Crush Step 2: The Ultimate USMLE Step 2 Review

Adam Brochert, M.D. Intern Department of Internal Medicine Eastern Virginia Medical School Norfolk, Virginia

t

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Chapter

Subject

Page

Introduction

vii

1

Internal Medicine

2

Cardiovascular Medicine

17

3

Pulmonology

25

4

Gastroenterology

29

5

Endocrinology

39

6

Nephrology

43

7

Rheumatology

47

II

Hematology

51

9

Oncology

59

10

Infectious Disease

69

11

Dermatology

79

12

Neurology

13

Immunology

95

14

Genetics

99

15

Geriatrics

103

16

Preventive Medicine, Epiderniology, and Biostatistics

105

17

Psychiatry

I 13

18

Gynecology

123

19

Obstetrics

13 I

20

General Surgery

147

21

Ophtbalmology

155

.

87

11

vi

Contents

Chapter

Subject

Page

22

Orthopedic Surgery

161

23

Neurosurgery

165

24

Elf. Nose, and Throat Surgery

169

25

Vascular Surgery

173

26

Urology

17S

27

Emergency Medicine

179

28 29 30 31 32 33 34

Pediatrics

181

Pharmacology

185

Radi.ology

191

Laboratory Medicine

193

Ethics

195

Photos

197

Signs, Symptoms, and Syndromes

203

35

Abbreviicltions

207

Index

219

This book was written because I felt there was not a good, c(Hnprehensive, high,. yield review book I()r the USMIE Step 2. The goal of tbe book is to provide inl(lfmation that has appeared on recent adIninistrations of Step 2. The eXaIn covers a lot of inf()rmation, hut if you know aU the concepts in this book, you should do much better than just pass: you should CRUSH THE BOARDS'

Step 2 is the same level of diflkulty as Step I, but tbe questions are more relevant to the practice of medicine. Step 2 stresses the things that are necessary to be a good first-year resident in the emergency room or a general clinic. Knowing how to diagnose, ll1anage, and treat COlTIIDon conditions is stressed. Not just theory, but practice--in other words, knowing the next step. The other topics tbat Irequently appear on Step 2 are treatable emergency conditions. Remember, these are the situations that you, as a future house officer, lllay have to diagnose and treat at three 0' clock in the morning while on call. Knowing how to ulanage exotic or rare conditions is low~yield. It is lunch more high~yield to know rare complications and presentations of common diseases. Usually, when you are asked about a rare disease, you siInply need to recognize it from a classic presentation.

Some information from Step I is high-yield for Step 2. Epidemiology and biostatistics are retested, as well as pharmacology and microbiology (which bngs cause which conditions in specific patient popnlations). Cardiac pathophysiology is high-yield, as is common EKG pathology. The behavioral science!psychiatry qnestions are also similar to those in Step I. Overall, though, Step 2 has a different l()Cus, and that focus is clinical. If a patient presents with chest pain, what would you do? What kinds of questions would you ask the patient? What tests would you order? What medications might yon give? There are also five general tips I would like to pass on to those preparing lor Step 2: 1. Always get more history when it is an option, unless the patient is unstable and itllmedi-· ate action is needed.

2. You must know cut-off values for the treatIllCl1t of cornlTlOll conditions (at what nlunbers do you treat hypertension and hypercholesterolemia, and at what CD4 connts do you need chemoprophylaxis in HIV?).

3. A presentation may be normal (especially in pediatrics and psychiatry) and ueed no treatrnel1t! 4. Don't I()rget to study your subspecialties. Just because you never took an ophthalmology rotation doesn't mean there won't be any questions about it on the exam. You don't have

vii

viii

Introduction

to be an expert, hut knowing common and life-threatening diseases in the sllbspecialties can increase you score substantially.

5. Remember that residency programs don't usually see the breakdown of your score, only those magic 2·· and 3-digit overall scores (in other words, dou't skip studying a suhject because you hate it and aren't going into it). Studying fbr Step 2 can seem like an overwhehning task. Given the time COIl'itraints of Iuedkal students in their clinical years, most need a concise review of the tested topics. It is my hope that CRUSH THE BOARDS will meet your needs in this regard.

Adam Brocbert, M.D.

HYPERTENSION Screening for hypertension should be done roughly every 2 years, starting at the age of 3. Whenever a patient COIneS in f()f any kind of medical visit or hospitalization, it is standard practice to measure the blood pressure. The current accepted eut-offvalue is BO/90 mmHg (lower in children). A blood pressure of 145/7 5 mmHg is still considered hypertension (isolated systolic hypertension) and should be treated if it persists. Both systohc and diastolic hypertension decrease life expectancy. Hypertension is not diagnosed until three separate measurements on three separate occasions are greater than 140/90 IlullHg (except in pregnancy. when waiting for a return visit could be devastating). Also, if hypertension is severe (> 210 systolic, > 120 diastolic, or end-organ effects). ilumediate trcatInent with 111edication is warran ted:

Basic studies and evaluation in a new hypertensive patient include urinalYSiS, chemistry panel 7, EKG, and hemoglobin/hematocrit. Do not tTeat hypertension until you have a diagnosis (hy~ pertension on three separate visits)! Once you have a diagnosis, first allow the patient 3-4 mouths of weight reduction, exercise. and other lifestyle modifications (low salt and ch01e5-terol diet, no alcohol or sJIlOking). If this approach is unsuccessful, only then do you start medicatioJl. There are f<)Hr first-line agents fl:.)T the treatment of hypertension: beta hlockers, thiazide diuretics, angiotensin--converting enzyme (ACE) inhibitors, and calcimn channel blockers. Which one you choose is olten based on the patient (see table, top of next page): For pI'egnant pati"nts, use hydralazine aud/or alphaIllethyJdopa. Labetalol is slowly gaining acceptance also. Remember that in patients with preeclampsia, I1lagncsium sulfate lowers blood pressure. For side effects of hypertension l1ll'dications (high yield), see phannaco]ogy section.

1

2

Internal Medicine

Hypertensive emergencies: usually occur when blood pressure is > 200/ I 00 mmHg. Defined as hypertension with acute end~organ damage (i.e., severe hypertension plus one of the fol~ lowing: acute left ventricular failure, unstable angina, myocardial infarction, or encephalopathy; syn1ptoms include one or Inorc of the follOWing: headaches, nlcntal status changes, vOlniting. blurry vision, dizziness, papilledelna). Hypertensive emergencies are all exception to the rule of measuring blood pressure 3 tinH:~S before treating! Use nitroprusside. nitroglycerin, diazoxide, or labetalol emergently. Secondary hypertension: clues include onset before 30 or after S5 years of age and other sug~ gestive history or lab values. In a young woman, the lTIosl COIlUllon cause .is birth control pills (discontinue them), f()llowed by renovascular hypertension due to fibrous dysplasia (renal bruit: use intravenous pyelogram or arteriograrD fl)f diagnosis; treat with balloon dilatation or angioplasty). In a young man, think of excessive alcohol intake or exotic conditions (pheo~ chromocylOma, Cushing's syndrome, Conn's syndrome, polycystic kidney disease). In elderly patients with new-onset hypertension, think renovascular hypertension due to atherosclerosis (renal bruit: ACE inhibitor precipitates reml failure). If you suspect secondary hypertension (95% of cases of hypertension are cssential, primary, or idiopathic), remember the follOWing hints and tests to order: 1. Pheochromocytoma: urinary catecholamines (vanillylmandelic acid, metanephrine) plus intermittent severe hypertension, dizziness, and diaphoresis 2. Polycystic kidney disease: flank mass, family history, elevated blood urea nitrogen, creatinine 3, Cushing's syndrome: dexamethasone suppression test or

24~hr

urine cortisollcvel

4. Renovascular hypertension: intravenous pyelogram or allgiograrn; look for bruit

5. Conn's syndrome: high aldosterone, low renin 6. Coarctation of the aorta: upper extremity hypertension only, unequal pulses, radiofemoral delay, associated with Turner's syndrome, rib notching onx·-ray Note: lowerirlg blood pressure lowers risk f(w stroke (hypertension .is the most important risk £1ctor), heart disease, lnyocardial infarction, renal failure, atherosclerosis, and dissecting "lOrtic aneurysllL Coronary disease is the nl0st comlUOll cause of death among untreated hypertensive patients. Don't f()rget to treat isolated systolic or diastolic hypertension if it persists. Note: Nitroprusside dilates arteries and veins, whereas nitroglycerin is a venodHator only and other rnedications are arterial dilators only (hydralazine, alpha[ antagonists, calcium channel

Internal Medicine

:I

blockers). Venodilators reduce preload, whereas arterial dilators reduce afterload (nitroprusside does both).

DIABETES Universal screening is generally not rccomnlended. Screening in patients who afC obese, > 45 years old, have a positive family history. or afC Il1Clnbers of certain ethnic groups (black, Aluerican Indian, Ifispanic) is filDre accepted but not lUli{()flnly. Classic symptoms of diabetes are polydipsia, polyuria, polyphagia and weight loss. Diagnosis is made by a fasting plasma glucose :0: 126 mg/dl (after an overnight fast) or a random glucose (no fasting) :0: 200 mg/dl. If the patient has classic symptoms, one measurement is enough to confinn a diagnosis, but in an aSYluptOInatic patient, the test should be repeated. Rarely, an oral glucose tolerance test (OGTT) is done and DM is diagnosed when levels :0: 200mg/dl are reached within or at 2 hours after a 7 S~gm gluc()se load is adruinistered orally.

The goal ofo'eatment is to keep postprandial glucose < 200 mg/dl and fasting glucose < 130 mg/ dl. Stricter control results in too many episodes of hypoglycemia (look for symptoms of sympathetic discharge and mental status changes) Important points: 1. Remelnber the inlportance of C--peptide in distinguishing between too Jundl exogenous insulin (low C-peptide with accidental overdose in a diabetic or factitious disorder) and an insulinoma (high C-peptide). 2. Because IV contrast agents can precipitate acute renal fajlure in diabetics and other renal patients, you should use contrast only if absolutely necessary. Make: sure that the patient is wen-hydra.ted befc)re using contrast agents in diabetics and renal patients to prevent renal damage.

3, Diagnosis of diabetie ketoacidosis(type I diabetes mellitus) requires hyperglycemia, hy~ perketoneU1ia, and rnetabolic acidosis. Treatulent involves flUids, IV regular insulin, and potassium and phosphorus replacernent. Do not usc hicarbonateunlcss the pH is < 7.0. Search fix the cause, which often is infection. The rnortality rate is about 10%.

4

Internal Medicine

4. Diagnosis of nonketotic hyperglycemic hyperosmolar state (type II diabetes mellitus) reo quires hypcrglycenlia and hyperosnlolarity without ketonelnia. Treatment involves fluids, fluids, fluids, IV insulin, and eleetrolyte replacement. The mortality rate is about SO%. Long-term cOlnplications of diabetes rnellitus include atherosclerosis, coronary artery disease. Inyocardial infarction, retinopathy, and nephropathy. Use of ACE inhibitors helps to prevent nephropathy; 30% of end-stage renal disease is caused by diabetes mellitus. Diabetes is associated with an increased risk of infections, peripheral vascular diseasc. (claudication, atrophy), gangrene (the lllosl common cause for nontraumic anlputations is diabetes), and neuropathy.

Peripheral neuropathy (autonomic and sensory) causes many problems in diabetics: IIlI

Gastroparesis (early satiety, nausea; treat with luetoclopromide and cisapride)

!Ii!

Charcot's joints (clef()flIH:d joints due to lack of sensation; patient puts too Inuch stress on

joints) II Irnpotence II

(from autonol11ic neuropathy as well as peripheral vascular disease)

Cranial nerve palsies (especially 3,4,6-ocular palsies; usually resolve spontaneously within

a few months) • Orthostatic hypotension (due to lack of effective sympathetic innervation; when patient sl:ands up, heart rate and vascular tone do not increase appropriately to maintain blood pressure) Note: Diabetics commonly have no chest pain with a rnyocardial infarction because of neu··· ropathy ("silent" MI).

Diabetics are also prone to foot infections, ulcers and gangrene because they cannot feel their feet and blood flow is poor so that infection does not heal well. Patients should wear comfortable, properly fining shoes and regularly inspect their own feet. When retinopathy becomes proliferative, the treatment is pametinallaser photocoagulation to prevent progression and blindness. All diabetics should be followed once a year by an ophthalmologist to monitor retinal changes. Know how to use regnlar and neutral protamine Hagedorn (NPH) insulin. Regular insulin "" 45 luinutes until onset, peak action at 3~4 hr, and duration of action for 6-8 hr. NPH insulin :::::: 1-1.5 hr until onset, peak action at 6'~8 hr, and duration of about 18-~20 hr. "Ifpatient has high (low) 7 AM glucose, increase (decrease) NPH insulin at dinner the night before. " If patient has high (low) noon glucose, increase (decrease)

AM

regular insulin.

.. If patient has high (low) S PM glueose, increase (decrease) morning NPH. .. Ifpatient has high (low) 9

PM

glucose, increase (decrease) dinner time regular insnlin.

Somogyi effeet vs. dawn phenomenon. The Somogyi effect is the body's reaction to hypoglycclnia. .If too IDuch NPI-I insulin is given at dinner time the night bd()re, the 3 AM glucose will be Jow (hypoglycemia).The body reacts by releasing stress hormones, which cause the 7 AM glucose to he high. TreatInent is to decrease insulin. The dawn phenomenon is hyperglycemia caused by norulal early AM growth hOnl10JlC secretion. 7 AM glucose is high, without: 4- AM hypoglyceIIlia (glucose normal or hi.gh at 4 AM). Treatment is to illCIease insulin. Follow cOlnpliann~ with henlOglobin Ale level, which is an accurate rneasure of overall con~ trol £()f the previous 3 lnonths. Patients are not: afraid to fudge their horne test nm:nber to please their doctors, and this is the way to catch them.

Internal Medicine

5

For surgery, patients with diabetes are allowed nothing by mouth (NPO). Give one~third to one~half of uormal insulin dose, then monitor glucose closely through case and postopera~ tively, using 5% dextrose in water (DSW) and IV regular insulin to maintain glucose control. Medications in diabetics: Chlorpropamide may cause syndrome of inappropriate secretious of antidiureti.c hormone (SIADH). Patients with type I DM are not helped by sulfonylurea medica ~ tions. Avoid beta blockers, which prevent many of the physical manifestations of hypoglycemia (tachycardia, diaphoresis); thereli)re, neither you nor the patient will know if the patient is becoming hypoglycemic.

CHOLESTEROL Measure total cholesterol and high-density lipoprotein (HDL) every S years (unless abuormal), starting at age 20 (although this recommendation is not universally accepted). Start earlier if the patient is obese or has a strong family history. Look for xanthelasma (know what it looks like), corneal arens (in younger patients), lipemic~looking serum, and obesity as mark~ ers of possible familial hypercholesterolemia. Family members should be tested. Also, look for pancreatitis with no risk factors (e.g., no alcohol, gallstones) as a marker fi)r familial hypertriglyceridemia.

Patient with xanthelasma in all four lids. (From Tasman W. Jaeger EA: The Wrlls Eye Hospital Atlas of Clinical Ophthalmology. Philadelphia, Lippincott-Raven, 1996, with permission.)

Risk factors for coronary heart disease (LDL and total cholesterol are risk factors for CHD, but do not count thelli in deciding to treat or not to treat high cholesterol): mAge (Iucn;<: 4.5, WOluen 2 SS or with prcrnaturc luenopause and no estrogen replacenlcnt

therapy)

Ii

Internal Medicine

M

Family history of premature CHD (defined as definite myocardial infarction or sudden death in father/firsHlegree male relative < 55 years old or mother/first-degree female relative < 65 years old)

• Cigarette smoking (> 10 cigarettes/day) w Hypertension ill

(:? 140/90 mmHg or on antihypertensive medications)

Diabetes lTlcllitus

EDL « 35 mg/dl) (Note: EDL :? 60 mg/ dl is considered to be protective and negates one risk factor.)

w Low

Iii1

IiI:I

-Male sex is also considered a risk factor because InCH develop coronary heart disease earlier than women (bnt postmenopansal women qnickly catch up with age-matched men). If you give a patient one risk fu:tor for being Inale, do not give hirn a second risk factor {-(.H' age (use one or the other in Ineo). Obesity is not an independent risk factor fe)f boards purposes. Stress, physical inactivity and type A personality (look fClT a hard.driving attorney) are controversial (presumed to be risk factors by some clinicians).

mHypertriglyceridenlia alone is not considered a risk factor, but when associated with high cholesterol causes nlore coronary heart disease than high cholesterol alone,

Note: Lipoprotein analysis involves measuring total cholesterol, HDL and triglycerides. I.DL can then be calculated from the formnla LDL = total cholesterol - HDL ~~ (triglycerides/ 5). Note: Always give new patients at least 3 1110nths to try lif:t~stylemodincations (decrease caIo-ries, cholesterol, and saturated fat iu diet; decrease alcohol and smoking; exercise) bef(lfe initi~ ating drug therapy. First-line agents are niacin (poorly tolerated but dICctive) and bile acid-binding resins (e.g., cholestyramine). HMG CoA-reductase inhibitors afC the 1110St effective drugs and are con5id-ered flrsdine agents by some, but for board purposes, they are used flrst only if the other two choices are not appropriate or if patient has extremely high cholesterol (> 300 mg/dl, which is a marker for fiunilial hyperchoJesttTolemia). Note: High HDL is protective against atherosclerosis and is increased by lnoderate alcohol consnmption (1·-2 drinks/day) but not high alcohol intake, exercise and estrogens. HDL is de~ creased by smoking, androgens, progesterone, and hypertriglyceridemia. Be aware of secondary causes of hyperlipidemia: uncontrolled diabetes mellitus, hypothy~ roidislll, urcluia, nephrotic syndrome, obstructive liver disease, excessive alcohol intake (increases triglycerides), and medications (oral contraceptives, glucocorticoids. thiazides, and beta blockers). Atherosclerosis is involved in about one-half of all deaths in the United States and one~third of deaths between ages 35 and 6S. Atherosclerosis is the Inost iInportant cause of penl1anent disability and accounts for m,ore hospital days than anyother illness (translation: understand atherosclerosis for the boords).

SMOKING Sll10king is the Single 1110St Significant source of preventable morbidity and premature death in

the United States. Whenever you are not sure which risk factor to eliminate, silloking is a safe guess.

Internal Medicine

7

Important points: 1. Smoking is the best risk factor to eliminate to prevent heart disease-related deaths (responsible for 30·45% of deaths due to coronary heart disease deaths). Risk decreases by SO% within 1 year cOlupared with continuing smokers and decreases to the level of pa-~ tients who never smoked in 15 years. 2. Smoking aJso increases risk f()r the f()lIowing cancers: lung (90% of cases). oral cavity, esophagus. larynx. pharynx. bladder (50% of cases), kidney, pancreatic, and cervical cancers. SlTlOking possibly increases stOluac:h cancer also.

3. Chronic obstructive puhnonary disease is often due to smoking. Emphysema. almost always is due to smoking (unless the patient is very young or has no smoking history. in which case you should consider alpha, antitrypsin deficiency). Although the changes of ernphysema are irreversible, risk of death still decreases after snloking ('cssatiol1. 4. When parents snloke, children at increased risk for asthrna and upper respiratory infections, including otitis luedia. 5. SIlloking retards healing of peptic ulcer disease, and cessation stops Buerger's disease (H..aynaud's symptOlus in a young cnale sIlloker). G. Sllloking by pregnant woman increases the risk of low birth weight, prelnaturity, spontaneous abortion, stillbirth. and infant lTlOrtality.

1. Smoking cessation preoperatively is the best way to decrease risk of postoperative pillmonary cOluplications. 8. Do not give birth control pills to WOlnen over 35 who sIlloke; do give women sH\()kers postmenopallsa] estrogen therapy

ALCOHOL Important points: 1. Alcohol increases the risk for the following cancers: oral, Jarynx, pharynx, esophagns. liver, and lung. It lIMy increase the risk f()f gastric, colon, pancreatic, and breast cancer. 2. Alcohol is the Illost conUTlon cause of cirrhosis and esophageal varices.

3. Alcohol is involved in roughly 50% of fatal car accidents, 67% of drownings and homi"" ddes, 70~80% of deaths in fires, and 35% of suicides.. 4. Always give thiamine before glucose in an alcohohc; if you give theIn in the reverse order,

you nlaY precipitate Wernicke's encephalopathy. Wernicke's vs. Korsakofrs syndromes. Wernicke's syndrome'" ophthalmopJegia, nystagmus. ataxia, and confusion; acute and often reversible; 111ay be fatal. Korsakoff's syndrome:::::: anten)grade amnesia and confabulation, chronk and irreversible. Both are due to thiamine deficiency. The most likely cause is darnage to the InalniUary bodies and thalacnic nuclei. Alcohol withdrawal can be. fatal. Treat on an inpatieut hasis. Use benzodiazepines (chlor· diazepoxide and other kmg·acting benzodiazepines) or, rarely. barhiwrates. Gradually taper the dose over days. Withdrawal stag("/symptoms. First comes acute withdrawal syndrome, 12~48 hours after last drink. Syrnptoms include tremors, sweating, hyperreflexia, and seizures (nUll fits). Next is ako"' holic hallucinosis, which consists ofhallucinat.ions (auditory/visual) and illusions without autonOInic SYlllptoms. Finally COllIes deJirimu tremens. which usually occurs 2--4 days after the last

8

Internal Medicine

drink and involves hallucinations and illusions plus confusion, poor sleep. and autonornic lability (sweating, increased pulse and temperature), which occasionally is fatal.Treat on an inpaticnt hasis.

Stigmata of chronic liver disease in alcoholics: varices, hernorrhoids, caput luedusae, jaundice, ascites, paln1af erythClna, spider angiomas, gynecOlnastia, testicular atrophy, encephalopathy, asterixis, prolonged prothr0111bin tilne, hyperbiliruhinemia, spontaneous bacterial peritonitis, hypoalbuminemia, and aneluia. Conditions commonly caused by alcohol include gastritis, Mallory~Weiss tears, pancreatitis (acute and chronic), periphcral neuropathy (via thiaminc deficiency), brain damage, and car~ diomyopathy (dilated). It also causes testicular atrophy, fatty change in tbe liver, hepatitis, cir~ rhosis, hepatocellular liver caucer, Wernickc/Korsakoff syndrome (via thiamine deficiency), cerebellar degeneration, and rhabdomyolysis (acute and chronic).

The best treatment for alcoholisrll is Alcoholics AnonynlOus or other support group. Disulfiram also may be tried (patients get sick when they drink because of alcobol dehydroge~ nase enzyme inhihition). Alcohol is a defInite teratogen. You should be able to recognize fetal alcohol syndrome: ruental retardation. micf()Ccphaly, rnicrophthal1uia, short palpebral fissures, ulidfacialhypoplasia, and cardiac defects. No alcohol is good akohol during pregnancy. An estimated 1 in 3000 births is affected by fetal alcohol syndrome, which is the 1110st common cause of preventable 111cntal retardation. Incidence. Alcohol abuse is Jnon~ COllunon in Ineu. Roughly 10-1 5 (Yo of people abuse alcohol. AlcohoJiS111 has a heritable component and is especially passed from fathers to sons. Important poiuts:

1. Skid-row alcoholics cOITnuonly develop aspiration pneumonia with weird bugs suelLas Klcb.liello species(currant ~jelly sputum) and enteric organisms (e.g., anaerobes, EschericlJio coli, streptococci, staphylococci). 2, Alcohol may precipitate hypoglycemia (but give thiamine first). 3, Alcoholics develop just ahout every type of vitamin and mineral deficiency; especially couuno]) afC deficiencies of £()late. luagncsitllll, and thiamine. 4, Bleeding varices are treated with stabilization (flUids, blood), then upper endoscopy and sclerotherapy with cauterization, banding. or vasopressin. The Tl10rtahty rate is high. and rcbleeding is common, especially early. Try transjugnlar intrahepatic portosystemic shunt (TIl'S) before portacaval sbnnting procedures (splenorenal is the most physiologic shunt type).

ACID/BASE STATUS You IUllSl know how to interpret: simple blood gases, when given pH, O'.~' CO 2 , and bicarbonate. Here are good basic hints: I, pH tells yOll whether you are dealing with acidosis or alkalosis.

2" Look at CO,.lfit is high, the patient has either respiratory acidosis (pH < 7.4) or is

com~

pensating f,,, metabolic alkalosis (pH> 7.4).II'CO, is low, the patient has either respira tory alkalosis (pH> 7.4) or is compensating f,)r metabolic acidosis (pH < 7.4). 3, Look at bicarhonate.lfit is high, the patient has either metabolic alkalosis (pH> 7.4) or is cOlnpensating {()[ respiratory acidosis (pH < 7.4-). If bicarbonate is low, the patient either has metabolic acidosis (pH < 7.4) or is compellsating f"r respiratory alkalosis (pH> 7.4).

Internal Medicine

!l

Clinical correlation: common causes of differeut primary disturbances 1. Respiratory acidosis: chronic obstructive pulrnonary disease, asthma, drugs (opioids, benzodiazepines. barbiturates, alcohol, and other respiratory depressants), chest wall problems (paralysis, pain), sleep apnea

2. Respiratory alkalosis: anxietyIhyperventilation, aspirin/saHcylal.e overdose 3. Metabolic acidosis: ethanol, diabetic ketoacidosis, uremia, lactic acidosis (sepsis/shock). lnethanollethylene glycol, aspirin/salicylate overdose, diarrhea, carbonic anhydrase inhibitors 4. Metabolic alkalosis: diuretics (except carbonic anhydrase inhibitors). vomiting, vohune contraction, antacid abuse/nlilk--alkali syndrorne, hyperaldosteronism

5. Salicylate/aspirin overdose causes two priInary disturbances (respiratory alkalosis and metabolic acidosis). Look flJr coexisting tinnitus and/orhypoglycerTlia, vOlniting, and history of IIswallowing several pills." Alkalinization of the urine (With hicarbonate) spet'ds excretion.

6. In certain patients wilb chronic lung disease, pH may be alkaline during the day (especially in pati(~nts with sleep apnea) because they breathe better when they are awake or have just recovered {forn an episode of bronchitis. The rnetabohc alkalosis that usually conlpensates for respiratory acidosis is no longer cornpensatory and bccollles the priulary disturbance (elevated pH and bicarbonate). 7. Sleep apnea, if severe, may cause right-sided heart failure (cor pulmonale). Treatment. Do not use bicarhonate to treat low pH. unless the pH is < 7.0 and other rneasures have failed (always try saline Hrst). Note: Beware the astilInatic whose blood gas goes from alkalotic to normal. The patient is prob-ably about to crash and needs intubation.

HYPONATREMIA Signs and symptoms ofhyponatreruia are confusion, lethargy, mental status changes, anorexia, seizures, disorientation, cramps, and cmna. The first step in detennining the caU5(:' of true hy~ ponatrernia is to look at the vohune status:

1. SyndrOlue of inappropriate secret:i<:m of antidiuretic hormone (SIADH) cOlTlrnonly results from head trauma or surgery, nleningitis, sInaU cell cancer of lung, postoperative or other painful states, puhnonary infections (pneumonia or tuberculosis), opioids, or chlor-propamide. Treatment is water restriction. Occasionally. when refractive to conservative management, SIADI-I is treated with clcnleclocycline (a tetracycline that caw;;es renal dia-hetes inSipidus).

2. With Addison's disease and hypoaldosteronisrn. potassium is elevated. 3. Hypovolemic hyponatrelnia shouhl he treated with saline. Euvolemic and hypervolemic hyponatrernia should be treated with free water restriction and pOSSibly diuretics for hypervolemia.

10

Internal Medicine 4. Never correct hyponatrexnia rapidly, because you 111ay cause brainstelIl dalnage (central pontine I11yeJinolysis). Hypertonic saline is used only when the patient has seizures due to hyponatremia and, even then, only briefly and cautiously. NOrIml! saline is (/ beuer choice 99 time,) out of ] 00 for board purposes. 5. Correct the sodiulYl when the patient has hyperglycernia (once glucose exceeds 200 mg/dl, sodinm decreases by 1.6 mEq/L for eacb increase of 100 mg/dl in glncose). Hyperlipidenlia and severe hyperproteinemia may cause a false (spurious) hyponatremia

by their OSIIlotic effect. 6. In a surgical patient, the most comrnon cause of hyponatremia is inappropriate or excessive fluid adIllinistration. 7. Oxytocin administration may cause hyponatrcrnia in pregnant wornen (oxytocin has an

ADHUke eff"ct).

HYPERNATREMIA The signs and sYlnptolns of hypernatrclnia and hyponatrelnia are silllilar: confusion, rnental status changes, hyperreflexia, seizures, and COlna. Common causes include dehydratioIl, inability to drink (paralysis, dementia), diuretics, diabetes insipidns (pitnitary or nephrogenic), diarrhea, renal disease, and iatrogenic administration of' excessive salt. Sickle cell disease also may cause hypernatrelnia due to kidney damage that irnpairs renal concentrating ability. lIypokaleulia and hypercalcemia also cause a sinlilax irnpairment in renal concentrating ability that may rrrhnic diabetes insipidus (DI). Treatment involves water replacenlent. Often the patient is so dehydrated that nonnal saline lnay be llsed at first until the patient is henlodynalnically stahle; then switch to one-half nonnal saline (O.4Y% NaCl). r"ive--percent dextrose in water should not be used

Pituitary vs. nephrogenic diabetes insipidus. Pituitary DI responds to vasopressin; nephrogenic])I does not. Nephrogenic ])I may be caused by medications (lithium, demee!ocyeline, methoxyflurane, and amphotericin B) and is treated wid, a thiazide diuretic (paradOXical effect). Central Dl Inay be caused by Sheehan's syndrOine (postpartmll hemorrhage causes shock aIId pi~ tuitary inf~J.xetion [apoplexy]); look f()r inability to breast feed and other endocrine deficiencies.

HYPOKALEMIA Hypokalemia causes uluscular weakness, including weakness of SI1100th muscles. The patient may have an ileus and/or hypotension. Mnseular weakness may lead to paralysis and ventila-

tory failure. Tbe most famous (and most tested) eH"ct of hypokalemia, however, is OIl the heart. EKG findings include loss of T wave, U waves, premature ventricular and atrial contractions and ventricular and atTial tachyarrhythrnias. Changes in pH Inay cause changes in senuu potassiurn (alkalOSis Clllses hypokalemia, acidosis causes hyperkalemia). For this reason, bicarbonate is given to severely hyperkaleruic pati~~nts. Nonnalization of deranged pH most likely will correct: the potasshllll derangernent autolnati cally (no need to give or restrict potassimn).

Important points: 1. The heart is particularly sensitive to htl)oka1elIlla whell tile patient: is taking digitalis. Potassitnn shcruld be watched (:arefully tn all patients taking digitalis, especially if they also takc diureUcs (a very COlnrnon occurrence).

Internal Medicine

11

2. Do nol replace potassium too qUickly! The best metbod of replacemeut is oral. but if potassiurn nlust be given IV. do not exceed 20 rnEqfhr. Monitor the EKG

if potasSiUITl

must be given qUickly 3. If hypomagnesculia is present, it is difficult to correct the hypokalerllia unless you also correct the hypomagneserlTia.

HYPERKALEMIA With hyperkalemia. especially if the patiem is asymptomatic and the liKG is normal. you should wonder whetber the lab specimen is hemolyzed, Hemolysis causes a false hyperkalemia, Repeat the test. Signs and. symptoms rnay include weakness or paralysis. but the lnosl irnportant (and most tested) effects are cardiac. EKG changes (in order of increasing potassium value) include tall. peaked T \II/aves; Widening of QRS; PH. interval prolongation; loss of P waves; and a sine wave

pattern. Arrhythnlias include asystole and ventricular H.brillation. ConlTIlOll causes of hyperkaleulia include renal failure (acute or chronic), severe tissue de~ struction. hypoaldostel'Onism (watch f()r hyporeninemic hypoaldosteronism in diabetes).

rnedications (potassiml1-Sparl.ng diuretics, beta blockers, nonsteroidal anti-.inflamrrlatory drugs, or ACE inhibitors). and adrenal insuf!lciency (associated with low sodium and low

blood pressure), Try stopping all implicated medications. The best method of therapy is oral (decreased intake. sodium polystyrene resin). If, however. potassium is very high (> 6.5) and/or cardiac toxicity is appareut (more than peakedT-waves). inunediate IV therapy is needed. First give calcimll gluconate, which is cardioprotective, even though it does not change potassium levels. Then give sodium bicarbonate (alkalosis causes potas-

sium to shift iuside cells) and glucose with insulin. which also f"rces potassium iuside cells, If the patient has renal failure or initial treatrnent is ineffective, prepare to institute dialysis elnergently.

HYPOCALCEMIA Hypocalcemia produces neurologic findings, the most tested of which is tetany. Tetany is evidenced by tapping on the facial nerve to elicit contraction of the facial muscles (Chvostek's Sign) or applying a tourniquet or blood pressure cuff and inflating it to elicit hand muscle (carpopedal) spasms (1i·ousseau's sign). Other symptoms are depreSSion. encephalopathy, dementia, laryngospasm. and convulsions. EKG shows QT interval prolongation. Cornmon causes: §

DiGeorge's syndrome (tetany shortly after birtb. absent thymiC shadow)

~

Renal failure (because of the kidney's role in vitamin D Iuetabolisrn)

• Hypoparathyroidism (watch f()r postthyroidectomy patients; all f(Hlr parathyroids Illay have been accidcntaUy removed) 'g!l

Vitalnin D deficiency

~

Pseudohypoparathyroidisrn (short Hngers, short: stature, Inental retardation, and nOfmal

levels of paratbyroid hormone [PHIl with end Ol'gan unresponsiveness to PHI) II:!

Acute pancreatitis

!llI

Renal tubular acidosis

12

Intornal Medicine

Important points: 1. Hypoproteinemia of any etiology rnay cause hypocalccrl1ia because the prolein- bound fiactlon of calchl1n will be decreased. In this instance, however, the patient is aSYlllpto-lll.1tic, llcGluse the ionized (unbound) fraction of calcium is unchanged. 2. Hypollugnesemia of any cause rnakes it diffJClllt to correct tllc hypocalcemia untiJ tIIC hy-porHaguesenlia is also corrected. 3. IZickcts and ostc()1nalacia arc the skeletal effecls of vitalnin D dcfkiency ill children and adults, respectively.

4. Alkalosis Inay cause syrnptOlT1S sirnilar to hypocalcemia because of effects on the ionized fraction of calcium. Treat by correcting the pI-I.

5. Phosphorus and calcillrn levels arc usually in opposite directions, and derangemcllts in one rnay cause prohleuJs with the otller, In renal fajlure, thcrdewe, you should not only raise calcilun but also restrict phosphorus and give POrbindin~~ agents, slrch as alu Illinum--hydroxidc,

HYPERCALCEMIA Hypercalcemia is usually asymptomatic and discovered by routine Jabs. When symptoms are present, rernernber "bones, stones, groans, and psychiatric overtones" (bone changes such as ostcopenia or pathologic fractures; kidney stones and polyur:ia; ahdoruinal pain, anorexia, constipation, ileus, nausea, vorniting; depression, psychosis, deliriulTI, and confUSion). EKe shows QT interval short.ening. Causes. l-lypercalcemia in outpatients is due most connIlonly to hyperparatllyroidisrn, In inpa~ tients, the Tllost COIIunon cause is rnalignancy. Other causes include vitamin A or D intoxica~ tion, sarcoidos:is, thiazide diuretics, fanrihal hypocalciuric hypercalcemia (look fbr low urinary calcium, which is rare with hypercalcernia), and inllHobiIization. Hyperproteineruia of any cause may cause hypercalcemia because of an increase in the protein--boul1d fraction of cal·durn, but the patient is asyrnptornatic because the ionized (unbound) fraction is unchanged.

Trcat:nlent. First, give IV f:1uids. Once the patient: is well-hydrated, give furosemide to cause

calcium diuresis (thiazides are contmindicnfn!). Other treatBlents include phosphorus adnlinistra·bon (oral; IV is rarely used because it is dangerous), calcitonin, diphosphonates (e.g., etidronate, ofi:en used in Paget's disease),plicarnycin, prcdnisollc (especially {e)l" rnaHgnancyinduced hypercalcenlia) , and gallimn nitrate, Note: Severe prolonged hypercalcemia Il1,q cause nephrocalcinosis and reJJa] fa.illlre fl'orn calCilUTl salt deposits in kidney.

OTHER ELECTROLYTE DISTURBANCES AND FLUID ADMINISTRATION Hypornagnesc.rnia is seen 1110St often in alcoholics. Signs and synlJ)torns, which irlclude EKG changes and tetany, arc similar to those of hypocalcernia. J--lypornagneseuliais notorious because

it rnakcshypokalunia aucl hypocalccrnia difIlcult to correct. Treat with oral replacement. Hyperrnagncsenlia ailnost always is iatr()~J,cnic ill pregnant patients treated fe)r preeclarnpsia (hut Illay also be seen ill paticIlts with renal hilurc), In patients treated with rnagnesiunl sulfate. check {()f decreased dccp-tend()lJ reflexes. hypotension, and respiratory depression.

Internal Medicine

13

'freatlncnt includes stopping ma~~llesilllll sulfate (flrsl step), supportive lreatment (intubate if necessary), IV hydration, furosemide, and dialysis as a last' resort.

Inlportant points: 1. Hypophosphatemia is seen primar.ily ill patients wilh diabetic ketoacidosis and alcoholics. Signs and symptoms are llcuronmscular disturhanct',s (encc-phah)pathy, wi..>akncss), rhabdOlny' olysis (especially in alcoholics) and dlH':IIlia with white blood cdl and platelet dysfunction. 2..Hyperphosphatemia is sC'ell almost always in paOCllts with renal failure. Treat with pIlus phatc restriction, dialys-js, and phosphatc--hinding resins (ahullillllIll hydroxile). 3. In traurHa patients, the fluid of choice is Ringer's lactate; the second choice is normal saline.

4. In hypovolemic patients, use norma! saline or Ringer l :, lactate, regardless of o~ heT elec'trolyte problems. 5. Maintenancc fluid ill NPO patients is uSllally S%) dexlrosl~ in onc:.half normal saline. In pediatric patients, use 5%l dextrose ill onc--f(Hlrlh or onc--third normal saline because of rCllal diffl::'fCnccs.

6. Add 20 mEq of potassimn chloride to each liter of maintenance fluid in an NPO patient (assulnillg absence ofpotassiull1 derangernents).

VITAMINS AND MINERALS

t~at,)ge

I:IYR-et:cak£n)~a;

nau'sea'--an:d 'V()l11itilig,.- r~:JlaI effect's,

Necrotizing _entrl'ocolitis(iIi.rants)

Elerriulysis-fl{ernlcte-rtls)

f!erij)htial-l1e:ql'OpathY. -(mlly B vitaruin wi(h

to:xidty)

~shap-ci_d

icity--also'u?ay cattsc -myxedema m6-ttling:.c')f I'eg:ih
Flu.orin\oZinc O~)Pf'1m~

fin

MenkelS :~_if,e_a$'(_~: (¥'~hnkf-d; _ki:i11ql halt: a'tld :n1e~lfaltttt;rfdaH0I1); "t.?Xicity ~_Wil$onj~'-~th;ea_se

Se]en'hil'n

C
Mahg-a~es~

~roxicity_e9ua.ls _ ~'jTIaJ)_gaIle,se

C)ir<)lB1Um

Impaired gtut:;ost'

toler~n_ce:

t~Xicity ;::I?Sf(of hair al1d nails OSSll ,in O',liners afore

14

Internal Medicine

Important points: 1. Deflciency of latsoluble vitamins (A, D, E, K) often is due to malabsorption (e.g., cystic fibrosis, cirrhosis, celiac disease, sprue, duodenal bypass, bile··-duct obstruction, pancreas insufficiency, chronic giardiasis). In such patients, parenteral supplements are required if

bigh-dose oral snpplements fail. 2. Alcoholics can have just about any deficiency, but check folate. thiarnillc, and 111agnesimll.

3. Vitaluin B 11. defIciency nlOst cOl1uIlonly is due to pernicious auunia, in which antipari, etal cell antibodies destroy the ability to secrete intrinsic factor. Conditions associated with pernicious anclIlia include hypothyroidisrn and vitiligo. Schilling's test is used to di-

agnose the cause of B" deflciency. Removal of the ileum and the tapeworm Diphyllobothtium latum also cause Ell. deficiency. 4. Isoniazid causes B6 (pyridoxine) defkiency~ Patients taking isoniazid (especially young

patients) are often given prophylactic B" snpplements. 5. Anticonvulsants (especially phenytoin) may cause folate defiCiency. 6. Vitamin A is teratogenic and any feruale patient given one of the vitamin A analogs as treatment fbr acne (c.g.. isotretinoin) must have a negative pregnancy test befbre medication is started and must be put on SOllie fC)fID of birth control as well as counseled about

the risks of teratogenicity if they become pregnant. Periodic pregnancy tests also should be olfered. 7. Rickets causes interesting physical flndings: craniotabes (poorly mineralized skull and bones that feel like a ping-pong ball), rachitie rosary (costochondral beading with small, round masses on anterior rib cage), delayed fontanelle closure. bossing of the skull, kyphoscoliosis, bowlegs, and knock-knees. Bone changes first appear at the lower ends of the radius and ulna. 8. Vitamin Kis given to all uewborns as prophylaxis against hemorrhagic disease of the newborn. Vitamin K is needed lor the synthesis of factors II, VII, IX, and X as well as proteins C and S. Chronie liver disease (cirrhosis) may cause prolongation of the prothrombin time (PI) because of inability to synthesize clotting factors. even in the presence of adequate vitaruin K. Treat with fresh frozen plasll1a; vitarnin K is ineffective.

SHOCK Definition of shock: a state in which blood flow to and perfusion of peripheral tissues is inad equate to sustain life. Although not included in a rigid definition of shock It)r board purposes, associated findings include hypotension and oliguria/anuria, Tachycardia is also usually present.

Pragmatically speaking, there are four clinical types of shock: 1. Hypovolemic 2. Cardiogenic 3. Septic 4. Neurogenic

Your job is to figure out why the patient is in shock while keeping him or her alive. Give fluids while you're thinking. If the patient doeSfl't respond to a fluid bolus and you are given the choice. use invasive helIlodynanlic monitoring (Swan--Ganz catheter) to help rnake diagnostic and therapeutic decisions:

Internal Medicine

15

Associated findings help to difkrentiate the etiology of shock: 1. Neurogenic shock: history of severe central nervous system trauma or bleed; flushed skin. I--leart rate may be nonnal. 2. Septic shock: fever, white blood cell count changes, skin fhlshed and wann to the touch, extren1eS of age. Use broad~·spectrum antibiotics after pan-culturing the patient (get blood, sputum and urine cultures plus others if history dictates). 3. Cardiogenic shock: history of lllyocardial infarction, chest pain, congestive heart failure, or several fisk factors fe)f coronary artery disease. The patient has cold, clalTIlny skin and looks pale. Distended neck veins, pulmonary congestion (on ('xalil and x-ray). Patients usually need diuretics··--.fluid rnaynlake them worse! 4. Hypovolernic:bistory of fluid loss (blood, diarrhea, vOIniting, sweating, diuretics, inar)ility to drink water). The paticnt has cold, clammy skin and looks pale. Flnid loss may be internal, as in a ruptured abdominal aortic aneurysm or spleen, pancreatitis, or after surgery. Other signs include orthostatic hypotension, tachycardia, sunken eyes, tenting of skin, and sunken f'llltanclle (in children).

5. AnaphylaXiS: look for bee stings, peanuts, shellfish, penicillins, sul£a.s, and other medica· tions. Treat with epinephrine and fluids, adnlinister 2 , intubate if necessary (do a tra· cheostOIny or cricothyroidotomy if laryngeal edenla prevents intubation). Antihistamines help only wIlen the reactloll is ruiId. Use corticosteroids when the reaction is prolonged or severe (not first·"line drugs f(w treatment of anaphylaxis). Monitor all patients for at least 6 hoursaner initial reaction.

°

6. Puhuonary embolus: look for risk factors for deep vein thrornbosis (Virchow's triad: endothelial damage, stasis, hypercoagulablc state), history of recent delivery (amniotic flniel embolus), fractures (fat emboli), deep vein thrornbosis (pOSitive HOlnan's sign with painful, swollen leg), and recent surgery (especially orthopedic or pelVic surgery). Patients have chest pain, tachypnea, shortness of breath. parasternal heave, right"axis shift on EKG, and positive VIQ scan, Heparinize to prevent further clotting and emboli. 7. Pericardial talTlponade: history of stab wound in left chest, distended neck veins. Do peri·cardioccntesis emcrgently. 8. Toxic shock syndrOlne: classic patient is woman of reproductive age who leaves tampons in place too long. Look fc)r skin desCjuaInation. Caused by St(Jphylomccus aurCl1S toxin. Note: ABCs (airway, breathing, circulation) come fIrst. Patients in shock often need heroic lueasures to survive. Intubate at the drop of a hat, and keep NPO, avoid narcotics if pOSSible (mental status changes arc often an important clue to IJnpcnding doom). Monitor EKG-, vital signs, Swan -Ganz paraulcters, urine output, arterial blood gascs (ABGs), hemoglohin, and henlatocrit.

16

Internal Medicine

Note: Most patients in shock need fluid. The standard bolus is 10--20 ml/kg of normal saline (roughly 1-2 L infused as fast as it will go). After the bolus, reassess the patient to determine whether the bolns helped. Do not be afraid to bolus twice if the first bolus has no effect. Of course, you nmst watch for fluid overload, which lllay cause congestive heart failure (especially in cardiogenic shock when the patient is already in failure). IV medications and their use to support blood pressure should be understood: 1, Dobutanline: betal agonist used to increase cardiac output by increasing contractility (leU equivalent of digoxin). 2. Dopamine: low doses hit doparnine receptors in renal vasculature and keep kidney per-· fi.lsed. Higher doses have beta] agonist effects to increase contractility. Highest doses have alpha I agonist effects and cause vasoconstrictioIl. 3. Norepinephrine: used f()f its alpha j agonist eH{~cts; given in hypotension to increase peripheral resistance so that: perfusion to vital organs can he :maintained. Also has beta agonist

eff(~cts.

4. Phenylephrine: used for its alpha] agonist effects. 5. Epiuephrine: used for cardiac arrest and anaphylaxis. 6. Milrinone/arnrinone: phosphodiesterase inhibitors used in refractory heart failure (not

first-line agents) because they have a positive inotropic effect.

Note: Relnemher Addison's disease as a cause of shock, especially in a postoperative patient who has taken steroids in the past year and received no extra steroids perioperatively. Give patient steroids! For shock in the setting oftraurna, see tramna section (Surgery).

CHEST PAIN AND MYOCARDIAl.. INfARCT When a patient presents with chest pain, your job is to lnake SUfe that the cause is not lik·threatening, which usually meallS that you try to Blake sure that the patient has not had a Illy-acardia! infarction (MI).

Findings tbat make MI unlikely: 1. Wrong age: in the absence of known heart disease, strong tinnily history, or risk factors I()f coronary artery disease (CAD), a patient uuder the age of 4-0 is extremely nnlikely to have had an MI. 2. Risk factors: a 50.-year-old lnarathon ruuner who cats well and has a high HDI. without other risk factors for coronary heart disease is unlikely to have had an MI. 3. l)hyskal characteristics of pain: if the pain is reproducible by palpation, its source is the chest wall, not an MI. Pain should not be sharp aud well~localized or related to certain I(}ods.

Findings tbat elevate suspicion of MI: 1, EKG: after an MI, you should see flipped or flattenedT waves, ST segment elevation, and/or

Q waves in a segmental distribution (e.g., leads II, IIJ, and aVF for an inferior infarct). 2. Pain characteristics: usually dcscr.ibed as crushing. poorly localized substernal pain that rnay radiate to the shoulder, ann, or jaw; not reprodUcible on palpation. Pain usually does not resolve with nitroglycerin (as it often does in angina). Pain usually lasts at least a half~h(lur.

3. Laboratory values: a patient with a possible MI should have serial detcrrninations of creatine kinase (the MIl isoenzyme) or troponin Ill' (usually drawn every 8 hours time's 3 bel()re MI is ruled OlIt). I.actate dehydrogenase (LDB) elevation and flip (LDB, > LDH,) also may be used, especially if the patient presents after 24 hours. Aspartate aminotransJ.l~rase is also elevated hut not used clinically f(>[ MI. X~ray may show cardiomegaly and/or pulmonary congestion; echocardiography may show ventricular walllllotioll abnonnalities.

4. Physical exaIn: puhnonary rales in the ahsence of other pncumonia--like syJnptOlllS, dis-tended neck veins, 53 or 54, flew lTmrmurs, hypotension, and/or shock should rnake you think along the lines' of an Ml. Patients are often diapboretic, tachycardic, and pale; nausea and vomiting Inay he present.

111

Cardiovascular Medicine

5. History: patients with MI often have a history of angina or previous chest pain, mur· Iuurs, arrhythluias, or risk factors for CAD. SOlIle are taking heart ITledications (digoxin, furoscrnicle, antihypertensives, cholesterol ruedications).

e

Treatment for an MI involves hospital adIuissl0Il to the intensive care or cardiac care unit: with adherence to several basic p6nciples: 1. Early thrombolysis (usually less than 6 hr after pain ouset) if the patient meets strict cri· teria f()f use; PTCA (percutaneous transhllninal coronary angioplasty) may be used if thrombolysis contraindicated,

2. EKG monitoring: if ventricular tachycardia develops. usc lidocaine (do not usc prophylactically). 3. Give OJ. by nasal cannula (maintain O 2 saturation> 90(%). 4. Pain control with lllorphine (which lllay help with puhnonary edema if present) 5. Nitroglycerin

6. Beta blocker (on which patient should remain f()r life if ItO contraindications: are preseIlt; proven to reduce inciderlce of second MI)

7. Aspirin (and possibly low· dose heparin) 8. Soft diet or NPO and stool sofieners

9. Begin anticoagulation with IV heparin in patients with cardiac thrornhus. large area of dyskinetic ventricle, or severe congestive heart failure (CHF). 10. Patients with CHF (ejection £i-action < +0%) should he started on an angiotensin--c(H1··

verting enzyme inhibitor. which has been shown to reduce ulOrtahty in this setting. Note: Rcrncrnber that the patient can reinfarct on the saIne hospital visit, even with adequate llledical management.

Other causes of chest paiu aud dues to diagnosis: 1. Gastroesophageal reflux disease and peptiC lIker disease: relation to certain {c>ods (spicy. chocolate). sl1lOking. caffeine, lying down; relieved by antacids or aCid.-reducing rned~ ications; positive for Helicobacter pylori (peptiC ulcer disease only).

2. Stable angina: pain begins with exertion or stress and rClnits with rcst or caln1ing down; relieved by nitroglycerin. EKG shows ST segment depression with pain. then reverts to normal when pain stops; pain lasts less than 20 nlinutes 3. Chest wall pain (costochondritis, bruised or broken ribs): reprodUcible on palpation and well localized. 4. Esophageal prohlems (achalasia. nutcracker or esophageal spasm): difficult diftercntiaL Question will probably mention a negative work-lIp f()f MI; look fc)Y barium swallow

(achalasia) or esophageal l11allOmetry abnonnahties, Treat achalasia with pneumatic dilatation; treat nutcracker/esophageal spasrn with calcium channel blockers. 111cn my otomy if calciulTl channel blockers are ineffective

5. Pericarditis: look f()[ viral upper respiratory inf(x:tion prodrOlne. EKG shows diffuse 51' segnlent elevation. Other signs include elevated erythrocyte sedirnentation ratc and lowgrade fever. The most COlnmon cause is viral (coxsackie Virus); others include tu.berculo·sis. urcrnia, rualignancy. and lupus or other au.toinUDllne diseases. 6. Pnelllnonia: chest pain due to pleuritis. Patients also have cough, production. with possible sick contacts.

f(~VCT.

and/or sPUtUIl1

Cardiovascular Medicine

19

Unstable angina usually presents with nonnal cardiac enzyrnes and EKG changes (ST depression) with prolonged chest: pain that does not respond to nilroglycerirl initially (hke MI). Pain often begins at rest. Treat like an MI, hut use IV heparin to anticoagulate and consider P'I'CA emergently if pain does not resolve. Alnl0st all patients have a history of stable angina and CAD risk factors. In strict terms, unstable angiJ1J is defIned as a change frorn previous stable angina; thus, if a patient who used to get angina once a week now gets it ()[lee a day, he or she has unstable angina. Variant (PriuZJuetars) angina is rare and associated with anginal pain at: rest with ST elevation (cardiac cnzylnes, however, are nOflTlal). The cause is coronary artery 5pas1n. Variant angina re-sponds to nitroglycerin; long-term treatnlcnt usually is with calcium channd blockers. Note: 25% of MIs are silent, rneaning that they present without chest pain (especially in diabetics, who have, neuropathy). Such patients present: with CHF, shock, or confusion/deliriurll (especially elderly patients).

VALVULAR HEART DISEASE

Note: Undersranding rhe pathophysiologic changes associated with longstanding valvular disease has a high yield (e.g., do you understand why Initral stenosis or regurgitation can cause right heart failure?). (See figures, top of next page.) Use endocarditis prophylaxis J'Jr people with known valvlllar heart disease (with mitral valve prolapse, use prophylaxis only if a IIlUflIlur is heard on phys:ical exam or if the patient has history of endocarditis) or prosthetic valves. For oral surgery, use amoxicillin bef()f(' and aHer the procedure (use erythrOIl1ycin in penjcillin--allergic patients). For gastrointestinal or genitouri-nary procedures, use aJ11picillin plus gentan1icill before and alnoxicHlin after procedure (sub~ stitute vanco:mycin fc)r penicillin--allergic patients).

DEEP VEIN THROMBOSIS, PULMONARY EMBOLISM, ANTICOAGULATION Important points: 1. Remember Virchow's triad (endorhelial damage, stasis, and hypercoagulahle state) as a cine to the the diagnosis of deep vein rhrombosis (DVT). 2.

causes or situations in which DVI occurs: surgc-:ry (especially orthopedic, pelvic, or abdoIUirtal), neoplasrns, trauma, in:unobilizaU_ol1, I)fegnancy, oral contraceptives, disseminated intravascular coagulation, lupus anticoagulant, and deHciency ()f antithrOlubin Ill, protein C, or protein S. COIlnI1011

20

Cardiovascular Medicine

v

V

Mitral stenosis: The mitral valve orifaceis narrowed, resulting in obstruction to flow out of the atrium and an increase in pressure in the left atrium and pulmonary veins. Pulmonary hypertension develops secondarily. A '" A wave, V

0:::

V wave, M :;: mean pressure. (From

James Ee, Gorry RJ, Perry JF: Principles of Basic Surgical Practico. Philadelphia, Hanley & Belfus, 1987, with permission.)

Aortic stenosis: The narrowed aortic valve results in high pressures in the left ventricle, which are transmitted to the left atrium and ultimately resulting in pulmonary hypertension. The left ventriCle is hypertrophied due to the chronic pressure overload. (From James Ee, Corry RJ, Perry JF: Principles of Basic Surgical Practic!~. Philadelphia, Hanley & Batfus, 1987, with permission.)

3. DVTs cOlnmonly present with unilateral leg swelling, pain or tenderness, and/ Or I-ioInan's sign (present in 30%).

II. The best way to diagnose DVT is doppler ultrasound or impedance plethysmography. The gold standard is venography, but it is invasive and usually reserved flJr settings in which the diagnosis is not clear. 5. Superficial thrombophlehitis (erytherna, tenderness, cderna, and palpahle dot in a super~ flcial vein) is not a risk: factor fe)}' pulmonary clnbolislTl (PH) and generally is considered a benign condition. Treat with NSAIDs or aspirin.

G. In patients with DVT, systelnic anticoagulation is necessary. Use IV heparin, f()llowed by gradual crossover to oral warfarin. Patients arc maintained on warfarin f(x at least 3 1110nths, possibly pennanently if they experh~ncc Illore than one episode.

7. The best DVT prophylaxis for surgery is pneumatic compression boots and early amimia· lion; use low-·dose heparin if anlblliation is 110t possible. Warfarin is an alternative, espe-cially jar orthopedic hip or knee surgery. 8. Pulmonary em bolLrs j()]]ows DVT, deli very (amniotic fluid embolm), or fbctures (fat emboli). Symptoms include tachypnea, dyspne", chest paiu, hemoptysis (iflung infarct), and hypotension, syncope, and death if severe. Rarely, on a chest x--ray you lnay sec a wedge-shaped defect due to a pulmonary infaxct. 9. Left--sided heart clots (fronl atrial HbrHlation, ventricular wall aneurysm, severe conges~ Live heart failure, or endocarditis) that elnbo!ize cause arterial--sided inbrcts (stroke and renal, Gr, and extremity infarcts), not PEs. Right sided clots that embohze (DVTs) cause PJis, nO( arterial emboli. The exception is a patent fI:Harnen ovaJc, in which the dOl: ITJay cross over to the left side of the circulation and cause an arterial infarct.

Cardiovascular Medicine

21

10. Use V /Q scan to screeIl fC)f PE. If positive, PE is diagnosed and treated. If indet.enninate,

usc pulmonary angiogram (the gold standard, but invasive). If low probability or negative, il is highly unlikely that the patient: has a signHkant PH. 11. Treat

Pl~

with IV heparin to prevent further clots and elnboli; then gradually switch to

oral warfarin. on which the patient wm remain feJr at least 3 months. If clots recur on anticoagulation or the patient bas contraindications to anticoagulat.ion, use inft'rior vena

cava Gltcr (Grcenlleld flItcr). 12. Heparin causes thrOJnbocytopcnia and arterial thrornhosis

ill SOllH'.

unlucky patien\s.

Disc<mtillllc heparin IHunediately!

13. Heparin is (l!lowed hy determination olpartial thromboplastin time (PTT)(illternal pathway), and warfarin is ()!lowed by prothromhin time (PT) (external p;uhway), whereas aspirin aHt~cts the hleeding titue. In emergencies, TeVerSI(', heparin with prot
DIS~A.Sl!

Pl\OLON(J~D'!'EST

l"l'T P1'T

Vlt4Ynh:i: K: i1d\;(;lertcy

Note: Uremia causes a qualitative platelet defect. Vitamin C deflc:icncy and chronic cortico steroid therapy may cause a bleeding tendency with nonnal coagulation tests. Mid;

CONGESTIVE HEART FAILURE AND ARRHYTHMIAS Symptoms and signs of CHF !ill

Fatigue

m Dyspnea

mOnhopnea (seen ill left ventricular failure; patient sleeps on more than one pillow) 1m

Paroxysmal nocturnal dyspnea (left ventricular failure)

Iij

Peripheral edema (right ventricnlar failure)

/ill

Jugular venous distention (rig-hI: ventricular failure)

flil

Peripheral edema (right ventricular failure)

00

S3 IS+

mVuhnonary congestion (ndes; seen in left vClltTicular failure)

22

Cardiovascular Medicine

~~~~~~~~.~~~

~

Hepatomegaly/ascites (right ventricular failure)

~

Chest xray abnormalities (cardiomegaly rseen in left or right ventricular failurel, Kerley B hues, puJrnonary vascular congestion, and bilateral pleural effusions are seen in left ventrk:ular failure)

Treatment: sodium restriction, angiotcllsin--collvcrting l~nzyrnc inhibitor (Cirst--line agents; proved to reduce mortality in CHF). bela blockers (also reduce mortality), diuretics, dif~oxil1 (not in hypertrophic ohstructive cardiornyopathy or atrioventricular conduction blocks; usnally re· served f(>r moderatt>Lo--sr'vere Cl IF with low ejection fraction), vasodilators (arteria] and venous). and IV synlpathomimetics (dobul<:unillc, dopailline. amrinone) f()f inpatients with severe eHE

Important points: ,. Many faxtors can precipitate cxaccrbatjoIl of CHF in a previolls1y stable cardiac patiellt. Noncompliance, Inyocardial inf~llTHon, hy))Crlcns.i01I, arrbytlHnias, inkCliolls/ft:vcr, pu]" !llonary cmhol-ism, anemia, thyrotoxicosis, and myociUditis afe cornTl1()ll causes. 2. Cor pulmonale ls right ventricular enlargement, hypertrophy, or failure due to primary lung disease. COl1nIlOl1 causes are chronic ohstructive pulmonary db ease and puhnonary embolisll1. In a young WOlllan (20-4-0) with no other medical history o[ risk factors, think of primary pulmonary hypertension, and treat with calciurn channel blockers while awaiting heart-lung transplant. Sleep apnea also may cause cor pulmonale (an obese snorer who is sleepy during the day). 3. Patients with cor pllbnonale have tachypnea, cyanosis, c111hhing, parasternal heave, loud P2, and right-sided S4 in addition to signs and symptoms of pulmonary disease. 4. Restrictive cardiOluyopathy usually results frOIll amyloidosis, sarcoidosis, helnochrOIuato~ sis or Inyocardia] flbroelastosis (ventricular biopsy abnormal in all of these <.:onditions). On the other hand, constrictive pericarditis can be simply treated by renloving the pericanliull1 (pericardial knock, calcifkation of pericardium, normal ventricular biopsy).

!i Dilated cardioIIlyopathy comnHmly is due to alcohol, lnyocarditis,

Of

doxorubicin.

Cardiovascular Medicine

23

Important points: 1. Sinus tachycardia and atrial fibrillation are cornrnon presentations check level of thyroid-stirnulating hormone.

f()f

hyperthyroidism;

2. Wolff-Parkinson-White syndrome cOllllllonly presents in childh.ood. The patient heeolnes dizzy or dyspneic or passes out aiter playing, then recovers and has no other sYlllptorns (transient arrhythIllias via accessory pathway). Look f()r the infarllous delta wave.

PEDIATRIC CARDIOLOGY

Note: Endocarditis prophylaxis is reqUired for aU of these cardiac ddh:ts except aSYlnptomatic atrial septal dekel. Important points: 1. A heart rate over 100 beatshnin IndY he nOHllal in children.

2. In the presence of a ventricnlar septal defect. think about the pOSSibility of f"ta! alcohol syndrorllc, TORCII syndrorne, or Down syndrmne. 3. Hypertropllic obstructive cardiomyopathy classically presents in a young male who passes out on exertion (watch {()f collapse Of sudden death in an alhle1e) and oft-cn is associated with a family history of sudden death. This disorder is considered a diastolic dysfunction and thus is treated with beta blockf~rs to give the heart rnore time to flll. Positive inotropic agents (e.g., digoxin), diuretics, and vasodilators are contmindicated, because they make the condition worse. 4. Oxygen content in the {(~tal circulation is highest in the lunbilical vein and lowest in the lUllbilical arteries; oxygen conterlt is higher in blood going to upper extrelnities than in blood going to lower cxtrernities. 5. Understand the changes in the circulation froln intra-· \() extrauterine life. First breaths in·Hate the lungs and cause decreased pulrnonary vascular resistance, which increases blood flow to the pulmonary arteries. This and the clamping of the cord increase left·~sidedheart pressures, whicJl functionally close the fora.men ovale. Increased oxygen concentration shuts ofr prostaglandin production in the ductus arteriosus, causing gradual closure.

CHAPTER

3

Imonology

Chronic obstructive pulmOlury disease (COPD) means that tht' FEY I Il'IiV ratio is Jess thall normal (0.7 5·A).30, usnally give)) value), whereas in restrictive lung disease, the FJ.:Y I IFFV ratio is often normal. FreV I may he equal in both conditioJls; it is lilt' FFVj/FEV ratio that is d; fltTe]] I. Ernphysenla alInost always is due to slunki ng (even jf sccoud--hand). In a young person whh rninimal snloke exposure « S years of silloking), think of alpha J antitrypsin ddkicJlcy. Astlnna: look f()f wheezing in children. Treat with beta;. agonists in the emergency departlnent. Use steroids if asthrna is severe, or docs not respond to beta] agonists. Cro!l1o}yn is !()f

prophylaxis, not acute attacks. The new lcukotricne inhibitors (zafJrlukast, l.iJenton) afC used regularly, not usually for acute attacks. Phosphodiesterase inhibitors (thcophyllinf, an1inophyJlinc) are older, second,~lillc agents. Note: Do not put patients with asthma or COPD

OIl

beta blockers, which block the beta) rccep--

tors needed to open the airways. WheeZing in ('hildrell under age 1. is OfiCll due to respiratory syncytial virus, especially ill the winter. Look f()l' coexisUllg f(~v('r. Important points: 1. Beware the asthmatic who no longer hyperventilates or whose COJ. is lloJ:rnal or rising (the patient should hyperventilate, which causes low CO).). Do not think that patients who seem calm or sleepy arc okay. They are probably crashing and need an .inunediatc arterial blood gas analysis and possible intubation. Fatigue alone is enough reason to intuhate an astlm1atic. 2. A patiellt with COPD may llOl'mally Jive at a higher CO! and lower 0,; treat thc patient, not tbe lab value. If the patient is asymptomatic and ta.lking to you, the lab value should not make you panic. 3. As a rough rule ofrhmnh. yOll should prepare to intuhate any patient whose- CO 2 is:> SO lllml'lg or whose OJ. js < SO mmHg, especially if the pH in either case- is < 7.30 vv-hile the patient is breathing room air. UsuaUy, unJcss the patient is crashing rapidly, a trial of OJ. by Basal canllula is given Hrst:. If this approach docs not work or if the patiellt bCCOllleS too tired (use of accessory muscles is a good clue to the work of breathing), intubate. With a solitary puh.uonary nodule, tll(' Hrst step is t() check f()r old HlfllS. )f tile lesion has 110t changed in more than J year, it is likely to be henign. Cen;tin clues point to ttw etiology:

25

26

Pulmonology

IiiI

Immigrant: think tuberculosis (do a skin test).

D S011tl1WCSt

U.S.: think Coccidioides immitis.

mCave explorer, exposure to bird droppings, hisLoplaslllOsis.

O[

Ohio/Mississi ppj River va]]eys: think

II

Sn1()ker over the age of 50: drink lung cancer (hronchoscopy and hiopsy).

II

Person under 4-0 with none of the above: think hamartoma,

A baseline chest x.-ray is standard preoperative evaluation {(Jf patients over 60 and patients with known puhrlonary or cardiovascular disease, but when to order pulnl0nary function tests is not as clear. Overall, the best indicator of possible postoperative puhnonary complications is preoperative pulnlonary function. Overall, the best way to reduce pulmonary postoperative cOJllplicatiollS is to stop smokio9 preoperatively. Aggressive pulmonary toilcting, incentive spironwtry, minimal narcotics, and early arnbulatlOIl help to prevent or minimize postoperative pulmonary c(HnpHcation.

Note: The most common cause ofa postoperative it'vel' in the nrst 24 hours is atelectasis. Adult respiratory distress syndrome (ARDS): aClite lung injmy that results iu uoncardiogenic pulmonary edema, respiratory distress, and hypoxculia. COIlllllon causes aTt' sepsis, Inajor tl'auIna, pancreatitis, shock, Hcar drowning, and drug overdose. Look for ARDS to develop within 24--·A8 hr of the initial insult. Classic symptoms include mottled/cyanotic skin, intercostal retractions, rales/rhonchi, and llO irrll)rovement of hypoxia with O 2 aclministrati()JI. X-ray shows pulmonary edema with l10rnlal cardiac silhouette (no congestive heart falJure). Treat with inllthatioll, rnechanical ventilation with high peru:'tll..1.ge of 0) and positive encl-·cxpira-. tory pressure (PEEP).

The diagnosis of pneumonia usually is based on clinical findings plus elevated white blood cell count and chest x--ray abnormalities. On physical cxatn, Jook to differentiate between typical (St(lphyJococcus pneumoniae) and atypical (other bugs) pneumonia, although the distinction is not always c1car--cut: TYPKAL PNEUMONIA

Shor' « 7. days)

Long (> 3 aaY$)""~headache, maLtise, other ach~s

High (> 107" F)

Low«

Age

> 40 yr

< 40yr

Clu::ist x":'ray

One dlstiuct lobe -inv01ve:d

Diffuse 'or mnltilobe- jiIl[olvetn.ent

lnfectiv(agent

S:. -pileuillOliiae

Miny (e.R, Hocmophila\ inll"nwe. Mywplosma sp.. Chlamydia sp.)

MedicatiOJls*

P(~nidHhi,

Erytn:romydi1

Prodrome

thii'd-gencration .cepha10sporin

Certain dinkal clues should make I!1l

I!1l

yOll

102'1')

think of certain bugs:

College stude]]!: think Mycoplasma sp. (look fbr cold a~~gllltillillS) Or Chlarnydi(l sp. Alcoholic: think Klebsiella sp. (currant jelly sputum), Staphylococcus (aspiration).

f@ Cystic nbrosis: think Pseudomonas sp. or S. (mrell.\;. f@Tmlnigrallt: tl.link tulwrl'ulosis. Il'!l

COPD: think H. jnfJueJlZtlc, Momxclla sp.

(lllrCUS,

o! her elltcric hugs

Pulmonology

27

IS

Patient with known tuberculosis and pulmonary cavitation: think A'lpergiUus sp.

Ii!I

Patient with silicosis (rnetal, granite. pottery workers): think tuberculosis.

II!I

Exposure to air conditioner/aerosolized water: think LegioncJ1a sp.

llliI

HIV / AIDS: think Pneumocystis cmil1ii or cytmnegalovirus (if shown a picture of koilocytosis).

llii

Exposure to bird droppings: tltlIIk Clllumydj(l psitt<Jci or HistoplasmCl sp.

• Child < I yr: think respiratory syncytial vir"'. • Child 2···5 yr: think parainllnenza (croup) or epiglottitis. Recurrent prlcuIllonia in a child, H'it always occurs in the, right middle and/or right lower lobe, is most likely due to foreign body aspiration (a foreign hody is most likely to go down the right bronchus). It should be a consideration especially if the patient has no other signs of irnmunodeflciency (e.g., other types of in{-(~ctions, cystic nhrosis sytnptolns) before or during the episodf~s. Sinusitis: usually S. pncumoniac or I--IacUlopl1i]us sp. Lookf()r purulent (green/yellow) nasal dis" charge with tenderness over the involved sinus. Associated symptolns are headache and/or toothache (maxillary sinusiLis).Yoll cannot transillurninatc the sinuses, and an x-Tay or CT scan shows opacifIcation of the [remla] or rnaxillary sinuses (order a sinus x-ray to confJrm the diag" nosis if it has not already been done). Treatment is with ])('nicillill/amoxicillin or erythromycin for 10-14 days. Note: The InOS! connnon CJuse of epistaxis in children is nose-picking (do not aSSurne low or defective platelets without evidence. Respiratory distress syndrome, due to atelectasis frolH deficiency cl surfactant, ahnost always occurs bl premature infants and infa_Ilts of diabetic lllOthers. Look f()r rapid, labored respirations, substernal retractions, cyanosis, grunting, and/or nasal flaring. Arterial blood gases show hypoxclnia and hypcrcarhia, whereas x···ray shows diffuse atelectasis. Treat with 0z, give surfactant, and intubate ifnce_dcd (often). Complications include intraventricular hClnorrhage and pnelunoth-orax/bronchopulmonary dysplasia (acute or chronic mechanical ventilation complications). Diaphragluatic hernia cOlIllTlonly causes respiratory problems because bowel herniated into the chest pushes on developing lung and causes lung hypoplaSia on the aff(~cted side. Look f()[ scaphOid abdonlCll and bowel sounds in the chest (herniated bowel also may be seen on the chest x-ray; 90% are lef\.sided). Note: Look f()r rneconium aspiration if the infant is covered with mcconiU1l1 when delivered. Suction the nose first (bulb suction); then suction the oropharynx under direct visualization. Intubate if necessary.

The most conllnon type (85%) of tracheoesophageal fistula is all esophagus with a blind pouch proxiJnaJIy and a fistula between a bronchus or the carina and the distal esophagus. I.()ok f()f a neonate w'itll excessive oral secretions, coughing and cyanosis with attelnpted f"t:cd-ings, ~bdOlninal distention, and aspiration pneurnonia. Diagnosis is 111ade by inability to pass a nasogastric tuhe; contrast x-ray shows the proxilnal esophagus only. Treatment is early surgical correction. Cystic fibrosis: autosornal recessive inheritance; the rnost cOll11nonlethal genetic disease in white children. Always suspect it: in chiklrcn with rectal prolapse, rncconiurn Heus, esopllageal varices, a "salty" taste, recurrent pulJl10nary inf\:x:tions, and/or failure to thrive. Diagnosis is made by an abnormal increase in the electrolytes of the patient's sweat (sodimn and dllc)ridc).

211

Pulmonology Patients also havf~ pancreatic insufficiency (give pancreatic enzyme replacements and fat--solu~ ble vitamin supplements) aud infertility (98% of affected males and many females) and may develop cor puhnonale (right heart fajlure). Look for S. (lUreUS and Pseudomonas sp. as the causes of the THallY respiratory inf{~ctions.'Treat with chest physical therapy, annual influenza vaccine, fat-soluble vitarnin sllpplements, pancreatic enzyme replacclnent:, JIld aggressive treatnlent of inf(:cUons with antibiotics. Pleural effusion: if ycm do not know the cause, always consider thoracentesis and examine the fJuid with GraIn stain, culture and sensitivity (including tuberculosis culture), cell count with dint'rential, glucose (low in infl~ction), and protein (high in infl'ction).

Gastroesophageal reflux disease (GERD) is due to inappropriate, int.t'nnittent lower esophageal sphincter (LES) relaxation. The i.ncidence is greatly incrl;:',
be repaired sllrgically.

~~l'" "~

GE junction __ ~_,'

(~Sl'd'-~h

~_

.

, ling erma

C(""-"--~

GE junction

Normal

Hiatal hernias. In the sliding type, the gastroesophageal jUrH:tion slides frnoly above and below the {ji.. aphragmatir. hiatlls. In Il p<1raesophagelll hernia, the gastl'Oosophageal junction is fixed below tho diaphragm, allowinn part of tllO stomach to herniate into the chost. (From Crapo JO, Hamilton MA, Edgman S: Medicine & Pediatrics. Philadelphia, Hanley & Belfus, 1988, with !wrmission.)

((:::Pomesophogeol hernia

29

30

Gastroenterology Peptic ulcer disease presents with chronic, intermittent, epigastric pain--burning. gnawing, or aching~-~that is localized and often relieved by antacids or milk. Look for epigastric tender~ ness. Patients may have occult blood in stool and nausea or vomiting. Peptic ulcer disease is 1110re comrnon in males. The two types are gastric and duodenal.

Important points: 1. Endoscopy is becOIning the fIrst-line diagnostic study (upper GI bariurn study is classically clone £lIst) and is more sensitive (but Inore expensive) than x-ray.

2. Always biopsy any gastric ulcer to exclude malignancy (duodenal ulcers do not have to be biopsied initially). 3. The rnajor cOluplication is perfc)fation. Look for peritoneal signs, history of peptic ulcer disease, or free-air on abdOIuinal x-ray. Treat with antibiotics and laparotOIIly with repair of perfoTation: .. If ulcers are severe, atypical, or nonhealing. think about gastrin level) or stomach cancer.

Zollinger~Ellison syndrome

(get

.. Dkt changes are not thought to help heal ulcers (but reduced alcohol or tobacco use may help). !II

Start treatnlent with antacids, H2blockers or proton-purnp inhibitors, as well as antibiotics to elinlinate H. pylori. Triple therapy (ampicillin or aUloxicillin, rnetronidazole, and hisllluth) is the gold standard, but many regimens are in use.

4. Surgical options should be considered after f~\ilure of rnedical treatment or in patients with cornplications (perfc)ration, bleeding). COIlUll011 procedures include antrectoluy, vago-tomy, and HiIlroth 1and 11. Aftel' surgery (especially Billroth procedures) watch for dnrnp~ ing syndrome (weakness, dizziness, sweating, nausea Of v0J11iting after eating). Patients also may develop hypoglycemia 2~~3 hr aher the meal, which causes the same symptoms to recur; aH(~rent loop syndrOIllc (bilious vmniting after a meal relieves abdOlllinal pain), bacterial overgrowth, and vitalnin deficiencies (H n and/or iron, causing anclIlia). 5. Achlorhydria, the absence of hydrogen chloride, is associated with pernicious anemia (anti parietal cell antibodies destroy parietal cells and thus cause achlorhydria and B12 defiCiency). Upper versus lower gastrointestin.al bleeding (see table, top of next page). Important points: 1. The flrst step is to make sure that the patient is stable (ABCs. IV fluids and blood if flCUlryl); then get a djagnosis. 2. l-Indoscopy is 11sually the nrst test perf()nned (upper or lower, depending on synlptoms). ('lassicaIl}:bariulll x,-ray studies were perf(-)nned first, hut endoscopy is 1110f(' sensitive.

Gastroenterology

31

Uppe, 's. lowe, Gastroiniostinalllleeding !.OWER

£ncat\on

Pi'oxirnal to ligaJ'nent :t)f1'teitz

GOlnrilon caUSR:$

Ga&tritis, variceS

Stool

Tarry> :black stOql.{lueJena)

Red blood:. s"een -Ju stool.(hcmatochezia)

1\Jasogastrit tube a;spif
Positive-K)!' blood

Negative Fo( blood

Jieptie ukcr disease;

l;:iis-tal t(digin:icl1t ofTreit£

Va-sew}ar e(;tiishi, divert1oilo~js> colon cancel', colttls!:inflamni.awry howel dbea;:<e',-h0mor:tnoius

mil

Endoscopically treatable lesions include polyps, vascular ectashs, and varices.

M

RadioJ]Hclide scans can detect slow Of intermittent hleeding if sonrce cannot he fOlJUd with endoscopy. Angiography caB detect more rapjd bleeding, and embolization of bleeding vessels call he done with this procedure.

3. Surgery is reserved f<>r severe or resist
Circular muscle

Diverticulum

Diverticulosis. Herniation of mucosa between two taeniae, Note the point of weakness where main blood vessel passes into the mucosa. (From James Ee, Corry RJ, Perry JF: Principles of Basic Surgical Practice. Philadelphia, Hanley & Belfus, 1987, with permission.)

Diarrhea has multiple etiologies aud is best hroken down into categories: 1. Systclnic canses: any illness can calise diarrhea as a systemic symptom (('.g., hyperthy-roidisrn, illf(~ctioll).

2. Osmotic diarrhea: lloflahsorhable solutes remain ill the bowel, where tbey retain water (e.g.. lactose or other sugar intolerances, Olestra in potato chips). When the patient stops ingesting the substance (e.g., no more rnilk or a trial ofNPO), the eli.arrhea stops- ·all easy diagnosis.

3. Secretory diarrhea: bowel secretes flUid. Causes inclnde hactcriall'oxills (cholera, SOIl1C strains of Esdlcrichj{l coli), vasoactjvc intestinal peptide--sccretillg {Ul1lOf (pancreatiC islet cell tumor), or bile acids (ahcr ileal resection). Diarrhea continues with NPO status.

32

Gastroenterology

4. Malabsorption: causes indude celiac sprue (look f<)f dcnnatitis herpetifc)rInis, and stop gluten in the diet), Crahn's disease, and gastroenteritis. Diarrhea stops with NPc) status. 5. Infectious causes: look for f(~ver, white blood cells in stool (not with toxigenic bacteria; only with invasive bacteria such as SllifjcJ!
6. Exudative diarrhea: inflammation in bowel lllllcosa causes Sl',epage of flUid. Due to flauunatory howel disease (Crohn's disease or ulcerative cotitis) or cancer.

in~

7. Altered intestinal transit: aher bowel resections or llledicatiolls that i L1tcrft~re with bowel functioll.

Important points: 1. With all diarrhea, watch f()f dehydration and electrolyte disturbances (eg., metabolic acidosis, hypokalcmb), a common and prevl'lltab1e cause of death in lInderdeveioped areas.

2. Do a rectal exam, look f()r occult hlood in stool, and exarnine stool f-()r ova or parasites, fat content (steatorrhea), and white blood cells.

3. If the patient has a history of antihiotic use, think ofClostridimn difficile and test the stool felT c. diffJcile toxin. If the test is pOSitive, treat with metronidazole (if it fails or is not a choice, use VaJ1Cmuyci.n). 4. Do not f()rgeJ about diabetic diarrhea, factitiuus diarrhea (surreptitious laxative abuse, usually by lJH:',dical personnel), hyperthyroidism, and colorectal caucer as causes of diarrhea. 5. Irritable bowel syndrome (IBS) is a COJ1nllOn calise of GI cOlnplaillts. Patients are anxiolls or neurotic and have a IJistory of diarrhea aggravated by stress; bloating; abdo111inal pain relieved by dcft:cat.ion; and/ or I1mcus in the stool. Look for psychosocial stressors in the history and norrnal phYSical flndings and diagnostic tests. This diagnosis of exclusion requires basic lab t.ests, rectal and stool exalllination, and siglnoidoscopy, but because it is very common, it is the 1I1Ost likely diagnosis in the absence of positive findings, especially i.n roung adults. IES lS three times more COlHmon in f(;lnales thall males.

6. After bacterial diarrhea (especially Ii. coli or Shigell" sp.) in children, watch

I,,, hemolytic

urenlic syndrOlllc: thrornbocytopenia, hentolytic aneluia (schistocytes, hehnet cells, fragmented reel blood cells). and acute ffllal failure. Treat suppoftively. Patients may need clial·ysis and/or transfusions.

Inflammatory howel disease

Site of origin ,Thickness _ofpatliofogy

ftectut'n MUt'osa aJld,~~~lblriucosa only

Progn:ssion

Ir~'egulax

Location

From-mouth to illms

-prOXimal, (9lititl\)qus £1'0111' rcct~l~.; no.ski11ped' a lnvolv'es.only Ct?lou;. rately e);'ten,ds to,Hl.tuJP-

'Change.in hQwd habi[s

Onstl'W;t'1oh,_ abd,6:rnioill- -Pil1l-\

J3:kj()dy'oiarrhe

Cla~f-lic,.l{,siOliS

F string

PSCilrlOpofyps:: l.ead-~pip:e. colon' 011 hal'i'um,xo..ray,)oxjC mtgilcolbn

_Colon canccl'-:dsk Surgl:2ry t:ures'bowd _d.iseil'ieJ

(skj_p-Iesions)

Slightly-increased No (m,y

, cobhlestoniri.g. him *""'fay

a

MarKedl -tncre

Gastroenterology

33

Both Crohn's disease and ulcerative colitis rnay involve uveitis, arthritis, ankylosing spondylitis, erythelna nodosUIn/multiforme, prinlary sclerosing cholangitis, failure to thrive or grow in children, toxic nlegacolon (1nore connnon in ulcerative colitis; look {()r markedly distended colon on abdominal x-ray), aneluia of chronic disease, and f(~ver. Both are treated with S~ASA with or without a sulfa drug (e.g., sulfasala:r.ine); steroids are used for severe flare-ups.

Toxic megacolon is classically seen with inflaunnatory bowel disease and infectious coliUs (especially C. dilficilc). It may be preCipitated by the lise of antidiarrbea medications. Symptoms include high f(~ver, leukocytosis, abdominal pain, rebound tenderness, and a very dilated segIncnt of colon on abdominal x·-ray. Ibxic Illegacolon is an emergency. Star,t treatnlent by dis.. contil1ui,ng all antidiarrhca rnedications; then place the patient on NPO status, insert a nasogastric tube, and adTninister IV fluids, antibiotics to cover bowel flora (e.g., arnpicillin or cef.nolin), and steroids if the cause is inflanunatory bowel disease. Go to surgery if perforation occurs (free air on abdOlninal x-ray). Liver disease, acute: elevated liver function tests, jaundice, nausea/vorniting, right upper quadrant pain or tenderness, and/or hepatomegaly.

Important points: 1. Alcoholic hepatitis: elevated liver function tests; aspartate aminotransferase levels are Hlore than twice as high as alanine aminotransferase levels in a patient who was just drinking. 2. [-Iepatitis A: look for outbreaks fronl food~borne source; no loug--tenn sequelae. Serology: pOSitive IgM antibody to hepatitis A virus dUring jaundice or shortly thereafter. 3. Hepatitis B: prevention is best t:reatment (vaccination); acquired through needles, sex, or perinatally. Transfused hlood is nc)w screened fc)r hepatitis H, but a history of transfusion years ago is still a risk £Ktor. Use hepatitis B imnmlloglobulin fl:)r exposed neonates and health care workers. Serology: HBsAg--positive with unresolved inf(~ction (acute_ or chroniC). HBeAg is a marker fi,r infectivity (HBeAbpositive patients have low likelihood of spreading disease). The flrst antibody to appear is IgM anti-fIBc, which appears during the window phase, when both HBsAg and HBsAb are negative. Positive HBsAb means that the patie_nt is inlmUlH~ (due either to recovery froni infection or vaccination) and never appears if the patient develops chronic hepatitis, Sequelae are cirrhosis and hepatocellular cancer (only with chronic infection).

4. Hepatitis C: the new king of chronic hepatitis; most likely cause of hepatitis alter a blood transfusion (used to be hepatitis B befbre blood was screened). More likely than hepatitis B to progress to chronic hepatitis, cirrhosis, and cancer. Serology: antibody to hepatitis C virus shows immunity. New test for HCV RNA detects virus (blood is now screened). 5. Hepatitis D: seen only in patie.nts with hepatitis B; may becOlue chronic wit.h hepatitiS B coinft~ction. Acquired in sallie ways as hepatitis B. IgM antibodies to hepatitis D antigen show recent resolutJon of :infect:iun; presence of hepatitis D antigen lHean!; chronici.ty. 6. Hepatitis E similar to hepatitis A (fbod -and water--borne. no c.hronic state). OH:eu Hual in pregnant WOlnen. 7. Drug-induced: look IC)f acetaJninopllcn, isoniazjd (other tuberculosis drugs), halothane, carbon tetrachloride, tetracycline. Stop the drug! 8. Reye's syndrome: develops in a child given aspirin for fever. 9. Acute filtty liver of pregnancy: develops in third trimester. Treat: with immediate delivery. 10. [schernia/shock: history of shock.

34

Gastroenterology 11. Idiopathic autoinnnunc hepatitis: 20---40--year-old WOlnen with anti-~smooth I1111Sde or antinudear antibodies and no risk factors or lab TnarkcTs of other causes for hepatitis. Treat with steroids.

12. Biliary tract disease: sec below; look f()rmarkedly elevated alkaline phosphatase. liver disease. chronic: often due to alcohol, hq.latitis, and metabolic diseases (hcrnochro 111atosis, Wilson's disease, alphat antitrypsin defkiency). Stigmata of chronic liver disease indude gyncu)1nJstia, testicular atrophy, palmar erytherna. spider angiolnas on skiTl, and ascites.

Important points: 1. Alcoholism: positive history. Mallory bodies

011

bistology (not specific).

2. Hepatitis B or C: positive history and serology. 3. HeulochrOluatosis: priInary few!"n is autosornal recessive disease (look f()r faTuily history) caused hy excessive iron that is deposited in liver (drrhosis, hepatocellular carcinoma), pancreas (diabetes), heart (dilated cardiomyopathy), skin (pigmentation, classically called "bronze diabetes"), and joints (arthritis). Men are symptOlnatic earlier and rnore often; WOlnen lose iron with lllenstTuatiol1. Treat with phlehotonlY. Secondary iron over·load also Inay cause a hemochroI1latosis-like picture, including anemia from inefft'.ctive erythropoiesis (e.g., thalassemia) and excessive iron intake. 4. Wilson's disease: autosmnal recessive disease caused by excessive copper. Serurn cerulo-· plasrnin is low. Sennn copper may be normal, hut liver biopsy shows excessive copper. Patients also have central nervous systeln/psychiatric tnanifestations (copper deposits in basal ganglia; another na111e for this disease is hepatolenticular degeneration) and Kayser· Fleischer rings in the eye, Treat with penicillamine (copper chelator). 5. Alpha l antitrypsin deHciency: younger adult who develops cirrhosis and eTllphysema without risk factors {e)r either; autosoll1al recessive inheritance.

Metabolic derangements that accompany liver failnre:

1. Coagulopathy: prolonged prothrornbin tiule; in severe cases, partial thromboplastin time luay be prolonged. Because the damaged liver cannot use vitamin K, patients must be treated with fresh frozen plasma.

l. Jaundice/hyperbilirubinemia: elevated conjugated and uncoujugated hilirubin with he·· patic damage (vs. biliary tract disease). 3. Hypoalbulllinemia: liver synthesizes albumin. 4. Ascites: due to portal hypertension and/or hypoalbuminelnia. Ascites can be detected OIl physical exam by shifting dllUness or a positive fluid wave. Possible complication is spontaneons bacterial peritonitis··infected ascitic f1uid that leads to sepsis. l.ook filr {t~ver and/ or change in 1nental status in a patient with known ascites. Do a paracentesis, and examine the ascitic fluid for white blood cells (especially neutrophils), Gram stain, culttlrC and sensitivity, glucose (low with inf(~etion), and protein (high with inf(~ction). Usually caused by E. coli, S. pnclIIlloniac, or other enteric hugs. Treat with broad'~spectrum ant.ibiotics. 5. Portal hypertension: seen w.ith cirrhosis (chron:ic liver disease); causes henlorrhoids, varices, caput rnedusae. 6. l---Iyperamrnoneuria: liver clears annnonia. Treat with decreased protein intake (source of NH 3 ) and lactulose (prevents al)sorption of Juullonia). Last choice is neolTlycin (stops bowel flora hom making NH]).

Gastroenterology

35

7. Hepatic encephalopatby: mostly dne to hyperammonemia; often precipitated by protein, GI bleed, or infection.

8. Hepatorenal syndrorne: liver failure causes kidney failure (idiopathic). 9. HypoglyccJllia: liver stores glycogen. 10. Disseminated intravascular coagulation: activated clotting factors usually cleared by liver.

Biliary tract disease: jaundice Inay be caused by bile duct obstruction. Look for markedly elevated alkaline phosphatase, conjugated bilirubin that is rnOrt' elevated than unconjugated bilirubin, pruritus, clay-.·colored stools, and dark urine that is strongly hilirubin--positive. Unconjugated bilirubin is not excreted in the urine because it is tightly bound to alblllnin. 1. Common bile duct: obstruction with gallstone: look fbr history of gallstones or the four l~s (ft~male, forty, fertile, fat). Ultrasound can often image the stone; i£'not, use enelo,· scopic retrograde cholangiopancrcatograplly.

2. CcnnmOIl bile duct obstruction from cancer: usually pancreatic cancer, sometirnes cholangiocarcinoma or bowel cancers. 3. ChoJestasis: often from medications (oral contraceptives, phenothiazines, androgens) or pregnancy. 4. Priulary biliary cirrhosis: rniddle-aged woman with no risk factors for liver or biliary disease, rnarked pruritus, jaundice, and positive antimitochondrial antibodies; rest of work-up is negative. Cholestyramine helps with symptoms, but no treatment (other than liver transplantation) is available. 5. Primary sclerosing cholangitis: young adults with inflammatory bowel disease (usually ulcerative colitis); presents like cholangitis. 6, Cholangitis: Charcot's triad = fever, right upper quadrant pain, and jaundiee. Treat with antibiotics, and relTI(We swnes surgically or endoscopically. Esophageal disorders: dysphagia is usually an esophageal complaint. Patients lIlay present with atypical chest pain. 1. Achalasia: hypertensive lower esophageaf sphincter (LliS), in~()Ihplete relaxation of LES, and loss or derangeulent of peristalsis. Achalasia is usually idiopathic but: ITlay he secondary to Chagas' disease (Sollth America). Patients have intermittent dysphagia for solids and liquids with 110 heartburn. Barium swallow reveals dilated esophagus with distal "birdbeak" narrowing. Diagnosis can be made with esophageallnanmnetry. Treat with cakiu111 channel blockers. pneumatic balloon dilatation, and, as a last resort, surgery (myotomy).

2, Diffuse esophageal spasm/nutcracker esophagus: both have irregular, forcdirl. painful esophageal contractions that cause interrnittent chest pain. Diagnose with esophageal Inanometry. Treat: with calcimn channel blockers and, if needed, surgery (InyotOll1Y). (See figure, top of next page.)

3. Sclerodenna: Inay cause aperistalsis due to fibrosis and atrophy of Sl1100th rnuscle. Lower LES becomes incompetent, and patients may develop GERD. Look f()r l)()sitive antinuclear antibody and rnask.-like facies, other autoinnllune symptom,s (CREST;::::: calcinosis, Raynaud's phenomenon. esophageal dysmotility. sclerodactyly, telangiectasias). 4. Barreu's esophagus: colurnnar metaplasia due to acid reflux; 111Ust be f()llowed with peri~ odic endoscopy and biopsies to rule out progression to adenocarcinorna.. Pancreatitis: more than 80%) of cases arc due to alcohol and gallstones. Other causes include hypertriglyceridemia, viral inf{;ctions (nullnps, coxsackie virus), trauma, and rnedications

36

Gastroenterology

Diffuse Esophageal Spasm

""'" j ,::.::'..

'.'

.....'

...••

.

'

..

" '

.' .'

Diffuse esophageal spasm. IFrom James Ee, Gorry RJ, Perry JF: Principles of Basic Surgical Practice. Philadelphia.. Hanley & Belfus, 1987, with perrnission.)

(steroids, azathioprine). Patients have abdorninal pain radiating to the back, nausea and vOInit~ ing that does not relieve the pain, leukocytosis, and elevated Jlnylase/lipase, Perfc)fatcd peptic ulcer disease also may have elevated amylase and presents sinlilarly, but patients have free air on abdonlinal x··ray and hi.story of peptic ulcer disease, Ill!

TreatlneIll: NPO, llJsogastric tube, IV fluids, narCOllcS (merperidinc, not morphine)

=

.. Grey··Turner's sign = blue/black flanks; Cullen's sign blue/black umbilicus (botb are due to hernorrhagic exudate; both indicate severe pancreatitis). I§iI

I§iI

COIl1plications include pseudocyst (drain surgically if symptomatic), abscess/infection (antibiotics and surgical abscess drainage), and diabetes (with chronic pancreatitis). Treat chronic pancreatitis with alcohol abstinence, oral pancreatic cnzynlC replacement, aud fat~solllble vitamin snpplcHtcnts,

Mallory-Weiss tears arc sllpcrHcial esophageal erosions that may cause a GI bleed. They usually afC seen with vorniting and retching (alcoholics and bulimics). Diagnosis and treatment are done endoscopicaJIy (sclerose any bleeding vessels). Boerhavc's tears arc fufl~thickness esophageal ruptures; if not iatrogenic (frorn endoscopy), they afC usually due to vomiting or retching (alcoholics and buliIllLcs). Diagnose with endoscopy or barium eneHla, and treat with imm.cdiate surgical repair and drainage. Note: With suspected GI perfc)ratioll, never use 11arillm (which may cause chemical peritonitis). Use water-soluble contrasl instead (e.g., GJstrograflll). The exception is esophageal pcrf(xatjon because the lungs tolerate barium well but develop chemical pneulllOnitis frolll water··soluble contrast.

Gastrointestinal malformations seen in rhildl'en: (see table, top of next page) Tracheoesophageal fistula: the rnost common variant (8-5%) of cases) has esophageal atresia with a flstula from the bronchus to the distal esophagus (hcnc(" gastric distention; each hrcaJh tTansnlits air to the G1 tract). Be able to recognizc a sketch of this most COllnllOn variant. Treat each of the f()llowillg conditions with surgical repair.

Gastroenterology

37

;Q~tectbd:by-ii1iiial-:¢X~tu:;n_ nurs~rf;::M_-:>--F

x~l~eve~_ btt;ryJIlg; tnab-ility it):pass

0-1 wk

Other gastrointestinal conditions in children:

Diaphragmatic hernia: more t:ornmon in males and on the left side. The InaiJ1 point to know is that bowel herniates into the thorax, cOInpressi.ng and irnpeding lung de\i('Jopmt~J)t (pullllOnary hypoplasia develops). Patients present with respiratory distress and have howel sounds in the chcst/bowe1loops in the thorax on chest x--ray. OUlphalotde vs. gastroschisis: omphalocde is in the midline. Sac cOlltains multiple abdollli-lId] organs, the 11mbiUcal ring is absent, and other anomalies are conunOJl. Gastroschisis is to the right of the midline. Only small bowel is exposed (no true hernia sac), the umbilical ring is present, aud other anornaJies are rare. Henoch~Schonle.in purpura

IndY present with GI hleeding and abdoJHinal paln. Look for his

tory of 11 pper respiratory iJlf(~ction, characteristic rash on lower extrcmities and huttocks, swelling in hands dnd f(~ct, arthritis, and/or hematuria/proteinuria. Trcat: suppOrlivdy.

Note: ChiJdrcn (more than adults) develop nau.sea and vOlniting aud/or diarrhea with allY systelllic illness. They a1s() I1lay develop inflalnrnatory bowel discas(:~ or irritahle howel syndrOllle and onen have GI complaints with anxiety or psychi.atric problcrns (separation anxiety, reJuclance to go to :o,dlOol, depression, child ahuse). Neonatal jaundice: may be physiologic or pathologic. The first step is to measmc t01;11, direct, and indirect hilirubin. The main COJlcern is kernicterus, which is due 10 high levels of unconjLlgated

38

Gastroenterology biliruhin and subsequent deposit into the basal ganglia. Look fl:lf poor feeding. seizures, Hac (jdity, opisthotonos, and/or apnea to accompany severe jaundice.

Physiologic jaundice: in SO%) of normal infants; even runrc COrnr11011 in premature infants. Bilirubin is ]}lostly unconjugated. In pretenn infants, biliruhin is < 15 lUg/ dl, peaks at J-~S days and may be elevated f{)r up to 3 weeks. In 1t,1I·term infants, bilirubin is < J2 mg/dJ, peaks at 2-4 days, and returns to normal by 2 weeks. Pathologic jaundice: levels rise higher than llonnal and continue to rise or fail to decrease appropriately. Any janndice present at birth is pathologic.

1. Breast milk janndice: breast·i',d infants with peak bilirubin of I{)-20 mg/dl occurring at 2-3 weeks of age. Treat with temporary cessation of breast f,'eding (switch to bottle) nntil jaundice resolves. 2. Illness: inf<::ctionl sepsis, hypothyroidisnl, liver insult, cystic fibrosis, and other illncsst:s may prolong neonatal jaundice and lower -the threshold f(H kernicterus. The youngest, sickest in6nts are at grcatcst risk f(){ 11ypcrbilirubincmia and kernicterus.

3. Hemolysis: frorn Rh incompati.bility or congenital red cell diseases that cause hemolysis in the neonatal period. Look for anemia, peripheral smear abnonnallties, farnily history, and higher level of nnconjngated bilirubin. 4. Metabolic: Crigler-Najjar syndrome canscs severe unconjugated hyperbilirnhinemia, Gilbert's disease causes rni1.d unconjugated hyperbilirubinernia, and Rotor and DubinJohnson syndromes cause conjugated hyperbilirubinemia. 5. Biliary atresia: li.,lI-term infants with clay· or gray-colored stools and high levels ofconjugated bilirubin. Treat with surgery. 6. Medications: avoid sulfa drugs in neonates (displaces bilirubin fron1 allHuuin and may precipi.tate kernicterus).

Treatment for nnconjngated hypc;rbilirubinemia that persists. rises higher than liS mg/dl, Or rises rapidly is phototherapy to convert the unconjtIgated bilirubin to a water-soluble forn} that can be excreted. The last resort is exchange transfusion (do not even think about it unless the level of unconjugated bilirubin is > ZO mg/dl). Note: Any infant born to a IllOther with active hepatitis B should get the first inununization shot

and hepatitis B inununoglobulin at hirth.

You must understand the hypothalamk~pituital'Yaxis so that yOll can distinguish primary frOlll secondary disorders. [Jl primary endocrine disturbances, the gland itself is I11alfunctioI1ing (e.g., from tumor, inflammation, enzyuw deflciency), but the pituitary and hypothalaUllls afe fUIlctioning llormally and exhihit the appropriate response to the gland's actiou. For example, thyroid-stinllllating hormone (TSI-I) is low in Graves' disease, because the thyroid is Inalhmctiouing and overproduces thyroid hormone. The appropriate reSp()I1Se is for the pituitary to secretc less TSH because of feedback inhibition. In a secondary endocrine disturbance, the gland is perf<xt.ly normal, but the pituitar}' or hypothalan1l1s is malfullctioning. For example, if the pituitary secretes low levc]s ofTSH or t.he hypothalamus secretes low levels of thyrotropin.. releasing hormone (TRH) in patients with hypothyroidism, the pituitary or hypothalamus is malf,Jllctioning, because it should be secreting high levels ofISH orTRH whcn the level of thy roid hormone is inadequate. HypothyroidislU: look for classic SYIDptOlns of fat.igue, bradycardia, lnenstrual disturbances (usually rnenorrhagia), slow speedl, cold intolerance, constipation, carpal tunnel syndrOlne, decreased rdlexes, anClllia of chronic disease, and/or coarse hair. Hypothyroidisllllllay be associated hypercholesterolc-mia, which resolves with treatment. Check thyroid function tests (TSH, thyroxine IT.l!, free thyroxine index [FTII). UsuaHy ISH is high, and T4 (primary) is low. Treat wilh thyroid honnoue (synthcticT4 ), Causes of hypothyroidism: 1. Hashimoto's thyroiditis: most comrnon cause; associated with other autoimnlUJlC diseases (e.g., pernicious allen-lia, vitiligo, lupus). Look f()f positive antimicrosomal antibod--

ies. Histology shows lymphocyte inflltration of the gland. 2. Subacllte thyroiditis: acute viral jnflauuuatioll with f(~ver and enlarged, tender thyroid gland. History of upper respiratory inft'ction or l1wmps is comrnon. Give NSAIDs f()[ symptom relief Patients often recover without treatment.

3. After treatment for hyperthyroidisrn, be awart' that it frequt'ntly occurs (second IHost conlJnon cansc in o.S.).

4.

Sick-~ellthyroid syndrorue: any illness may decrease T4- and/or triiodothyronine (1"3)' but rSH is normal. The condition is self,limiting. and no treatment is necessary except f()r the underlying disorder.

5. Toel.inc defkiency: rare in US. May cause cretinism in children (stunted growth and mental retardation).

39

40

Endocrinology

HyperthyroidislU: symptoIlls include nervousness, anxiety, insomnia, tachycardia, palpitations, atrial fibrillation, heat intolerance, weight loss, diarrhea, Iucnstrua.l irregularities (hypomenorrhea), increased appetite, and "thyroid stare." Check thyroid fnnction tests. Usually TSH is low, and T+ (primary) is high. Exophthalmos and pretibial myxedema are specifIc If)r Graves' disease. Trealrnent begins with antithyroid drugs (propylthiouracil or ITldhi111JZole). Most patients eventually require fllrther therapy. Use surgery fcn patients under Z5 or pregnant WOlnen and radioactive iodine {()[ patients over 40. For patients 24~40. {TeaUncnt is contIn·· versial, and either approach is acceptable. Propranolol is llsed for thyroid stunn (the patient decompensates, physically and mentally, from very bigh thyroid hormone levels) and syrnptomatic tachycardia, palpitations, and arrhythmias. Causes of hyperthyroidism: 1. Graves' disease: by far,most ccnmnon cause. Exophthalntos and pretibial myxedeJua are specHk f()f Graves' disease. Pafien.tshavc positive thyroid-stimulating innnullogJobu lins/thyroid--stinnllating antibodies, which activate the TSH receptor. Nontender, diffuse goiter also is present. Whole gland takes up excessive radioactive iodine. 2. Phulllner's disease/toxic rnultinodular goiter: hyperfunctioning nodules cause a hunpy goiter without positive antibodies or cxophtllahnos/pretihial 111yxedclna. Radioactive iodine uptake is high in nodules, but decreased in the rest of the gland.

3. Toxic adenoma: one nodule is palpable and has high radioactive iocline uptake; the rest of the gland shows decreased uptake (thyrOid cancer is rarely hyperhmetional). 4. Thyroiditis: Hashiuloto's or subacute thyroiditis rnay produce a transient hyperthy-· roidism due to inflammation be{-(wc converting to hypothyroidisnl.

Note: In pregnancy and other states (administration of oral contraceptives/estrogens, illft~(> tions), thyroid-hinding globulin (THC;) may be elevated. Altbough this causes devation of total thyroid honnone levels, free thyroid hormone is not elevated, and TSH is normal. Do Hot treat. Nephrotic syndrome or large protein losses of any kind and anabolic steroids can decrease TBG (again, TSH is normal and you should not treat). Hypoadrenalism (Addison's disease, primary adrenal insufflciency): the ITlOst cornmon cause is idiopathic (probably autoifmHune). Look for increased skin p.igrnentation, weight loss, de-hydration, anorexia, nausea and vomiting, dizziness and syncope, hyponatrernia, and hypcrkalcluia. Under metabolic stress (i:n{t;ction, surgery) patients may have an adrenal crisis-~--­ abdoluinal pain, hypotension/ cardiovascular collapse, renal shutdown, and death. Treat with hydrocortisone and IV flUids to avoid adrenal crisis. The diagnosis of llypoadrenahsII1. when not obvious, is done by administering adrenocorticotropic honnone (ACTI-I) and seeing whether levels of plasma cortisol increase over baseline. 1)0 not tle/oy giving steroids to do this test if the patient is doing poorly; the patient Inay die while you wait for the results. Secondary adrenal insufficiency: commonly tested disorder, and is lnost often due to previ·OtIS taking of steroids. Once patients take steroids fe)f luore than 1 rnonth, they nuy not be able to 1110UUt an appn)priate increase in ACrH when needed f-()r up to 1 year! The classic setting is the patient on ~tcroids wh.o stops taking aU rnedications beft)re surgery, then develops refractory hypotension and electrolyte disturbances after surgery. Give (:orticosteroids! Other sec ondary causes of adrenal insufficiency arc Sheehan's syndrOlne (history of IJost,parturn hypotension, inability to breast--f(~ed, and other endocrine insumciencies) and. ncoplasrfls (pituitary adcnoHrJs and craniopharyngiom,as). In secondary hypoadrenahsrn, :mineralocorticoid (aldosterone) secretion is not aff(~cted, because it is not directly under pituitary control; thus, the electrolyte disturbance is not as severe, and there is no skin hyperpjgnH~ntation. ACTH is

Endocrinology

41

decreased, as is melanocyte-stimularing hormone (MSH), which is thought to cause the skill hyperpigmentation in primary adrenal insu!llciency. Hyperadrenalism (Cnshing's syndrome): usually due to prescribed steroids in the u.s. l.ook i<')f moon facies, truncal obesity, "buffalo hurnp". striae, poor wound healing. hypertension, osteoporosis, secondary diabetes or glucose intolerance, Inenstrual abnonnalities, and psychiatric disturbances (depression, psychosis). Cushing's disease is Cushing's syndrmne caused by pituitary overproduction ofACTH, which usually is due to a pituitary adenorna. Get an MRI of the brain ifJevels ofACTH and cortisol are high. Other causes are adrenal neoplasms that produce steroids and small cell cancer of the lung, which may produce ACTI-l. Treat by curing the neoplasm. Diagnosis is made by first doiug a screening test. The best choice is usually a 24· hour urine lest for free cortisol; plasIna cortisol is not a good test because of wide inter- and intrapatient fluctuation. Then do a dexauwthasone suppression test. Hyperaldosteronism: primary disease is known as Conn's syndn)Ine and is due to an ade-1l00ua. Look for hypertension, hypernatrernia. hypokalemia and low renin. Get a CT scan of the ahdOIuen. Secondary hyperaldosteronisrll is much Inore (mDrnOIl, and is related to hyperten~ sion (especially with renal artery stenosis) and edematous disorders (congestive heart failure, cirrhosis, nephrotic syndrome). Look for hypertension, edema, renal bruit, variable sodimn and potassium, aud high reuin. Treat the underlying cause.

Pheochrolnocytolua: popular on the boards. Look for intermittent hypertension that is very high, wild swjngs in blood pressure, tachycardia, postural hypotension, headaches, sweating, dizziness, mental status changes. and! or feeling of impending doom. Patients also rnay have glucose intolerance due to high catecholamines. If you are suspicious. first screen with a 14-hour urine test to look for catecholaluines and their break-down products (vanilIylmandelic acid [VMAJ and! Of homovanillic acid [HVAI, rnetanephrines). If the screen is positive, do an abdOIuinal CT, and cut out the tUlnor after stabilizing the patient with alpha and beta blockers. Diabetes insipidns (DI): Symptoms include severe polydipsia and polyuria (patients may urinate 25 Uday). When access to water is restricted, patients rapidly develop dehydration aud hyperllatremia, which may cause death. Giving antidiuretic hOfIllone (ADH) detennines whether the cause is central or nephrogenic. Central disease responds to ADH, whereas nephro~ genic disease does not. m Nephrogenic

DI: look for Ilwdications as cause (hthimll, methoxyflurane, clenwclocy" cline). Treat with thiazide diuretics (paradoxical efh'ct; ADH does not help).

i!f

Central DI: look for traunla, neoplasrIl, or sarcoidosis, although central DI is often idio·pathic. Treat with ADH!vasopressin, and treat underlying cause, if possible.

Syndrome ofillappropriate secretion ofantidinretic hormone (SIADH): symptoms include hyponatremia as well as low levels of every other eleetrolyte (and lab value) because of dilution from excessive water retention. Look f(x medications (morphine, chlorpropamide, oxytocin~·'·­ be careful in pregnant patients), small cell lung cancer, postopcmtivc .Iriltus (watch fi,r all electrolyres to fall after surgery), traU111J, hmg inf(~ctions, and pain. Treat with water restriction. For board purposes, do not give hypertonic saline, and do not try to correct hyponatremia aggressively or quickly. Rapid correction may cause hrainstcul dalliage. Obesity: causes an increased risk of the f()llowing problenls: 1. Overall mortality (at any age)

2. Insulin resistance/diabetes Il1eUitus

42

Endocrinology

3. Hypertension 4. Hypertriglyceridemia (also weakly associated with hyperchol(~sterolelllia) 5. Bean disease/ co[{)uary artery disease

6. GallstollPs (cholesterol stones)

7. I-lypoventilati()ll, pickwick ian syndrmnc, sl('cp apnea 8. Syrnptomatlc osteoart hritis 9. Cancer, vspccially cndonwtrjal C,lneer

10. Thro111b(H'Jllbolism 11. Varicose veins

CHAPTER

6

Nephrology

Acute reual failure (ARF): progressive rise in crealinine and blood urea nitrogen (BUN), lnetabolic acidosis, hyperkalemia, and hypervolem ia. SyJnptOllls include rales, elevated jugular venous pressure, and dHutional hyponatremia. Three cate'gories: 1. Prerenal: most

COllUnOIl exa_tnple is hypovo]en1.ia (dehydration, hCTnorrhage). Look flJf BUN/ creatinine ratio> 15 or 20, Patients have signs of hypovolemia (e,g., tachycardia, weak pulse. depressed foutaneUc.). Give IV-fluids and/or blood. Other prerena( causes are sepsis (treat the sepsis and give IV lluids), heart lailure (give digitalis and diuretics), and liver failure (hepatorenal syndrome; treat supportively),

2. P()strcllal: most common exarnple is benign prostatic hypertrophy (BPH). The patient is a man over 50 with BPH symptoms (e,g., hesitancy, dribbhng), and ultrasound revcals bi lateral hydronephrosis. Treat with catheterization (suprapubic catht'terizati<)}l if neces~ sary) to relieve obstruction and prevent further renal damage; thell consider surgery (transurethral prostatectomy [TURP]), Nephrolithiasis is also a possible cause, but stones have to be bilateral to cause renal failure.

3. }{t:'nal: acute tubular necrosis is the most common type. Examples of renal causes: !ill

IV contrast: do not give to diabetics or reual patients if you cau avoid it; yOll Illay precipitate acute renal failure. If you lllllst give it, give Jots of hydration.

IIlIl\IIyoglohiI1uria/rhahdo,myolysis: from strenuous exercise (e.g., marathon), alcohol, burns, muscle trauma, heat stroke, or neuroleptic malignant syndrome. Muscle breaks down and plugs up the rellal filtration systern. Look for very high levels of crealine phosphokinase (GJK) ,Treat with hydration and diuretics, Il/I

f!i'l

!!l

Iii!

Lupus erythclnatosus: look {-()f malar rash and arthritis. Renal failure is a major caus(-' of morbidity and rnoriality. Toxins/ulcdications: sides, methicillin.

clUOJ]1c

NSAID

liSt'.

(papillary necrosis), cydosporiuc, amiIl()glyco--

Goodpasture's syndrOJllc: due to antiglomernlar basenlent membrane antibodies (lillear inmruIlot1uOft'sC('llce pattern on rC]Jal biopsy), which also rcact with the lungs, Look for a young luaIc with hemoptysis, dyspnea, and renal failure. 'frcat with steroids and cyclophosphamide, Wegener's granulomatosis: also has lung and kidney involvement. Look for nasal iJ].~ volvenlcul: (bloody nose, nasal perf()fation) or hcnlOptysis and plctLrisy as pn'selltillg synlptoms. Patients have positive antiIl('utrophil cytoplasm antihody (ANCA) titer. Treal with cyclophosphamide.

113

44

Nephrology

Ifil

Glofncrulonep,hriUs: prototype is post--strepwcoccaJ syndrome; usually seen in chil~ drcn with history of upper respiratory infection or strep throat 1-3 weeks earlier; they present with edema, hypervolernia. hypertension, hernaturia, and oliguria. Red blood cell casts 011 urinalysis clinch the diagnosis. Treat supportively.

Note: In aU cases of ARF, dialysis Inay be required. Indications for dialysis include ureuric en-

cephalopathy, pericatditis, severe metabolic acidosis (roughly, pH < 7.25), heart lailure, and hyperkalemia severe enough to cause arrhytlunia. Nephrotic syndrome: proteinuria (> 3.5 gm/day), hypoalbuminemia, edema (classic exam pie is morning periorbital edema), and hyperlipidernia/lipiduria. In children, it is usually due to minimal change chsease, often ancr an iuft'elion. Measure 24:--hour urine protein to dincJl the diagnosis, and trcat with steroids. Causes in adults include diabetes mellitus, hepatitis E, amyloidosis, lupus, and drugs (gold, penicillamine, captopril). Nephritic syndrome: oliguria, azoternia (rising BUN/creatinine), hypertension, and helna~ turia. Patients may have S0111e proteinuria, but not in the nephrotic range. The usual cause is P()st-streptococcal glolTlerulonephritis.

Chronic renal failnre (CRF): any of the canses ofARF may cause CRT' jf the jnsult is severe or prolonged. The majority of cases of CRF afe due to diabetes lllclhtus (number one cause of CRF) and hypertension. Another counTIon cause is polycystic kidney disease (ITIultiplc cysts in kidney). Look for positive f~uni1y history (usually autosOlnal dominant; autosomal recessive fonn presents in children), hypertension, hematuria, palpable renal lllaSSes, berry aneurysrns in tlle circle ofWillis, and cysts in liver. Metabolic derangements due to CRF: 1. Azotemia-·-high BUN/creatinine

2. Metabolic acidosis 3. Hyperkalemia----know EKG changes 4. Fluid retention-' -may cause hypertension, edenIa, congestive heart failure, and monary edelHa

5.

Hypocalcenlia/hyperph()sphatemia-~vitamin

pul~

D production illlpaired; bone'loss leads to

renal osteodystrophy 6. Aneluia--from lack of erythropoietin (synthetiC crythropoietinmay correct) ], Anorexia, nausea, vOll1iting---from build--up of toxins 8. Central nervous systell1 disturbances-- --mental status changes and even convulsions or

coma frolll toxin buiId--up 9. Bleeding-- --due to disordered plateJet function, patients IIlay have prolonged bleeding tiIHC test

10. Uremic

pericarditis~

-may hear a {fiction rub

11. Skin pigmentation and

pruritus-~----skin turns

yeHowish ,browll and hches due to

Inl~ta-­

bolic byproducts 12. Increased susceptIhihty to infl::cUon-- ~due to decreased ceHular imn:umity

Treatment of'CRI": regular dialysis, wattT--soluble vitaluins (removed during dialysis), phosphate rcstrictioIllbinders (aluIllinu1l1 or cakiuHl CarbOJlatc), erythropoietin, and hypertension control. The only cure is renal transplant.

Nephrology

115

Urinary tract infection (UTI): much 11l0fC common in fenlalcs. Usually caused by Iischerichia coli (also by other enteric organisms). Look for urgency, dysuria, suprapubic/low back pain, and low-grade fever. The gold standard for diagnosis is urine culture (at the least, get a Inidstreanl salIlple; hest is catheterized sample or suprapuhic tap). Udnalysis shows wh'ite blood celts, bacteria, positive leukocyte esterase, and/or positive nitrite. Treat with trimethoprirn/sulfamethoxazole, amoxicillin, nitrofurantoin, or flrst--generation cephalosporin for about] week.

Important points: 1. In patients less than S years old, UTI is a cause for concern because it nlaY be the presenting sYJnptom of a genitourinary malf(Jrmation. The most comnl0n examples are veslco ureteral reflux (VUH.) and posterior urethral valves. Get an ultrasound and a voiding qTstourethrogram to evaluate any male under 6 with a UTI and any female under 6 with recurrent UTIs or pyelonephritis. 2. Some women get recurrCJlt UrIs related to sex and can be giV(')Jl antibiotics to take aHerwards. 3. Conditions that promote uri nary stasis (BPH, pregnancy, stones, neurogenic bladder, VUH.) or bacterial colon.ization (indwelling catheter, fl'cal incontinence, surgical instru-

mentation) predispose to UTI. They also predispose to ascending UTI (pyelonephritis) and bacteremia/sepsis. 4. ASyJuptornatic bacteri.uria is treated "in pregnancy (high risk of progression to

pyelonephritis) . Pyelonephritis: almost always ffom an ascending UTI and due to E. coli (> 80% of cases). Patients present with high fever, shaking chills, costovertebral angle tenderness/flank pain, and/or UTI symptoms. Urinalysis and urine and blood cultures establish the diagnosis. Treat on an inpatient basi.s willl IV antibiotics while awaiting test results (penicillin or cephalosporin pIllS aminogl ycoside). Kidney and hematologic disorders in children

NQri:n£l , $J~t~itl$;-:sgl~neet:61'1)~'1~ rne:dh;a(iQJl;S', (aut

). (bleeding)

Or

platelets < 20,000.

Renal stones: present with severe, intermittent, uniJateral fhnk and/or groin pain. M'osl stones show ,up on abdominal x--ray and are composed of' calciulll. Most cases are idiopathic

116

Nephrology and should he treated with lots of hydration and pain control (to see if stone will pass). If stone does not pass, it needs to be removed surgically (pref''fably endoscopically) or by lithotripsy. Underlying canses of stones:

1. Hypcrcalceluia: due 10 hyperparathyroidiS1l1 or rnaJignancy (nletastases or squamous cell lung cancer--secreting parathyroid hormone).

2. lnkction: from ammonia-producing bugs (Proteus, Staphylococcus spp.). Look fi)r Staghorn calculi. 3. Hyperuricemia: fronl gout or frorn leukemia treatment (allopurinol and IV flUids are given befc)re cheruotherapy as preventive rneasures). 4. Cystinuria/aminoaciduria: suspect if the stone is made of cystine and in repetitive stonefbrnling patients.

CHAPTER

7

Rheumatology

Arthritis: the large lllajority of cases are due to osteoarthrltis (OA). When in doubt O[ if you suspect some_thing other than OA, aspirate fluid frOIn the affected jOjrlt for eXJlnl.natioll. Exanline the fluid fl.Jf cell counL and differential, glucose, bacteria (Gram stain and culture), and crystals:

MATOID RI'rlS

OSTEOARTHRITIS

GOUT

PSEUPOGOUT

Slll''rlC ARTHIUTIS

--------~

Older :rne-n

Older -adults

Any age

PlifMCP, wris"t

Big'-IDe

KI1l~es,

Knee

< Z,OOO

> 2,000

> 2,000

> Z,OOO

>50,000

< 25

>-,5:0

> 50

> 50

> 75

H~i:1al1~g~!sex

Olde, adults

Classic -J:()lnis

Dlit-PIP; hil);J(ii¢e.

JOiin Huld -whit~ blood ¢e~l cQunt % l:W:t±mro,prl.iIs

dbows

Other key difleren<:es/points: 1. OA: few signs of inflammation on exam (lacks hot, red, tender joints seen in all the others of IIIis group). Symptoms include Hcbcrdcn's (DlP) and Bouchard's (1'11') nodes, worseuing of symptoms ill evening and after use, and bony spurs. Incidence increases with age. Treat with weight reduction and NSA1Ds as needed. 2. Rheumatoid arthritis (RA): positive rheumatoid factor clinches the diagnosis in most pa~· tients, hut children afe oft-en negative. Look for systemic syrnptoms (f(~ver, Inalaise. sub-· cutaneous nodules. pericarditis/pleural effusion. llVeitis), prolonged morning stiflJlcss, and swan neck and boutonniere deformities. The buzz word is pannus (articular cartilage looks like granulation tissue due to chronic inflammation). Treat with NSAIDs, hydroxy-chloroquine, gold, penicillamine, and steroids (fo)' bad flare-ups). 3. Gont: classically starts with podagra (gout ill tbe big toe). Look for tophi (subcutaneous uric acid deposits, punched~()ut lesions in bone x--ray) and needle-shaped crystals (ohen inside leukocytes) with negative birefringence. COtlt is Inore C011ll11on ill Jllell than wornen. Patients should avoid alcohol (nlaY precipitate an attack). Colchicine or NSAIDs (not aspirin, which causes decreased excretion of uric acid by the kidney) are used f()J: acute attacks. Maintenance therapy include.s high fluid intake, alkalinization of the nrtne, and/or prohenjcidlallopurinol (neil her {()r acute attacks). 4. Pseudogont: rhomhoid- shaped crystals with weakly positive bi.refrillgencc·.

47

48

Rheumatology

5. Septic arthritis: synovial fluid has bacteria on Graul stain. Staphylococcus aureus is the lTIOst common organism. except in sexually active young adults (Neisseria gonorrhocac is 11l0St common in this group). Do blood cultures in addition to joint cultures, because the bug usually reaches the joint via the hematogenous route. Do urethral swabs and cultures in appropriate patients.

Other causes of arthritis 1. Psoriasis: in the presence of skin lesions, diagnosis is easy. Arthritis usually affect hands and feet, and the arthritis resembles RA but rheUlnatoid factor is negative. Treat with NSAIDs, gold, penicillamine, or steroids. 2. Lupus erythematosus or inflanlmatory bowel disease: other syrIlptoms of the priInary disease nuke diagnosis easy. 3. Ankylosing spondylitis: associated with HLA~B27. Most often a 20~~40~year~01d man with a positive farnily history presents with back pain and 1110rning stifIness; the patient Inay assume a bent-over posture. Sacroiliac joints arc priInarily an(~cted, and x--rays m,ay reveal a baJnboo spine. Patients have other autoiInlDuneAype syInptOlTls. such as fever, elevated erythrocyte sedimentation rate (ESR) , and anemia; some develop uveitis. Treatment is exercise and NSAIDs. 4. Reiter's syndrome: also associated with 1~ILA~B27. The classic triad is urethritis (due to Chlamydia sp.), conjuuctivitis, aud arthritis ("can't pee, can't see, can't climb a tree"), but Reiter's syndrome also may follow enteric bacterial infections. Superficial oral and penis ulcers also are comnIOn. Diagnose and treat the sexually tranSIllitted disease, and treat sexnal partners. NSAIDs are llsed ]()r arthritis. 5. Hemophilia: recurrent hemarthroses may cause debilitating arthritis. Treat with acetaminophen (avoid aspirin). 6. Lyme disease: look for tick bite, erythema chronicum nligrans, and lnigratory arthritis later. Treat with doxycycline/tetracycline (amoxicillin in pregnant women).

1. Rheumatic fever: look for prevIous streptococcal pharyngitis. Migratory polyarthritis is one of the rnajor Jones criteria. 8. Sickle cell disease: patients frequently develop arthralgias and avascular necrosis of the [,'moral head. 9. Tranma 10. Childhood orthopedic problem: slipped capital t<,moral epiphYSis, congenital hip dys~ plasia, and Legg~Calv{'~Perthesdisease may cause arthritis as adulthood. Use history (age of onset) and x~rays to determine which disease the patient had as a child.

11. Charcot joint: most commonly seen in diabetes Inelhtus; also in other neuropathies. Lack of sensation causes patient to overuse or Inisuse joints, which bcccHue defonned and painfuL The best treatment is prevention. After even seerningly rnild traurna, patients Wlth neuropathy in the area of the trauma need x··rays to rule out fractures.

12. Hernochronntosis/Wilsou's disease: both Inay be associated with arthritis due to deposition of iron/copper. AutoiInmnne diseases: aff(~ct WOluen of reproductive age unless otherwise specified. For board purposes, classic disease findings diH(~rentiate one condition limn the other. Ahnost all patients have systemic signs of inflammation (elevated ESR/(>reactive protein, ft~ver, anen1ia of chronic disease, fatigue, weight loss).

Rheumatology

49

1. Systernic lupus erythematosus: Inalar rash, discoid rash, photosensitivity, kidney damage, arthritis, pericarditis/pleuritis, positive antinuclear antibody (ANA), positive anti-Slnith antibody, positive Venereal Disease Research Laboratory or rapid plasma reagin test f,lr syphilis, positive lupus autieoagulant, blood-penias (thrombocytopenia, leukopenia, anemia or pancytopenia), neurologic disturbances (depression, psychosis, seizures) and oral ulcers may all be presenting syn1ptOlTIs. Use ANA titer as a screenIng test, anti-Smith antihody to confirm. Treat with NSAlDs, hydroxychloroquine, and steroids. 2, Scleroderma/progressive systemic sclerosis: look for CREST symptoms (calcinosis, Raynaud's pheuomenon, esophageal dysmotility, sclerodactyly, telangiectasia), heart, burn and lnask-like, leathery facies. Screening test is ANA; conflnnatory tests are anticentromere antihody (for CREST) and antitopoisomerase (scleroderma). Steroids may help. 3. Sjilgreu's syndroulc: dry eyes (keratoconjunctivitis sicca) and dry Inouth (xerOSl01111a). often associated with other autoinunune disease. Treat with eyedrops and good oral hygiene. 4. Dermatomyositis: polymyositis (see below) plus skin involvement (heliotrope rash around the eyes with associated periorbital edema is classic). Patients classically have trouble rising out of a chair or climbing steps (proximal muscles atTeeted). Musde enzymes are elevated, electromyography is irregular. Muscle biOpsy establishes the diagnosis. PatieUL<; have increased incidence of 111alignancy.

5. Polyarterit.is nodosa: associated with hepatitis B infection and cryoglobulinerl1ia. Patients present with fever. abdorninal pain, weight loss, renal disturbances, and/or peripheral neuropathies. Lab abnormalities include high ESR, leukocytosis, ane-Inia, and heIDa·turia/proteinuria. Vasculitis involves rnedium-sized vessels. Biopsy is the gold standard for diagnosis. 6. Wegener's granulcnnatosis: resembles Goodpasture's syndrorrlc. but instead of antiglomerular antibody, there is a positiveANCA titer. Look f()r nasal (nose bleeds, nasal perf(lration), lung (hemoptysis, dyspnea), and kidney (hematuria, aeute renal failme) involvement. Treat with cyclophosphamide.

1. Kawasaki's syndrOIne: affects children less than 5 years old (more C01111non in Japanese and females). Patients present with truncal rash, high fever (lasts> S days). conjunetival inject.ion, cervical lymphadenopathy, strawberry tongue, late skin desquamation of palms and soles, and/or arthritis. Patients develop coronary vessel vasculitis and snbse~ guent aneurysllls, which may thrombose and cause a lnyocardial infarction (suspect Kawasaki's disease in any child who has a myoeardial infaretion). Treat during aeute stage with aspirin and intravenous iuununoglobulin to reduce the risk of coronary ancurysIll development. 8. Takayasu's arteritis: tends to affl~ct Oriental WOlncn between 15 and 30 years old. It is called "pulseless disease" because you may not be able to fl~el the patient's pulse or 111eaSUrc blood pressure on one side. Vasculitis aff-(>cts the aortic arch and the branches that arise frorn it.. Carotid involvement may cause neurologic signs ()[ stroke, and congestive heart failure is not uncommon. Angiograrn shows the characteristic lesi.ons. Treat with steroids and/or cyclophosphaITIide. 9. BelWet's sylldrOlne: the classic patient is a l.O--something rnan with painful Oq} and geni-tal ukers. Patients l11<\Y also have uveitis, arthritis, and other skin lesions (especially ery-thema nodosum). Steroids may help.

50

Rheumatology Fibromyalgia vs. polymyositis vs. pOlymyalgia rheumatica POLYMYALGIA lUIEI1MATICA Wonlt~o: >'

Classj{"~el:sex

.SO years old

Location

'Vat.h"ms

.Pectoralan d pdv ic 'gtrdJesi nee\::

ESR

No'fJitill '

Markedly elevated (often> 100)

j\1usdt:,,'bi"()psyiBMG

N'ornftli·

Classk-findings _

Ahrloiina1

Nortriill

Elevated CpK. abnormal EMG and biopsy; greater-dsk of-ca~c{;t

'.fetnporaI-.arteritis, great resp(>Use to, stelX)'ids, vety' high EsR. elderly

Steroids

Paget's disease: a disease ofbollt' in which bone is broken down and regenerated, often simultancously; seen in patients> 40 years old, more ('oflnnon in men. Often discovered in an asymptOlnatic patient through an x-ray; be able to recognize a Pagetoid skull (frontal bossing). Classic sites of involvement are in the pelvis and skull. Watch f()f a person who has had to buy larger-size hats. Patients Inay cOInpJain of bone pain, osteoarthritis, nerve deafness, or paraplegia. Alkaline phosphatase is markedly elevated in the presence of normal calcium and phos~ phorus. The risk of osteosarcoma is increased in aflt~cted bones. Treat with NSAIDs, possibly etidronate or calcitonin for severe disease. Note: With juvcnlle RA, rhCUIllatoid factor is often negative. Watch f()f uveitis (especially in pauciarticular f()nn).

CHAP11lR

8

atology

Anemia is deflned as hemoglobin < 12 rug! ell in WOH1Cn Of < 14 lUg! dl in men. Symptoms include fatigue. dyspnea on exertion, light-headedness, dizziness, syncope, palpitations. angina, and claudication. Signs include tachycardia, pallor (especially oflhe sclera and IllUCOUS In(j'.1ubranes). systolic ejection nnumurs (frmll higb flow), and signs of the underlying cause (e.g., jaundice in hemolytic anelnia, positive stool guaiac in GI bleed). Medication history is important; lnany InedicatiollS can canse anemia through various Inechanisms. Classic examples include methyldopa, which causes red blood cell antibodies and hemolysis; chloroquine and sulfa drugs, which cause hemolysis in G~-6-PD deficiency; phenytoin, which causes Inegaloblastic aneluia; and chloramphenicol, which cau~es aplastic allenlia. Other irnportant points of the history include blood loss (trauma or surgery, melena, hematemesis), chronic diseases (anemia of chronic disease), family bistory (e.g., hemophilia, thalassemia, G-6-PD deficiency), and aJcoholisnl (which tends to caUse iron, f()late, and B u_deficiencies as well as G1 bleeds). Steps to diagnosing the canse of anemia: 1, Complete blood coLlnt (CBC) with difl'''cntial and red blood cell (Rl~C) indices. ViISt and foremost, heIllogJohin and hematocrit must be below nonnal. The meaD corpu~cular volume (MCV) tells you whether the anemia is microcytic (MCV < 80), normocytic (MCV C~ 80-100), or macrocytic (MCV > 100). 2. Peripheral smear: Look for classic findings to give you an easy diagnosis. You must know what the f(lllowing look like:

• Sickled cells (sickle cell disease) • Hypersegmcnted neutrophils (f(Jlate/BI) deficiency) " Hypochromic and microcytic RlKs (iron def\ckncy) • Basophilic stippling (lead poisoning) • Heinz bodies (G-6·PD deflciency) I!!l

HEile cells" (hemolytic anemias)

"Howell·Jolly hodies (asplenic patients) m Iron

inclusions in l{BCs of bone rnarrow (sjderohlast'ic aneInia)

"'lhrclrop-shaped RBCs (myeloflbrosis) Wi

Schistocytes, helmet c('Hs, aud fragmented RBCs (intravascular hemolysis)

" Spherocytes and elliptocytes (lwrcditary spherocytosis/elliptocytosis) M

Acanthocytcs!spur cells (ahClaHpoprotcincmia)

51

52

Hematology

• Target cells (thalassemia, liver disease) • Bchinocytes an d burr cells (uremia) • Polychromasia (hom reticulocytosis; should alert yon to possibility ofhemolysis) .Rouleaux I,mnation (multiple myeloma) II

Parasites inside RECs (malaria, babesiosis)

3. Reticulocyte index (RI) should be> 2% with anenlia; otherwise, dw marrow is not responding properly. A reticulocyte index> 3%) should lnake you think of henlolysis as the cause (the marrow is respondiug properly, so it is not the problem). Witllthese three parameters, you can make a reasonable differential diagnosis if the cause is not obvious.

Other clues to the presence ofhemolytic anemia: II

Elevated lactate dehydrogenase

II

Elevated bilirubin (nnconjugated as well as conjngated if the liver is working)

iii

Jaundice

II

Low or absent haptoglobin (intravascular hemol ysis)

I!Il

Positive urobilinogen, bilirubin, hClnoglobin in urine (only conjugated bilirubin appears in the urine, and hCITwg]obin appears only when haptoglobin has been saturated, as in brisk intravascular hemolysis)

Causes of anemia: 1. Iron deficiency (hypochromic, microcytic): the most common cause of anemia in tbe U.S. Look for low iron/ferritin level, elevated total iron-binding capacity (TIIlC; also known as transferrin). and lowTIBC saturation. Rarely patients have a craving for ice or dirt (pica) or Plummer~VinsoJlsyndrome (esophageal weh producing dysphagia, iron deflciency anemia, and glossitis). In a patient over 40, rule (Jut colon cancer as a canse of chronic blood loss. Iron deficiency anemia is COlTUl1on in women of reproductive age hecause of m.enstrual irregularities. Give i.ron supplements to all i1lfants except full·-term infants who are exclusively breast-·fed; giving COWlS rnilk before 1 year of age fIlay cause anemia through CI bleeding. Start iron supplernentation at +···6 rnonths fbr fuIJ·-tenn in·· {ants and at 2 months fe)l' preterm infants. Iron supplements also are cornrnonly gjven during pregnancy and lactation because of increased demand. To treat iron deHeieney anemia, correct the underlying cause if possible and t.reat with oral iron supplelnentatjoll for roughly 6 months.

Hematology

53

2. I:olate deficiency (rnacrocytic): cornrnonly seen in akohol:ics and pregnant ·women. All women of reproductive age should take folate suppleUlents to prevent neural tube deft~cts. Rare causes include poor diet (e.g., tea and toast), rnethotrexate. prolonged course of trinlethoprinllsulfaulethoxazole, anticonvulsant therapy (especially phenytoin). and Illalabsorption.l.ook f()f macrocytes and hypersegmenteclllcutrophils (even one should make you think of the diagnosis) md low f()Jate levels (serum or RIle). Treat with oral folate. 3. Vitamin B n deficiency (rnacrocytic): Illost commonly due to pernicious allenlia (anti~ parietal cell antibodies). Relnember the physiology ofB]2. absorption and the association with vitiligo and hypothyroidism. Other causes include gastrectOIny, tenninal ilemll re~ section, diet (strict vegan), chronic pancreatitis, and Diphyllobothrium lalUm (fish tapeworm) infectioll. Peripheral smear looks the same as in f()1ate deficiency (olacrocytes, hypersegnwnted neutrophils), but the patient has neurologic defIciencies (loss of sensation or position sense, paresthesias, ataxia, spasticity, hyperreflexia, positive Babinski sign, de~· Tllcntia). Look f()r low serum B n , achlorhydria (no stornach acid secretion, elevated st0111 aeh pH). and antibodies to parietal cells. A Schilling test usually determines tbe etiology. Usual replacement route is intranluscular, because most patients cannot absorb Bu.

4. l'halasseluia (microcytic, hypochromic): Inust be differentiated froln iron deficiency. Iron levels are Donnal in thaiasselnia; iron is contraindicated because it may cause overload. Look I()r elevated hemoglobin A}. (f3-thalassemia only) or hemoglobin F (13thalassemia only), target cells, nucleated RHCs, diffuse basophilia 011 peripheral smears, K-ray of the skull showing a "crew-cllt" appearance, splenolnegaly, and positive family history (ul()re COIlllnOil in blacks, Mediterraneans, Asians). No trtannent is required for ruinor thalassemia; patients often are asymptOInaticas they afe used to living at a lower level of hernoglobin and helnatocrie Thalassernia Tuajor is more dramatic and severe. Treat with as-needed trallsfusions and iron chelation therapy to prevent hernochroInatosis. Diagnosis is Inade by hernoglobin electrophoresis. 'There are f(-Jur gene loci for alpha~chain and only two fe)r heta···chain thalassemia. Alpha thalassemia is symptOlnatic at birth, or the fetus dies in utero (hydrops); beta thaiasselnia is not SYIllptomatic until 6 Illonths of age. 5. Sickle cell aneluia: smear gives it away. Look few very high percentage of reticulocytcs. Sickle cell anemia ahnost always is seen in blacks (80Al are heterozygotes in lIS.). Watch for classic rnanifestations of sickle cell dise,ase: III

Aplastic crises (due to parvovirus B19 infection)

1m

Bone pain (due to n1icroinfarcts; the classic exanrple is avascular necrosis of the femoral head)

m Dactylitis

(hand-foot syndrome; know what it looks like)

IIlI

Renal papillary necrosis

iii

Splenic sequestration crisis

!!II

Autosplcnectomy (increased

Ii!!l

Acute chest syndrome (mimics !)neUlllonia)

Ill!

Pigment cholelithiasis

fill

Priapisrn

!!II

Stroke

illf(~ctions

with encapsulated bugs)

Diagnosis is made by heIl1oglobin electrophoresis. Screenirl.g is done at birth, but synlptoms usually do not appear until around 6 months of age because of lack of adult

54

Hematology hernoglobin production. Treat with prophylactic penicillin (start as soon as the diagnosis is lnade), proper vaccination (including pneumococcal vaccine at the age of 2 years old), f()late supplementation, early lrealUlent of inf(~ctions, and proper hydration. Sickle crisis is characterized by severe pain in various sites due to RBC sickling. Treat with oxygen, lots of IV fluids, and analgesics (do not be afraid to use narcotics). Consider

transfusions jf syrnptoms and/ or findings are severe. 6. Acute blood loss (normocytic, normochromic): innnediately after blood Joss, hemo-globin and hematocrit arc nonnal; it takes at least 3---4 hours (often more) f()f reequili-bration. l.ook fbr pale, cold skin; tachycardia; and hYIJotellsion. Transfuse if indicated, even with norn1al hcrl1oglobin and herl1atocrit in the acute setting.

1. Autoimmune hemolytic anemia (nonnocytic, normochromic)"-----can have multiple etiologies: lupus (or IIleds that cause lupus, hke procainamjde, hydralazine, and isoniazid), drugs (classic is methyldopa, also peN/ cephalosporins/ sulfas and quinidine), leukclllia/lympholl1J or il1fl:ctioll (classic is Mycoplasm(J, also EBV and syphilis). Coornbs' test is positive, IndY have spherocytes due to incolnplcte IlIJCrophage destruction in extravasclllarhernolysi s

8. Lead poisoning (hypochromic, rnicrocytic): classically seen in children. With acute poiscming, look f(H' vOlniting, ataxia, colicky abdOlninal pain, irritability (aggressive, behavioral regression), and encephalopathy, cerebral edema, or seizures. Usually, however, poisoning is chronic and low~level; look f()f pica (especially paint chips and dust in old buildings, which may still have lead paint); residence in an old or neglected building; residence near alead--slneltillg or battery-~recyding plant; family lnelnbcrs

who work at slIch plants; basophilic stippling; and elevated free erythrocyte protoporphyrin (FEP). Screening asymptomatic children f"r serum lead level at I and 2 years of age is ilnportant (screen at 6 1110uths if risk factors are present) because chronic low·-levcllead exposure Inay lead to pern1allent neurologic sequelae. Screen and Inea-sllre symptomatic lead exposure with serllm lead levels (should be < 10 fig/ cil). Treat with decreased lead exposure (best and nrst treatlucllt) as well as as-needed lead chelation therapy (succimer in children, dimercaprol in adults; in severe cases, use diJnercaprol plus edetate f(Jr children or adults).

9. Sideroblastic anemia (ulicrocytic, hypochrornic): increased or normal iron, ferritin, and TIBe saturation (which distinguish it: from iron defIciency), polychromatophilic stippling, and the classic !l ringed sideroblastl! in the bone rnarrow (know what it looks like). Sideroblastic anelnia Inay be related tOl1lyclodysplasia or future blood dyscrasia. Manage supportiveIy; in rarc cases the anemia responds to pyridoxine. Do not give iron!

10. Anemia of chronic disease (Illicrocytic, hypochromic, or nonnocytic): look

£(H dis,,· eases that cause chronic inflammation (rheumatoid arthritis, lupus erythematosus, cancer, tuberculosis). Senun iron is low, but so bTIBe (thus, the % saturation may be nearly normal). Scnull ferritin is elevated (because ferritin is an acute--phase reactant, the level should be increased). Treat the underlying disorder to correct the anenlla. Do

not give iron!

11. Spherocytosis (nonnochromic): look fbr spherocytes, family history (autosolnal donli-nant) , splenomegaly, positive oSluotk fragility test, and increased Inean corpuscular heIlloglobin concentration. Treatment often involves splenectomy. Spherocytes also nlay be seen in extravascular hemolysis, but llle osmotic fragility test is nornnl.

12.

Chroni(~

renal disease: the kidney produces erythropoietin; thus, you rnay give erythropoietin in end-stage renal disease to correct the anemia.

Hematology

55

13. Aplastic anemia: usually idiopathic; may be caused by chemotherapy, radiation, malignancy (especially leukemias), benzene, and I1lCdications (chloranlJlhcnicol, carbaIIlJZepine, phenylbutazone, sulh drugs, zldovudine, and gold). Look fe)f decreased white blood cells and platelets to accompany anemia. Stop any possible causative medication; then try antithynlocyte globulin or bone rnarrow transplant. 14. Myelophthisic aneluia: usually due to rnyelodysplasia/myelofibrosis or malignant invasion and destruction of bone rnarf(lW (most connnon cause). Look f()r lnarke.d anisocytosis (different size), poikilocytosis (chm,rent shape), nucleated RBC5. giant and/ or hizarre~lookillgplatelets,and teardrop--shaped RBCs on the peripheral Slllcar. A hone lnarrow biOpsy is usually done and rnay reveal no cells ("dry tapt! because marrow is Hbrotlc) or Inahgllant--Iooking cells. 15. G-6-PD deficiency: X-linked recessive (males affected); most common in blacks and Mediterrancans. Look {()r sudden hunolysis or anemia after fava bean or drug exposure (antiJnalarials, salicylates, sulfa drugs), or after infhtion or diabetic ketoacidosis. Heinz bodies and bile. cells also are seen on peripheral Slnear. Diagnosis is nnde with RBC enzyme assay. Do nol perf(Wl11 the assay illllnediatdy after hcnlOlysis~you Inay get a false negative ,,'suit because all of the older 1\BCs already have been destroyed and the younger RBCs are not afI(~cted. 'Treat by avoiding precipitating foods and rnedicatic)ns. Discontinue the tJiggering medication firstl

16. Other canses: endocrine failure (especially pituitary and thyroid); mechanical valves (whIch hemolyze RBCs); disserninated intravascular coagulation, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrorne (look f()f schistocytes/RBC frag-· ments and appropriate otber flndings); other hemoglobinopathies (hemoglobins C and E fajrly common); paroxysmal nocturnal or cold hemoglobinuria; Clo,stridium perfringens, mabria, babesiosis, and hypersplenism (always aCCOJnpanied by spleuOlnegaly and often by low platelets and whitc blood cells).

Transfusions: always based on clinical grounds. Treat the patient, not the lab value; there is no s,\lch thing as a "trigger value" for transfusion. Different: blood components have different indications:

1. Whole blood; used only !()f rapid, nlassive blood loss or exchange transfllsioIlS (poison-ing. throlnbotic thrombocytopenic purpura) 2. Packed RBCs: used instead of whole blood when the patient needs a transfllsiofl 3. Wasbed RBC" free of traces of plasma, whlie blood cell" and platelers; good f,)r IgA de· Hciency and allergiC or previously sensitized patients 4. Platelets: given f,)r symptomatic thrombocytopenia (lisually < 10,000//11) 5. Granulocytes: rarely used f()r neutropenia with sepsis caused by chemotherapy 6. l
7. Cryoprecipitate: contains fibrinogen and factor 8; llsedin hemophiha, von Willebrand disease, and disserninated intravascular coagulation

The 1110st common cause of blood transfusion reaction is lab error. Type 0 negative can he llsed wIlen you can:not wait felf blood typing or the blood bank docs not hav\,:' the patient's type. If a ITansfllsion reaction occurs, the first step is to stop the Imns{usiou! Look f()r f(~brile reaction (chills, f(~ver. headache, hack pain) frorH antibodies to white hlood cells; hernolytic reaction

56

Hematology

(anxiety, discomfort, dyspnea, chest pain, shock, jaundice) from antibodies to RBCs; or allergic reaction (urticaria, edema, dizziness, dyspnea, wheezing, anaphylaxis) to an unknown component in the donor serum. Patients with associated oliguria should be treated with IV fluids and diuresis (mannitol or furoserrtide). Massive transfusions Iuay lead to bleeding diathesis frorn thr0111bocytopenia (look for oozing frorn puncture/IV sites) and citrate (calcium chelator). Patients also may develop hyperkalemia. Disseminated intravascular coagulation (DIC): most commonly due to pregnancy and obstetric complications (50%), malignancy (33'%), sepsis, and trauma (especially head trauma, prostate surgery. and snake bites). DIC usually Inanifbts as bleeding diathesis. Look few the das,· sic oozing/bleechng fron1 puncture or IV sites, but patients may have thrombotic tendencies. Look f(lr prolonged prothrombin time (PY) , parita] throm boplastin time (PTT) , and bleeding time (B1'); positive I)-dimer and increased ,fibrin degradation products; thrombocytopenia; and decreases in fibrin and dotting factors (including factor 8, which is normal in hepatic necrosis). Trealthe underlying cause (evacuate uterus, give antibiotics). Patients may need transfusions, fresh frozen plasma, or, rarely, heparin (only in the presence ofthn)111bosis). Eosinophilia: causes include idiopathic etiology, allergy, eCZCllla, atopy, angioedema, drug reo actions, parasitic infections, blood dyscrasias (especially lymphoma), Loffler's syndrome (pulInonary eosinophilia), autoilnmune diseases (e.g., lupus erythematosus, rheUlnatoid arthritis, inflammatory bowel disease), IgA def\ciency, and adrenal insuf!lciency.

Basophilia: think of allergies, neoplasm, or blood dyscrasia. Bleeding problems: lupus anticoagulant may cause a prolonged PTT but the patient has a tendency toward thrombosis. Look for associated lupus, positive Venereal Disease Research Laboratory o,r rapid plasma reagin test for syphilis, and I or history of miscarriages. Defkiencies in protein C. protein 5, or antithronlbin III also may cause increased tendency toward throIllbos1s. Patients arc treated with anticoagulant therapy to prevent deep venous thrornbosis, pul-lllonary clnbolis111, and other cornplications. Clotting tests: PT for extrinsic system (prolonged by warfarin), PTY f(l[ intrinsic system (prolonged hy heparin), and liT f(l[ platelet flmction:

ThrOInbocytopenia Hlay be caused by idiopathic thrornbocytopcllic purprua. thrOlnbotic tluombocytopcnic purpura, henlolytic uremic syndrome, disseminated intravascular coagulation,

Hematology

57

HIV. splenic sequestration, heparin (treat by first stopping heparin), other medications (especially quinidine and sulfa drugs), autoimmune disease, and a!eohoL Bleeding from thrombocytopenia is in the form of petechiae, nose bleeds, and easy bruising. Note: Do not give platelets to a patient with throD1botic thrombocytopenic purpura or associated thrOlnbocytopcl1ia. They may cause thrOl11bosls.

heparin~

Vitamin C deficiency (scurvy) may canso bleeding similar to that seon with low platelets (splinter and gum hemorrhages, petechiae); porif()llicu]ar and subperiosteal hemorrhages are unique to scurvy. Patients have a poor dietary history (the classic example is hot dogs and soda), myalgias and arthralgias, and capillary fragility. Bleeding is due to collagen problems in vessels. Treat with oral vitalnin C. Bleeding tendency also Inay be due to inherited connective tissue disordt~r (Ehkrs--Danlos syn-drome, Marfan syndrorne, pseudoxanthoma elasticum, osteogenesis imperfecta) O[ chronic steroid use, but it is rarely a clinical problem.

Oncology

Blood Dys<:rasias TYPE

AGE (Yl\)

Children (peak ~

> 30

3~5)

WHAT TO LOOK fOR IN CASE DESCRIPTIONITRIGGElI WORDS ----~ i)~ncyto1?(:'uia, (bleeding,' fever, -anemia;) l,histOry 'pf radiatknl t.~xpo$ure; Pl>Wu.sj'iidrt)me

- P-aticyJopenia: (flleclling;)'ev:er; aX1~mia},:l\tict rods., (ijS$t"lIllriatedin;tra-~ yagcufar.\;t1ag1l1at}oll

-

3(HO > 50

H~itXce1l'Jeil~e~l.ia

_

'

Mytosis}u:ngoidcsVS6~ry: syn_dro~l~_-

Adults

> 50

,blood

it:U$---{in-Afrk~)

Children Adults H6dgkJIJ:

cr Yitl;l~zMDS

Adtilts

Hutnfin T:cql1l~u-keJn'fA Yir\.ls~ Lis i\ -cau.se

I S-34

-Reed-S.mrn:bexg ~w.tt, (;_erYlf~llyn'q?hadellopatll% __njght sweats

NOl1-'fToj::lgkhj':i,lymiJ hO'!U,i

_Sm~ll-, l()l1kl,1at'typ~:l)as". -GI tractJllay. be-loti~tinn,of'p

MultIple myeloma

osis;.. largeJ dijfu$Q Wt)€ is ',\'i,':orst,. tumor

> 50

!\iieJ:uia,.teitrdf:QJ) ceUs,;: °qtY.tart :~n p()Tje rriarrow hlO:psy; 111gh: Ji1.c;m G?rp.uscr(]ttr :vohuJ:lC and re~ blv,0
>+0

Oence-Jo

> 4'.0

(ein (lgG " 50%, IgA ~ 25%), osteolydc 1«10"',

H?'rtervh;(:o8,i~Y.}~iN{sptke> colq NHIluttOliis. (R.ayna:t!d:~, -:ph~~rlJiineJi,Qri vtith cptd'serlsitiVity) Tligh'heiliatdcflt,' henldgl'tj&iri,. ptniihiS' (¢spedally,.an~r no"tbatll' (;it shQvv-erJ;'.use-.phJI?hotol1w

> >

so

Rlatdet-:£Oltni' \is1J ,t..oOO;bOO; may, have: bleeding or, tbiombosiK w

Cam:er Statistics (See tahle, top of next page) Important points: 1. In childre-n and yOlwgcf adults, leuK\:',rnla is the most common cancer. Remember, how·e-vcr, fhat (lflc has the most signiHgant 'ilnpacl on the inC'idcJlcc and mortality rittc of cancer.

59

60

Oncology

EEMALE

MALE

J. J;!fQ.'it'ate

-1; :Breasl

1,-Lung

l.l.u.ng

2. Lung

.2._Ltl~i_g

1. Plo~tate

;LJ~reast

3. Colon-

3:.'0bloii

3--<;taLon

3: Colon

'The incidence of cancer in tbe U.S. [oughly doubles every 5 years after age 25; thcref()re, cancer most cornlllonly afh'cts older adults. 2. In most organs, the most comIllOn Inalignancy is metasl.Mic Do not be fooled into saying that hepatocellular cancer is the rnosl. common mal ignancy of the liver if nlctastatic cancer is a choic('. Unless the question specif-ically asks for a primary tumor, do not aSSllll1C that a primary tumor is the answer. 3. Metastases 10 tll{' spine can cause cord cOlnprcssion (locaJ spinal pain, reflex changes. weakness, sensory Joss, paralysis). Cord compression is an nucrgency, and tht::, fhst step is to start high"dose corticosteroids. Then get an MRL The next step is to treat with radi"atiOll. Surgical decompn'ssion is llsed if radiation fails or the tumor is known not to be radiosensitive. Prcnnpt interVl'lltion is essential, and outcome is closely linked to pretreatrnent function.

Genetic predisposition to cancer

:RerinobJast(imaMEN, typ" lla

Aut08()trial-dbminan:r - AU1:01>01n,il-do-mitiant

MEN, 'ype JIb

A'utoscimal- domill~\.itt

FarnH-(al polyposis coIF

Aut6so:rtl.al-"dtJltiiffant AiltOs'cima.!-dotl1i)_iant

MEN, type I

Gai:diii~J

sYndroJl1¢

TUrcot syixdtbrne

AUt05Qthal (19ni~.rl'ahf

l~eutz"'Jegher&

Auto$()-mal.d(~minal1t

_synoroine

Neur type 2 TU:beY(;hiS sClerQsis

VOlllllppel:,Lirvftail _ disease Xe-r~)'de~:ma--pi'gtnentosillri

_AiltQ$()Jllal recess.iVe

Skin caXlcer

Albillfsm

}\ut()s9lnal recessiYe

Skih c-alicere

Down sylidromg

'I'riS(lIh

MEN -~

ri:lUltipH~

t\ndocrine-.ne,6p!asia.

or

Other diseases with increased incidence cancer arc in1J1H1llodeflcicllcy syndrOJ)lc's, Bloom syndrome, and Fancol1i allcrnia. Breast, ovarian, and colon Cancer have well--known hmjlial tendencies (along with smne other types of cancer), but rarely call a Mendelian i1l1w.ritallcc pattern be dctllOTlSlratcd (yet).

Oncology

61

Avoidable risk factors fi,r cancer development

Smqk±I1g, asbcsws' (also nickel~ tadon,- toiU,-ars!ZlJic,.<:hrom1tun,. Hl'illiium)

Lmlg

Ashmifos

Mesot1jelioni"

_ Che~10iheral))\ Tadidtheral'Y}_9thct-- inlln1JuosUptH'0ss1've ~r~gs, hel)u:-rif

I,c:ul;ginia lliadder

Sm?kJ.ng, ~rnili:r:t,"-{lr~s (rubher a~Hi_ d;ye_t11du~try), schbWJsonJlasis (in .iuunigtants)

Skiri -Liver,

t1:hl'aiilokt: imhtfxposuJ? (e;g,,:&lUl~ i __coai .tar, -iltSeJljc Ah~bhoJ; vinyl fnl(}j~i-de' (liY0tAngios;(t.t.()ni".s), $.l;1tOKlJ,}S

Onil-'caVity

Sroykin
P1Jctr-ym:,;-XI~rynx

Sm(~kiIig,_jJROh(Al

Esophagus Pancreas-

Sl:noking; Ji:'kohOl

Renal_cell

S-~i:lOking

SlHC)k.lilg Ak6hol,Jlitrt;).sin'nbjis,-riitrites (froni sri)ok~'(r Meats -alid fish)

S.tomac;h

MQtlu:rs,should avoid -dietnylstm)e$(roJ (DES) d\lring-pregitanc'y High-f~t_ami lovv:,:fibef di~~t

Cer:vical

HlgIi··fat arid low:flber dh;~t Smoking; -iex,-high' parity

'phyroid

Chjl(lh()odhead~_ n~c~,oE

Bl.'0
che(iJ jrl,:adiario.h

}'<;11dorm:trl;l1

t-lriopp()!ie_d t:,str:Qgpit s.timula"tiol1:, {)QCf:;i.ty

An Ci,v):cer 'overall

Sm()king (st;eoitd hi probably akohoI) ,

LUNG CANCER Lung cancer is the nUlnher--one cause of overall cancer lnortality ill the u.s. The incidence is rising ill WOlIlen (due to increased smoking). Look for change in a chronic cough in a sillokcr. The rnor(' pack years of tohacco lIse, the [nort' suspicious you should he. Patients also may present with hemoptysis, pneumonia, and/or weight Joss. Chest x--ray Inay show pkural CffllSiou; put a needle jn the fluid and examinc for 1l1alignant cells. After chest x,~ra}', get a tissue biopsy to confirm the diagnosis and define the histologic type. NUll--small cell cancer Illay be treated with surgery if the cancer rcmahls within .-he IUJlg parenchyma. SmaU cel! callcer is treated with chel110therapy only; early lnetastascs Illakc surgery inappropriate.

Weird and frequently tested consequences ofhmg cancer: 1. Horner's syndrol1w: from invasion of cervical sympathetic chain by an apical (Pancoast) tmnor. Look for unilateral pt-osis, miosis, and anhidrosis (no sweat.ing) 2. l)iaphraglll paralysis: from phrenic nerve involvement 3. Hoarseness:

frOID

recurrent laryngeal nerve involvement

4. Superior vella cava syndr01ne: look for edema and plethora (redness) nfthe neck and face

and central lWfVOtiS system symptoms (headache, visual symptoms, altered mental status); dUl' to compressioJl ofsllperior vena cava with impaired venous drainage 5. Cnshing's syndrornc: {-~om adrcnocorticotropic hormone production by a sl11a11 cdl carcinoma 6. Syndrome of inappropriate secretion of antidiuretic hormone: h0111 JllitdiurHic hormOlle production by a small cell carcinoma

62

Oncology

7. I-Iypcrcalcernia: fronl bone Tlletastascs or production of parathyroid honnone by a squa-· nIOUS cell carcinorna 8. Eaton--Lalnbert syndrome: lnyasthen:ia gravis--hke disease due to lung cancer that spares the ocuIar muscles; the nmsc1es become stronger with repetitive stilliulation (opposite of 11lyasthenia gravis) Solitary pulmonary uodule ou chest x~ray: the first step is comparison with previous chest x-rays. If the nodule has remained the sanH:~ size fC)f > 2 years, it is not cancer. If no old films afC available and patient is older than 3S or has a long sIllOkinghistory, get a biOpsy of the nodule (via hronchoscopy or transthoracic biopsy if possible) for tissue diagnosis. If the patient is younger than 3S or has no s1110king history, the cause is rHost likely infectious (tuberculosis or fungi), hamartoma, or collagen vascular disease. The patient Inay undergo coreful observation and follow--up with repeat chest x---ray.

BREAST CANCER Incidence: roughly 1 in 10 WOlnen will develop breast cancer in their hft;ti111C. Risk factors for breast caucer: 1. History of breast cancer (biggest risk !actor) 2. Family history in flr.st·-degree relatives

3. Age (breast cancer is rare before age 30; the incidence increases with age). Greatest risk in WOlnen > 75 years old.

4. Early ntenarche, late lnenopause, and late fIrst pregnancy or nulliparity (more menstrual cycles :~.:::: more risk) 5. Atypical hyperplasia of the breast 6. Radiation exposure before age 30

J. Prolonged use of oral contraceptive pills (> 5 years) only if nulliparous or hel()re first pregnancy (controversial) Signs and symptoms that suggest a mass is breast cancer until proved otherwise: fIxation of breast Inass to the chest wall or overlying skin, satellite nodules or ulcers on the skin, lym-phcdema/peau d'orange, matted or flxed aXillary lymph nodes, inflarnmatory skin changes (red, hot skin with enlargeJnent of the breast due to inflanunatory cancer), prolonged unilateral scaling erosion of tbe nipple with or without discharge (may be Pagct"s disease of the nipple), rnicrocalciflcations on mannl1ography, and any new brensl moss in a poslmenoptlllS(l! woman.

The conservative approach is to biopsy every palpable breast lnass in WOHlerl over 35 when in doubt, especially if they have any risk factors. If the hoard question docs not want you to biOpsy the mass, it will give you dues that: it is not cancer (e.g., bilateral, lmnpy breasts that hecolne symptornatic with every rnenscs and have no dorninant lnass, age < 30). Important points: 1. In women under 30, breast cancer is extremely rare. With a discrete breast ITlaSS in this age group, think of Hbroadenmna and observe the patient over a :f(~W rnenstrual cycles bcfbre considering biopsy, Fibroadenomas arc usually roundish, rubbery"fceling, and freely movable. 2. The most common histologic type of breast cancer is invasive dnctal carcinoma.

Oncology

63

3. In patients with a palpable breast mass, the decision to do a biopsy is a clinical one. A Iuammogram that looks benign should not deter you from doing a biopsy. On the other hand, a lesion that is detected on mammograpby and looks suspicious should be biopsied, even if not palpable (needle localization biOpsy),

4. Do not do mamnlograms in WOll1en under 35 (breast tissue too dense to see cancer).

5.

raInoxlfi~n

(or other endocrine therapy) generally ilnproves survival jf the turnor is estrogen receptor-positive (FR+) and even more so if the tumor is also progesterone rcceptorpositive (PR +).

6. Mastectomy and breast-conserving surgery plus radiation are considered equal in efficacy. In either case, do an axillary node dissection to deternline spread to the nodes. If nodes are positive, give chemotherapy.

PROSTATE CANCER Risk factors:

1. Age (not seen in men < 40 years old; incidence increases with age; 60% of men> 80 have prostate raIlcer) 2. Race: black> white> Asian Patients present late, because early prostate cancer is asynlptomatic Look fe)f SyUlptOlllS sugges-· tive of benign prostatic hypertrophy (heSitancy, dysuria, frequency) with hematuria anellor elevated prostate-specific antigen (PSA) or acid phosphatase. Acid phosphatase is elevated only when the cancer has broken through the capsule; ior this reason, it was replaced with the lllore sensitive PSA as a screening tool. Look for prostate irregularities (nodule) on rectal exam. Patients also commonly present with hack pain from'vertebral metastases (osteohlastic). Local prostate cancer is treated with surgery (prostatectomy). Patients with 111etastases have sev~ eral options for horulo11al therapy: orchiectomy, gonadotropill~releasiIlghormone agonist (leuprolide), androgen-receptor antagonist (flutamide), estrogen (diethylstilbestrol), anel others (e.g., cyproterone). Chemotherapy docs not work, and radiation therapy is used for local disease or pain frOlll bony lnetastases.

COlORECTAl CANCER Risk factors: 1. Age (incidence hegins to increase after age 40; peak incidenre between 60·-75 years) 2. Family history (especia]]y with familial polyposis or Gardner, 111rcot, or Lynch syndrome) 3. Inflammatory bowel disease (ulcerative colitis> Crohn's disease, hut both increase risk) 4. l.ow-fiber, high-fat diet (weak evidence)

Important points: 1. Patients 11lay present with aSyLuptolllatic hlood in stool (visihle streaks of blood on stool or gllaiac~positive), anerrlia with right~sided colon cancer, change in stool caliber ("pencil stool") or frequency (alternating constipation and frequency) witb left-sided colon cancer. As with any cancer, look fe)f weight loss. 2. Occult blood in tbe stool of a patient> 40 years old should be considered colon cancer until proved otherwise. Tb rule out colon cancer, either do flexible sigmoidoscopy and a

64

Oncology harimll enema Of do a total cololloscoP)( If you see any lesions with a flexible sigrl1oidoscope or barimD enema, you need to do a total colonoscopy with rernoval and histologic exarnination of all polyps/lesions. For this reason, most people now start with colonoscopy. 3. Carcilloembryonic alltigm (eliA) is often elevated with colon cancer. and a preoperative level is usually measured, After surgery to renlove the tumor, CEA should return to normal levels. Periodic rnonitoring of CEA postoperatively helps to detect recurrence before it is clinically apparent. CEA is not used as a screening tool for colon cancer; it is used only to follow known cancer. 4. Treatnlent is pril1larily surgical, with resection of involved boweL Adjuvant chClDotherapy is sometimes done with 5-fluorouracil (5-FU) and levamisole or leucovorin. 5. Colon cancer frequently metastasiz.es to the liver; if the metastasis is solitary, surgical resection is often attempted. With I1letastases elsewhere, chenl0therapy is the only option. Prognosis is poor.

6. Colon cancer is a common cause of a large bowel obstruction in an adult.

PANCREATIC CANCER II

Classic presentation: a smoker in the 40-80-year-old range who has lost weight and is jaundiced. Symptoms may inclnde epigastric pain. migratory thrombophlebitis (Tronsseau syndrome. which al", may he seen with other visceral cancer), or a palpable, nontender gallbladder (Courvoisier's sign)

II

Epidemiology: males> females. diabetics> nondiabetics, blacks> whites

• Surgery (Whipple procednre): rarely curative; the prognosis is dismal • Cell oforigin: dnctal epithelinm Islet cell tumors: 1. Insnlinoma (beta cell tumor): most commOn islet cell tumor. Look for two-thirds of Whipple's triad: hypoglycemia (glncose < 50 mg/dl) and central nervons system symptoms due to hypoglycemia (confnsion, stupor. loss o[ consciousness). As the good doctor. yOlI will provide the third part o[Whipple's triad: give glncose to relieve symptoms. Ninety percent of insuhumuas are benign and cured with resection, if possible. In your work-np. take history and check C-peptide first to make sure that the patient is not a diabetic who accidentally took too much insulin or a patient with factitious disorder; C-peptide is high with insulinorna, low with other conditions. 2. Gastrinoma: Zollinger..ElJison syndrome is gastrinoma plus acid hypersecretion and peptic nicer disease (gastrin canses acid secretion). Peptic nlcers are often multiple and resistant to therapy; they may be in an unnsnallocation (distal dnodennm or jejunnm). More tban one-half are malignant. 3. Glucagonoma (alpha cell tumor): hyperglycemia with high glucagon level and migratory necrotizing skin erytheu1a.

OVARIAN CANCER Ovarian cancer usually presents late with weight loss, pelvic luass, ascites, and!or bowel ob-struction in a postnlenopausal woman. Any ovarian enlargement in a postulenopausal WOl1UIl is cancer until proved otherwise. In WOInen of reproductive age, most ovarian enlargements arc

Oncology

65

benign. Ultrasound is a good tlrst test to evaluate an ovarian lesion. Treatn1cnt includes debulklng surgery and chemotherapy; prognosis is usually poor. Most ovarian cancer arises [1'0111 ovarian epithelium. Serous cystadenocarcinoma, the Inost connnon ovarian cancer, often has psammoma bodies on histopathology.

Germ cell tumors lnake good questions: 1. Teratmna/ dermoid cyst: look for a description of the tm110r to include skin, hair, and/or teeth/bone; lllay show up on x-ray. 2. Sertoli-Leydig cell

tUITlOf:

causes virilization (hirsutism, receding hairline, deepening

voice, clitoromegaly). 3. Granulosa/theca-ceH tun1or: causes feminization and precocious puherty. Terms worth knowing: iii

Meig's syndrome: ovarian fLbroma, ascites, and right hydrothorax.

III

Krukenberg's tlnIlor: stmnach cancer with Inetastases to both ovaries.

Note: Oral contraceptives have been shown to reduce the incidence of ovarian cancer by 50%; they also reduce endOInetrial cancer.

CERVICAL CANCER Papanicolaou smears decrease the incidence and rllortaHty of cervical cancer. Give female pa-· tients a Pap SUlCar if they are due, even if they present with an unrelated cOIuplaint.Follow up any dysplastic Pap smear with colposcopy-directed biopsies and endocervical curettage. If the Pap SHlear shows luicroinvasive cancer, proceed to conization. Frankly invasive cancer needs surgery and/ or radiation. Risk factors for cervical cancer: 1. < 20 years old at first coitus, preguancy, or marriage 2. Mnltiple sexual partners (role ofhnman papillomavirus aud possibly herpes) or coitus with a promiscuous person 3. Smoking

4. Low socioeconomic status 5, High parity (which protects against endometrial cancer) Important points:

1. Invasive cervical cancer begins in the transformation zone and usually presents with vaginal bleeding or discharge (maybe postcoital, intermenstrual spotting, or abnormal nlen~ strnal bleeding). 2. Treat with surgery and/or radiation. 3, Maternal exposure to diethylstilbestrol causes daughters to get clear cell cancer of the cervix or vagina.

UTERINE CANCER Postm,enopausal bleeding is cancer until proved otherwise; endometrial cancer is the roost cornmon cancer to present in this fashion (f()urth rn05t cornrnOl1 cancer in women). Get an cndOlTIetrial biopsy for any patient with posuuenopausal bleeding (as well as a Pap srnear and

66

Oncology endocervical curettage). Any wonlan with unexplained gynecologic bleeding that persists needs a Pap smear, endocervical curettage, and endometrial biopsy.

Risk factors for endometrial cancer: 1. Obesity 2. Nulliparity 3. Late rncllopallse

4. Diabetes mellitus

5. Hypertension 6. Gallbladder disease 7. Chronic. unopposed estrogen stimulation, as in polycystic ovary/Stein-Leventhal syndrome, estrogerh,ecreting neoplaslll (granulosa--theca cell tumor), and estrogen replacement (increases risk of cancer only if taken without: progesterone).

Important points: 1. Oral contraceptives have been shown to reduce the incidence of uterine as wen as ovarian cancer. 2. Most uterine cancer is adenocarcinoll1a and spreads by direct extension.

3. Treat "ith surgery and radiation.

MISCELLANEOUS NEOPLASMS Brain tumors: ill adults two-thirds of primary turnors (lnetastases are more COInmon than primary turnoys) are supratentorial, whereas in children two-thirds are infratentorial (posterior fossa/ cerebellar). In either group, look fbr new,·onset seizures, neurologic deficits, or signs of intracranial hypertension (headache, blurred vision, papilledema, projectile nausea and vomit.. ing). In children, also look for hydrocephalus and ataxia. The most COmmon type in adults is glioma (most are intraparenchymal astrocytomas with little or no calcification), followed by meningioma (usually calcified, external to the brain substance). In children, the 1110St (OrrnDOn types are cerebellar astrocytOlua and rnedulloblast0111il, f()l1owed by epeudynl0ma. Treatment is surgical removal, which may be followed by radiation and chemotherapy. depending on the WnWf. Important points to renlenlber: 1. A yonng, obese woman who has headaches, papilledema, and vomiting with a negative CT/MRI has pseudotumor eerebri, not a malignancy. 2. The most common posterior fossa tumors in children are astrocytoma and medulloblastoma; in adults, it is acoustic neuroma (wateh for neuroflhromatosis). 3. In children, watch f')f eraniopbaryngioma (a remnant of Rathke's pouch). It is heavily calcifled altd shows up on skull x..ray (most likely tumor in children if it shows lip 011 skull x-ray). Testicular cancer: the most COlnrllon solid Inalignancy in Tnen < 30 years old. The Inain risk factor is cryptorchidism.lbusillumination and ultrasound hclp to distinguish hydrocele (fluid~ filled, transiIh1l11inates) {rorn cancer (solid). The most conlInon type is seminoma (radiosensitive). Sarcoma hotryoid.,.: female child with a "huuch of grapes" coming out of her vagina. Pituitary tumors: look f,)r bitemporal hemianopsia (order an MRI if the patient has it). The most type is prolactinOIna (high prolactin levels with galactorrhea and Inenstrual/sexual dysfunction). Other types nray cause Cushing's disease or hyperthyroidisJ1l. COIl1mOn

Oncology Nasopharyngeal cancer: seen in Asians; remember association with

67

Epstejn~Barr virus.

Esophageal cancer: weight loss, possible anernia, and complaints that "my f()od is sticking," which progress to dysphagia f/x liquids in a chronic smoker and drinker (blacks> whites). Patients present late, because car]y cancer is asyrnptolnatic. The 1110St common type is squanlOUS cell. Barrett's esophagus (cohunnar metaplasia of esophageal sq uarnous epithelium due to acid reflux) may give rise to aderlOcarcinorna of the esophagus, and the incidence is increasing.

Thyroid cal1ce,r: the patient presents with a nodule in the thyroid gland. Be suspicious of cancer in any of the f()llowing scenarios: "cold nodule" on nuclear scan, male patient, history of childhood irradiation, nodule described as "stony hard,"recent or rapid enlargernent, and increased calcitonin level (lnedullary thyroid cancer--~--usually in patients with multiple en-docrine neoplasia type II). To evaluate a nodule in the thyroid, get thyroid function tests. Thyroid·-stimulatlng honnone is the best screening test; "toxic" or functional nodules arc un-likely to be cancer. On a nuclear scan, a "cold" nodule or aTca of decreased uptake is 11101'(:' suspicious than normal/increased uptake. Consider flne--needle aspiration or open biopsy Bladder cancer: look fbr persistent, painless flClnaturia. Patients often arc smokers or work in the rubber/dye industry (aniline dye exposure). Cystoscopy is usually done £lrst to evaluate a potential bladder cancer,

Live.r tumors: hepatocellular cancer is caused by alcohol, hepatitis, and anything else that causes cirrhosis (heulOchrOlnatosis is especially known to cause liver cancer). Hepatitis C is lT10re likely to cause cancer iTl the chronic setting than hepatitis B. For hepatitis B, the best prevention is proper vaccination; for hepatitis C, the best prevention is avoidance of blood transfu·, sinns. Alpha.-h-.toprotein is often elevated and can be measured postoperatively to detect recurrences. Patients have a history of akohohsrll, hepatitis, and/or hemochrOlnatosis or other causes of cirrhosis and present with weight loss, right upper quadrant pain, and an enlarged liver. Surgery is the only hope fc)r cure; prognosis is poor. Other tlUnors of the liver: 1. HemJngiOll1a: the most C0111mon priInary tumor of the liver; generally left alone. Surgery is done if the patient is synlplOIl1atic.

2. Hepatic adenoma: wornen of reproductive age taking oral contraceptives. Stop the pills! (The cancer may regress.) 3. Cholangiosarcoma: 50% of patients have a history of ulcerative colitis; liver flukes (Clonorchis) may be found in inunigrants. 4. Angiosarcoma: look for industrial exposure to vinyl chloride. S. Hepatoblaston1J.: the luain prinlary liver turnor in children. Adrenal tun\ors: lln-y be functional and cause primary hyperaldosteronisrn (Conn's syndrOll1e) or hyperadrcnahsm (Cushing's disease). Patients also nlay have a pheochrOIuocytOlna (int<.-.'TInittent, severe hypertension with mental status changes, headaches, and diaphoresis). Check 24-hour urine catecholarnines (vaniUylnlandelic acid, homovaniHic aCid). Stomach cancer: risk factors are Japanese race, increasing age, smoking, and consmnption of smoked nleaL HeJicobactcr pylori also is implicated. Krukenberg's tumor is stenna-eh cancex with bilateral ovarian metastases. Virchow's node is left supraclavicular node enlargement due to visceral cancer spread (classically stornach cancer). If a gastric ulcer is seen on upper GI barium series or endoscopy, it must be biopsied to exclude malignancr Osteosarcoma: 1 ()'~30---year-olds; recogrlize x -ray fmdings ("sunburst" appearance in distal fenmf or proxirllal tibia).

611

Oncology

Carcinoid tUlnors: the most common location is the small bowel, but carcinoid I::>' the most cornlnon appendiceal tumor. The liver hreaks down serotonIn and other vasoactive secreted suhstanct.'s to make the tumor asyrnpt01natic, hut when cardnoid luetastasizes to the liver and vasoactive products reach the systemic circulation, syrnptoms begin (carcinoid syndrome): episodic cutaneous flushing, ahdominal cramps, diarrhea, and right-sided heart valve damage. Urinary S-hydroxyindoleacetic acid (SHIAA) is increased (a product of serotollin breakdowu)_ Kaposi's sarcoma: ill HIV~positivt' patients; a vascular skin tUlllOr tbat starts as a papule or plaque, cOlnmonJy on the upper body or in tIle oral cavity. TIl(' classic deSCription is a rash that docs not respond to 111ultiple treatIllcnts. Skin can.cer: ultraviolet light increases risk of basal, squam()llS, and melanoma skin cancer. The AReDs of Inelan()Jna should nuke you suspicious of malignancy. Biopsy any lesion with any of these characteristics: asynlInetry, borders (irregular), color (change LIl color or multiple colors), or dialllekr (the higgcr the lesion, flIt:' mOfe likely that it is malignant). Know the classie appearance of basal cell cancer (pearly, mnbilicated, telangiectasias). Basal cell cancer is ex tremely common and aln10st never metastasizes. Squanlous cancer rarely metastasizt's, whereas melanoma cmnulOnly metastasizes. Biopsy any suspicious lesion (excisional biopsy).

Wihns' tUlnor vs. neuroblastolna: both present as flank masses in children at a peak age of around 2 years old- The classic way to differentiate the two (although yOll should always get a tissue diagnosis to rnake sure) is intravenous pyelography: neuro11lastomas tend not to distort the calyces of the kidney (most come frolll the adrenal gland), whereas Wilms' tumor arises in til(' kidney and thus distorts the calyceal architecture. Rarely, neurohlastomas Juay regress SPO])taneously (fC)f unknown reasons)_ Oral cancer: due to smoking or che_wing tobacco and drinking; also look fIjI' poor oral hygiene. Oral cancer often starts as leukoplakia (know the appearance), which Illust be daferentiated from oral hairy leukoplakia, which is associated with Epsteil1-~Barrvirus and affects HIV-posilive patients.

Histiocytosis: CD I positive, Birbeck granules (cytoplasmic inclusion hodies that look !ike tennis rackets). Unicalllcral hone cyst: expansile, lytic, welJ.--demarcated lesion in fhe proxhna] portion of the humerus in children and adolescents. It is benign but nlaY weaken bone enough to cause a pathologic fracture. Retinoblastollla: lellkocoria in a young child (red reflex is white whh a penlight) or Ullilatcral exophthalmos; inherited ]emn may be hilateraL

Note: Patients with cancef, like an others, have the right to refllse treatment However, watch fC)f and treat depression, even in tenninal pati.ents.

TUlllorlllarkers 101

)eillh~,y:til:i1¢_llP;tig~u'-,­

Prbshite-'spectfic,'il:ntigc_u

C,o!oh, Jl:a11creas; {)tht~r GX Junibfs

CA,125

Ovarian

Prostate (early)

$,10Q

M:danom'a" ~htral I1('-l'VOl'lS sys(ernlrter:«e n.ni1O'fS

Pl'Q':i-tate _(only witli -eirt¢tlBiori DutSide, tHe: capsUle)

Pancreas

CHAPTER

10

Fmpiric therapy while awaiting culture and sensitivit}' results MAIN~VG(S)

CONDftION

;;; t!:xa~nples t HiWIl10p1JiJu.l'iriflu:etlJ(ae type, l? is-·tic _ __," '.' ,': p'.'ca,ust6 totyqf vacciqati.6Il, H, hUluenZ'ac hcthli):, fi1o~t_1ik~ly-c,l:tlse-_ of +Thh,~k of', staB:~yl(){;r{)pd jf', the- patY:'pt -is: 1;19't: se>tuaJ.ly::ayttv~ a({tive\lud/or p:r<:lJl:i'iScnO\ls.

-

-

Empiric: antibiotics of choice f(H' difl(-'.fcI1t bugs (always use culture sensitivities to guide ther-apy if available.) (See flgure, top of next page.)

Staining hints: bhll~/purpJe;

liiIl

Granl--positive organisms are

gram-negative organisms are fed.

IiJ

Gram--positive cocci in chains = streptococci

rn

Gram~-pos"itjve

I1i!l

Gram-posilive cocci in pairs (diplococci) :.: ": SlfCl'tococcm: pncumoniae

!lII

Gram- negative coccohacilli (small rods)

cocci in dusters "'; staphylococci

~~..:

HacmoJlhilus sp.

69

70

Infectious Disease

ANTl&IOTIC

:Thil'g-gcinerati()l}_cephalos'pQrln m&ttl~~f~' Spi ,

;tetracycline

Mt(;'~bti~retitim--ttlb:erf:tiJosis

l.\;?ilmiidtr-ifltmpf ll

;$treptMo'cCUS pne~rtion.i9e:

:Pentcin1n

~taph-yloc()ctf

Anti~taphy16co¢qil

lvlellingococcus

PenkilliIllap1pici!lin

tiox, c<'phalo.s:p6rin-

-J?~-eutIQm_t#lclS--~p.­

Pel1i~il1iri

.My(;opJl1s~a, sp.-

Erydlroinydil Te:tracytHne

Chlamydia sp.

peuipl1hli

:+-,amilioglyQo$'ide

T'errac-yc1hi.:¢

Thfud"Oge~(q:~tl()n _cel~liilIQ~pOrijr

II

Granl-negative diplococci::::: Neisseria (gonorrhea, septic arthritis, meningitis) or MomxelJa sp. (lungs, sinusitis)

• Gram-negative rod that is plump and has thick capsule (mucoid appearance) = Klebsiella .II'. .. Grarn·,posltive rods that form spores:=: Clostridium, Bocillus spp, • Pseudohyphae III ill!

Acid-fast organiSJ11S == Mycobacterium tuberculosis, Nocardia sp. Granl--positivc oragnism with sulfur granules = Aclillomyces sp. (p('lvic inflanunatory disease in WOlnen who usc intrauterine devices; rare cause ofueck mass/cervical adenitis)

II Silver-staining D

= Candid" sp.

=:

Pneumocystis carinii (PCP) and cat-scratch disease

Positive India ink preparation (thick capsule) == Cryptococcm sp.

• Spirochete = Treponema, Ieplospim spp. (both seOIl only on dark· field microscopy), Borrelia sp. (regular light microscope)

Pneumonia: look fc)r classic clues to differentiate. The gold standard for diagnosis is SPlltUll1 culture; do blood cultures, too;

1. SlrqJlOCOCcus pncurnoni(Jc: nlOst common cause, especially in old~'r adults. Look f()r rapid onset of' shaking chllls after an upper respiratory infection, then ft.'ver, pleurisy, and productive cough (yellowish--green or rust-colored from blood). X--ray shows lobar consolidation. White blood cell COllllt is bigh, with large percentage ofllcutrophils. Gjvc vaccine to pa-tients older than 6S yr, splenectomized patients, patients with sickle cell disease, im-UWIlOC0l11pr01nised patients (Hrv. lTlalignancy, organ transplant), and all patients with chronic disease (diabetes rneBitus, cardiac, pulmonary, renal, or liver disease). j-,'or board questions, penicillin is still the cI11piric drug of choice. 2. Hacmophilus infJucnwe: second only to S. pncumoniae as most COmltlOJl cause of pncumo nia; mOre conHllon ill young children. Resemhles S. pncumoniac clinically. Treat with

Infectious Disease

11

J,lnpicillin/al1l0xicillin, cephalosporin, or trinl(~thopriIl1/sulf~llnethoxazoleif granl-negative coccobac~lli are seen Oll sputmll Graul stain.

3. Staphylococcus aureus: causes hospital-acquired pneulnonia and pneulnonia in patients with cystic fibrosis (second to P,m,doJllonas sp.), intravenous drug abusers, and patients with chronic granulomatous disease (look for recurrent lung abscesses). Etnpyema and lung absct.sses are TelaUvelycoIll111on. Cultures usually are positive. 4. Graul-negative organisrns: Pseudomonas sp. classically -is associated with cystic Hbrosis; KlcbsieJlo 3p. is classic cause in skid--row alcoholics and hOlndess people; enteric gram-negative organisIns (e.g., E, coli) afC cmUlHon with aspiration, neutropenia and hospital-acquired pneumonia. High mortality rate because of patients affected and severity of puc,umonia (abscesses COUlll1011). Treat empirically vvHh third-generation penicillin/ cephalosporin plus aminoglycoside. 5. Mycoplasma sp: most coun11on in adolescents and young adults (the classic case is a college student who lives in the donn and has sick contacts). Called l1atypicaI" penumonia he-cause it is different frOIn S. pneumoniae, with long prodrome and gradual worsening of lIlalaise, headaches, dry nonproductive cough. and sore throat. Chest x~ray shows a patchy, diffuse bronc!lopneurnonia (the x-ray classically looks terrible, although the patient does not feel that bad). took f(,r positive cold· agglutinin antibody titers (may cause henlolysis/ anernia). EInpiric treatnlent of "atypical!! pneultlonia is erythronlydn. 6. Ch!(Imydi(l pneumonioc second only to Mycop]asID<1 sp. as cause of pnemnonia in adolescents

and young adults; presents similarly but has negative

cold~,agglulinin antibody

titers.

7. Viral pnemnonia: viruses commonly cause respiratory infections (respiratory syncytial virus, influenza, parainfluenza, adenovirus) 8. Pneumocystis carinii pneumonia (PCP) and cytomegalovirus (CMV): always suspect in HIVpositive patients. PCP is rrlOre c01111110n; hronchoalveolar lavage often is required to obtain the diagnosis. PCP shows up with silver stains·--know what it looks like. Treat with trinlethoprim/ sulfalnerhoxazole; the alternative is pentamidine, PCP is acquired when the CD4 count is below 200, at which point you should institute PCP prophylaxis ill an HIV-positive patient. CMV has intracellularinclusioll bodies. Treat with ganciclovir; f()s-carnet is an alternative,

Classic infectious disease questions: .. Patient stuck with thorn or gardener: Sporothrix schenckii (a hmgus). Treat with oral potas sinn1 iodide or ketoconazolc. Bl

Aplastic crisis in s:ickle cell disease

Of

other hcrlloglobinopathy: parvovirus B19

after splenectomy (}f atltosplenectomy in sickle cell disease): S. pneuIlloniae, H. influenme, N. mcnillaitidi.\ (encapsulated bugs)

m Sepsis

fi

Pneumonia in the Southwe·st (Cahf()rnia, Arizona): coccidioides immitis. Treat with aruphotericin B.

9 PneunHmia after GiVe exploring or exposure to bird droppings in Ohio and Mississippi

River valleys: Hjstoplosmo capsul
Pnemnonia after exposure to a parrot or exotic hird: Chlamydi(l psi/taci

.. Fungus baU/hemoptysis after tubercular cavitary disease: Aspcraillus sp. m PnClmlonla in a patient with s.ilicosis: tuberculosis I!!I

Diarrhea after hiking or drinking frorn a stream: Giardia /C1mlJlia; cysts in stool; tfeat with nwtronidazole

72

Infectious Disease

HI

Pregnant women with cats: Toxoplasma gondii

.. En deficiency and abdominal symptoms: Diphyllobothrium latum 1/1

Seizures with ring-enhancing brain lesion em CT: Taenia solium (cysticercosis)

II Bladder II1II

I!!I

Il!1!

cancer (squamous cell) in Middle East and Africa: Sc),istosomo haemotobium

Worm infection in children: Enterobius sp. (positive tape test, perianal itching) Fever, rnusde pain, eosinophilia, and periorbital edclua aHer eating raw lueat: Trichindla spi",lis (trichinosis) Gastroenteritis in young children: rotavirus

mFood poisoning after eating reheated rice: Bacillus cereus ll\1

Food poisoning after eating raw seafood: Vibrio purahocmolyticus

G

Diarrhea after traveling to Mexico: liscbericbia coli (MonteI.UIna'S revenge)

liIIl

Diarrhea after antibiotics: Clostridium diftkile; treat with InetronidazoJe or vancornycin

.. Infmt paralyzed after eating honey: Clostridium botulinum (toxin blocks acetylcholine release) III

Genitallesiolls in children in the absence of sexual abuse/activity: molIllscml1 coutagioswu

.. Cellulitis alter cat or dog bites: Posteurello multoeido (tn"t cat and dog bites with prophylaetic ampicillin) .. Slanghterhonse worker with fever: Brucello sp. Ilt

Pneunlonia after bdngin a hotel, near air conditioner or water tower: I,egioncl!o pneumophila; treat with erythromycin)

III

Burn wound infection with bluel green color: Pseudomonas sp. (5. aurcus also without blne.green color)

cOJnnV)Jl,

but

Syphilis: screen with Venereal Disease Research Laboratory (VDRL) or rapid plasma reagin (RPR) test; if positive, confirm with Iluorescent treponemal antibody, absorbed (I'YA-ABS) or mierohemagglutination Trcponemo pallidam (MHA-TP) test. Treponema pallidum also can be seen with darkfield microscopy but not with a Gram stain. Screen all pregnant women with VDRLlRPR. Treatment is penicillin; use erythromycin for penicillin allergy. Three stages: 1. Primary: look fbr painless chancre that resolves on its own within 8 weeks. 2. Secondary: roughly 6 weeks to 18 lnonths after infection; look fc)r condylOlna lata, rnaculopaplllar rash (especially involving palms and soles of feet), and lymphadenopathy. 3. Tertiary: occurs years after initial infection; between secondary and tertiary stages is the latent phase, when the disease is quiet and asymptomatic. Look fClr gummas (granulomas iu many differeut organs), neurologic symptoms and signs (nenrosyplli!is, ArgyllRobt.'rtson pupil, dcrnentia, paresis, tabes dorsalis, Charcot joints), and/or thoracic aortic aneurySl11s. Note: Watch f()f false.~positive VDRl.IRPR in patients with Illpns erythematosus. For other ally transrniued diseases, see gynecology section.

sexu~

Infectious rashes (rnosl often in children; supportive treanllent only unless otherwise specified): 1. Measles (rnbeola): look IClT a reason IClr patient not to be immunized. Koplik's spOts (tiny white spots on buccal mucosa) are seen 3 days afler high fever. Other symptoms include cough, funny nose, and conjunctivitis/photophobia. On the next day, the rash (maculopapular) begins on the head and neck and spreads downward to cover the tTunk (cephaJocaudal progression). COInplications include pneumonia (giant-cell pneulllonia,

Infectious Disease

13

espec.ially in very young dlHl irmnullocnnlprolnised pa.tients), otitis -media, and ('n-~ ccphaHlis (which Inay he acute or cause subacute sclerosing p
2. Rubella (Germ,an Ineasles): 111ostiJnportallt because of inf(;ction in pregnan.t women. Screen and inllllunize any wcnnan of reproductive age before she heeolnt's pregnant; the vaccine contraindicated in pregnant wOlDen. Rubella :is InUder than Trwasleswith low fever, malaise, tender swelling of the suboccipital and postauricularnodes, and arthral gias. Aht'r a 2---·3--day prodrome, the rash (nIJcnlopapular, hint) starts on the Glee and neck and spreads to the trunk (cephalocaudal pn)gression). ConlphcaLions include en cephalitis and otitis lnedia.

3. Roseola inf~m.tu:nl (exanth{~m subitum): easy to recognize hecause of progression: high fc~vcr (rnay he > 4:(fl C) with no apparent cause f()[ 4: days (patient rnay get {t"brilc seizures), then an abrupt return to nor1nal telllperature as a diffuse 1l1
4. Erythenla infectiosulu (fifth disease): classic "slappcd-clleck rash (confluent erythema over the checks) appears around the saIne tllne as mild constitutional syrnptOIns (low fc;vcr, lnalaise). One day later, a rnaclllopapular rash appears on the anns, legs, and trunk. It is caused by llarvovirus B19 (the SMne virus that callses aplastic crisis in sickle cell disease).

5. Chickenpox (varicella): the description and progression of the rash itsdfshollid lead

to

the diagnosis: discrete 111acu1es (usually OJl the trunk) turn into papules, whiclt turn into vesicles that rupture and crust over. These changes occur withinl day. The lesions appear in successive crops; therefore, the rash is in (liffcrcnl: stages of progression in (lifferent areas. The patient is infl'ctious until the last lesion crusts over. A CCHllplication is infl~ction of the lesions (streptococci, staphylococci-- --erysipelas, cellulitis, sepsis). The patient should be instructed to keep clean to avoid inft.'ction. Other cornplications include pncu-lnonia (especially in very young childrerl and imnnnlocoinpromised adults) , cncephalitis, and Reye's syndrorne. 1)0 not give aspirin to any child with a fever unless the diagnosis requires ils usc. A Tzanck smear of tissue from the hase of a vesicle shows Hlullinucleated giant cells. VariceHa zoster innrlullOglobuhn (VZIG) is available J(}f prophylaxis in pa-tients with dcbilitatingillness (e.g., leukemia, AIDS) jf YOll sec thenl within 4- days of exposure or in newborns of rnothcrs with chickenpox. Acyclovir may he nsed in severe G1Ses. The varicella zoster virus can reactivate years later to cause shingles (zoster), which is characterized by denTldtolnal distribution of rash. Pain and paresthesias often precede tbe rash. 6. Scarlet: fever: look for a history of lmtr(~ated streptococcal pharyngitis (caused only by Streptococcus species that produce erythrogeniC toxin), f()llowcd by a sandpaplT-like rash on the abdomen and trunk witlI classic circumoral pallor and strawberry tongue. The rash tends to dcsq uarnate once the f(~ver subsides. Treat with penicillin to prevent rhclUnatic f(;ver. 7. KawasaJdts syndr()lnt~ (n1ucoclltaneolls lYlTlph node syndrorne): [afe; llsuaIly occurs in patients younger than .5 years. Diagnostic criteria include fever> S days (lnandatory for diagnosis); bHateral conjunctival injection; dlanges in the lips, tongue, or oral mucosa (strawberry tongue, fissuring, injection); changes in the extreurtties (desqudlnation, edelna, erythelna); polynl0rphous truncal rash (usllallybcgins one clay ann the fever starts); and cervicallYll1pbadenopathy: Also look for arthralgia or
74

Infectious Disease

ft:~arcd cornphcJtions

involve the heart (coronary artery aneurysms, congestive h.eart bil nrc, arrh ythrnias, rnyocarditis, rnyocarclial infarction). Think of Kawasaki's syndrolne in the difh:rcntiaJ diagnosis of any child who has a ITlyocardial inbrction. If suspicion is high, give aspirin and IV inlITIU[]()glohllHn, hoth of which reduce cJrdiac IcshJfls. Follow up with cchocardiogr
cut). The rash starts as thin--wallcd vesicles tlHt rupture and fonn yellowish crusts.I'he skin often is described as "weeping." Ctassically, lesions arc on the bee and tend to be 10calizccl. 1111JJetigo is illJ(:ctiollS;look f()f sick contacts. Treat with oral anti staphylococcal penicillin to cover streptococci and staphylococcl, the most COlmnon causative bugs. Endocarditis: eitber acute (fltlminant, ltlosl CC)llHnonly caused by S. aurcus) or subacute (insidious onset, rnost connnonly caused by Streptococcus virit!oJ1S). Look {c)r general signs of inf:(~ction (e.g., £1:.:ve1', tachycardia, rnalaise) plus nevv--onsct heart rnunnur, embolic phenolllerla (stroke and other infarcts), Osler's nodes (painful nodules on tips of flngers) , IZoth spots (round reti-nal helnorrhages with white centers), and septic shock (nlOre drarnatic with acute than sul)<:\" olte disease). Diagnosis is lnade by blood cultures. Elnpiric treatment is begun with wide--spectflllTl antibiotics until clrlture and sensitivity fC'SUItS arc known. A third--gcneration penicillin or cephal()sporin plus aUlinoglycc)side is a reasonable choice. J)atients 1110re likely 1:0 be afh~ctcd include IV drug ahusers (who develop riglJj>.-~;idcd lesions, although Jeft---sicledl(>sions are Iuuch Tnorc cornrnon in the gencral poplrlation), patients with abrlorlnalhcart valves (prosthetic vatves, rlleUlTlatic valvular disease, congenital heart deft:cts, such as ventricular septal defc:ct: or tetralogy of J;a11ot), and postoperative patients (especially aftcr genitourinary, gastrointestinal, or dental surgery). IIellCC the need f:(Jr pl'ophyla:x.h; in sllsceptible people. Any patient with known valvular disease is given oral arnoxicHhn (crythrornycin if the patient is PCllicillilJaJlergic) hef-()rc and after dcntal procedures (to cover S. viridolJ.<;) and IV dll1picillin (vancolnycin if thcpatielrt ispenicilliIl--aHcrgic) hcf(}rc and Jrnoxicillin/ gentarnicirl bd()rc and after gastn)jJlteslinal or gcnitollrin c()Vcr cllt:cn)Cocci). Patients with seCUl1drllll atrial sept
~~iven

clldocarditis proJ)hylaxis.

Meningitis: the highest incidence of JIleningitis is scen inneolutes; > 7S?{} of ca,'.;cs arc seen in I)atients YOlluger than 2 years, 'fJuls the decision about when to do a IUlnhar taJl is diHlcult, hecause such patients ()ften do not have classic physical fiuclings CKcnli~(<; and _Brudzinski's

Infectious Disease

75

signs). Look for lethargy, hyper·. or hypothermia, poor tone, bulging fontanelle, vomiting, photophobia, altered consciousness, and signs of generalized sepsis (hypotension, jaundice, respiratory distress). Seizures may be seen, but silnrIc ft'brile seizures also are possible if the patient is between 5 months and 6 years old and has a {c'vet > 102 0 l' :in the absence of other signs of Incningitis. If seizures occur in the presence of other signs of meningitis or sepsis. proceed to lumhar puncture iuuuediatdy and begin hroad..speetnuTl antibiotics irumediately after the procedure. The 11105t cornman ne.urologic sequela of rneningitis is hearing loss. All patients need fonnal hearing evaluation after a bout of meningitis; vision testing also is recommended. Other sequelae includemcntal retardation, rnotor deflcits/paresis, epilepsy, and learning/hehavioral disorders. iii

Murnps and measles are possible causes of aseptic (nonbaeterial or culture-negative) meningitis. The best prevention is irnrnunization.

mWatch f()r herpes encephalitis if the rnother has herpes simplex lesions at the tinle of the infant's birth. Look f(x ternporallobe ahnonnalities on a C1' or MRl scan oCthc head. Give acyclovir. Ii!I

If rneningHis is due to NeisserJn sr., give all contacts rdalupin as prophylaxis.

ll!I

For cerebrospinal fluid Hndings in lneningitis, see the neurology chapter.

Pediatric respiratory infections: the big three are croup, epiglottitis, and respiratory syncytial virus (RSV)--high yield! 1. Croup/acute laryngotracheitis: look for patient to be [--2 years old; usually occurs in fall or winter. About 50---7 S% of cases afe due to parainflucnza virus; the other causative agent is influenza. Patients start with synlptolllS of viral upper respiratory infection (rhinorrhea, cough, and I,,,er) and roughly 1--2 days later develop a "barking" cough, hoarseness, and inspiratory stridor. The "steeple sign" is classic on lateral x~ray of the neck. Treat supportively with a Illist tent and racenllc epinephrine.

2. Epiglottitis: the patient usually is 2~5 years old. The main cause by lar llsed to be HaemophilusinfJucnzoc type b, but wHh widespread vaccination, H. inf1uCJlZ
76

Infectious Disease

Rabies: in the U.s., usually due to hites from bats, skunks, raccoons, or f()X('s. Vaccination has elijuinated dog rabies. 'The incubation period is usually around 1·----2 lTlonths. Classic synlptorns are hydrophobia and central nervous systelll signs (paralysis). After a bite, several steps should be taken: 1. Local wound trcatluent: cleanse thoroughly with soap; do not cauterize or suture the wound.

2. Observe the animal. If possible, capture and observe a dog or cat to sec ifit develops rabies. If a wild animal (bat, sktmk, raccoon, I"x) is caught, it should be killed and the tissue exan1ined f()r rabies. 3. Prophylaxis with rabies immunoglobulin and vaccine: II!!

If a captured or killed animal has rabies, defInitely give prophylaxis and vaccinate.

!!!I

If a wild anhnal (bat, skunk, raccoon, fox only) bites and escapes, give prophylaxis and vaccine.

!!II

If a dog or cat hites and escapes, do not give prophylaxis or vaccine unless the aniInal acted strangely and/ or bit the patienht without provocation Hod rabies is prevalent in the

arca (rare). i!!l

Do nOl give prophylaxis

O[

vaccine f()r bites by rabbits or other rodents (rats, filice,

sqUirrels, chipmunks).

STREPTOCOCCAL INFECTION Streptococcus pyogenes (strep A) causes multiple important inf<,ctions: 1. Pharyngitis: look f()f sore throat with fever, tonsillar exudate, enlarged tender cervical nodes, and leukocytosis. Streptococcal throat culture conftrnls the diagnosis. Elevated an,tistreptolysin a (ASa) and anti-DNase titers also arc usce! retrospectively when needed (rheumatic fever, post-streptococcal glornerulonephritis). Treat with penicillin to avoid rheulnatic fever and scarlet fever. I'ilIRh(~umatic

fever: diagnosis is rnade by history of streptococcal pharyngitis and Jones criteria, rnajor (migratory polyarthritis, carditis, chorea, erythema rnarginatlun, subcutaneous nodules) and minor (elevated erythrocyte sedimentation rate, C>.reactive pro-tein, white blood cell count, andASO tiler; prolonged PR interval; arthralgia).'I'reat with

aspirin; steroids arc used for severe carditis (e.g., congestive heart failure). After rheuloatic fever, patients need continuous prophylaxis against streptococci (heuzJthine penicillin intramuscularly every month or orally Ii" compliant patients; erythromycin for penicillin-allergic patient) until age 18 (or longer) plus endocarditis prophylaxis hefc:)rc surgkal procedures. Treatment of streptococcal pharyngitis reduces the incidence of rheumatic f(·ver. l[Ij

Scarlet f(~ver: some untreated cases progress to scarlet fc'veT if the streptc)coccal species produces erythrogenic toxin. Symptorns include red flush iIr skin (which blanches with pressure, classically with circurIloral pallor), truncal rash, strawberry tongue, and late skin desquamation. Kawasaki's syndrolneis another cause of this set of symptoms.

f£l

Post--streptococcal glomerulonephritis: occurs Jl10St cornmonly after a skin infection but may occur after pharyngitis. The patient presents with history of streptococcal infection (by anephritogenic strain) 1"--~3 weeks earlier and abrupt onset of hematuria, proteinuria (mild, not in nephrotic range), red blood cell casts, hypertension, edema (especially

Infectious Disease

77

periorhital), and elevated .BUN / creatinine. 'Ih:at supportive1y: control blood pressure, and use diuretics for severe edcu1a. Treating strevtococcal infections does not reduce the incidence. 2. Skin infections: often occur after a break in the skin due to tranllla, scahies, or insect bite. Watch ft)}' development of post-streptococcal gloJl1enllonephritis:

lI:ilrnpetigo: Inaculopapules, vesicopustules/bu!Jac, or honey--colored, crusted leslcHls. Staphylococci are a more frequent cause than streptococcL DefInitely think of staphylo-cocci jf a furuncle or carlnmcle is present:; think of streptococci if glomerulonephritis occurs. Infc.ction is contagious; watch for sick contacts. Treat empirically with antistaphylococcal penicillin (e.g., dic!oxacil!in). I:1iErysipelas: a superficial cellulitis that is red, shiny, swollen, and tender; may be associated with vesicles and hullae, fever, and lymphadenopathy. !!\lCclluHtis: involves subcutaneous tissues (deeper than erysipelas). Streptococci arc the most comrnon cause, hut staphylococci also lnay he implicated. Treat empirically with antistaphylococcal penicillin or vancomycin to cover both. If Pseudomonas sp. is suspected (diabetic foot ulcers, burns, severe, trauma), treat with broad--spectnl111 penicillin plus an amino glycoside. If Pasteurella multocida is suspected (after dog or cat hites), treat with IV alnpicillin. If Vibrio vuJnificus is suspected (fishennen or other salt--water exposure), treat with tetracycline. !!\lNecrotizing fascijtis: progression of cellulitis th necrosis and gangrene, crepitus, and systemic toxicity (tachycardia, fever, hypotension). Often rnultiple organisms (aerobes and anaerobes) are involved. Treat with IV fluids, incision and drainage or debridernent, and broad-spectrum antibiotics (broad--spectflllll penicillin or cephalospOrin plus an arninoglycoside) . 3. Endoluetritis/puerperal fever: postdelivery fever and utedne tenderness. Treat with anloxicillin/anlpicillin.

StreptocoCCLlS Clgalactiae (strep B): f~unous as the Inost COlTl1l10l1 cause of neonatal lllcningitis and sepsis; acquired [rmll luaternal birth canal, in which it is part of the nC)fl1lal flora. Penicillinsensitive. Streptococcus viridans: causes subacute endocarditis and dental cartes (Streptococcus mutans).

Enterococcus faecalis: normal bowel flora; causes endocarditis, urinary tract

inf(~ctioTl,

and sepsis.

Streptococcus pneumoniae: COlllmOn cause of pnelunonia, otitis lllcdia, meningitis, sinusitis, and sepsis.

STAPHYLOCOCCAL INFECTION Staphylococcus aureus: C(Hnmon cause of various 1m Abscess

inf(:~ctions:

(especially in the breast after breast"f(~eding or in the skin after a furuncle)

mEndocarditis (especially in drug users) !ill

Osteomyelitis (Inost connl1on cause except in patients with skkle cell disease)

!j

Septic artbritis

Y-Pood poisoning (pref()rmed toxin)

73

Inlectious Disease -Toxic shock syndrOlne (preformed toxin, classically in a woman who leaves tarnpon in place too long and develops hypotension, fever, and a rash that desquamates) m Scalded

skin syndrome (preformed toxin that alleets yonnger children, who often stan with impetigo, then desquamate)

II

Impetigo

• Cellulitis Iilil

Wound infecti\Hls

IIIPneumonia (often [onlls ltmg abscess or empyema) III

Furuncle or carbuncle

Note: Health care workers who

chronic nasal carriers may cause nosoccnnial infections. Treat carrier with antibiotics. Treat abscesses with incision and drainage, other infl~ctions with anti staphylococcal penicillin (e.g., methicillin, dicloxacillin) or vancOlllycin. afC

StalJhylococcus epidermidis: causes IV catheter infections. infections of prosthetic implants (hean valves, vascular grafts), and sepsis. Treat empirically with antistaphylococcal penicillin Or vancolllycin.

Staphylococcus saprophyticus: C01111110n cause of urinary tract infection. Treat empirically with standard urinary tract infection antibiotics.

CHAPTER

11

Common tel'lTIS to des(~ribe skin finding

-

l'RIMARY'J:;:USION

DIDJJNIl'ION

---

X\i10RPJIOtOGY

liXAMPt'fJS

'~~""-~~_'-':':"-'~--~--='~-~--~~'~-"~-"--"~-~-~~-'~-->"-~~~~~~-~~--

t';lat:_(ir(ull1~crjhed r;khi dtscolOT,iltion that ]ac.~s -surface efevation or

Cale-a:u~1ait

Vitilig<) }ireckJe


]lU1cliorittlllcvi

Yak tattoo

Acrochordon (skjn tag)

Elevate'l, so'Ud Jqsli)J1 <:: 0:5 cut

-Bas,at cell carcinoimi - Molhiscum :conta'gjOS1;ni: Intraden:nal nevi L'.tchen: plam.is

in -di;lIneter:

HIe' of

B6wen's dh;e'a(>('; Mycosis'fungciidC!:i

di corilponent

Psoriasis :Eczcllia Tinea-corporis

!'lat, dn;umcscr"bo,l skin

Nevus -fhn111uem: Vitiligo

disc61p:Nnion; .:i:-Ycry,largp macule?* I,:ern -iu: -dial:)iletttr

Elev;itt'd, ;>: 0:-5- i';i'u, in dia,m.",'r;

a-larger, de'bper,papule,'

l' -Wheal

Rheiunatoid nodl:lle 1'endtntxanthoma 1!rythml'1a'119dbsnm HpC)u1J

M(jtast
Nnn, 'cdematouqillaque that

Urticaria

is e:V_ilnescent al1d pi"mith:.; 'a hive

Derntogr"'phiS1H Ui.tlcarjJ pigmentosa

79

80

Dermatology

EXAMPLES

_ _::PR::::IMARYl:::.E::Sl:::O:::N:.-_..:D::E::F::IN:::I:::T:..IO::N::-__ VCs-ide

Papnk that c;c)1¥ta"ins clear fluid;- a blister

Hetpes simplex: Herpes zost~r nyshiA:lrol:tC .CC?:01Ua ConnKt dermatitis Vesiclo

Bulla

t-QtaUzed fluid-collection

Pemphigus vulgatJs"

>d:J,S'cm fn diameter;

Bul1o'us peli:rpltigoid. Bullous impetigo

it

Pustule

large vesiCk

Papult; that contains purulent maH:l'ial

rmpet-igo A{me Pustular psori.asis

wme w", contains fluid (jr semisolid- materIal

Acne Epidj)rmoid (;'}'st Pilar cyst'

Vitiligo: dcpiguwIHatioll of unknown etiologv; associated with pernicioHs anemia, hypothy-roidisrn, Addison's disease, and diabetic mellitus; lnay have autoirmnune basis. Patients often have antihodies to Juelanin, parietal cells, or thyroid. Pruritus: rnay be a clue, to diagnosis of serious and common conditions; seen in obstructive biliary disease, uremia, pnlycytheIllia rubra vera (classically after a warm shower or hath), con tact or atopjc dermatitis, scabies, and lichen planus. Contact dcnnatitis: ofh~n dne to a type IV hyperS{'Ilsitivity reaction; also may be due to irritat~ ing or toxic substancl'. Look for question that mentions new exposure to a classic nfh_'neling agcllt (polson ivy, nickel earrings, deodorant). The rash is well circumscribed and f(Hllld only ill the area of exposure; skin is red and itchy and often has vesicles Of bullae. Avoidance of the agent is required; patch testing can he done, ifncedcd, to determine the antigen.

Atopic dermatitis: look lew lamily and persollal history of allergies (e.g., hay kver) and astluna. This chronic condition begins in the first year of lift: with red, itchy, weeping skin on d~e head, llpper extremities, and somctinlCs around the diarwr area. The higgest prohlem h scratching, which leads 10 skin hreaks and possible hacterial infection. Treatmtnl involves avoidance

()f

dr)ling soaps, antihistarnines, aJld topical steroids (see figure, top of next page).

Seborrheic dernlatitis: causes the ('onHllOJl conditioJls know)] as cradle cap and dandruff as well as blepharitiS (c}Tclid inflanunatjon). Look f()r scaling skin on the scalp and eyelids, and tfeat with dandruff shampoo. Fungal skin infections (dermatophytE' infections, ringworm), depending on location, arc known as: 1. Tinea corp0rls (bodyltrunk): look t(lr red ring--shapcd lesions that have raised borders and Il'lld to dear ct'Jltrally while they ~'xpand peripherally.

OIJrmatology

81

Phases of atopic dermatitis. A, In1antile phase. Typicalefythematous, oozing, and crusted plaques seen on the cheek of an infant with atopic dermatitis. B, Childhood phase. Close-up view of a lichenifind, excoriated, crusted, and secondarily infected plaque on the right knee of a 4-year-oJd girl. C, Adolescent O( young adult phase. {From Fitlpmrkk JE, Aeling JL: Dermatology Secrets. Philadelphia, Hanley & Belfus, 1996, with permission.)

2. Tinea pediS (athlete's foot): look f,)r macerated, scaling web spaees between the toes that often itch and thickened, distorted toenails (o'nychomycosis). Good 1'001 hygkne is part of'treatlnent. 3. 'Tinca unguium (onychornycosis): thickened. distorted nails with debris under the nail edges. 4. Tinea capitis (scalp): mainly aflects children (highly contagiOUS), WllO have scaly patches of hair loss and may have an inflamed, boggy granuloma of the scalp (known as a kerion). which usually resolves on its own.

5. Tinea cruris (jock itch): rnnIc connnon in obese Ina]es, Llsually in the crural fblds of the upper inner thighs. M"ost fungal skin infections are due to Trichophyton species. Infections are diagnosed by scrap, illg the. lesion and doing a potassium hydroxide (KOH) prc.paration to visualize the ftlngus Of a culture. Griseofulvin (oral) must be used to treat tinea capHis and ollychornycosis~ the others can be treated with topical antifllHgals (e.g., miconazolc. dotrimazo)e, ketoconazole) or griseofulvin, which is better f()! severe or persistent inkctious, In tinea capitis, jf the hair fluoresces under Wood's larnp, Microsporum sp. is the cause; if it does not, the probable CJuse is Trichophyton sp_ Candidia~is: thrush (cn.:'an1Y whife patches on the tongue or buccal mucosa that can be scraped off) may be seen in normal chiJdren, and candidal vulvovaghlitis is Seen in nonnal wOfllcn, cs-pccially when they are pregnant Of afl-er taking antibiotics. However, at ot1](~r times and in die fercn! paticnts. candidal infections may he a sign of diabetes mellitus or imm.unoddkicncy. For example. thrush in iln adult male should make you think ahout the possihility of mv/ AlDS. Treat with local or topical nystatin or inridazoles (e.g., miconJzole, c}otriJnazo}e); oral therapy (nystatin or ketoconazole) is llsed {())' extensive or resistant disease,

112 Tinea versicolor: Pityrosporon sp: infection that presents TIlosl conllTlonly in young adults with llmltiple patches of various size" and color (brown. tan, and white) on the torso. It often becOIlles noticeable in the SUITnuer because the aH(~cted areas fail to tan and look white. Diagnose froul lesion scrapings (KOH preparation). Treat with sdeninm sulf)de shampoo or topical imidazoles.

linea versicolor demonstrating hvpopigmented scaly papules. (From

Htzpatrick ,IE, Aeling Jl: Oennatology Secrets. Belfus, 1996, with permission.)

Philad(~lphfa,

Hanley &

Scabies: caused by the mite Sarcopte., scabei, which tunnels into the skin and leaves visible bur· rows on the skin (know what tbey look like), classically in tbe finger web spaces and llexor surface of the wrists. Facial involvclnent somethnes is seen in infaIlts. Patients have severe prn-titus, and itching rnay lead to secondary bacterial infection. Diagnosis is made by scraping the mite out of a burrow and viewing it under a microscope. Treat with perrnethrin cream applied to the whole body. Rememher to treat all contacts (e.g., the wbole family). Do noluse lindane to treat unless pennetbrill is not a choice. Lindarlc used to be the treatnlcnt of choice but can cause neurotoxicity, especially in YOlIng children. Lice (pediculosis): lice can inft:ct the head (Pediculus capitis, which is COIln110n in schooJ -aged children), body (Pcdiculm corporh', which is unusual in people with good hygiene), or pubic area (crabs, caused by Phthirus pubis and transnlitted sexually). Infected areas tend to itch, and diag·Hosis is Inade by seeing the Uce on hair shafts. Treat with pennethyjn cream (preft.:rred over lio--

dane because of lindane's neurotoxicity), and decontarninatc sources of reinfixtion (wash or sterilize combs, hats, bed sbeets, clotbing). Warts: caused by hmnan papiIlornavirus (HPV); infections are rnost cornnwnly seen in older children, on,en on the hands. Treatments include salicylic acid, Hquid nitrogen, curettage, and others. Genital warts also are caused by HPV (types 16 and 18 are associated with cervical cancer). (Sce figure, top ofnexi page.) MoJhlsr:nm (:()ntagiosunl~ a poxvirus infection that is cmnmon in children hut also Inay be transIllit:t:ed sexually. A child who has genitallIloUuscmn Inay or rnay not 11ave contracted the inft~ction frorn sexual contact; autoirlIloculation is possible. Do not JUlornaticaUy assume child abuse, although it luust be nlled out. Diagnosis is made by the characteristic appearance of the lesions (skiJl"colored, srllooth, waxy papules with a central depression [uInbilicated] dIal: arc roughly O.S em) or hy l()()king at COntents oftlle lesion, which include cells with characteristic inclusion bodies. Usually treated with freezing or curettage·.

Dermatology

83

Some common types of warts. A. Flat warts afthe face. B, Wart of the hand. (From Fitzpatrick JE, Aeling Jl: Dermatology Secrets. Philadelphia, l1anley & Belfus, 1996, with permission.)

Acne: know the description of acne: comedones (whiteheads, blackheads), papules, pustules, inflamed nodules, superficial pus-Hlled cysts with possible inflammatory skin changes. Propionibacterium acnes is thought to be partially involved in pathogenesis as well as blockage of pilosebaceous glands. Acne has not been proved to be related to f()()d (but if the patient relates it to a food. yOll can try discontinuing it). exercise, or sexhnasturbation. hut cosmetics Inay aggravate it. Treatment options are multiple. Start with topical benzoyl peroxide, then try topical clindamycin, oral tetracycline, oral erythr0111ycin (fc)r P. aenes eradication), and topical tretinoin. The last resort is oral isotretinoin. Isotretinoin is highly eH(~ctive but teratogenic (pregnancy testing before and during therapy is man(hltory) and filay cause dry skin and mucosae, Inuscle and joint pain, and liver function abnormalities. Rosacea: looks hke acne but starts in middle age. Look I()f rhinophyma (bulbous red nose) and coexisting blepharitis. Treat with topical n1ctronidazole or oral tetracycline. The pathogenesis is unknown, but it: is not related to diet.

Hirsutism: lnost commonly idiopathic, but other signs of virilization (deepening voice, clitoromegaly, frontal balding) indicate an androgen--secreting ovarian tumor. Other causes in-elude corticosteroid administration, Cushing's syndrOlne, 'Stein-·Leventhal syndrOlne (polycys~ tic ovary), and drugs (minoxidil and phenytoin). Baldness: watch out for trichotillomania (psychiatric patients pulling out thcir hair) and alopecia arcata (idiopathic and associated with antimicrosoI11al anel other autoantibodies, lnpus, syphilis, or chemotherapy) as exotic causes of irregular, patchy balduess. Male-pattern baldness is considered a genetic disorder that requires androgens to be expressed. Psoriasis: know what classic lesions look like and how they are described (dry, not pruritic, well-circumscribed, silvery, scaling papules and plaques). l'amily history is often positive. Psoriasis occurs rnostly in whites with onset in early adulthood. Classic lesions arc f(nlIld on the scalp and extensor surfaces of the elbows and knees. Patients may have pitting of the nails and arthritis that resembles rheulnatoid arthritis but is rheumatoid faetor--negJ.tiv{~.Diagnosis is made by appearance, but biopsy can be used {-()f doubtful cases, 'l'reatnlcnt is cornplex but involves exposure to ultraviolet light (e.g. sunlight), lubricants, topical corticosterOids, and kera-·tolytics (coal tar, salicylic acid, anthralin). (See figure, top of next page.)

84

Dermatology

Psoriasis. Elbow involvement of psoriasis vulgaris, demonstrating typical well-demarcated, red plaques

with silvery scale. (From Fitzpatrick JE, Aeling Jl: Dermatology Secrets. Philadelphia, Hanley & Belfus, 1996, with permission.)

Pityriasis rosea: scenlS to be a popular dennatology question. Pityriasis rosea "is seell in adults. Look leJr a "herald parch" (slightly erythematous. ring-shaped or oval, and sealy patch classically seen on the trunk) followed 1 week later by many similar lesions that tend to itch. Look lelr lesions on the back with a long axis that parallels the Langerhans' skin cleavage lines. typically in a "ChristInas tree" pattern. Pityriasis rosea usually remits spontaneously in 1 rnont11. Think about syphilis in the differential diagnosis. Treat with reassurance. Lichen planus: look lelr tbe four Ps (pruritie. purple, polygonal papules) and oral mucosal lesions. Drug reactions: pen.iciJHn, cephalosporins, and sulfa drugs commonly cause rashes; tetracycline and phenothiazines cOl1ullonly cause photosensitivity. Erythema multiform", look f()J' classic target (iris) lesions. Usually caused by drugs or infections (e,g.. herpes). The severe fc)rrn is known as Stevens·-Johnson syndrOlue. which often is lataL Treat supportivcll' (See figure below.)

A, Stevens-Johnson syndrome. Typieal mucosal inflammation of the mouth, lillS, and conjunctiva. B, El'ythemn multiforme or Stevens-Johnson syndrome. The eruption Gonsists of annular ,HId Ilupular erythema over the ac;ral areas. (From Fitzpatrick .IE, Aelino Jl: Oermatofoov Secrets. Philadelphia, HanlflY & Belfus, 1996, with permission.)

115 Erythema nodosurn: inflanunation of the subcutaneous tissue and skin, classically over the shins (prctib.ial). T<.-.~nder, red nodules are present. Look fl)f exotic diseases such as sarcoidosis, coccidioidornycosis, and ulcerative colitis as the cause, although rnOTe conunonly the cause is unknown or clue to a streptococcal :infection. Pem.phigus: all autoinunune disease of themidcllc---agcd and elderly that: presents with lnnltiple hullae, starring in the oral mucosa and spreading to the skin of the rest of the body. Biopsy can be stained for antibody and shows a linear inUllUTlofluorescence pattern. 'freal with corticosteroids. Dermatitis herpctifol'lnis: should alert you to the presence of gluten--sensilivity; look for diar rhea and weight loss. Skill has IgA deposits even in unaffl~cted areas. Patients preseIlt with in"

tensely pruritic vesicles, papules, and wheals on the extensor aspects of the dbows and knees, possibly on the face and neck. Treat with gluten---frce diet. Decubitus ulcers (hedsores or pressure sores): dne to prolonged pressure against the skill. The best treatrnent is prophylaXiS. Periodic turning of paralyzed, bedridden, or debHHated patients prevcnts bedsores. Cleanhness and dryness also help to prevent this condit.ioIl, and perj odic skin inspcction makes sure that you catch the problell1 early. When Inissed, llH'. lesions can ulcerate down to the bone and hermne infl'.cted, possihly leading to sepsis and death. 'Treat major skin breaks with aggressive surgical debridement and antibiotics if signs of infl:cti_on arc present. Ex<:essive perspiration: think of hyperthyroidism and pheochrOlllocytoma. Moles: COIl1HlOll and benign, hut Inalignant tralls{bnnation is possible. Excise any mole (or do a biopsy if the lesion is very large) if it enlarges suddenly, develops irregular borders, darkens or becomes inflall1cd, changes color (even if only one small area of the 1nolc changes coh)r), begins to bleed, begins to itch, or becomes painful. DysplastiC nevus syndn)llH'. is a genetic condition with multiple dysplastic appearing nevi (usually> 1(0); also look for a family bis~~ tory of Inelanorna. Treat with careful fc)llow-~up and excision/biopsy of any suspicious··looking lesions as well as sun avoidance and sunscreen use_. Kerar.oacantholna: mainly iInportant: because it lnilnics skin cancer (especially squarnous cell callccr).'I'his flesh--colored lesion with a central crater contains keratinous Ina1:e1'ial claSSically is f()llfld on the face. The best way to differentiate it from cancer is that a keratoacantllOlll<.l has a \rcry rapid onset, and grows to full size in 1-~2 months. Such rapid growth almost never occurs with squamous cell cancer. The lesion involutes spontaneously in a few rnonths and requires no tTcatluenl. If you
Keloid: an overgrowth of scar tissue after an inj my, most freq Llcntly scen in blacks. Usually slightly pink and claSSically I'mnd on tbe upper back, cbest, and deltoid area. Also look I,,], keloids to develop after ear pitTcing. Basal <:cll canc:e.:r: begins as a shiny papule and slowly enlarges and develops an uUlbilicatcd center (which later may ulcerate) with peripheral telangiectasias. Basal cell cancer rarely 111cta5tasi'les. As with all 51<;j1"1 cancer, SU11hght exposure increases risk. It is lI10re cornnlOn in light skinned people. Treat with excision. Biopsy any SUSIJkiollS skjnlesiofl in elderly patients.

Squalllous cell c:an(~er: look fc)r preexisting actinic keratoses (hard, sharp, red, often scaly Ie sions in sUI1--exposcd areas) or burn scars that becorne nodular, warty, or ulcerated. Do a biOpsy if tllishappens! Squarnous cell cancer :irl situ "is krlowIl as Bowen's (Hseasc. (Sec figure, top of next page.)

86

Dermatology

Squamous cell carcinoma of the ear, demonstrating a nodule with contral scale 8hd crust. (From Fitzpatrick JE, Aeling JL: Dermatology Secrets. Philadelphia, Hanley & Belfus, 1996, with permission.l

Malignant melanoDla: usually arises fi'orn preexisting llloies. RClncruber your ARCDs: aSYlnmetry; irregular borders, color change, and increasing dtameter). Prognosis is directly related to the depth of vertical invasion. Superf\cial spreading melanoma tends to stay superflcial and has the best prognosis. Nodular ruelanoma is the worst because it tends to grow downward first. Although UllCOrnrnOl1 in blacks, meJanOlna tends to be of the acrolentiginous type. Look f()f black dots on tilt' palrns and soles or under the fingernail. Treat with surgery; if surgery fails, the prognosjs is poor. Kaposi's Sal'COllla: seen in AIDS patients. Look f()t classlc Inucosal lesions or an expanding, strange rash or skin lesion that does not respond to multiple treatments. (See flgure helow.)

Kaposi's :;an:oma. Multiple violaceous pla{/lws are seen on the trunk of an HIV-positive patient. (From Fitzpatrick JE, Aeling JL: Dermatology Secrets. Philadelphiil, Hanley & Belfus, 1996, with permission.)

Fagees disease ofth.e nipple: watch f()]' a unilateral red, oozing. and crusting nipple in an adult woman. An underlying breast cancer with extension to the skin rnust be ruled out. Stoln'ltitis~ watch

vitamin C

for deficiencies of B--cOlnplex vitarnins (riboflavin, niacin, pyridoxine) or

Classic <.:<"cbrospinal fluid findings in dillere"t conditions CONDITION

CIlLLStt
GLUCOSE ("'still)

PROTEIN (mg/dl)

PlUlSSURE (mmHg)

Norin;d -ce1'(~bro$phi,ar fluid

10-45

100-lOO

-Bact{~riar rnelilngWs

A;ci::mixcl- Lon"

> 200 Nntrniil' or sliglitry

~''''-~~~--~,,:--"~~-~--'--,~_'''=~~_~'~_~~,,",,,,",-~_~~~~~_",","o",, ....,,~.-;,~~

VimJ

Of

__~«-_''''''~~~~__ "~~_

ase-pridnetliUgitls

inGr'ciasft
>200

,:Cx1tiUtrhl:-Barre: syni;Jton\e,

tzer.ebrid hern:()rrhagt~ t :Mu1til~lt:i;Ckl;oii~ij:

Important points: 1. Do not do hunbar puncture in patients with acute head trauma or signs of intracranial hypertellsion until you have a CT'/MRI. You Inay cause death. Z" Tuberculosis and fungal meningitis have low glncose « SO) with bigh cells (> 100), which arc predominantly lymphocytes. VVatch f()r a positive India ink preparalion {()r

CryplococCUS sp. Multiple sclerosis: look fc)r insidious OllSet of neurologic symptoms in women aged 20-- 4-0 with exacerbations and remiss_ions. Common presentations include parcsthcsias and lHlmb·· ness, weakuess and dUIllsincss, vislJal disturhances (decreased vision and pain duc to optic neuritis, diplopia due to cranial nerve involvement), gait disturbanccs, inco.utjlleIlcc or ur· gency, and vntigo, Also look f()!' ('lllotionallahility or other lnental status changes. fntertllidcar ophthalmoplegia and scanning speech are classic; Babinski's sign may be positive. MRI, the most sensitive didgllostic tool, shows demyelination plaqlLCS.l.ook f()r increased IgC Of oligoclOlJ.11 hands and possihly mydjll basic protein in the cerebrospinal fluicLTreannent is not very effective hut -includes C(Hticosteroids alld blterf't'xons.

Guilbin·-llan'c s'YlHlrm:ne look

f()!· history of Jllild hdt'ctjon

or immllnizatioI1 rOllghly 1 vveck

bef()JT onset of symmetric, distal weakness, paralysis, or Ill.lld parcsthcsias with Joss of deep

117

tendon reflexes in affected areas. As the ascending paralysis and weakness progress, respiratory paralysis may occnr. Patients rnust be watched carefully; usually spirometry is used to follow inspiratory ability. lntubat ion Juay 1-)<:: required. Diagnosis depends on clinical signs and synlptolllS, JIJa)ysis of cerehrospinal fluid (usually llornlal except fe)f Inarkedly iU
Iilcctromyography (EMG): measures the electrical (CCJJJtraeliJe) properties ofumscle.l.ower motOr neuron Jcsjons afC associated with fasciculations and Hbrillations al rest. When the dis case is in the muscle Itself: no electrical activity is SeCll at rest (which is normal), Inn amplitUde is decreased with contr
Syncope: the rnost common canse (after stress, fear, or other emotional states) is vasovagal. Other causes include cardiac events (especially arrhythmias; get an liKCn, transient jschcmk at·, tacks (consider carotid art.ery duplex scan), and neurologic disorders (especially seizures; consider CT or MRI of head jfothcr neurologic symptoms arc present).

I.ocalizing pathology of the central nervous system

poriiftobe* Memory trtipa~rment or agg.l'e:5sive.- st:xual be'havidt

temp()'caJ lobes

Iri.abil~ty to

Ddnli11al1t'paii(~tal

te;xd; write naiJie,-:or:-do math

Iohe*

19nt):tfrlR one·sid(;) ofhody, troub1~ .witrr d~~ssiJlg

NOildoXfifn,).nt: -lJ.arie.taJ -lob.c*

Visila.! ha,llpCtnaribiis O:r:illll~ions:

OcCi.j:;.ltaIlci!;ler;

Chmiaf ne~Y~s 3. "aJl
l\1idbra:hi

For deliriou's or uncousc1on,s IJadeuts i.n tlH~ enlcrgency departtnent with no history of trauma, think. flrst of hypoglycemia (give glucose), opioid overdose (give naloxonc), and tbi~ amine defIdency (give thiaH/inc bd()]'c !~jvillg glucose ju a suspected alcoholic). Otlwr common causes are alcohol, illjc.it drugs, prescript'iOll medications, diabetic ketoacidosis, stroke, and l'pilcpsy or postictal state.

Deliriunl vs. dern.cl1tia (Sec table, lop orncxl page.)

Neurology

89

Important points: 1. Both deliriurn and dementia Inay have hallucinations, illusions, delusions, melllory irn-painnent (usually global in dehrimll, whereas rCluote m,ernory is spared in early dementia), orientation difficulties (time, place, person), and "sundowning" (worsened delirium or clClnentia at Hight). 2. In the elderly watch f()y pscudodcn1Cntia, whiCll is caused by depression and reversible with treatrnenL 3. Treatable causes of dcrucntia include vitamin H12 deficiency, endocrine disorders (espe-tially thyroid and parathyroid), uremia, syphilis, brain tnnwfS, and normal-pressure hydrocephalus. Treatrnent of Parkinson's syndrcnne also Inay reverse dernentia. 4. Watch for thiarnine deficiency in alcoholics as the calIse of delirium (Wernicke's en-cephalopathy, which claSSically presents with ataxia, ophthalmoplegia, nystagmns, and confusion). If untreated. it Illay progress to Korsakoff's syndrome (meJIlory loss with confabulation; usually irreversible). Give thiamine bek>fe glucose in an alcoholic to pre-vent precipitating Wernicke's encephalopathy. Causes of headache:

1. Tension headaches: lTlOst counl1on cause. Look {-(Jr long history of headaches and stress plus a feeling of tightness or sUlli,css, usually frontal or occipital and hilateral. Treat with stress reduction and acetalninophen or NSAID. 2. Cluster ht'adaches: unilateral. severe. tender; occur in dusters. Oxygen Inay abort an attack acutely. 3. Migraine headache: look fC)f aura. photophobia. nausea and vOlniting, and positive falnily history: Patients nlaY have neurologic symptoms during attacks. which usually begin hctweert ages 10 and 30. Treat and prophylax with antirnigrainc medication (e.g.. smnatriptan). 4. Tumor or rnass: look for progressive neurologic sYlnptonls, papilledema, intracranial hypertension (classically with nausea and vomiting. which Inay be projectile). I11ental status changes. and headache every day (classically worse in the morning). CT/MRI should be ordered. 5. Pseudotumor cerebri: may mimic tunl0r or ruass; botll cause intracranial hypertension. papilledenla. and daily headaches that: claSSically are worse in the lnornirlg and rnay be accOlnpanied by nausea and vomiting. Found in young obese f(~males. who are unlikely to have a brain tmllor; CT and MRJ are negative. Pselldonunor cercbri Inay cause permanent vision loss. Treatment is usually supportive; weight loss usually helps. Large doses of vitamin A. tetracyclines. and withdrawal frorn corticosteroids arc pOSSible causes. 6. Meningitis: look for fever. Brlldzinski's or Kernig's sign, cerebrospinal fluid findings (see above).

1. Subarachnoid hernorrhage: "worst headache" of patient's life; I1ny he due to congenital berry aneuryslll, rupture. or trauma. Look f()r grosslyhloody ccrehn)spiIJal fluid. Treat supportively.

90

Neurology

8. Extracranial causes: eye pain (optic neuritis, eyestrain from refractive errors, iritis, glaucoma), .middle ear pain (otitis media, nlastoiditis). sinus pain (sinusitis), oral cavity pain

(toothache), herpes zoster with cranial nerve iJwolvernent, and nonspecific (malaise from any illness). Cranial nerve (CN) lesions: 1. Olfactory (CN I): rarely important clinicaUy. Kalhnann's syndrome is anosmia plus hypogonadisrn due to deficiency of gonadotropin-releasing hOTJnone. 2. OptiC (CN 2): you must be able to localize the lesion through the resultant visual deficit (e.g., biteluporal hemiau()pia due to a lesion at the optic chiasln). as on Step I boards. Most comn10nly tested are bitelnporal hemianopia and Inonocular loss of vision (see ophtbalmology cbapter). 3. ()culoruotor (CN 3): the canse lnay be benign (hypertension, diabetes melli/us) or serious (aneurysm, tumor, uncal herniation). With beni.gn causes the pupil is spared (normal), and no treatment is needed. With serious causes the pupil is dilated and nonreactive ("hlown"). Urgent diagnosis and treatnlent are reqUired. 4. Trochlear (CN 4) and abducens (eN 6): see ophthalmology chapter.

S. Trigeminal (eN 5): innervates 111uscles of mastication and facial sensation (including the afferent limh of the corneal rdlex). Patients may have trigeminal neuralgia (tic douloureux), which is characterized by unilateral shooting pains in the face in older adults and often triggered by activity (e.g., blushing teeth). Treat with carhamazepine and anti-epilepsy medications. If the patient is young and/or female and/ or the disease is bilateral, COnsider urultiple sclerosis. Make sure to rule Out other causes, such as trllnor or stroke, 6. Facial (eN 7): innervates muscles of facial expression, taste in anterior two-·thirds of tongue, skin of external ear, lacrimal and ,,!ivary glands (except parotid gland), and stapediUS Illusde. Difh'rentiate between upper I1Iotor neuron lesions (the f()Tehead is spared on the affected side, and the cause is usually stroke or tUHlOf) and lower Juntor neuron lesj.ons (the foreJH:'ad is involved on the affected side, and the cause is usually Bell's palsy or tumor) of the facial nerve. Patients may be unable to close the eye; give artifIcial tears to prevent corneal ulceration. Patients with Bell's palsy IndY get hyperacllsis dne to slapedialmusc1e paralysis. rfCNs 7 and 8 arc afl"cted, think of possible cerebelJo.. pontine angle tunlor (e.g., acoustic neUrOll1a, especially in neuroflbroI1Iatosis), 7. Vestibulocochlear (CN 8): f<)f hearing and balance, Lesions cause deafness, tinnitus, and vertigo. In children, think (A' uleningitis as a cause. In adults, think of toxins and Illedicalions (aspirin, aUIinoglycosides,loop diuretics, cisplatin), tmllors (with CN 7 coinvolve·" ment, think of acoustic neuroIIld), or strok(~, 8. Glossopharyngeal (eN 9): innervates pharyngealll1Usdes and mucous IIlembranes (ar f\..~rent lhnb of gag reflex), parotid gland, taste in posterior third of tongue, skin of external ear, and carotid body and sinus. Look for loss of gag reflex and loss of taste in posterior third of tongue.

9. Vagus (eN JO): innervates IIluscles ofpaJate, pharynx, larynx (el1,"ent limb of gag reflex), taste buds in base of tongue, abdoluinal viscera, and skin of external ear. Look f()l' hoarseness, dysphagia, and loss ()f gag Or cough rdlex. Think of aortic aneurysms or tumors, especially Pancoast lung tlnnors, 10. Spinal accessory (eN 11): innervates stenl(JdeidOlnastoid and trapeZius muscles. With a eNl I lesion, the patient has trouble turning the head to the opposite side of lesion and Shoulder droop,

Neurology

91

11. Hypoglossal (CN 12): innervates muscles of the tongue. With a CN 12 lesion. a protfuded tongue deviates to the side of the lesion. Vitalnin deficiencies may present with neurologic signs and symptOIl1s:

1. Vitalnin B n .: denll:ntia. peripheral neuropathy, loss of vibration sense in lower extrclnities, loss of position sense, ataxia, spasticity, hyperactive reflexes, and positive Babinski's sign 2. Thiamine: peripheral neuropathy. confllSiotl, ophthalrnoplegia, nystagmus, ataxia, confusion, delirimn, dementia 3. Vitalllin E: loss of proprioception/vibratory sensation, areflexia, ataxia, and gaze palsy 4. Vitamin A: vision loss 5. Vitaulin B6 : peripheral sensory neuropathy (watch for isoniazid as a cause and give pro-phylactic 1\ to patients taki.ng isoniazid if given the choice) Five main types of seizures are tested on boards (although there are others): 1. Simple partial (local, lexal) seizures: may be motor (e.g., Jacksonian march). sensory

(e.g.. hallucinations), or psychic (cognitive or affective syrnptoms). The key point is that consciousness is not irnpaired. Treat with phenytoin, carbamazepinc, or valproate. 2. Complex partial (psychomotor) seizures: any simple partial seizure f(vllowed by impairment of consciousness. Patients perform purposeless mOVClllents and Inay becotne aggressive if restraint is attempted (people who get in fights or kill people are not having a seiz.ure!). The first~line agent is carbarnazepine; phenytoin and valproate also are eHt~ctive. 3. Absence (petit mal) seizures: never begin after the age of 20. They are brief (I 0--·30-seconcl duration), generalized seizures in which the main manifestation is loss of consciousness, often with eye or Inuscle flutterings. The classic description is a child in a claSSr00111 who stares off into space in the rniddlc ofa sent.ence (the child is not: daydreanling hut: having a seizure), then 20 seconds later reSUlnes the sentence. There is no postictal state (an ilnportant differential point). The flrst-line agent is ethosuximide; valproate also is eflective. 4. Tonic clonic (grand rHaI) seizures: the classic seizures that IndY have an aura; tonic muscle contraction is followed by clonic contractions, usually lasting 2--Sminutes. Patients often have incontinence and a postictal stale (drowsiness, confusion, headache, muscle soreness). Treat with phenytoin, carbarnazepine, or valproate. 5. Febrile seizures: between the ages of 6 1l1onths and S years old, children lnay have a seizure

due to fl~ver. The seizure is usually of the tonic-clonic, generalized type, and no specific seizure treatment is reqUired. Treat the underlying cause of the fever, if possible, and give acetaminophen. Such children do not have epilepsy, and the chances of their getting it afe just harely higher than in the general population. Make sure that aflected children do not have rneningitis, tmTIor, or other seriolls cause of seizure. The board question will give dues in the case description if you are expected to pursue work-up f()r a serious condition. Secondary seizure disorder may be caused by: M

Mass (ttum)f. hemorrhage)

M

Metabolic disorder (hypoglycemia, hypoxia. pbenylketonuria. hyponatremia)

Ii!l

Toxins (lead. cocaine, carbon

1!11

lnonoxid{~)

Drug withdrawal (alcohol, barbiturates, henzocliazepines, too-rapid anticonvulsant withdrawal)

Ill!

Cerebral cdeUla (severe or malignant hypertension; also watch fe)f phcochrornocytOlna)

j)

l~dalnpsia

92

Neurology

I!II

Central nervous systenl infections (rneningitis, encephalitis, toxoplasmosis, cysticercosis)

m Trauma m Stroke

Cysticercosis is due t() infection with the larval {()fm of Taenia solium, the pork tapeworrn, and Ill0St (Atcn is seen in patients with AIDS and inunigrants. On CT scan the lesion often is described as "ring-enhancing." 'treat with niclosanlide or praziquantel. In secondary seizures of any etiology. treat the underlying disorder and use diazeparll, or phenytoin acutely to control seizure. Note: For aU seizures, secure the airway and, if possible, roll the patient onto his or her side to prevent aspiration. Status epilepticus: when seizures of any type f()Uow one after the other with no intervening periods of consciousness. May occur spontaneously or result from toc}--rapid withdrawal of auticonvulsant~.Treat with IV diazepaIll, lorazepan1, or phenytoin. Rernember to protect the airway and intubate if necessary. Important points:

1. Hypertension Tnay cause seizures or convulsions, headache, and confusion, stupor, or mental status changes. 2. All anticonvulsants are teratogenic, and women need counseling about the risks of preg"~ nancy. Do a pregnancy test before starting an anticonvulsant. Cerebrovascular disease (stroke. cerebrovascular aCcident): the lnost corIlmon cause ofneuTo~ logic disability in the U.S. and the third leading cause of death. Ischemia from atherosclerosis is by far the most COll1l110n cause; other classic causes include atrial fibrillation with resultant clot formation and elnboli to the brain and septic emboli from endocarditis. Treatrnent fc)r acute stroke in evolution is supportive (e.g., airway, oxygen, IV fluids). Heparin is controversial and should be avoided if the patient is hypertensive; it should not be given until a hemorrhagic stroke has been ruled out by CT The vascular surgery chapter discusses the role of carotid endarterectmny, which is not done ell1ergently.

Transient ischcIUic attack (TIA): f(Jcal neurologic deflcit that lasts minutes to hours, then re~ solves spontaneously; often a precursor to stroke, The classic presentation is ipsilateral blind-ness (amaurosis fugax) and/or unilateral hemiplegia, hemiparesis, weakness, or chunsiness. Get a carotid duplex scan to look f()f stenosis. Heparin BUy be given acutely (if not contraindi-· cated), but f(Jf long-term therapy use aspirjn~-antiplatelet medications, or carotid endarterectmny (if carotid stenosis> 70%). Huntington's disease: autosonul dominant condition that usually hegins at 35-50 years of age. Look f(:Jr choreifonn movements (irregular, spasmodic, involuntary 11lOVenlenls of the ltmbs or facialmnsclcs) and progressive intellectual deterioration, dernentia. and psychiatric disturbances. Atrophy of the caudate nucleus Inay be seen on CT IMRI. Treatment is supportive; antipsychotics Tnay help. Parkinson's disease: classic tetrad of slowness or poverty of Illovemellt, muscular rigidity ("lead pipe" and "C()g--wheel"), resting "pill--rolling" trenlor (which disappears with IDovelnent and sleep). and postural instability (shuffling gait: and f(~stination). Patients also may have dementia and rIepression. The mean age of onset: is around 60. The calIse is loss of dopanlinergic neurons, espe-ciaHy in the substantia nigra, which projects to the hasal ganglia; the result is decreased dOpalI1ine in the basal ganglia. Drug therapy aims to increase dopa1nine. Options include lcvodopalcarbi·dopa, brorn(xTiptinc/pcrgolide, monoamine oxidase·-B inhibitors (sele~~iline), amantidine, anti~ ehohnergies (trihexyphenidyl, benztropine), and antihistamines (diphenhydramine).

Neurology

93

Note: Antipsychotics Inay cause Parkinson-like sYlnptoms in Sdlizophrenics. Treat with anti-cholinergics (henztropine, trihexyphenidyl) or antihistamines (diphenhydramine).

Tremor and chorea: resting lrCInor generally due to basal ganglia disease (chorea), intention tremor due to cerebellar disease, JndhemiballisIl1US (random, violent, unilateral flailing of the limbs) due to a lesion in the subthalamic nucleus. Besides Parkinson's disease, a resting lr{'Innr lnay be due to hyperthyroidislll, anxiety, drug withdrawal Of intoxication, or a benign (essential) hereditary trelllor (usually autosomal dorninant; look for a positive faInily history, and lIse beta blockers to reduce the tremor), Watch f()I'Wilson's disease (hepatolenticlliar degeneration) and asterixis (outstretched hands flap slowly and involuntarily) in patients with liver and kidney failure. Cerebellar disorders: in children, think of hrain tumor (cerebellar astrocytoma, mednlJoblastOlnas), hydrocephalus (enlarging head in infants younger than 6 ITlonths, Arnold-·Chiari and Dandy~Walker syndrornes), Friedreich's ataxia (autoscHnal recessive), or ataxia--tclangiectasia (the diagnosis is in the nalne). Friedreich's ataxia starts between 5 and J 5 years of age and pre-sents with areflexia, loss of vibration .or position sense, and cardiOlnyopathy. In adults, think of alcoholism, tumor, ischenlia or hemorrhage, or multiple sclerosis. Amyotrophic lateral sclerosis (Lou Gehrig's disease): an idiopathic degeneration of both upper and lower motor neurons that is 1norc common in males. The rnean age at onset is 55. The key is to notice a cOInbination of upper 1110tor neuron les_ion signs (spasticity, hyperreflexia, positive Babinski's Sign) and lower lDntor neuron lesion signs (fasciculations, atrophy, flaCCidity) in the saIne patient. Treatlnent is supportive, but 50% of patients die within 3 years of onset. "Floppy baby"syndrome (infants with hypotonia or flaccidity) may be caused hy two disorders: 1. Werdnig-Hoffnlann disease: a.utosonlal recessive degeneration of anterior horn cells in the spinal cord and brainstern (lower lllotor neurons). Most infants arc hypotonic at birth, and all are affecteel by 6 months. Look ft)r a positive family history, long and slowly progressive course of disease. T'reatrncnt is supportive.

2, Infant hotulism: look for sudden onset and a history of honey ingestion (or other home, canned f(x)ds). -Diagnosis is lnade by finding Clostridium botulinum toxin or organisms in the f(~ces. Treat on an inpatient basis with dose JIlonitoring of respiratory status. Patients may need intubation for respiratory llmsclc paralysis. Spontaneous recovery usually occurs "vithin 1 week. Peripheral neuropathies have multiple causes: 1. Metabolic: diabetes lllellitus (autollOlnic and sensory neuropathy), urcInia, hypotllyroidism

2, Nutritional: deficiencies of vitamin B /2' J\, (look !t)[ history o!'isoniazid), thiamine (dry beriberi), or vitamin B

3. Toxins and medications: lead (classic SyIUpt0111 is wristdrop or fc)()tdrop; look fc)}' coexist-ing central nervous systeul or abdominal synlptorns) or other heavymetaJs, isolllazid, vincristine, ethamhutol (especially optic neuritis), amilloglycosides (especially eN 8) 4. Postinfectionliunl1unization and autoiunllune: Guillain·-Barrc, syndrome, systernic lupus erythclnatosus, polyarteritis

HOeIOS,},

sclerodenna, sarcoidosis, amylOidosis

5. Trauma: carpal tunnel syndronlc (median nerve erltrapnlc:ut: at the wrist), pressure paral-ysis (radial nerve palsy in alcoholics), or fi'acturcs. Carpal tunnel syndrorne usually is due to repetitive phYSical activity but Inay be a presentation of aCf(}megaly or hypothyroidlsnI.

I.ook for positive Tind and Phalen Signs. 6.

lnf(~ctious:

Lyme disease, diphtheria, IllY, tick hite, leprosy

94

Neurology

Note: Nerve conduction velocity is slowed with a peripheral neuropathy.

Myasthenia gravis (MG): autoimmune disease that destroys acetylcholine receptors. MG usually presents in women aged 20-40. Look le)r ptosis, diplopia, and general muscle fatigability, especially toward the end of the day. Diagnosis is made with the Tensilon test. Injection of edrophoniurll (trade naIlle: Tensilon), a short-acting anticholinesterase, inlproves llluscle weakness. Watch f()[ associated thYlnOlllaS; lnosl patients with MG ilnprove after removal of the thynlus, which is considered part of standard treatrnent. Most patients have antibodies to acetylcholine receptors in their serum. Treat with long·-acting anticholinesterase (pyridostigInine, neostigmine). Eaton-Lambert syndrome: a paraneoplastie syndrome (classically seen with small cell lung cancer) characterized by rnuscle weakness, with sparing of the extraocular muscles (MG almost always has prOIllinent involvernent of extraocular rnuscles). Eaton--Larn bert syndrome has a dif ferent rl1echanisll1 of disease (ilnpaired release of acetylcholine fronl nerves) and a differential response to repetitive nerve stimulation (MG worsens, Eaton-Larnbert irnproves). Important points: 1. Do not fiJrget organophosphate poisoning as a causefbr ruyasthenic-likemusdc weakness. Usually it occurs with agricultural exposure. SynlptOllls of parasympathetic excess also are present (e.g., rniosis, excessive bronchial secretions, urinary urgency, diarrhea). Edrophoninn1 caUSes worsening of the rnuscular weakness. Treatrllent is atf()pine and pralidoxilne.

2. Alninoglycosides in high doses Inay cause myasthenic-like rnuscular weakness and prolong the effects of muscular blockade in anesthesia. Muscular dystrophy: most commonly dne to Duehenne muscular dystrophy, an X-linked recessive disorder of dystrophin that usually presents in boys aged 3~7. Look for mnscle weakness, markedly elevated creatine kinase, and pseudohypertrophy of the calves (due to fatty and Hbrous infiltration of the degenerating muscle). IQ often is less than noruIaL Gowers' sign is classic (when the patient tries to rise from a prone position, he "walks" the hands and feet toward each other). Musde biopsy establishes the diagnosis. Treatment is supportive; most pa~ tients die by age 20. Other muscnlar dystrophies: 1. Becker muscular dystrophy: also an X-linked recessive dystrophin disorder, hut milder

2. Facloscapulolnuneral dystrophy: autosomal dominant disease that affects the areas in the name (face, shoulder girdle) and begins hetween ages 7 and 20. Ufe expectancy is normal. 3. Limb-girdle dystrophy: affects pelvic and shoulder museIes; begins in adulthood. 4. Mitochondrial myopathies: interesting because they are inherited mitochondrial defects (passed only fimn mother to ompdng; males cannot transmit). The key phrase is "ragged red fibers" on biopsy specimen. Ophthalmoplegia usually is present. 5. Myotonic dystrophy: autosomal dominant disorder that presents between 2.0 and 30 years of age. Myotonia (inability to relax museIes ) classically presents as inability to relax the grip (inahility to release a handshake). Look Ihr coexisting menta! retardation, baldness, and testicular/ovarian atrophy. '[rcatnIent is supportive and includes genetic counseling. Diagnosis is clinical.

Note: Do not fbrget the rare glycogen storage diseases (autosomal recessive) as a cause l()r 1I1UScu!ar weakness (especially McArdle's disease, a delkiency in glycogen phosphorylase that is relatively nlild and presents with weakness and crarnping after exercise).

CHAPTER

13

Immunology

Types of hypersensitivity reactions: 1. Type I (anaphylactic): dllC to pref()nned IgE antibodics, whicb callse release of vasoactive amines (e.g., histamine, leukotrienes) [rol11 mast cells and basophils. Examples arc anaphylaxis (bee stings, food allergy [espccially peanuts and shellnsbj, medications [especially penicillin and snlfa drugs], rubher glove allergy), atopy, hay fever, urticaria, allergic rhinitis, and some forms of astlul1a. H

With chronic type I hypersensitivity (atopy, some asthma, allergic rhinitis), look felf eosinophilia, elevated IgE levels, family history, and seasonal exacerbations. Patients also ll1ay have allergic "shiners" (bilateral infraorbital edenla) and a transverse nasal crease (from frequent nose Tubbing). Pale, bluish, edeluatous nasal turbinates with l1UllY eosinophils in clear, watery llasal secretions also are classic.

ll!I

If patients have nasal polyps, do not give aspirin; you attack.

11

'Treat anaphylaxis imrnediately by securing the airway. Laryngeal edema lnay prevent intubation, in which case do a cricothyrotOluy, if needed. Give subcntaneous epineph~ rine, then an antihistamine. Steroids are Sotnetilnes given f(,n' severe reactions, but only if other options afe not present.

Ilm

rtl

filay

precipitate a severe astluuatic

Watch f()f C I esterase inhibitor (cOlllplement) deHciency as a cause of hereditar)' an~ gioeclema. Patients have dilh,se swelling of lips, eyelids, and possibly the airway, unrelated to auy allergen exposure. The deficiency is autosomal dorninallt; look fOf a positive tunily history. C4 c0l11plenlent i,s low. Treat acutely as anaphylaxis; androgens afC llsed for long-term treatment to increase liver production ofel esterase inhibitor. Skin testing may identify an allergen if it is not obvious.

2. Type II (cytotoxic): due to pref(mned IgG ancllgM, which react with antigen and cause secondary inflammation. Exanlples are autoimmune henlOlytic anemia (classic causes are methyldopa or penicilJin/sulfa drugs) and other cytopenias caused by antihodies, such as idiopathic thromhocytopenic purpura, transfusion reactions, erythroblastosis f(;talis (Rh incOlnpatihility), Goodpasture's syndrmne (watch for Hnear innllunof1uoresccnce on kidlley biopsy), myasthenia gravis, Graves' disease, pernicious anelnia, peIllphigus, and hyperacute transplant rejection (as soon as the anastOl1lOsis is made at transplant surgery, the transplanted organ deteriorates in frollt of your eyes).

Note: With anemia, watch {()Y a positive Coombs' test; in pregnancy, watch rect Coombs' test.

f()f

a positive indi--

95

96

Immunology

3. Type III (immune complex-mediated): due to deposits of antigen-antibody complexes (usually in vessels) that cause an inflalunlatory response. Examples are serum sickness, lupus, rheumatoid arthritis, polyarteritis nodosa, cryoglobulinemia. and glomerulonephritis (e.g.. chronic hepatitis). 4. Type IV (cell-mediated [delayed]): due to sensitized T lymphocytes. which release inflammatory mediators. EXJlnples include tuberculosis skin test, COntact dennatitis (especially poison ivy, nickel earrings, coslnetics, luedicati0I1-;), chronic transplant rejection, granulomas. Hnman immunodeficiency virus (HIV)/ AIDS: iuitial seroconversion may present as a mononucleosis-type syndrome (fever. malaise, pharyngitiS, rash, Iymphadenopathy)_ Keep se· roconversiol1 in the back of your lnind as a differential diagnosis for any sore throat or EpsteinBarr virus-type presentation. Diagnosis is 111ade with the enzYlne-·linked irrnnunosorbent assay (ELISA), which,ifpositivc, should be eonfirmed with a second assay. If the second assay is positive, conHnn with a Western blot test. Do all tests before you tell the patient anything! It takes at least I month for antibodies to develop; therd()re, jf a patient COlnes to you for testing because of reC(~nt risk-taking behavior, you should retest the patient in 6 rllonths if the initial test is negative. Important points: 1. Once the diagnosis of HIV inl"ction is made, the patient should get a CD4- count every 6 111onths.

2. Antiretroviral therapy should be started when the CD4 count falls below SOD (or sooner). 3. Once the CD4 count is less than 200, start prophylaxis f()f Pneumocystis carinii pnemTIonia (PCP). Use trimethoprim-sulfamethoxazole (TMP-SMX) or pentamidine (if the patient is allergic to or intolerant ofTMP·SMX). 4. Once the CD4 count is less than 100, start prophylaxis for Mycobacterium avium intmcellularc with rifahlltine; consider cryptococcal and candidal prophylaxis with fluconaz.ole. 5. Once CD4 < 200, the patient automatically is considered to have AIDS (even without opportunistic inf:t~ctions). 6. Give measles, mumps, and rubella (MMR) vaccine to HIV·positive patients (the only live vaccine given toHIV patients!). 7. Give pneumococcal, hepatitis B, inactivated polio vaccine, and annual influenza vaccines to all HlV-positive patients. 8. Do annual purified protein derivative test f()r tuberculosis in HIY'patients; get an annual chest x·-ray if the patient is anergic. 9. Do not give oral polio vaccine to }-IIV--positive patients or their contacts. 10. Watch I,,, Kaposi's sarcoma or non-Hodgkin's lymphoma (especially primary B·cell lyrnphOlnas of the central nervous systern). 11. A positive India ink preparation of the cerebrospinal fluid means Cryptococcus neoformam rnenin gi tis. 12. Riug---enhancing lesions in the brain usually luean toxoplaSl1lOSis or cysticfTcosis (Yaenin soHum) . 13. Other conunonly seen lIlV sequelae include wasting syndrorl1c (progressive weight loss), dernentia, peripheral neuropathies, thrOll1bocytopenia, and loss of delayed hyper-sensitivity (type IV) on skin testing (anergy).

Immunology

97

14. Give pregnant HIV·positive patients zidovudine (AZT) , and give the infant AZT ft)f 6 weeks after birth. This protocof reduces mother-to-child transmission from roughly 25% to 8%. The infant may have a positive HIV test for 6-12 months because of maternal antibodies. Retest after 6--12 111onth5. Recent studies indicate that: cesarean section also may reduce tran~anission. 15. HlV-positive mothers should not breast-feed, because they can transmit the disease to their infants through breast milk. 16. the ganciclovir fl)T cytmuegalovirus retinitis; foscarnet is the second choice. 17. In any patient with HIV and pneumonia, think of PCP nrst. took for severe hypoxia with normal x-ray or diffuse, bilateral interstitial infiltrates. Usually the patient has a dry, nonproductive cough. PCP may be detectable with silver stains (Wright-Giemsa, Giemsa, Dlethenalnine silver) of induced sputurn; jfnot, use bronchoscopy with bronchoalveolar lavage and brush biopsy to make the diagnosis. 18. Any adult patient witb thrush should make you think of HIV or lenkemia. 19. Any young adult who presents with herpes zoster shonld make you think ofl-UV 20. Cryptosporidium and Isospora spp. are diarrheal infections uniquely seen in HIV-positive patients Primary immunodeficiencies: because they are rare, your job is simply to recognize the classic case presentation: 1. IgA deficiency: most COInmon priluary imlTIUnodefkiency. Look for recurrent respiratory and GI infections. IgA is low, and IgG subclass 2 may be low. Do not give im .. munoglobulins; you may cause anaphylaxis due to development of anti..IgA antibodies. Alternatively, in any patient who develops anaphylaXis after immunoglobulin exposure, you should think ofIgA deficiency. 2. X-linked agammaglobulinemia (Bruton's agammaglohulinemia): X-linked recessive disorder that affects males. B-cells are low or absent; infections begin after 6 months when luaternal antibodies disappear. Look for recurrent lung and sinus infections with Streptococcus and Hacmophilus spp. 3. DiGeorge syndrome: caused by hypoplasia of the third aud fourth pharyngeal pouches. Look for hypocalcemia and tetany (hom absent parathyroids) in the first 24-48 hours of life. Also look for absent or hypoplastic thymus and congenital heart defects. 4. Severe combined immunodeficiency (SClD): may be autosomal recessive or X..linkecL Many cases are due to adenosine deaminase deficiency (autosollul recessive). Patients have B- and Tcell defects and severe infections in the first few months of life, and cutaneous anergy usually is present. Other signs include an ahsent or dysplastic tbymus aud lymph nodes. 5. Wiskott-Aldrieh syndrome: X-linked recessive disorder that affects males. look for classic (dad: eczema, thrombocytopenia (look for bleeding), and recnrrent infections (usually respiratory). 6. Chronic granulolnatous disease: usually X-linked recessive disorder that affects inaies. Patients have a defect in reduced nicotinamide adenine diuucleo(ide phosphate (NADPH) oxidase activity and thus get recurrent inf<,.ctions with catalast.>positive organisills (e.g., Staphylococcus aureus, Pseudomonas sp.). The diagnosis is clinched if the question luentions de~ fkient nitrohlue tetrazolhml dye reduction by granulocytes. This test lneasures respiratory burst, which patients lack.

911

Immunology

7. Chediak-Higashi syndrolllc: usually autosornal recessive, look for giant granules in neutrophUs and associated oculocutaneous albinislll. The cause is a defect in microtubule polynlcrization. 8. Complement deficiencies: C5--C9 deficiencies cause recurrent Neisseria infections; specific c0111plement cOIllponent is low~ 9. Chronic mucocutaneous candidiasis: a cellular immunodeficiency speciH.c f()f Candida sp. Patients have candidal thrush, scalp, skin, and nail infections and anergy to Candido sp. with skin testing. Hypothyroidism is often an associated flnding. The rest of imnnlne function is intact.

10. Hyper IgE syndrome (Job-Buckley syndrome): patients get recurrent staphylococcal in·· lectioJls (especially of the skin) and have extremely high IgA levels. They also commonly have fair skin, red hair, and <:czenla.

Questions otten ask yon to give genetic connseling to a parent or to predict the likelihood of having a second affected child after the first is born with a given disease. Because it is assumed

that you know the inheritance pattern of the disease, the following lists should come in handy: Autosomal domiuant: look for affected mother or father who passes the disease to SO% of offspring: .. von Willebrand disease III NeurofibrOIllatosis:

cafe·-au-lait spots, profuse peripheral nerve ttnnors, acoustic neurOlna

.. Mnltiple endocrine neoplasia (MEN) type I and II syndromes .. Achondroplasia: diagnosis by picture of a patient .. Marian syndrome: tall patient with arachnodactyly, mitral valve prolapse, aortic dissection, lens dislocation S

Huntington's disease

mFamilial hypercholesterolemia: look for xanthomas, early coronary artery disease,

markedly elevated cholesterol .. Familial polyposis coli .. Adult polycystic kidney disease .. Hereditary spherocytosis _Tuberous sclerosis: hypopigmented skin macules. seizures, lllental retardation, central nervous system hanlartomas, rhabdomyOlnas, renal tumors

.. Myotonic dystrophy: muscle weakness with inability to release grip, balding, cataracts, mental retardation, cardiac arrhythmias Autosomal recessive: look for family history and unaffected parents who pass the disease to 25(% of children: .. Sphingolipidoses (e.g., Tay-Sachs disease, Gaucher's disease; exceptiou is l'abry's disease, which is X linked) ~

Mucopolysaccharidoses (e.g., Hurler's disease; exception is Hunter's disease, which is. X~linked)

"Glycogen storage diseases (e.g., Pompe's and McArdle's disease.) .. Cystic fibrosis

99

100

Genetics II

Galactosctnia: look for congenital cataracts, neonatal sepsis; avoid galactose"" and lactose-

containing foods • Amino acid disordcrs (e.g.. phenylketonuria. alkaptonuria) .. Sickle cell disease _Children's polycystic kidney disease II Wilson's

disease

.. Hemochromatosis (usually) .. Adrenogenital syndrome (e.g.. 21 ~hydroxylase deficiency) X~linked

recessive: look [or affected fathers to pass the gene only to their daughters. who become carriers but do not gel disease. Carrier mothcrs (family history in male relatives) who

pass the gene to their sons, who get the disease:

.. Hemophilia II

G~6~P··D defiCiency

.. Fabry's disease III II

II

Hunter's disease Lesch~Nyhan syndrome: hypoxanthine~guaninc phosphoribosyltransferase enzyme defi~ ciency. Look for mental retardation and self~mutilation (patients may bite off their own fingers)

Duchenne muscular dystrophy

"Wiscott~Aldrich syndrome III

Bruton's agalIlruaglobulinemia

II Fragile X syndrome: second ,most COffir11011 Cause of rnel1tal retardation in rnales (after

Down syndrome). Patients have large lestes. Polygenic disorders: relatives are more likely to have disease, but there is no obvious heritable pattern: III

Pyloric stenosis

• Cleft lip andlor palate .. Type II diabetes • Obesity .. Neural tn be defects mSChizophrenia

• Bipolar disorder iIlI

Ischcrnic heart disease

I!i!

A1coholisrn

Chromosomal disorders M

Down syndrome (trisorny 21): lnost COlnnlon known cause of rnental retardation. The

major risk factor is age of the mother (I 11 sao offSpring of 16~year·oJcll110thers. J I2S f(lf 4·S-year~old

rIlothers).At birth look f()r hypotonia, transverSe palnlar crease, and

character~

istic facies. Congenital cardiac defects (especially ventral septal defect) are C0l11nlOn, and patients arc at increased risk f()r leukemia, duodenal atresia, and early Alzheimer's disease.

Genetics

101

Ili Edwards syndrome (trisomy 18): more common in females than males. Patients are small for their age and have mental retardation, small head, hypoplastic mandible, low-set ears, and clenched fist with index finger overlapping third and fc)Urth fingers (almost pathognomonic). Iil

Palau's sylldrOlue (trisomy 13): nlental retardation, apnea, deafness, holoprosencephaly (fusion of cerebral hemispheres) > myelolnenil1gocele. cardiovascular abnonnalities, rocker-bOllOIIl

feet.

IliTurner's syndrome (XO instead of XX): lymphedema of neck at birth, short stature, webbed neck, widely spaced nipples, amenorrhea, and lack of breast development (due to priInary ovarian failure). Coarctation of the aorta is horse··shoe kidneys or cystic hygrorna. Ili

liS

COD11110n,

and patients

lllay

have

Klinefelter's syndrome (XXY): tall patient with microtestes « 2 ern in length), gynecomastia, sterility (the classic presentation is for infertility), and decreased IQ. Cri--du--chat: due to a deletion on the short ann of chromOSOlne S; look f()r high~pitched cry like a cat along with severe mental retardation.

Important points: 1. The most rapid increase in population in the u.s. (percentage·wise) is in people over 65. Within this group, the over-85 suhgroup is increasing nl0st rapidly 2. At age 80 patients have half the lean body mass of a 30-year-old. Because basal metabolic rate depends on lean body mass, elderly patients need fewer calories. Tliey also need lTIOre sodium. vita111in Bn . vitaulin D (and/or calciUln). f(Jlate. and nonhclne iron.

J. Normal clianges in elderly: slightly impaired immnne response, visual (presbyopia) and hearing (presbycusis) iInpairnlcnt, decreased muscle Inass, increased fat deposits, osteoporosis, brain changes (decreased weight, enlarged ventricles and snlci), and slightly decreased ability to learn new rnaterial. 4. Nonnal sexual function changes in men: elderly men take longer to get au erectiou and have an increased refractory period (after ejacnlation it takes longer bcfore the patient can have another erection). Delayed ejaculation is comnlOD, and th,e patient may ejaculate only 1 of every 3 times that he has sex. Impotence and lack of sexlIal desire are not normal and should be investigated. Look felr psychological (depression) as well as physical causes, Medications, especially antihypertensives, are notorious culprits. 5. Nonnal sexual function changes in WOlnen: for decreased lubrication, advise water~soJ" uble lubricants. Atrophy of clitoris, labia, and vaginal tissues Inay cause dyspareunia; treat with estrogen creaIn. Delayed orgasm is conunon, hut lack of sexual desire is not normal and should he investigated (psychological or physical (:anses). 6. The best prophylaxis for pressure ulcers in an immobilized patient is frequent turning. 7. Sleep changes: elderly people sleep less deeply, wake up more frequently during the night, and awaken earher in the morning. They take longer to fan asleep (longer sleep latency) and have less stage 3 and 4 and rapid-eye-movement sleep. 8. Depression in the elderly JUdY present as den1el1tia (i.e., pseudodeIl1entia). Look fijr a history that would trigger depression (e.g., loss of a spouse, terminal or debilitating disease).

9. In 1993, 12 'i/o of the U.S. popnlation was over age 65. 10. Fifteen percent of people over age 65 suH(~r from dementia. The rnost COlnrnOll causes of dernentia, in order, are Alzheirner's disease (gradually progressive, neurofihrillary tangles) and Itmltiinfarct (step·-wise, risk factors for cerebrovascular accident). Other causes inclnde EIV and Pick's disease. 11. Only S% of people over the age of 65 live ill nursing homes.

103

CHAPTER

16

PREVENTIVE MEDICINE American Cancer Society guidelines for cancer screening in asymptomatic patients*

Important points: 1. Colonoscopy is equal to flexible' sigmoidoscopy plus barium Cr1elna (BE) f()r colon canc('}' screening. Colol1oscopy is llwn' sensitive and 1110rc expensive. Choose flexihle SigOlOidos·copy + BE over COIOHOSCOpy If)r hoards, Double--contrast (air--contrast) BE is superior to sjngle~contrast (no air) BE.

105

106

Preventive Medicine, Epidemiology, and Biostatistics 2. In general, urinalysis (scr~ening fi)! urinary tract cancer that results in heu1aturia). acid phosphatase (prostate cancer), alpha-fetoprotein (liver and testicular cancer), and other serum markers are not appropriate f()f screening asynlptornatic patients with no physical findings, but look for these abnonnallab values to show up jn questions as a clue to diagnosis. Prostate-specific anlJgen (PSA) is beC0111ing popular as a prostate cancer screening test, 1mt does nol replace rectal eXJID.

Immunizatioll.f\ in adults

FI~,mQqx~Y$ti;

'Herndphili;lCS ,an of International 1:raveleni

Inmates

,

-AhyOJle whoasks}or}t

InfhtenrJl

Given

Patt¢t

ts over u.s em _cardit?vasQilat -dis¢:rl;b:~r-s

Tetanus

ster

iugomplete vacdrmtloil,Ond;1topdean/ ns

Preventive Medicine, Epidemiology, and Biostatistics

107

EPIDEMiOLOGY Per-year rates commonly used to compare groups: 1. Birth rate: live hirths/ ] 000 population 2. Fertility rate: live births/ I 000 population of females age 15-45 yr 3. Death rate: deaths/ I 000 population 4. Neonatal mortality rate: neonatal deaths (in the first 28 days) / 1000 live births 5. Perinatal mortality rate: neonatal deaths + stillbirths per] 000 total births ., The major cause is prematurity "The neonatal mortality rate is roughly 6/ I 000 (higher iu blacks) M

The fetal mortality rate is roughly 9/1000 (higher in nonwhites)

"The perinatal mortality rate is roughly 15/1000 II

A stillbirth (fetal death) is defined as a prenatal or natal death after 20 weeks' gestation

6. Infant mortality rate: deaths (from 0-1 year old)/IOOO live births (the top three causes, in descending order, are congenital abnormalities, low birth weight. and sudden infant death syndrome) 7. Maternal mortality rate: maternal pregnancy-related deaths (deaths during pregnancy or in the first 42 days aher delivery)/ 100,000 live births liThe top three causes are pulmonary embolism, pregnancy-induced hypertension, and hemorrhage II

The rate increases with age and is higher in blacks

Important points: 1. Medicare is health insurance for people who are eligible for Social Security (primarily people> 65 years old as well as the permanently and totally disabled and patients with end-stage renal disease). Nursing home care is paid by Medicare only in the short term after a hospital admission; then it is paid by the patient (if the patient has no money, the state usually pays). 2. Medicaid covers the indigent and poor who are deemed eligible by the iudividual states.

BIOSTATiSTICS Review this section of Step I material for some easy points. Sensitivity: ability to detect disease. Mathematically, sensitivity is calculated by dividing the number of true positives by the number of people with the disease. Tests with high sensitivity are used for scretoning. They may have false positives but do not miss many people with rbe dis· ease (low f:tlse··negative rate). Specificity: ability to detect health (or nondisease). Mathematically, specificity is calculated by dividing the number of true negatives by the number of people without the disease. Tests with high specificity are used for disease confinnation.They may have false negatives but do not call anyone sick who is actually healthy (low false-positive rate). The ideal confirmatory test must have high sensitivity clUd high specificity; otherwise, people with the disease may be called healthy.

lOll

Preventive Medicine, Epidemiology, and Biostatistics The trad.e-offbetween sensitivity and specificity is a classic statistics question. Understand how changing the cm·-off glucose value in screening f()f diabetes (or changing the value of any of several screening tests) will change the llmnber of true and 61<.;('. negatives and Lruc and false positives. If the cut--off value is raised, f(~wer people will be called diabetic (lllOfe false nega-lives, ftwer false pOSitives), whereas if the cut-off value .Is lowered, more people will be called diabetic (fewer f~11se negatives, Inure false positives). Positive predictive value (PPV): when a test comes back positive {by disease, the PPV mea-" SlIreS how likely it is that the patient bas the disease (probability of having a condition, given a positive test). Mathematically, PPV is calculated by dividing the number of true positives by the mnnber of people with a positive test. PPV depends on the prevalence of a disease (the higher the prevalence, tbe greater the PPV) and the sensitivity/specificity of the test (e.g., an overly sensitive tcst that gives rnore false positives has a lower PPV). Negative predictive value (NPV): when a test comes back negative f()f disease, the NPV mCd-slln~s how likely it is that the patient is healthy and does not have the disease (probability of not having a condition, given a negative test). Mathematically, NPV is calculated by dividing the munber of true negatives by the rHllnber of people with a negative test. NPV depends on prevalence and sensitivity / specificity just like PPV: Tlie higher the prevalence, the lower the NPV In addition, an overly sensitive test with lots of false positives willrnake the NPV higher.

Attributable risk: nuntber of cases attributable to one risk factor; in other words, the al110unt by which you can expect the incidence to decrease if a fisk factOf is removed. For example, if the incidence rate of lung cancer in the general population is 1/ 't 00 and in smokers it is J 01 100, the attributable risk of smoking in causing lung callcer is 91 I 00 (assuming a properly matched contro]).

Relative risk (RR): cOlllpares the cHsease risk in the exposed population to the disease risk in the unexposed population. RR can be calculated only aft-er prospective Of experimental studies; it cannot be calculated frOJn retrospective data. RR greater than '1 is clinically signiflcant. Odds ratio (OR): ust:d only for retrospective studies (e.g., case-control). OR compares disease in exposed and nOlldisease in unexposed populations with disease in unexposed and nondisease in exposed populations to deLennillC whether there is a dint~rencc between the two. Of' course, there should be, J110re disease in exposed than unexposed populations and more llondisease in unexposed than exposed populatioll,:). OR is a less than perfect way to estimate relative fisk. Get in the habit of drawing a 2 X 2 table to Inake calculations easier: DiseaSe

(+) r-~~-r-~-,

Sensitivity", AI(A Specificity;= D/(B

+ C)

+ D)

PPY '" A/(A + B)

NPV "' D/(C + D) Exposure

Oddsratio ;~ (A X D)/(B X ' - - - - - ' - - - - ' Relative risk'" (AI(A Attributablerisk "' [AI (A

B)]

+

C) [C/(C + D)] B)] ~

+ D)]

Preventive Medicine, Epidemiology, and Biostatistics

109

Standard deviation (SD): wi th a normal or bell-shaped distri hution, 1 5D holds 68'10 of values, 2 5D holds 9S% ofvalues, and 3 5D holds 99.7% of values. The classic question gives you the mean and standard deviation and asks you what percentage of values will be above a given value; variations on this question are abo COllunOll. In a normal distribution, the mean ==median : : :; rnode. The lnean is the average, the lnedian is the middle value, and the lTl()de is the ITJOst COUllllon VahH\ Questions may give you several nUlnbcrs and ask f<)r their I1H'an, lucchan, and l11ode.

Skewed distribution: a positive skew is asymmetry with an exccss of 11.1gh values (tail on right, lucan > median > mode) ; anegalive skew is asymrnetry with an excess of low values (tail on len, rncan < lnedian < nlOdc). These are not nonnal distributions; tillIs, standard deviation and lllean afe less meaningful values.

Reliability of a test (synonYlnous with precision) : measures the reproducihility and consis-tency of a test (e.g., the concept of interrater reliability: if two diH(:rent pe()ple administer the saIne test, they will get the same score if thc, test is reliable). Random error reduces reliability/precision (c.g., limitation in Significant figures). Validity of a test (synonymous with accuracy): JlleaSUrcs the trueness ( ) f llH:.'asurenlent--whether the test measures what it cJairns to measure. For example, if you give a vaHd 1Q test to a genius, the test should not indicate that he or she is retarded, Systenlatic error reduces valid ity / accuracy (e.g., miscalibrated equipment). Correlation coeflicient: rneasures the degree of relationship between two VallIeS, The range of the coefHcient is -1 to + I. The inlportant point in detennining the strength of the relationship between the two variables is how br the nUlnher is from zero. Zero equals no association what-soever; positive one (+ 1) equals a perfi:.'ct positive correlation (when one variable increases, so cloes the other); and negative one (---1) equals a perfect negative correlation (when one vari-able increases, the other decreases). Use the absolute value to give you the strength of the cor-, relation (e.g., ~-0.3 is a stronger correlation than +-0.2). Confidence interval (CI): when you take a set of data and ~alculatc a mean, you want to say that it is equivalent to the 1nean of the whole population, but usually they are not exactly equal. The CI (usually set: at 95%) says that you are 950;() confident that the population luean is within a certain range (usually within 2. SD of the experimental or derived lucan). For cxaluple, if you sample the heart rate of 100 people and calculate a mean of 80 bpnl and a 5D of 2, your confidence interval (confidence 1inlits) is written as 76 < X < 84- =:.: O.95.This ]lH.'JllS that you are 95°!l) certain that the luean heart rate of the whole population (X) is between 76 and 84. Different types ofstndies (listed in decreasing order of quality and desirahility): 1. ExperiInental: the gold standard, which compares two equal groups in which one vari~ able is lnanipulated and its effect is rneasured. Renwluber to use double-blinding (or at least single-blinding) and well-matched controls.

2. Prospective, longitudinal. cohort, incidence, follow- up: choose a salnple and divide it into two groups based on presence or absence of a risk factor and f()llow the group over tiTne to see what diseases they develop (c.g., f()llow people with and without asynlpto~ matic hypercholesterolenlia to see whether people with hypercholesterolcluia have a higher incidence of myocardial infJ.rclion later in life). This approach sOlnetirnes is called an ohservalional study because all you do is observe. Relative dsk and incidence can be calculated, Prospective studies are tilne,,-cOIlsluni:ng, expensive, and good fc)r COlnlllon diseases, whereas retrospective studies are less expensive, less time--consuming, and good f()f fare diseases.

110

Preventive Medicine, Epidemiology, and Biostatistics

3. Retrospective/case-control: samples are chosen after the fact based on presence (cases) or absence (controls) of disease. Information can then be collected about tisk factors; for example. look at people with lung cancer vs. people without lung cancer and see if the people with lung cancer smoke more. An odds ratio can be calculated, but you cannot calculate a true relative risk· or measure incidence frOl}} a retrospective study. 4. Case series: good for extremely rare diseases (as arc retrospective studies). Case series simply describe tbe clinical presentation of people with a certain disease and may suggest the need for a retrospective study. 5. Prevalence survey/cross-sectional survey: looks at prevalence of a disease and prevalence of risk factors. When comparing two diHerent cultures. you may get an idea about the cause of a disease, whtch call be tested with a prospective study (e.g., more colon cancer and higher· fill diet in U.S. vs. less colon cancer and low··fat diet in Japan). Incidence: the number of new cases of disease in a unit of time (generally I year, but any time frame can be used). Incidence rate also equals the ahsolute risk (to he differentiated from relative or attributable risk). Prevalence: the total munber of cases of disease that exist (new or old). Important points: 1. The classic question about incidence and prevalence: when a disease can be treated and people can be kept alive longer but the disease cannot be cured, what happens to the incidence and pn~valence? Answer: nothing happens to incidence, but prevalence will increase as people live longer. In short-term diseases. such as tbe flu, incidence may be higber than prevalence. whereas in chronic diseases. such as diabetes mellitus, prevalence is greater than incidence, 2. An epidemic occurs when the observed incidence greatly exceeds the expected incidence. Comparison of data: 1. Chi-square.d test: used to compare percentages or proportions (nonnumeric dat.a, also called nominal data) 2. T--test: used to compare two Ulcans

3. Analysis of variance (ANOVA): used to compare three or 1110re means P-value: the board eXaItl always contains one or lnore questions about the significance of the p-value. If someone tells you that p < 0.05 for a given set of data, there is less than a S% chance (because 0.OS=5%) that these data were obtained by random error or chance. Ifp < 0.01. the chance that the data were obtained by random error or chance is less than I %. For example, if I tell you that the blood pressure in my controls is J 80/! 00 mmHg but decreases to 120/70 mmHg after administration of drug X and that p < 0.10. tbere is less than a 10% chance that the difference in blood pressure was due to random error or chance. However, there is up to a 9.99999% chance that the result is due to random error or chance. For this reason, p < 0.05 is comnlOuly used as the cut-,off fix statistical significance. Three points to re-· member: (I) the study lllay still have serious flaws, (2) a low p-value does not imply causation. and (3) a study that has statistical signiflcance does not necessarily have clinical Significance. For example, if! tell you that drug X can lower the blood pressure from 130/80 to 128/80, P < 0.000000000000000001, you still would not use drug X. 'The p-value also ties into the null hypothesis (the hypothesis of no diflerencc). For example. in a drug study about hypertension, the null hypothesis is that the drug docs not work; any diflereuce

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111

in blood pressure is due to random error or chance. When the drug works beautifully and lowers the blood pressure by 60 points, I bave to reject the null hypothesis, because clearly the drug works. When p < O.OS, I can confidently reject the null hypothesis, because the pvalue tells Inc, that there is less than a 5% chance that the null hypothesis is correct----·.and if the I1Ull hy.. pothesis is wrong, the difference in blood pressure is not due to chance and 1I1USt: he due to the new drug. In other words, the p-value represents the chance ofmaking a type I error (clainl-tng an eff{~ct or diH(~n:nce when none exists, rejecting the null hypothesis when it is true). If p < 0.07, there is less than a 7% chance that you are nuking a type I error if you claim a dHf(:r" cnee in blood pressure between control and experimental groups. Type II error is to accept the null hypothesis when it is false (the hypertension drug works, but you say that it does not). Power: probability of rejecting the null hypothesis when it is false (a good thing). The best way to increase power is to increase sample size. Experimental conclusions and errors: the exam Inay give you data and the experinlenter's conclusion and ask you to eXIllain why the conclusion should not he drawn or to point out flaws in the experimental design: 1. Confounding variables: an unmeasured variable af!t:cts both the independent (manipulated, experimental variable) and dependent (outcorne) variables. For eXJrl1ple, an experimenter rneasures nmuber of ashtrays owned and incidence of lung cancer and finds that people with lung cancer have nl0re ashtrays. He concludes that ashtrays cause lung cancer. Sl110king to-bacco is the confcmnding variable, because it causes the increase in ashtrays and lung cancer. 2. NonrandOlll or nonstratifiecl sanlpling: city A and city B can be cOlnpared but :may not be equivalent. For example, if city A is a retirement cor11l1Ulnity and dty B is a college town, of course city A will have higher rates of mortality and heart disease if the groups arc not stratified into appropriate age--specifIc cOluparisons. 3. Nonresponse. bias: people fall to return surveys or answer the phone for a phone survey. If nonresponse is a significant percentage of the results, the experilnent will sufft:r. The nrst strategy is to visit or call the nonresponders repeatedly in an atten1pt to reach theln and get their response. If this strategy is unsuccessful, list the nonresponclers as unknown in the data analysis and see if any results can be salvaged. Never luake up or assume responses! 4. Lead tilue bias: due to tinle clifferentials. The classic example is a cancer screening tcst that claims to have prolonged survival cOluparecl with old survival data, when in fact the difference in survival is clue only to earlier detection, not to iInproved treatment or prolonged survival. 5. Admission rate bias: in cOlnparing hospital A with hospital B f()r 1110rtality due to l11Yocardial infarction, you find that hospital A has a higher mortality rate. But this Hnding may be due to tougher hospital admission criteria at hospital A, which admits only the sickest patients with myocardial infarction and thus has higher rl10rtaHty rates, although their care ll1ay be superior. The same hias can he f(mud in a surgeon's mortalityIITI(rrhid.ity rates if the surgeoll takes only tough cases. 6. Recall bias: risk f()f retrospective studies. When patients cannot rcrllember, they lnay inad-vertently ovcr-· or underestixllate risk factors. For example, John died of lung cancer, and his angry wife rernembers hilTl as sillOking "like a c.hinlney," whereas Mike died of a nOll"~srnoking-related cause andltis loving wHt, denies that he smoked "mucll." In fact, both men smoked 1 pack per day. 7. Interviewer bias: due to lack of blinding. A scicntoist gets big rnouey to do a study and wants to find a cliflt'fence between cases and controls. Thus, he or she inadvertently labels

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the Satne patient conmlcnt or outcorne as "no significance" in controls and "serious dif

ference" in treated cases. 8. Unacceptahility bias: patients do not admit to ernbarrassing behavior or claim to exercise ruorc than they do to please the interviewer·--or they 111ay dainl to take experimental medications when they spit then} out.

Schizophrenia: 1. The diagnostic criteria provide clllCS: delusions, hallucinations, disorganized speech, grossly disorganized/catatonic behavior, and negative sYInpt(HllS (flat aiTect, refusal to

talk, avolition, apathy). 2. Tilne period iIllportant: < 1 rnonth :.:::: acute psychotic disorder, 1--6 Jllonths :::: schizo" phrenif(JfIll disorder, > 6 1110nths :::: schizophrenia. 3. Positive s)'lnptOlns: delusions, hallucinations, bizarre behavior, thought dis()rder (e.g.,

tangenUality, clanging). These symptoms respond to traclitional anti psychotics dol, chlorprOlnazinc).

(haloperi~

4. Negative symptoms'" flat aflect, alogia (no speec!J), avolition (apathy), an!Jedonia, poor attentioll. These sYlnptoD1S respond poorly to tradltional alllipsychotics but Iuay respond to clozapine or rispcridonc. 5. Good prognosis features: good prcll10rbid functioning (most important); late onset; ob-vious precipitating factors; married; finnily history of mood disorders; positive symptoms; good suppor\ systClYL

6. Poor prognosis features: poor premorhid functioning (IllOst important); early onset; no precipitating factors; single, divorced, or widowed; family history of schizophrenia; negative symptOll1s; poor support systenl. 7. Typical age of onset: ] 5-25 years for men (look for someone going to coHege and dete· riorating); 25-35 years fi)r women. 8. Roughly 1% of people have schizophrenia (in all cultures). 9. In the U.S., most schizophrenic patients are horn in the winter (not known why). 10. Up to 10% of schizophrenics even wally COJ1uuit suidde (past attc.mpt is best predictor of eventual success),

11. Antipsychotic med ications arc the rnainstay of therapy, but psychosocial treatlnent has. bCt'll shown to ilnprove, outCOHlt:. Medications are llsed fJrst, hut the best treatnlC'.JIt (as in most of psychiatry) is lncdications plus therapy. Antipsychotic: medications (see tahle, top of next page)

113

114

Psychiatry

Extrapyramidal side effects: 1. Acute dystonia: first few hours or days of lrealrnent. The patient has muscle spasms or stiffness (e.g., torticollis, triSlTIUs), tongue protrusions and twisting. opisthotonos, and oculogyric crisis (fC)fCed sustained deviation of the head and eyes). Acute dystonia is rnosL COlnmOll in young men. Treat by giving antihistamines (diphenhydramine) or anticholinergics (benztropine, trihexyphenidyl). 2. Akathisia: first few days of treatment. The patient has a subjective ["cling of restlessness. Look for constant pacing, alternate sitting and standing, and inability to sit still. Beta blockers can be tried for treatment. 3. Parkinsonisln: first ft~W lllonths of treatInent. The patient has stiffness, cogwheel rigidity. shufTling gait, lnask-like faCies, and drooling. ParkinsonislD is rnost con11110n in older women. Treat by giving antihistamines (diphenhydramine) or anticholinergics (benztropine, trihexyphenidyl). 4. Tardive dyskinesia: after years of treatment. Most COllu11only, the patient has perioral movements (darting, protruding movements of the tongne, chewing, grimacing, puckering), The patient also rnay have involuntary, choreoathetoid rnovements of head, lirnbs, and trunk. There is no known treatment for tardive dyskinesia. If you have to InaIce a choice when the patient develops tardive dyskinesia, discontinue the antipsychotic and consider switching to clozapine.

5. Neuroleptic malignant syndrOlne: life-threatening condition that can develop at any tilne dur;ng treatmellt. The patient has rigidity, mntism, obtundation, agitation, high fever (up to 107°F), high creatine phosphokinase (often> 5000), sweating, and myogJobhmria. Treatment: first discontinue antipsychotic; then provide supportive care for fever and renal shutdown dne to myoglobinuria; finally, administer dantrolene (jnst as in malignant hyperthermia) . Other antipsychotic medication pearls:

1. Dopamine blockade causes Increases in prolactin (dopamine is a prolactin·-inhibiting factor in the tuberoinfunclibular tract), which may cause galactorrhea, iInpotence, rnen~ strnal dysfunction, and decreased libido. 2. Individual antipsychotic side effects: thioridazine causes retinal pigl11(~nt deposits; clozapinc causes agranulocytosis (white blood cells counts HlUst be rTIollitored); chlorproInazine causes jaundice and photosensitivity. Bipolar disorder: 1. Mania js the only synlptOID reqllired f()r a diagnosis of bipolar disordel~ but a history of

depression is COlnmon.

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2. Look I<)f classic symptoms such as decreased need for sleep, pressured speech. sexual promiscuity, shopping sprees. and exaggerated self-importance or delusions of grandeur. 3. Look for initial onset between 16-30 years old. 4. Lithiurn and valproic add are first-line treatments. Choose lithium if both are options; choose carbamazepine if lithimll fails. If valproic acid is a choice, choose valproic add over carbarnazepine.

5. Autipsychotics may be needed if the patient becomes psychotic; nse at the same time as mood stabihzer. 6. Bipolar II disorder is hypomania (mild mania without psychosis that does not cause occupational dysfnnction) plus major depression. 7. Cyclothymia is at least 2 years of hypomania alternating with depressed mood (no fldl·· blown rnania or depression),

8. Lithium causes renal dysfunction (diabetes inSipidus), thyroid dysfunction, tremor, and central nervous system effects at: toxic levels. Valproic acid causes liver dysfunction. and carbamazepine lllay cause bone rnarrow depression.

Snicide: 1, The major risk factors are age> 45 years, alcohol or substance abuse, history of rage or violence, prior suicide attempts, male sex (men commit suicide 3 times more often than women, but WOlTIen attempt it 4 tiules more often than men), prior psychiatric history, depression, recent loss or separation, loss of health, unemployment or retireluent, and

single, widowed, or divorced status. 2. If you have to choose, the best predictor of furure suicide is a past attempt. 3, Always ask patients about suicide (it does not make them more likely to commit suicide). If you need to do so, hospitalize acutely suicidal patients against their will. 4, When patients come out of a deep depression, they are at increased risk of suicide. The antidepressant may begin to work, and the patient gets more energy-just euough to carry out suicide plans.

5. Suicide rates are rising the fastest in 15--24--year-olds, but the greatest risk is in people over age 65. Depression: 1. Patients may not directly say, "I'm depressed." You have

to watch for clues: chauge in sleep habits (classically, insomnia), vague somatic complaints, anxiety, low energy or fatigue, change iu appetite (classically, decreased appetite), poor concentration, psy· chomotor retardation, and/or anhedonia (loss of pleasure).

2. Patients mayor rnay not have obvious precipitating factors in history, such as loss of loved one, divorce or separation, unemploYlnent or retirement, chronic or debilitating disease. 3. Depression is more COn11ll011 in females.

4. Treat with both antideprt"sants and psychotherapy (combination works better than lnedications alone).

5. Adjustment disorder with depressed mood: when a bad situation ocenrs, the patient does not handle it well and feels "bmnmed out" fe)T < 6 rnonths, but does not meet cri-teTia for full-blown depression. Iior example, the patient gets a divorce, seeins to cry a lot

I"r the next few weeks, and leaves work early on most days.

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6. Dysthymia: depressed lnood on most days f()t Illorc than 2 years, hut no episodes of 111ajor depression, Inania, hyponlania, or psychosis. 7. Antidepressants can trigger mania or hypomania, especially in bipolar patients.

8. Tricyclic antidepressants (TCAs; e.g., nortriptyline, amitriptyline) prevent reuptake of norepinephrine and serotonin. They also block alpha-·adrenergic receptors (watch for orthostatic hypotension, dizziness, and falls) and 111uscarink receptors as well as cause 5\:.> dation and lower the seizure threshold (especialJy bupropion, wbich tedInically is not a tricyclic). TeAs are dangerous in overdose prilnarily because of cardiac arrhytlnnias, which IndY respond to bicarbonate. 9. Selective serotonin reuptake inhibitors (SSRls; e.g., fluoxetine, paroxetine) prevent reo uptake of serotonin only and have less serious side effects (insOlnnia, anorexia, sexual dysfunction) . 10. M:onoamine oxidase inhibitors (MAOIs; e.g., phencJzine, tranylcypromine) are older medications and not flrst--lint'_ agents. They may be good {():r atypical depression (Iook {()[ hypersOlnnia and hyperphagia, the opposite of classic depression). When patients eat tyran1ine-containing {()ods (especially wine and cheese), they Inay get a hypertensive crisis. Do not give MAOI at the Saine thue as SSRIs or Illcperidine; severe reactions Inay occur, possibly death.

11. Trazodone is famous because it can cause priapism (persistent, painful erection without sexual arousal or desire). Normal vs. pathologic grief, mourning, bereavement: 1. Initial grief after a loss (e.g., death of a loved one) may incJnde a state of shock, feeling of nUlubness or bewilderment, distress, crying, sleep disturbances, decreased appetite, difHculty with concentrating. weight loss. and gunt (survivor guilt) for np to I year~-·iIl other words, the saIlle symptoms as depression.

2. It is normal to have an illusion or hallucination about the deceased, but a normal griev"ing person knows that it is an illusion or hallucination, whereas a depressed person believes that the illusion or hallucination is real. 3. Intense yearning (even years after the death) and even searching f"r the deceased are normal. 4. Feelings of worthlessness, psychOlnotor retardation, and suicidal ideation are Ilotnonnal expressions of grief; they are signs of depreSSion. Panic disorder: Look f,)r 2.0-40-year-old patient who thinks that he or she is dying or having a heart attack but is healthy and has a negative work-up far organic disease. Patients often hyperventilate and are extremely anxious. A COillIIlal1 association is agoraphobi.a (f(~ar of leaving the house). Ireat with SSRls (e.g., fluoxetine). Generalized anxiety disorder: patients worry ahout everything (e.g., career, family, future, relationships, money) at the saIne time. SynlptoIllS are not as dramatic as in panic disorder; patients arc just severe worriers. Treat wi.th buspirone (nonaddictive, nonsedating) or hcnzodiazepiues (addictive, sedating). Simple phobias: f,)r example, to needles. blood products, anirnals. or heights. Treat with be· havioral therapy (floodiIlg, system,atic desensitization, hiof(~edback, mental ilnagery- -lnow what these tennsmean). Social phobia: a specific Simple phobia that is best treated with behavioral therapy Beta blockers may be 11S(xl to reduce symptOllls bef()re a public appearance that cannot be avoided.

Psychiatry

Posttraumatic stress disorder: look for

50111cone

117

who has been through a life-threatening

event (Vietnanl vetexan, victhn of severe accident or rape) who recurrently experiences the event in nightnures or flashbacks, tries to avoid thinking about it, and has depression or poor concentration as a result. Treat with group therapy; if yuu have to choose a medication, use imipramine or phenelzine (MAOI).

Homosexuality and homosexual experimentation afC not considered a disease at any age; they arc normal variants. Kinky fantasies or occasional kinky activities (a luau wearing wonlcn's un·" derwear, rnild f()ot fetish) arc 11ofll1aL Somatoform disorders: patients do not hehave inappropriately on purpose. Treat with frequent return chnic visits and/or psychotherapy. 1. Sornati_zation disorder: :multiple different cornptaillts in multiple different organ systerns over many years with extensive work ~ups in the past.

2. Conversion disorder: obvious precipitating factor

(n~~ht

with boyfriend) f()llowed by un·explainable neurologic symptOllls (blindness, swcking--and-glove numbness).

3. Hypochondriasis: patients keep believing that they have the same disease despite extensive negative work"-up.

4. Body dysrnorphic disorder: preoccupation with imagined physical delect (e.g., who thinks that his or her nose is too hig when it is ofnonnal size).

J

teenager

Somatoform disorders YS, factitious disorder YS. malingering: 1. Somatofc)rm disorders: patients do not :intentionally create Sylllpt0111S.

2. Factitious disorders: patients intentionally create their illness or sylnpt0l11S (e.g., injecl themselves with insulin to provoke hypoglycemia) and suhje.ct then1selves to procedures to assmne the role of a patient (no financial or other secondary gain).

3. Malingering: patients intentionally create their iUness for secondary gain (e.g., lTlOney, to get out of work). Dissociative fugue/psychogenic fugue: the patient has anmesia and travels, assuming new identity. Mnltiple personality disorder: most likely

to

he associated with childhood sexual abuse.

Adjnstment disorder: normallik experience (e.g., relationship hreak~up, laihng grade, loss of joh) is not handled well. Patients often are depressed (adjustmcnt disorder with depressed mood) but do not meet tlJe criteria for full~blown depression. For example, a high~schooi girl who breaks np with her boyfr.iend may mope around tbe house, crying and not wanting to attend school or go out with her friends for 1 week, Personality disorders are iildong disorders with no rea! treatment, although psychotherapy may be tried: 1. Paranoid: patients think that everyone is out to get theln (friellds. too) and often start law~suits.

2. Schizoid: the classic loner; no fri.ends ,lnd no interest in having friends. 3. Schizotypal: bizarre beliefs (extrasensory perception, cults, supcrstitio:n, illusions) and [nanner of speaking but no psychosis. 4. Avoidant: patients have IV) friends but want tllcln; they are afrai.d of CritiCi.Slll or tion and avoid others (inf(~riority complex).

rejec-~

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Psychiatry

5. Histrionic: overly dramatic, attention-seeking. and inappropriately seductive; the patient must be the center of attention. 6. Narcissistic: egocentric and lacking enlpathy; patients use others for their own gain or have a sense of entitlement.

7. Antisocial: IlUlst frequently tested personality disorder. Patients have long criIl1inal record (con-Iuen) and torture anirnals or set fires as children (a history of conduct disorder is required for this diaguosis).They are aggressive. do not pay bills or support cbildren, often lie, and have no reillorse or conscience. Strong association with alcoholism or drug abuse and SOlIlatization disorder. Most patients are lnale. 8. Borderline: nnstable mood. behavior. relationships (many bisexual). and self-image. Look for splitting (people are all good or all bad and may frequently change categories). suicide atten1pts, rnicropsychotic episodes (2 lninutes of psychosis), impulsiveness and constant crisis (see Gknn Close in Fatal Attraction). 9. Dependent: patients cannot be (or do anything) alone; a wife stays with an abusive husband; highly dependent on others. 10. Obsessive-compulsive: anal-retentive, stubborn; rules rnore important than objectives; restricted affect. cheap. Obsessive-compulsive disorder: patients have recurrent thonghts or impnlses (obsessious) andlor recurrent behaviors I acts (compulsions) that canse marked dysfunction in occupational andlor interpersonal lives. Look for washing (wasb hands 30 times a day) andlor checking rituals (check to see if door is locked 30 times a day). Onset usually is in adolescence or early adultbood. Treat with SSRls or clomipramine. Behavioral therapy also may be effective (e.g.. flooding). Narcolepsy: daytime sleepine&s; decreased rapid-eye-movement (REM) latency (patients go into REM as soon as they fall asleep); cataplexy (loss of muscle tone. falls); hypnopompic (as patient wakes up) and hypnagogic (as patient falls asleep) hallucinations. Treat with amphetamines. Note: Patients can be hospitalized against tbeir will if they are a danger to themselves (suiCidal or unable to take care of themselves) Of others (homicidal). Many different psychological tests are available to aid in a difficnlt diagnosis; they are not used Ibr a straightfclrward case. There are two types of tests: objective (rnultiple choice, scored by a computer) and snbjective (no right answer. scored by test giver): 1. Stanford-Binet: objective IQ test for adnlts. 2. Wechsler Intelligence Scale for Children: objective IQ test Ibr children (4--17 years old). 3. Rorschach tcst: subjective test in which patients describe what they see in an inkblot

4. Thematic Apperception Test: subjective test in which the patienls describes what is going on in a cartoon drawing of people. 5. Beck Depression Inventory: objective tcst to Screen for depression. 6. Minnesota Multiphasic Personality Inventory: objective test designed to rneasure person-

ality type. 1. Halstead-Reitan Battery: used to determine the location and effects of specific brain lesions. 8. Luria-Nebraska Neuropsychological Battery: assesses a wide range of cognitive functions and tells you the patient's cerebral dominance (left or right).

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CHILD PSYCHIATRY 85% of cases of mental retardation are mild (IQ range: 55-70) and are usually idiopathic. Patients often have a reasonable level of independence with assistance or guidance during peri·ods of stress, Fetal alcohol syndrome is the number-one preventable cause, whereas Down syndrOlue is the number-one overall caUse. Fragile X syndrOlne (in males) is another conunon cause of mental retardation.

Autism: usually starts at a very young age. Look for iInpaired social interaction (isolative, unaware of surroundings), impaired verbal/nonverbal cornumnication (strange words, babbling, repetition), and restricted activities and interests (head banging. strange rnovelnents). Autisrn is usually idiopathic, but look for congenital rubella as a potential cause. See Dustin I-Ioflinan in the lnovie Rain Milll. Learning disorder: impairment in math, reading, writing, speech, language, or coordination, but everything else is nonnal and no mental retardation is present ("Johnny just can't do lnath'} Conduct disorder: pediatric [onu of antisocial disorder. Look for fire setting, cruelty to ani·· mals, lying, stealing, and/or Ilghting. As adults, patients often have antisocial disorder. Note: Conduct disorder is required to Blake a diagnosis of antisocial personality disorder in adults. Attention-deficit hyperactivity disorder (ADHD): as the name implies, affected children are hyperactive and have short attention spans. Males are affected more often than females. Look for a fidgety child who is iInpulsive and cannot pay attention but is not cruel. Treat with stirrmiants (paradoxical calming effect) such as methylphenidate (Ritalin) and dextroampbetamine, both of which lllay cause insomnia, abdominal pain. anorexia, and wd,ght loss or growth suppression. oppositional-defiant disorder: negative, hostile, and deflant behavior toward anthority Ilgures (parents, teachers). The child misbehaves around adults bIll behaves normally around peers and is not a cruel, lying criminal. Separation anxiety disorder: look for a child who refi!ses to go to school. Basically, affected children think that something will happen to them or their parents if they separate; thus, they will do anything to avoid separation (stOIuachache, headache, telnper tantrmlls). Anorexia: look for a female adolescent who is a good athlete and/or student with a perfectionistic personality. Patients have body weight at least 1 S(YO below nonllal, inteuse fear of gaining weight (or "ft'ellat" even though emaciated), and amenorrhea (all three are required for diagnosis). Death occurs in roughly 10-15 % of patients as a result of cou1plications of starvation and/or bulimia (electrolyte imbalances, cardiac arrhythmias, infections). Some patients are hospitalized against their will for IV nutrition. Roughly one-half of anorexics also have bulimia. Bulimia: look for a f(~male adolescent who is of nornlal weight or overweight (unless anorexia coexists). Patients have binge eating episodes during which they leel a lack of control and then engage in purging behavior (vomiting, laxatives, exercise, fasting). Patientsrnay require hospi.. talization for electrolyte disturbances. In the classic patient tooth enan1e! has been eroded because of frequent vorniting; skin lnay be eroded over the knuckles from putting fingers into the throat. Tourette's disorder: only 10-30% of patients utter obscenities. Look f()f rnales with 111otor tics (eye-blinking, grunting, throat-dearing, grimacing, barking, or shoulder shrugging) that are exacerbated by stress and rernit during activity or sleep. of interest, Tourette's disorder can be

120

Psychiatry

caused or unmasked by use of stimulants (e.g., f()r presumed ADHD). Antipsychotics (haloperi· dol) are used if SYll1ptOlns are sevcre.lburettf..'s disorder tends to he a lift>-long problelll. Encopresis/enuresis: not a disorder until afte.r age 4- (encopresis) Of 5 (enures,is). This i,s ohviously an jUlportant diagnostic point to rernember when the lnother cOTnpiains (nonnal finding if the child is 3 years old). RIlle out physical problem (e.g.. Hirschsprung's disease, urinary tract inf('clion), then treat with behavioral therapy ("gold star f()l' being good" charts, alarms, biofeedback). hnipraInine is used only f()[ refractory cases of enuresis; it: is not a Hrst--l:ine agent. Important points: 1. Depression in children often presents as irritable instead of depressed 11100(1. Depression in the elderly may present as pseudodc1l1entia (cognitive decline), which is reversible with treatnlent.

2. The top three causes of adolescent deaths in order arc accidents, hOlukide, and suicide. 'J(Jgether they account fc)r about 75%) of teenage deaths.

DRUGS OF ABUSE Marijuana: nlOst cOllullonly abused illegal drug. Look f(JT a teenager who listens to yock ulusk, has red eyes, and acts "weird."Other synlptOllls include "arnotivational syndrOIne" (chronic use rnay cause laziness and lack of _motivation), time distortion and "munchies" (eating binge when intoxicated). No physical withdrawal syrnpto1Us aYc noted, although patients may have psychological cravings. Overdose is not dangerous, although patients Inay have temporary dysphoria. Marijuana is nota teratogen. Cocaine: look for sympathetic stirmdatioll (insonlnia, tachycardia, mydriaSiS, hypertension, sweating) with hyperalertness and pOSSible paranoia, aggressiveness, ddirimn, psychosiS, or fc)nnications ("cocaine bugs"----,patients think that bugs are crawling on them). Overdose can he fatal (arrhytlllllia, myocardial infarction, seizure, or stroke). On withdrawal, patients hecorne sleepy, hungry (vs. anorexic with intoxication), and irritable, possibly with severe de pression. Withdrawal is not dangerous. hut psychological cravings usually arc severe. Cocaine is teratogenic (vascular disruptions i.n ft,(us).

Amphetamines: classically associated with psychotic symptoms (patients may appear to be fhll-blown schizophrenics), but eff(".cts arc similar to cocaine. Opioids: heroin artd other opioids cause euphoria. analgesia, drowsiness, miosis, constipation, and central nervous system depression. Overdose can be fatal (respiratory depreSSion); treat with naloxone. Be-cause the drug is usually taken intravenously, there are associated lllOrbidities or lllortalities (endocarditis. I-HV, cellulitis. talc damage). Withdrawal is nc)t Ii[E>-threatening, hut patients act as though they are going to die. Symptoms include gooseflesh, diarrhea, in-sonwia, and cramping/pain. Methadone treattnent sOlnetimes is given f()r addicts. Methadone is a longer-acting opioid that allows patients to hU1clion by keeping thern on a chronic, free, low--dose. Its use is controversial.

Lysergic acid diethylamide (LSD) and mushrooms: symptoms of intoxication include haH" dnations. rnydriasis, tachycardia, diaphoresis, arId perception/mood disturbances. HaUucina' tions usually are visual rather than auditory, whereas in schizopllrenia they afC alJditory. Overdose :is not dangerous (unless the patient thinks that he or she CaII fly and jumps out a window). No withdrawal synlptoms are noted. Patients may get "flashbacks"months to years later (brief f(~ding of being on drug again, although nOlle was taken) or a "had trip" (acute

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121

panic reaction or dysphoria), Treat bad trips with reassurance or benz.odiazepine/antipsychotic

medication (if needed).

Phencyclidine (PCP): LSD/mushroom symptoms in intoxication plus confusion, agitation, and aggressive behavior. Also look for vertical and/or horizontal nystagmus, plus possible schizophrenic~like symptoms (paranoia, auditory hallucinations, disorganized behavior and speech). Overdose can be fatal (convulsions, coma, respiratory arrest). Treat with supportive care and urine acidification to hasten eliInination. No withdrawal syrrlptoms are noted.

Inhalants (e,g" gasoline, glue, varnish reIllover): intoxication causes euphoria, diz:z.i.ness, slurred speech, a feeling of floating, ataxia, and/or a sense of heightened power. Intoxication usually is seen in younger teenagers (11-15 years). Can be fatal in overdose (respiratory de~ pression, cardiac arrhythmias, asphyxiation) or cause severe permanent sequelae (centra) nervous system, liver, kidney toxicity, peripheral neuropathy). There is no known withdrawal syndrome. Benzodiazepines/barbiturates: cause sedation and drowsiness as well as reduced anxiety and disinhibition. Overdose may be fatal (respiratory depression). Treat with flumazenil if symp~ toms are due to benzodiazepine. Withdrawal also may be fatal (just as with alcohol) because of seizures and/or cardiovascular collapse. Treat withdrawal on an inpatient basis with a long·. acting benzodiazepine; gradually taper the dose over several days. Benzodiazepines and barbi~ turates are especially dangerous when mixed with alcohol (aU three are central nervous system depressants) .

Note: Caffeine can cause headaches and fatigue in withdrawal.

CHAPTER

1

Gynecolo

Pelvic inflammatory disease (PIl»: look {')f 0 {,'male aged 13~~3 S years with abdominal poi n, adnexal tenderness, and cervical motion tenderness (aU three l1111st be present). PIn also requires one or more of the following: elevated erythrocyte sediJ1H:'ntalioIl rate, leukocytOSiS, fever, purulent cervical discharge, or purulent fluid from culdocC'lltesis. Treat with 1I1Ore than one antibiotic (e.g., ce!()xitin/ceftriaxone and doxycyclille on outpatient basis; c.liuctllnycin and gentamicin on an inpatient hasis) to cover I.1mltiple organisills (e.g., Neisseria f/ollorrhocac, Chlamydia sp., Hw:ll{Tichja coli). With a history ofiutrauterinc device usc, think Actinomycf..'i hmeJii. Imp6rtant points:

1. PIJ) is the nlosl common CJuse ofprcvcIltable infertility (callses scarring of lubes). 2.

W~lt(h

for progression to tuhoovartan abscess (palpable on exanl) and its rupture. Treat with emergent laparotomy with excisjon of afff:'ctcd tube (unilateral disease) or total ahdOlllinal hysterectomy aud hilateral salpingo--oophorectOlny (bi.lateral disease)

3. PID is the nlOst likely cause of inf(~rtjlity in a normally IIlenstruating WOlnan under age 30.

Vaginal Infections 101: :aUG

CuUdiMsp.'

"Cot.tag(~ thei-$e'.~

history ,0£ diaL

ge, pse:udohyphat :011 KOB

pt(~pa:r;l.nO'n;

HitW:\,iPltibiotk ,treatri1NHi__piegllancy

See' hlig's SWlnjTl1Cing-tU1der'mh;rQscope; r,ia;le gre:en, fro'thy, wali,:ty dis\=h<1rge! "str~lwberry" ct'rYix _

Gurdnerdlu"sfi

.Nfalddorous dfsc1i~Tge;, fYsJiy;"snl~1l t~n Kc)~.:p'Nparati(}il;'due' cells

Hmnail.p.apjl1b,mavirus

Vi~neteal, warts,:ktjilp'cytosJs

tlerfi¢s

]vl\lltIpld"shaJlo:'w, :palrifhl'run; ~

inence :a:il(f'resoitltt<;iD ~nd(l

.on: :PaR.smear

Metr:onidai:Qle Many {aqj
prir11al'y'sy.phi1i:s

Pajn!es$

Sc('ondary ·syph:i1i~

CondyloriiaJata; r:na(itik)fiapular J'<.l.sh ortijithns; $t~rnlogy

)?:tnJciHin

Chl(l,l~xdia

Most: Cortlr~on sc'Xtia}ly ttani;nJit"le~l discas~':;:d-Yf;uria, positive cl\'ltnre/ antibody U>')l

:D9x-y£yclin(" (see' }).d{Jw)

Ndsscr1i.i ~lonorrh{)c{lC,

Mucopmult;m ceiyjcHj~i graUl~nt~gative bH~fs .on Gram: stain

Ceftriaxcmc' (see 'b:ek)w)

Mollusell1u

Ch,mH;t(~rj%.tic, appearance

M,n),y

sp.

!)iictoseopy-

of lesions".iiltrat::,dhilar ,hiclusl{}1'w

(nlreit()~·

cryotherapy,

tXJagulatKm) PediciilosiS KOB ';::;:,potassi.til1l hydrox-H:!(\

!)enllcthrin ,cream (6t:Ltndanc)

124

Gynecology

Important points:

t Chlamydia is treated with erythromycin if the patient is pregnant. If compliance is an issne (alcoholic, drng~abusing, bomeless, or unreliable patient), you can give aZ.ithromycin, 1 grn orally all at once, and watch the patient take it. 2. Any patient with gonorrhea is generally treated f(lr presumed chlamydial coinfection (give ceftriaxone and doxycycline). 3. With aU infections but Candida sp., treat the patient's sexual partners and give counseling (e.g., condoms). 4. All of the above inf()fInation is similar f())' men (except candidal i))fection), but any sicms and di'icharges arc on or emne from the penis.

le~

Endometriosis: endometrial glands outside the uterus (ectopic). Patients usually are nulh·, parous and over 30 with the folloWing symptOllls: dysmenorrhea, dyspareunia (painful intercourse). dyschezia (painful defecation), andlor perimenstrual spotting. The rnost cmUUIon site is the ovaries (look for tender adnexae in an aIebrile patient), f(lliowed by the broad or uterosacralligarnent (classic signs are nodularities on physical cxanl and sequela of retroverted uterus), and peritoneal surface. The gold standard of diagnosis is laparoscopy with visualization of endon1Ctriosis.

Important points: 1. Endometriosis is the most likely ,cause of infertility in a menstruating wornan over age of 30 (in the absence of a PID history). 2. Treat first with oral contraceptives (danazol and gonadotropin--releasi_ng hornlOne agonists are seconel-line agents). 3. Surgery and cautery Illay be used to destroy endOllletriOlua and improve fertility markedly. In an older patient, consider total abdominal hysterectomy and bilateral saIpiuga-oophorectomy for severe symptoms. Adenomyosis: endOlllctrial glands within the uterine musculature. Patients usually are over 40 with dysruenorrhea and rueuorrhagia; physical exam reveals large, boggy uterus. Do dilatation and curettage (D&C) to rule out endometrial cancer, and consider total abdorninal hysterectenny to relieve severe SYlllptoms. Gonadotropin-releasing hormone agonists also luay relieve symptoms. Leiomyoma (fibroids): benign tUlllors; Iuost COl1lInOn indication for hysterectomy (when they grow too large or cause symptoms). Malignant transf'mnation is rare « 1%). Look for rapid growth during pregnancy or use of oral contraceptives with regressi.on after menopause (cstrogen~dependent).Fibroid" lllay cause infertility; myomectomy may restore fertility. Other syrnptoms include pain and menorrhagia/rnetrorrhagia. Anemia due to leicHnyoma is an indi-· cation f()T hysterectOlny. D&C rules out endOInetrial cancer and lnalignant tranSf()rnlation in WOlnen> 40. Patients Illay present with polyp protruding through cervix. Note: Any sexually activc WOlnan of rcproductive age with abnormal uterine bleeding should have a pregnancy test first. Dysfunctional uterine bleeding (DUB): defined as abnormal uterine bleeding not associated with tmnor, inflamnlatioIl, or pregnancy. DUB is the most COlnmOIl cause of abnorrnal uterine bleeding and is a diagnosis of exclusion. Over 70(% of cases are associated with anovulatory cycles (unopposed estrogen). The age of the paticnt is irnportant. After fnenarche and just bef()fc menopause, DUB is extremely COIlllllOq and, in fact, physiologic. Most other patients

Gynecology

125

hav~

polycystic ovaries. Always do a D&C to rule ont endom,etrial cancer in women over 35. Also get hemoglobin/henutocrit to make SUfe that the patient is not aneluic from excessive blood loss. Uncommon causes of DUB arc infections, endocrine disorders (thyroid, adrenal, pituitary/prolactin). coagulation defects, and estrogen-producing neoplaslu.

Important points: 1. In the absence of pathology. treat first with NSAIDs (flrst~line agents for DUB and dys~ l1wllorrhea) ,

2. Oral contraceptives are also a first-line agent for Jnenorrhagia and DUB if the patient: does not desire pregnancy and cycles are irregular. 3. Use progesterone only for severe bleeding. Polycystic ovarian syndrome (peaS): look for heavy woman who has hirsutism, amenor~ rhea, and/or inft~rtihty. PCOS is the m.ost likely cause ofinft'.rtility in a WOl:nan, under 30 with abnormal1uenstruation. Multiple ovarian cysts ohen are seen on ultrasound. The primary event is androgen excess. The ratio of luteiniz.ing hormone (I.I-I) to f(lliide-stimulating honnone(FSH) is greater than 2.: 1. Unopposed estrogen increases the risk f(>r endornetrial cancer. Treat with oral contraceptives or cyclic progesterone. If the patient desires pregnancy, use clonliphene. Infertility:

1" In two-thirds of couples infertility is a female problenl; in one-thiTd, it is a male problem. 2. If nothing is apparent after history and physical exam, the first step is semen analysis (cheap, easy, noninvasive). Nonnal SClneIl has the f()llowing properties: IilI

Ejaculate volmne: > 1 lni

II

Sperm concentration: > 20 InilHon/ml

IIluitial f(,rward motility: > 50% of sperm m Normal

morphology: > 60% of sperm

3. The next step is documentation of ovulation. History luay suggest an ovulatory problem (irregular cycle length, duration, or amount of flow, lack of premenstrual symptoms). Basal hody temperature, luteal phase progesterone levels, and/or endOluetrial biopsy can be done to check f<:)f ovulation. II. Tubal/uterine evaluation is doue by a hysterosalpingogram. History may suggest a tubal problem (PID, previous ectopic pregnancy) or a uterine problem (previous D&C may cause intrauterine synechiae, history of fibroids or endoluetriosis SYUlptOlllS). 5. Cervical factor may be a cause of infertility and is suggested by a history of cervicitis. birth traurna, or previous cone biopsy. Evaluate cervical mucus, and do a postcoital test:.

6. l.aparoscopy is a last resort or is done in patients with a history suggestive of endometriosis. Lysis of adhesions and destruction of endOlnetriosis lesions can restore fertility. 7. Medical therapy is usually clomiphene citrate to induce ovulation, but this approach n>quires that the woman is producing adequate estrogen. If the WOIllan is hypoestfogellic, use humanmeuopausal gonadotropin (hMG) , which is' a cOIubinatioll of FSH_ and LB. If these Inethods fail, use in vitro fertilization.

Secondary amenorrhea: in a previously Inenstruating, sexually active WOluan of reproductive agc, the diagnosis is pregnancy until proved otherwise (with a negative tnunan chorionic go-· nadotropil1 assay). Amenorrhea is not UllCOmnlOl1 in hard--training athletes (duc to exercise induced depression of gonadotropin-releasing honuone). Watch for amenorrhea as a presenting

126 syrnptolll for anorexia (arnenorrhea required fC)f a diagnosis of anorexia), especially in a ballet dancer or 111odel. Another common cause is peas (sec above). Secondary arnenorrhea also IndY be due to endocrine disorders (headaches, galactorrhea, and visual fidd dcf(:'cts I1ny indicate a pituitary lUlll0r), anti psychotics (due to increased prolactin), or previous chernotherapy (which causes prernature ovarian fJihlrc/nH:'JlOpause). The £lrst step after a negative pregnancy test and no obvious abnorrnahty in the history or physical exanl is to administer progesterone, which teUs you the patient's estrogen status: mIf the patient has vaginal bleeding within 2. weeks, she has sufHcient estroge:n. Next, check LB. If the level is high, think of peos. If it is low or normal, check the prolactin level to rule out pituitary adenOlua and the thyroid·-stimulating honnone CTSH) level to rule out hypothyroidism (high TSH level causes high prolactililevcl). If prolactin is high with nonna! TS1-1, get an MYU of the brain. If prolactin is :normal, look f<)r drug--, stress-·, or cx('xcise-~indllced depression of gonadotropin--rcleasinghornlollc. Any of these patients may try clcnniphene to become pregnant. m If the patient: does not have vaginal bleeding, she lIas insuffIcient estrogen. Check FSl--l next. If the level is elevated, the patient has prClnature ovarian fajlufe; check !()[ dUlutHl THune disorder, karyotype ahnormalities, and history of cllClnotherapy. If FSH is lc)w or Honnal, the patient IndY have a craniopharyngioma; get: an MRI of the brain.

Primary aluenorrhea: any female who has not lllenstruated by age 16 has pri.Hufy amenor rhea. In the absence of secondary sexual characteristics by age 14- or absence of rnenstruation withi.n 2 years of developing secondary sex characteristics, patients also should be evaluated. Important points:

1. The Hrst step is to rule out pregnancy! (Yes, pregnancy call present as primary amenorrhea.) 2. If the patient is older than 14· and has no sec()udary sexual characteristics, she most likely

has a congenital problern.

3.

a phenotypically norrnal fcrnale (nonnal breast development) wi.th an absence of both axillary and pubic hair, think of androgen insensitivity syndrome. The uterus is absent:. [n

4. In the presence of normal breast clevelopllH'nt and a uterus, the first step is to get a pro--

Iactin level to rule out pituitary adentnna. If prolactin is high, get an MIU. If it is Jlonnal, adUlinister progesterone and f(Jllow the saIne procedures as f()r evaluation of secondary alllenC)rrhea, When in doubt, f()llow these steps in order to evaluate any alTlenorrhea:

1. Do a pregnancy test.

2. Administer progesterone. 3. Furth.er testing depends

Ofl

results of progesterone challenge (bleeding or no bleeding).

Note: SOlne clinicians Inay do a TSH andlor prolactin level befc)l'e a progesterone challenge. For board purposes, choose progesterone challenge over TSH/prolactin levels unless obvious dues point to a'I'SI-I or prolactin problclll (synlptoms of hypothyroidism or pituitary tmllor).

Any sexually active wom(ln of reproductive oW who has amenorrhea should have a preHnancy test evaluation.

(IS

the first step in

Menopause: tlw average age at lnenopause is around 50. Patients have irregular cycles or al101en·orrhea, hot flashes, lIIood swings, and an elevated IjSH level. (Sec pharmacology clJapter.) A bone density test rnay show osteoporosis and help the patieTIl to make a decision about whether to take IHrrmone replacemellt therapy. Patients also Inay cOluplajn of dysuria, dysparetlllia,

Gynecology

127

incontincnce, and/or vaginal itching. burning, or soreness-~symptOlnsthat often arc due to atrophic vaginitis in this age group. l.ook for vaginal mucosa to he thin, dry, and atrophic with increased parabasal cells on cytology. Estrogen, either topical or systeinic, inlproves symptOlTls.

Breast discharge: first get the patient's history of oral contraceptives, hormone therapies, antipsychotic medications, or hypothyroidism symptoms, all of which can cause discharge. When bilateral and non bloody, the discharge is not due to breast cancer; the cause may be a prolactin~ oma (check prolactin) or endocrine disorder. A discharge that is unilateral and hloody and/or associated with a luass should raise concern abo~lt possible breast cancer. Nipple discharge sec~ ondary to Carc1JlO1na should contain hemoglobin. Do a biopsy of any ITlass. Breast mass in a woman under 35: 1, Fibrocystic disease: bilateral, multiple, tender (especially premenstrually) cystic lesions. Most COlIlmon of all breast diseases. Generally, no further work-up is needed-~"jllst routinc follow-up. Progesterone for 1 week at the end of each rl10nth or danazol may help to rc-· lieve syluptorns.

2. FibroadenoJna: painless, discrete, sharply cirnunscribed, rubbery, mobile rn;t55. Most COlnmon benign tUllior of the fenlale breast. Observe the patient f()r one or rnoremenstrual cycles in the absence of sYlnptOllls. Pregnancy or oral contraceptives may stimulate growth; menopause causes regression (estrogen-dependent). Excision is curative but not required. 3. Mastitis/abscess: look for lactating woman with reddish, painfi.I!, fluctuant mass. Culture breast luilk, discontinue breast-feeding, and start on antistaphylococcal antibiotics (e.g., cloxacillin). Staphylococcal infection is hy far the most common cause. If symptoms do not resolve, aSSUlne that the patient has an abscess, which requires incision and drainage. 4. Fat necrosis: history of tramna. Note: Do not do mam1uography in WOlllen under 35 (breast tissue is too dense to give inter--

pretahle films). If suspicious of cancer (exceedingly rare in this age group), proceed directly to hiopsy. Breast mass in a woman 35 or over: 1. Fibrocystic disease: as above, but aspiration of cyst fluid and haseline mammography are recommended. If the cyst fluid is nonhloody and the mass resolves after aspiration, the patient needs only reassurance, follow-up, and a baseline mammogram. If the fluid is bloody or the cyst recurs quickly, do a biopsy to rule out cancer. 2. Fibroadenoma: get haseline mammogram. Observe hriefly if the mass is small (lJ]d seems benign clinically (lnd the WOlllan is premenopausal and has no risk factors for breast cancer. Otllerwise, do a biopsy. Watch out fbr cystosarcoma phylloides that lllasquerades as a fihroadenoma.

3. Fat necrosis: as above. 4. Mastitislabscess: as above.

S. Breast cancer: you rnay not get a classic presentation of nipple retraction and! or peau d'orange in a nulliparous wornan with a strong family history. In a woman 3S or older, you will never be faulted for doing a hiopsy of any mass. In the ahsence of a classic benign presentation -(snch as trannta to the breas.t with fat necrosis or bilaterality with preluenstrual Inastalgia), always consider biopsr Also get a baseline rnanlTnograrn. (See oncology chapter.)

128

Gynecology Important points: 1. If the patient is postmenopausal (or over age 50) and develops a new lesion, you should proceed directly to biopsy. 2. In patients with a clinically evident breast mass, mammography is a poor test to evaluate the mass. although it should be done in a woman over 35 to have a baseline for fiIlure comparison. Mammography is used to deteet nonpalpable breast masses (as a screening tool). not to evaluate masses that are already present. 3. Any suspicious lesion f(mud on mamlllograill should be biopsied, even if it seeIns benign or is inapparent on physical exam. Pelvic relaxation/vaginal prolapse: due to weakening of pelvic supporting ligaments. Look for history of several vaginal deliveries. feeling of heaviness or fullness in the pelvis. baekache, worsening of sympt0111S with standing, and resolution with lying down: 1. Cystocele: bladder bulges into the upper onterior vaginal wall. Symptoms: urinary urgency. frequency. incontinence. 2. Rectocele: reclUm bulges into the lower posterior vaginal wall. Major symptom: diffieulty with defecating. 3. Enterocele: loops of bowel bulge into the upper posterior vaginal wall. 4. Urethroeele: urethra bulges into the lower onterior vaginal wall. Symptoms: urinary urgency, frequency. incontinence. Note: Conservative treatment involves pelvic strengthening exercises and/or a pessary (artificial device to provide support). Surgery is used for refractory or severe cases. Birth control: 1. The best choice is oral contraceptives if the patient is a candidate and does not desire sterilization. Oral contraceptives do not reduce translnission of sexually transmitted diseases. 2. An intrauterine deviee should be used only in older women. preferably those who are monogamous, because it increases the risk of ectopic pregnancy and PID (look for Actiuomyces sp.). 3. Condoll1s are good because they prevent transmission of sexually translnitted diseases.

PEDIATRIC GYNECOLOGY Ambiguous genitalia: look for adreuogenital syndrome and congenital adrenal hyperplasia, which usually are due to 2 I-hydroxylase deficiency (90% of cases). Patients are female; males with this disease show precocious sexual developn1ellLPatients with 21 .. hydroxylase deficiency have salt."wasting (low sodium levels). hyperkalemia. hypotension, and elevated 17-hydroxyprogesterone. Treat with steroids and IV fluids immediately to prevent death. No patient with ambiguous genitalia should be assigned a gender until the work-up is complete. A karyotype must be done. Important points: 1. Any child with a "bunch of grapes" protruding from her vagina probably bas sarcoma botryoides. a lnalignant turnor 2. Prelnature or precocious puberty is usually idiopathic but may be caused by a hormoncsecreting tumor or central nervous systenl disorder, which must: he ruled out. By definition,

Gynecology

129

the patient must be younger than 8 (9 f()f males). Treat nnderlying canse or. if idiopathic, treat with gonadotropin-releasing hormone analog to prevent premature epiphyseal closure and to arrest or reverse puberty until appropriate age.

3. Most cases of vaginitis or vaginal discharge are nonspecific or physiologiC. But look for foreign hody, selmal abuse (especially with sexually transmitted disease). or candida! infecdon (as a presentation of diabetes; lT1CaSure senun glucose and/or check for glycosuria).

4. Imperforate hymen: patient of menarche age with hematocolpos (blood in vagina) that cannot escape (hymen bulges outward). Treatment is snrgical opening of the hymen. 5. Vaginal bleeding in the neonate is usnally physiologic as a result of maternal estrogen withdrawal and resolves by itself.

Important points: 1. The Inns!" common cause of secondary amenorrhea is pregnancy_ AJw(Jys do a pregnancy test fhst when a patient presents with arnenorrhea. Pregnancy also IllllSt be ruled out as a

cause of primary amenorrhea. 2. A 'woman may say that she is taking oral contraceptives and still be pregnant. No contraception is J 00% eHf.~ctive, especially when you factor in poor cOl1lpliant:t'..

3. Signs of pregnancy: Jll1CIlOrrhca, morning sickness. Hegar's sign (softening and C0111pressihiUty of the lower uterine segment), Chadwick's sign (dark discoloration of the

vulva and vaginal walls), linea nigra, chloasma, auscultation of fetal heart tones, visibilHy of gestational sac and/ or f(~tus on ultrasound, uterine contractions, weight gain, and palpaliou/ballottcnlcnt of f(,-tllS. 4. Give all pregnant patients f()]at.c to prevent neural tube defects. Ideally, an women of reproductive age should take f()late, because it is most efft~ctivc in the Hrst trimester when nlOst WOlI1Cll do not know that they are pregnant. Iron is oficn given routinely to prevent anemia. 5. Macrosomia (or positive ]ljstory ill preVious cbi]dren) is caused hy maternal diabetes Ille]]itus 1mtil proved otherwise.

Routiue laboratory tests iu a pl'egnaut patient: 1. Pap SI11eaI': give to every paticnt at £lrst visit; unless she had a normal Pap smear in past 6 1110nths.

2. Urinalysis: at first visit and every visit (screen f<-)r preeclampsia and bacteriuria; not a good screen fC)T diabetes mellitus). 3. Complete blood count: at Hrst visit to sec if tht,_ patient is anemic (pregnancy Illay aggra-vate it). 4. Blood type, Rh type, and antibody screen: at first visit (fC)T idclltiflcation of possible isoimrnunizatiOIl ). 5. Syphi.lis test: at first v.ish (ulJlldated in nlOst states) and subscqupnt visits if the patient is at high risk.

6.

R\lbt~lIa

antibody screen: in 1he abscnce of a good vaccLnation history, obtain at fIrst visit (otherwise not Dceded).

I'll

132

Obstetrics 7. Glucose screen: at first visit if the patient has risk factors fC)f diabetes Iuellitus (obesity. family history, age> 30 years); otherwise, do at 24--28 weeks. Screen with fasting serum glucose
1. At every prenolaJ visit, listen for fetal heart tones and evaluate uterine size for any size/date discrepancy. Uterine size is evaluated by measuring the distance frOIll the sYlnphysis pubis to the top of the fundlls in centitneters. Between roughly 20--35 weeks, the rnea,~ sureIIlent in ern should equal the nmuber of weeks of gestation. A discrepancy greater than 2-·3 Cln is called a size/date discrepancy, and ultrasound should be done to evalu-ate further. Possible explanations include intrauterine growth retardation and rllultiple gestati.0!l. 2. At 12 weeks' gestation, the uterus enters the abdomen; at roughly 20 weeks. it reaches the umhilicus. 3. Between 16 and 20 weeks, ultrasound is most accurate at estimating fetal age (using the biparietal diameter).

Hydatidiform mole: in a sense, the products of conception become a tnulOt. Look for preeclanlpsia before the third trilnester; an HCG that does not return to zero after delivery or abortion or that rapidly rises during pregnancy; flrst- or second-trimester bleeding with possible expulsion of "grapes;" uterine size/date discrepancy; and/or a "SlIow"-starm" pattern on ultrasouud. Complete moles are 46 XX (all cbromosomes from the father) and have no f"tal tissue; incomplete moles are usually 69 XXY and contain fetal tissue. Gross appearance suggests a bunch of grapes. Treat with uterine dilatation and curettage, then follow HCG until it falls to zero. IfHCG does not fall to zero or rises, the patient has either an invasive runle or choriocarciuOIua; in either case, the patient needs chemotherapy (usually methotrexate or actinomycin D). Intranterine growth retardation (IUGR): deflued as size helow the tenth percentile for age. The causes are Juauy and are best understood in broad tenus as caused by one of three factors: maternal (e.g., smoking, alcohol or drugs, lupus erythematosus), fetal (e.g., TORCH infections, congenital anomalies) or placental (e.g,. hypertension, preeclampsia). TORCH infections con·· sist of toxoplasmosis, other (congenital syphilis and viruses), ruhella, cytomegalovirus. and herpes simplex virus. Do ultrasound on aU patients who have a size/date discrepancy greater than 2--3 cm or risk factors for pregnancy prohlems (e.g.. hypertension; diahetes mellitus; renal disease; lupus erytheIl1atosus; cigarette, alcohol, or drug use; history of previous problems). Ultrasound pararnetersmeasured f()I' IUGR determination include biparietal djameter, head circurnference, abdominal circmuference, and femur length. Evaluation oHelal wdl-being: 1. Nonstress test (NilT): with the mother n"ting, fetal heart rate tracing is obtained fby 20 -minutes. A normal strip has at least two accelerations of the heart: rate, each of which is at least 15 bpm above baseline and lasts at least 15 seconds. This is the flrst screening test to evaluate fetal weU--bcing; it is ofl:en done in the context of a biophysical profile.

Obstetrics

133

2. Biophysical profile (BPP): includes lemr measurements: • NST (see above). II Amniotic

fluid index (AFl): measures vertical pockets of amniotie fluid (iu em) iu each of the f'JUr quadrants. The sum oftbe highest vertical pocket in each quadrant is used to determine whether oligohydramnios or polyhydramnios is present (AFl < 5 em '" oligohydramnios,AFI> 25 cm '" polyhydramnios) .

.. Fetal breathing movements: fetlls should have at least 30 breathing movements in 10 lninutes. "Fetal movements: fetus should have at least three body movements (e.g.• flexion. body rotation) in 10 I11inutes. Note: If the fetus scores low on the BPp, the next test is the contraction stress test. With high-risk pregnancies (e.g., IUGR, diabetes mellitus, hypertension, a!cohol or drug use, postlerm pregnancy, history of problcrtl pregnancies. maternal or physician concern), the BPP often is done once or even twice a week until delivery. 3. Contraction stress test (CST): a test for utcroplacental dysfunction. Give oxytocin, and monitor the fetal heart strip. IfIate decelerations are seen on the letal heart strip with each contraction, the test is positive, and usually a cesarean section is done,

Note: In women with antiphospholipid antihodies and previous problem pregnancies, lowdose aspirin may help in subsequent pregnancies. Normally, aspirin and other NSAIDs should be avoided in pregnancy; use acetaluinophen instead. Postterm pregnancy: > 42 weeks' gestation. Generally, if gestational age is known to be accurate,labor is induced (e.g., by oxytoCin) if the cervix is lavorable. If the cervix is not favorable or the dates arc uncertain, do twice-weekly NST and BPP. At 43 weeks. most authorities advise induction of labor or cesarean section. Both prernaturity and postrnaturity increase perinatal rHorbidity and Illortality. Prolonged gestation is common in association with anencephaly and placental sulfatase deficicncy. Normal pregnancy changes: nausea and vOlniting (morning Sickness), amenorrhea. heavy (pOSSibly cven painhll) feeling of the breasts, increased pigmentation of the nipples and areolae (and Montgomery tUbercles), backache, linea nigra, chloasma, striae gravidarum, mild ankle edema, heartburn, and increased frequency of urination. Alpha~fetoproteinlevels: Ilil

Low AFP = Down syndrorne, ft"-tal deluise. or inaccurate dates.

.. High AFP '" neural tube defccts (e.g., anencephaly, spina bifida), ventral wall defects (e.g., ornphalocele. gastroschisis). rllultiple gestation, or inaccurate dates. .. IfAFP or triple screen is positive (at J 6-20 weeks), the patient sbonld undergo amniocente· sis (also done at 16·-20 weeks) le)r a defInitive diagnosis of chromosomal disordcrs (cell cuI· ture) or ueural tube defects (amniotic f1uidAFP). Chorionic villus sampling (CVS): can be done at 9~12 wecks (earlier than amniocentesis) and generally is reserved for WOUlen with previollsly affected oHspring or known genetic dis-, ease. CVS gives WOlUCl1 the advantage of Hr5t""triInester abortion jf a fetus is affected. It is associated with a slightly higher rniscarriage rate than amniocentesis and ((lU!1ot detect neural tube def"cts. Teratogenic agents (see table, top of next page)

134

Obstetrics

-J\lcouol AGENT

DEFECT CAUSllD

,~----

',Fetal'alCohQl syndiorhe

p,eafriess Jnti'Jljti~fijle_-_8i(jwth

.retatdiuiQn,_ central J).(\rv{)lls ,systeii'i 'p!oBlvtris,. deft lip/pal?-t(~

:M.~J;i}£.,

""_'" ",'_,

IJiiigernail-lT0p()'plti1i~_a.:z;rJni{)taG_i,i1

_

4efectfi

111ixiHlteri!1li gnJwtlf-retaxdariQu; ,lQw:'hil'th. ;w~dgJit, ,ptX'ttiattirlty·_

Cetebri\lJn:l1

lnen'trd rctardati611

Cleft lip Clear ¢ell\/agina[.-callcer, -aaeilOsis, cer-vJcaJ inCOll1:pf;ieuce V-0\1$ -sys~eni; crant6t:aciidlcj),t; and/or- c_ardi()va~i.\;:bhit .defects }--~'rio:tl1a.li<~s

,(qral'-(;oiitta~~ijtlyes 'Pne1tyioln FiogesliN'onc Yellov/ or:: biowiLt{l'etn Phdcoinclia

'0raniofihiiaI'iuiBY()c- 2atd~6.viii0ii1af
utcl'lue- giuYfth J.'ftJtd,
t:r.ach¢oesop]J4gea ;,rcllaJ, an

nnatiOl1S,

* Vhaniin P('i:11',getler.aI ii co:nsidt~nidte:r:ati)g~nicwhen-xe¢OHH1).eu,ded iut-ae- .levels ,Yre exceedtid. Important points: 1. Hyperglycemia and diabetes lllelJitus cause cardiovascular anorua]ies, den lip/palate, caudal regression, neural tube defe.cts, left colon hypoplasia, and macrosonlia (early dia-beLes) or microsomia (long-standing diabetes). 2. Radiatjon(> S cGy) causes lUG-H., central nervous system flltnre malignancies (especially leukcmia).

dd{~'cts,

eye lua]fC)rnlatioLlS, and

3. Drugs that arc generally safe in pregnancy: acetaminophen (not NSAIDs or aspirin), penlcilli.Jl, cephalosporins, crythrOTnycin, nitrofurantoin, rr) blockers, antacids, heparin, hydralazine, nteLhyldopa, labetalol, insulin, dOcllsate. 4. l\1ost TORCH intrauterine fetal infl'l~tjons can cause mental retardation, microcephaly, hydrocephalus, hepatosplenomegaly, jaundice, anemia, low birth weight, and/or IUGR:

;; Toxopl(fswa Hondii: look fe)r exposure to cats; spf'cHk dt'feets include intracranial calcifka Lions, chorioretinitis. I'M

Other: varicella zoster (lImb hypoplasia and scarring of the skin) and syphilis (r1linitis, saber shins, Hutchinson's teeth, intnstitial keratitis, skill lcsions).

mRubella: worst in fIrst trimester (some authorities rcc01l1mend abortion if the tllOtllcr

contracts rubella in the first trirnester). Always check antibody status 011 first visit jf the patient has a poor immunization history. Look {()[ cardiovascular dcf{~ct,,;' (patent dlICius arteriosus, ventra.! septal deft:ct), deafiwss, cataracts, and microphthalnlia. Ii!! M

Cytomegalovirus: lllost common; look

f()f

deaflwss, cerebral calcifications, rnicrophtllallllia.

Herpes: look f()l' vesicular skin Iesiol1s (with positiveT'zanck sna'ars), history herpes lesioJls.

ofrnaterl1~tl

Obstetrics

135

Note: With all in utero infections that can cause probleITlS 'with the fetus, the mother may be asymptomatic (subclinical infection) and the infant may even be asymptomatic at birth. only to develop symptoms later (e.g.. learning disability. mental retardation). 5. In untreated HlV-positive patients, translnission to the fetus occurs in roughly 25% of cases. With prenatal zidoV"udinc (AZT) treatlnent f()T the mother and adrninistratioll of AZT to the infant feJr 6 weeks after birth. HIV transmission is reduced to roughly 10%. A noninfected inHmt may still be HIV-positive on testing because rnaternal antibodies can CfOSS the placenta. Within 6 1110nths, the test reverts to negative. HlY-positive ul0thers should not breast--h~ed because milk can transmit vIrns to the infant.

6. When the mother has genital herpes simplex. delay the decision of whether to do a cesarean section until the mother goes into labor. If at the thne of trne labor she has lesions ofHSV; do a cesarean section. lfat the time of true labor the rllother has no HSV lesions, deliver vaginally. 7. If the 1110ther ha...<; hepatitis B, g:iV(~ the infant the flrst hepatitis 1) vaccine shot andhcpatitis B imIllunog}()bulin at birth. S. If the mother gets chickenpox in the last S days of pregnancy or first 2 days afler delivery, give the infant varicella zoster in1D1ullOglobulin,

9. In pregnancy. treat chlamydial infection with erythromycin (not tctracycline).

10. Signs of placental separation: fresh show of blood from vagina; umbilical cord lengthens; the fundus rises and becomes finn and globular.

11. After a cesarean section with classical (vertical) uterine incision, the patient rnust have cesarean sections fbr all future deliveries because of the increased rate of uterine rupture. After a cesarean section with a lower (horizontal) uterine incision, the patient may deliver future pregnancies vaginally. 12. For the fhst several days afler delivery. it is normal to have some discharge (lochia). which is rcd on the first few days and gradually turns to a white or yellowish-whit" color by day 10. If the lochia is foul-smelling. suspect endometritis. Breast-feeding: 1. Ifa wornan does not want to breast~ft.~cd, prescribe tight-fitting bras, ice packs, and anal·· gesia. Brornocriptine and estrogens or oral contraceptives also Inay he used to suppress lactation. 2. If a woman does hreast·-feed, watch for mastitis, which usually develops in the first 2 months of breast-feecling. lIreasts are red. indurated. and painhll; oftcn the patient has a low-grade fever. Staphylococcus oureus almost always is the cause. Treat by stopping breast feeding; obtain 111ilk f()r culture and sensitivity; and begin antibiotic (pcniciUinase--rcsistant penicillin such as cloxacillin) fbr 7~ 10 days while awaiting culture results. Evaluate iIlfJnt for staphylococcal colonization if given the option. If the breast is fluctuant, the condition Inay have progressed into an abscess, in which case incision and drainage are needed. 3.

Breast~feeding

is contraindicated in patients with EIV or hepatitis B and in patients who use the f()llowing: benzodjazepines. barhiturates, opiates, alcohol, caffeine or tobacco (in large amounts), antithyroid medications, hthium, chlorarnphcJlicol, anticancer agents, or ergot and its derivatives (e.g., rnethysergide).

Important points: 1. Epidu.ral anesthesia is the prdt'rrcd Incthod in obstetric patients. General anesthesia involves a higher risk of aspiration and resulting rmeuHlonia, because the gastroesophageal

136

Obstetrics sphincter is relaxed in pregnancy and most patients have not been NPO Spinal anesthesia can interfere with the mother's ability to pnsh and has a higher incidence of hypotension than epidural anesthesia. 2. Treat asymptomatic bacterinria hI pregnaney (20% of patients develop cystitis andlor pyelonephritis if untreated because progesterone decreases the tone of the ureters and the uterus cOlnpresses the ureters. 3. Treat group B streptococcal (GBS) carriers only during labor and delivery. For example, if the patient is GBS-positive at 26-28 weeks, wait nntillabor and give ampicillin. The goal of treatment is to prevent neonatal sepsis and endOlnetritis.

4. If a woman has tuberculosis in pregnancy (positive purified protein derivative [PPD] test and suspicious chest x~ray, plus a positive sputmn culture), treat as you would any other patient. If the patient is a known recent PPD converter or has additional risk factors (such as HIV positivity or household contact with an active case e)f tuberculosis), treat with isoniazid like a nonpregnant patient. Make sure to give the r110ther vitaluin B6 with isoniazid to prevent nutritional defect in her and the fetns, Avoid streptomycin, which may cause deafness and nephrotoxicity in fetus, 5. Marijuana and lysergic acid diethylamide (tSD) have not been confirmed as teratogens.

Preeclampsia: look for hypertension (in patients with preexisting hypertension, blood pressure shonld increase by > 30/tS mmHg over baseline); urinalysis with 2+ or more proteinuria; oliguria; swelling or edema of hands andlor face; headache; visual disturbances; and HELLP syudrome (hemolysis, elevated liver enzymes, low platelets). Pre eclampsia often involves right upper quadrant and epigastric pain and develops in the third trimester, The main risk factors (in order of importance) are chronic renal disease, chronic hypertension, family history, multiple gestatiou, nullipal'ity, age> 40 (although the classic case is a young woman with her first child), diabetes mellitus, and black race. Treatment is delivery if the patient is at term. If tbe patient is premature and has mild disease, treat hypertension with hydralazine or labetalol and bed rest. Observe the patient carefnJJy. If the patient has severe disease (oliguria, 111ental status changes, headache, blurred vision, pulmonary edema, cyanosis, I'lEUP, blood pressure> 1601 ItO mmHg, or progression to eclampsia [seizures]), deliver regardless of gestational age because both mother and infant may die. m Mild

ankle edema is normal in pregnancy, but severe ankle edema or hand edema is likely to be preeclampsia.

m If preeclampsia

symptoms develop before the third trimester, think of hydatiform mole and! or choriocarcinoma.

II!I

Hypertension plus proteinurIa in a pregnant patient is preeclampsia until proved otherwise, pins seizures = eclampsia. Eclampsia can be prevented by regnlar prenatal care. Catch it in preeclamptic stage, and treat appropriately.

m Preeclampsia

m Use

magnesium sulJate fc)r eclamptic seizures (also lowers blood pressure). Toxic effects include hyporeflexla (first sign of toxicity), respiratory depression, central nervous systeIu depression, COlna, and death.

I1iI

Do not rerneasure v(:ry high blood pressure in a pregnant patienL Err all the safe side; assurne that it represents preeclarnpsia and start treaUnenL

m Do

not try to deliver the infant until the ruother is stable (do not do a cesarean section while the fnother is having a seizure).

Obstetrics

I!:l

I\!l

137

Preeclampsia and eclampsia cause llteroplacelltal insufIJdency, IUGR, fetal demise, and in-creased I11aternal rnorbidity and 111ortality. Preeclarnpsia and eclampsia are 1101 risk factors cnd-organ efIt~ClS ofhypertellsioll.

f()[

future d('ve]opuwnt of hypertcTlsion or

Important points: 1. The top causes of maternal mortality ;ue pulmonary clnholislll, pregnancy induced by-pcrtensjon, and hemorrhage (most texts say ill that order). 2. When a postpartum lllotlwr develops shortness of breath, tachypnea, chest pain, hypotcu--

sinn, and/or disserninated intravascular coagulation, think of anlniotic fluid (AI'). pulmonaryemholisl1L

3. Oligohydramnios, AF < ',00 /lIl,AI' index < 5. Callses inclnde IUGR, pH'mature rllp lure of membranes, postmaturity, and renal agenesis (Potter's d-iseasc). Oligohydramnios may cause fetal problems sneh as pulmonary hypopJas_ia, cutaneOllS and skeletal abnormalities dut' to compression, and hypoxia due to cord compressiOlL 4. Jlolyhydranluios

.--:-_~

AF > lOOO mJ, AF index> 25. Causes Lnclude maternal di
litus, nwltiple gestation, neural tube defects (anencephaly, spina binda), GI anol.naLics (oI11pllalocele, esophageal atresia), and hydrops f(~taHs. Polyhydramnios Inay canse post--

partmll uterine atony \-vith resultant postpartum hf~morrhagt' and maternal dyspnea (ovcrdistcndecluterus compromising JHlllnonary function).

Normal Labor STAGE

OJAIlACTElusrlCS

NULLlGRA"lOA

MUtTrGIlA"lOA

fiitst

Om;et -r){': true labor to: frill eervieal d1l
<,20 -np'm:s

111.'oni D"to

B;igh1:y va:riablli

14 h6urs Highly'variable

taten(ph4se

~~

n :(s!l>yv,-:irreglllar)

Acttve _phas:e Second

From ftilh:H1atign

Third

From 4~livf,ry--,of infant -to, dci1iyei:y of-placenta

lJJ:dni~ 3;::'4

011 :_(rapid.;

t()

reztdat)

M'rth ofhlfant

From placent~l delivt:r:y to ltiat:f;fn:;il st,abJlizadon

cfn/hi. di1
',> 1'2 -t~!fi/hr

dilation

5-.3Q--rt).jIJ\1teS O~-30_-ininutes

Up to -+8 hQurs'

Protrattion disol'dt~r: occurs once true lahor has begull if the IllotlJer takes longer than she should, according to lbe above table. Arrest disorder occurs once tru(' labor has begun if no change in dilation (as opposed to the slow change ofprotraction disorder) occurs over 2 llOurs or jfno change occurs·m descent: over] hour. First, rule ont abnormal lie or cephalopelvic dis-proportioll. If everything is okay, treat with labor angmentation (ox}'tocin, prostaglandin gel, allllllotOIny). If this approach docs not work, mallagt' ('xjwetanlly and do a cesarean section at the first sign of trouble. Tht' lnost common cause of faj)uft' to progress (protractioll or arrt'st disorder), also known as dystocia (diHkult birth), is cepba!ope-lvic disproportioll, ddJned as (J-isparity between the size of tht' jllfJ.nt's head and the llIother's pelvis. Labor C'I_ugmclJtation is cOJltraindicated ill this setting. Tn](~ labor:

llonnal contractions occur at least every 3 Jl\iIllllCS, arc f:rirly regular, aud arc asso ciated with cervical changes (dbn"HH:'llL al1d dilation). I
138

Obstetrics

decelerations, and water intoxication (frolll the antidiuretic horrnOlH>like etltx:l of oxytocin). Treat of these symptonls by first: discontinuing oxytocin inhJsion (half-life::: < 10 minutes), Prostaglandin Ii, (dinoprostone) also may be used locally to induce ("ripen") the cervix and is highly em~:ctive :in cOInhination with (often bef:()fc) oxytocin. ProstaglandiIl E] also Inay cause uterine 11ypcTstinmlatic)Jl. Arnniolorny hastens labor but exposes the fc:tus and lite-rine cavity to possible inf(;ction if labor docs not occur.

an

Contraindications to labor induction and augmentation (siInilar to contraindications to vaginal delivery): mPlacenta or vasa previa HI

Umbilical cord prolapse or presentation

1!l1

Prior classic uterine cc'sarean section lncision

~

Transverse ft,tal he

m Active

genital herpes

Il!!

Known cervical cancer

IIll

Known cephalopelvic disproportion

Abortion: defined as termination ofa pregnancy at < 20 weeks (f"tus < SOO gm) ,The fl>Uow ing speciHc terms also imply that the event occurs at < 20 weeks' gestation: 1. Threatened abortion: uterine bleeding without cervical dilation and no expulsion of tissue, Treat with IV fluids (or blood, if needed), bedrest, pelvic rest, and Rhogam iftbe patient is Rh--negativc. Do dilatation and curettage if the fetus dies. 2. Inevitable abortion: uterine bleeding with cervical dilation and crarnpy abd01ninal pain and no tissue expulsion. Treat with IV fluids, Rhogam if the patient is H.h--negative, and eli-latation and curettage. 3. IncOlnplete abortion: passage of S()Ine products of conception through the cervix. Treat with IV flUids, Rhoganl if the patient is lUl,--negative, and dilatation and curettage.

4. CCHllplete abortion: expulsion of all products of conception from the uterus. Treat with serial BeG testing to nuke sure that BeG drops to zero, do dilatation and cur(~ttage, and give Rhogam if the patient is Rh~llegalive~ 5. Missed abortion: (('tal death with no expulsion of tissue (often fc)r several weeks). Treat with dilatation and curettage if less than 14 weeks, attempted delivery if greater than 14weeks. Give Rhogam if the patient is

Rh~negatjve.

6. Induced abortion: intentional ternlination of pregnancy < 20 weeks; may be elective (requested by patient) or therapeutic (to maintain the health of the mother)~

7. Recurrent abortion: two or three successive unplanned abortions. IEstory and physical exam may show: 61

Inf(~ctious

ll!!

Environmental (alcohol, tobacco, drugs)

!in

Diabetes mellitus

00

Hypothyroidism

I!!!l

Systemic lupus erythenlatosllS (especially with l)ositive anti.phosphohpid/lupus anttco agulant antibodies)

!in

C:ervical illcolnpctence (watch {e)r history of patient's mother taking diethylstilbestrol (hIring pregnancy and patient with rCCLlrrcnt sccond·-trimcstcr abortions; treat hIturc pregnancies with a cervical cerclage at 14----[ 6 weeks)

etiology (listeria, Mycoplasma, or Toxoplasma spp., syphilis)

Obstetrics f{~Inalc

tract abnormalities (correct if possible to restore

ill

Congenital

l1lI

Pibroids (rernove (hun)

139

f(~rtiHt:y)

mChroiTlosolllal abnonnaHlies (e.g., rnaternallpatcrnal translocaUons) MeG roughly doubles every two days in the first trimester of pregnaney. An tICG that stays the saIne or increases only slowly with serial testing indicates a ft~tllS in trouble or f(,tal delnisc. A

rapidly increasiJlg 1-1CG or 011(-; that docs ll(Jl decrease after delivery rnayindicatc a llydatjf{)rm 11101e or choriocarciuOlna. The standard lICG hOllle pregnancy test hccornes positive roughly Z weeks after conception. Transvaginal ultrasound: detects intrauterine gestational sac at roughly 5 weeks, fetal buage at 6---7 weeks, and a beating heart at: 8 weeks. Use this infc)rmation in trying to dct:ernline the possihility of an ectopic pregnancy. If the patient's last llwnstrual period (LMP) was 4· weeks ago and the pregnancy test is positive, you cannot rule 011t a uterine IHcgnancy with ultra· SOlIUci. If, however, the patient's IJvlP was 10 weeks ago and an ultrasound of the uteHIS shows no gestational sac, think ofcctopic pregn,ancy. lfBeG is > "lOOO mIll, you sllould be able to vi· suahze a gestational sac with transvaginal ultrasound.

The major risk factor for ectopic pn~gnancy is a history of pelvic inflauunatory disease (10fold increase in ectopic pregnancies). Other risk factors include previous ectopic pregnancy, history of tubal sterilization or tuboplasty, pregnancy thilt occurs with an illtrautcrirle device in place, and diethylstilbestrol exposure (\ivhichmay cause tubal abnormalities in women ex·· posed in ntero).

Classic SyUlptOlTIS of ectopic pregnancy are alnenorrhea, vaginal bleeding, and abdominal paiu. Patients also have p()sitiveHCG test. If you palpate an adnexal In<1ss, pHI may be palpat~ jng an ectopic pregnancy or a corpus Juteum cyst, which Jnay coexist with a tubal pregnancy or a threatened abortion (both lllay have silnilar symptoll1s). When :in doubt and the patient is doing poorly (hypovolelllia, shock, severe abdominal pain, or rebound tenderness), do a la-paroscopy fc)r definitive diagnosis and treatment, if necessary On rare occasions, culdocentesis is done in a stahle patientto check for blood in the pouch of Douglas (with a ruptured ectopic pregnancy), but it: Ius a high false--lIcgative ratc. When culdocentesis is negative, laparoscopy is still required. Therd()rc, do not choose culdocentesis 11nless laparoscopy is not a choice. Tubal pregnancy, if stahle and less than 3 CITI in greatest diameter, can be treated with salpin-. gostomy and rernoval, leaving the tube open to heal on its ovvn. If the paUent is unstable or the ectopic pregnancy has ruptured or is greater than 3 ern, salpingectomy is requirt·_d. Give Rhogarn after treatment f()f Rh--negative patic_l1ts.

Pregnaney and diabetes mellitus: 1. Prohlenls with diabetiC pliGttions of diabetes.

1110thtTS

in pregnancy: polyhydramnios, preeclampsia, and com-

2. ProbJenls in infants bon1 to diabetic 1nolhers: IllacrOSOlllia and HJGH.. ; respiratory distress syndrome; cardiovascular, C01011, craniofacial, and lI('ural tube dd(~cts; caudal regression syndrome (lower half of body incompletely f(>nncd), and postdelivel'Y hypogly<:emia in the ktus (fi'om f(:tal isleH:cIJ hypertrophy due to maternal and thus f(-tal hypcrglycCJnia). Alter birth, the infant is Cl11 off hom the 1not1ler's glucose arId the hypergly-cClnia goes away, but islet ceIls still overproduce insuUn and cause hypoglyccrrlia. 'freal willI IV glucose.

1110

Obstetrics

3. Ireat diabetes mellitus with cliet, exercise, and/ or insulin (no oral hypoglycenrics). Tighter control results in better outcomes for l110thcr and infant. Check HbA 1c to deterrnine compliance and glucose fluctuations. 4. In evaluating anllliotk fluid to detennine ft~tallung maturity, phosphatidylglycerol concentration is much better dtan the lecithin:sphillgonlyclin ratio when the lnother is diabetic.

Fetal heart monitoring: TfHltinely done, but its b(:uefh is cOlltroversial. Fetal heart toues can he heard with Doppler at I 0~~12 weeks and with a stethoscope at 16~20 weeks~ At term the Honna.] heart rate is 12.0---160 bpnl. Any value outside this range is worrisOlne. Know what a basic f(~tal heart strip wi.th uterine contraction patterns looks like, and know the following abnonnalities: 1. Early deceleration: peaks tHatch up (f(~tal heart deceleration nadir and uterine contraction peak). Early deceleration signiHes head cOJllpression (probable vagal response) and is normal. 2. Variable deceleration: variable with relatioI\ to llterinc contractions. The most comIllonly encounten:::-d abnormality, variahle deceleratic)J1 signifles cord cOlnpressio:n. Place the lllothcr ill a lateral decubitus position, adminlster oxygen by face mask, and stop any oxytocin infusion. If bradycardia is severe « 80---90 BPM) or docs not resolve, Ineasure fetal scalp pH. 3. Late deceleration: f(~tal heart deceleration COInes after uterine contraction. Late deceleration signifies uteropiacental insufficiency and is the luost worriSOllle pattern. First, place the mother in a lateral decubitus position, give oxygen by face mask, and stop oxytocin if it is being g:iven. Next, give a tocolytic (heta) agonist such as ritodrine or magnesimn snl-

fate) and IV fhlidsif the mother is hypotensive (especially with epidural anesthesia)~ If late decelerations persist, lueasure fetal scalp pH. 4. Short--term variabi1ity (beat~-to'"beat variability): reflects the interval between successive heart beats. The Honnal value is S----25 bpm. Variability consistently less than S hpn1 is worrisome, especially when cOlnbined with decelerations. Measure f(,tal scalp pH. S. Long-·t(TITl variability: a I--minute strip normally shows changes in the baseline heart ratc. l.ess than 3 cycles per IIlinu_teis worriSOlTI(', especially when cOlnbincd with decelerations. Measure fetal scalp pH. SpeCial walDing: long- term variability is decreased nonnally during letal sleep~

6. Feral tachycardia: > 160 bpIl\~ Poor indicator of f"tal distress \llliess prolonged or marked~ OfieI~_ associated with oxytocin achninistration, Inatcl11aI f(:vel~ or intrauterine inf(~ction. Note: Any ktal scalp pH < continue to observe.

7~2

is an indication f(lI immediate cesarean ddivery.lfpH >

7~2,

When shoulder dystoda occurs, the fIrst ste[Jis MclZobcrt's lllancuver (see figure, top of next page). Ask the lund-ler to flex her thighs sharply against her ahdolllCll.This maneuver IDay free the impacted shoulder. If it does not wcrrk, options arc lilnited. A cesarean section is usually the procedure of choice (after pushing the infant's head back up into the birth canal).

Third-trimester bleeding (very high yield): olwlIYs do all ultrasound bef()re a pelvic exarn~ Always do an ultrasound bdbre a pelvic e:xanl (writtert twice on purpose). The diHl_'rential diagnosis includes the f()Howing: 1. Placenta previa: predisposing {actors include lllultiparity, increasing age, ITlultiple gestation, and prior previa. lItis condition is why you (Jlwoys do an ultrasoundbef:()fC a pelvic exam. Bleeding is painless and Inay he profuse. Ultrasound is 9S---l OW~{) accurate ill diagnosis.

Obstetrics

141

c

B

A, McRobert's maneuver with legs flexed on the maternal abdomen and chest. Angle of inclination of the pelvic tHea is increased when the legs are flexed re) compared to the legs being extended in lithotomy (B);thllS, the shoulder of the infant may become disengaged. (from Ratcliffe SO, Byrd JE, SakornbutEl: Handbook of Preynancy and Perinatal Care in Family Practice. Philadelphia, Hanley & Belfus, 1996, with permission.)

Cesarean section is Iuandatory f()]~ delivery, but you Inay try to adlnit w:ith hed and pelvic rest and tocolysis jf the patient is preterrn and stable and the bleeding stops. 2. Abruptio placentae: predisposing factors include hypertension (with or without pre-eclampsia). traU111a, polyhydramnios with rapid decompression after membrane rupture, cocaineltobacco use, and pretenn prernature rupture of rncrnbranes. Do not fc)rget that the patient can have this condition without visible bleeding (blood contained behind pla,~ ceuta). Patients have pain, uteriJle tenderness, and increased uterine tone with hyperactive contraction pattern. Fetal distress also is preSCJlt. Abruptio placentae 111ay cause disscrninated intravascular coagulation if fetal products enter the maternal circulation. Ultrasound detects only 2';10 of cases. Treat with IV fluids (and blood if needed) and rapid delivery (vaginal preferred).

3. Uterine rupture: predisposing factors include previous uterine surgery, traml1a, oxytocin, grand multiparity (several previous deliveries), excessive uterine distention (e.g., I1mlti-· pie gestation, polyhydramnios), abnormal f<,tallie, cephalopelvic disproportion, and shoulder dystocia. Uterirle rupture is characterized by extreme pain of sudden onset and often associated with rnaternal hypotension or shock. I-etal parts rnay he felt in the ab-dOlnen, or the abdorninal contour rnay change. Treat with iunnediate laparotOlny and usually hysrerectomy after delivery.

4. Fetal bleeding: usually frorn vasa prev:ia or velamentous insertion of the cord. The major risk !actor is multiple gestarion (tbe higher rhe number oflemses, tbe higher the risk). Bleeding is l>ainlcs.s, and tbe IUorher is completely stahle while tbe fetus shows worsening distress (tachycardia initially, then bradycardia as the fetus decompensates).Tbe Apt test is positive on uterine blood and diHerentiates fetal from maternal blood, Treat with irnme-diate cesarean section. 5. Cervical/vaginal lesions: exa1llples include herpes sinlpJex virus, gonorrhea, chlamydial or candidal infection. 6. Cervical/vaginal tramna: usually fronl intercourse.

1. Bleeding disorder: rarely presents befc)re delivery (more

COnllTlOl1

after delivery).

8. Cervical cancer: IIlay occur in pregnant patients too! 9. "Bloody show": with cervical cffaceulCnt, a blond.-tinged 1111lCOUS plug may be released ff{)J11 the cervical canal and heralds the onset of labor. This event is normal and a dia~~nosis of exclusion.

142

Obstetrics

Treatment: in all patients with third-·trinlcster bleeding, start IV fluids and give blood, jf needed. Give oxygen, and get complete hlc)()(l count, coagulation pn)files, and ultrasound. Set up f(:tal and Inaternal J1lOnitoring. Do drug screen jf you arc sllspicious (cocaine causes i)laccn~­ tal ahruption). Give Rhogalll if the IHother is Rll--negativc. The KJeihauer--Betke test quantifIes {(>lal blood in lnatcrnal circulation and is used to calc'ulatt' the dose of Rhoganl. l'reterullabor: defIned as labor between 20----37vveeks. 'Treat with lateral decubitus position, bed and pelvic: rest, oral or IV flUids, and oxygen administration (all rnay stop the contractions). Then give a tocolytic (beta i agonist or 111agneSil1l11 sulfate) if no contrairldicatiolls are present (llean disease, hypertension, chaberes mellitus, h(;nlOrrha~~l', ruptured IIH:.'111hranes, cervix dilated> 4 em). Patients Inay be discharged on oral tocolytic. Do nol tocolyze the Illother if it is dangerous to do so (preeclampsia; severe _henlorrhage, chorioamnionitis, IUGR, fcta] demise, or ft.::tal anomalies incOlllpatible with survival). Often steroids arc given with tocolysis (if the inbnt is 21~-34 weeks old) to hasten f(·tal hmg maturity. Important points: 1. Quickening (wIlen the lllother first detects f(:tal 1110velnents) usually occurs <1tI8---20 weeks in a prirnigravicla and 16----18 weeks in a lnultigravida. 2. Order of labor positions: dest:ent, Hexioll, internal rotation, extension, external rotation, and expulsion 3. IgG is the only maternal antibody that crosses the placenta. An elevated neonatallgM con~ centration is never nonnal, whereas an elevated neonatal IgG often represents rnateflOlal antibodies. Rh ineompatihiHty and hemofytic disease of the newhorn: occur when the motber is Rh negative and the i:nfant is Rh--positive. If both nlothcr and father are Rh--negative, there is noth·ing t(l worry about~,--the illfantwill be Rh-'llf'gative. If the father i's Rh--positive, the infant has a 50/ SO chance of being Rh-·positive. If the potential fbr hemolytic disease exists, check maternal1.Zh antibody titers every IllOTlth, starting in the seventh mOllth. Give RhogalTI automatically at 28 weeks and within 77. hours ahcr delivery as well as after any procedures that1uay cause transplacental hernorrhage (e.g., anuriocentesis). An irnportant point is that development of disease requires previous foicnsitizatioIl. In other words, if a Tlulliparous 1nother has never re cdved blc)od products, her £lrst Rh--positive infant will not be aff(~cted byhemolyOc disease (except in tllt' rare case of sensitization during the fhst pregnancy frolll undetected fctornatt.T-nal bleeding, which usually occurs later in the pregnancy and can be prevented by Rhogam ad ministration at 28 weeks in most instances). The second Rh-·positive infant, however, will be a1T(~cted---~unless you, the astute board taker/physician, administer Rhogarx1 at 1.8 weeks and within 72 hOUTS after delivery during the Grst pregnancy. Any history of blood transfusion, abortion, ectopic pregnancy, stillbirth, or delivery can cause sensitization. If you check nUler-nal R11 antibodies and they are strongly positive, Rhogam is wortbJess, because sensitization has already (>CCUlTccL Rhogarn adnlillistralion is a good example ofprhnary prevention. fii!l

l1i!

If not: detected and prevented, Rh incompatibility leads to f:(~t:al hydrops (ederna, ascites, pleural and pericard:ial dhISioJls). Arnniotic fluid spectrophotolnctry ganges the severity of fc;tal herIlolysis.

rn 'frcatulCllt of _hen101ylic disease; involves delivery if the ft;tus is rnature. Check lung lnatu dty with the lc.'cithin:spllingornyelill ratio. Intrauterine transf1.1sion is risky; phenobarbital helps the fc;talliver to break down bilirubin by inducing cnzym_cs. §l

ABO hlooc! group incompatibility also may CUlse henlOlytic disease c£ the newborn when the mother is type 0 and tIle infant is type A, B, or AB. Previous sensitization is not

Obstetrics

143

required, because IgG antibodies, which can cross the placenta, occur naturally in patients wirh blood type O. Usually the disease is less severe than with Rh incompatibility, bnt treatlnent is the same. Otller minor blood antigens also cause a reaction in fafC cases. SlUUIUaI'Y: Give Rhogarn only when the mother is Rh--negative and the tather's blc)()(:l type is unknown

O[

Rh--positive. l)uring routine prenatal care, check for 1\11 antibodies at the {-lrst vtsH.

If the test is positive, do not give Rhoganl-----you afC too late. Otherwise, give routinely at 2.8 wecks -and il1uuedialely after delivery. Also give Rhogam aner an abc)rtion, stillbirth, ectopic pregnancy, anlniocentesis, chorionic villus sampling, and any other invasive procedure during pregnancy that may cause transplacental bleeding. Note: If fetal lungs arc immature (lccilhin:sphingomyclin ratio < 2: 1 or prostaglandin--negative) and the ft,tus is between 24----34- weeks, corticosteroid acllninistrationmay hasten lung rna-eUTity and thus reduce the risk of respiratory distress syndn)rne. PrClnatnre rupture of menlbranes (PROM): rupture of tIle amniotic sac bd()re the onset of tabor. Diagnosis of Tupture of the luernhranes (whether prcrnature or not) is based on history and sterile speculum exam, which will show (1) pooling of amniotic fluid, (2) {"ruing pattern when the fluid is placed on a Inicroscopic slide and allowed to dry, and/or (3) positive nitrazine test (nitrazine paper turns blue in presence of arnniotic flUid). Ultrasound also should be done to assess anuliotic fluid vohllne (as well as gestational age and any anomalies that may be present). Spontaneous labor often follows mcrnbrane rupwre.lflabor does not occur within 6---8 hours and the patient is at tenn, labor Sllould be induced. If the cervix is highly unfJ.vor"" able, you can wait 24- hours to attenlpt induction. PROM carries an increased risk of iuft'ction, both to the mother (chorioamnioniLis) and infant (neo:natal sepsis, pnelullonia, meningitiS), usually frclln group B streptococci, Escherichia coli, or Iisteria sp.

Preterm premature rupture ofmembraues (PPROM): PROM that occurs befc)re 36-·37 weeks. Risk of infection increases with the duration of ruptured mernbranes. Do a culture and Gram stain of anmiotic fluid. If they are negative, treat with pelVic and bed rest and frequellt follow-up. If positive f()f group B streptococci, treat the lTlOthcr with penicillin, even if she is asympton1at:ic. Chorioamnionitis: presents with ft~ver and tender, irritable uterus (usuallypostparturn but may be antepartum in pa!ierlts with PROM or PPROM) _Do a culture and GraIn stain of alnniotic fluid, and treat with anlpicilJin while awaiting culture results.

Postpartum hemorrhage: defined as estimated blood loss> .500 ml during a vaginal delivery (> 1000 m] dUring cesarean section). The m.ost C(llInnOn cause is uterine atony (75---80% of cases). Hemorrhage also may be cansed by lacerations, retained placental tissue (placenta accreta, increta, or percreta), coagulation disorders (e.g,. disseminated intravascular coagulation, von WiUebrand d:isease), low placental implantation, and uterine inversion. The Jnajor risk factor fiJI retained placental tissue is previous uterine surgery or cesarean section. T'rcaunent: is usually a hysterectolIlY. Uterine atony: caused by overdistentioJl of the uterus (nulltiple gestation, pc)lyhydrarnnios. 1I1JCrOsornia), prolonged labor, oxyt:ocinusage, grandmultiparity (history of S or lllorc delivcries), and precipitous labor « 3 hr). Treat with dilute oxytocin infusion, and nse biInanual compression and massage of the uterus while the infusion is running. If this approach fails, try ergonovine or another ergot drng (contraindicated with maternal hypertension) or prostaglandin 1"70;' If this approach aIsc) fails, do a hysterectomy (ligate the uterine vessels if the patient wants {c'rtility).

144

Obstetrics

~~~~~~~-

With retained products of conception (which is probably the most common cause of delayed postparturn herllorrhage), remove the placenta manually to stop bleeding; then do curettage in the operating TOOTn under anesthesia. If placenta aecreta or pcrcrcta is present (placental tissue grows into/through the InYOlllctriuln), a hysterectomy is usually necessary to stop the bkeding-

Manual removal of placenta. Tho fingers are alter-

nately abducted, adducted, and advanced until the placenta is completely detached. (From Ratcliffe SO, Byrd ,lE, SakornbutEL: Handbook of pregnancy and

, \

Perinatal Care in Family Practice. Philadelphia,

Hanley & Belfus, 1996, with permission.)

With uterine inversion (the uterus inverts and can be seen outside the vagina, usually as a result ofpulJing tOQ hard on the cord), put tbe uterus back ill place nlanually (anesdH'sia may be ne"dl'd) and give ]V fluids and oxytocin_ Postpartum fever: deflned as temperature> I OO.4°F (38°C) {()f at least two consecutive days.

Postpartum f{:ver usually is due to endometritis. Irnportant predisposing factors are PROM/PPROM, prolonged labor, frequent vaginal exams during labor, and rnarmal removal of placenta or retained placental fragrnents (good culture medium). Patients with endometritis have a tender uterus ill addition to fl~v('r. Anaerobes usually are j nvolvcd. Treat with broad--spcctrum penicillin or cephalosporin; add c!ind,llllycin, Juetronidazolc, and geutalnicin if the pa,· tient is doing poorly. Bd()rc antibiotics, dn cultures of cndometrilllll, vagina, blood, and llrinl~. Do not f()fW~t the easy causes of postpartum ft've:r, such as uri nary tract i]Jfl~ctiOll Of atelectasis and pneumonia, especially after cesarean S('CUOLl. I®

lfa post part urn f('.ver docs not resolve with broad--spectrurn antibiotics, there are two main possjbilities: pro,grcssion to pelvic abscess or pelvic thrombophlebitis. Get a CT scan, which show all abscess. If an ahscess is present, it needs to he (lrair~l'd. If no abscess is seen OJ) cr, think of pel vi<: thnnubophlebitis, which presents with persistent spiking kve]"s and Jack ofrespollsP to antibiotics. Give heparin f()r all easy cure (and dia~{nosis in

wm

retrospect) .

Obstetrics

l!!I

145

If a postpartlun patient goes into shock and you see no hlccchng. think of amniotic fluid ernbolisIl1, uterine inversion, or concealed helnorrhage (e.g., uterine rupture with bleeding into the peritoneal cavity)

Normal physiologic changes in pregnancy: 1. l.aboratol·y tests: erythrocyte sediulentation rate is luarkedly elevated (worthless test in pregnancy). Thyroxine and thyfoxillc·-binding globnhuincrease, but free thyroxine is normal. Hernoglobin increases, but plasma vohuneirlcreascs Illorc, so net result: is a de~ creased hCll1Jtocrit and hernoglobin; HUN and creatinine decrease/G}'R increases (high end ()fnonnal range {()[ BlJN/creati.nine indicate renal disease in pregnancy); alkaline

phosphatase increases markedly. Mild proteinuria and glycosuria are NORMAL in pregnancy, electrolytes and L.FTs remain nonnaL

2. Cardiovascular changes: blood pressure decreases slightly, heart rate increases by 10-,20 bpUl, stroke vohune increases, and cardiac output increases (up to 50%). 3. Pulnl0nary ehange.s: rninute ventilation increases because of increased tidal volulne with the saIne or only slightly increased respiratory ratc, Residual volurne and carbon dioxide decrease (physiologic hyperventilation/ respiratory alkalosis). 4. The average weight gain in pregnancy is 1.8 lh (12.5 kg). With a greater weight gain. think of diabetes JllelHtus, With a slnaller weight: gain, think of hyperemesis gravidarml1 or psychological or Hujor systernic disease,

Note: Treat asyrnplO1nalic bacteriuria in pregnancy (because of the high rate of progression to pyelonephritis) with penicillin, cephalosporin, or nitrofurantoin. HypCI'Clnesis gravidarunl: intractable nausea and vomiting leading to dehydration and possi-~ ble electrolyte disturbances. The condition presents in the nrst trjnlcster, usually in younger patientswith their first pregnancy and underlying social stressors or psychological prohlcIllS. Treat with supportive care, including slnall, frequent lTlcals and antieluetics (fairly safl~ :in pregnancy). Outpatient treatillellt s01netilnes is acceptable unless the patient has severe dehydration andlor electrolyte disturbances, in which case aclrnit fbI' treatment.

Cholestasis ofpregnaney: presents with itching, ahnonnalliver flulCtion tests, and lor jaun~­ dice during pregnancy. The only tfeatlnent is (lelivery, but cholestyramine may help with symp' toms. Acute, fatt.y liver of pregnancy is a more seriolls disorder that presents in the third triJnester or after delivery and usually progrC'sses to hepatiC COlna. Treatment includes IV fluids, IV glucose, and fresh frozen plaslua. Vitamin K docs not work, because the liver is in te:mporary failure. Surgical con.ditions: Pregnant WOJllen can have the sarne surgical conditions as nonpregnant women. In general, treat the disease regardless of pregnancy. This rule of thUTUh always works with acute surgical conditions (e.g., appendicitis, cholecystitis). With semiurgent conditions (e.g., ovari.an 1IeopJasm), it is best to wait until the second trimester, when the patient is most stable. Purely elective e:ases arc avoided. Appendicitis Inay present with right upper quadrant pain or tenderness due to displacernent of the appendix by the uterus. Do a laparotoIlly jf you arc lmsure and tIle patient has peritoneal signs. l'etallna]l)n~sentations: although under specific guidelines sorne frank and cornplct:c breeches Inay be dehvcred vaginally, it is acceptable to do a cesarean section f(Jr (Jny breech presentation. Witb shuulder presentation or inCOluplctc/f(wtling breech, cesarean section ismarldatory. For face and brow presentations, watchful waiting is hest, as most convert ttl vertex presenlations; if they do not convert, do a cesarean section.

146

Obstetrics

Multiple gestations: if sex or blood type is different, twins arc dizygotic. If the placentas are rl1onochorion:ic, the twins are rllonozygotic. These three si111ple factors differentiate monozygotic [r0111 dizygotic twins in 80%) of cases. rhe remaining 20(% require HLA--typing studies. Complications of rlluItiple gestations (the higher the llUI11her of fetuses, the higher the risk of tIlost of these conditions) :include the fc)Uowing: 1. Maternal: anernia,hypertcIlsion, premature labor, postparturn uterblC atony, postpartmn

hemorrhage, preeclampsia 2. Fetal: polyhydraxnnios. rnalpresentation, placenta previa, abruptio placentae, velamentolls cord insertion or vasa previa, PROM, prClnaturity, mubilical cord prolapse, IUGR, congell'· i tal anornalies, increased perinatal Tnorbidity and Tllortahty. 3. With vertex-·vertex presentations, you call try vaginal delivery If)r both infants; with any other cornbinatlo!1 of pn:sentations, do a cesarean section.

CHAPTER

2

Acute ahdonlcn: an infhlllcd peritolli':'l11l1 often leads to J. ]aparotOlny because it signifIes a potentially ]if(,-thrcat('!lillg COllditio]l (ilnp<,rtant exceptions to Japarc*>1ny arc pancreatitis, many cases of eli vertiC'ul iUs, a ud spontaneous hacterial peritoniti.s). The best physical C()I)Hrrnat ions of peritonitis are rebound tenderness and involuntary guarding. Vohmtary guarding and ten-dcrness to palpation are softer signs because both are oflen present in benign dis('Jses. Wh(~n yOll are in doubt and the paUcnt is stable, withhold pain medications (do not mask symptOlns before yOll have a dtag-nosis), and do serial abdominal exams. If the patient is unstable or worsening, proceed 10 laparoscopy or laparotOlny.

Localization of acute ahdmncu: mRight upper quadrant: think of gallhladder (cholecystitis, cholangitis) or liver (abscess) III

teft upper q uadranr: think of spleen (rupture with blunt trauma)

mRight lower quadrant: think of appendix (appeudicitis) mI

Left lower quadrant: think of sigmoid colon (diverticulitis)

!!II

Epigastric thillk of stomach (penetrating ulcer) or pancreas (pancreatitls)

AUQ

LUQ

ALQ

LlQ

Topographic anatomy (4-quadrallt construct) of the abdomen. (From James Ee, Corry R.I, Perry JF: Principlos of Basic Surgical Practice. Philadelphin, Hanley & Beltus, 1987, with perrnission.)

147

11111

General Surgery

Gallbladder disease: 1. Cholecystitis: the classic patient is fat, f,)rty, I';ftile, f',male. flatulent aud now fehrile (es.. pecially with gallstones on ultrasound or history of gallstones and/or gallstone-·type symptoms, such as postprandial right upper quadrant colicky pain with bloating and/or nausea and vOlniting). Look f(Jf Murphy's sign, Do a cholecystectomy.

2. Cholangitis: right upper quadrant pain, f(~ver and shaking chills, and jaundice. Patients often have a history of ga]]stones. Start antibiotics, and do a cbolecystectomy 3. Ultrasound Is the best first imaging study for suspected gallhladder disease in the aCllte abdomen. For cholecystitis, a nllclear hepatobiliary/scintigraphy study (e.g., a hopato· Imlnodlacetic acid rHIDAl scan) clinches a dlJncult diagnosis (nonvisualizatiol1 of the gallbladder).

Splenic rupture: history of bhuH abdominal trauma, hypotension/tachycardia, shock, and Kerr's sign. Patients with Epsttin--Barr virus inft~ctjon should not play contact sports. InununiL,c all patients after splenectorny (see section OIl immun.izations). Appendicitis: peaks in 1O~-30-'Yearmolds. 'The classic history is crampy, poorly localized perhmlbHical pain followed by nausea and vOllliting. PaiD then localizes to the right lower quadrant, and patients develop peritonea] signs with worsening of nausea and vomiting. Patients who are hungry and ask for fc)od do not have appendicitis. Rerneluber positive Rovsing's s_ign and fvkBurney point tenderness. Do an appcndcct01llY.

Umbilicus

McBurney's _ point Anterior superior iliac spine

McBurney's point: usual point of maximal tenderness in right lower quadrant. (From James Ee, Corry RJ, (lerry JF: Principles of Basic Surgical Practice. Philadelphia, HanJny & l3e(fus, 1987, with pennission.l

Diverticulitis: left: lower quadrant pain in a patient over SO is diverticulitis unless you have a good reason to think otherwise. Treat nH:~djcaJ]y with avoidance of oral ingestion (NPO) and broad-·spectrurn antibiotics.1fthe disease is recurrent or l'eh'actory to medical therapy, consid(~r sigmoid resection.

General

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149

Pancreatitis: look fel[ epigastric pain in an alcohol abuser or patient with history of gallstones. Pain 1uay radiate to the back, and SCfllIn arnylase Of lipase, if given, is elevated-~--"if they have not been given, order thernl Cormllon syrnptoms indude decreased bowel sounds, local ileus (sentinel loop ofbowe1 on x--ray). and nausea and vomiting with anorexia. Treat with narcotics (meperidine, not rnorphine). NPO, nasogastric Lube, IV fluids, and supportive carc. Watch f()J" cOlnphcations of pseudocyst and pancreatic abscess, which Iuay require surgical intervf'ntioll. Perforated ulcer: patients oft:en have no history of alcohol consumption or gallstones. X-ray classically shows free air under the diaphragln, and patients have a history of peptic ulcer disease. Treat with surgery. Small bowel obstruction: synlptOll1S include bilious vomiting (seen early), abdOIllinal disten-tion, constipation, hypexactive bowel sounds (high--pitched, rushing sounds), and pain that usually is poorly localized. X-ray shows multiple air-,·I:luid levels. Patients often have a history of previous surgery; the l110st connllOll cause of srnall bowel obstructions in adults is adhe-SlOllS, whidl usually develop fJ~OIn prior surgery. In children, think of Meckel's diverticnlmll or incarcerated hernia. Start trcatruent with NPO, nasogastric tube, and IV fluids. If symptorns do not resolve or peritoneal signs occur, laparotOlny is needed to relieve the obstruction.

large bowel obstruction: gradually increasing abdominal pain, abdolllinal distention, constipation, f(~culent vOIniting (secnlate). In older patients the most COllllnon causes are volvulus and diverticulitis, but colon cancer is a possibility. Treat early with NPO and nasogastric tube. A signlOid volvulus often can be decompressed with an endoscope. Other causes or rc-· fractory cases require surgery to relieve the obstruction. In children, watch for Hirschspnmg's disease. FoUl' types ofhernia (all are treated with surglcal repair): 1. Indirect: most common type in both sexes and aU age groups. Hernia sac travels through the inner and outer inguinal rings (protrusion begins lateral to the inf(~rior epigastric vessels) and into the scrotum because of a patent processus vaginahs (congenital defect). 2. Direct: hernia (no sac) protrudes medial to the inft~rior epigastric vessels because of weakness in the abdominal musculature of Hessell:>ach 's triangle. 3. Femoral: ITlOfe cOllunonin WOlnen. Hernia (uo sac) goes through the f(~nlOral ring ontc) rhe anterlor thigh (located hdow the inguin,1! ring), This type is most susceptible to incarceration and strangulation. 4. Indsional: after any wound (especially surgical), a hernia can occur through the site of the incision. Ii!l

Incarceration: when herniated organs become trapped and swollen or edeulatous. Incarcerated hernias are the most common cause of slnall bowel obstruction in a patient who has never had abdominal surgery and the _~econd most common cause in patients wh() have had abd01llinal surgery.

mStrangulation: the cntraplncllt becornes so severe that the blood supply is cut off; necro-sis may occur. Stra:ngulatiOIl is a surgical Cll1ergency; th.epaticnt Inay present w.ith symp·touts of sinaIJ bowel obstruction and shock. hnportant preoperative and po§toperativc points:

1. Bd()rc surgery the patient should avoid oral ingestion {()f at least 8 hOllTS to reduce the risk of aspiration. POI' this reason, general anesthesia is dangerousiJl obstetrics, lwcause patients have not heen NPO when thc:y go into labor.

150

General~""nn,n,

2. Spirometry and a good history are the best preoperative tests Ii),. assessment of pulmonary fUIlction. SpirOIllctry evaluates forced vital capacity (I~VC), forced expiratory volurne in one second (FEY I), FEV! IFVC C%), andmaxhnal voluntary ventilation. 3. Usc cOlnprcssivc or elastic stockillgS. early amhulation, and/ or low--dose heparin to help prevent deep vein thrornbosis andpullnonary crnbohslll, Warfarin ohen is used {(H Of·thopcdic procedures. 4. The most conUllOll cause of postoperative fever in the Hrsl: 24- bouTs is atelectasis. Treat or prevent with early arnbulatioll, chest physiotherapy and percussion, incentive spirometry. and I)[opcr pain control. Both too much pain and too InJIJY narcotics irlcrcase risk of at dectasis. 5. "Water, wind, walk, wo-und, andvveird drugs" h.elps to rccaB causes of postoperative f<'-;ver.Wat:er :::..--= urinary tract infectl(Hl, wind == atelectasis or pneuJll0nia, walk :::::: deep vein thrombo~;is, wound surgical wound inf(:ction, and weird drugs --- drug {(>ver. If daily fever spikes occur, think about an intraabdolninal abscess. Order a CT searl to locate; drainage is required.

6. Fascial or wound dehiscence: o(:curs S~--J 0 days poslopcrotivdy. Look for leakage of sero"sanguinous fluid from the W011rtd, particularly after the patient coughs or strains,vvhich is often associated with infection. Treat with antibiotics (if secondary to inf(X:lion) and reclosure of the incision.

TRAUMA If you spent your free time during your surgery rotation trying to catch up on lost sleep, go back and read a chapter about traulna frOTn a general surgery text. Trauma and its Iuanagement are high yield Ii" Step II. ABCDEs are the key to rnanagen1Cut of patients with traurna. Always do them in order, For ex"alnple, if the patient is bleedjng to death and has a blocked airway, you must cll()oSC which problcrn to address first. The answer is airway Inanagerncnt: A : : :.: Airway: provide, protect, and :maintain an adequate airway at allthnes. If thc. patient cal] answer questions, the airway is flne. You can usc an oropharyngeal airway in UIICoIllpHcated cases and give supplelllcntal oxygen. When you are in doubt or the patient's airway is blocked, intuhate. If intubation fails, do a cricothyroidotonlY; B :--::. Breathing: siuIHar to airway, but even WhCJl the airway is patent, the patient Inay not he breathing spontaneously. The end result is the sarne: when you are in doubt or the patient is nol breathing, intubate. If intubation fails, do a cricothYfoidotolllY.

C Circulation: if the patient seen}s hypovoJernic (tachycardia, hleeding, weak pulse, 1)(11<> ness, dtaplloresis, capillary fenll > '2 seconds), give IV Huicls and I or blood pn)ducts. The initial procedure is to start two large-bore IV catheters aud give a bolus of 10-20 mllkg (roughly I I.) oflactate.d Ringer's solution (IV fluid of dlOicc in traurna). Reassess the patiellt after bolus f()r jrnprovelnenl:. Give another l)olus if needed.

n = Disability: check neurologic function

(Glasgow COUla scale).

E Exposure: strip thepatienJ J1Jd "Ilul: a finger in ever V oriflce" so thaI yen! do not Iniss any occult injnries.

General

SIII-,,";rV

151

Important points: 1. All trauma patients generally get cervical spine, chest, and pelvic x-Tays. 2. Evaluate any head trauma with a noncontrast CT (bener than MIU f(Jf trauma). 3. In blunt abdominal traUllla, initial flncHngs determine the course of action: !'!'I

Il!!

Ii1I

If the patient is awake and stable and }Tour ('xanl is benign, observe and repeat the ab-clcnninal exam later. If the patient is hemodynamically unstable (hypotension and I or shock that de) not re-spond to a fluid chalJenge), proceed directly to laparotomy If the patient has altered Inental status, the abdornen cannot be exarnined, or an obvious sources of blood loss explains thehemodyrl<1I11k instability, eitIH:.'T do a diagnostic peri~ toneallavage (DPL) or get a CT scaIL

4. In penetrating abdoluinal traU111a, the type of injury and initial fIndings dctennine the course of action: j@With any gunshot wound, pf()ceed directly to laparotoIlly. mWith a wound fi'Olll a sltarp instrumcnt,lltanagcrnent is mC)f(:' controversial. Either proceed directly to laparotomy (the better cboice if the patient is unstable) or do a DPL If the DPL is positive, do a laparotOllly; if it is negative, observe and reveat the ahdorninal exanllater. Six thoracic injuries that can he rapidly fatal and that you shOl'tld be able to recognize:

1. Airway obstruction: no audible breath sounds. Patients cannot answer questions even though they arc awake and gurgling. Treat hy intuhation_ Ifintubation lails, do a cricOlhyroidotomy (or a tracheostOJllyin the operating [001"11 if titIle allows). 2. 0Pt'D pneumothorax: open defect in the chest wall that causes poor ventilation and oxy

genation. Treat with intubation, posi.tive--pressure ventilation, and closure of the def(~ct in the chest wall. You can usc gauze. Tape it on three sides only to allow excessive pressure to escape. Otherwise you Iuay convert an open pneurnothorax into a tension pllCllrnothorax. 3. Tension pneunlothorax: usually after blunt trauma. Air forced into pleural space cannot escape and collapses the aHt,etcd lung, then shins the nlcdiastinmll and trachea to the opposite side of the chest. Know what this condition looks like on x-ray. There are no breath sounds on the affected side, and chest percussion produces a hypcrtympanic sound. Hypo-tension and distended neck veins may result: from irnpaired cardiac fHling. Treat with needle thoracentesis, loJ!owed hy insertion of a chest tube. (See lJgure, lOp of next page.) 4. Cardiac tamponad.e: the classic history is penetrating trawna to the left: chest (where the hean is located) _Patients have hypotension (due to impaired cardiac lJlling) , distended neck veins, Iuuffled heart sounds, pulsus paradoxus (exaggerated fall in hlood pressure on inspiration) , and nonnal breath sounds. Treat with pericardiocentesis if the patient is unstable (put a catheter in the pericardial sac, and aspirate the blood or lluid) _If the patient is stable, you can do an echocardiogram to confinn the diagnosis Hrst. 5. Massive hemothorax: loss of rno1'e than 1 L of blood into the thoradc cavity~ Patients have decreased (not absent) breath sounds in tIle an(~cted area, dllIlnote on percussion, hypotension/collapsed neck veins (frOlll blood leaving dIe vascular tTee), and tachycar·, (lia. Placernent of a chest tube causes the blood to COlIle out Give IV fluids and/or blood bef()re you placc the chest tube. If bleeding stops after the initial outflow, get an x,~ray to check fc)r relnaining blood or pathology and treat supportivcly. If bleeding docs not stop, pcrfc)nn an cIncrgeut: thoracolOlny.

152

General

left tension pneumothorax. (From James Ee, Corry RJ, Perry JF: Principles of Basic

Surgical Practice.

Philad{~lphja,

Hanley & Belfus, 1987, with permission.)

6. Flail chest: when several adjacent rib, arc broken in multiple places, the affected parr of the chest walllllay rnove paradoxically during respiration (in during inspiration, out dUf.Lllg expiration). There is almost always an associated puhnonary contusion, which, combined with pain, may nlake respiration inadequate. When yOll are in doubt or the tient is not doing well, intubate and give positive pressure ventilatioll.

pa~

Other injuries: 1. Aortic rupture: the

lUOSl

common cause of illunediatc death after an autOlnobile acci-

dent or fall from a great height. Look for widened mediastinum OIl x··ray and appropriate trauma history. Get an angiogranl if yOll are suspicious. Treat with surgical repair.

2. Diaphragm. rupture: usually occurs on the left because the Hver protects the right side. Look f(Jr howel herniated into the chest. Fix surgically, 3. Neck fl'aUlna: the neck is divided into three zones {or tralllua:

lONE iii lONE Ii Cricoid cart~ago~~ _ _L

ZONE I

Neck zones for trauma. (hom Markovchick V, Pons P: Ernergency Mndicine Secrets, 2nd ed. Philadelphia, Hanley & Belfus, 1999. with permission.)

General Sura,,,v

153

.. ZOlle I: base oflhe neck (hom 7. em above the clavicles to the level oflhe clavicles) !iii

Zone IT: Inidcervical1\.'.gioll (2

C111

above the clavicle to the angle of the TIlandible)

.. Zone III: the angle of the mandible to the base of the skull I!li l1\l

With zone 1 and III injuries, do an arteriograrn beI:()re going to the operating rO()]}L With zone n injuries, proceed to the operating roorn for surgical exploration (do not do an arteriograll1 first).

l5JIn the presence of obvious bleeding or a rapi.dly expanding hematoma, proceed directly to the operating 1'00111, flO matter where the injury is. 4. Choking: leave choking patients alone if they are speaking, coughing, or breathing. If they stop doing all three, do the l:leiInlich Il1aneuver. 5. Tooth avulsion: put the tooth back in place with no clcanirlg (or rinse it only in saline), and stabihze as soon as possihle. The sooner thjs pnJcedure is done, the better the prognosi.s fcw salvage of the tooth.

CHAPTER

21 logy

Conjunctivitis causes conjullctival Vt'sscl hyperemia. The three main causes arc allergic (cOJlnllnn), viral (COlnmon), and bacterial (rare): ETIOLOGY

UNIQUE SIGNS/SYMPTOMS

TRllA'tMENT

Allergi
}fching';:--bilateiill,-spasonal, btig dnratimi

'Vas()copsttictors ifuce-oed

Yiral*

Pream:icul,ilx adDftopathy. highlycqntagtous (hlsl6ty' of tilfectctL cQlltact.s) -; \;:leaf, :",iatery: -disch.arge

SupportiVe, hand-,vashl'ng (prevents- srin:ad)

Bacterial

p\u'ule-m discharge: riRwc common Jtr-nerjnates

~_ _ ~_ _ ~ ~_ _ ~

o

~,

~

~

_

Neonatal conjunctivitis is usually due to one oftbree causes: 1. Chenlical: silver nitrate (or erythromycin) drops are giVCIl prophylactically to all new-horns to prevent gonorrheal COlljUllctivi.tis. The drops JJlay cause a cheInical conjunctivitis (with no purulent discharge) that develops within 12 hours ofinstilliug the drops and rc solves within 4-8 hours (pick this answer ifcoiljUllctivitis occurs ill tile first 24- hours o[Iife).

2. Gonorrheal: look fe)f symptoms of gonorrhea in the mother. The inb.nt has an extremely purulcnt discbarge at 2~-S daysofoHc.Trcatment is topical (c.~., erythromycin oinllIlcnt).lnfimts that are giVt'Il prophyLlCtk drops should not get gonorrheal CO.l1jl1Jlctlvitis. 3. Chla.lnydial (inclusion conjunctivitis): the llwther often reports fi() sylnptollls. The infant has mild-·to--severe conjunctivit.is beginning at S-·-I4- (Joys of (lgc. Patients must be treated

w,ith topical and systemic antibiot.ics (oral erythromycin usually is Llsed) to prevent chlamydiaJ pneumonia (a COrnnlOtl cOlnplication). Prophylactic eycdrops do not effec-~ tively prevent chlalllydial conjunctivitis.

Important points: 1. IfyOll f()rgct everything else, rernCluber the age at which the three diseases preseJlt. 2. Conjunctivitis involves

110

loss of vision (other than transient blurriness due to tcar fJhn

debris that resolves with blinking). [floss of vision is present, think of other, nlOfe serious conditions.

Glaucoma: best thought of as oCllbr hypertension whh its resultant eff(:cts. H-isk factors afC age > 'to, race (black), aml family history TWll types: 1. Open angle: althougb it is traditional to talk ahout painful attacks, they arc rarc. Open--angle glaucoma causes 90 r1(l of cases of glallcoma, 'is JwinJess, and does Hot involve acute attacks.

155

156

Ophthalmology

The only signs are elevated intraocular pressure (usually 20··-30 mmHg). a gradually progressive visual field loss, and optic nerve changes (increased cup-to~disc ratio on fUlldoscopic exaln). Treat with several different types of medications (beta blockers, prostaglandill[latanoprost]. acetazolamide, pilocarpine) or surgery. 2, Closed angle: presents with sudden ocular pain, Ilaloes around lights, red eye, high intraocular pre~sure (> 30 rnrnHg), nausea and vomiting, sudden decreased vision, and a fixed, mid--di1ated pupil. Treat immediately with pilocarpine drops, oral glycerin. and acetazolamide to break the attack. Then usc surgery to prev"nt flmhcr attacks (peripheral iridectomy). In rare cases, anticholinergic medkations IlldY cause an attack of dosed---angle g]au-, ('OUla iII a susceptible, previously untreated patient. Medications do DOt cause glaucoma attacks in open-angle glaucorna or pattents previously treated surgkally for dosecl--angle glaucolna.

Important points: 1. Steroids, whether topical or systemic, call cause glaucoma and cataraets.1bpical steroids can worsen ocular herpes and fungal inft:ctions. For board purposes, do not give topical steroids (especially if the patient has a dendritic corneallllcer stained green by fluorescein). 2. Exposure to ultraviolet light can cause keratitis (corneal inflanlInation) with resultant pain, foreign body sensation, red eye, tearing, and decreased vision. Patients have a history of welding, using a tanning bed or sunlarup, or snow~skiing ("snow-blindness"). Treat with an eye patch (24 hours). topical antibiotic. and possibly with an anticholinergic (cycloplegic agent that reduces pain).

3. Uveitis is COlUlnon in juvenile rhemnatoid arthritis (especially the pauciarticular f()rul). Patients need periodic ophthalmologic exarnination to check fcx uveitis.

4. Cataracts arc the Inost common cause of a painless, slowly progressive loss of vision. Treatrnent is surgicaL Cataracts in a neonate should Blake you think ofTORCH inf{;cliollS or an inherited rnetabolic disorder (c.g., galaCtoserIlja). 5. Know the retinal and fundus changes seen in diabetes (dot- blot hemorrhages, rnicroa~ ncurysms, ueovascularization) and hypertension (arteriolar narrowing, copper/s,ilver wiring, colton wool spots, papHlederna with severe hypertension).

Background diabetic retinopathywitll oxudate, hemorrhages, alld edmna (dot and blot). (From Vander ,JF, Gault JA: Ophthalmology Secrets. Philadelphia, Hanley & Belfus, 1998, with pefrilissioll,)

6. Diabetes is the ullll1ber--one causE' ofbhndness in adults uuder SS. Sf.'nHc Inaenlar degen-eration (look fbr macular drusen) is the nlOst COllunon cause of blindness in adults over age 55. Glauc(nna is the nurnber---ollc cause of hlilldJlCSS in blacks of any age and the IllJJnber~tbree overall cause of blindness.

7. Treannent for prolift'rativc diabetic rettnopathy (with neovasculariz,ltion) is application of a laser bearn to the peripllcry of the whole retina (panrctinal photocoagulation). Focal laser trtannent is COlnnl0}l for nonprolifcraUve_ retinopathy with ulacular ederna; the laser is applied only to the afkcted afea. Be able to ddkfentiate orbital cellulitis hom preorbital ceUulitis (preseptal cellulitis). Both may involve swollen lids, fl--'ver, chemosis, and a history of facial laceration, trauma, insect bite, or sinusitis. Ophthaln-lOplegta, proptosis, severe eye pain, or decfeased visual aCllity indicates orbital cellulitis (a medical E'lllergency). 'I'hemost common bugs in both arc Streptococcus pneumo-niae, Haem0l'hilus inJJucnzae type b, and staphylococci/streptococci with a history of traurna. Cornplicalions of orbhal cellulitis include extension into the skull, vein thro111boscs, and blindness. Treat either condition with blood cultures and adlninistratjon of broad--spectrum antibiotics to C()ver tIle likely hugs until culture results are known. Inpatient IV antibiotics are needed {()f orbital ce.llulitis.

With chemical burns to the eye (acid or alkaline), the key to rnanagen1cnt is copious irriga tion with the closest source of water. The longer you wait, the worse the prognosis. Do not get additional htstory in this justallCC. Alkali burns have a worse prognosis, because they tend to penetrate rnore deeply into the eye. HordeoluITl (stye) is a painful, red lUlTlp near the lid lnargin.l'rcat with wann compress(~s. Chalazion is a painless lump away horn the lid rnargin. Treat witl1warul cornpresses. If the compresses do not work, use incision and drainage f()r both conditions. Herpes simplex keratitis usually startswitb conjunctivitis and a vesicular lid eruption, then prc)gresses to the classic dendritic keratitis (seen with Huorescein--- -know what this looks like). Treat with topical antivirals (c.g., idoxuridine, triJluridine). Corticosteroids afe colltmindicated with dendritic keratitis, because they Iuay make the condition worse. Ophthalolic herpes zoster should be suspected Witll involvenlCl1t: of the lip of thc nose and/ or 1neclial eyelid with a typical zoster dennatolnal I1atterJ1. Treat with oral acyclovir. CompHcations include uveitis, keratitis, and glaucolua. Central retinal artery oedusioll presents with sudden (within a f:l.'W ITI,inllles), painless, uni~ lateral loss of vision. The fundoscopic appearance is classic (know it!). No trcatHlent: is satisfactory. The Inosl: COmnlon cause is ernhoh frOIn carotid plaqllc or the heart, but look Jt.)J' coexisting synlI)[oIns of temporal arteritis (elderly patients with jaw claudication, torUlOUS temporal artery, rnarkedly elevated erythrocyte sedinlentation rate, and co--existing polyrnyalgia rllclunatica syrnptoll1S of proxilTlal nluscle pain a[ld sIHJilcss). If l:cmporal arteritis is SllSpcctcd, start corticosteroids immcdin1cJy before conIirming the diagnos:is with a ternporal artery biopsy. The patient lnay Jose vision ill the mIter eye if you wail to corrHrm the diagnosis. Central retinal vein occlusion also presents with sudden (within a f(~w llours), paiI1less, uniJateralloss of vision. TIle fundoscopic aIJfH:.'arance is classi.c (know it!). No trcatrncllt is satisfactory. The rnost C01111non canses arc hypcrtc_Tlsioll, diabetes rncUitlls, glaucorna, and in crcased hloc)(l visc()sity (e.g., leukenlia). COlnplicatiolls (vision loss, glaucol1la) arc related to ncovascu\arizatic)Jl .

158

Ophthalmology

Retinal detachlnent causes a sudden (instant). painless. uIJilateralloss of vision. History usually includes "floaters" and seeing flashes of light. Often described as a "curtain or veil coming down in front of lny eye." This history should prompt inunediate ref(~rral to an ophthalmolo-. gist. Surgery to reattach the retina rnay save the patient's sight. In elderly patients, both macular degeneration and cataracts cause painless loss of vision, often bilateral, but OIle side may be worse than the other. Know what the red reflex looks like in a patient with a cataract and what the fundus looks like in a patient with macular dcgencra-lion (nucular drusen). Treat cataracts with surgery. M,acular degeneration currently has no treatment".

Drusen are the byproduct of retina! metabolism and manifest as focal yellow..white deposits deep to the retinal pigment epithelium. They serve as a marker of nonexudative age-related macular degeneration. (From Vander JF, Gault JA: Ophthalmology Secrets. Philo" delphia, Hanley & Belfus, 1998, with permission.)

Optic neuritis and papillitis present with a fairly quick (over hours to days), paiuful (unless retfobulbar), unilateral or bilateral loss of vision. If loss of vision bilateral in a 20---40 year"old VI/oman, think of multiple sclerosis; the, same apphes to internuclear ophthalmoplegia. Worry about tumor if the patient is male and has signs of intracran ial hypertension or other 11CllfO'-logic deficits. LyIlJC disease and syphilis are rare causes. Disc margins nlaY appear blurred on fundoscopic exam, just as in papilledema. M

Note: Know your visual pathway lesions and location, just as f()f Step I (e.g., hOIllonYIllolls hOl1ianopia, bitelnporal helnianopia). The most cmnmonly tested exan1ple is bitcrIlporal hemianopia, which usually is due to a pituitary turnor.

Ophthalmologic
Ophthalmology

159

2. Trochlear (CN 4): when the gaze is medial, the patient cannot look down. 3. Ahdueens (CN 6): the patient cannot look laterally with the alfeeted eye. II. CN Sand 7 palsie., also affect the eye beeallSe of corneal drying (Joss of corneal blink reflex). Children with a "lazy eye" or strabisluuS (deviation of the eye, usually inward) that persists beyond 3 1l1fmths need ophthahnologic reft~rral. The condition does not resolve and may cause

blindness (amblyopia) in the afl(,eted eye. For this reason, visual sereeniug must be done in pediatric patients; the visual system is still developing afier birth until the age of 7 or 8. If(llle eye does not sec well or is turned outward, the brain cannot fuse the two different images that it seeS and suppresses the bad eye, which will not develop the proper neural connections. Thus, the eye will never sec well and cannot be corrected with glasses (neural rather than refractive

problem). Presbyopia: between the ages of 40 and SO years, the lens loses its ability to accommodate. Patients need bif()Cals or reading glasses for near vision. Presbyopia is a TlOflllal part of aging.

Important points: 1. PeJvic fracture is the fracture with the highest mortality rate, Patients call hleed to death. If the patient is unstahle, consider heroic measures such as military antishock trousers and external {halor. 2. For any {facture, always do a neurologic and vascular ('xaUl distal to the fracture site to see if there is any neurologic or vascular cOlnpromise. Either may he 111 enlergency. 3. For any fracture, get two x--ray views (usually anteroposterior and lateral) of the site, and consider x--rays of the joint above and below the fracture site,

4. Open fracture (compound fracture): skin is broken over the fracture site. Closed fracture: skin is intact over the fracture sileo 5. For open fractures (lacerated skin), give antibiotics (cefazoIin or cefazolin/gentaJllicin if the laceration is large or cOlltan1inated), do surgical debridemellt, give letal1us vaccine, lavage fresh wounds « 8 hours old), and do an open reduction and internal fixation. The Inain cornplication in open fractures is infection.

Compartnlcnt syndronlc: usually occurs aHer fracture, crush injury, burn, or other trauma or as a reperfusion injury (e.g., after revascuJarization procedure). The lIlost common site is the calf SymptOlns and signs include pain on passive lllOvement (Ollt of proportion to injury), paresthesias, cyanOSiS or pallor, £inn-·feel ing IDusdc cornpartllient, hypesthesia or numbuess (decreased sensation and lWo--point discrimination), paralysis (late, ominous Sign), and cIt'·· vatec! compartment pressure (> 30·~+O mmHg). The diaguosis usually is made clinically with~ out a need to lueasurc pressure. C0111partrneUl syndrome is an emergency, and qUick action can save an otherwise dOCHned limb. Pulses are usually palpable or detectable with Doppler ultrasound, 'l'reatluent is an inunediate fasciotomy; iJlcising the fascial cmupartmcnt relieves the pressure. Untreated, the condition progresses to permanent nerve dalnage and llmsc1c necrosis. The cbs·sic clinical scenarios associated with COlnpartmeut syndrome are supracondylar elbow fracttlrt~S ill children, proxilualhnidshaft tibial fractures, electrical bunts, arterial or venous disruption, and f('vascularization procedures. Open vs. dosed reduction: 1. Reasons to do open reduction fM

Intraarticlilar fractures or articular surface malalignment

1M

Open (compound) fractures

!l'8

NOllunion or ClUed dosed reduction

161

162

Orthopedic Surgery

• COInpromis" of blood supply • Multiple trauma (to allow mobilization at earliest possible pOint) • Extremity function rcquiring perfect reduction (e.g., professional athlete) 2. Closed reduction shoufd be done for all other fractures.

Important points: 1. To test u)r anterior crudate ligaulent (ACL) integrity, do the anterior drawer test. The knee is placed in 90" of flexion and pulled f()rward (like opening a drawer). If the tibia pulls forward, the test is positive, and the patient has an ACL tear.

2. Pain in the anatomic snull~I)()x after trauma (fall on an outstretched hand, especially in young adults) usually is due to a scaphOid bone hacture. 3. After falling on an ontstretched hand, the most likely hacture in older adults is a Colles' fracture (distal end of radius). Lumbar disc herniation: COl1nnon correctable cause of low back pain ll!iI

The most common site is the L5--51 dtsc. Herniation affects the S1 nerve root. Look. f()f decreased ankle jerk, weakness of plantarflexors in f()ot, pain from midgluteal area to the posterior calt: and sciatica with the strajg.ht~leg raise test.

u The second most COInlll0n site is L4-L5. Herniation affects the L5 nerve rool, Look f<:)f de~ creased biceps fculOris reflex, weakness of foot extensors, and pain in the hip or groin.

• Diagnosis is made by CT/MRI or myelogram. Ii!l

Conservative treannent consists of bed rest and analgesics. Surgery (discectOluy) is an option if conservative treaU11C'nt fails.

" Cerv.ical disc disease (classic synlptom :.:;:; neck pain) is less common than lumbar disease. The C6--C7 disc is the lilost COllUIlon site. Herniation affects the C7 nerve root. Look f()r decreased triceps reflex!strength and weakness of fc)rearm extension.

Charcot joints and neuropathic joints are seen luost conunonly'in diabetes 111ellitus and other conditions causing peripheral neuropathy (e.g., tertiary syphilis). Lack of proprioception causes gradual arthritis and arthropathy and joint defonnity. Do x-rays for any (even minor) tramua in neuropathic patients, who may not feel even a severe fracture. The most (mnmon cause of osteomyelitis is Stophylococcus Qureus, hut think of graul--ncgative or-

ganisms in innnullocoinpromised patients and IV drug abusers, and Soimonciia sr. in sickle cell disease. Aspirate the joint and do Granl stain, cultures and sensitivities, blood cultures and cornplele blood cell count with difI(~reIltial if you arc suspicious.

Septic a.rthritis also is 1110St conmlOnly due to S. aureus, but in a sexually active adult (especially if promiscuous), suspect gonococci. Aspirate the joint and do Granl stain, culture and sensitivities, blood cultures and complete blood cell count with differential if ycm are suspicious.

Orthopedic Surgery

163

Important points: 1. With a true posterior knee dislocation, get an Jngiogralll 2. The Illost C01l1mOIl type of bone tumor is Inetastatic (from the breast, lung, or prostate). 3. The most COIlUllon cause of a pathol/)gic fracture is osteoporosis (especially in elderly, thin wOlnell). 4. A hip dislocation, fracture, or inflannnation call refex pain to the knee (classic in children). Pediatric hip problems

Note: All three of the above pediatric hip prohlclllS may present in an adult as arthritis of the

hip. Given the correct history (especially age of onset of symptoms!), you shollid be able to tell which disorder they had. X-rays may be taken, but history gives it away. Osgood-Schlatter disease is osteochondritis of the tibial tubercle. It is often bilateral and llSU ally presents in males 10---1 S years old with pain, swelling, and tenderness in the knee. Pediatric hip problems (see above) give ref(~rred pain to the knee, but the patient has no knee swelling or pain with palpation of the knee. Treat with rest, activity restriction, and NSAIDs. The disease usually resolves (m its own. Scoliosis usually an-ects prepubertalf(~lnales and is idiopathic. Treat with a brace unless the def()fmi ty is severe (with rapidly progressive respiratory compromise); then consider surgery. Ask the patient to touch her toes, and look at the spine. With scoliosis, a lateral curvature is seen.

Wh(~nev('r "tIp

intracranial hemorrhage is suspected, order a CT wilhout contrast. Blood shows as whitt' and may cause a rnidHne sbift.

1. Subdural hematolna: due to bleeding from veins that brklge the cortex and dural si· lluses; crescent-shaped; COITI111011 jn alcoholics and after head tralul1a. Patients may pre·· sent i_mnwdiate1y after trauma or up to 1---2 nlOnths later. If the question includes a history of head tramna, always consider the diagnosis ofa subdural hernatoma. Treat with surgical evacuation.

2. Epidural hematoma: due 10 bleeding hom meningeal arteries (classically, the middle meningeal artery); lenticular-shaped; alI-nost always associated with a skull fracture (classically, fracture of the teluporal hone; see below). More than SO(~;;} of pat.ients ha\le an

ipsilateral "blown" pupil (see below). Tbe classic history is a head trauma with loss of cOllsdousness, f()Jlowed by a lucid int.erval of rninutes to hours, then neurologic deterioration. Treat with surgical evacuation.

3. Subara(:hnoid henlorrhage: due to blood between the arachnoid and pia nuter. The most COl1UllOn cause is tramna, fc)lIowed by ruptured berry aneurysms, Blood is seen in ventricles and around (but not in) tlw hrain/brainstE'Hl. Patients classkaUy present wid] the "worst headache of my lift;," although rnany dit: bef()re they reach the hospital or may be unconscious. Awake patients have signs of meningitis (positive Kernig's and Brudzillski 's Signs). Remember the association between polycystic kidney disease and herry aneurysms.

Although CT is the tcst of choice, a lumbar tap sllOws grossly bloody cerebrospinal fluid. Treat with support, anticol1vuJsants, and observation. Once the patient is stahle, do a cerebral angiogranllMH angiograul to look f<)r aneurysms and arteriovenous 11lalforrna· tion5, which are treated with surgical clipping.

4. Intracerebral hemorrhage~ results frOlTl bleeding into the hrain parenchyma. The most comll1on cause is hypertension; other causes include arteriovenous rna}fbnnations, cnag-llJopathies, tumor, and traluna. Two--thirds Occur ill the basal ganglia. Patients ofl:en pre-sents with corna, jf awake, they have contralateral herniplegia and hcrnisensory deficits. Blood (white) is seen in brain parenchyma alld perhaps in the ventricles. Surgery is reserved [C)l' large bleeds that arc accessihle.

Afier a history of trauma, a dilated, unreactive ("blown") pupil on only oue side most likely represents impingernent of the ipsilateral third cranial nerve and impending uncal herniation due to increased intracranial pressure. Of the difll'rent intracranial hleeds, this is most comulOnly secn wilh epidural heluatolllas. Do not do a lumbar tap on any patiellt with a "blown" pupil; you lIMy precipitate uncal hem iation and death. :[Iirst do a CT/MRl.

165

166

Neurosurgery

Basilar skull fracture has four classic signs: 1. Raccoon eyes: periorbttal ecchyrnosis 2. Battle's sign: postauricular ecchymosis

3. Henlotynlpamun: blood behind the eardnun 4. Cerebrospinal fluid otorrhea/rhinorrhea: clear fluid frOlll tIle cars or nose

Important points: 1. Skull Ifactnres of the ealvariulD are seen on CTscan (prefc:rred) or x-ray, generally as a depressed fracture. Surgical indications are contamination (cleaning and debridc111e11t:). iln-

pingenlcnt on brain parenchyma, Of open fracture with cerebrospinal fluid leak. Otherwise, fractures 111ay be observed and

genf~rally heal

on their own.

2. Head traullla also may cause cerebral contusion or shear injury of the brain parenchyn1a. Neither IUdY show up on a CT scan, hut hoth l11ay cause lcrnporary orpennanent neurologic ddkits.

Increased. intracranial pressure (intracranial hypertension): normal intracranial pressure is 5--] 5 mmHg). An increase is suggested by bilaterally dilated and flxed pupils. Other symptoms include headache, papilledenu, nausea and vOlniting, and :mental status changes. Look also for the classic and important Cushing's triad (increasing blood pressure, bradycardia, respiratory irregularity). The flrst step is to put the patient in reverse Trelldelenburg (head up) and intubate. Once intubated, the pati.ent ShOllld be hyperventilated to rapidly lower the intracranial pressure. This approach decreases intracranial blood volume by causing cerebral vasoconstrie-tion. If the decrease in pressure is not suffIcient, mannitol diuresis can be tried to lessen cerebral edelna. FuroscJnide is also used but less effective. Barbiturate coma and deCOlTlpressive craniol:onlY (burr holes) are Iast--ditch llleastlres. ProphylactiC antlconvulsants are controversial. !II

Cerebral perfusion pressure equals blood pressure lninus intracranial pressure. Tn other words, do not treat hypertension initially in a patient with increased intracranial pressure; hypertension is the body's way of trying to increase cerebral perfusion.

I!!I

Never do a hunbar tap on any patient with signs of increased intracranial pressure until a CT scan is done £lrst. If the CT is negative, you can proceed to a tap,i£' needed.

Spinal cord traulna: often prescrl1s with spinal shock (loss of reflexes, loss of lnotor function, and hypotension). Get standard traulna x-rays (cervical spine, tllorax, pelvis) as well as addi-tional spine x--tays based on physical exam.. Also give steroids (proved to ilnprove outCOlTIC). Surgery is done for incomplete neurologic injury (some residual function is maintained) with external cOlnpression (e.g., subluxation, bone chip).

Spinal cord compression: subacute cOlnpressiol1 (vs. acute compression in traurna) is onen due to m,etastati.c cancer but also may he due to a prhnary neoplasm or subdural or epidural abscess or hernatoma (especially after lUlTlbar tap or epidural! spinal anesthesia in a patient with a bleeding disorder or on anticoagulation). Patients present with local spinal pain (especially with bone rnetastases) and neurologic deHcits below the lesion (hyperrefleXia, po.sitive Babinski's, weakness, sensory loss). The £lrst steps in the emergency department are to give hjgh~dosc corticosteroids and get a CT/MRL Then give rachotherapy to Jlletastases froln a known primary that is radic.lsensitive. Alternatively, surgical decOlnpression Inay be done. !ill

Prognosis is rnost closely related to pretreatment function; the longer you wait to treat, the worse the prognosis.

IIil

For henlatOlTla or subdural/epidural abscess (seen especially in diabetics and usually due to S'taplIy/OCOCCtlS aureus), surgery is indicated fbI' decompression and drainage.

Neurosurgery

167

Syringomyelia: central pathologic cavitation of the spinal cord, usually in the cervical or upper thoraclc region. Syringolllyelia is idiopathic but rnay follow trauma or congenital cranial base maJ!()[]nations (e.g., Arnold~Chiari or Dandy~Walker syndrome). The classic presentation is a bilateral loss of pain and tenlperatllre sensation below the lesion in the distribution of a "cape" due to involvenlcnt of the lateral spinothalamic tracts. The cavitation in the cord gradually widens to involve other tracts, causing IllOtor and sensory deficits. MRI is the bnaging study of choice, and treatll1cnt is surgical (creation of a shunt). Neural tube defects: triangular patch of hair over the hllnbar spine indicates spina bHlda occuIta. More seriOUS deft'cts afe obvious and occur Illost cOJlnnonly in the lurnbosacral region. Meningocele is defined as Illeninges outside the spinal canal; myelo1l1enigocele, as central ner~ VOl1S SystCIll tissue plus Ineninges outside the spinal canal. Most iInportantly, giving folate to potential rYlothers reduces the incidence of neural tube defects. Hydrocephalus: in children, look for incfeasinghead circmnference, illcreased intracranial pressure, bulging f(Hltanellc, scalp vein engorgement, and paralysiS of upward gaze. The most cormnon causes include congenital malfonnations, tumors, and inDanunation (hernorrhage, Ineningitis). Treat the underlying cause, if possible; otherwise, a surgical shunt is created to deC01l1press the ventricles.

HeIrs palsy: Illost commOlt cause of facial paralysis; suddell ullilatf'ra] onsct, usually after an upper respiratory inkction.The cause is unknown but Illay he viral, inllllllllt', or ischemic. .Patiellts Illay have hyperacusis; everytbing souuds loud because the stapedius muscle in tile ear is paralyzed. In severe cases, patients 111ay be unahle to clost' the afhxtcd eye; use drops to protect the eye. Most cases resolve spontancouslr ill abollt 1 rnont11, although SOlIle have pennallcnt sccrue]ae.

Other causes ofunilatcral facial paralysis: I!il

Herpes (Rcullsay Hunt s}flldrollle): e_ighth cranialncrvc is commonly involved also. Look vesicles OIl pinna and inside. ('ar, encephalitis and llw_ningitis rnay be present.

f()}" llil

Lyall' disease: probably the IIlost comrnon calise of bilateral facialneTve palsy.

!1':I-Middlc car and mastoid inf(-;ctions Ii!

Mcningitis

ml

Fracture (tenlporal bone): patients rnay have Battle's sjgn and/or bleeding from the car.

!@]

TurBor: especially in the cerebellopontine angle (acoustic neuroma; consider IlCllrofihroInatosis) or glomus jugulare.

Note: Gel CT/"MRI scans of til(' bead to evaluate if the caus(' -is not apparent or seems sLispiciollS (especially ifaddilionalilcurologk signs arc presult) after history and/or physical exam. Hearing loss: the most common cause is aging (preshycusis); a hearjllg aid call be used, if needed. l-Jistory may suggest other causes: !l:ll

Exposure to prolonged or intense loud noise

ti1.I

Congenital TORe] I inft:ction

~ M(~lliere's

disease: accompanied by severe vertigo, tinnitus, nausea and vorniting; treated with anticholinergic,';, antihistamines (mediziue), or sLlfgery (ifrcfractofY)

!iii

Drngs (JIllilloglycosidcs, aspirin, q-l1lnillc, loop diuretics, cisplatin)

113 TUllIOI' (usually aCOllstic Tlt'lIn)llla)~ rei

Labyrinthitis: lllay be viral or f()lIow/exlt'lld f)'om meningitis or otitis media

l!,l

Diabetes Llldli tlIS

w Hypothyroidism

m:Multlp.le sclerosis liil

Sarcoidosis

113

Pscudottullor cerehri

169

110

Ear, Nose, aod Throat s"'''''''" Sudden deafness: develops over a ft':w hours; 1nOst ohen due to a viral cause (endolyrnphatic labyrinthitis from rHumps, Ilwasles, influenza, chickenpox. adenovirus). Hearing usually f<::>turns withitt 2 weeks, but Joss IlJay be pennanenL No treatment has proved CfftTtive; crnpiric steroids often are used . . Note: Bacterial meningitis is the 111051: COHllllon cause of acquired hearing loss in children. Pollow all children with hearing testing after a bOllt of Il1ellillgitis. Vertigo: may he due to the same eighth cranial nerve lesions that cause hearing loss (Meniere's disease, tumor, inkcti.on, rIlultiple sclerosis). Anoth{~r COlTnllOn cause is benign positionaJ/paroxysn1al vertigo, which is induced by certain head positions and may be accompanied by nystagrnus without associated hearing loss. The condition often resolves spontaneously; treatment is llot necessary.

Note: IJcviated IlasaI surgical correction.

SC'_ptUIH

or odlcr congclJital dc[c:cts fnay cause recurrent sinusitis. Treat ,vith

Causes of rhinitis (edernatous, vasodilatednasal JllU.cosa and turbinates with dear nasal discharge) :

1. Viral (commoII cold): frO-In rIriuo-virus (most cornrnou), influenza, parainfluenza, COXm sackie virus, adenovirus, respiratory syncytial virus, coronavirus, echovirus. Treatment is symptornatic; vasoconstrictors such a phenylephrine are used f()r short--tenn trcatInent hut rnay cause rebound congestion.

2. Allergic (hay f(~ver): associated with seasonal flare- ups, boggy and bluish turbinates, early onset « 2.0 years old), nasal polyps, sneezing, pruritis, conjunctivitis, wheezing, astluna, eczema, positive funny history, eosinophils in nasal ruucns, and elevated 19B. Skin tests Inay identify an allergen. 'Treat: with avoidance when the antigen (e.g., poUen) is known, antihistarnines, crolnolyn, ancl!or steroids fc)r severe syrnptcHns. Desensitization is also an option. 3. Bacterial inkction: frOln Streptococcus A, Pneumococcus, or Staphylococcus spp. Do streptococ-cal throat culture, and treat with antibiotics if appropriate (sore throat, f(;ver, tonsillar

exudate).

The most COlnnlOn cause ofnos(~blec:d in children is nose-picking (trauma), hut watch out f()r local tUIIlor, lcukernia, and other causes of thrOlnbocytopcnia (idiopathic thrombocytopenic purpura, hCIIlOlytic uremic syndrcnne). Nasopharyngeal angiofibroma should he suspected ill adolescent rnales with recurrent nosebleeds and/ or obstructi-nJl but no history of trauma Or blood dyscrasias. Leukemia may resttlt in pancytopenia; look f()l' associated fever and anemia.

1. 75{% are benign in chilclren, and 75%) are malignant in patients> 4-0 years old.

2. Causes: M

Branchial deft cysts: lateral; ofteltbecOl:ne inf(:cted.

~

'I'hyroglossaI duct: cy~t:s: midline; elevate with wngucpTotrusioIl.

llil

Cystic hygrcnna: lyrnphangiOlna; treat with surgical resection.

Ii)l

IN

CCfvi.callylnphadenilis: frOTH streptococcal pharyngitis, Epstcin--Barr vlrus (cmnrnon in adc}lcsccnts and adults in 20s), cat--scratch di.sease, Mycobacterlum sp. (scrofula). NcopIas1l1: rnay be IynlpJladeIJOpathy due toprilnary (lynlpllOlna) or Inctast:atic neo-plasrn, or tbe rnass Inay be the. turnor itself

Ear, Nose, and Throat

3.

Work~up of unknown cancer in the neck includes random biopsy oftlw nasopharynx, palatine tonsils, and base of the tongue as well as laryngoscopy, brolH..:hoscopy, and esophagoscopy (with biopsies of any suspicious lesions)-----the so~-caned triple endoscopy with triple biopsy.

Otitis e.xterna (swinuner's ear): most cOIluTlonly due to PSCUdOInOlWS neIufjinosQ, Manipulation of the auricle produces pain; the skin of the auditory canal is erytheulatous and swollen. Patients Inay have foul--smeUing discharge and conductive hearing loss. Treat widl topical antibiotics (neornycin, polyrnyxin H); steroids rnay reduce swelling. Otitis m,cdia: Illost conmlcmly due to Streptococcus pneumonic/c, Hacmophilus influcnzac, and MoraxeHa C
Recurrent otitis 1l1edia is a common pediatric clinical problern (as well as prolonged SetTe-> tory otitis, a. resnlt ofincom_plete1y resolved otitis) and can cause hearirlg loss with resultant devcloprnental problerus (speech, cognitive functions). 'I'reatrnent consists of prophylactic antibiotics or tyrnpanostouly tubes. Adenoidecto:my is thought to help in some cases by preventing blockage of the eustachian tubes.

m Inf('.ctiousmyringitis (tynlpanic mernbrane i1Jflarnmation) is caused by MycojJhl.~m(l sp., Streptococcus I)l1cumoniac, or viruses. Otoscopy reveals vesicles on the tympaniC lTIClnbranc. Treat. as otitis lnedia (with antibiotics).

Sinusitis: often due to S. lmeumoniac, H. infJuenz(Jc, and other streptococci or staphylococci. Look f()r tenderness over aff(:cted sinus, headache, and purulent nasal discharge (yellow or green). X-"ray shows opacification of the sinus; C1' is used to evaluate chronic sinusitis or suspected extension of infection outside the sinus (su~~gest:ed by high f(~ver and chHls).l'reat with antibi-otics (penicillin/anloxicHlin or erythromycin i(H 1 weeks, up t:o 6 weeks f()f chronic cases). Operative intervention for resistant cases (drainage procedure, sinus obliteration). ReUICJIlber that: the frontal si,nuses arc not well developed until after the age of 10 years. Otosclerosis: the rnost cornUlOn cause of progressive conductive hearing loss in adults (vs. presbyacusis, the most common cause of sensorineural hearing loss in adults). Otic bones becorne Hxed together and ilnpede hearing. Treat: with hearing aid or surgery.

Parotid swelling: the rnost ccnnmon cause is nUIlups. The best tl\:attnenl {()r lTmrnps and the c01nplicalion of inf(~rtihty is prevention tbrough irmnunizatioll. Parotid swelhng also Inay be due to neoplasm (pleornorphic adellorna is the 1nost comrnon), SFJgren's syTldrorne, sialolithiasis (rnore COB1JllOn in the sulJTnandibular gland), and sarcoidosis. Note: After nasal fr;-\Cture (which you should be able to recognize on x--ray), rule hematmna, which Inust be removed to prevent pressurc--induced septal necrosis.

(HIt

a septal

Carotid stenosis: the classic presentation is a transient ischemic attack (TIA), especially drnaurosis fugax, which is characterized by sudden-onset and t.ransient unilateral blindness, smne times described as a "shade pulled over one eye." Patients Illay have a carotid hruie If a bruit is heard or the patient has a 1'IA, ultrasound of the carotid arteries should be done to dctennine the degree of stenosis. §

If stenosis is greater than 70%) and the patient has had aTIA/amaurosis fugax or a small, nondisabli.ng stroke, carotid endarterectomy (CliA) should be perfc)rmed. Patients should not undergo CEA after a stroke that leaves them severely disabled, nor should tbey undergo eEA during a TIA or stroke ill evolution. CEA is an elective, not emergent, procedure.

IiiIl£' stenosis is less than 70%, whether the patient bas syrnptOIns or not, do not do CEA. Treat with daily aspirin instead. iii!

If the patient has greater than 70% stenosis, with or without symptOllls, CEA provides the, best loug-·tenn prognosis. If the patient is asyrl1ptomatic, you have to weigh the risk.-benef] t ratio IliOT(:' carefully.

m Carotid

stenosis is a generalized rnarker for atherosclerosis. Virtually all patients have signiflcant coronary artery disease; peri operative myocardial infarction is tile 1110st COlnmon cause of death in patients llndergoing vascular sllrgery. Make sure to evaluate fjsk factors fc-)r atherosclerosis (cholesterol, hypertension, smoking, diabetes Iudl It ns).

Abdominal aortic anenrysm (AAA): look {C)f a pulsatile abdominal mass that may GUIse ab~ dOlninal pain. If pain is present, sllspect possible rupture ofAAA, although eveIl an unruptured AAA rnay cause SOlIle pain. c1' scan usually is lIsed ft.)]" initial eval uatioJl. If tbe AAA is smaller than S ern, follow it with serial ultrasound to make sure t.hat it is uot enlarging. If the AAA is larger than S em (or YOll are told that it is rapidly enlarg.ing), surgical correction should be donE'. pulsatile abdominal lllass plus hypotcnsion ::~ cmergent laparotomy (means ruptured AAA, which has a mortality fale of roughly 90%).

mA

II@:

A dissecting AAA, if not ruptured, can be treated conservatively witll antihypertensive nICdications, aIt-hough an elective repair should be considered iftbc patient is in good health.

leriche's syndrolne.: claudicat.ion in the buttocks, huttock atrophy and lJllpotl~I1Ce in men; a classic marker f()r aortoiliac occlusive disease'. Patients nsually need an aortoHiac bypass graft. Claudication: pain the the lower extremity (usually) brought on by exercise and relieved by res!. Claudication is an indicator of severe atherosclerotic disease. Associated physical flndings

114

Vascular S'"'IIA,rv include cyanosis (with dependent rubor), atrophic changes (thickened nails, loss of hair, shiny skin), decreased temperature, and decreased (or absent) distal pulses. The best treatment is conservative: cessation of snlOking, exercise, and control cholesterol, diabetes nwlhtus, and hy~ pertension. Beta blockers Inay worsen claudication (due to beta~2 receptor blockade) and

sbould be avoided, If the patient progresses to rest pain in the f(xef()ot, which generally occurs at night and is relievf:d by hanging the [bot over the edge of the bed, or cannot continue lllrrent lifestyle or work obl:igations, consider re-vascularization procedure. lIll

mSevere pain in the foot that: has a sudden onset without previous history, tramna. or any associated chronic physical findings is generally rnore serious, and lIMy represent an elnbo·, Ius (look for atrial Hbrillation) or COTllpartrnent syndrome (collullonly occurs after revascularization procedures). m Claudication

and peripheral vascular disease are generalized markers for atherosclerosis. Check patients fCll' other atherosclerosb risk factors.

Mesenteric ischelnia: the classic patient has a long history of posq)randial abdOlninal pain. which causes a fear of f(wd and thus leads to extensive weight loss. This diagnosis is diHkult hecause, like all atherosclerotic disease. :it presents in patients over 40, who lIlay have other disorders that cause the probleul (e,g., peptic ulcer disease, pancreatic cancer, stomach cancer). Look {()r a history of extensive atherosclerosis (previous rnyocarcHal infaxctions, cerebrovascular accidents, known coronary artery disease, or peripheral vascular disease with several risk factors). possihle abdominal bruit. and a lack of jaundice (which would steer you toward pancreatic cancer). Usually Cl' scan of the abdornen is negative and should make you more suspi-cious of ischemia, Patients should be treated surgically with revaseularization because of the risks of bowel infarction and lnalnutrition. Note: After a penetrating trauma in an extremity (or iatrogenic catheter damage), an arteriove-nous fistula rnay result. Look f()r bruits over the area or a palpahle pulsatile Illass (aneurysIll). Such flstulas can be left alone if they are slnall; otherWise. surgical correction is needed. Venous insufficiency: generally refers to the lower extrcITllties. Patients may have a history of deep vein throrubosis; swelling in the cxtrenlity with pain, bt.igability, and heaviness, which are relievc,d by elevating the extremity; and/or varicose veins. Skin pigl11Cutation rnay increase around the ankles with pOSSible skin breakdown and ulceration. Initial treatnlent is conservative: clastic ccunpression stockings, elevation with minimal standing, and treatment of any ulcers with cleaning. wet-to-dry dressings. and antihiotics (ifcellnlitis is present). Patients with varicose veins. localized leg pain with cord··like induration, reddish discoloration. and luild fever have superfleial thromhophlebitis (not deep vein thrombosis), which rarely leads to pulrnonary clnbolisllL Patients do not need anticoagulation. Treatment is often thro111 bectorny under local anesthesia; lnedical treatlnent (NSAIDs) is used if pain is mild or the patient does Tlot want surgery. Pain generally subsides in it few days OIl its own. Suhdavian steal syndrolne: usually due to left subclavian artery obstruction proximal to the vertebral artery. 'lb get blood to an exercising ann, blood is "stolen" from the vertebrobasilar systeln; it flows backward into the distal subclavian artery instead of f(>rward into the brain~ stem. As a result, the patient develops centrol nervous system ::''YJllJJtoms (syncope. vertigo. confusion, ataxia, dysarthria) and upper extremity claudic(ltioll.'I'reat with surgical bypass. C{~rvical

rib: Inay cOlnprmnise subclavian vessel blood flow. Patients may deveJop upper ex-trenlity paresthesias, weakness. and cold telnperature (arterial cOlnprornisc) or edema/venous distention (venous compromise) without eNS symptoms. Treat with rib n.'scction.

CHAPTER

2

Testicular torsion vs. epididymitis

> 30

, s~v0nell" testi;; ".:(!vcrlyJnge-t,ythmna,_ positive- nti:nalygis, nrethraI4iSCh(l.rge; urethritis, DrostatHis F(!JlJ de(;:t~<1se$ wltlt !estiJ;u!llr elcv«tjo/J

.An-ttbldth;:s* Htrht:a); treat acco:tdingl(ln xiiole {Ji:-si~pj;ofloxildn,

Testicular cancer: usually pres{>I1ts as a painless maSs in a young I11an (age 20---40). The ulain risk factor is cryptorchidism.. Roughly 90<1'0 arc: genn cell tnrnors, the Illost cornrnon being seminoma. Treatment generally consists of orchiectomy and radiation. If disease is widespread, use chenlOtherapy. Alpha-fctoprolt'in is a tumor lnarker f()£ yoJk sac tWllors, whereas hmnan chorionic gonadotropin is a Hl.arker fe)]' choriocarcinorna. Leydig I...J'JI HllBors lHay secrete an drogens and cause precocious puberty.

Note: Remember mmnps as a canse of orchins (painful, swollen testis, usua]Jy unilateral, ill a postpubertalmale).Thc best treatment i_s prophylactic (illlI1Umization). -Murnps alnlOst never causes sterility because it is usuaIly unilate-ral. Benign prostatic hypertrophy (BPH): 1. Symptorns: urinary lwshancy, intermittency, terminal dribbling, decreased size and fc:)rce of stream, seJlsation of incomplete emptying, nocturia, urgency, dysllria, hequency. 2. BPI-I may result ill urinary l'Hcntion_, urinary tract jnft'_ction~;, bydron\:.'_phrosis, and even

kidney dalnage

Of

fail ure

ill

s(;vere cases.

3. Drug therapy is started when the patient" hecomes symptomatic. Options include alphaOtH'. hlockade (pIJ_'l.osin, t\~razosin, doxaz.osi-n) and anttaudrogens (gonadotropin -releasing honllone analogs, flutarllide, flllasterjdc). 4. Transurethral resection of the prostate (TURl') is llsed f()l' more advanced cases, cspe-dally wjth repeated ur1l1ary tract infections, nrinary ret"ention, and hydrouepluosls or kidney damage dne to r(-'flux. Prostatectomy also may be llsed but is a mOTe complicated opcratJ()Jl.

115

176 Note: With acute urinary retention (pain, palpation of full bladder on abdominal exam, history of BPH, no urination in past 24- hOUIS), the first step is to empty the bladder. If you cannot pass a regular Foley catheter, do a suprapubic tap. Then address the underlying cause. hnpotence: Dlost cOllllnonly caused l)y vascular problcrns. Medications are also a COlTunon culprit (especially antihypertensives and antidepressants). Diabetes mellitus Inay he a vascular (increased atherosclerosis) err neurogenic cause of irnpotence. Rcrnclnber point and shoot: parasynlpathetics 11lcdiatc erection, sYll1pathetics mediate ejaculation. Patients undergOing dialy·sis also are COllHllonlyirn.potenL Theh~story oiterl gives you a due jf the cause of irnpotf'J1Ce is psychogenic. Look f()[ a Honnal pattern of nocturnal erections, selective dysfunction (a patient who has norrnal erections when masturbating but not with his wif:t~), and stress, anxiety, or ft'ar. In patients with trauma, look for signs of urelhralinjury (high riding, ballottable prostate, blood at the urethral rneatus, severe pelvic fracture, eccllYlnosis) hefore trying to pass a Foley catheter. If allY of 1:11es(' signs are present, do not try to pass a Foley catheter u:ntil you have gotten a retrograde urethTograrrl to rule out urethral injury. Urethral injury is a cOJltraindica-tion to a Foley catheter. Hydrocele vs. varicocele:hydroccle represents a rClllnarlt of the processus vaginaHs (rcmeTnber embryology?) and transiIIUl11inates. It generally causes no synlptOll1s and does not require treatll1cnL A varicocele is a dilatation of the palnpin]f()fITl venous plexus ("bag of worms," usu-· any on the len), does not transilluminate, disappears in the supine position, and may be a cause of male jnf(~rtility or pain (in which case it is surgically treated). Nephrolithiasis: 1. Signs and symptonls include severe flank pain, which often radiates to the groin and is colicky in nature;helnatnria; and stone on abdon1inal x--ray (90% of stones radiopaque). 2. 7 S%) of stones are calcium (look f()r hypercalcelnia and hyperparatllynJicUSln; sInaU bowel bypass also increases oxalate absorption and thus calcimll stone f(>nnation), I 5 (Yo are struvitc/magnesimYl--alnrnonimn--phosphate stones (think of infection), 7°1t) are uric acid stones (look for history of gout or leukernia), and 2(% are CYStillC storIes (think of cystinuria) . 3. Treat stones with lots of hydmtion, narcotics {()f pain, and observation. Most stones pass by theJnseJves, If not, do lithotripsy, ul:e:r()Scopy with stone retrieval, or open surgery (if needed). Cryptorchidism: arrest of descent of the testicle(s) smnewhere between the renal aIea and the scrotmn. The l110re prCulatnrc the inf~mt, the greater the likelihood of cryptorchidism_. Many ar·rested testes eventually descend on their own within the first yeaL After J year, surgical inter-vention (orclliopexy) is warranted to attempt to preserve fertility as well as facilitate flIture testicular exanIS (because of increased cancer risk). Cryptorchidisrn is a Iuajor risk factor f()f testicular cancer (4-0--f(}ld increased risk), and bringing the testis into the scrotlun docs not alter the incrcased risk £()r tcsticular Glncer. The higher the testicle is f()lInd (the furtllCr away {forn the scronun), the higher the risk of developing testicular cancer and the 10\'\Ier the likelihood of retaining ft~rtihty. Note: 1'llc righllesticular/ovaria:n vein drains into the inf(:rior vena cava,wl1ercas the left ovafia:nltest:icular veill drains irlto the left renal vein. Renal t:ransl)]ant: an option {(}rpatient:swith end ,stage renal disease. unless tlley have active ink'ct:io1ls or other lif(> threatening c:on(Jitions (e.g., AIlJS,maliglIancy). Lupus and diabetes

177 llleJIitus afC not contraindications to transplant. hYing, relalt"'.d douors afC best (siblings or par-

ents). especially whcnlILA--siInilar, hut cadaveric kidneys are l110re common because of availability. BeI(lf(, the transplant, pcrf(lfln ABO and Iymphocytotoxic (lILA) cross~matching. I!!iI

I!!l

A transplanted kidney is placed in the iliac fossa (f()[ easy hiopsy access in case of prob·, kl-ns as well as f()f technical reasons); usually the recipient's kidneys are left in place to reduce ITlorbidity. Unacceptable kidney donors: newborns, age over 60, history of generalized or inl:raabdOlTlinal sepsis, history of disease with possible renal involvenJcnt (e.g.. diabetes ITH::Jlitus, hypertension, lupus), and history of Inalignancy.

Rejection: 1. Hyperacute rejection: pref(rnned cytotoxic antibodies against donor kidney (happens with ABO TuiSlnatch as wen as other prefonned antibodies). CIassicpicture: surgery is cOlnplete, vascular c1alnps are released, and tbe kidney quickly turns bluish~hlack.Treatby removing the kidney: 2. Acute rejection: T-cell-- -mediated rejection that presents during first seVerall110nths with feveT, oliguria, weight gain, tenderness and enlargerncnt of the graft, hypertension, and/ or renal function lab derangenleut:. Tre,at by increasing steroids or using antithymo-cyte globulin or other inununosuppressants.

3. Chronic rCjcction:T-·cell- or antibody--Iuediated. Late cause of rena! deterioration present-ing with gradual decline in kidney function, protcirluria, and hypertension. Treatrnenr is supportive and not effective, but the graft Inay last several years hefc)re it givcs out completely. The patient may be retransplanted with a new kidney.

4. Follow creatinine to assess asymptolllJtic rejection (Inure rehable than blood urca nitrogen) . 5. Inununosuppressive ruedications: steroids (inhibit interleukin -1 production), cydo-

spaTine (inhibits interleukin- 2. production), azathioprine (antineoplastic tliat is cleaved into mercaptopurine and inhibits DNA/RNA syuthesis, which decreases B~eeU and Tcell production), antithymocytc globulin (antihody agaiIlStT-ceUs), and OKT3 (antibody to CD3 receptor on T~ce)Js).

6. Cydosporine causes nephrotoxicity, which can be diHlclilt to distinguish froll1 graft: rc~ jection clinically. When in doubt, a percutaneous needle biopsy of the grail should be done if the patient is taking cydosporinc, because the two usually can be dls,tinguished histologically. Renal ultrasound also helps to distinguish between the two. Practically speaking, if yOll lncrease the irmnunosuppressive dose, acute rejection should decrease, whereas cyclosporine toxicity stays the same or WOrSCJ1S.

7. Inullullosuppn'ssioll carries the risk of infection (with COlllIuon as weB as the strange bugs that inl"et patients with AIDS) and increased risk of cancer (especially lymphomas and epithelial cell cancer). Penile auolualies: hypospadias occurs when the urethra opens on the dorsal side of the pen:is; epispadias, when the urethra opens on the ventral side of the penis (also associated with extrophy of the bladder) .Ire,t both surgically. Potter~s syndrome: hilateral rcnal agcnesis causes oligohydramnios in utero (the fetus swal lows fluid hut can.not excrete it), lhnb defC)flnities, ahnonnal facies, and hypoplasia of th.c lungs. It: is generally :incolnpatible with lifc,.

Burns Inay be thermal, chclIlical, or electrical. Initial Jnanagement of all burns includes lot~ anv fluids (usc lactated Ringer's solution or nonnal saline lfRinger's solution is not a choice), re-nlOval of all clothes and other smoldering iteIlls on the body, copious irrigation of chenlical burns, and, of course, the ABCs (airways, breathing, circulation). You should have a very low threshold for intubation; use 100%) oxygen untH significant carboxyhemoglobin fi·OJu carbon rflonoxide inhalation is ruled out. Ill!

Electrical burns: most of the destruction is internal and Inay lead to myoglobinuria, acido-sis, and renal !ailun'. Usc lols of IV fluids to prevent such cOlnplications. The inllnediate, 1ift~··threatcn.ing risk with e1cctr.icity exposure Of burns (including "lightning and putting the fmger in an electrical outlet.) is cardiac arrhythulias. Get an ,EKG.

I!iI

Chemical burns: alkah burns are worse than addic burns, because alkali penetrates more deeply. Treat all chelnkal burns with copious irrigation fronl the nearest source (e.g., tap water).

Burned skin is nmch l110re prone to infection, llsually by Staphylococcus aureus or Pseudomonas sp. (with Pscudomon(Js sp., look f()r a fruity smell and/or blue..green color). Prophylactic antibiotics are given topicaHy only Give tetanus booster to all burn patients unless they received it recently (within the past S years). Severity dassifkation: 1. _First~dcgree burns involve epidenllis only (painful, dry, red areas with no blisters). Keep clean.

2. Second-degree burns involve epidermis and

SOlllC dennis (painful and swollen, with blisters and open weeping surfaces). Remove blisters; apply antibiotic ointnlt.:'l1t (e.g., silver nitrate, silver sulfadiazine, nc-ol1Iycln) and dreSSing.

3. Third·degree bunls involve aU layers of the skin, incJud.i.ng nerve endings (painless, dry, and dlarred). Surgical excision of eschar and ski n grafting are reqUired. Watch for C0111partment syndrome; treat with escharotorny. Hypothermia: body temperature < 9Sor; (3S°C), lIsually accompanied by mental status changes and generalized neurologic ddkits. If the pati.ent is conscious, use slow rewarming with blankets. If the patient is unconscious, consider immersion in a tuh of wan11 water. It is lllost illlportant to lllOuitor the P.KG f()[ arrhythmias, wbich arc conlIllon with hypothcnnia. You also lnay sec the classic J wave, a small, positive deflection f()llowing the QRS cOInpJex. Also 11loniwr electrolytes, renal flulctiol1. and acid·-base status. ~

With frostnip (cold, painfirl areas of skill, mild) ane! Jfostbite (cold, anesthetic areas of skin, l11or(' severe), treat with wanl1jn~~ of aH(~ctcd areas using warm waleI' (not scalding hot) and generalized warming (e.g., blankets).

179

1811

Eme. I"fWnrCV Medicine

f!

A patient is not considered dead until "warm and dead"; in other words, do not give up re-suscitation efforts until the patient has been wJr111cd.

Hyperthermia: may be due to heat stroke.l.ook for history of heat: exposure and high temper" atlue (> 104 0 F). Treat with immediate coobug (wet blankets, icc, cold water). The iunuediatc threats to life are convulsions (which should be treated with dii17,epalll) and cardiovascular C()l-lapse. Rule out infection and other classic culprits:

1. Malignant hyperthcnnia: look for succinylcholine or halotharlc exposure. Treat with dantrolene. 2. Neuroleptic Inalignant syndrOlne: caused by taking an antipsychotic. Pirst, SLOp the Ined-icatioll. Second, treat with SUppOrl (cspcdally lots of IV Huids to prevent renal shutdown frorn rhabdOll1yolysis) and dantrolene. 3. Drug fi:~ver: idiosyncratic reaction to a medication that lJsually Was started within the past

week. Near-drowning: fresh water is worse than sea water, because fresh water, if aspirated, call cause hypervolemia, electrolyte disturbances, and h<::11101ysis. Intubate such patients -jf they are unconscious and monitor arterial blood gases if they arc conscious. Patients who drown in cold water often do better than those who drown in wanTl water because of decreased 111ctaboHc needs. Death usually results from_ hypoxia and/or cardiac arrest.

Milestones: there are a million of' th(~m, bUl concentrate on the table gives rough average ages when lllilestoncs are achieved.

Whih;~ proiie, litis ht:a,tl.l1p- 90'" :Rolls ,fron(tQ :b~rck

t·mb

Vdluntary."g~iiSP. :\ll').~l'et~;a,sQ;)

$·n16

Vohn>.'taty:grat.qi yvitll vbluma1JY: release,

lO"mo

Pla:y~ 'pai:.a--cakf:,

9--10 roo

tower,:{~F2' cltbe,s

13-15 mo

2 y' ~I-S',mQ '

Can b\IHd tower, 'of (i CUbl"'S '(;OO(tlt"(~.

0'1' pup ailcl"spo()Jl

-'9,m9.

t1liderstarid~ l--:st~p,'CQjllin:atids '(nd S'~?tute)

* R{,)ug~, a:v(·ra.w~: ages

ones. The following

J;HO

Sits wit1( i)o .Sllppoxt

C.ail, build

COmnH)})

6~9'lti.o

or . . .to

:lJse,a ,f! is.Jsstss(id, ):i()r ~:Xaln:ple,.-iiHbtt, gestatioi1;' t:heref\:ire, Hit; infant' is expJ:c,tf:d. to pI'.rfonn:ilt

ftmctional.developl11ent.-Wh,!:o'\n· '~vheh,dc\'tJ()pitient

Screening and preventive fare: an ilnportant part ofwell~baby cxalllS that also may provide all answer to a question ahollt a cllild with a cornplaillt. For example, the Inother cOIllplains of' a 4-~y('ar--old child who sleeps] I hours every nigl1t (this is normal). The answer to the question, "What should yOll do next?" may be to gel an objective hearing exam, which is a routine sCH'ening procedure in a +.. ycar--old child. Height, weight, brood pressure, developmental and behavioral aSSf'SSlllCIIt, history and physical exam, and anticipatory guidance (counseling and dis· cllssiOll abollt agl>-appfopri.ate concerns) should h(' done at every vi,sit. Renlt'lnbl'T tbe f()\}owing: 1. M'eta[)o!ic/ulllgenita] disorders: all states mandate screcIl'ing tl.H hypothyfOidistll and plwnylkctolluria at birth (with tIl the Hrst month). Most rnandatc screl',ning f()l' galac-· toscmia and sickle eel! disease. If any of'the screens arc pos-itive, tbe fhst step is a conHr matory test (0 nuke sure that the screen gave yOll a trlle positive. 2. Anticipatory guidance: remernber to tell parents the f()lIovving: " Keep the water heater < 1&

j

IO--120 o E

Use car restraints.

181

182

Pediatrics

f!I

Put baby to sleep on the side or back to help prevent sudden infant death syndrOlne (1110St C01111n011 cause of death in children aged 1-~12 lllonths).

II

Do not use infant walkers (which cause .injuries).

M

Warcl, out f"r small objects (risk of aspiration).

IiII

Do not give cow's fnilk before 1 year of age.

l\il

Introduce solid f()ods gradually, starting at 6 months.

Ii1!I

Supervise children in a bathtub or swiJnrning pool.

3. Height, weight and head circumference: lH_'ad circm,nf(~rence should be lneasured rou-tindy in the Hrst 2 years, height and weight routinely ulltil adulthood. All are rnarkers of general well-being. The pattern of growth along plotted growth curves (which you need to know how to read) tells you luorc than any raw Illunber. If a patient has always been low or high compared with peers, this pattern is generally henign. Parents c01111110nly bring in a child with delayed physical growth or delayed puberty, aBcl Y0L11TlUst know when to H> assure and fc)llow up and \Nhen to do further testing and questioning. If a patient goes limn a nonnal curve to an abnormal CIJfVe, this is a uluch lnore worrisorllc pattern. Ii

ml

Ii

Failure to thrive « 5th percentile for age): most COl11111only due to psychosocial OJ' functional problems. Watch fbr child abuse. Organic causes usually have specific clues to trigger your suspicion. ObeSity: usually due to overeating; < ,51% of cases are due to organic causes (Cushing's or Prader..WiIli syndrome). Increased head circumft'rence nuy mean hydrocephalus or tumor,whereas decreased head circumference may IHcan lllicrocephaly (e.g., frotn congenital TORCH infection).

4. Hearing and vision: should be measured objectively once by or at 4 years old. Measure every few years until adulthood, more often if history dictates. III

After a bout of rneningitis, an children should be screened ()bjectivcly f()r hearing loss (the Illost COlTlIUOn neurologic cOIuplication of lTleningitis). Hearing screening is also inlportant after congenital TORCH infections, Ineasles and rHumps, and chronic lniddle ear effusions and otitis rlledia.

!JlI

Check the red reflex at birth and routiuf'ly thereafter to detect congenital cataracts (usually due to congenital rubella, other TORCH inf(~ctions, or galactosernia) or retinoblastoma (know wbat they look like on exam).

ml

It: is normal for children to have occasi,onal ocular rnisalignment (strahisrnus) until 3 Ino11t1ls; after that, it should be evaluated further to prevertt possible blindness in the affected eye.

5. Anemia: routine screening (hemoglobin and hClnatocrit) is sOlnewhat controversial; traditionally screening was done once in the fhst year (8---12 Illonths), once between 4~6 years, and once during adolescence. If any risk factors f()r iron deficiency are present during infirtcy (prcrnaturity, low birth weight, ingestion of cow's milk befc}rc 12 rnonths, low dietary intake, low socioeconornic status), definitely scree!} th.e hCInoglobin and helnatocrit jf given the optio:n. Give all infiults prophylactic iron supplements; start full-terrn jnfants at 4~6 lXlOIlths, preterm infants at 2. rnonths. 6. lead: initial screen at 12 JIlonths :jJ risk is low, at 6 months jf risk is 11igh (residence i.n old building, paiut chip cater, home ncar battery-recycling plant). lflevel is < ] 0, re screen at 2.4- lT10llths if risk is low or at 12. months if risk is high. After 2. years old, screen annually only .in high·,risk patients. If level> 10, <..loser f()llow up and intervention are Ilceded. 'fhe best Hrst course of action is to stop the exposure.

Pediatrics

183

7. Fluoride: start supplenlentation in 1:lrst few years of life if water is inadequately fluoridated (rare) or if the patient is fed exclusively hom a premixed, ready-to-eat I'mnula (nonfluoridated water is used in such products). Most children need no supplementation. 8. Vitanlin D: some authorities still recornmend that aU breast ~ fe.d infants should n:ceive vitamin D; most reconunend it only {t}r high-risk patients (inadequate lnaternal vitanlin D intake, little snnlight exposme and/or dark skin, exclusively breast-fetl beyond 6 [nouth,s of' age). Start SUpplClllents by 6 rnonths. I'ornlula-fed infants do llot require vitaInin 1) supplcule-nts, which the f(Jnnula already contains.

9. Tuberculosis: screen f()r tuberculosis irlllnediateJy jf it is suggested by history or atIn.l]-ally at any age if risk hctors are present (HIV, incarceration) _If the only risk lactor is living in a high-risk area or hnmi.grant parents, screen once at 4--{) years old and once at 11----16 years old.lfuo risk factors arc present, do not screen. 10. Urinalysis: universal screening is not reconunendcd. Do, however. screen fbr renal disease when a boy < 6 years old develops a urinary tract infection or a girl < 6 years old has repeated urinary tract infections. Get a voiding cystourethrogram and a renal ultrasound. 11. Imnlunizations: when to give normal imlllUllizatiol1s is constantly being updated, so the adnlinistration schedule for COlInnon vaccines is usually given. SpeCial patient populations (pnemllococcal vaccine f()f patients with sickle cell disease or splenectomy) and vaccine contraindications (no measles, nuunps, and rubella or influenza vaccine f(:>r egg~ allergic patients, no live vaCclnes to pregnant WOlDen or immunocornprOluised patients) are high yieH 12. Other: give sexually active adolescents an annual ]'ap smear and screen for sexually transIlljtted diseases. The Hrst dental referral should be nude around 2-~3 years old. Tanner stages: Stage 1 is preadolescent, stage S is adult. Increasing stages are aSSigned fhr testicular and penile growth in lnales and breast growth in felnales; pubic hair developnlcnt is used for both sexes. Average age of puberty (when the patient first shows changes from stage 1 status) is 11.5 years in males (the first event usually is testicular eulargement) and 10_5 years in fClnaies (the fIrst event usually is breast develolnnent). Delayed puberty: no testicular enlargement in lnaies by age (+, no breast developrnt'llt or pubic hair in females by age 13. The usual cause is constitutional delay. Parents often have a similar hi,story. In this norrnal variant, the growth curve lags behind others of saIne age but is consistent. Delayed puberty is rarely due to priulary testicular failure (Klinefelter's syndrome, cryptorchidism, history of chemotherapy, gonadal dysgenesis) or ovarian failure (Turner's syndrome, gonadal dysgenesis)_ Other rarc causes inclnde hypothalamic/pituitary deI"cts, such as Kalhnann's syndrome or tu.n1or. Prewcious puberty: usually idiopathic but may be due to McCuIle--Albright syndrome (in females), ovarian tumors (granulosa, theca cell, or gonadoblastorna) i testicular tumors (Leydig celt), cc.ntral nervOus system. disease or tranrna, adrenal neoplasITl, o~ congenital adrenal hyper·plasia (males only; usually 2.1-011 deIlciency)_ Most patieIlts with au uncorrectable, idiopathie precocious puberty are given longo-acting gonadotropin-·-releasillg honnone agonists to sup'-press progression of puberty and thus prevent preulature epiphyseal closure. Caveru
1114

Pediatrics Caput: succedal1clun: diffuse swelling or edema of the scalp that crOsses the lllidlinc and is benign. CephalhernatOluas are subperiosteal he,morrhages that are sharply linlited by sutures and do not CYOSS the midline. CephaUlcrnato,lllctS a[c llsuallybenign and self~resolving but rarely Inay indicate an underlying skull fracture; get an x--ray to rule it out.

Large anterior fontand.le: Inay indicate hypothyroidisll1, hydrocephalus, rickets. or intrauterine growth retardation, It usually is closed by 18 nlOllths; delayed closure fnay be due to the saIne factors. Important points: 1. Check the urnbihcal corel at birth f(x two arteries, one vein, and the absence of the uraelms. If there is only one artery. consider the pOSSibility of ccrngenita1 renal Jualfbrrnations.

2. FCTnale infants :may have a lllilky",white (arid possibly bloc)dAinged) vaginal discharge iJl the first week of lif(~. This discharge is fJhysiologic and due to Hlatcrna! !JOrmOllC withdrawaL Child abuse: watch f(yr failure to thrive; llUlItiplc fractures, bruises, or injuries in different stages of healing; shaken baby syndrorne (subdura'IIClnatornas .llld retinaJ hemorrhages with no external trauTua signs); behavioral, emotional, and interaction probIcJllS; sexually transTnit· ted diseases; and multiple personality disorder (sexual abuse). Consider abuse whenever tIle injury does not fh the story. Reporting any suspicion is rnandatory; yOll do not need proof and cannot be sued.

Note: Children have diflcorent nonnallahoratory and physiology values (normal values usually arc given): lower blood pressure, higher heart and respirat()ry rates. and diflereJJt hemoglobin and hematocrit vain(~s (higher at birth, 10"vc1' throughout childhood). Renal, Inl1mollJry. hepatic, and central nervous systtm still not fully IIlJturc and flmclional at birth. APGAR Sf.ore: COIluTIon!y done atl and S mLIlIHcs. Do not wait until the 1 ·lTlinute rnark to evaluate the newborn; you may have to suction or intubate the infimt 3 seconds after delivery. The APGAR score induclcs nvc categories with a Inaxirnal score of 2. points per category and a total Il1axiUllUn of 10 points:

1. l1eart rate: 0

= ahsent, I = < 100, 2 =, > 100

2. l\cspiratory cH()rt: 0

none, 1 :::: slow, weak cry. 2.

3. Muscle toue: 0 :::; liInp. 1

~-~ SOBle

=~

good, strong cry

flexion of extreulities. 2.

:::~

active Inotion

4. Reflex irritability (response to stimulation of sole of f()()t or catheter put in nose): 0 ::::: none, J :;::; griIllace, 2. := grimace and strong cry, cough, or sneeze

5. Color: 0 := pale, blue, I

body pink and extrenlitJes blue, 7.

cornpletely pink

ContimH.-', to score every S Ininutcs until the infant reaches a score of 7 or more (while resuscitating).

Important: points: 1. Reye's syndroHlc rnay cause encephalopathy and/()r liver failure in children taking as~­ pirin. The syndn)lne usually develops after infhlCnZJ or varicella inf(:ction. Avoid aspirin in children; use acetarnirlophen instead. 2. 'Moro and palmar grasp reflex should disappear by 6 rnonths.

CHAPTER

29 logy

Pharm

Side. eHects: bizarre, unique, and fatal s'lde effects are tested as wcJl as l'OIllIllOll side cnl'us of cOJlJJllOn drugs.

unUG

SlDEEFFEC'f(S)

Trazodmw Aspirin Bh:orpyc:ili Cy:cloplwspha..~i'.\

Bupropioi", ts()rtiaiid

Priapism Gf blc(:ding. -hYP~l.;S,e:nsitivlt:Y -Pulni Seizures VitanYh1'Bf\'
toxidty

Cyc16:::porlne Piitiil-:dlrris

R.l:naf tbxkity

AngJ otensin~Cq])'lik~r6ilg

Cough

t'l~Z:Yr!'H:',

UnDG

SlOE EFFECf(S)

~"--~,~~----~~~~-~----~-~,-~-~~-"----~-~~--"'"'"~~--~---~-'--~-~'~~-~-

Anil.pJlylax-ts, rash with Epstein.,Barr yirus

hlhiWwrs

Dem0doQydinf~

Ut-hiuin Mf:thoxyflufane Sulfa dntgs Halothane tocal -aiwstlretlc Phenytoin

"Vincristine Amiodaiime

ValjjroJc add IsottetimAI\

ThioridazhH} Heparin Vancomycin Clofibrate 'fetracydines

Dfabt:tes irisipidtIs Dlabetes'insipfd\l$, thyroid (JysPllI1ctiuu -OJ aht'tes 'itis,tpidus Allergies, kernicterus' in nconate)'> hv(~r imcrosis Seizures

l'olill.e det1cit;.nc~';'t0ratogeri; hirsutism:

Periiiheri\{ neuropathy Thyxoid dys'fimdioil NC\lral tuh{< defects' to oHsprjlig Tt;Uible ,terJtog(~;U Rr;tJ.l1ro dep{),~its,'l/ardiil.C wxidty

Thronibocylopel1ia, thrombosii Red inan syridroriH; Iucfeilse.d GJ neuplasmJ> PhO'to$(msitiv1t}~ 'H~(;tll stililling

in childrei) Quim)lone~£

.Qrllntnt: M6l:phtnc CHndamydlf

'feratog<ms (cJHilage d~nnage) CjuChi:HlisttJ (e:g..• tinnitus, vt."rtigo) Sphincter of Qddi spasm Pscl1domem.branO'tls coHos(milX be caused by any l:H'O;u'J-speetrum atltlbiqtte)'

.Chloramphenicol ])n::{oruhIclll

nU$ulfim:

j\plaSti.p'a,uenUa, gra'y-luby s)'l1dtome Cardiomyopathy Bitlrn.t)uary, fibros,is, 'ldrenjl fitilt'lre Tyrainiii«crisis '(¢hc(jse,: wine) LIlrus'crythemat&sus Lupus erytht'nl;ato~qs

Nfonoaniin("'o¥idiise ilihibitc!l'~ Hyol'illazIne PTocaillatuide MinoJddtl Birsuttsrn AUJjuoglycbsJdf;s l:cadng-)()ss, ren;il t~xidty )\(:etttnHI1ophel1 J,iverwxicil y ,(in' high -lloset) ChJ6t'pl'opamide. SYlld£(')})~{:, ..(A' inaplJrnpriate se'creti.on of antidLoreti:c hormone SyiKtrom{~, of:i:napprt)pifate secreti6u-' of dl1tidhrretic hOrni()ue Sy':(td:rj)tne 'of iiu:ip,pi'Opriafe secteti()ll Opiates Qfanddrurt\tic: hormone Di
Clozapin('.

l~g:ranurQcytosis

S2leCl1YG 'serot<min re.'tlptake iJtUihitors (SSRls) WarHuin,

Anxiqty; agitaticiJl,' h180m:nJa

Niacin

3-By drox y.. 3~ methy!glutary( ~

W{:O'()gl:\,; te"'qtog~t1

Skin f)ushinHI PIllTit'CI::; Livcrand'ffit1Sck t.oxicity

cbtllzynw A redU(:tas(~ (HMO eoA Red) Etllamhutpt

0J)tit neuritis'

MctrorridazoJt~

J)j:<;i,tl6ral1l~hke reactioH

Cisplatiri Methyldopa

Nephrotoxicity Hcriwlyl ic ,mC1:rxta

with ak\ihol

(CoOJllbs' posJ,tivt)

185

The side cflc(ts of diuxctics arc high yield.Thiaziclcs calise hyperglycemia, hyperuricemia, hyperlipidemia, hyponatremia, hypokalernic metabolic alkalosis, and hypovolenlia. Thiazides also C
cff(~clS ofpsYI[:hiatrkJ.:n~dkatio:nsare

also high yide!. Sec psychiatry chapter.

Antidote' OVERDQSE

Acetaminophen Cholinesterase inhfMtOTS Ql.rini(liIi~, or tricyclic antidepress~J1ts Iron: Digoxin-

Acetykystdhe Atropinei )?:ralido,XMt Sodi nm bfcarhtHiato''(car.dioptottx.:ti\T)" Dt'-feroxi.l',mine

Norniidiie poffis.<;iU'~11 antl.other eledfQlytt,s; digoxin, ailtkbodies

Lead

Ethanol Fluinazenll Glucagon Edctate

<::oppel'!g01d

Peilidllamine

Opioids

N;ilQxon~;

Methanol 61' ethylene glycol Benzo:di.a7;cpjm~s

]~fu b}()cRe'rs

G1r\)(m' monoxide

()xygt~ll':-(liypei'baricif sev(;re)

MtlScartnh:: r-eceptor'!j]ockei's

PhYJ;osdgnJine

A few drug interactions are high yield. Do nol give the f()llowing drugs together: 1.

M()llO~\Inille

oxidase inJlibitors and lllepcridilll' (cmna)

2. Monoamine oxidase inhibitors aud selective serotonin reuptake inll.i.bitors (serotonin syndrome: hyperthermia, rigi.dity, myoclollus, autonomic instability)

3. KetoconazoJc and astemizok/tcrIt'nadine (lethal arrhytlunias) 4.

ArniHo}~lycosides

and loop diuretics (cnh;mccd ototoxicity)

5. Thj,-17.ides and lithium (Hthiuru toxicify)

hnpo:rtant points: 1. Hifampin 'is given {(H' propllyl;uis ill contacts ofa patient with Ndsseriu meniuHitidis.

2. Barbiturates, antiepileptks, ;mc1 rifampin induce hepatiC enzymes; CilllCtldine and keto" conClzok inhibit hepatic cuzyrnes (inlportant {tH dTug--drug interactions). patic[lt r(,spolld~; to placebo, it does 110t meal! that the disease is psychosomatic; it llWaltS Simply that the patiellt respollded to plan~b()! Normal pc'oplc with real diseases oftcn have all llllprovcnlCnt in symptoms with placebo.

3. J f

d

Pharmacology

1117

HORMONE REPLACEMENT THERAPV AND ORAL CONTRACEPTIVES Hormone replacement therapy (HRT) should be considered li)r al/ postmenopausal and posthystereclomy VVOlnen. Observation during therapy is necessary, because eslrc)gen and progesterone are Ilothannless. Every patient should rnake the decision on her own after weighing the risks and benclits.

Known benefits of estrogen therapy: decreased osteoporosis, decreased fractures (especially hip fractures), decreased coronary heart disease, reduced rlsk of all-cause Illortality, reduced hot flushes or flashes, and reduced genitourinary symptoms (dryness, urgency, atrophy--ineluced incontinence, frequency). The decrease in coronary heart disease is probably due to the fact that estrogen increases high--density lipoprotein cholesterol; this bendkiaJ eff(~ct is reduced or negated by coadrninjstration of progesterones. Known risks of estrogen therapy: increased risk of endometrial caucer (eliIninated by coad-~ Iniulstration of l)fogcsterones), increased risk of venous throlliboembolism, possible increased risk of breast cancer (not with short-·tennuse) and increased risk of gallbladder disease. The risk of breast cancer Inay increase aner 1() Or' 1110re years of use, but this risk is controversial. Other side effects of estrogen therapy: endOlnetrial bleeding, breast tenderness, nausea, hloating, and headaches. Absolute contraindications to estrogen therallY: unexplained vaginal bleeding, active liver disease, history of throrubophlebitis or thr0l11boembolism, history of endOlnetr:tal or breast cancer.

Relative contraindications to estrogen therapy: seizure disorder, hypertension, uterine leimnyOlnas, farnilial hyperlipjdcnlia, rnigraines, thrOlubophlebitis, endollletriosis, gallbladder disease. Important points: 1. WOlIlen who take estrogen therapy need an ClldOlllctrlaJ biopsy and dilatation and curettage at the onset of treatlnent to rule out hyperplasia and cancer and an evaluation of any unexplained bleed:tllg, even whHe on therapy, unless they have had a nonnaJ evaluation in the past 6 months. 2. The nraiu reason to give progesterone with estrogen .is to elirninate the increased risk of endornetrial cancer. If a women has no uterus, do not give progesterone. Oral contraceptiv(~s s.hould not be given to women over 35 who srnoke or have other cardiovascular risk factors (hypercholesterolernia or untreated hypertension) because of an increased risk of sudden death. Oral contraceptives afC the nlOst connnon cause of secondary hyperten,sionin women, and any patient who is noted to have increased blood pressu,re should discon~ Hnuc oral contraceptives and have her blood pressure rechecked at a later elate. Absolute contraindications to oral contraceptives: sInoking after age 35, pregnancy (dc) pregnancy test before prescribing), breast-·f{:eding, active liver disease, hyp(~dipjdemia, uncontrolled hypertension, diabetes 111ellitus with vascular changes, l)rolonged irnlIlobilization of an cxtrernity, history of thrornboemhohsm or throrn.bophlebitis, coronary artery disease, stroke, sickle cell disease, cstrogen"depcIHknt neoplasll1 (breast, cnOOlllctrillnl), liver adenoma, and history of cho1estatic jaundice of pregnancy.

188

Pharmacology

Relative contraindications to oral contraceptives: depression, rnigraine headaches (l1laY trigger attacks), olig0111Cnorrhca, undiagnosed anlcnorrhea, gallbladder disease, and heavy cigarette srnoking under age 35. Side effects of oral contl'aceptives: glucose intolerance (check fi:Jf diabetes 111ellitus annually in patients at high risk), depression, edema (bloating), weight gain, cholelithiasis, henignhver ad.enolnas, Ine1aslna ("the rnask of pregnancy"), nausea, vOlniting, headache, hypertension, and drug interactions (drugs such as rifarnpin and antiepileptics 11lay induce rnetabolism of ()ral contraceptives and reduce their dl(~ctjveness). Important points: 1. Because of the risks of thrOlnboembolislll, oral contraceptives should be stopped 1 month bef()re elective surgery and not restarted until 1 IlIOlith aher surgery.

2. The risk of breast cancer does not seem to be increased with oral contrdcepUvcs, except in long--terln users (controversial; unlikel)! to he asked on boards). Cervical neoplasia HId}! be incn~ased,possiblybecause of the conf()unding factor of increased sexual rela00115 and number of partners; nonetheless, oral contraceptive users should have at least annual Pap smears. Other benefits of oral contraceptives: 50% reduction iu ovmiou coueer; decrease in the incidence of 111eUOIThagia, dyslnenorrhea, benign breast disease, flmcUoIlal ovarian cysts (often prescribed for the previous four effects), premenstrual tension, iron--deficiency anelnia, ectopic pregnancy. and salpingitis.

ASPIRIN, NONSTEROIDAl. ANTI-iNfLAMMATORY DRUGS, ACETAMINOPHEN Effects: aspirin and nonsteroidal anti~inflalnnlatory drugs (NSAIDs) inhibit cyclooxygenase centrally and peripherally, giving then1 anti-inflamlnatory. antipyretic, analgesic, and antiplatelet properties. Aspirin inhibits cydooxygcnase irreversibly and thus for the lift, of the platelet, whereas other NSAIDs inhihit cydooxygcnase reversibly. Acetarninophen is Inostly central~acting; thus, it is only an analgesic and antipyretic with no platelet or anti-inflanlmatory eff"cts. Toxicity: 1. Aspirin rnay cause GI upset and hleeding, gastric uIeers, Jnd gout. Always consider Gl bleeding and ulcer in any patient taking aspirin or NSAIDs. 2. Higher aspirin doses cause ti.nnitus, vertigo, respiratory alkalosis and rnetabolic acidosis. hyperthermia, COl1la, and death. 3. Aspirin can be rcrnovedby dialysis in severe overdose. 4. Do not give aspirin to people with Tlasal polyps. Fl:ypersensitivity reactions are extrel11cly conl111on in this group; look f()r nasa] polyps in anyone widl all asdnnatic--type rc_action to aSpir111. 5. People witll astlnna lnaybavc an asthma attack after taking aspirin--- --even those without nasal polyps. 6. Do not give aspirin to children younger than 8 years with feveT or viral infection; it may cause Reye\; syndrome (look fe:Jr encephalopathy and liver dysfunction).

Pharmacology

1119

7. Other NSAIDs also cause GI upset, bleeding, and LIleers. Always cousider GI bleediug and ulcer in any patient taking aspirin or NSAID.

8. NSAIDs also rnay cause renal damage (interstitial nephritis and papillary necrosis), especially in patients who take theIn chronically and have preexisting renal disease. 9. Phenylbutazone can cause fatal aplastic anemia and agranulocytosis and should nol- be used chronically. 10. Acetanlinophcn causes liver toxicity in high doses dne with acctylcysteine.

1:0

depletion of glutathione. Treal

Note: New NSAID/prostaglandin E[ combinations help to prevent GI damage. I~{)w-dose aspirin

has been proved to be of beneflt in reducing the risk of stroke in patients ·with a transient ischemic attack or previous stroke, in reducing the risk of myocardial infarction in patients who have had a previous lnyocardial inf:lrction and patients with stable or unstable angina who have not had a myocardial infarction. Many als() recoTlllllcnd daily aspirin {()r patielltswith known coronary artery disease. Generally, use of aspirin {()r prhnary prevention of myocardial infarction or stroke in a patient with no definite history of rnyocarchal infarction, angina, or coronary artery disease is not alJpropriate. Studies have not shown a clear benefit:, and there may be an increased risk of heulOrrhagic stroke and/or sudden death. If the patient has a history of liver or kidney disease, peptic ulcer disease, GI bleeding, poorly controlled hypertension, or bleeding disorder, the risks of aspirin prophylaxis may outweigh the benefits.

Important points: 1. Give aspirin to any pati.ent i.n the crnergency de.partrncnt with unstable angina,myocardial infarction, or transient ischernic attack. 2. Stop aspirin I week bef()fe surgery, other NSAIDs

011

the day bef()re surgery.

'-,- J

CHAPTER

3

Screening and/ or confinuatory radiologic tests f()r suspicion of difft:'renl cliscJses

SImI! (yacture (depre>scd)

CO,Inrhlte.d toritograpl\y.-CCl') stall

Tie.ad 'ttallma

C't witliant co:ntt'ast

11ltj'aerat)ial·he'lio:rrh~:e

(IT .witheiUt· contrast

:Acme stroke

CT wldlo.ut contrasl'

Mu{tiple sclirosis

Magl1ctiC ,tesOilft\li::e iIU~lging (MRI) of b,rahi

.Brain turnqr-hvetasmses

CT 'or 'N1:lU wtth: contrast

PneUint211ta

Cllt\st x-ray

ObStttVf :ifinild

x~ray

Chest- tra'uma'

Cliest

CheSt.m;lSS

Cr-scan

HlemofH;ysi$

Chest .x'~ray

Bnm.Ghoscopy

Pl1h'l.lonary'enibolisl11 Aoitic:,ane-ilry.srn](jisse:ctlon

Venri!;lti6n!:p/irrusibH:scan .(ilUdt.;ar scan) ,

Pulmonary arteiiogram

C1'- with contr,ist

A:D1,~tic t~::-ar:{tra;Uli1d)

Angiofp:anl

Ca.rotid stel10sis

Du.ple,x liltrasotl110

Esophage,a:lohsiructj()11

Bariurn':x-~ray

o-r endost:opy

E,nAo8\;Oriy

ES(tphageal-tear.

Badiul1 Jl>,(ay (If'endoscopy.

:Endoscopy

Howe} 'r10rforatj611

Chest :alld,'abdouiinal x- rays (free air)

Laparoscopy

Ht:ixiateUlesis

EndoscI>f}Y Upper GJ .st.fl~S

):jndoscopy

!?,eptk:uker' disease }(l5dcIUliil,11 tratH1la

orell~losCQpy

Cf or ,qiagriQstic per,itolltiaJ hi.nge (scc:'ettlergency -riwdicirie, chapt~r)

l,apar.oscofiy

tVbi'!tHHUlal ,a'Qitlc, a:nenr:ysm·

er with ('()fitJ'
Abdominal absce.ss

Cf with cQ1'itnwt

Ch()Jdithiasls

Ultrasound

Gholedochohrhiasis:'

UItnisdutid

E!\CP

Chdlecystitjfi

Ultrasound.

ElDA. scan (nuclear hcpalobiHary study)

Intest~nal

obstruction

j-\bdomiha} x~l'ay (supine amI 'iipright)

Appeildicins

Abdomilfa:i x~tay; then ultrasound

taparOSf:Opy

Nephrolithi~lsts

Abdomin,al x~ray; then ultrf;sotmd

Il1Jl'il.ve11Ous pyelogram

Clinical diagnosis

191

COND.i1'lON

SCl~JlliNING (OR ONtY) t,ES't TO ORDER

~~~---' ----~-~-~~~---.~,'--~---~~~_.~----

Ova'ria'n pat.llology

Ultrasolll1d

Di\'ertiCulitis

Barium

CONFIRMATOWY TEST

_.~-----,._----,---=",..----"--~-,_.---_._---'-~~---

l.apar6scop¥

0Jlt~f\1J: (not

acntdy)

CT

Upper'GI'bkediJlg

tJpper-GT serks or endoscopy

Hild()s~:opy

Lower (;1 bleeding

Barium t:Jlema

{'ndosC()ljy

Ent]t)SCU!lY

hkeding'si,lJd)'

L"parotntiT}C

UllknojVJj.

Gl'-Bleed

COMMENTS --'---~~-----'-'---

(II'

Nncl!~.'H il}tdicint~

No scope ;KlHdy!

Or 3ngiox-niphy (brisk bleed) I'I~((tr01iephrr><;j::;

IntravqlloW; pyelogram Illitially, \lltrasolmd

to I()llmv H{'w,ltuda (peniistent)

Inu'avenous pyelogram

I;jbroid utt:flis

lJltl'<1sound

Pelvic mass (ft'malej

Uhoiionnd

'hone nlC(.lst,lS('~

Bont s<;an

Pregn,\Ui;yevaluaIJoll

Ultrasound (tf41lSvagiJli11 dCICCl~ BOOne1: thou trawi,lhd,omjnal)

li racturc

X-ray

OSfeomyelitis

X-ray

Artht'itis

X-r~y

Pyloric sten,osis

Ultrawund

MCI:hl's diverticulum

Meckt'l's scan (mH_']ear rnedkin0 scan)

CT

First do

neG test

HIDA;:::: hep
hnportant points: 1. r'-Iyperkalemia may he caused by a hemolyzed blood sample or rhabdomyolysis (due to high intracellular potassillIll concentration).

2. J--Iyponat:rcIIlia Iuay he caused by hyperglycemia. hyperproteinemia, or hyperhpidcJuia; these forms of secondary hyponatrcillia wHl correct with correction of tbe glucose, lipid, or protein levels. 3. Correcting hyponatremia aggressively (esptTially wit h hypertonic saline [3%1) may cause hrainstcm damage (central [Jon line myelinolysis).

4. AI kalosis may cause hypokalemia and symptoms of hypocalcemia (perioral numbness. tetany) due to cellular shift:; acidosis lllay cause hyperkalemia by the saUle IIlfchanism. 5. High levels of amylase and lipase may he due to sources other than the pancreas (salivary glands, GI tract, renal fiilure, ruptured tubal pregnancy), but elevation of both in the saUle patient usually is due to pancreatitis. 6. Alkaline phosphatase can be elevated by biliary (Hscase, bone disease, or pregnancy. If the elevation is due to biliary disease, g J 5 usually implies dehydration. 12. Positive results OIl the rapid plasma reagin or Venereal Disease Hesearch Laboratory test f()r sypbilis IIlay he due to systemic lupus erythematosus, 13. 11] patients with isosthenuria and hyposthenuria---~the inabilit.y to concentrate urinethink of diabetes insipidus or sickle cell diseascltrait. 14. The erythrocyte sediulcntation rate (ESR) is a worthless test in pregnancy; fiSH. is elevated by pregnancy itself A high---lIormal hlood urea nitJ'ogen:creaUninc ratio nlay mean renal disease in pregnancy.

193

Important points: 1. Do not fbrce adult jehovah's witness patients to accept blond products. 2. If a chHd has a lift~-threat:ening condition and the parents refuse a shnple, curative treatIIlcnt (antibiotics for meningitis), {lrst try to persuade the parents to change their mind. If you cannot, your second option is to get a court order to give the treatIl1t.'nt. But do Hot give the treatlnent until you talk to the courts ifynu can avoid it. 3. Let COlnpctent people die if they want to (10 so. Never force treatments on adults of sound Inind. Respect wishes {C)f passive euthanasia, but avoid active euthanasia. 4. Do not tell anyone how your patient is doing unless he or she is directly involved with

cafC and needs to know or is an authodzed family meInber. If a colleague asks ahout a. friend who happens to be your patient, refuse to answer. 5. Break confldentiality only in the f()llowing situations: Drhe patient asks you to do so. iii

Child abuse is suspected.

gThe courts mandate you to tell. mYoll have a duty to protect

lift::. (Iflhe patient says that he or she is going to kiH someone

or hiIn-- or herself, you have to tell the intended victiIn, the authorities, or botb.) mThe patient has a reportable disease. You must report to authoritks, let thelll deal with it.

patient is a danger to others. If the patient is blind or has seizures, let the proper authorities know so that they can take away the patient's license to drive. If the patient is an airplane pilot and a paranoid, hallucinating schizophrenic, authorities need to know.

I!!I Tbe

6. lnfonned consent involves giving the paUent information about the diagnosis (his or her condition and what it means), the prognosis (the natural course of the condition without treatlnent), the ]Jroposecl treatment (description of the procedure and what the patieIlt will experience), the risk/benefits of the treatment, and the alfernativc /rcafmems. The patient is then allowed to Inake his or her own choice. The documents seen on hospital wards that patients are rnade to sign are ncither required nor sufficient fc)r infc)rrned consent; tiley are used for J11CdicoIcgal purposes (i.e., lawsuit paranoia). 7. When the patient is inCOlnpetent, a guardian (surrogate decision maker or health care power of attorney) should be appointcd hy the court.

195

196

Ethics

8. Living wills and do-not~-resuscitateorders should be respected and f()llowed if done correctly. For example, ifin a living will the patient says that a vcntilator should not he used if he or she is unable to breathe independently, do not put the patient on a ventilator, even jf the spouse, son, or daughter lnakes the request. 9. Depression always should be evaluated as a reason fl)I the patient's "incompetence." Patients who are suicidal rnay refuse all treatment; this decision should not he respected until the depression is treated.

10. Patients can he hospitalized against their will in psychiatry (if they are a danger to sciI' or others) £()f a liInited time. After 1-3 days, patients usually get a hearing to determine whether they have to rcrnain in custody. This practice is based on the principle of benefice,ce (a principle of dOing good felf tbe patient and avoiding harm). 11. Restraints can be used on an incompetent or violent patient (delirious. psychotic) if needed, but their use should be brief and reevaluated often. 12. Patients under 18 do not rc.quircparental consent in the f()llowing situations: II

If they are ernancipated (rnarricd, living OIl their O\,vn and nnancially indcl-K'.ndent, parents of children, serving in the anned f()fces)

mIf they have a sexually translniUed disease, want contraception, or are pregnant. e If they want drug treatlne11t or coullseling. SOlne states have exceptions to these rules, but for hoards let such minors nuke their Own decisions. 13. If a patknt is COInatosc and no surrogate decision Tnaker has been appointed, the wishes of the family generally should he respected. If there is a family disagreement or nlterior motives arc evident, talk to your hospital ethics cOJlnnittee. Use Courts as a last resort. 14. In a pediatric cluergeUc.:y when parents afC not available, treat the patient as you sec fit.

15. Do not hide a diagnosis from patients (including pediatric patients) if they want to know the diagnosis·--~evenif the falnily asks you do so. Do not lie to any patient because the family asks you to do so. The flip side also applies: do not fc)rce patients to receive iufor-lnation against their will. If they don't want to know the diagnosis, don't tell them. 16. If a patient cannot connnunicale, give any required (~Inergency care unless you know that the patient does not want it. 17. Withdrawing and withholding care are no different in a legal sense. Just because the patient is on a respirator does not 111ean that you cannot stop it. 18. In terminally ill patients, give enough medication to relieve pain. Opioids are C0111monly used.

Although some questions with photos can be flgured out without looking at the photo, this is not always the case. You should be able to recognize the entities listed bclovv. The Color Atlas tIIld Text of Clil1icul Medicine by l~orhes and Jackson is a great source. l~or other good atlases, seC your lihrarian.

Blood smears: ~

Howell-Jolly bodies (asplenia/splenic dysfunction)

~

Basophilic stippling (lcad poisoning. thalassemia)

II!I

Malaria

II!I

Spherocytosis

l1i!I

Target: cells (thalassemia, severe liver disease)

~

Heinz bodiesl"bite cells" (G6PD defiCiency)

e Schislocytes/heJrnet cells (disseminated intravascular coagulation, thro.rnbotic topenie purpura, microangiopathic hemolysis) 00

Multiple myeloma

l1i!I

Acute lymphoblastic leukcluia, chronic lympbocytic leukemia

fiji

Auer l'ods (acute lnyeloid leukeIIlia)

Iii!

Chronic Inyelocytic leukemia

00

Acantbocytes (abetalipoproteinernia)

~

Teardrop cells (myelollhrosis. myelodysplasia)

fill

Iron deficiency anemia

m Sideroblastic

thromhocy~

anernla

~

1;ol-atc!I\J2 anemia (rnacrocytic, hyperse.gmented ncutrophHs)

00

Sickle cell disease

mil

Reticulocytes

C' dry" bone mal'row tap)

00 Aplaslic anenlia m Chediak-Higashi

cell

"

Reed~Stcrnberg

mil

Dohle bodies (toxic lymphocytes; f()r boards, think ofEpstein~-Barr virus)

cell (Hodgkin's lymphoma)

00 Hairy cell leukemia

191

198

Photos: The Glossy Book

Ophthalmology: m Kayser~Fleischer

ring (Wilson's disease)

1'1

Bacterial conjunctivitis (especially in neonates)

IJ!I

Glaucoma (closed--angle attack or acute)

Ill!

Graves' disease (exophthalmos)

IfiJ

Diabetic fimdus

II

Hypertensive fundus

!!II

Central retinal artery occlusioIl (fundus)

H

Central retinal vein occlusion (fundus)

II!!

Papillexlema

liM

Retinoblastoma (white reflex instead of red)

I!\Il

XanthelaSIlla

IliI

Corneal arcus (in patients < SO, a lnarkerf()f hypercholeslerolernia)

m Roth III

spots (think of endocarditis)

Herpes simplex keratitis (dendritic uker seen with fluorescein; avoid sterOids)

mCataracts (bad enough to notice with the naked eye)

"Orbita] cellulitis Dermatology I skin findings: IiIl

Pityriasis rosca

!I

Neisseria sp. septiceUlia (severe purpura)

!is

LyIne disease (erythelna chronicurn rnigrans)

~

Tinea ca.pitis

M

Scabies

!!14

Fsoriasis (ski.n £lndings and nail pi,Uing)

M

Cheilitis/stomatitis (think ofB vitamin deficiencies)

00

Tbxic shock syndrorne, scalded skin syndrOlue

!lil

AbdOlninal striae (Cushing's syndron1e)

w Erythema rnarginatum (rheumatic ft~ver) U

Janeway and Osler lesions (endocarditis)

li!1

Acanthosis nigricans (marker f()r visceral malignancy)

• Syphilis (chancre, condylOlna lata, secondary syphilis rash) " Herpes (I and II) !ill

Varicella zoster virus (chickenpox, shingles, trigeminal and ophthahIlic invc)lveluent)

• HenochSchDnlein purpura (rash) I1iil:

Erythclna rnultifbrme (target lesion)

• Malar rash (lupus) gj

Heliotrope rash (dennatm:nyositis)

!ii!l

Oral hairy leukoplakia (caused by Epstein-Barr virus; seen in HIV--I)()sitive patients)

m Basal

cell cancer

Photos: the Glossy Book

j§J

Squamous cell cancer

mI

'Melanorua

Ii1l

Actinic keratosis

I!ll

Stasis dennatitis/venous insuffkiency (skin changes, ulcers)

199

mArteria] insulliciency (skin changes) fJI

Diabetic f()()t ulcers (sinlilar in appearance to arterial insufficiency ulcers but usually jJojnJess)

Ii',;

Vitiligo

(ass()ciat(~d with

pernicious anelnia and hypothyroidisrn)

m Impetigo

~

Raynaud\ phenornenon (Huger autoJluputation; often seen in sclerodenna)

w Ternporal

arteritis (tortuous-looking temporal artery)

lfil

Clubbing of the Hugers

Illi1

Acne

IIil

Adcnorna scbaccmll (tuberous sclerosis)

ll!I

CcmdylOlua acmninata

l!il

Molluscml1 contagiosmll

mBitot's spots (vitamin A defkiency) II!I

IIil

Cafe-·au-lait patches (neuroHbrOluatosis in patients with Bonnal IQ, McCune-Albright syndrollH:.: with Inental retardation) Varicose veins

m Cullen',

sign

Ji',j

Grey~'I'urner

II

Erythema infectiosurn (slapped cheek rash with fever resolution just bef()re rash appears)

I!il

Sturge-Wcber syndrOIne (hemangioma; port-wine stain on one side of face)

M Cavernous

sign

hernangiomas (in children, rnost lesions resolve

!f1i

Hirsutism_ (know conditions associated withit)

IIIl

Rocky Mcmntain spotted

f(~ver

OIl

their own)

rash

mPyodenna gangrenosUlll (think of ian amrna tory bowel disease) l@

Erythema nodosmn (think of jnflannnatory bowel disease, infections [the classic exalllple is Coccidioidesimmilis or tuberculosis], or sarcoidosis)

WI

Pretibial rnyxedclna (Graves' disease)

l!ll

NeurofibrOIuatosis (skin)

m Keloids

(usually in blacks)

l!!l

Allergic contact dennatitis

!&J

Tinea corporis and tinea cruris

Microscopic findings: red, grarll--positive :::::. hlue) plus clustering tendencies

§j

Granl stain (grarn--Tlcgative

I!!I

Caseating granulOluas (tuberculosis, fungi)

I!il

Noncaseating granulomas (sarcoidosis)

l@]

Goodpasture's di.sease (linear imrnunofluorcsccncc i.n kidney)

WI

Gout (needle-·shaped crystals from a joint with no birefringence)

200

Book

Photos: The

llil

Pseudogout (needlc--shaped crystals with positive birefringence)

I'!.I

'TrichomoJlas sp.

w Clue cells

(C;mdnercJl(J sp. vaginitis)

Ii

Giardj(J sp.

l!ll

Koilocytosis (think ofhUluan papillo1l1J.virus or cytcHllegalovirus)

Radiologic findings: !!il

Lobar pneulllonia

w Sinusitis (rnaxHlary or frolJtal sinus opaciflcation) ijI

Sarcoidosis

!l!!I

()steoJrthritis (o!-;lcophytes, interphalangeal joint changes)

• Multiple myeloJna (puncbed-out skull ,-rays) mOsteosarcoma (sunray

()f

sllIlbnrst pattern)

• Duodenal and jejuual atresia ("double-bubble" sign) • Esophageal atresia (bariulll x-ray) /!IiI

Pleural efIilsion

mCongestive heart failure on dlest x--ray • Small bowel obstruction (air-flnid levels) l:1II

Toxic lnegacolon (Hirschsprung's disease or 1n£1anu11al:ory bowel disease, inf(~ction)

I'!.I

Pneumothorax

II!I

Grossly ahnornlal ventilation-perfusion scan

GIl

Classic chest x--ray of tuberculosis

• Chest x-ray findings of asbestosis (pIaq Ltes) m Abdominal

aortic aneurysm

011

angiogram

l@I

Achalasia (esophagus on barium enema)

!.lI

Volvulus on barium enerna (birers beak)

-Epidural hematoma (CT) • Subdural hematoma (CT) mAppearance of a 111ajor cerebrovascular acci.dent (stroke) m Berry

OIl

CTIM_Rl

aneurysllls on angiogranl

• Slipped capital f"moral epiphysis (x-ray) mNephrolithiasis on abdolninal x-Tay (radiopaque) III

Coarctation of aorta (on angiograrn)

• Multiple sclerosis (plaq lies on MRI) l'i\!

Pancoast tumor On chest x-Tay (know associated Horner's syndrome)

I]

Lung abscess

OJl

chest x-ray (air--fluid level)

~

Bossing of the skull on x--ray (think of hemolytic anemias, espec.ially thalasscrnias and sickle cell disease; and in elderly patients Paget's disease)

Ii'Il

Colon cancer on bariuIll enema (apple--core and napkin-Ting lesions)

Ii'Il

Liver tumors on CT (nletastasis 20 titnes rHore likely than priruary tmllor)

Photos: the Glossy Book

I!I

Severe carotid artery stenosis on angiogranl

IIll

Shoulder separation on x,-ray

M

Lytic lesions of bone on

x~ray

201

(think of malignancy)

Other plIotos: III

Rhemuatoid arthritis (swan~neck defonnity, boutonniere defonnity, ulnar deviation, rhemnatoid nodules)

M

Osteoarthritis (Heberden's and Bouchard's nodes)

m Gout

(podagra, tophi)

mDactylitis (sickle cell disease) mDown syndnnue (facies, silnian crease) m Turner's !Ill

syndrorne (body habitus, widely spaced nipples, webbed neck, cubitus valgus)

Horner's syndrome (unilateral ptosis and l11iosls and history ofhernianhydrosis)

II Bell's

palsy (facial asymmetry)

m Cushing's m Graves'

syndrome (facies, striae)

disease (exophthafmos)

D

Acromegaly (facies)

M

Peutz~Jeghers

iii

Achondroplasia (overall appearance; usually autosolnal dominant)

M

Candidal infection (vaginal, thrush)

D

Gonorrhea (yellowish discharge)

D

Erb's palsy (waiter's tip)

m PolycystiC

syndrome (freckling pattern on face)

kidneys (gross appearance)

mFetal alcohol syndrome (facies) I!lI

Decubitus ulcers (best prevention is frequent turning of patient)

ll!l

PseudohermaphrotidisIll (picture of alnbiguous genitalia; look for II-hydroxylase

deficiency) mTanner stages (male and fbnale) m Congenital

syphilis (Hutchinson's teeth, saddle nose delclrInity)

M

Osteomyelitis extending to the skin (think of Staphylacoccus or Salmonella sp. in sickle cell disease)

M

Scleroderma (late-stage facies)

!!Ill

Spina bifida (gross appearance; encephalocele_.meningocdeocele, rneningolnyelocele, oc--

culta/patch of hair) M

Strawberry tongue (scarlet fever and Kawasaki's disease)

!!Ill

Acute tonsillitis (Streptococcus sp. or Epstein---Barr virus; rarely diphtheria in UninlI1Umized

patient) .. Acute pharyngitis (viral or streptococcal) IIl\I

Gynccornastia (nonnal finding in pubertallua1es)

@11(~nosynovitis (think M

of gonorrhea if the patient is sexually active)

Hypertrophy of the heart (gross specimen: severe disease)

202

Photos: The Glossy Book

i!!I

Dilated cardiOluyopathy (gross speciInen; severe disease)

mKaryotype showing Down (trisomy 21), Turner's (XO) , or Klinefdter's (XXY) syndrome IiiIl

Fetal heart strips (normal, short--term, and long-tenn variability; early, variable, and late decelerations)

Babinskfs sign: stroking the f()ot yields extension of the big toe and fanning of other fOes ill patients with upper n1otor neuron disease. Beck's triad: jugular vein distention, 111uffled heart sounds, and hypotension in cardiac ponade; do pericardiocentesJs.

tam~

Brudzinski's sign: pain on neck flexion with lueningeal irritation.

Charcot's triad: fever and chills, jaundice, and right upper quadrant pain in patients with cholangitis. Courvoisier's sign: a: pain less, palpable gallbladder should make you think of pancreatic cancer. Chvostek's sign: tapping on the facial nerve elicits tetany in hypocalcernia, Cullen's sign: bluish discoloration of periurnbilical area due to retroperitoneal hemorrhage (pancreatitis) .

Cushing's reflex: hypertension, bradycardia. and irregular respirations with very high intracranial pressure. Grey~ Turner

sign: bluish discoloration of fla.nk from retroperitoneal hemorrhage (think of

pancreatitis) . HOITIW'S

sign: calf pain on f()rced dorsiflexion of the f()ot in patients with deep veil} thrornbosis.

Kehr's sign: pain in the lefl shoulder with a ruptured spleen. Leric:he's syndrome: claudication and atrophy of the buttocks with in1potence (seen with Jor·, toiliac occlusive disease). McBurney~s sign:

tenderness at McBurney's point with appendicitis.

Murphy~s sign:

arrest of'jnspirati.on when palpating right -upper quadrant under the rib cage in patients with cholecystitis. Ortolan;'s sign/test: a palpable or audible dick with abduction of an infant's flexed hip means congenital hip dysplaSia. Prehn's sign: elevation of a painful testicle relieves pain in epididymitis (vs. torsion). Rovsing's sign: pushing on the lefi lower quadrant produces pain at McBurney's point in pa·tients with appendicitis.

203

204

Signs, Symptoms, and Syndromes

Tiners sign: tapping on the volar surface of the wrist elicits paresthesias in carpal tunnel syndrOlne. Trousseau's sign: pumping np a blood pressure cuff canses carpopedal spasm (tetany) in bypocalcemia. Virehow's triad: srasis, endorhelial damage, and hypercoagulability (three broad categories of risk factors for deep vein thrombosis).

WORD ASSOCIATIONS Word associations are not all 100% accurate, but they are useful in cll1ergencies. iii

Friction rub: pericarditis

breathing: deep, rapid breathing seen in rnetabolic acidosis (think of diabetic ketoacidosis)

IW KUSSJ113Ul

III

Kayser~Fleischer

I!!I

Bitot's spots: vitalnin A deHciency

III

Dendritic corneal ulcers: herpes keratitis (seen best with fluorescein; avoid steroids)

ring: Wilson's disease

mAlnaurosis fugax: temporary, painless, monocular blindness seen in transient isch(~mic attack (watch out far telnporal arteritis; ifu is suspected, start steroids before biopsy COIlfIrmation to prevent blindness) III

Cherry..,red spot on the rnacula: Tay-Sachs disease (no hepatospleuOlnegaly) or Nienlann" Pick disease (hepatosplenomegaly)

II

Bronze (skin) diabetes: helllochrmnatosis (look also for cardiac and liver dysfllllction)

1/1

Malar rash: lupus erythenutosus

I!I

Heliotrope rash: derrnatomyositis

M

Clue cells: Gatdnerella sp. infection

1\1 Meconium M

ileus: cystic fibrosis

Rectal prolapse: cystic llbrosis

mSalty-tasting baby: cystic llbrosis IIlI

Cafe--au-Iait spots: neurofibrOlnatosis (if mental retardation is present, think of McCuneAlbright syndrome or tuberous sclerosis)

m Worst

headache of patient's lif,,, suharachuoid hemorrhage

.. Abdominal striae: Cushing's syndrome (or possible pregnancy) HI

Honey: infant botulislll

II

Left lower quadrant tenderness/rebound: diverticulitis

m Children

who torture animals: conduct disorder (Inay be antisocial as adults)

Ii!l

Currant jelly stools in children: intussusception

iiJ

Arnbiguous genitalia and hypotension: 21 <-hydroxylase deHciency

!11

Cat-like cry in children: cri-du--chat syndrOlne

.. > I 0 lb. baby: maternal diabetes .. Anaphylaxis from immunoglobulin therapy: IgA deficiency Ij

Postparturn fever unresponsive to broad-spectrmll antibiotics: septic pelvic thrOlTI-bophlebitis (give -heparin f{)f an easy cure and retrospective diagnosis)

Signs, Symptoms, and Syndromes

Illl Increased A2 hemoglobin and aneluia: thalassemia lI\1

Heavy young WOlnan with papilledclua and negative radiology: pseudotulDor cerebri

iii

Low-grade ft:~ver in first 24 hOUTS after surgery: atelectasis

III

Vietnam veteran: posttraunntic stress disorder

B

Bilateral hilar adenopathy in a hlack patient: sarcoidosis

B

Sudden death in a young athlete: hypertrophiC obstructive cardiomyopathy

B

Fractures or bruises in different stages of healing (children): child abuse

!!i

Decreased breath sounds in a traUl1la patient: pneumothorax

OJ

Shopping sprees: mania

OJ

Constant clearing of throat (children): rOmelle'S syndrome

mIntermitt.ent bursts of swearing: Tourctte's syndrOnlE.'. Ill!

Koilocytosis: human papillOInavirns or cytol1wgalovirus

• Rash after anlpicilhn or arnoxicilhn f()r a sore throat: Epstein-Barr virus infection OJ

Daytime sleepiness and occasional falling down (cataplexy): narcolepsy

205

AAA

abdominal aortic aneurysln

Ab

antibody

abx, Abx antibiotics

ABC, ABCD, ABCDE

airway, breathing, circlliation, disability, exposure (trallrna protocol)

abd

abdominal

ABG

arterial blood gas

ABO

bloocltypes (A, B, AB or 0)

AC

abdominal circumference

ACE

allgiotensin--converting enzylne

ACI~-I

angi()tcnsill-convertillg enzylne inhibitor

ACL

anterior cruciate ligament

ACTH

ad renocorticotropic hormone

ADH

antidiuretic hormone

ADHD

attention~dellcit

AF

am niotic fluid

API

amniotic fluid index

afib

atrial flhrillation

AFP

alpl1a~felOprotein

AIDS

acqUired irrllllunodeflciency syndrmne

ALL

acute lymphoblastk leukemia

ALT

alallillc aluinotransferase

AMl.

acute lllye]oid leukemia

ANA

antinuclear antibody

ANCA

antincutrophil cytoplasmic antibody

ANOVA

analysis of variance

ANS

alltonomic nervous system

AP

a1lteroposterior

ARDS

adult H'spiratory distress syndrome

hyperactivity disorder

2117

2011

Abbreviations ARF

acute renal failure

ASA

acetylsalicylic acid (aspirin)

ASAP

as soon as possible

ASD

atrial septal defect

ASO

antistreptolysin 0 (in streptococcal infection)

AST

aspartate aminotransferase

ATG

anti thymocyte globnlin

Aut

autosolllal

AV

arteriovenous or atrioventricular

AVM

arteriovenous malfonnation

AXR

abdominal x--ray

AZT

azidothymidine (zidovudine)

B.1l

beta

BAl.

dimercaprol

BE

barimll enema

8M

bone lnarrow

BMR

basic metabolic rate

BP

blood pressure

BPD

biparietal diameter

BPH

benign prostatic byperplasia/hypertrophy

RPM

beats per minute

BPP

biophysical profile

BSO

bilateral salpingo-oophorectomy

BT

bleeding time

BUN

blood urea nitrogen

Bx. bx

biOpsy

C

centigrade (e.g., 37° C) or complement (e.g., C1, C3, C4)

C&S

culture and sensitivity

c-section cesarean section

c-spiue

cervical spine

Ca

calcium

CA

cancer

CAD

coronary artery disease

CRC

cOlnplete blood count

cc

cubic centiIneter

ceu

coronary / cardiac care unit

CD

cluster of differentiation (e.g., CD4, CD8)

CEA

carcinoembryonic antigen

cGy

cClltigray

Abbreviations

enn

coronary heart disease or congenital hip dysplasia

Chem

chemistry

chenlo

chemotherapy

CHF

congestive heart failure

CK

creatine kinase

Cl

chloride

CLL

chronic lymphocytic leukemia

em

centimeter

CMl.

chronic myelocytic (or rnyelogenous) leukemia

CMV

cytomegalovirus

eN

cranial nerve

eNS

central nervous system

CO

carbon monoxide or cardiac output

CO 2

carbon dioxide

COPD

chronic obstructive puhnonary disease

CPD

cephalopelvic disproportion

CPR

creatine phosphokinase

Cr

creatinine

CRF

chronic renal failure

eRP

c-reactive protein

eSF

cerebrospinal fluid

CST

contraction stress test

CT

cOlnputed tomography scan

CV

cardiovascular

eVA

cerebrovascular accideut (stroke)

CVP

central venous pressure

CVS

chorionic villus sampling

ex

culture

CXR

chest x-ray

D&C

dilation and curettage

DDI

dideoxyinosine (HIV medication)

Dern1., derm

dernlatology

DliS

diethylstilbestrol

DJ

diabetes insjpidus

DIe

disserninated intravascular coagulation

dilI

difkrential or difflcile (e.g., Clostridium dill or C. difl)

Dig

digoxin

DIP

distal i.nterphalangeal (joint)

DKA

diabetic kCl(lacidosis

209

210

Abbreviations

dl

deciliter

DM

diabetes mellitus

DMSA

2,3-dinlcrcaptosuccinic acid, sllccirner

DNA

deoxyribollucleic acid

DNR

do not resuscitate

DU

diagnostic peritoncallavage

DUB

dysfunctional uterine bleeding

DVT

deep venous throlubosis

Dx,dx

diagnosis

EBL

estiInated blood loss

EIlNA

Epstein-Barr nuclear anUgen

EIlV

Epstein--Barr virus

EeG

electrocardiogranl

EDTA

edeLate

EEG

electfocncephalogranl

EF

ej cetion fraction

EKG

electrocardiogralh

ELISA

enzyme-linked inununosorhent assay

EMG

electromyogram

EPS

extrapyranlidal systeIll

ER

emergency roorn

BRCP

endoscopic retrograde cholangiopancreatography

li'RT

estrogen replacernen t therapy

ESP

extrasensory perception

ESR ESRD

erythrocyte sedirnentation rate

BtOH

alcohol (ethanol)

I'

fluoride or feluale

FDP

.fibrin degradation product

Fe

iron

FEP

hee erythrocyte protoporphyrin

FEV

forced expiratory vol-tune

FEV,

f(xced expiratory volurnc in 1 second

FFP

fresh frozen plasrna

I'SH

f()llicle--stimulating horJnone

FTA~AIlS

fluorescent: treponelIlal antibody-absorption test (f(l[ syphilis)

FTI

free thyroxine index

I'Ve

J()fced vital capacity

end~stage

fracture

renal disease

Abbreviations

g.gm

gram

G-6-PD. G6PD

glucose- 6-phosphatase defkiency

GBS

group B Streptococcus

GERD

gastroesophageal reflux disease

GFR

glornerularfiltration rate

GGT

gamma-g]utamy]transpeptidase

GH

growth honnone

GI

gastrointestinal

GnRH

gOl1adotropin--releasing honnonc

GU

genitourinary

GYN

gynecology or gyDecologic

H2

histamine type 1 receptor

H&H

henlOglobin and hernatocrit

H&Phistory and physical examination HAY

hepatitis A virus

HhAle

glycosylated hemoglobin

HBIG

hepatitis B immnne globulin

HBeAhlAg

hepatitis B core antibodylantigen

RBeAhl Ag

hepatitis B "e" antihody/antigen

HBsAhl Ag

hepatitis B surface antibody/antigen

HBY

hepatitis B virus

He

head circunlference

HCC

hepatocellular carcillODla

HCG

]uunan chorionic gonadotropin

HCI

hydrochloric acid

Hct

hcrnatocrit

HCV

Jlcpatitis C virus

HDJA

high-density lipoproteins

HDV

hepatitis D virus

HEl.LP

hemolysis. elevated liver enzymes, low platelets

Hep

hepatitis

HEY

hepatitis E virus

H,

fln

H(Jemophilll~

influcnwe

Hgh

heulOglohin

_~-HIAA

S~-hydroxyindoleacetic acid

Hih

Hacmo],hilllS inflllcllZuC type b (vaccine)

HIV

human irnn.mllodeHciency virus

HLA

hUlTlan leukocyte antigen

hMG

Inunan lllcnopausal gonadotropin

211

212

Abbreviations

HOCM

hyperrrophic obstrucriv" cardiomyopathy

HPV

human papillOIna virus

hr

hour/hours

HRT

honnone replaccluent therapy

HSP

Henoch-·Sch6ulein purpura

HSV

herpes simplex virus

HTN

hypertension

HUS

hernolytic uremic syndrome

HVA

homovanilhc acid

Hx

history

I&D

incision and drainage

IBD

in£lannuatory bowel disease

IBS

irritable bowel syndro111c

ICP

intracranial pressure

Ig,IG

immunoglobulin (e.g., IgA, IgM, IgG, IgE)

1M

intramusLlllar

lOP

intraocular pressure

IPV

inactivated poliovirus vaccine

IQ

intelligence quotient

IU

international units

IUD

intrauterine device

IUGR

intrauterine growth retardation

ITP

idiopathic thrombocytopenic purpura

IV

intravenous

IVC

inferior vena cava

IVDA

intravenous drug abuse

IVF

intravenous fluids

IVIG

intravenous imrllunoglobulins

IVP

intravenous pyelograIIl

JRA

juvenile rheumatoid arthritis

JVD

jugular venous distention

JVP

jugular venous pressure

K

potassiurn

KCI

potassimll chloride

kg

kilogram

KOH

potassiurn hydroxide

I,

liter

IA

left atrium

LAE

left atrial enlargement

Abbreviations

lb

pound

LEW

low birth weight

LCP

Legg-Calve-Perthes syndrome

LDH

lactate dehydrogenase

IDI

Jow~dellsity lipoproteins

lES

lower esophageal sphincter

IFT(s)

liver function testCs)

IGI

lower gastrointestinal (below the ligament ofTreitz)

IH

luteinizing hormone

UQ

left lower quadrant

IMN

lower motor neuron

IMP

last nlellstru al peri od

lOC

loss of consciousness

lR

lactated Ringer's solution

1,S

lecithin:sphingomyelin ratio

lSD

lysergic acid diethylamide

LUQ

left upper quadrant

IV

left ventricle

IVF

left ventricular failure

IVH

lel; ventricular hypertrophy

M

lnale

MAl

Mycobacterium avium·-intracdlulare complex

MAO

monoalnine oxidase

MAO-l

monoalnine oxidas.e inhibitor

MAST

rnilitary antishock trousers

MBP

myelin basic protein

MCHC

lucan corpuscular hemoglobin concentration

MCP

metacarpophalangeal (hand joint)

.MCV

ITleall corpuscular vohune

MEN

multiple endocrine neoplasia

Inet

metabolic (e.g., met. alkalosis)

mets

llletastasis

MG

rnyasthenia gravis

MHA-TP microhemagglutination assay li)r antibodies to Treponema pollidum (l'lT syphilis) MI

myocardial infarct

ml

milliliter

mm

lnillilueter

MMR

measles, mumps, rubella (vaccine)

mo

month/lnonths

213

214

Abbreviations

MR

rncntal retardation

MRA

magnetic resonance angiogralll

MRI

lllagnetic resonance iInaging scan

MRSA

ITlcthicillin-resistant Staphylococcus aureus

MS

rnllitiplc sclerosis

MVP

Initral valve prolapse

Na

sodimll

NEC

necrotiiing enterocolitis

NG

nasogastric

NGT

llasogastric tube

NH3

amnlOnia

NHL

non~Hodgkin's lymphoma

NPH

isophal1c insulin suspension

NPO

nothing by mouth

NPV

negative predictive value

NS

normal saline

NSAID

nonsteroidal anti ~inflanlInatory drug

NST

nonstress test

N/V

nausea/vOluiting

O2

oxygen

OA

osteoarthritis

OCP

oral contraceptive pill

OD

overdose

OGT

orogastric tube

OGTT

oral glucose tolerance test

OM

otitis lnedia

OPV

oral poliovirus vaccine

OR

operating room

PAC

premature atrial contraction

PAN

polyarteritis nodosa

Pap

Papanicolaou Sluear

PCL

posterior cruciate hgaruent

PCN

penicillin

pcos

polycystic ovary syndrome

PCP

phencyclidine or Pneumotysti.\' carinii pneumonia

PCWP

pulmonary capillary wedge pressure

PDA

patent ductus arteriosus

PE

pulmonary clnbolus

PEEP

positive end--expiratory pressure

Abbreviations

I'G

prostaglandin (e.g., PGE2, PGF) or phosphatidylglycerol

PH

puhnonary hypertension

PID

pelvic inflalnInatory disease

PIH

pregnancy·-induced hypertt'_nsion

PIP

proximal interphalangeal (joint)

PRU

pbenylketonuria

PMN

polymorphonuclear leukocyte

PMS

premenstrual syndrOlue

PO.

phosphate

PPD

purified protein derivative (tuberculosis skin test)

PPROM

preterm preluature rupture of the membranes

PPV

positive predictive value

prn

as needed

PROM

premature rupture of the IlICl11hranes

PSA

prostate--speciHc antigen

pUpt.

patient/patients

PT

prOthr0111bin thue

PTCA

percutaneous transluminal coronary angioplasty

PTH

parathyroid hOfll1one

PTT

partial thrOluboplastin tilne

PUD

peptic ulcer disease

PVC

prernature ventricular contraction

PVD

peripheral vascular disease

PZA

pyrazinamide

RA

right atriurn

RAE

right atrial enlargenlCllt

RAI

radioactive iodine

MC

red blood cells

RDW

red blood cell distribution width

Rec

recessive (e.g., autosomal rec.)

REM

rapid eye movement (dream sleep)

RI'

rheumatic fever

Rh

Rhesus blood-group antigeu

RI

reticulocyte index

RLQ

right lower quadrant

RNA

ribonucleic acid

RPR

rapid plasma reagin test (It)r syphilis)

RSV

respiratory syncytial virus

RUQ

right upper quadrant

Of

rheumatoid arthritis

215

216

Abbreviations

BV

right ventricle

BVF

right ventricular £lilure

RVH

right ventricular hypertrophy

SI, S2, 53, 54

heart sounds 1-4

SEO

small bowel obstruction

SCD

sickle cell disease

SCFE

slipped capital fc'moral epiphysis

SCID

severe cOlubined irmnunodeficiency disease

SD

standard deviation

SES

socioeconornic status

SIADH

synd.rome of inappropriate antidiuretic honnone secretion

SIDS

sudden infant: death syndrolne

Sl.E

systernic lupus erythullatosus

SOB

shortness of breath

SIP

status post (after)

SSRI

serotonin-selective reuptake inhibitors

Staph

Staphylococcus

Stat

immediately

STD

sexnally transmitted disease

StIep

Streptococcu\

SVC

superior vena cava

Sv0 2

systernic venous oxygen saturation

SVR

systclnic vascular resistance

Sx (Sxs)

symptom (symptoms)

T3

triiodothyronine

T4

thyroxine

TAH

total abdominal hysterectomy

TB~

tuberculosis

Tb

TBG

thyroid hinding globulin

TCA

tricyclic antidepressant

TE

tracheoesophageal

Tet

tetralogy (ofFallot)

TFTs

thyroid function tests (usllally means TSH, T4 , free T+ index, '1'] resin uptake)

TIA

transient ischelnic attack

TIRe

total iron-binding capacity

TIPS

transjugular intrahepatic portosysternic shunt

TM

tympanic membrane

TMI'/SMZ

TORCH

trimethoprimsulfamethoxazolc toxoplasma, other, rubella, <:ytomegalovirus. herpes

Abbreviations

tPA

tissue plaslllinogen activator

TRlI

thyroid-·releasing honnone

TSH

thyroid~stilnulating horrnone

TTP

thrOlnbotic thr0111bocytopenic purpura

TURP

transurethral resection of the prostate

Tx

trealUlent or therapy

UA

urinalysis

DC

ulcerative colitis

UGI

upper gastrointestinal (proxima] to the ligament ofTreitz)

UMN

upper nlOtor neuron

URI

upper respiratory infl~ction

US

ultrasound

UTI

urinary tract tuft'cHon

UV

ultraviolet

VACTERL

vertebral, anal, cardiac, tracheoesopbageal, renal, limb (malformations)

Vaneo

vallcOlnydn

VCA

viral capsid antigen (in Epstein-Barr virus)

VDRL

Veuerea] Disease Researcb Laboratory test (for syphilis)

VFib or Vfib

ventricnlar fibrillation

VIP

vasoactive intestinal peptide

VIPoma

pancreatic lurHor that secretes vasoactive intestinal peptide

Vit

Yltan1in

VMA

vanillylmandelic acid

V/Q

">;Jcntilatioulperfusion (ratio)

vs.

versus

VSD

veutricular septal del"ct

VTach or Vtach

ventricular tachycardia

VUR

vesicoureteral reflux

vWF

von Willebrand's factor

VZIG

varicella zoster ilnmunoglohulill

WBC

white blood cells

WPW

Wolff·Parkinson-White syndrome

yr

year I years

ZES

Zollinger--Imtson syndrome

217

ABCDEs, trauma management, J SO Ahdominal aortic aneurysrn (AAA), 173 Abducens nerve, I S9 AhortiOlJ,138--139 AbruptiO placentae, 14-1 Acetaminophen, toxicity, 188~ 189 Acid/hase status hlood gas interpretation, 8 causes of primary' disturbances, 9 laboratory testing, 193 tn'atmenl for diswrhances, 9 Acne,83 Acquired inm1unodctlciency syndrome (AIDS), 96~9 7 Acute: abdomtn appendicitis, 148 gallhladder disease, 148 laparotomy, 147

lOG1.lization of; 147 pancreatitis, ] 49 splenic rupture, 11-8 stomach ulcer, 149 Acute fatty liver of pregnancy, 11-.5 ACute renal failure (ARF) postreual, 4-3 prerenal, 43 renal causes, 4-3- 44Addison's disease, 40 Adenomyosis, I 24· Adjustment disorder, ] 17 Adrenal insufficiency, 40 Adrenal tumor, 67 Adult respiratory strcs~ syndrome (ARDS),

26 Aging, J 03 Aifway obstruction, 151 Akathisia, antipsychotic drug side eff{~ct, 114 Alcohol f(~tal alcohol syndrome, 8 incidence. of ahuse, 8 risk fiKtor for diseases, 7--8, 6 I treatrrH.'_nt of abuse, 8 Wernicke's verSllS Korsakoff's syndromes, 7 withdrawal, 7----8

Alkalillc phosphatase, ] 93 Alpln-ft,tol)wtdn, C'valuation \)1 pregn;\lley, 133 Ambiguous genitalia, 12.8 AmCW)IThea primary, 11.6 secondary, ] 25--126, 131 Amphetamine abuse, 12.0 Amrinone, imravenous therapy (()r sllock, 16 AmyotrophiC lateral sclerosis, 93 Analysis of variance (ANOVA), liO Auemia causes of: .52--55 ddini tion of, S] diagnostic steps, S ]--S 2 hemolytiC anemia, 52. screening in children, 182 Angina stable, 1g unstable, 19 variant, _\ 9 AnkyJosing spondylitis, arthritis with, 48 Anorexia, l t 9 Anterior cruciate ligament, 162 Anticoagulation therapy, 20-~2l Antidotes, tahle of; 186 Antipsychotic nledicatiOlls extrapyramidal side eHects, I] 4types of, 1 14 Aortic aneurysm. Se.e Abdominal aortic aneurysm Aortic rupture, 152 APGAH score, I 84 Aplastic anemia, 5S AppendicitiS, 14-8 Arrhythmia causes of; 23 EKG abnormalities, n trcafmcms ic)r, 22.

Arthritis, 4-7---1-8 Ascites, 34 Aspirin protective eff('(:[s, 189

toxicity, 188---189 ASlhrna,25

AtheroscJel"()sis, 6 Atopic dermatitis, 80 Altentioll~defkit hyperactivity disorder (ADED), 119 Attributahle risk, 108 Allti.:->m, It9

AntoinunUIle hemolytic anemia, 54 Babinski'S sign, 203 Baldness, 83 Barbiturate abuse, ] 21 Barrett's esophagus, 35 Basal cdl canCN, skin, 85 Basilar skull fracture, J 66 Basophilia, 56 Beck Depression Inventory, 118 Btck's triad, 103 Belu;et's syndrome, 1-9 Bell's palsy, 169 Benign prostatic hyperplasia (BPH) , 175---176 lknzodiazepine abuse, 121 Bereavement, 116 Bias, ]1]-~]12

Billary tract dis('Jse, 35 Biophysical proHle (BPP), fi:~tal evaluation, 133 Biostatistics, glossary of terms, ] 07"-112 Bipolar disorder, 114--115 Birth control, 128 Bladder cancer, 67 Blindness, causes in adults, 157 Blood dyscrasias, .59 Blood smears, photo recognition fllr hoards, 197 Blown pupil, 165 Brain tumor, 66 Braxton--I-1icks contractions, 137 --138 Breast cann'r biopsy, 62~--63 incidence o( 62 risk factors fC)I", 62 signs and symptoms of; 62 treatment, 63 Breast discharge, 12.7

219

220

Index

Breast fceding, 13 S Breast DlaSS women over 35, 127 wOIncn under 35, 127 Hrudzinskl's gign, 703 Bulimia, 119 Bulla, skin, 80 Burns

degrees. 179 types of 1 79 Cafh:ine withdrawal, 121 Candidiasis, 81, 98 Caput succedaneum, 184Carcinoid tumor, 68 Cardiac taruponade, J f) 1 Cardiovascular medicine, I 7~23 arrhytlnnia,1.2--23 congestive heart failure, 21---22 deep vein thrombosis, 19--20 myocardial infarction, 17--19 pediatric cardiology, 23 pulmouary embolism, 20 -21 valvular heart disC<1.~e, 19 Carotid stenosis, 173 Cataract, 156, 158 Cavernous hemangioma, 183 Central retinal artery occlusion, 1 S 7 Central retinal vein occlusion, 157 Cerebellar disorders, 93 C(~rebrospinal fluid blo()(l}~ 165 flndings, 87 C(;rvical cancer, 65 Cervical rib, 174Chalazion, 157 Charcot joint, 4-8, 162 Charcot's triad, 203 ChediakuHigashi syndrome, 98 Chest pajn, diagnosis of; .l7~19 Chi-squared test, 110 Chickenpox, 73 Child abuse, J84-, 19S Chlorpromazine, 111Choking, 153 Cholangitis, 14-8 Cholecystitis, 148 ChoJest
Chvostek's sign, 203 Claudication, 173- 174 Clotting tests, 56 Clozapine, 114 Cocaine abuse, 120 Coloreclal cancer, 63 -64Compartment sy'ndrome, 161 Complement deficiency, 98 Conduct disorder, J 19 Confldence interval (Cl), 109 Confounding variables. 1 I I Congenital heart defects, 23 Congenital hip dysplasia (CHD) , 163 Congestive heart failure symptoms and signs of; 21 -22 trealJIH;nt of; 21 Conjunctivitis etiology of 155 neonatal, 155 Conn's syndrOlne, 41 Contact dermatitis, 80 Contraction stress test (CST), [33 COt pulmonale, 22 Correlation codHciem, 109 Courvoisier's sign, 203 Cranial nerve ksions, 90-~9 J palsies, 1 S8·-~ 1S9 Cn:'atine kinase, 193 Cri--du--chat, 101 Crohn's disease, 32~·-33 Croup, 75 Cryptorchidism, 176 Cullen's sign, 203 Cushing's reDex, 203 Cushing's syndrome, 4-1 Cyst, skin, 80 Cystic fibrosis, 27--2.8 Decullitus ulcer, 85 Deep vein thrornbo:-;is (DVT) causes of; 19 diagnOSis ot:1 9·,,20 prevention of, 20 Delayed pnberty, 183 Delirium, 88--89 Dl:meotJa, 88--89 Depression, 115--116, 118 Dermatitis herpeU{()fmis, 8S Dermatology, 79~86 conditions, 80-~8b lesion types, 79--·-80 photo recognition f(xboards, 198 -199 D{~rmatomy()siJis, 49 Developmental mik~stones, 181 Diabetes insipidus, 10, 1-1, 193 Diabetes mellitus compliance monitoring, 4complications oJ: 4 diagnosis of, 3----4f()ot problenls with, 4insulin types, 4-

Diabetes mellitus ((ont) medication interactions, 5 peripheral neuropathy in, 4in pregnancy complicatiOns, 139 macrosomia, 131, 139 treatment, 139-1 +0 retinopathy with, 4,156-157 screening, 3 Somogyi effect versus dawn phenomenon, 4surgery preparation, .5 symptoms, 3 treatment goals, 3 Diaphragnnlic hernia, 2. 7, 37 Diaphragm rupture, 152. Diarrhea causes oC 31~u31. tnanagernenl 32 DiGeorgc syndrome, 97 Diptheria, 75,106 Disseminated intravilscular coagulation (DIe),16 Dissociative hIgUt', 117 Diuretics, side effects, 186 Diverticulosis, 31 Dobutamine, intravenous therapy {(X shock,

at:

]6 Do--not-reSllscitatc order, 196 Doparnine,intravel1ot'ts therapy lor shock. 16 Down syndrome, 100 Drowning, 180 Dysfunctional uterine bleeding (DUB), ]24--]25 Dysthymia, 11 6 Dystonia, antipsychotic drug side eHeet, ]]4

Ear, nose, and throat surgery, 169-171 Eatlm-Lamhert syndrome, 94 Eclampsia, 136-·137 lictopk pregnancy, 139 Edwards syndrome, 100 Iilectromyography (I(MG), 88 Emergency medicine, 179-180 burns, 179 drowning, 180 hyperthermia, 180 hypothermia, 179-180 Emphysema,2S Em;opresis, 220 Endocarditis, 19, 71Endocrinology, 39---42 hypothalamic--pituitary axis, 39 Endometriosis, 124 Enuresis, 11.0 EOSinophilia, S6 Epidemiolo~:y, per--year rate overview, 107 Epididymitis, 17.5 Epidural anesthesia, in labor, 136 Epidur;t1 hematoma, 165

Index

Epiglottitis, 7 S Epinephrine, imravenous therapy.I()f shock, 16 Epstein-Harr virus, 74Error types, 111 l;;rythema inf{:ctiosUIn, 73 Erytherna multif(mne, 84Erytberna nodosum, 85 lirythrocyte sedimentation rate (ESR), 193 Esoph"'geal c",neel', 67 Esophageal disorders, 35 Estrogen, honnone replaCCluent therally, 187 Ethics, 195-196 Facial nerve, 90 Facial paralysis, causes of~ 169 riactitious disorder, 1 J 7 I'alnccrosis, 12.7 Fetal alcohol syndrome, 8 Fihroadenoma,! 17 Fihrocystic disease,! 27 Fibroids, 121Ti'ihromyalgia, 50 Fifth disease. See Erythema infectiosum Flail chest, J 52 Floppy baby syndrome, 93 Fluoride supplementation, l83 Folate defkiency, anemia with, 53 V(lut3.ndh-::, largl: anterior, 184Foreign body aspiration, 27 Fracture open versus closed, 16 I open versus dosed reduction, 161--162 Fungal skin inf('ction, 80--81 Gallbladder disease, 1+8 Gastrinoma, 64 Gastroenterology, 29-~38 bleeding, 30--31 Hl<\Hi.)flnalions in children, 3(,. -38 Gastrocs(lphageal reflux disease (GEfl,D), 29 Gastroschisis, 37 Generalized anxiety disorder, J 16 General sllr~~ery, 147-15.3 acute abdomen, 147--·150 preparation and f()l1ow-up, 149-~1 SO trauIna, 150·~IS3 GeTleiics, 99-101 autosomal dominant diseases, 99 alltosonlal recessive diseases, 99-~ 100 chromosomal dborders, 100 -101 polygenic disorders, 100 Genitalia, ambiguous, 128 Geriatrics, 103 G-bucorna dosed angle glaucoma, 56 open angle glaucoma, .55- 156 GJossopharynge;11 nerve, 90 GlllClgonoTna, 64Gl1,lcoscn6-phospllate dehydrogenase deJiciency, anemia with, -':is

Gout, 47 Grey-Turner sign, 203 Grie( 116 Group B streptococcus (GBS), treatment in pregnancy, 136 Growth charts, pediatrics, J 82 Guinain-Barr{~ syndrome, 87--88 Gynecology, 123-129 breast rnass diagnosis, 127--128 tertiHty, 125----12/ infections, 123--J 2.4 lwdiatric gynecology, 11.8·--119 Haloperidol, 114 Halstead-Reitan Battery; 118 Headache, causes of~ 89--90 Hearing loss, causes of; 169--] 70 HELLP syndrome, 136 IIemato logy,51-57 anemia causes o( 5).,-,')5 ddInition o( S1 diagnostic step>, 51 -S2 hemolytic anemia, S 2 transfusions, 55---56 Hemochromatosis, 34 Hemolytic uremic syndrome, 32,4-5 Hemophilia, arthritis with, 4-8 Hemothorax, luassivc, ] S J Henoch~Sch(mlcin purpura (HSP), 37,45 Hepatitis, 33·-34, 106 Hernia, types of: 1+9 Herpes simplex keratitis, 157 Hiatal hernia, 'L 9 Hirsutism, 83 Histiocytosis, 68 Homan's sign, 203 Hordeolum, IS 7 I-Iormone replacement therapy (I-IItr), 187 I-Iuman chorionic gonadotropin (hCG) , changes during pregnancy, 139 Human immunodeficiency virus (HlV) (h3.gnosis ()fi.nh~ction, 96 Tnanagement of in feet ion, 96- 97 pregnancy managemem, t 35 Huntington's disease, 92 Hydatidiform mole, 132 Hydrocele, 176 Hydrocephalus, 167 Hyperammonemi,l, 34 Hypercalcemia, 12 Hypercluesis gravidannll, 1'I-S Hyperintl1lunoglobulin Ii syndrome, 98 Hyperbk'mia, II, 193 I-Iypennagnesemia, 12---13 Hypernatreluia, 10 Hyperphosphaternia, 13 HypersensitiVity reactions type 1. 95 type II, 95 type m, 96 type IV, 96

Hypertension, 1---3 drug therapy, 2. emergl':ncies, '2 evaluation of; in pregnan('.y, 1 screening, 1 secondary hypertcllsion, 2--3 Hyperdwnnia, 180 Hyperthyroidisln, 39 -4-0 Hypocalcemia causes of: 11----12 symptoms, 11 treatment oJ: 12 HypoglossallWrVt\ 9 \ HypokalerniJ, 'j 0----11, 193 Hypomagne~emia, 12

Hypomania, I 15 IIypOlMtremia, 9 ..,,] 0, 193 J-Iypophosplntemia, 13 Hypospadias, 177 IIypothermia, 179----180 Hypothyroidism, 39,193 Idiopathic thrombocytopenia (ITP), 4-.5 Ilmnuni'l.ation adults, 106 children, 183 Immunoglobulin A (rgA) detlciency, 97 Immunology, 95-98 AIDS, 96---97 hypersensitivity reactions type I, 95 type II, 95 type lll. 96 type IV, 96 prima.ry inununodt.-.fkiendes, 97·~98 Imperforate hymen, J 29 Impetigo, 74 Impotence, J 76 Incidence, definition oJ; 110 Infant botulisIli, 93 fnf(cetiollS disease, 69~78 classic hoard que~tions, 71-- n, empiric therapy; 69 endo-card-itis, '74meningitis, 74----75 pediatric respiratory inf0ction, 75 pneumonia diagnosis, 70.--71 rabies, 76 rashes, 72---74staining of bacteria, 69----70 staphylococcal inlt~ction, 77· -7 g streptococcal illf(~ction, 76--77 syphilis, 72 Infectious rllononucleosis, 74 Inflammatory bowd disease, 32.~-33 Influenza, imnTllnization in adults, 106 Infl:H'lllecl conscllt, 195 Inhalant abuse, r 2. -I lllsulinorna, 64Internallnedicine, 1-. 16 ;lCid/base status, 8---9

221

222

Index

Internal medicine (cont.) alcohol, 7-8 cholesterol management, 5--6 diabetes, +"..5 hypercalcemia, 12 hyperkaJcloia, I] hypernatrc.I1lia, 10 hypertension, ],,--3 hypocalcemia, 11-12 hypokalemia, 10--- 11 hyponatremia, 9---10 magnesium and phosphate disturbanc(;s, 12--13 shock, 14--- 16 smoking, 6---7 vitamin and milH'ral deficiency llOxidty, 13-]4

Leukemia, 59 Lice, 82. Lichen planus, 84 Ljv(~r disea5;e acute, 33---34 chronic, 34metabolic derangenH.·ntsin, 3+ 35 Liver tumor, 67 LiVing will, ] 96 Lumbar disc herniation,162 Lung eanCer, 61-,,62 Luria-Nebraska Neuropsychological Ballery, 118 Lyme dis(';tse, arthritis with, 48 Lysergic acid diethylamide (LSD) abuse, I WI Zi

Intracerebral hemorrhage, 165 Intracr,10iaf hypertension, 166 Intrauterine growth retardation (lUGH.), 132. Iron deficiency, anemia with, S Z

Macular (h'generatioll, J 58 Macule, skin, 79 Malignant meianomJ, 68, 86 Malingering, 117 Mallory--Weiss tcars, 36 Mania, 114--115 Marijuana abuse, 120 Mastitis, 127 McBunwy's sign, 203 Measles, 72-13 Medicaid, W7 Medicare, 107 Meig's syndrome, 65 Melaoorna, 68, 86 Meningitis, 74----75 Menopause, 12(r] 27 Mental retardation, 119 Mesenterk ischemia, 174Metastases, 60 Milrinone, jntravt~nous therapy f(Jr shock,

Jaundice, 34, 37-38 Jehovah's witness, 195

Job-Buckley syndrome, 98 Kaposi's sarcoma, 68, 86 Kawasaki's syndrome, '1-9, 73~ 74 Kehr's sign, 2.03 Keloid,85 Keratitis, 156---157 Keratoacanthoma, 85 Kidney stOHl'S. See Renal stones Klindelter syndrome, 101 Korsakoff's syndrome, 7 Krukenberg's tumor, 65- -66

J6

Labor abortion, 138-,-139 arrest disorder, 137 epidural anesthesia in, 136 false labor, [37--·]38 ft·tal nlillpresel1tation, 14-5 induction, conuaindications, 138 Tlonnal characteristics, 137 placenta removal, 144 peematuee rupture of memhnnes, 143 preterrn labor, j 42 protraction disorder, 137 shoulder dystocia, l40 true labor, j 3 7 Laboratory Inedidne, 193 Large bowel obstruction, J +9 Lazy ('}'e, 159 Lead poisonillg anemia with, 51screening in children, 182 Learning disorder, 119 Legg--Calvc,Perthes disease, l63 LeiolnyonJiI, J 2+ Leriche's syndrOlne, 173,203

Minerals, deHeiency and toxicity, J 3---14 MinnesolaMultiphasic Personality Disorder, 118 Moles,8S Molluscum cOlltagiosum, 82 Monoamine oxidase inhibitors, drug ilW~l'actions, J 86 Morphy's sign, 203 Mourning, 116 Multiple gestation, 14-6 Multiple personality disordel~ 117 Multiple sclerosis, 87 Muscular dystrophy, 94 Myasthenia gravis, 94· Myelophthisic anenlia, 55 Myocardial infarcti(m findings in, 17--18 silent, 19 treatrnent ftw, 18 Narcolepsy, I 1g Nasopharyngeal cancer, 67 Neck HlaSS, I 70--,- J 7 J Neck triUJrm, 152-153

Negative predictive value (NPV), 108 Nephritic syndrome, 44 Nephrolithiasis. Sec Renal stones

Nephrology, 43-46 acute renal f:riJure, 43-~4-4 children, 4,5 chronic renal failure, 44stones, 45--46 urinary tract inflxtion, 4-4-A-S Nephrotic syndrome, 41

Nerve conduction velocity, 88 Neural tube dd<:'ct, 167 NCtlroblaswrnil, 68 Neuroleptic malignant syndrome, ill1tipsychotic drug side dtt:ct, J 14 Neurology, 87~94 Neurosurgery,1(,S·--167 intracranial hemorrhage, 165 jntrJcrJfliaJ hypertension, 166 spinal cord, J 66 Nodule, skin, 79 Nonsteroidal ilnti~infhmmatorydrugs (NSAIDs), toxicity, 188---189 N onstress test (NST) , I 3 I Norepinephrine, intravellOUS therapy ft)}' shock, 16 Nosehl('e(l, canses of, 170 NuB hypothesis, J 10--] J J Obesity, risk iactor in diseases, 4-1--11 Obsessive-cOJnpulsive disorder,J 1 g Obstetrics, 131-146 breast feeding, 135 ICtal evaluation, J32~]35, ]39"140 labor, 137---· 144 laboratory tests in pregnancy, 131--132 IImhiple gestation, 14-6 preeclampsia, 136---137 Oculomotor nerve, 90, 158 Odds ratio (OR), J 08 Olfactory nerve, 90 Oligohydramnios, J 37 Omphalocde, 3'7 Oncology, 59~68 blood dyscrasias, 59 breast cancer, 6L--63 cervical cancer, 6S f'OJorectaJ cancer, 63- ,64 genetic predisposition, 60 lung cancer, 61---61. ovarian canetr, 64--,,6S pancreatic cancer, 64prostate cancer, 63 risk 1;\CIOr5 felf cance]~ 61 screening, American Cancer Society guidelines, J05---106 statistics J()I" cancer, S9 --60 tumor markers, 68 ntrriue: cancer, 65---66 Ophlh;llIllO.lOgy, 15,1)---1,'>9 photo recognition f(n boards, 198

Index

Opioid abuse, 120 Oppositional-defiant disorder, I 19 Optic nerve, 90 Optic neuritis, 1S8 Oral cancer, 68 Oral contraceptives bendhs of: 188 contraindicatJons,187---188 sick eHt'cls, 188 Orbital cdlulilis, 157 Orthopedic surgery, 161-163 Ortolan]'s sign, 1.03 Osgood-Schlatter disease, 163 Osteoarthritis, 41 Osteomyelitis, J 62 Osteosarcoma, 67 Otitis externa, 17 j Otitis media, 17 J OHY.:'>derosb, 171

Ovarian cancer, 64 - 6S Paget's disease, SO Paget's disease of lbe nipple, 86 Pain management, ')6 Pancreatic cancer, 64 Pancreatitis, 35~-3 6, 149 Panic disorder, II 6 Papani.colau smear, 65 Papule, skin, 79 Parkinson's disease, 92--93 Parkinsonism, antipsychotic drug side em'ct, 114 Parotid swelling, 171 Palau syndrolne, 10] Patch, skin, 79 Pediatrics, 181-184 child abuse, I 83 developmental milestones, 181 ethics, \ 9 5--} 96 puberty, 183 ~cr('_ening and ptl·vl·nti\'l~ 111cdkine, 181-183 Pediculosis. See Lin' Pelvic inflammatory disease (PID) , 123 Pelvic relaxation, 128 Pemphigus, 85 Penile anomalies, 177 Peptic ulcer disease, 30, 14-9 Periphcral oemuIMtby, 93--94 PeT":;onaht y disonkrs, 1 1 7 --11 8 Perspiration, excessive, 8S Pertussis, '1 S Ph,1rlnacology, 18S~189 analgesics, 188--] 89 antidotes, 186 drug interactions, 186 hormone replacement: therapy, 187 oral contraceprives, 18/---188 side effects,! 85---186 Phencyclidine (PCP) abuse, 121 Plwnylephrillc, intravenous therapy for shock,16

}llleodwomocytoma, 4-1, 67 Phobia, 16 Photo recognition, 197-202 blood smears, 197 dermatology, 198--199 microscopic findings, 199- -200 miscellaneous photos, 20 I ··202 ophthalmology, 198 radiologic fIndings, 200---201 Photosensitivity, drug reactions in, 84Pituitary tumor, 66 Pityriasis rosea, 84J)laccnta previa, 140 Plaque, skin, 79 Pleur,-.} effusion, 1.8 Pneumococcal inflxtioJl, imJllUnization in adults, J 06 Pneumonia, 26---27, 70---7] Pneumothorax, 151 Polyarteritis llodosa, 49 Polycystic ovarian syndrome (PCOS), 12S Polyhydramnios, 137 Poly myalgia ,hcurnatica, 50 Polymyositis, 49-50 Positive predictive value (PPV), 108 Postpartum fever, ] 44-145 Postpartum hemorrhage, 143 Posttraumatic stress disorder, I 17 Potter's syndrome, J 77 Precocious puberty, 128-129, 183 J)reedampsia, ] 36~·13 7 l}n:gnancy bleeding iuthird trimester, 140-142. diabetes. See Diabel~':s rnellitus drug salt:ty, ] 34ectopic pregnancy, J 39 fetal evaluation, I 32--1 35, 139-- 140 folate supplenwntadon, 13] laboratory tests, 131 ----13 2 multiple gestation, 146 nonnal changes in, J 33, 14-S posttenn pregancy, 133 signs oC \31 surgical conditions, 145 TORCH infh:tions of fetus, 13+~ 135 tubal pregnancy, 139 ultrasound evaluation, 132 viral infections in, J 35 Prehn's sign, 203 Premature nlptnre of nlC_mbrauC"_s (PBOM), 143 Preorbital cdiuhtis,J .5 J Presbyopia, 159 Prevalence, dd\nition of: ] 10 Preventive medicine cancer screening, 1OS·--1 06 Imnlunization in adults, ] 06 pediatrics, 181 -183 Prostate cancer, 63 Pruritus, 80 PseudogoLlt,47 Psoriasis, 48, B3

Psychiatry, 113~121 child psydliatry; 119··-120 disorders, 1 13---118 dnlg abuse, 120----] 21 te<;ting, Il8 Psychogenic fugue, I 17 Puherry onset o( ] 83 'limner stages, 183 Pulmonary embohsm, 20~2] Pulmonology; 25-~28 function tests, 26 nodules, 25---26, 62 Pustuk, skin, SO l)~value, 110--1 II Pyelonephritis, 4_ 1; Rabies, 76 Radiology photo recognition ft)r boards, 200--201 screening or conflnnalory tests, 191--192 Reiter's syndrome, arthritis with, +8 Relative risk (RR) , 108 Reliability, of tests, 109 Renal srones caust's of 46 composition of: 176 signs and synlploHls of. 176 treatment of; 4-5, I 76 Renal transplant indkations hr, 176-~177 rejection, ] 77 Respiratory distress syndrome, 27 Respiratory syncytial virus, 25, 75 Retinal detachnw,nt, 158 Retinoblastoma, 68 Reye's syndrome, 33, 18+ Rhemnatic fi,ver, arthritis with, 4-8 Rhenmatoid arthritis, 47 HheUlnatoJogy, 41·-50 arthritis, 47-·-+8 autoimmune disease, 48-~4-9 Rh incompatibility, 142~- 143 Rhillitis, Lanses of, 170 Rhogam, 14-2-14-3 Risperidone, 114 Rocky Mountain spotted fever, 74Rorschach test, I] 8 Rosacea, 83 Roseola inlillltunl, 73 Rovsing's sign, 203 Huhdla,73,106

Sarcoma bonyoidt'.'i, 66 &:ahies,81 Scarlet fever, 73 SchiL,ophrenia, 113 Sderodenna, 3.5,49 Scoliosis, J 63 Scurvy, S7 Sehorrheic dennatitis, 80

224

Index

Seil,urCS

secondary seizure disorder, 91--92 types of; 91 Sensitivity,] 07",,1 08 SeparatiCm anxiety disorder, I 19 Septic arthritis; +8,162 SerloH cell tumor, 6S Severe cornbined irnmunoddkh"llcy, 97 Shock associated findings with, 1S definiton o( 14treannent of; I S---16 types of: 14---1 5 Shoulder dystocia, 140 Sickle cell anemia, S }_mS4 Side dh:cts antihypertc'nsives, 186 diuretics, 186 tabk of drugs, I8S Sideroblastic anemia, 5<j-

Signs, symptoms, and syndromes, 203-1.0S Sinusitis, 27, 171 Sj6gren syndrome, 4-9 Skewed distribution, 109 Skin cancer, 68 Slipped capital {('woral epiphysis, 163 Small bowf.,j obstruction, 14-9 Sllloking, risk factor fbI' diseases, 6--7, 6 ( Social phobia, 116 Somatof(mtl disorder, 117 SOll1ogyi eff(~ct:, versus dawn phenomenon,

+ Specificity, 1 07-~1 08 Spherocytosis, 54 Spinal accessory nerve, 90 Spinal cord. compression, 166 syringomyelia, 167 tra~lma, 166 Splenic rupture, 148 Squamous cell cancer, skin, 85 Standard deviation (SD), 109 Stanfimj--Binet IQ tcst, 1 j 8 Staphylococcal injection, 77~~78 Status epilcpticus, 92. Stomach cancer, 67 Stomach ulcer, 30, 149 StOluatHis, 86 Strabismus, j S9 Streptococcal infix:tion, 76- 77 Stroke cerebrovascular dis(~ase overview, 92 intracranial hemorrhage typ~'s, 165 Study design, 109--- I I () Stye, i57 Subarachnoid hemorrhage, 165

Subclavian steaL syndrome, j 74 Subdural hematoma, 165 Sudden deafltess, 170 Suicide, 11 S Surgery ear, nOSe, and throat surgery, 169 -171 Surgery (tollt.) general surgery, 147~153 neurosurgery, 16_5--167 orthofwdic surgery, t61~163 vascular surgery, 173~-174 SWinuner's ear, 171 Syncope, 88 Syndrome ofinapproriate secretion of antidiuretic hormone (SIADH), 9,41 Syphilis, 72 Syringomyelia, 167 Systemic lupus erythematosus, 4-8- +9 'Takayasu's arteritis, 49 l~rdive dyskinesia, antipsychotic drug side efkcl, J 14 Tension pneumothorax, 15 1 Teratogenic agents, types and defects, IH-I34 "Itratoma, 65 ll'slicular cancer, 66, 17.5 T<'sticuJar torsion,! 7.5 Tetanus, irnmunil,ation in adults, 106 Thalassemia,S:) Thematic Appercepcion Ib(, J 18 Thrombotic thro1l1bocytopeUic purpura

(TTl') , 45 Thrush, S 1 Thyroid cancer, 67 Tinea, 80--~82

'find's sign, 204Tboth avulsion, 1S3 TORCH infi.'ction {(,(US, J 34-'" J 35 hearing screening in children, 187. roureue's disorder, 119~--120 'lhxic rneg,lcolon, 33 Tfdchcoesophageal fistula, 2.7 Transf'usion indications f(ll~ 55 transfusion reaction, 55-- S6 Transient ischemic attack (TIA), 92 Transurethral resection of the prostate (TU 1<1'), 17 5 Trauma, general surgery, 1SO----1 53 Tremor, 93 T'rigeminalnerve, 90 Trochlear nerve, 90, 1.59 'll"()(l!;se,w's sign, 20£j'f.-test, 110

Tubal pregnancy, 139 'I'uherculosis screening in children, 183 trealrnco( in pregnancy, 136 Tumor markers, 68 Turner syndrome, 101 Ulcerative colitis, :n~-33 Urethral injury, 176 Urinary fmC( infection (U'n), 4-4---4-5 Urology, 175--177 Uterine alOny, J4-3 Uterine cancer, 125 postmenopausal bleeding with, 6S -66 risk factors, 66 IT(\1trncnt, 66 Uterine inversion, 144Uterine nlpture, 14-1 Uveitis, 156 Vaginal in!(_'ction, 123-- ] 24Vaginal fnolapsc, 128 Vagus lwrve, 90 Validity, of tests, 109 Valvular bean discJ..)e endocarditis prophylaxis, 19, 74 lllUnnur characteristics;, 19 Var1c(K'ele, 176 Vascular surgery, 173~174 Venous insufficiency, j 74Vertigo, 170

Vesicle, skin, 80 Vestibulocochkar nerve, 90 Virchow's triad, 204Vitamic C deHeieney. See Scurvy Vitamin B12. deficiency, anemia with, 53 Vitamin J) ,mpplClnentatioJl, J 83 Vitamins, dehciency and toxicity, 13--14, 9j Vitihgo,80 Warts, 82 Wegener's granulomatosis, 4-9 Werdnig--IlofhlliIHll disea»e, 93 Wernicke's syndrome, 7 Wechsler Intelligence Scale for Childrell, 118

Wheal, skin, 79 Wilms' tumor, 68 Wilson's disease, 34 Wiskott-Aldrich deficiency, 97 Word a,';.,,;ociatic)J]s, 204----205 X--linked agammaglohulinemia, 97 Zoster, 73