born to a mother addicted to methamphetamines. Which complications are you most likely to ­see? A. Cleft palate and ­hypoglycemia B. Hyperglycemia and ­bradycardia C. Feeding intolerance and ­hypothermia D. Complete heart block and feeding ­intolerance 94. Billie and John both smoke 1 pack of cigarettes daily. You are completing the discharge teaching for their son, Aaron, born at 26-weeks gestation and who is now 36-weeks adjusted age. Aaron has had several episodes of respiratory difficulty and one episode of pneumonia. Which of the following statements must be included in their ­teaching? A. “CPR classes are optional.” B. “Hearing and vision screening should only be done at discharge and at 3 years old.” C. “Aaron is at higher risk for sudden infant death syndrome.” D. “Aaron only needs antibiotics for 5 days after discharge.” 95. Barbara is a 35-week gestation infant just born and diagnosed with fetal alcohol syndrome. Her mother reports drinking “heavily” while pregnant and does not want any interaction with the baby. Due to the baby’s presentation and history, you would anticipate withdrawal symptoms to ­occur A. Within the first 12 hours of ­life B. Between 24 and 36 hours of ­life C. Between 36 and 48 hours of ­life D. Between 48 and 60 hours of ­life 96. The type of echocardiography used to detect a PDA or ventricular dysfunction is ­echocardiography known as A. M-­mode B. ­Contrast C. ­Color-­flow ­Doppler D. Two-­dimensional

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97. Which of the following infants is at the greatest risk of developing necrotizing ­enterocolitis? A. A 38-week gestational age breastfed ­baby B. A 34-week gestational age baby with ­GER C. A 26-week gestational age baby who is currently ­stable D. A 30-week gestational age whose mother had salmonella food poisoning just prior to giving ­birth 98. Over the last 2 weeks, you have been teaching Sid and Marne about behavioral cues and interventions when caring for their 26-week gestation neonate. You would document effective learning and application ­if A. They call you to the bedside when the baby ­cries B. They provide hand containment when they see arms and legs ­failing C. They adjust the neonate with extended extremities while providing kangaroo ­care D. They no longer ask about treatments the baby is ­receiving 99. You are caring for a 33-week gestation infant in acute respiratory failure. The patient is receiving O2 therapy and has ABGs scheduled every 4 hours. As a NICU nurse, you know that the arterial oxygen tension should be maintained ­between A. 40 and 60 m ­ mHg B. 50 and 80 m ­ mHg C. 70 and 90 m ­ mHg D. 80 and 100 m ­ mHg 100. If positive inspiratory pressure (PIP) is increased on a mechanical ventilator, what effect should this have on your patient’s blood ­gases? A. Raise the PaCO2 and decrease the ­PaO2 B. Lower the PaCO2 and lower the ­PaO2 C. Raise the PaCO2 and raise the ­PaO2 D. Decrease the PaCO2 and increase the P ­ aO2 101. An important consideration when teaching a parent about the care of an infant with thrush ­is A. Use a tongue depressor to scrape off the ­plaques B. Swab skin lesions with gentian ­violet C. Swab oral lesions with gentamycin ­ointment D. Use a ­cotton-­tipped applicator to swab the ­plaques 102. When utilizing transcutaneous PO2 measurements, some conditions may make the results unreliable. Underestimation of oxygenation ­may A. Occur when there is an air bubble between the electrode and the ­skin B. Occur when the alignment is ­improper C. Occur when skin is ­hypoperfused D. Be profoundly underestimated due to an air ­leak

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103. Micrognathia, a cleft lip and palate, along with a large protruding tongue are classic characteristics ­of A. Turner ­syndrome B. Trisomy ­13 C. Pierre Robin ­syndrome D. Williams ­syndrome 104. A ­full-­term infant born breech has extensive molding of the head. The infant has a ­high-­pitched cry and is feeding poorly. What type of neurovascular hemorrhage would you expect in this ­infant? A. Periventricular hemorrhagic ­infarction B. Infratentorial subdural ­hematoma C. Subarachnoid ­hemorrhage D. Epidural ­hemorrhage 105. A contraindication for the use of indomethacin to close a PDA in a premature infant would ­be A. Necrotizing ­enterocolitis B. A BUN , 30 mg/dl C. A urine output , 0.8 ml/kg/hour D. A serum creatinine clearance of . 1.8 mg/dl 106. Which of the following statements is true about ­high-­frequency jet ­ventilators? A. ­High-­f requency jet ventilators deliver ­high-­flow, s­ hort-­duration ­pulses B. No conventional breaths can be delivered during ­HFJV C. Exhalation is active and ­forced D. No PEEP can be ­provided 107. You are participating in the delivery and resuscitation of a 28-week gestation neonate. The heart rate is 140 with a respiratory rate of 80 with moderate retractions and circumoral cyanosis. Your next step is ­to A. Provide positive pressure ventilation with 100% ­FiO2 B. Provide CPAP using a s­ elf-­inflating ­bag-­mask ­valve C. Provide CPAP using a T ­ -­piece ­resuscitator D. Provide ­flow-­through ventilation with an anesthesia bag at 100% 108. Intubation of a term infant was just completed. The infant has a heart rate of 20 bpm, no spontaneous respiratory effort and is flaccid and mottled. The chest rises equally and breath sounds are auscultated in all lung fields. The CO2 detector does not indicate the presence of exhaled CO2 . What is the next step in this ­resuscitation? A. Continue to provide positive pressure ventilation and observe for a rise in heart ­rate B. Remove the endotracheal tube and reintubate after 30 seconds of positive pressure ­ventilation C. Remove the endotracheal tube and insert a laryngeal mask ­airway D. Extubate the infant and perform a ­tracheostomy

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109. Your patient has required h ­ igh-­frequency oscillating ventilation. The patient’s PaO2 remains low. Which of the following nursing actions would be appropriate at this t­ ime? A. Increase the ­M AP B. Assess for an air leak or ­pneumothorax C. Use a bag mask and ventilate the ­patient D. Replace the ventilator ­circuit 110. An advantage of h ­ igh-­frequency ventilators over conventional ventilators i­ s A. HF ventilators can transport CO2 out of the lungs, utilizing smaller ­pressures B. Conventional ventilators will cause ­barotrauma C. HF ventilators will not cause barotrauma because of lower ­pressures D. Mean airway pressures do not have to be m ­ onitored 111. A fellow nurse in your NICU is 16 weeks pregnant and tells you her fetus was diagnosed with a cystic hygroma. A cystic hygroma ­is A. A large, ­wart-­like ­growth B. A ­t umor C. A virus filled ­nodule D. A fluid filled ­sac 112. Rejection of a transplanted organ usually occurs as a result o ­f A. Cellular ­immunity B. Humoral ­immunity C. Delayed hypersensitivity ­reaction D. Mediated ­immunity 113. Developmental care is based on which of the following standards of critical ­care? A. All infants receive the same care with slight ­differences B. Parents play a critical role in infant care once the infant is ­stable C. Parent and infant interaction is continuously assessed and interactions ­supported D. All interactions in the critical care setting are dependent on the parents’ coping ­skills 114. A genetic disorder with cystic hygroma as a presenting disease ­is A. Fetal alcohol ­syndrome B. A ­parvovirus C. Turner ­syndrome D. Potter’s ­syndrome 115. Which of the following conditions could cause a 31-week gestational age infant you are feeding to suddenly gag and become ­apneic? A. Tracheoesophageal ­fistula B. Duodenal ­atresia C. Gastroesophageal ­reflux D. An improperly sized ­nipple

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116. To increase parental involvement in their neonate’s care, teaching ­should A. Provide detailed pathophysiological changes occurring at any one ­time B. Provide short bursts of developmental cues and parent ­intervention C. Provide lengthy explanations of cues and nursing ­actions D. Provide a set time for teaching every ­day 117. A ­moderate-­sized ventricular septal defect results ­in A. A ­r ight-­to-­left s­ hunt B. A decrease in pulmonary vascular ­edema C. A ­left-­to-­r ight ­shunt D. An increased ­PVR 118. Tet spells may be treated with which of the following ­medications? A. ­Gentamycin B. ­Digoxin C. ­Morphine D. ­Phenobarbital 119. Your patient required placement of a left atrial pressure monitoring line. The pressure reads 14 mmHg. This value might ­indicate A. Tricuspid s­ tenosis B. Pulmonic ­stenosis C. Tricuspid r­ egurgitation D. Mitral valve ­dysfunction 120. Baby Laura is a 25-week gestational age female who had prenatal benzodiazepine exposure. She is lethargic with poor respiratory effort and poor muscle tone. You anticipate which of the following medications to be administered to this ­infant? A. ­Flumazenil B. ­Naloxone C. N-­acetylcysteine D. ­Neostigmine 121. Your preterm patient has become increasingly restless. You note that the infant’s chest has become more barrel shaped and overdistended. Breath sounds have become distant on the left side. These findings are indicative ­of A. A tension ­pneumothorax B. A ­pneumomediastinum C. Pulmonary interstitial ­emphysema D. A ­hemidiaphragm 122. You are caring for a term infant and note that the PR interval on the EKG is 0.14 second. This ­measurement A. Is normal for a term ­infant B. Indicates r­ ight-­sided heart ­failure C. Indicates a fi ­ rst-­degree heart b ­ lock D. Indicates the onset of ­SVT

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123. Georgia is a 4-day-old, 37-week gestation infant who appeared to be doing well at home until she began having seizures. On initial assessment, you note Georgia is lethargic and has poor muscle tone. She appears to be having muscle spasms. Georgia will not feed and has a “burned sugar” smell to her urine. You believe Georgia is suffering f­ rom A. Carnitine ­deficiency B. ­Tyrosinemia C. ­T hyrotoxicosis D. Maple syrup urine ­disease 124. Which of the following conditions are common side effects of ­pentobarbital? A. Respiratory depression, ­Stevens-­Johnson syndrome, and ­agitation B. Respiratory depression, bradycardia, and ­hypertension C. Respiratory depression, tachycardia, and ­hypertension D. Hypertension and ­agitation 125. You are part of the patient safety team for your neonatal intensive care unit (NICU) and are responsible for evaluating compliance with ­hospital-­acquired infection (HIA) procedures. To prevent ­ventilator-­associated pneumonia (VAP) in the NICU, the Centers for Disease Control and Prevention (CDC) recommends that respiratory equipment that comes in direct contact with patient mucosal membranes ­be A. ­Wet-­heat pasteurized at temperatures . 158° F for 30 minutes and packaged sterile for next ­use B. Washed with sterile normal saline at 90° F for 5 minutes prior to ­use C. Steam sterilized or autoclaved by the facility regardless if the item is multiuse or single ­use D. Washed in a bleach solution 15 minutes prior to ­use 126. The parents of your 23-week gestation patient are asking why the baby’s chest is vibrating while on h ­ igh-­frequency oscillator ventilation. You should tell the ­parents A. “The ventilator uses a ­microprocessor-­controlled pneumatic valve to create pulsation of airflow.” B. “The ventilator injects air into the ETT in short, rapid pulses that make the baby’s chest wobble.” C. “This ventilator causes the vibration when the pressures are too high.” D. “The ventilator uses a piston to move small amounts of air to and from the baby at a rapid rate, causing the chest to vibrate.” 127. Your patient is a 32-week gestational age male infant with hydrocephalus who received a ventriculoperitoneal shunt 2 days ago. On your initial assessment you find him febrile, tachycardic, and lethargic. You should anticipate orders for this patient to i­ nclude A. CBC with differential, CRP, sed rate, ceftazidime, and cooling ­measures B. CSF cultures, acetaminophen, and ­azithromycin C. CT scan, CSF cultures, and ­ciprofloxacin D. Levofloxacin, CT scan, and ­CRP

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128. Chronic hypoxia usually results in which of the following electrolyte ­imbalances? A. Decreased ­chloride B. Decreased ­potassium C. Decreased ­calcium D. Decreased ­phosphorus 129. Expiratory grunting is an infant’s attempt t­ o A. Maintain a normal functional residual ­capacity B. Decrease the ­PVR C. Remove a bronchial ­obstruction D. Clear ­secretions 130. Type II truncus arteriosus is characterized ­by A. A longer pulmonary artery arising from the base of a common ­trunk B. The right and left pulmonary arteries having separate ­origins C. The right and left pulmonary arteries are joined at the base of the common ­trunk D. The left pulmonary arising from the middle segment of the common ­trunk 131. Your patient is a 35-week gestation infant who has developed respiratory distress and sepsis. The infant is being mechanically ventilated and has had a varying pH (from 7.22 to 7.30) and a base deficit that has also been fluctuating from 25 to 26 over the last 10 hours. The infant has an indirect bilirubin of 22.9. This infant will be receiving a d ­ ouble-­volume exchange transfusion. Which of this infant’s problems must be addressed prior to the start of the ­transfusion? A. The bilirubin ­level B. Weaning from the ­ventilator C. Extubating the ­infant D. The a ­ cid-­base ­imbalance 132. You are to administer a single dose of chloral hydrate to a 30-week gestational age infant for sedation. What is the most common side effect of this drug in premature i­ nfants? A. CNS ­depression B. ­Bradycardia C. ­Seizures D. Complete heart ­block 133. Spinal reflexes ­indicate A. Functional upper motor ­neurons B. Nonfunctional lower motor ­neurons C. Functional lower motor ­neurons D. Nonfunctional upper motor ­neurons

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134. What assessments and positioning should be done for an infant with hydrocephalus after a ventriculoperitoneal shunt ­placement? A. Frequent neuro signs, HOB elevated at 30° degrees, head m ­ idline B. VS Q15 minutes, HOB flat, positioned on the left ­side C. Neuro signs hourly, HOB elevated at 30° degrees, positioned on the right ­side D. Head midline and prone ­positioning 135. Genetic disorders associated with advanced maternal age ­include A. Marfan syndrome, achondroplasia, and Apert’s ­syndrome B. Osteogenesis imperfecta, Treacher Collins syndrome, and Waardenburg’s ­syndrome C. Trisomy 21, Trisomy 13, and Klinefelter ­syndrome D. Apert’s syndrome, Marfan syndrome, and Waardenburg’s ­syndrome 136. Your team is resuscitating a 35-week gestation infant with severe respiratory distress. The infant has unilateral chest rise, deviated heart tones, and diminished breath sounds on the unaffected side, with a ­stretched-­to-­scaphoid abdomen. Which of the following actions should be taken ­immediately? A. Needle d ­ ecompression B. Vigorous oral ­suctioning C. Provide continuous positive airway ­pressure D. Endotracheal intubation and orogastric tube ­placement 137. Vitamin K deficiency is also known as hemorrhagic disease of the newborn. A factor that will negatively influence the production of vitamin K ­is A. ­Hyperparathyroidism B. Overproduction of coagulation factors II and ­X C. Excess circulating extracellular ­calcium D. A lack of sufficient intestinal ­flora 138. An example of an autosomal dominant disorder would ­be A. Sickle cell ­disease B. Fragile X ­syndrome C. Adult polycystic kidney ­disease D. Neural tube ­defects 139. Intubation of a term infant was just completed. The infant has a heart rate of 20 bpm, no spontaneous respiratory effort, and is flaccid and mottled. The chest rises equally, and breath sounds are auscultated in all lung fields. The CO2 detector does not indicate the presence of exhaled CO2 . What is the next step in this ­resuscitation? A. Continue to provide positive pressure ventilation and observe for a rise in heart ­rate B. Remove the endotracheal tube and reintubate after 30 seconds of positive pressure ­ventilation C. Remove the endotracheal tube and insert a laryngeal mask ­airway D. Replace the CO2 ­detector

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140. Tetralogy of Fallot manifests itself by which of the following combinations of ­defects? A. Right ventricular hypertrophy, tricuspid atresia, and ­A SD B. Aortic stenosis, atrial septal defect, coarctation of the aorta, and ­PDA C. ASD, mitral prolapse, PDA, and pulmonary ­stenosis D. VSD, overriding aorta, pulmonary stenosis, and right ventricular ­hypertrophy 141. Which nursing intervention is critical to maintaining patency and proper functioning of h ­ igh-­frequency jet ­ventilation? A. Monitor the chest wall excursion and immediately report any chest ­wobble B. Kink the tubing when turning the infant to prevent a drop in ­pressures C. Monitor respiratory rate every ­hour D. Irrigate the jet port with 0.5 ml of normal saline every 3–4 ­hours 142. Retractions may best be described ­as A. The chest wall caving inward with a large increase in pleural ­pressure B. The chest wall caving inward with a small decrease in pleural ­pressure C. The chest wall movement is corresponding to increases in pleural ­pressure D. The chest wall caving inward with a moderate decrease in pleural ­pressure 143. Which of the following actions should be done routinely when caring for an infant with a pulse ­oximeter? A. Eliminate all arterial blood gas draws to prevent unnecessary pain for the ­infant B. Recalibrate the pulse oximeter every 2 ­hours C. Check the disposable sensor q 24 ­hours D. Use disposable ­cuffs 144. A medication utilized to close a patent ductus arteriosus (PDA) ­is A. ­Prostaglandin B. ­Indomethacin C. ­Lysine D. ­Cisplatin 145. Today you are performing the duties of the charge nurse and are attending an emergency cesarean section. The infant is a 26-week neonate whose mother is a 17 year old gravida 1 with placenta previa. The infant was delivered pale, floppy, and apneic with a heart rate of 50 bpm. The neonatal nurse practitioner intubated the neonate immediately, and positive pressure ventilation was provided with effective chest rise. The heart rate increased to 80 bpm with minimal spontaneous effort. The patient is pale and hypotonic. What is the next step to be performed by your resuscitation ­team? A. Provide chest ­compressions B. Prepare to give epinephrine via the endotracheal ­t ube C. Administer lactated Ringer’s at 20 ml/kg via a continuous ­infusion D. Insert an umbilical venous line and administer normal ­saline

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146. Brian is a 24-week gestation neonate at day 25 of life. He is intubated on conventional ventilation with a chest tube post pneumothorax. TPN, intralipids, and fentanyl are infusing via a r­ ight-­arm PICC line. You note mild general edema, strong and bounding peripheral pulses, and capillary refill is , 3 seconds. Brian’s heart rate is 180, respiratory rate is 72, and the O2 saturation is 92% on 30% FiO2 . Cuff blood pressure is 96/67. Your first action should be to A. Call the physician for a hydralazine ­order B. Verify that the cuff size you are using is half the arm ­diameter C. Check the IV site to verify ­patency D. Immediately discontinue the ­fentanyl 147. The most common ­single-­gene disorder (autosomal recessive) in Caucasian populations i­ s A. Adult polycystic kidney ­disease B. Cystic ­fibrosis C. ­Retinoblastoma D. Potter’s ­syndrome 148. During your first assessment, you note that your 27-week gestation patient on day 2 of life has a right flank mass with frank blood noted in the diaper. You do not see any open wounds and the anus appears normal. Your patient is NPO and receiving nutrition via TPN and intralipids via a UVC. Arterial blood pressure is monitored via a UAC and indicates hypertension. Phototherapy was started yesterday for a bilirubin level of 6.7 mg/dl. Morning labs show a Hgb of 12.9 gm/dl, Hct of 45%, and PLT count of 34,000. You notify the physician immediately because you ­suspect A. Renal vein ­thrombosis B. Renal artery ­thrombosis C. A Wilms’ ­t umor D. Liver ­injury 149. You are called to the delivery of a 40-week gestation infant from a mother with gestational diabetes. On arrival, the labor nurse reports that the infant is having frequent decelerations and that an amnio infusion ran over 4 hours for thick meconium. The baby is delivered vaginally with thick particulate meconium. You note that the baby has not cried and is limp in the obstetrician’s arms. Your first priority is ­to A. Rapidly dry and stimulate the baby to prevent cold ­stress B. Aggressively suction the airway with the bulb ­syringe C. Immediately begin chest ­compressions D. Avoid stimulating the baby and assist with intubation and tracheal ­suctioning 150. The K ­ leihauer-­Betke test ­identifies A. Common blood group ­antigens B. The s­ ickle-­cell anemia ­trait C. Bone marrow lymphocyte ­precursors D. Fetal hemoglobin in maternal ­blood This concludes the Neonatal CCRN Practice ­Examination.

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Neonatal CCRN Practice Examination A ­ nswers 1. Correct answer: ­C The need for corrective surgery is dependent upon the cause and severity of the hydronephrosis. This patient has unilateral hydronephrosis with moderately low urine output. Further testing and time will provide support for or against surgery. The need for surgery is dependent upon the level of kidney function, presence of an obstruction, whether hypertension is present, and/or if the hydronephrosis ­worsens. 2. Correct answer: ­A Brian is exhibiting symptoms of SIADH (syndrome of inappropriate ADH secretion). The positive pressure ventilation, pain, opiate administration, and pneumothorax are contributing factors. Presentation of an infant with SIADH includes rapid weight gain, edema, hemodilution, urine loss, and elevated urine osmolality. Treatment includes free water restriction, Lasix administration, and Na supplementation if serum Na levels are , 120 mEq/L. Answer B is the treatment for nephrogenic diabetes insipidus (NDI). 3. Correct answer: ­D This collection of symptoms is classic for esophageal atresia and often occurs with a tracheoesophageal fistula. This condition occurs in 1 in 25,000 infants. Esophageal atresia is a failure of the esophagus to complete the connection between the throat and stomach, forming a blind pouch. There is often a fistula between the esophagus and the trachea called a tracheoesophageal fistula. Assessment of infants with this condition includes insertion of a nasogastric tube into the blind pouch, then taking a chest ­X-­ray. Depending on the severity of the atresia, it is possible to close the fistula. It is important to close the fistula as soon as possible to reduce the risk of aspiration pneumonia. A ­G -­t ube may be inserted for feedings and the nasogastric tube placed on low suction. Approximately half the infants born with esophageal atresia have other congenital problems such as VATER association, other GI abnormalities of the anus and rectum, renal malformations, cardiac abnormalities, and skeletal deformities. Nursing care includes elevating the head of the bassinet, providing IV fluids, oxygen, and maintaining the NG ­t ube. 4. Correct answer: ­C TORCH is a collection of similar clinical conditions seen with HIV infections. Toxoplasmosis is a protozoan infection passed from the mother to the infant. Other infection includes Hepatitis B, syphilis, ­varicella-­zoster virus, HIV, and parvovirus B19. The complex may also be written TORCHES and includes syphilis. Blood, urine, and CSF are tested, and the infant is considered positive if the IgM for one or more of the aforementioned infections are present in a symptomatic baby. Symptoms include chorioretinitis, microcephaly, hydrocephalus, small for gestational age, petechial rash, jaundice, hepatosplenomegaly, mental retardation, seizures, autism, and hearing ­loss. 5. Correct answer: ­C Pulmonary atresia with an intact ventricular septum is also known as PAIVS. The most common finding upon physical examination is central (perioral and periorbital) cyanosis. Following ductal closure, profound generalized cyanosis is present, and the

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apical left ventricular impulse may be pronounced. The first and second heart sounds are single. A pansystolic murmur is often heard at the left lower sternal border, consistent with tricuspid regurgitation. If severe, the murmur of tricuspid regurgitation may be associated with a thrill and a diastolic ­r umble. A systolic ejection murmur of the patent ductus arteriosus may be heard at the left second or third intercostal space, particularly after initiating prostaglandin ­infusion. 6. Correct answer: ­C Adequate perfusion must exist for the probe to register an accurate evaluation of hemoglobin saturation. If the infant is hypothermic or has decreased peripheral perfusion, the results will be inaccurate. Phototherapy, position of the probe, and patient motion can also cause inaccurate readings on a pulse ­oximeter. 7. Correct answer: ­D Placental function and inherent maternal resources are critical maternal factors to consider when planning care of the neonate prior to and after delivery. Placental function determines the rate and flow of nutrients and oxygen to the fetus as well as cellular waste products flowing away from the fetus. The placenta also plays a critical role in the physiologic changes within the mother that increase glucose and essential elements to the fetus during development. Maternal resources comprise the mother’s ability to obtain and maintain adequate and appropriate nutrition, cardiovascular and respiratory function in order to transport oxygen, carbohydrates, fats, vitamins, minerals, amino acids, and more to the fetus. If maternal resources are compromised, then fetal health becomes compromised. Additional complications to adequate maternal resources include preexisting medical and mental health conditions, drug ingestion, and ­exercise. 8. Correct answer: ­A Examples of maternal conditions that will cause a neonate’s body to use or destroy platelets more quickly than they are produced are lupus erythematosus, rheumatoid arthritis, and ­pregnancy. Hemolytic uremic syndrome can cause a decrease in platelets, destruction of red blood cells, and impairment of kidney function. Sometimes, this can occur in association with a bacterial Escherichia coli (E. coli) infection. Idiopathic thrombocytopenic purpura (ITP) is a condition in which the immune system mistakenly identifies platelets as a threat and forms antibodies that attack them. Thrombotic thrombocytopenic purpura (TTP) is a ­life-­threatening condition that occurs when small blood clots suddenly form throughout the body, using up large numbers of platelets. TTP sometimes happens as a result of a genetic deficiency, but more often the cause is unknown. In some cases, it may be associated with infection or a chronic illness. Blood poisoning is very rare in a neonate. However, severe bacterial infections may cause destruction of ­platelets. 9. Correct answer: ­D An increase in serum calcium, glucagon, or magnesium will stimulate the release of calcitonin. Calcitonin will reduce serum calcium levels. Calcitonin targets bone and kidney cells to inhibit bone cell lysis, and it decreases calcium reabsorption by the ­kidney.

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10. Correct answer: ­B B represents the normal range of values for pulmonary artery pressures— ­R AP 3 mmHg, LAP 8 mmHg, and PAOP 6–10 ­mmHg. 11. Correct answer: ­A Furosemide may cause nephrocalcinosis, renal calculi, hypotension, hypokalemia, hyponatremia, contraction alkalosis, and ­PEA. 12. Correct answer: ­D Hydrops fetalis is severe, total body edema and occasionally is accompanied by pleural effusions and ascites. Most research concludes that this condition is the result of congestive heart failure and intrauterine hypoxia from severe anemia. The patient will also have low serum albumin levels. Most infants with hydrops have portal and umbilical venous hypertension secondary to hepatic hematopoiesis. This infant will probably develop respiratory distress fairly quickly. Initial efforts should be directed toward airway management and ­ventilation. 13. Correct Answer: ­A In this type of anemia, the erythrocytes are very thin and fragile. They are known as target cells. The serum bilirubin is very elevated, so you should caution the parent about trying to prevent the infant from scratching himself. Cool water and lotion may be used for skin care. Oral stimulants could lead to vasoconstriction. The integrity of the skin is weakened, so careful positioning is paramount. The patient may require ongoing transfusions. Multiple transfusions may actually lead to too much iron that might have to be chelated ­out. 14. Correct answer: ­D Sickle cell anemia occurs primarily in the African American population. People with the condition are homozygous for HgS and have more HgS than HgA. This causes some of the cells to form a “sickle” shape—­curved with rough edges. A crisis can occur when the low oxygen tension (postulated) causes a proliferation of these cells. The sharp edges of these cells travel through the microcirculation and damage capillaries. A simple thing like cold weather can precipitate massive sickling. Other identified factors include dehydration, vomiting, diarrhea, high altitude, excessive exercise, and stress. When these cells break apart, they occlude the microcirculation and lower oxygen tension, which initiates more sickling. This is a very painful time for the patient, and oxygen, pain management, and fluids are very ­important. 15. Correct answer: ­A A diaphragmatic hernia is a medical emergency. If there is a defect in the development of the diaphragm, the abdominal contents can herniate into the thorax, preventing lung development. Because of this, it is often difficult to tell if the lungs are developed enough to sustain life. An infant with a diaphragmatic hernia is at great risk for pneumothorax. Pulmonary hypertension is a postoperative risk that may require special ventilation. ­One-­third of the infants with this condition die even with ­treatment.

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16. Correct answer: ­D Baby C. is probably suffering from the syndrome of inappropriate antidiuretic hormone (SIADH). This condition is a disorder of the posterior pituitary gland. Free water clearance is impaired by an increased secretion of vasopressin (ADH). ADH levels may become elevated in infants with fetal distress, respiratory diseases, surgery, central nervous system infections, injury (e.g., birth asphyxia), and pain. Prematurity, intracranial hemorrhage, and meningitis may also be ­factors. Infants with SIADH will exhibit weight gain, dilutional hyponatremia, edema, and a serum osmolality of , 275 mOsm/L. Urine osmolality will be high because the urine will be concentrated. Blood pressure can be normal as can adrenal, thyroid, and cardiac function. 17. Correct answer: ­B In addition to teaching the parents about gastroesophageal reflux, it is important to remember that the patient could also suffer other potential side effects such as growth restrictions, recurrent diaphragmatic hernias, and delayed neurologic ­development. 18. Correct answer: ­D The best staff member to speak with the parents is the neonatologist or nurse practitioner with or without the obstetrician present to answer questions regarding prognosis. The nurse can only speak to nursing plan of care and should not be discussing medical plan of care with the parents. It is important to establish effective communication and trust with the parents prior to delivery if time is available to improve overall understanding and patient ­outcomes. 19. Correct answer: ­B According to current NRP guidelines (5th edition) from the American Academy of Pediatrics, if, after 10 minutes of resuscitation, there is no heartbeat and each resuscitation step has been completed correctly and verified, then the team may stop resuscitation efforts. More time may be needed to ensure that each step of resuscitation was completed correctly and effectively. Additional time may also be need to fully treat identified causes of fetal arrest such as hemorrhagic or hypovolemic shock and asphyxia. Ethical dilemmas are frequent challenges during resuscitation as providers balance legal, ethical, and moral elements in delivering care. If the prognosis is unknown, continual communication with the parents by the physician is critical in determining best outcome for the infant, even if that choice is to withdraw support. After any resuscitation, it is vital for staff members to discuss the resuscitation to identify team strengths and opportunities for improvement and any ethical challenges. Discussion may identify more effective ways to communicate and improve competence between various disciplines, departments, and ancillary ­staff. 20. Correct answer: ­C Lorazepam is the drug of choice for neonatal subtle seizures. Subtle seizures can mimic normal movements of the infant. Some of the common movements seen with this condition include apnea, tonic deviation of the eyes, repetitive eyelid fluttering, drooling, chewing, sucking, swimming arm movements, pedaling of legs, and paroxysmal laughing. Dilantin and phenobarbital may also be used to treat subtle seizures in this

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infant. Carbamazepine is not indicated for subtle seizures. Diazepam is used for status epilepticus. Propofol is a hypnotic, not an ­anticonvulsant. 21. Correct answer: ­B Mechanical ventilation (possible barotrauma) and oxygen (toxicity) can predispose an infant to bronchopulmonary ­dysplasia. 22. Correct answer: ­C Myelomeningocele is the most severe form of spina bifida. The defects occur in the lumbar sacral area and develop in the first month of gestation. The clinical features of a myelomeningocele include a protrusion of meninges, spinal cord, and cerebrospinal fluid contained in a sac. Hydrocephalus and paralysis, including loss of bowel and bladder function, are common manifestations of myelomeningocele. It is imperative to keep pressure off this area, keep it moist, and avoid infection. Other nursing actions include daily head circumference measurements, careful I and O, and emotional support of the ­family. 23. Correct answer: ­C ECMO should not be used for intravascular hemorrhage because systemic heparinization is required. ECMO also increases the risk for intravascular hemorrhage in premature infants, especially those less than 35 weeks ­gestation. 24. Correct answer: ­C Contractility of heart muscle in neonates is positively influenced by exogenous catecholamine release. Exogenous catecholamines, such as dopamine and dobutamine, will increase cardiac output and blood pressure by increasing intracellular calcium, which increases myocardial contractility. Contractility is decreased by hypoxia, electrolyte disturbances, hypoglycemia, and ­acidosis. 25. Correct answer: ­D When caring for any infant in respiratory distress, it is important to hold oral feedings until gastric function has been determined. If the infant is tachypneic, there is an increased risk of aspiration. Aspiration may occur as a result of air trapped in the stomach from oxygen delivery devices, poorly coordinated suck, swallow, and breathing due to hypoxia, and diminished activity tolerance. Additional reasons to delay oral feedings include hypoxic effects and hypotension on the intestines that may inhibit or stop peristalsis. Although IV nutrition will be initiated, the answer given in option B does not acknowledge or address the parent’s question. Increased stress and physiologic distress increase glucose utilization by the infant, resulting in initial hypoglycemia, not ­hyperglycemia. 26. Correct answer: ­D Hirschsprung’s disease is a congenital abnormality seen with meconium plug. It is a neural tube defect, in which the ganglia that enervates the distal bowel and rectum do not migrate caudally (toward the feet). There is often a strong family history of Hirschsprung’s disease especially in males, who are affected 5:1 over females. Symptoms include vomiting (sometimes bilious), obstipation, and abdominal obstruction.

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A rectal biopsy shows a lack of ganglionic cells in the rectum. Surgical resection of the affected area may ­help. 27. Correct answer: ­C Oxygenation will improve because the PGE1 will maintain patency of the ductus arteriosus. In tricuspid atresia, pulmonary circulation is dependent on the ductus. As the ductus remains open, pulmonary blood flow and oxygenation ­improve. 28. Correct answer: ­A A chylothorax is a collection of lymphatic fluid in the pleural space. Lymphocytes are lost when the fluid is removed, which increases the risk of patient ­infection. 29. Correct answer: ­B A ­left-­to-­r ight shunt exists, causing increased pulmonary blood flow and pulmonary congestion. This results in tachypnea and hypercarbia. There is also decreased systemic blood flow, resulting in oliguria, mottling, and prolonged capillary ­refill. 30. Correct answer: ­A Because of hypoxia in patients with pulmonary atresia, polycythemia may be present. Prothrombin time and activated partial thromboplastin time may be abnormal secondary to the polycythemia. Cardiomegaly is usually present, with a prominent right heart border that reflects enlargement of the right atrium. Sinus rhythm is generally present, with tall P waves indicative of atrial enlargement. ­First-­degree atrioventricular block may be observed. Because of the origin of the left bundle branch from a common bundle, the frontal plane QRS axis may be leftward or ­superior. 31. Correct answer: ­D Asphyxia is increased inadequate tissue perfusion. Eventually, lactic acidosis occurs, so the progression is hypoxia, hypercarbia, and ­acidosis. 32. Correct answer: ­D It is the responsibility of the transport team to function and care for the neonate within the standards of care for stabilization. The ER physician may not be aware of or current to the standards of care in newborn stabilization. It would be appropriate to politely decline the order and contact the accepting neonatologist for orders while following standard stabilization practice. Dextrose concentrations of 25% and 50% provide a bolus of glucose that would cause profound hyperglycemia followed by rebound hypoglycemia, as well as severe damage to the ­vessels. 33. Correct answer: ­B A nursing action appropriate when administering amphotericin B intravenously is to not mix the drug with normal saline. Mixing in normal saline will cause the solution to precipitate. It is also necessary to protect this drug from light. Amphotericin B injectable is compatible with D5W, D10W, D15W, and D20W. Monitoring serum levels daily is not routinely necessary. When the infant is receiving amphotericin B, it is necessary to augment the sodium intake to at least 4 mEq/Kg per day to help prevent ­nephrotoxicity.

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34. Correct answer: ­B CMV depresses the bone marrow leading to the petechiae or purpura. This condition usually appears within the first few hours after birth and lasts for a few days. It may or may not be associated with ­thrombocytopenia. 35. Correct answer: ­C This infant has probably acquired bacteria from the birth canal and generally these bacteria are responsible for ­early-­onset ­pneumonia. 36. Correct answer: ­C If the infant is handled, it would increase oxygen consumption. If the infant becomes hypothermic, she may become peripherally vasoconstricted and go into anaerobic metabolism and metabolic ­acidosis. 37. Correct answer: ­D Erythropoietin is a hormone responsible for the synthesis of hemoglobin. Erythropoietin levels increase in response to anemia and low oxygen states and levels decrease in response to hypertransfusion. Levels are also increased in infants with Down syndrome and intrauterine growth restriction. Erythropoietin may also be elevated in infants born to mothers with ­pregnancy-­induced hypertension or ­diabetes. 38. Correct answer: ­D Teen mothers are more likely to become pregnant again within 2 years if not educated regarding contraception and birth control measures. This may include abstinence, oral contraceptives, intrauterine devices, hormone injections, and vaginal contraception. Information should be presented without judgment or pressure, but in conversation regarding future planning. Teens with infants may find it difficult to complete schooling and obtain employment while raising a child. Additional topics and education should include parenting classes, day care options, home health visits, community support groups, and ­follow-­up clinics. It may be easier for the nurse to plan all the ­follow-­up visits, but this does not help the mother in building confidence in becoming a mother or provide information to learn to manage the healthcare system. Teen education should focus on providing information, building confidence and knowledge, and guiding the teen through care. Teaching sessions should be in clear, understandable terminology and should include the mother, the father if involved, and ­grandparents/support ­persons. 39. Correct answer: ­B A phenotype is what you can observe about your patient (or any organism). The phenotype is comprised of the atoms, molecules, macromolecules, cells, structures, metabolism, energy utilization, tissues, organs, reflexes, and behaviors—­anything that is part of the observable structure, function, or behavior of your ­patient. 40. Correct answer: ­A Cardiac glycosides possess positive inotropic activity, which is mediated by inhibition of ­sodium-­potassium adenosine triphosphatase. Also, cardiac glycosides reduce conductivity in the heart, particularly through the atrioventricular node, and therefore

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have a negative chronotropic effect. The cardiac glycosides have very similar pharmacological effects but differ considerably in their speed of onset and duration of action. They are used to slow the heart rate in supraventricular arrhythmias, especially atrial fibrillation, and also are used in chronic heart ­failure. 41. Correct answer: ­B Prostaglandin E1 may cause respiratory depression, apnea, hypotension, hyperthermia, flushed appearance, and ­bradycardia. 42. Correct answer: ­A Arnold-­Chiari syndrome is a central nervous system condition that can affect the spinal nerves, resulting in bilateral vocal cord paralysis. The brainstem displaces downward and actually stretches the vagus nerve. This condition may be corrected surgically by relieving compression of the affected ­area. 43. Correct answer: ­D Pink, frothy secretions may indicate multiple factors such as traumatic intubation, carina irritation, and pulmonary hemorrhage. The presence of ectopy on the monitor indicates a misplaced UVC. If the UVC is inserted too deep, the catheter may travel into the right atrium and then into the ventricles or left atrium, causing cardiac irritation and ectopy. The chest ­X-­ray will assist in determining the UVC and the endotracheal tube position. Additional complications of a deep UVC placement may include emboli formation, infarction, pericardial effusions, endocarditis, pulmonary infarction, and pulmonary hemorrhage. The physician should be notified of your findings, and you should anticipate the physician ordering the line pulled back to a position just above the right atrium and a ­follow-­up chest X-­ray. 44. Correct answer: ­C Prior to starting any medications or fluids, confirm line placement with the physician or radiologist to avoid injury to the patient. The umbilical artery catheter tip should be located within T6–T9 for a high line and between L3 and L4 for a low line. The umbilical venous catheter tip should be located just above the diaphragm or only 2–5 cm in depending on gestation and weight. Never advance a catheter if the sterility has been broken. This patient should have the arterial line withdrawn to T6–T9. Repeat the chest ­X-­ray to confirm the new tip placement. Originating from the aortic arch, the coronary and subclavian arteries (above T5), celiac and superior mesenteric arteries (T11–T12), renal artery (L1), the inferior mesenteric arteries (L2), and iliac arteries (L4–L5) may be damaged by fluids and medications administered via misplaced umbilical artery catheter. A venous catheter tip in the liver may cause irreversible damage to the ­liver. 45. Correct answer: ­A The ECMO pump does not have the pulsatile force of a normal heart. After the pump is discontinued, renal function should improve. A CVA or intraventricular hemorrhage could occur, but these would be ­long-­term ­effects.

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46. Correct answer: ­C Selected side effects of methylxanthines include gastroesophageal reflux, jitteriness, wakefulness, tachycardia, diuresis with loss of sodium and calcium, and ­hyperglycemia. 47. Correct answer: ­B Mandibular hypoplasia prior to 9 weeks gestation is the hallmark of Pierre Robin sequence. The characteristics of Pierre Robin sequence include glossoptosis, micrognathia, and a cleft ­palate. 48. Correct answer: ­A Infants with choanal atresia often have other defects, like CHARGE syndrome. The echocardiogram would be appropriate to help rule out congenital cardiac ­abnormalities. 49. Correct answer: ­C The pH is above 7.45, indicating alkalosis. The PaCO2 is on the alkalotic side of normal, indicating a respiratory ­alkalosis. 50. Correct answer: ­B Digoxin (Lanoxin) is a frequently used cardiac glycoside that inhibits sarcolemmal ­Na-­K adenosine triphosphatase, which leads to an increase in intracellular Ca concentration and increased myocardial contractility (the inotropic action). Digoxin has direct inotropic effects in addition to indirect effects on the cardiovascular system. Digoxin acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any increase in mean arterial ­pressure. Patients who are hypersensitive to digoxin may develop atrioventricular block, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, hypokalemia, or renal ­failure. 51. Correct answer: ­D Normal temperature for an infant is between 36.5 and 37.5° C (97.7–99.5° F). Mild hypothermia begins when neonatal temperature falls below 36.4° C or 97.6° F. Moderate hypothermia begins at temperatures below 35.9° C or 96.6° F. Severe hypothermia and death result when the temperature drops below 32° C or 89.6° F. Premature infants are at greater risk of temperature instability at considered normal ranges if there is concurrent sepsis, cardiac anomalies, or respiratory distress. Brown fat deposits are incomplete in the premature infant as the majority of brown fat deposits occur during the third trimester. Similar to a match, brown fat burns fast and hot and requires oxygen to be metabolized. If the neonate is hypoxic, then metabolism of brown fat is further restricted. Environmental factors greatly impact the neonate’s ability to conserve heat and ­self-­regulate temperature. Bed type, humidity, moisture, ambient temperature, air drafts, and the temperature of items touching the skin (equipment, fluids, clothing) all influence neonatal ­temperature.

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52. Correct answer: ­A The pH is outside the normal range, so it is uncompensated. The pH is elevated, indicating alkalosis. The HCO3 is normal, the PaCO2 is decreased, and that indicates respiratory ­alkalosis. 53. Correct answer: ­D The bilious vomitus is the hallmark of malrotation and volvulus. This is a surgical emergency to prevent necrotic bowel. Duodenal atresia may also have bilious vomitus, but a dilated abdomen and duodenum are seen on abdominal ­X-­rays. Meconium plug and Hirschsprung’s disease have abdominal distention and delayed stooling as their ­hallmark. 54. Correct answer: ­B Chronic hypoxia leads to chronic respiratory acidosis. The kidneys then retain bicarbonate in the form of sodium bicarbonate. The bicarbonate is exchanged for sodium chloride. Ammonia is an acid, and excess amounts must be removed from the body. This is done by releasing ammonium chloride. In chronic hypoxia, there is an increase in bicarbonate levels and a decrease in chloride levels. Other causes of hypochloremia are NG suction, vomiting, and ­diarrhea. 55. Correct answer: ­D Myocarditis is clinically defined as inflammation of the heart muscle. There are numerous infections, systemic diseases, drugs, and toxins that associate with the development of myocarditis. Viruses, bacteria, protozoa, and even worms have been implicated as infectious agents. Coxsackie B1 has been more prevalent as a frequent cause of myocarditis. The normal WBC count in neonates varies, but values of , 4,000/μL or . 25,000/μL are abnormal. The absolute band count is not sensitive enough to predict sepsis, but a ratio of immature:total polymorphonuclear leukocytes , 0.2 has a very high negative predictive value. A rapid fall in a known absolute eosinophil count and morphologic changes in neutrophils may indicate sepsis. The platelet count may fall hours to days before the onset of clinical sepsis but more often remains elevated until a day or so after the neonate becomes symptomatic. 56. Correct answer: ­B If one individual carries two different cell lines, this is known as mosaicism. Mosaicism occurs during mitosis (after fertilization) and results in two different cell lines in the same person. Selected disorders include hemophilia A and B, Marfan syndrome, Duchenne muscular dystrophy, neurofibromatosis type I and type II, and ­pseudoachondroplasia. 57. Correct answer: ­D The radiant warmer should be set to ­servo-­controlled temperature regulation based on neonatal skin temperature. If a heat lamp is utilized at the same time, the ­servo-­probe may register the heat lamp and not accurately interpret the infant’s temperature, resulting in a failure to heat the warmer properly, and the neonate may become cold. Premature neonates should always be placed in ­double-­walled isolettes to minimize heat loss via convection and radiation. Never use any blanket or fluid warmed in the

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microwave. The heat element heats unevenly and cannot be regulated from unit to unit. Hot spots in the blanket that come in direct contact with skin will cause burns to the fragile dermal layers. This is also true of water bottles, gloves filled with hot tap water, and chemical mattresses in direct contact with ­skin. 58. Correct answer: ­B An infant with Turner syndrome will have monosomy—­the loss of all or part of one copy of the X chromosome. Often, the paternal X chromosome is missing. Turner syndrome is a condition associated with small stature, narrow palate, ­low-­set ears, hearing loss, ptosis, and a broad nasal bridge. This infant may also have a broad chest with ­wide-­spaced nipples, pectus excavatum, lymphedema of the extremities, and insufficient circulating concentrations of female sex steroids. The infant may also be infertile and have normal intelligence. Diagnosis is based on phenotype combined with a genotype that includes partial or complete ­X-­chromosome monosomy (45,X). 59. Correct answer: ­B Acute pyelonephritis can cause acute intrinsic renal failure. Fluid management should focus on fluid restriction to prevent fluid overload. Urine output of 30 ml/kg/d plus for term infants and up to 70 ml/kg/day plus urinary output for preterm infants is the goal of fluid management. With a fluid restriction, the patient should have a 20–30 g/d weight loss. Once normal kidney function is established, strict fluid monitoring should include daily or twice daily weights. Nutritional support at 100 cal/kg with fat and carbohydrates and 1 to 2 g/kg/d of protein is necessary. Blood pressure should be closely monitored to provide early indication of congestive heart failure and renal ­damage. 60. Correct answer: ­D Duodenal atresia is seen in about 30% of infants born with Down syndrome. On ­X-­ray, a “double bubble” of gas is often seen. Treatment will include an NG tube, NPO status, and IV fluids. Darsey may also receive ­antibiotics. 61. Correct answer: ­C Four cardiorespiratory problems are most commonly associated with periventricular leukomalacia (apnea, PDA, pneumothorax, and RDS). The ­hypoxic-­ischemic injury caused by these problems is seen in the vulnerable areas of the white matter around the lateral ventricles and the foramen of Monro. Because the white matter in myelinated nerve tissue that carries messages to the body is affected, there can be severe motor problems, delayed mental development, hearing and vision deficits, and coordination problems. There is no treatment for PVL, only supportive care. The prognosis depends on the severity of the brain ­damage. 62. Correct answer: ­C Erythroblastosis fetalis is the result of Rh incompatibility in the fetus. This condition usually results in anemia and hyperbilirubinemia. Too many circulating immature red blood cells (erythroblasts) are forced into the fetal circulation to compensate for rapid destruction of fetal blood cells. Anemia and hyperbilirubinemia are the result. Jaundice is secondary to the rapid rise in bilirubin and the infant may have hepatosplenomegaly. Hydrops fetalis may also be ­present.

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63. Correct answer: ­D The best approach to parental concerns is to address them directly and then provide the reason for your actions. Isolette walls may be clear, but are a physical barrier to the parents as compared to an ­open-­access radiant warmer. To encourage parent bonding, support open communication, and provide teaching, the nurse’s best response is, “We will assist you in touching and holding your baby as he gets better, and the isolette allows for better temperature control and reduces stress to the baby.” Leaving an infant in the warmer may meet the mother’s needs, but it does not address the infant’s need for a stable, neutral thermal environment. Stating policy and ignoring parent concerns closes communication and removes parent involvement in patient care and further compounds barriers to ­bonding. 64. Correct answer: ­B Possible adverse effects of fentanyl include respiratory depression, chest wall rigidity, apnea, laryngospasm, abdominal distention, loss of bowel sounds, and muscle ­r igidity. 65. Correct answer: ­B After plain films, a barium enema may be administered. The rectal biopsy is the best diagnostic tool for Hirschsprung’s disease. The biopsy shows a lack of parasympathetic ganglions in the distal bowel and rectum. This problem usually develops around the 12th week of gestation. Hirschsprung’s is seen in males four times more often than in females, and it may also be seen in ­families. 66. Correct answer: ­B Hypoxemia is a decreased oxygen level in the arterial blood or a PaO2 of , 80 mmHg. Hypoxia is defined as a decreased oxygen level at the cellular level. 67. Correct answer: ­B Four days of high FiO2 has resulted in a nitrogen ­wash-­out resulting in atelectasis. Nitrogen’s high partial pressure is necessary to maintain alveolar inflation. It is important to titrate FiO2 to maintain saturations within a prescribed range when oxygen therapy is utilized. Pulmonary edema would result in coarse breath sounds. With a unilateral pneumothorax, there would probably be tracheal ­deviation. 68. Correct answer: ­D Trisomy 18 severely affects all organ systems. The physical appearance of the infant at birth will suggest the diagnosis of trisomy 18. However, most babies are diagnosed before birth by amniocentesis. Ultrasounds of the heart and abdomen can detect abnormalities, and ­X-­rays may detect deformities of the skeleton. Low birth weight even at term is common. The following areas may be ­affected: Nervous system and brain—­mental retardation and delayed development, high muscle tone, and physical malformations such as brain ­defects Head and face—­small head, small eyes, ­wide-­set eyes, small lower jaw, ­low-­set e­ ars Heart—­ventricular septal defect and patent ductus ­arteriosus Bones—­severe growth retardation, clenched hands with second and fifth fingers on top of the others, unfolded ­thumb Digestive tract—­malformations Urinary tract—­malformations Genitals—­malformations

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69. Correct answer: ­A Characteristics of an infant with fetal alcohol syndrome would include microcephaly, cardiac defects such as a ventricular septal defect, small for gestational age, small eye openings, hyperactive behavior, and sleep and sucking disorders. Additional defects may include a sunken nasal bridge, shortened nose, flat cheekbones, smoothing and elongation of the ridged area (the philtrum) between the nose and lips, and a smooth, thin upper ­lip. 70. Correct answer: ­B A shift to the right means hemoglobin has less affinity for oxygen. In other words, O2 will be given off more readily to the tissues. A chemical called 2,3 DPG (2,3 diphosphoglycerate) helps force O2 off the hemoglobin molecule. So if 2,3 DPG is decreased, the hemoglobin will hang on to the O2 . If the temperature is increased, the tissues need more O2 . Therefore, if the temperature is decreased, the O2 will not be given off to the tissues as readily. If the PCO2 is elevated, the tissues need more ­oxygen. 71. Correct answer: ­D Excess fetal insulin may be the cause of delayed maturation of type II alveolar cells and pulmonary surfactant deficiency. The excess insulin may cause congenital cardiac anomalies such as cardiomyopathy and intraventricular septal ­hypertrophy. 72. Correct answer: ­C Prematurity is present in almost all cases of respiratory distress syndrome. Surfactant production is usually diminished, and this leads to atelectasis and pulmonary edema. The patient worsens, an alteration in the ventilation perfusion ratio occurs, and functional residual capacity is diminished. Serum protein leaks into the alveoli and inhibits surfactant function. The immature lung lacks the ability to clear this fluid, and they are more compromised because of decreased surface area for gas exchange. The collapsed alveoli fill with a ­protein-­r ich fluid that forms hyaline ­membranes. Spasm of the pulmonary arteries results in blood being shunted away from the lungs via the foramen ovale and ductus arteriosus. These abnormalities all result in respiratory failure with poor oxygenation of the ­blood. 73. Correct answer: ­D Energy drinks contain massive amounts of caffeine and a variety of ingredients that will break down into methamphetamines and amphetamines with effects on the fetus similar to fetal alcohol syndrome. Immediate presentation includes small for gestation, dehydration, poor coordination for suck and swallow, hyperactive behavior, and sleep disturbances. ­Long-­term effects include learning disabilities, speech and language delays, poor judgment, and low intelligence ­quotient. 74. Correct answer: ­C Answers A, B, and D are not signs, they are tools for diagnostics. Grunting is frequently seen in infants with RDS because they are trying to prevent alveolar collapse. The grunting sound is produced from the closure of the glottis in the attempt to maintain normal functional residual capacity. Other signs might include tachypnea (above 60/minute), retractions, flaring of nares, open mouth (decreases resistance), cyanosis on room air; PaO2 below 60 mmHg (torr) in FiO2 . 0.4, reduced air entry, stridor, and ­apnea.

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75. Correct answer: ­A Milrinone is a class III phosphodiesterase inhibitor with inotropic, lusitropic (myocardial relaxation), and vasodilator ­properties. 76. Correct answer: ­C In an infant with TTN, the pCO2 tends to be lower than in an infant with RDS. TTN may require positive pressure support of short ­duration. 77. Correct answer: ­B Congenital diaphragmatic hernia is a herniation of the abdominal viscera through the diaphragmatic defect during fetal life. The herniation impairs lung development, causing pulmonary hypoplasia. This affects the number of bronchial divisions and alveoli, the pulmonary vasculature, and both lungs are affected. One lung is affected to a lesser degree than the other. The decreased alveolar surface and reduced available vascular area lead to hypoxia, hypercarbia, and pulmonary hypertension after birth. Not only is the pulmonary vascular area reduced, but there is also increased thickness of the peripheral pulmonary arteries, into ­intra-­acinar arteries. This results in increased vulnerability to pulmonary vasoconstriction, which results in pulmonary hypertension and ­r ight-­to-­left shunting through the foramen ovale and the ductus ­arteriosus. 78. Correct answer: ­C Positive pressure ventilation and reduced lung compliance are significant predisposing factors for the development of PIE. Infants are often mechanically ventilated for other conditions such as respiratory distress syndrome (RDS), meconium aspiration syndrome (MAS), amniotic fluid aspiration, and infection. However, in extremely premature infants, pulmonary interstitial emphysema can occur at low mean airway pressure and probably reflects increased sensitivity of the stretch in an underdeveloped lung. Pulmonary interstitial emphysema has been rarely reported in the absence of mechanical ventilation or continuous positive airway pressure. PIE is a collection of gases inside the connective tissue surrounding the lung ­structures. 79. Correct answer: ­C Congenital lobar emphysema is caused by bronchial narrowing. Weakened or absent bronchial cartilage is present, so that there is inspiratory air entry but collapse of the narrow bronchial lumen during expiration. This bronchial defect results in lobar air trapping. Surgery is indicated in most cases, but rarely the condition may resolve with ventilatory therapy. Emergency lobectomy may be required if the lobe cannot be decompressed by intubation of the opposite side or by placing the infant in the decubitus position with the involved lobe ­dependent. 80. Correct answer: ­B Calcium channel blockers act primarily on arteries to arterioles. Large lumen vessels in the arterial system are affected. The advantage of this action is that both systolic and diastolic pressures are reduced, and the patient will not have a drop in blood pressure. The blood pressure may be lowered slightly and cause a reflex baroreceptor response to speed up the heart rate to maintain cardiac ­output.

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81. Correct answer: ­A Ideally, this infant should have arterial blood gases drawn as ordered. However, this is not possible. A newer ETCO2 device provides continuous inline CO2 monitoring. This new device allows continuous measurement with minimal dead space with improved accuracy. Capillary blood gases provide very little information about oxygenation status. Guessing the patient’s ­acid-­base status is dangerous. A twice daily chest ­X-­ray provides no information about a patient’s ­acid-­base ­status. 82. Correct answer: ­D This baby has likely been exposed to cocaine during the pregnancy and prior to delivery. Cocaine increases the risk of fetal cerebral vascular accident and intraventricular hemorrhage due to the stress of the vaginal delivery. Cocaine use should be considered with any mother presenting with eclampsia without proteinuria. Maternal urine should be tested for benzoylecgonine, which is a metabolite only seen with ­cocaine. 83. Correct answer: ­A The infant should be treated for respiratory depression and the airway supported with positive pressure ventilation and intubation if needed. Time will resolve the respiratory depression. The sudden collapse of a young woman at a party without explanation is suspicious for Gamma hydroxybutyrate (GHB) or ketamine ingestion. These are known date or party rape drugs. Due to the anesthetic and amnesic effects of the drug, an affected female may be unable to describe events leading up to the collapse. To determine exposure, the drug screen should be repeated looking specifically for Gamma hydroxybutyrate (GHB) and/or ­ketamine. 84. Correct answer: ­D A high serum magnesium or phosphate level, low serum calcium, with catecholamine release results in secretion of parathyroid hormone. Bone breakdown is accelerated, releasing calcium into the blood. Calcium reabsorption is increased in the intestine, and kidney tubule reabsorption is decreased. Phosphate loss is increased in the urine, which decreases serum phosphate. Parahormone increases reabsorption of magnesium by renal tubules. Renal calculi may ­develop. 85. Correct answer: ­C Elimination of CO2 is controlled by the oscillatory amplitude and the rate (hertz). 86. Correct answer: ­C In congenital lobar emphysema, the left upper lobe is affected 41% of the time, the right middle lobe 34% of the time, and the right upper lobe 21%. There are rare cases of both lungs affected. Congenital lobar emphysema has two forms—­hypoalveolar (less than the expected number of alveoli) and polyalveolar (greater than the expected number of alveoli). 87. Correct answer: ­D The B lymphocytes originate in bone marrow and mature there. The B lymphocytes form antibodies (immunoglobulins) that formulate a response to a specific antigen that binds itself to the B cell’s receptor sites. The B cell then forms a specific antibody for that particular antigen. The following five different types of immunoglobulins

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are available: IgG, IgA, IgM, IgE, and IgD. After the antibodies are synthesized, the specific antibody can attach to its antigen and set off the reaction to allow for phagocytosis. The cells retain a “memory” for the specific antigen and if another exposure occurs, the response will be quicker and ­stronger. 88. Correct answer: ­D Intrauterine growth restriction is the most common fetal effect if the mother abuses and is addicted to solvents. Infants may also deliver prematurely, have low birth weights and potential acid-base ­imbalances. 89. Correct answer: ­D Airway management is your priority as this infant had no spontaneous ­respirations. 90. Correct answer: ­B The type of echocardiography that shows the quantity of flow across an obstruction is known as ­continuous-­wave echocardiography. This type of echocardiography is used to detect direction of shunting, estimating cardiac output, and assessing ventricular diastolic function. This type of echocardiography provides a good estimate of pressure ­gradients. 91. Correct answer: ­D The most important nursing consideration in the neonatal patient with HHNS is to prevent aspiration. The severe hypokalemia is the result of no ­intracellular-­to-­extracellular shift occurring because the patient is not usually acidotic. When the patient is hypokalemic, hypocalcemia and hypomagnesemia also occur. Muscle activity is compromised, and the patient is at high risk for aspiration because of a paralytic ­ileus. 92. Correct answer: ­D The mechanism of this injury is often seen with subgaleal hemorrhage especially with the prolonged labor and vacuum extraction attempts. Subgaleal hemorrhage occurs in the potential space between the aponeurosis and the skull. Whenever there is a prolonged labor, vacuum extraction, forceps use, or a coagulopathy, a subgaleal hemorrhage can occur. This is a medical emergency requiring blood transfusion, frequent coagulation lab tests, and vitamin K administration. The infant may develop hemorrhagic shock. The hemorrhage generally resolves spontaneously in a few ­weeks. 93. Correct answer: ­A Methamphetamines create an increased risk for cleft palate/lip, hypoglycemia, seizures, poor sucking, and feeding intolerance. Cardiac rhythm disturbances such as supraventricular tachycardia may occur. In addition, the baby may have hyperthermia, agitation, tremors, intraventricular hemorrhages, and a ­high-­pitched ­cry. 94. Correct answer: ­C Infants of smokers are at higher risk for sudden infant death syndrome (SIDS) with the risk increasing if the parents smoke with the infant in the same room and with the number of cigarettes smoked. Parents should be given literature and referrals for smoking cessation classes and support prior to discharge. If the parents smoked regularly

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during the pregnancy, additional ­follow-­up teaching should include keeping appointments to monitor growth (due to intrauterine growth restriction), neurologic function, vision and hearing screening due to malformations, and speech therapy if language delays are ­noted. 95. Correct answer: ­A Alcohol withdrawal symptoms may occur within the first 12 hours of life. Presentation includes hypertonia, tremors, feeding intolerance with poor coordination and weak suck, and opisthotonos (severe arching of the back and neck). Comfort measures should include decreased external stimulation with a darkened and quite room, containment, and grouped ­cares. 96. Correct answer: ­D A PDA or ventricular dysfunction in a premature infant is done using ­t wo-­dimensional ­echocardiography. 97. Correct answer: ­D A maternal infection such as salmonella can lead to necrotizing enterocolitis in an infant. The infant in option A should be carefully monitored for abdominal distention, hypotension, lethargy, and bloody ­stools. 98. Correct answer: ­B After teaching, parents should have the knowledge and confidence to identify and intervene when negative cues are present. Additional teaching is needed if the parents only call the nurse’s attention to the behaviors, but fail to become involved. Teaching during kangaroo holding should also include positioning in the flexed position with ­skin-­to-­skin touching from chest to umbilicus to maximize ­benefits. 99. Correct answer: ­B In preterm infants, the arterial oxygen tension should be maintained between 50 mmHg and 80 ­mmHg. 100. Correct answer: ­D If positive inspiratory pressure (PIP) is increased on a mechanical ventilator, the result ­ aO2 . will be to decrease the PaCO2 and increase the P 101. Correct answer: ­D When swabbing the mouth of an infant with thrush, it is very important to use a ­cotton-­tipped applicator. It is necessary to distinguish between thrush and milk curds. If the white areas are thrush, they will bleed and be very raw after removal. Administer gentian violet (1–2%) swabbed on oral lesions. Do not get the gentian violet on normal mucosa because it burns the tissue. Gentian violet will also stain the mouth and any clothing. After 1 hour (or after feeding) nystatin can be applied to the baby’s oral mucosa. Swab skin lesions with topical nystatin and instruct the mother to treat her nipples with nystatin if she is ­breastfeeding.

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102. Correct answer: ­C When utilizing transcutaneous PO2 measurements, underestimation of oxygenation may occur when the skin is hypoperfused or the calibration is improper. Overestimation of oxygenation may occur if there is an air bubble or leak between the electrode and the ­skin. 103. Correct answer: ­C Micrognathia, a cleft lip and palate, along with a large protruding tongue, are classic characteristics of Pierre Robin syndrome. Upper airway obstruction is common, and feeding can be quite ­difficult. 104. Correct answer: ­B Infratentorial subdural hematoma can occur in this patient due to the shearing forces associated with a breech presentation. The symptoms may not be seen for as long as 12 hours. A change in the rate and depth of respirations occurs first. Later, the baby’s cry becomes high pitched or hoarse, followed by decreased feeding and sucking. The fontanelles become firm and bulging due to the increased intracranial pressure (ICP). 105. Correct answer: ­D Contraindications for the use of indomethacin to close a PDA in a premature infant would be a serum creatinine clearance of . 1.8 mg/dl, a BUN . 30 mg/dl, and a urine output , 0.6 ml/Kg/hour. Necrotizing enterocolitis is not a contraindication. 106. Correct answer: ­A High-­frequency jet ventilators (HFJV) deliver ­high-­flow, s­ hort-­duration pulses of pressurized gas directly into the upper airway through a specially made endotracheal tube. The pulses are delivered to the upper airway and are superimposed on gas flow from a conventional ventilator that provides positive ­end-­expiratory pressure (PEEP). In addition, conventional breaths may be delivered in conjunction with the jet ventilation. The systems operate at rates of 150–600 breaths per minute and exhalation is ­passive. 107. Correct answer: ­C AT ­ -­piece resuscitator or ­flow-­inflating b ­ ag-­mask-­valve would be appropriate methods for providing continuous positive airway pressure (CPAP). A ­self-­inflating ­bag-­mask-­valve requires the user to squeeze the bag to provide oxygen. Current NRP guidelines recommend that the initial oxygen begin at less than 100% FiO2 during resuscitation of a premature neonate and that oxygen be titrated for saturation via pulse ­oximetry. 108. Correct answer: ­A If neonatal circulation is compromised, the lungs may not have exhaled adequate quantities of CO2 to register a change in the device. The resuscitation team must use primary confirmation (equal chest rise, audible breath sounds in all lung fields, absent air movement over the stomach, rise in heart rate, and visualization of the tube between the vocal cords) to determine positive endotracheal tube ­placement.

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109. Correct answer: ­B If the PaO2 is low, check chest wall movement (it should be symmetrical) and breath sounds, establish ETT patency, transilluminate, check for water in ETT or ­T-­piece, and obtain a stat chest ­X-­ray. The problem could be an overinflated lung, so check blood pressure and reduce the MAP to see if oxygenation ­improves. 110. Correct answer: ­A High-­f requency ventilators have the advantage of transporting CO2 out of the lungs with smaller pressure and volume fluctuations than are required during conventional mechanical ventilation. Originally it was thought that adequate gas exchange could be obtained at relatively low mean airway pressures, but now it is known that recruiting lung volume is essential in not only optimizing oxygenation but also in minimizing lung injury. Studies have shown that if both types of ventilators had matched mean airway pressures, PaO2 was significantly greater during ­high-­f requency oscillation than during conventional mechanical ­ventilation. 111. Correct answer: ­D Cystic hygromas are ­fluid-­filled sacs that result from a blockage in the lymphatic ­system. Cystic hygromas are single or multiple cysts found mostly in the neck region. A cystic hygroma in a developing baby can progress to hydrops and eventually fetal death. Some cases of congenital cystic hygromas resolve, leading to webbed neck, edema, and ­lymphangiomas. Sometimes, the hygroma can progress in size to become larger than the ­fetus. 112. Correct Answer: ­C Thymus-­derived (T cells) lymphocytes initiate a response to delayed hypersensitivity reactions and ­graft-­versus-­host disease. The function of T cells is cellular immunity. These cells recognize the transplanted organ cells as foreign, resulting in a mounted attack, leading to rejection. Immunosuppressive drugs suppress this normal ­response. 113. Correct answer: ­C Standards of critical care should focus on both infant and parent response and interaction. Interventions of care should be individualized for each infant–parent group. Interventions are continually adjusted based on ongoing assessment of infant cues and response as well as parental involvement and interaction. Parental support and bonding should be encouraged and facilitated at every ­opportunity. 114. Correct answer: ­C The majority of prenatally diagnosed cystic hygromas are associated with Turner syndrome. Turner syndrome is a chromosome abnormality in which a female has only one X chromosome instead of two. Cystic hygroma is also associated with chromosome abnormalities such as trisomies 13, 18, and 21, as well as Noonan’s syndrome. The pattern of inheritance for these syndromes varies depending upon the specific syndrome. Rare cases of cystic hygroma can be inherited as an autosomal recessive disorder for which parents are carriers. A cystic hygroma can also be idiopathic. Parvovirus and fetal alcohol syndrome are potential causes of cystic ­hygromas.

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115. Correct answer: ­C Gastroesophageal reflux is considered a common physiologic condition that occurs in most people. In infants, the condition may spontaneously resolve within 18 months. It becomes a problem when they develop failure to thrive, apnea, aspiration, or stridor. Treatment involves using a thickening agent, feeding smaller volumes but more frequently, raising the head of the crib 45–60% after feeding, and keeping the baby still during ­feedings. 116. Correct answer: ­B Parental involvement is crucial at every stage of care. The neonatal intensive care setting may be overwhelming and frightening to parents. The nurse needs to offer short bursts of information with appropriate parent intervention to increase parental confidence and knowledge. Detailed and lengthy explanations at the physiologic level may be overwhelming and intimidating to new parents and hinder interaction. Parents can be taught about all stages of sleep and wake cycles during each visit by pointing out both positive and negative behavioral cues when present. Parents should then be taught what actions would assist in establishing stability. While at bedside, encourage and direct parent interactions without doing an action for ­them. 117. Correct answer: ­C A ­moderate-­sized ventricular septal defect causes the PVR (pulmonary vascular resistance) to be less than the SVR (systemic vascular resistance). This will cause a ­left-­to-­r ight shunt. Too much blood can enter the lungs, increasing edema and possibly preventing or delaying development and maturation of arterioles. A pansystolic murmur can be heard over the left sternal ­border. 118. Correct answer: ­C Tet spells may be treated with morphine to promote venous dilation. The dose is usually 0.1 to 0.2 mg/kg IM. IV fluids are used for volume expansion with an increase in systemic BP. If this does not control the spell, systemic BP can be increased with phenylephrine or ketamine (this has the added benefit of being sedating). Propranolol may prevent or mitigate ­spells. 119. Correct answer: ­D The mitral valve dysfunction is often seen with a postendocardial cushion repair. The LAP is usually around 8 mmHg, so 14 mmHg is high. The right atrial pressures would be increased by pulmonic stenosis or tricuspid ­regurgitation. 120. Correct answer: ­A Flumazenil is the reversal agent for benzodiazepines. Monitor the infant closely for seizure ­activity. 121. Correct answer: ­B This infant probably had some degree of PIE initially. However, the air has migrated from the ruptured alveoli and has traveled to the mediastinum, causing a pneumomediastinum. If a tension pneumothorax existed, the infant would desaturate, become hypotensive and bradycardic, and be ­c yanotic.

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122. Correct answer: ­C The PR interval on the EKG is 0.14 second and indicates a ­first-­degree heart block. A ­first-­degree heart block in a term infant is usually benign. The PR interval for a normal, term infant is 0.09 to 0.12 seconds. ­R ight-­sided heart failure usually results in tall, peaked P ­waves. 123. Correct answer: ­D Georgia is probably suffering from maple syrup urine disease. Her urine has the characteristic “burned sugar or sweet maple syrup” smell. Other symptoms include lethargy, poor feeding, muscle weakness, spasms, seizures, and poor weight gain. During acute episodes, the infant will exhibit hyperglycemia and profound ­acidosis. 124. Correct answer: ­A Barbiturates in general can cause respiratory depression. ­Stevens-­Johnson syndrome (SJS) is a serious, ­life-­threatening dermal reaction to a medication. SJS causes bullae and skin sloughing similar to a second- or ­third-­degree burn. Patients with more severe forms of SJS are often treated in burn units. Agitation is an idiosyncratic response to ­pentobarbital. 125. Correct answer: ­A The 2003 guidelines from the Centers for Disease Control (CDC) recommends that any equipment that comes in direct contact with patient mucosal membranes undergo ­wet-­heat pasteurization at . 158° F for at least 30 minutes and packed in sterile wrapping for the next use. An autoclave may also be used for reusable equipment. Any m ­ ulti-­use equipment is recommended for resterilization per FDA guidelines by a ­third-­party agency prior to use with another patient. If equipment must be washed after sterilization, the CDC recommends the use of sterile water, not normal saline. Although many units may direct respiratory therapy to maintain and sterilize all respiratory equipment, as a patient advocate, the bedside nurse should be aware of and ensure equipment is properly sterilized prior to use on his or her ­patient. 126. Correct answer: ­D The ­high-­f requency oscillator uses a rapidly moving piston to deliver gases to and from the baby, causing the chest to vibrate or wobble. This chest movement indicates that the ETT is in correct position as breath sounds and respiratory rate cannot be assessed while the ventilator is in use. Prolonged standby mode or disconnection of the ventilator for assessment or repositioning may cause the airway to collapse and increase air ­trapping. 127. Correct answer: ­A Establishing that there is an infection is important. Antibiotics such as ceftazidime are used due to their ­broad-­spectrum coverage. Cooling measures and acetaminophen are important to reduce the fever and therefore reduce the risk of febrile seizures. The other three options suggest fluoroquinolone antibiotics, which have a narrow antibacterial spectrum and are not appropriate for this ­infant.

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128. Correct answer: ­A The kidneys try to correct the imbalance by retaining bicarbonate. Chronic hypoxia results in increased CO2 , creating a chronic respiratory acidosis. The bicarbonate exchanges for the chloride to maintain a ­balance. 129. Correct answer: ­A Grunting results from a forceful closure of the glottis in an attempt to maintain functional residual capacity. FRC is the lung volume at the end of a normal expiration, when the muscles of respiration are completely ­relaxed. 130. Correct answer: ­B Type II truncus arteriosus is characterized by the right and left pulmonary arteries having separate origins. A short pulmonary artery may arise from the base of the common trunk (type I), or the branch pulmonary arteries may arise separately but in close proximity from the posterior surface of the common trunk. The right and left pulmonary arteries may arise widely separate from the lateral aspects of the truncal artery (type III). Truncus arteriosus results in ­left-­to-­right shunting of blood and pulmonary overcirculation. Congestive heart failure and pulmonary artery hypertension are common. Pulmonary vascular obstructive disease will occur early if no intervention is ­made. 131. Correct answer: ­D This patient is in metabolic acidosis. The acidosis increases the risk of bilirubin toxicity, so it must be corrected prior to the transfusion. Increased hydrogen ions and an acidotic state impede bilirubin binding. At a serum pH of 7.1, bilirubin binding drops to half its potential for binding at albumin binding sites. During acidosis, free fatty acids are formed and compete with the albumin binding ­sites. 132. Correct answer: ­B Bradycardic episodes may occur for up to 24 hours after administration of chloral hydrate to a premature infant. Other potential side effects include agitation, gastro­ intestinal effects, plus depression of the cardiac, CNS, and respiratory systems may occur with repeat ­doses. 133. Correct answer: ­C The knee jerk, also known as the patellar reflex, is an example of a functional lower motor neuron. These reflexes are also known as deep tendon reflexes. The nerve impulse makes an arc from the tendon to the sensory portion of the spinal cord to the motor root and back to the patella, causing extension of the lower ­leg. 134. Correct answer: ­A Frequent neuro checks are strongly recommended. The HOB elevated at 30° promotes drainage of CSF; and the head midline maintains optimal CSF drainage. It is imperative to notify the neurosurgeon at once if there is a change in the neuro signs, especially if the baby becomes ­lethargic.

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135. Correct answer: ­C Genetic disorders associated with advanced maternal age include trisomy 21, ­trisomy 13, and Klinefelter’s syndrome. All the possible selections in answers A, B, and D are associated with advanced paternal age. These disorders also carry an increased risk of new ­mutations. 136. Correct answer: ­D The clinical presentation is consistent with diaphragmatic hernia. Bowel tones may or may not be auscultated in the thoracic cavity if hypoxemia occurred during the labor process or resuscitation. Auscultation of sounds over the epigastrium may reflect air passage through the esophagus to the stomach rather than gastric motility. The degree of respiratory distress, unilateral chest rise, deviated heart tones, and abdominal appearance are dependent upon the severity and extent of the hernia. Although typically located on the left side, ­r ight-­sided hernias do occur. Endotracheal intubation and the placement of an orogastric tube to low intermittent wall suction are required to maximize ventilation and oxygenation. To decrease the risk of pneumothorax related to elevated airway pressures, the physician may purposely intubate the unaffected side to allow for lower ventilation pressures. Transillumination and chest ­X-­ray may be used to rule out secondary ­pneumothorax. 137. Correct answer: ­D A more appropriate term for hemorrhagic disease of the newborn is vitamin K deficiency bleeding (VKDB) disease. Vitamin K production depends on sufficient intestinal flora. Vitamin K is necessary for the production of calcium and various coagulation ­factors. Newborns are relatively deficient in vitamin K. Contributing factors to this deficiency include low vitamin K stores at birth, poor placental transfer of vitamin K, low levels of vitamin K in breastmilk, and sterility of the gut. Basic commercial infant formulas contain supplemental vitamin K, so VKDB is almost exclusively a condition resulting from breastfed infants. Infants with inadequate intake are at higher risk. The most common sites of bleeding are the umbilicus, mucous membranes, GI tract (melena), circumcision, and venipuncture sites. The infant may also present with a distended abdomen, jaundice, and signs of increased intracranial pressure. Hematomas, such as large cephalohematomas, along with bruising, are also common findings. Intracranial hemorrhage (ICH) is responsible for nearly all deaths from ­V KDB. 138. Correct answer: ­C An example of an autosomal dominant disorder would be adult polycystic kidney ­disease. Autosomal dominant inheritance means that the gene carrying a mutation is located on one of the autosomes. This means that males and females are equally likely to inherit the mutation. Dominant means that having a mutation in just one of the two copies of a particular gene is all it takes for a person to have a trait. So, if the infant is Dd, the trait will be expressed. The trait will not be expressed if the infant is ­dd. An important characteristic of dominant gene mutations is that they can have variable expression. This means that some people have milder or more severe symptoms than others. In addition, which systems of the body the mutation affects can vary as can the age at which the disease starts, even in the same ­family.

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Dominant gene mutations can have reduced penetrance. This means that sometimes a person can have a dominant mutation but not show any signs of disease. The concept of reduced penetrance is particularly important in the case of autosomal dominant cancer susceptibility genes. Sometimes, breast cancer occurs eventually in all or most generations in a family. This has led to genetic testing and to some women having mastectomies even if they do not exhibit the ­cancer. 139. Correct answer: ­A If neonatal circulation is compromised, the lungs may not exhale adequate quantities of CO2 to register a change in the device. The resuscitation team must use primary confirmation (equal chest rise, audible breath sounds in all lung fields, absent air movement over the stomach, rise in heart rate, and visualization of the tube between the vocal cords) to determine positive endotracheal tube ­placement. 140. Correct answer: ­D Tetralogy of Fallot results in low oxygenation of blood due to the mixing of oxygenated and deoxygenated blood in the left ventricle via the VSD and mixing of blood from both ventricles through the aorta due to the obstruction of blood flow through the pulmonary valve. This causes a ­r ight-­to-­left shunt. The primary symptom is low blood oxygen saturation with or without cyanosis from birth or developing in the first year of life. If the baby is not cyanotic, then it is sometimes referred to as a “pink tet.” Other symptoms include a harsh grade II to IV systolic murmur with a thrill, difficulty in feeding, failure to gain weight, and retarded growth and physical development. Polycythemia may be present with dyspnea on exertion, and clubbing of the fingers and toes is also seen. Children with Tetralogy of Fallot may exhibit “tet spells”. The precise mechanism of these episodes is unknown, but may result from a transient increase in resistance to blood flow to the lungs with increased flow of desaturated blood to the body. Tet spells may be precipitated by activity and are characterized by paroxysms of hyperpnea, irritability and prolonged crying, increasing cyanosis, and decreasing intensity of the heart murmur, and may result in hypoxic brain injury and death. Older children may squat during a tet spell, which cuts off circulation to the legs, raises intrathoracic pressure and systemic vascular resistance, and therefore improves blood flow to the brain and vital ­organs. 141. Correct answer: ­D The jet port of the endotracheal tube may become occluded with secretions, so a critical nursing intervention would include flushing the jet port every 3–4 hours with 0.5 ml of normal saline. The tubing should never be kinked when turning, repositioning, or moving the patient as it may cause a collapse of the alveoli. Respiratory rate cannot be directly monitored when ­high-­f requency ventilation is used. Instead, the chest should be monitored for continuous wobble or vibration to indicate patent airway and correct endotracheal tube ­placement. 142. Correct answer: ­D Retractions occur when parenchymal disease is present and the chest wall produces a greater negative pressure. The chest wall is very compliant and caves inward with a moderate decrease in pleural pressure. Retractions are best seen at the xiphoid and intercostal markings of the ­chest.

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143. Correct answer: ­D When caring for an infant with a pulse oximeter, it is important to use a disposable cuff. A reusable cuff will wear down and allow ambient light to get in and invalidate the readings. The disposable sensor should be checked at least every 8 hours. The pulse oximeter is useful for trending, but arterial blood gases must be drawn to correlate with the pulse ­oximeter. 144. Correct answer: ­B Indomethacin is a medication utilized to close a patent ductus arteriosus (PDA). The ductus arteriosus is a normal connection between the pulmonary artery and aorta; it is necessary for proper fetal circulation n. At birth, the rise in PaO2 and decline in prostaglandin concentration cause closure of the ductus arteriosus, usually within the first 10–15 hours of ­life. Patent ductus arteriosus (PDA) is a persistence of the fetal connection (ductus arteriosus) between the aorta and pulmonary artery after birth, resulting in a ­left-­to-­r ight shunt. Symptoms may include failure to thrive, poor feeding, tachycardia, and tachypnea. A continuous ­machine-­like murmur in the upper left sternal border is common. Diagnosis is by echocardiography. It is very important that you remember that the PDA results in a ­left-­to-­r ight shunt and a ­“machine-­like” murmur is heard. Administration of indomethacin (a prostaglandin inhibitor) with or without fluid restriction may be used in premature infants with a significant shunt but not in term infants with PDA. If the connection persists, surgical or ­catheter-­based correction is indicated. Endocarditis prophylaxis is recommended before and for 6–12 mo following a c­ orrection. PDA accounts for 5–10% of congenital heart anomalies, and the ­male-­to-­female ratio is about 1:3. PDA is seen in 45% of premature infants with birth weight , 1750 grams. 145. Correct answer: ­D The history indicates hypovolemia related to maternal blood loss and poor neonatal response to resuscitation. Since the airway has been secured with a moderate heart rate increase, volume expanders are now indicated. Initial resuscitation with 10 ml/kg of normal saline is appropriate if packed red blood cells are not immediately ­available. 146. Correct answer: ­C Blood pressure may be elevated due to several factors. The first action should be to check IV patency of the fentanyl drip. Vital signs are elevated and may indicate pain related to the chest tube and intubation. Brian also has generalized edema that may mask a PICC line infiltration. Treatment of potential causes of hypertension, including pain, hypervolemia, and titrating of inotropic medications should be initiated prior to starting antihypertensive medications. Manual cuff size should be ­t wo-­thirds the extremity diameter. Since the baby is now 25 days old, the cuff size should be increased for growth. Moving the cuff site does not identify probable causes for the hypertension and may delay appropriate ­treatment. 147. Correct answer: ­B The most common ­single-­gene disorder (autosomal recessive) in Caucasian populations is cystic fibrosis. Autosomal recessive inheritance means that two copies of an altered gene located on one of the autosomes (that is, not the X or Y chromosomes)

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must be present for an individual to be affected with the trait. The parents would both have the gene pattern Rr—­this is a ­heterozygote. An affected individual would get an rr pattern—­this is a ­homozygote. The risk of two heterozygotes, or carriers, having an affected child is 25% (1 in 4) for each child whom they have. There is a 3 in 4 chance that each child will not be ­affected. Males and females are at equal risk for being ­affected. Two affected individuals usually produce children who are all ­affected. 148. Correct answer: ­A Clinical presentation for this patient is consistent with renal vein thrombosis. This includes right flank mass, hematuria, hypertension, anemia, and thrombocytopenia. Management includes review of the history and physical for maternal diabetes, fetal asphyxia, maternal thiazide therapy, polycythemia, sepsis, RDS, or congenital heart disease. Treatment is focused on correcting the underlying illness, fluid therapy, possible dialysis, and close continuous ­monitoring. 149. Correct answer: ­D According to current NRP guidelines (5th ed.), the baby is not vigorous and should be immediately intubated and tracheally suctioned using the appropriate sized endotracheal tube and meconium aspirator. An additional indicator for immediate tracheal suctioning of meconium is the presence of a heart rate under 100 bpm. The priority is to open and clear the airway. If a baby is stimulated prior to clearing the airway of meconium, the baby is likely to aspirate the meconium, possibly leading to meconium aspiration syndrome. Once the airway is clear, the team should dry and stimulate the infant, using the bulb suction as appropriate. Due to the fragile mucosal membranes of the oral cavity, it is inappropriate to be aggressive while suctioning. If the baby has copious secretions, it would be appropriate to use wall suction to clear oral ­secretions. 150. Correct answer: ­D The K ­ leihauer-­Betke test identifies fetal hemoglobin in maternal blood. The results of this test allow for calculations to determine the amount of ­fetal-­maternal hemorrhage and the amount of immune globulin (RhoGAM) necessary to prevent sensitization. If a patient has a positive test, ­follow-­up testing at a postpartum ­check-­up should be done to rule out the possibility of a ­false-­positive result. For example, a ­false-­positive result in the mother could be caused by sickle cell trait, which causes persistent elevation of fetal ­hemoglobin.

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Neonatal CCRN Practice Examination Answer Sheet Use this sheet to test yourself with the practice examination.

1. A  B  C  D

26. A  B  C  D

51. A  B  C  D

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13. A  B  C  D

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14. A  B  C  D

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69. A  B  C  D

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71. A  B  C  D

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25. A  B  C  D

50. A  B  C  D

75. A  B  C  D

Turn the page to answer questions 76–150.

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80050_Rogelet_05Pass.indb 434

76. A  B  C  D

101. A  B  C  D

126. A  B  C  D

77. A  B  C  D

102. A  B  C  D

127. A  B  C  D

78. A  B  C  D

103. A  B  C  D

128. A  B  C  D

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104. A  B  C  D

129. A  B  C  D

80. A  B  C  D

105. A  B  C  D

130. A  B  C  D

81. A  B  C  D

106. A  B  C  D

131. A  B  C  D

82. A  B  C  D

107. A  B  C  D

132. A  B  C  D

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108. A  B  C  D

133. A  B  C  D

84. A  B  C  D

109. A  B  C  D

134. A  B  C  D

85. A  B  C  D

110. A  B  C  D

135. A  B  C  D

86. A  B  C  D

111. A  B  C  D

136. A  B  C  D

87. A  B  C  D

112. A  B  C  D

137. A  B  C  D

88. A  B  C  D

113. A  B  C  D

138. A  B  C  D

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114. A  B  C  D

139. A  B  C  D

90. A  B  C  D

115. A  B  C  D

140. A  B  C  D

91. A  B  C  D

116. A  B  C  D

141. A  B  C  D

92. A  B  C  D

117. A  B  C  D

142. A  B  C  D

93. A  B  C  D

118. A  B  C  D

143. A  B  C  D

94. A  B  C  D

119. A  B  C  D

144. A  B  C  D

95. A  B  C  D

120. A  B  C  D

145. A  B  C  D

96. A  B  C  D

121. A  B  C  D

146. A  B  C  D

97. A  B  C  D

122. A  B  C  D

147. A  B  C  D

98. A  B  C  D

123. A  B  C  D

148. A  B  C  D

99. A  B  C  D

124. A  B  C  D

149. A  B  C  D

100. A  B  C  D

125. A  B  C  D

150. A  B  C  D

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