CK Flashcards

Question

``` [Endo] Hirsutism - clinical presentation, diagnosis, and treatment of: CAH Sertoli-Leydig tumor Aromatase deficiency vs 5AR deficiency ```

Answer 1

Congenital adrenal hyperplasia (CAH) - MCC is 21OH deficiency --> Decreased cortisol, aldosterone but increased sex hormones A. Clinical - - at birth - hypotension, ambiguous genitalia and salt wasting - at puberty - precocious puberty in men and virilization in women B. Diagnosis - elevated morning fasting 17hydroxyprogesterone, increased renin / low BP, high K+ C. Tx - replace cortisol, aldosterone; antiandrogens (spironolactone, OCPs) *17alphaOH deficiency has HTN with high aldosterone, decreased cortisol and sex hormones *11betaOH deficiency has HTN with low aldosterone and cortisol, increased sex hormones (HTN bc there is still a mineralocorticoid being produced) Sertoli-Leydig tumor - androgen-secreting ovarian tumor A. Clinical - rapid onset hirsutism, virilization, adnexal mass B. Diagnosis - elevated testosterone level C. Tx - surgical *estrogen counterpart is granulosa cell tumor --> precious puberty in girls, AUB in women (high estradiol and inhibin) Aromatase deficiency - cannot convert androgens to estrogens A. Clinical - masculinization of XX infants --> normal internal genitalia but ambiguous external genitalia, external virilization (clitoromegaly) - osteoporosis, delayed puberty - maternal virilization during pregnancy B. Diagnosis - high levels of FSH, LH, testosterone, androstenedione but undetectable estrogen levels C. 5alphareductase deficiency (AR) - cannot convert testosterone to DHT --> feminization of XY infants until virilization at puberty; increased testosterone, LH, estrogen

Answer 2

A. HSV - genital is HSV2 - primary episode is systemic (fever, malaise) and local, vs just local (syphilis) - PAINFUL burning and paresthesias, then small, superficial groups of painful ulcers on a red base w mild tender LAD - do viral culture (multinucleated giant cells) or PCR - tx - oral acyclovir B. Chancroid - due to H. ducreyi - papules --> pustules --> erodes into multiple PAINFUL deep ulcers with ragged edges on necrotic, friable base with gray yellow exudate + severe / tender lymphadenopathy that suppurates - school of fish on Gram stain - tx - oral azithromycin or IM ceftriaxone C. LV - C. trachomatis L1-L3 - primary - PAINLESS papule or small shallow ulcers - secondary - unilaterally painful inguinal LAD --> buboes (enlarged tender nodes) and groove sign (separation of lymph nodes by inguinal ligament) - tertiary - fibrosis/strictures of anogenital tract - tx - doxy or erythromycin D. Granuloma inguinale - Klebsiella granulomatis (Gram -) - Donovan intracellular inclusion bodies in macrophages - extensive PAINLESS beefy red ulcers w/out LAD and base of granulation tissue - seen in India, Guyana, New Guinea - tx - doxy or TMP-SMX E. Syphilis - primary is single PAINLESS ulcer (chancre) with regular borders and hard clean base (indurated) with no systemic sx, mild b/l inguinal LAD

Answer 3

Developmental milestones: 2 months - sustains head, social smile, recognizes parents, coos, follows object past midline 4 months - sits with trunk support, hands to midline, laughs, looks around and turns to voice 6 months - rolls over, crawl, sit with support, stranger anxiety, transfer objects hand to hand 9 months - sits unsupported, pulls to stand / cruises, 3 finger pincer grasp, holds cup, says dada and mama, waves bye 12 (1 year) - stands, throws ball, 2 finger pincer grasp, says words other than mama/dada, comes when called *weight triples, height increases 50% 15 - walk alone, 3 cube tower, scribble w crayon 24 (2 years) - walks up stairs with both feet, 1/2 speech comprehensible, 2 word sentences, holds spoon, 7 cube tower, parallel play, follows 2 step commands 36 (3 years) - walks up stairs with alternating feet, rides tricycle, copies circle, 3 word sentences, 3/4 speech intelligible, knows age and sex, counts to three 48 (4 years) - hops on 1 foot, copies cross, throws ball, goes to toilet alone, group play, speech 100% intelligible 60 (5 years) - skips, copies square, dresses independently, 5 word sentences, counts to 10

Answer 4

FHR decels - do nonstress test (nl = 2+ acc in 40 min) if decreased perception fetal movements --> high NPV to r/o fetal acidemia 1. Early - symmetric - starts when ctx begins and recovers when ends and nadir corresponds to peak of ctx --> physiological, benign (2/2 vagal tone from fetal head compression) 2. Late - begin at peak of ctx --> due to fetal hypoxia (uteroplacental insufficiency 2/2 chronic HTN, postterm) - recurrent late --> due to fetal acidemia - sinusoidal --> severe fetal anemia, or hemorrhage; do umbilical cord sampling to check HCt - move mom to LLD, give 02 and IVF, d/c pitocin - C-section if needed 3. Variable - abrupt in decline and resolution (<30 sec from onset to nadir) --> due to umbilical cord compression, cord prolapse, or oligohydramnios - if persistent (with >50% ctx) --> maternal repositioning to LLD, then amnioinfusion for repetitive variable decels to decrease risk C-section

Answer 5

RTA - normal AG met acidosis with low HCO3, high Cl, and high K out of proportion to renal dysfunction Type 1 (distal) - poor H+ secretion into urine - pH >5.5 - a/w kidney stones, sickle cell trait - causes - congenital, amphotericin B Type 2 (proximal) - poor HC03- reabsorption - causes - Fanconi syndrome (lots of things not reabsorbed), acetazolamide - pH <5.5 - leads to osteomalacia (defective mineralization 2/2 phosphate wasting --> 2 hyperPTH) Type 4 - aldosterone resistance - causes - ACEI/ARB, NSAIDs, heparin/cyclosporine, Addison / congenital adrenal hyperplasia, obstructive uropathy - hyperchloremic, hyperkalemic metabolic acidosis --- Renal tubular defects 1. Fanconi syndrome - generalized reabsorptive deficit in PCT 2. Bartter - TAL of Henle, like chronic loop use --> met alk with hypokalemia and hypercalciuria 3. Gitelman - DCT, like chronic thiazide use --> met alk with hypokalemia and hypocalciuria 4. Liddle - collecting tubules, presents like rddo with HTN, met alk with hypokalemia; tx - aldosterone

Answer 6

Hyperaldosteronism - suspect with HTN, easily provoked hypokalemia (eg falls/leg cramps after starting thiazide), mild hypernatremia (bc of aldosterone escape) vs renal artery stenosis - suspect with severe HTN after 55 yo, recurrent flash edema or resistant HF, unexplained rise in Cr, and abdominal bruit; dx - captopril renal scan measure PAC (plasma aldosterone concentration) / PRA (plasma renin activity) ratio: - PAC/PRA >20 and high PAC >15 --> primary hyperaldo --> adrenal suppression testing --> (+) then adrenal CT to determine cause --> if no discrete adrenal mass, then adrenal vein sampling to differentiate hyperplasia (medical tx - spironolactone, eplerenone) vs adenoma (surgery) - PAC/PRA ~10 and high PAC and PRA --> secondary hyperaldo eg diuretics, CHF, renin-secreting tumor, malignant HTN, aortic coarct, renal artery stenosis, fibromuscular dysplasia (do CT A/P) - low PRA and low PAC --> CAH, glucocorticoid resistance, Cushing (do dexamethasone suppression test), Liddle syndrome

Answer 7

Spontaneous bacterial peritonitis - bacterial infection of ascitic fluid without obvious source 1. Causes - E. coli, Klebsiella MC due to intestinal bacterial translocation 2. Clinical - fever >100F, diffuse abd pain, AMS (abnormal connect-the-numbers test) - can be hypotensive, hypothermic, paralytic ileus - occurs in patients with cirrhosis, CKD on peritoneal dialysis 3. Diagnosis - paracentesis to do cell count, cytology, Gram stain and culture - shows PMN >250, positive culture, protein < 1 (seen in cirrhosis, nephrotic syndrome), SAAG >1.1 (indicates portal HTN --> increased capillary hydrostatic pressure) 4. Tx - 3rd gen cef eg cefotaxime or ceftriaxone; fluoroquinolones eg cipro for prophylaxis - d/c BBs after SBP (increased rate mortality) - no protein free diets (increase mortality, only recommended post TIPS) * SAAG <1.1 indicates increased capillary permeability eg TB, peritoneal carcinomatosis, pancreatitis, malignancy * protein >2.5 indicates CHF, peritoneal carcinomatosis, TB, fungal eg coccidio, Budd-Chiari

Answer 8

TB presents with subacute sx - f/c, weight loss, fatigue, night sweats, cough / hemoptysis - CXR - apical cavitary lesion - skeletal - spondylitis (back pain, vertebral fractures), arthritis, osteomyelitis - consider with coming from developing country, homeless, or immunosupressed (CKD, DM2, HIV, steroid use) >5 mm - HIV (+), recent contacts of known active TB, prev healed TB signs on CXR eg abnl calcifications, immunosuppressed eg transplant, glucocorticoids >10 mm - recent immigrants in 5 yrs from endemic areas, IVDA, high-risk settings eg jails nursing homes hospitals, high risk reactivation eg DM2, ESRD, leukemia or other heme malignancies, kids <4 yo >15 mm - all other people Tx latent TB - INH alone for 6-9 mos or rifampin for 4 mos *Add pyridoxine if DM2, uremia, ETOH, malnutrition, HIV, pregnant, or epilepsy Tx pulm TB - RIPE (rifampin isoniazid pyrazinamide ethambutol) for 2 mos, then INH + rifampin for 4 mos

Answer 9

1. ADPKD A. Clinical - usually asx but hematuria / proteinuria, b/l palpable abdominal masses, resistant HTN, flank pain 2/2 stones cyst rupture or UTI - extrarenal - berry aneurysms, hepatic cysts (most common), MVP/AR, diverticulosis, ventral/inguinal hernias B. Dx - US C. Mgmt - ACEI/ARBs, close follow-up, dialysis or transplant if ESRD (MCC death is cardiovascular dz) 2. ARPKD A. Clinical - cystic dilation of collecting ducts, can present in utero with Potter syndrome or in infancy with HTN, progressive CKD, portal HTN 2/2 congenital hepatic fibrosis 3. Wilms tumor - MC renal neoplasm of childhood A. Clinical - asymptomatic, firm smooth abdominal mass that does not cross midline + HTN + hematuria (classic triad) - Beckwith Wiedemann syndrome - Wilms, macroglossia, omphalocele, hyperinsulinism (hypoglycemia --> seizures), hemihypertrophy - WAGR - wilms, aniridia, GU probs, and retardation B. Dx - US then CT to look for pulm mets C. Mgmt - surgery + chemo +/- radiation D. Neuroblastoma - MC cancer in 1st year of life, arises from neural crest cells commonly in adrenals or as posterior mediastinal mass - calcified abdominal mass that crosses midline with systemic symptoms (opsoclonus-myoclonus syndrome) - raccoon eyes (periorbital mets)

Answer 10

1. Infective endocarditis causes and associations: - RFs - MVP then aortic stenosis, catheters, IVDU (tricuspid regurg) - clinical - fever/chills, pleuritic CP, dyspnea, cough + FROMJANE (fever, roth spots, osler nodes, murmur, janeway lesions, anemia, nail bed hemorrhage, emboli) - if there are conduction abnormalities on EKG eg AV block or syncope --> think perivalvular abscess! - glomerulonephritis, RF (+), high ESR - Tx - vanco for empiric coverage; do NOT give oral abx 2. Causes and associations - Strep bovis -- colon ca --> do colonoscopy - Strep viridans -- dental or URI --> ceftriaxone or IV penicillin G; also Eikenella (anaerobe in normal oral flora) - Staph epidermidis - prosthetic valves or implants eg ICD, catheters --> vanco - Staph aureus - above + IVDU; Staph is MCC of healthcare-associated IE --> cefazolin if sensitive, vanc for MRSA - Enterococci - UTIs --> amp + gent - fungi eg Candida - immunocompromised --> amphotericin - culture negative (HACEK, MCC Coxiella) --> ceftriaxone 3. Ppx - prosthetic heart valve, prev hx IE, unrepaired cyanotic CHD, repaired CHD w residual defects - dental -- ammo - GI/GU -- amp - sx on infected skin/muscle or prosthetic heart -- vanc

Answer 11

false labor - irregular ctx w no cervical change 1st stage - onset until cervix dilated 10 cm A. latent - onset until 6 cm - give oxytocin if long (hypotension, hyponatremia, tachysystole) B. active - 6 to 10 cm - if cervical change slow --> oxytocin - arrest if no cervical change for >4 hrs w/ ctx (200+ MVU in 10 min) or >6 hrs w/out ctx --> C-section 2nd stage - 10 cm until delivery - arrest if >3 hrs in nulli or >2 hrs in multip - MCC is malposition (nl is occiput anterior), other causes maternal exhaustion, cephalopelvic disproportion, inadequate contractions * presentation = vertex, breech, etc 3rd stage - delivery until placenta - arrest if >30 min Vaginal delivery c/i in: 1) prior classical C-section 2) abdominal myomectomy for fibroids with uterine cavity entry 3) prodromal sx or active herpes lesions 4) HIV >1000 5) breech presentation Prenatal testing - First trimester combined (9-13 weeks) - PAPP, bHCG, nuchal translucency; screening - cell free fetal DNA (10+ weeks) - screening - Chorionic villus sampling (10-13) - definitive karyotype - 2nd trimester quad screen (15-22) - AFP, bHCG, estriol, inhibin *decreased AFP, increased bHCG inhibin = Down - amniocentesis (15-20) - definitive karyotype

Answer 12

Thyroid lymphoma - rapidly enlarging, firm, tender goiter with compressive sx (hoarseness) and B sx - Pemberton sign - raising arm compresses veins bw enlarged thyroid due to retrosternal extension --> JVD and plethora - increased incidence with co-existing Hashimoto thyroiditis (pos antithyroid peroxidase Ab) Subacute (de Quervain) thyroiditis - follows acute viral syndrome with fever, neck pain, tender + diffuse goiter - thyrotoxic due to release of preformed thyroid hormone - low RAIU but high thyroglobulin (Vs exogenous thyroid hormone, which has low RAIU and low thyroglobulin) Thyroid nodule - do TSH and US - if suspicious or cancer RFs --> FNA - normal or elevated TSH --> FNA - low TSH --> RAIU scintigraphy - -> hot nodule is hyperthyroid --> diffuse uptake is Graves, nodular is adenoma or MNG - -> cold nodule needs FNA

Answer 13

Clean wound I. 3+ tetanus doses - Tdap only if last dose was >10 years ago II. <3 doses or uncertain - Tdap Dirty wound I. 3+ doses - Tdap only if last dose was >5 years ago II. <3 doses or uncertain - Tdap AND TIG

Answer 14

Pericarditis 1. Causes - idiopathic, viral (MCC eg Coxsackie) - connective tissue eg RA, SLE - cardiac eg Dressler (1-6 weeks post MI) - uremic - @ BUN >60 - malignancy - due to cancer (lung, breast, lymphoma) or treatment (chemo, radiation) 2. Presentation - pleuritic CP relieved by leaning forward, friction rub, tachycardia / dyspnea - EKG changes: diffuse ST segment elevation and PR depression (first 2 weeks) --> normalization of ST and T wave flattening --> inverted T waves --> return to normal * viral pleurisy also has friction rub but it disappears on breath holding 3. Dx - echo. Tx: - idiopathic or viral - NSAIDs and/or colchicine - uremic - hemodialysis *classic EKG changes not seen 4. Constrictive pericarditis A. Causes - post cardiac surgery or radiation, TB B. Clinical - signs of RHF (peripheral edema, ascites, clear lung fields), Kussmaul sign (increase instead of decrease in JVP on inspiration), pericardial mid-diastolic knock, hepatojugular reflex (sustained elevation of JVP >3 cm during liver compression 2/2 failing RV that cannot accommodate increase in venous return; how to differentiate cardiac vs liver cause of peripheral edema) C. Dx - calcification and fibrosis on CXR, JVP shows prominent x and y descents --- II. Myocarditis 1. Causes - viral eg Coxsackie B, adeno 2. Presentation - viral prodrome --> respiratory distress (tachypnea, dyspnea) from LH failure, mitral regurg murmur, hepatomegaly 2/2 RH failure - CXR - cardiomegaly (Eccentric), pulm edema - echo - global hypokinesis, decreased EF - biopsy (gold standard) - inflammation and necrosis 3. Tx - supportive (diuretics, ACEI/BB) + IVIG

Answer 15

1. RV failure - acute, RCA, hypotension + clear lungs + hypokinetic RV * also see vfib acutely (MCC cardiac arrest in acute MI 2/2 reentry) 2. Papillary muscle rupture - acute to 5 days, RCA, pulm edema + new holosystolic murmur at apex from mitral regurg with flail leaflet 3. IV septal rupture - acute to 5 days, LAD or RCA, CP cardiogenic shock (pulm edema hypotension) + VSD -> new holosystolic murmur at left sternal border with palpable thrill; echo shows L to R shunt or equalization of pressures 4. LV Free wall rupture - 5 days to 2 weeks, LAD (anterior>>inferior MI), CP SOB Beck's triad + pericardial effusion with tamponade - suspect with PEA after 1st MI and no signs HF 5. LV aneurysm - up to several months, LAD, stable angina + HF, echo shows thin + dyskinetic LV wall; EKG shows persistent STE and deep q waves; risk of mural thrombus 6. Dressler syndrome - autoimmune fibrinous pericarditis weeks - months with persistent fever, friction rub, pleuritic CP; tx - NSAIDs, colchicine

Answer 16

I. HLD - screening nonfasting total and HDL cholesterol - kids at 9-11 and 17-21 yo - screen M > 35 yo or F > 45 yo or either M or F > 20 yo with risk factors (smokers, DM, obesity, FHx, HTN) q5yrs - if >240 or 200+ with risk factors - get fasting lipid panel II. Statin guidelines 1. ASCVD (ACS, s/p CABG, angina, stroke/TIA/PAD) - LDL >75 - high intensity eg atorva 40-80 or rosuva 20-40 - LDL <75 - moderate intensity eg atorva 10-20, rosuva 5-10, simva 20-40 2. LDL 190+ --> high intensity 3. age 40-75 with DM2 - ASCVD risk 7.5%+ - high intensity - ASCVD risk <7.5% - moderate 4. estimated 10-year ASCVD risk 7.5%+ and LDL 70-189--> moderate III. Medications: A. statins - inhibit HMG CoA reductase, lower LDL, reduce mortality; monitor LFTs, can cause myopathy and increase in CPK; teratogen B. Niacin - best at increasing HDL, moderately lowers TGs; do not use in diabetics bc worsens glycemic control; causes flushing (take NSAIDs) and pruritus 2/2 hyperuricemia, check LFTs and CPK C. Cholestyramine - bile-acid binding resin (liver must use cholesterol to make more); use w statins or niacin to lower LDL - does increase TGs and causes constipation and bloating, decreased absorption of ADEK D. Gemfibrozil - fenofibrate, lowers TGs in severe by changing hepatic metabolism of lipoprotein (increasing LPL) BUT statins preferred for mildly high TG levels - can cause cholesterol gallstones, myopathy --> do not use w statins E. Ezetimibe - inhibits absorption of cholesterol in small intestine; 2nd line for decrease in LDL and can be used with statins; can cause increase LFTs and diarrhea

Answer 17

1. Nipple discharge - Unilateral, bloody, or a/w skin changes (mass, LAD) --> pathologic --> do cytology and US (if <30) or mammo (if 30+) * intraductal papilloma MCC of nipple discharge, ~2x increased risk of cancer - b/l, nonbloody, no skin changes --> likely physiologic --> UPT, serum prolactin, TSH * hyperprolactinemia MCC, consider MRI brain to r/o pituitary mass; other causes hypothyroidism, pregnancy, meds eg antipsychotics, cimetidine 2. PPH >500 mL post vaginal >1000mL post C-section - MCC is uterine atony, then retained placenta or tract lacerations - palpate uterine fundus --> if boggy --> oxytocin and bimanual massage - uterotonic agents --> rectal misoprostol / Cytotec, ergot alkaloid eg Methergine (c/i in HTN), prostaglandin F2ef eg carboprost / Hemabate (c/i in asthma) 3. Chorioamnionitis --> maternal fever and 1+: fetal (>160) or maternal tachycardia, maternal WBC, uterine fundal tenderness, and/or malodorous vaginal discharge - RF - PROM - baby can be septic (pale, lethargic, high temp) - tx - IV amp and gent and induce labor regardless of gestational age (doesn't need C-section); if ROM >18 hrs --> give amp - vs endometritis (fever 2-3 days postpartum, uterine tenderness, foul smelling lochia) - tx clinda/gent

Answer 18

1. Solitary pulm nodule - <3cm, round, no adenopathy or atelectasis - first compare to previous X-rays --> if stable 2-3 years --> observe - otherwise, CTchest: - -- benign features eg smooth, central calcification, <30 nonsmoker --> serial CTscans - -- suspicious eg scalloped, eccentric calcification, large >2 cm, and RFs incl >40 yo, smoking hx, B sx --> biopsy or PET - -- high suspicion eg scalloped, large and RFs incl age >60, smoker --> skip biopsy and do surgical excision * MC is adenocarcinoma, squamous cell more likely spiculated, squamous and small cell are centrally located usually 2. Increased A-a gradient (A-a normal with high altitude, hypoventilation eg opiate OD) - intracardiac shunt eg cyanotic HD - does not correct with 02 - intrapulmonary shunt (V/Q=0) eg PNA, atelectasis - hypoxemia does not correct with 02 - dead space ventilation (V/Q = infinity) eg PE - does correct with 02 - diffusion limitation eg COPD - does correct with 02

Answer 19

Erysipelas - MCC is GABHS infection of superficial dermis and lymphatics --> raised demarcated margins of red, indurated, shiny plaque with rapid spread and onset; a/w fevers, regional LAD - tx - oral dixloc, cephalixen, clinda; if fever --> IV ceftri cefazolin or clinda - Strep skin infections can cause PSGN (but not ARF) Cellulitis - MCC is Staph, then strep; infection of deep dermis and subQ fat --> flat edges with poor demarcation and indolent, localized; if purulent, think MRSA Furuncle - MCC is S aureus; folliculitis ---> dermis --> abscess; carbuncle = multiple furuncles Impetigo - GABHS and S aureus, most superficial skin infection; non-itchy pustules and honey-crusted lesions; tx - mupirocin, oral cephalexin if severe, can be c/b PSGN

Answer 20

PTH --> Vit D (Activated in kidney) --> - increased Ca and P04 reabsorption from bone and intestines - increased Ca and decreased P04 (more excretion) from distal renal tubules 1. Hypocalcemia - prolonged QT, cramps and tetany - first confirm low Ca with repeat, can be due to low albumin (Corrected Ca = serum +0.8(4-serum albumin), low Mg (induces resistance to PTH), alkalosis - then measure PTH: - -- high PTH --> Vit D deficiency, CKD (secondary hyperPTH), sepsis, pancreatitis, resistance eg pseudo - Albright hereditary osteo * tumor lysis syndrome - low Ca high K uric acid and P04 - -- Nl to low PTH --> iatrogenic/postop (MC!), Wilson / hemochromatosis, metastatic cancer (eg prostate -Ca2+ released from osteoclast activity inhibits PTH) * chronic hypoPTH complications - nephrocalcinosis, cataracts, EPS 2/2 deposition of Ca2+ in basal ganglia 2. Hypercalcemia - shortened QT, stones (and polyuria) bones groans (constipation) psychiatric overtones (confusion, depression, psychosis) - also do repeat measurement - then measure PTH: - -- high PTH --> 24hr urinary calcium - -------> high - primary hyperparathyroid (high Ca low P04); resect if <50 yo or symptomatic - -------> low - familial hypocaliuric hypercalcemia - --- low PTH --> measure PTHrP (malignancy eg squamous cell lung ca, RCC and bladder ca, breast ovarian and endometrial ca), calcidiol (excessive intake eg milk alkali syndrome), calcitriol (ectopic production), consider osteolytic mets eg breast, MM, NHL, NSCLC tx - if >14 or symptomatic - IVF + calcitonin 3. renal osteodystrophy (secondary hyperPTH) CKD and low GFR --> decreased production of 1,25 dihydroxyVit D --> decreased intestinal absorption of Ca2+ and excretion of P04 --> stimulates PTH synthesis and parathyroid hyperplasia --> increased bone resorption --> osteitis fibrosa cystica (bone pain, fractureres); PTH resistance can also lead to tertiary hyperPTH with autonomous adenoma (high PTH and high Ca2+) - mgmt - low phosphate diet, phosphate binders (calcium carbonate, sevalamer), supplement Ca and Vit D

Answer 21

1. Myasthenia gravis - autoAb from thymus block or destroy postsynaptic nicotinic ACh receptors at NMJ --> impaired action potential propagation - ptosis, diplopia, fatigable chewing dysphagia, myasthenic crisis is respiratory muscle weakness (plasmapheresis) - triggers - fluoroquinolones, aminoglycosides, NMJ agents, BBs, procainamide, mag, pregnancy, surgery, infection - positive tensilon test and ice pack test - tx with pyridostigmine or immunotherapy (cyclosporine, corticosteroids) - a/w thymoma - do CTchest 2. Lambert Eaton - autoAb against presynaptic Ca2+ channels at NMJ --> decreased ACh release - slowly progressive prox muscle weakness, decreased DTRs, autonomic sx (dry mouth, impotence) that improve with use - a/w SCLC (also SIADH and Cushing) 3. Sjogren syndrome - anti-Ro, anti-La Ab, (+) ANA --> lymphocytic infiltrate of exocrine glands --> Dry eyes and dry mouth --> cavities, parotid enlargement, dyspareunia 2/2 vaginal dryness; chronic urticaria, vasculitis - affects F 40-60 yo, if onset after 60 - think age-related atrophy - a/w other autoimmune eg scleroderma, RA, SLE - risk of developing NHL

Answer 22

1. Bronchiectasis - infectious insult with impaired bacterial clearance --> cycles of infection inflammation and damage --> permanent anatomic abnormality - MCC is CF, also due to rheum eg RA Sjogren, hypogammaglobulinemia, congenital eg antitrypsin, cancer, mycobacteria, allergic broncho aspergillosis (eosinophilia, needs oral prednisone and itraconazole) - chronic cough with copious daily sputum production +/- hemoptysis and fatigue, weight loss, dyspnea - not a/w smoking - FVC <80% predicted - exacerbations are bacterial and require abx - best initial test is CXR, most accurate is CTchest (not PFTs unlike COPD) --> shows bronchial thickening and dilation 2. Chronic bronchitis - "blue bloater" - productive cough (not as much sputum as bronchiectasis) with dyspnea, cyanosis - exacerbations are viral - clinical dx - cough 3+ months over 2 years, obstructive pattern on PFTs -- high TLC, normal FVC but FEV1/FVC <70% and normal DLCO - CXR - prominent bronchovascular markings and flattened diaphragm 3. Emphysema - "pink puffer" - centriacinar airway destruction 2/2 smoking, panacinar 2/2 alpha1 antitrypsin - CXR - decreased vascular marking, hyperinflated lungs - high TLC, FEV1/FVC <70%, low DLCO

Answer 23

4. COPD exacerbation - only things proven to improve mortality: smoking cessation, home oxygen if Sa02<88% or p02<55 -- <89% or p02<59 IF cor pulmonale, RHF, or HCt >55% - goal Sp02 88-92%, inhaled bronchodilators (B2 agonist + muscarinic antagonist), systemic glucocorticoids - MCC viral but add abx eg azithro, levo, augmentin if 2+ cardinal sx (increased dyspnea, cough, or sputum production) OR mechanical ventilation required (NPPV or intubation eg if pH<7.1) - can lead to multifocal atach, too much 02 --> C02 retention 2/2 loss of vasoconstriction (worsens V/Q mismatch), Haldane effect, decreased RR (decreases minute ventilation) --> C02 narcosis at >60 with AMS, seizures 5. COPD vs asthma - both obstructive lung diseases (FEV1/FVC < 70%), increased TLC 2/2 hyperinflation - Diffusion capacity of CO (DLCO) normal or increased in asthma, but never increased in COPD (normal in bronchitis, decreased in emphysema) - only asthma shows complete reversibility (>12% or >200mL increase in FEV1) in PFTS with bronchodilators - asthma best first test: PEF or ABG, COPD best first test: CXR; most accurate diagnostic test for both = PFTs * COPD MCC cor pulmonale 2/2 pulm HTN (elevated pulmonary artery systolic pressure >25 2/2 constriction; loud S2 on PE) 6. Restrictive lung diseases - poor muscular effort (MG GBS) structure (scoliosis ankylosing spondylitis obesity) - interstitial dz with low TLC RV and low DLCO and increased A-a gradient and loud P2 (pulm HTN) --> idiopathic fibrosis, asbestosis, silicosis, sarcoidosis, Goodpasture / Wegener, drugs (bleomycin mtx amiodarone), SLE scleroderma, infection (TB) - FEV1 and FVC both decrease --> FEV1/FVC >70%

Answer 24

Syphilis 1. Primary - painless genital ulcer (chancre) with mild b/l painless inguinal LAD 2. Secondary - diffuse maculopapular rash that starts on trunk and involves palms/soles + widespread LAD (Esp epitrochlear on elbow) + condyloma lata (raised gray-white papules on mucous membranes (genitals, mouth) 3. Tertiary - neurosyphilis (eg dementia), tabes dorsalis (sensory broad-based ataxia with impaired proprioception --> (+) Romberg and absence of DTRs), aortitis (vaso vasorum destruction), gummas, Argyll Robertson pupil 4. Congenital - Hutchinson teeth, mulberry molars, snuffles, saber shins, saddle nose, CN VIII deafness Dx - darkfield microscopy for primary, RPR / VRDL followed by FTA-ABS *cannot be cultured* Tx - penicillin G benzathiene - IM in primary and secondary, IV in tertiary - if severe PCN allergy, give doxy - need 4x decrease in titers at 6-12 months

Answer 25

1A. Multiple Myeloma - MC primary bone tumor in >40 yo - M spike is IgA or IgG on SPEP - CRAB - hyperCalcemia, Renal (AKI, Bence Jones Ig light chains in urine), Anemia, Bone pain / osteolytic lesions - also hyperuricemia - a/w amyloidosis, can be preceded by MGUS (Asx) - Rouleaux formation on smear; increased beta 2 microglobulin (increased cell turnover) - most accurate test is bone marrow biopsy - >10% clonal plasma cells - susceptible to encapsulated organisms eg Strep pneumo, H flu, Neisseria - tx - dexamethasone, lenalidomide, bortezomib, mephalan if >70; if <70 most effective is BMT B. Waldenstrom - M spike is IgM on SPEP - hyperviscosity syndrome (tinnitus, diplopia), neuropathy, mucosal bleeding, Raynaud - HSM + LAD + anemia + thrombocytopenia - also see Rouleaux formation on smear - >10% clonal B cells on bone marrow bx - tx - urgent plasmapheresis to remove IgM; rituximab, prednisone 2. Myeloproliferative - due to Jak2 mutation A. Polycythemia vera - all 3 blood lines elevated and low EPO - aquagenic pruritus, HTN splenomegaly and thrombosis 2/2 hyperviscosity, erythromelalgia (painful red hands - tx= aspirin); a/w gout B. Essential thrombocytosis - platelets > 1M --> thrombosis AND bleeding; tx - hydroxyurea C. Myelofibrosis - splenomegaly, teardrop cells; tx - thalidomide

Answer 26

1. Factor V Leiden -(AD) - MC esp in whites - Factor V participates fine in coag cascade but cannot be turned off by activated protein C --> continued thrombin formation - PT and apTT normal 2. Prothrombin mutation (AD) - 2nd MC - increased prothrombin biosynthesis and levels - PT and apTT normal 3. Antithrombin deficiency -acquired, not inherited 2/2 DIC cirrhosis nephrotic syndrome 4A. Protein C or S - decreased inactivation of factors V and VIII - with C, see warfarin-induced skin necrosis B. HIT (autoimmune) - causes skin necrosis at injection sites, thrombosis, or anaphylactoid rxn, or platelet count down >50% with 5+ days on heparin - d/c heparin and start argatroban or fondaparinaux - gold standard dx - serotonin release assay 5. Antiphospholipid syndrome - lupus anticoagulant, anticardiolipin ab - cause venous and arterial thrombi, recurrent abortions (2+ in 1st tri or 1 in 2nd tri) --> give heparin - prolonged apTT but normal PT/INR, fails to correct on mixing study (shows deficiency is not in clotting factor) - russel viper venom most specific for lupus anticoagulant

Answer 27

1. IDA - MCC microcytic anemia in kids, can be due to excessive cows milk intake >24oz daily - low MCH, high RDW --> anisocytosis and hypochromia - low MCV, low RBCs --> Mentzer index (MCV/RBC) >13 - low retics (not enough iron to make new RBCs) - normal Hb electrophoresis - decreased iron ferritin and transferrin saturation (iron/TIBC) and increased TIBC 2. Thalassemias - low MCV, normal RDW and high RBCs --> Mentzer index <13 - Normal iron, low TIBC, high ferritin, high transferrin sat (iron / TIBC) - normal to high retics - alpha thal minor - normal Hb electrophoresis, 3 alleles deleted shows HbH - beta thal minor - increased HbA2 on Hb electrophoresis - smear shows target cells 3. Anemia of chronic disease - normal MCV - low iron, low TIBC, high ferritin (bc acute phase reactant), normal transferrin sat - tx - EPO 4. Sideroblastic anemia - causes - ETOH, B6 deficiency eg INH, copper deficiency, myelodysplastic syndromes - HIGH iron, normal/low TIBC, high ferritin - smear shows basophilic stipping due to iron; bone marrow has ringed sideroblast (iron laden mitochondria) - tx - pyridoxine B6

Answer 28

1. HIV A. Testing - HIV screening (Ab and p24 antigen) for 15-65 once, yearly if IVDU or MSM, and when pregnant <18 months - HIV DNA PCR testing (cant do ELISA bc of transfer of maternal Ab) >18 months - ELISA to screen for HIV Ab, Western blot to confirm Disease status - HIV viral load, CD4 count, clinical findings B. Presentation - recurrent infections eg thrush, chronic diarrhea (Crypto <180, microsporidium <100, CMV colitis <50, MAC with high fever) + weight loss, LAD - opportunistic infections / AIDS-defining illnesses eg MAC, CMV, toxo, PML (do PCR of CSF), TB, Histoplasma, Candida esophagitis (give empiric fluconazole), Crypto - --- bacillary angiomatosis - caused by Bartonella (also causes cat scratch disease), in HIV with CD4 <500 --> red, firm, friable, exophytic nodules; tx - oral erythromycin - --- Kaposi sarcoma - caused by HHV8 in HIV with CD4 <500 --> violaceous plaques/nodules on skin GI and lungs D. Mgmt i. pregnant women - if viral load <1K --> mom gets ART + Vaginal delivery, infant gets zidovudine for 6 weeks - if viral load >1K --> mom gets ART+ zidovudine + C-section, infant also gets ART, test until 6 mos ii. vaccinations / ppx - if HIV positive - give TMP-SMX PCP and toxo ppx for CD4 <200 and <100, azithro for MAC ppx for CDF <50 (high fever, weight loss, cough, diarrhea + HSM and elevated ALP) - HAV in MSM IVDU and cirrhosis; HBV if no immunity, HPV if 11-26, influenza yearly, meningococcus boosters q5, Td q10 yrs with one Tdap, and sequential conjugate PCV13 once then polysaccharide PPSV23 2 months and five yrs later and again at 65 (for immunocompromised, CKD, SCD, asplenia, CSF leaks, cochlear implants) iii. treatment - HAART (2 NRTIs eg AZT or didanosine and 1 NNRTI eg nevirapine, OR protease inhibitor eg indinavir) - side effects: AZT (anemia), abacavir (HSN), didanosine (pancreatitis), NNRTIs (lactic acidosis, hepatotoxicity), -navirs (hyperglycemia, lipodystrophy), indinavir (hematuria), -gravirs (increased CK),

Answer 29

2. SCID A. Etiology - deficiency of T cell development --> B cell dysfunction --> both cellular and humoral immunity affected - MC is XLR but mutation in adenosine deaminase is AR B. Diagnosis - - labs: low absolute lymphocyte (CD19 B and CD3 T cells), very low IGs and low CD4/CD8 T cells - absence of thymic shadow on CXR, germinal centers on lymph node biopsy, and T cells on flow cytometry C. Presentation - FTT and recurrent severe infections eg chronic viral diarrhea (similar to AIDS presentation) - loss of T cells --> viral, fungi (eg Candida oral thrush), PJP - loss of B cells --> pulmonary, GI bacterial - susceptibility to GvHD, NHL - adverse rxn to live vaccines (rotavirus, varicella) D. Mgmt - definitive tx is hematopoietic stem cell / bone marrow transplant, otherwise death by age 1-2

Answer 30

3. DiGeorge syndrome A. Etiology - 22q11 microdeletion --> abnormal 3rd and 4th pharyngeal pouch formation --> absent thymus and parathyroids B. Diagnosis - detect via FISH - absent thymic shadow on CXR - no response to intradermal test with Candida (bc of T cell deficiency) C. Presentation - CATCH22 (cardiac, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia) - absent parathyroid --> hypocalcemia --> seizures, tetany - absent thymus --> decreased T cells --> recurrent viral / fungal (Candida) infections, also PCP - velocardiofacial abnormalities - truncus arteriosus, transposition of great arteries (TGA), VSD + cleft lip, palate - characteristic facies - short philtrum, low-set notched ears, mandibular hypoplasia (ie micrognathia), slanted palpebrae - can also have TEF - developmental and speech delay D. Mgmt - surgery, Ca and Vit D supplements impaired cell-mediated immunity ie T cell disorders: - IL-12R deficiency (decreased IFNgamma --> disseminated infections with non-TB mycobacteria, Salmonella) - chronic mucocutaneous candidiasis (also has absent rxn to Candida intradermal test)

Answer 31

4. Leukocyte adhesion deficiency A. Etiology - see above --> impaired chemotaxis B. Diagnosis - neutrophilia C. Presentation - delayed separation of umbilical cord, no pus formation and delayed wound healing, recurrent bacterial skin eg cellulitis and mucosal eg peridontal infection (eg staph, candida) 5. Chronic granulomatous disease (CGD) A. Etiology - XLR defect in NADPH oxidase --> impaired oxidative burst --> phagocytes filled with bacteria on Gram stain B. Diagnosis - abnormal dihydrorhodamine or nitroblue tetrazolium (NBT) dye tests C. Presentation - recurrent pulm (PNA, empyema) infections and SSTI (S aureus skin abscesses, swollen infected lymph nodes, aphthous ulcers) with catalase (+) organisms eg pseudomonas, candida, e coli, listeria, staph aureus, serratia, aspergillus, nocardia D. Mgmt - IFNgamma immunomodulators, abx ppx with TMP-SMX and itraconazole 6. IgA deficiency A. Etiology - most common primary immunodeficiency B. Diagnosis - decreased IgA C. Presentation - anaphylaxis to blood products (contain IgA) - concomitant atopic (asthma, eczema) and autoimmune d/o (vitiligo, thyroiditis, RA)

Answer 32

7. Bruton (X-linked) agammaglobulinemia A. Etiology - XLR defect in tyrosine kinase --> Defective B cell maturation into plasma cells --> defective opsonization B. Diagnosis - absent lymph nodes and small tonsils - decreased B lymphocyte cells, plasma cells, and all classes of IGs, but normal T cells - NO response to vaccines, live vaccines c/i C. Presentation - recurrent sinopulmonary (esp otitis media by SHiN), and GI (eg Giardia, Salmonella, Campy) infections after 6 months - often simultaneous infections D. Mgmt - monthly IVIG and ppx abx 8. CVID A. Etiology - acquired abnormal B cell differentiation --> multiple IG classes deficient, like in Bruton's but less severe B. Diagnosis - normal T cell and B lymphocyte cell counts but low Igs in all lines (esp IgG and IgA); normal lymphoid tissues eg tonsils, lymph nodes - no response to vaccines C. Presentation - recurrent sinopulmonary infections esp SHiN, also GI - Giardia, Campy, sprue-like enteropathy - concomitant autoimmune dz eg RA, atopic eg asthma - presents 20-45 yo, increased risk of NHL D. Mgmt - IVIG *total lymphocytes = T cells (CD4 + CD8) + B cells (CD 19 + CD20)

Answer 33

9. Job syndrome A. Etiology - AD, impaired neutrophil recruitment B. Diagnosis - increased IgE, normal B cell count C. Presentation - FATED (coarse Facies, cold staph Abscesses, retained primary Teeth, hyperigE, Derm prob eg eczema) 10. Chediak Higashi A. Etiology - AR LYST gene mutation --> microtubule dysfunction --> defective phagolysosome fusion B. Diagnosis - giant granules in platelets, pancytopenia C. Presentation - partial albinism, peripheral neuropathy, recurrent staph and strep infections 11. Wiskott-Aldrich A. Etiology - XLR, T cells cannot reorganize actin cytoskeleton in WBCs or platelets B. Diagnosis - increased IgE, IgA and low IgG, IgM + small platelets C. Presentation - WATER (Wiskott Aldrich, Thrombocytopenia, Eczema, Recurrent infections) - bloody diarrhea, bruising, bleeding from umbilical stump - increased risk autoimmune and malignancy D. Mgmt - hematopoietic stem cell transplant; MCC death is EBV --> malignancy 12. Hyper IgM A. Etiology - XLR, defective CD40 ligand on Th cells --> cannot class switch B. Diagnosis - Increased IgM and low IgA, IgE, IgG; normal lymphocyte count C. Presentation - pyogenic infections + crypto, PJP, CMV

Answer 34

1. GERD - upper endoscopy if M > 50 yo with 5+ years of sx and cancer RFs eg tobacco - to exclude Barett - upper endoscopy if alarm sx eg persistent vomiting, melena, weight loss, anemia, dysphagia - otherwise, trial daily PPI x2 mos --> then can increase dose or switch PPI --> then consider endo or 24 hour pH monitoring 2. Dysphagia - if hx of difficulty initiating swallow with cough, choking, nasal regurgitation --> do videofluoroscopic modified barium swallow - if dysphagia with solids then liquids --> likely mechanical obstruction; if hx radiation caustic injury stricture (eg 2/2 GERD, systemic sclerosis) or surgery for esoph/larynx cancer --> do barium swallow first. otherwise upper endoscopy - if dysphagia with solids and liquids --> likely motility disorder eg achalasia --> barium swallow then esophageal manometry 3. Dyspepsia ie gastritis - episodic upper abdominal pain, postprandial fullness, belching, pain awakens at night (circadian acid secretion) --> inflammation / erosion of gastric lining - causes - ETOH, H pylori (MCC PUD), NSAIDs (2nd MCC PUD) - do upper endoscopy if >55 yo no matter what, OR if alarm sx (weight loss, bleeding, anemia, dysphagia) otherwise can do urea breath or stool test for H pylori; if no improvement after ammo, clarithro, PPI --> then upper endo

Answer 35

1. Childhood flow murmur - grade I or II mid-systolic ejection murmur, can be monitored w/out workup * any diastolic or continuous murmur needs w/u with echo 2. VSD - harsh holosystolic murmur at left midsternal border (loudest at 3rd/4th intercostal space) + palpable thrill + apical diastolic rumble - do echo to determine size and location - most small ones close spontaneously by age 2 - decreased weight gain, tired out while feeding or dyspnea with respiratory distress ~2-3 months (becomes audible as pulm vascular resistance drops bc lungs expand --> decreased R heart pressure --> increased L to R shunting) 3. PDA - continuous machine-like "to and fro" murmur best heard at 2nd left infraclavicular / intercostal space, also bounding peripheral pulses 2/2 increased LV stroke volume from L to R shunt, loud S2 2/2 pulmonary HTN, and widened pulse pressure - infants have recurrent respiratory tract infections 4. ASD - wide and fixed splitting of second heart sound, ejection murmur over left upper sternal border (increased flow across pulmonic), mid-diastolic rumble (increased flow across tricuspid) - a/w Down syndrome, fetal alcohol syndrome - asx until adulthood --> DOE, afib, stroke, RHF, cerebral abscess 5. HOCM (AD) - high-pitched systolic crescendo-decrescendo murmur at apex and left lower sternal border, similar to AS but no radiation to carotids; carotid pulse w dual upstroke - louder with Valsalva and standing (more obstruction with decreased venous return), softer with squatting leg raise handgrip, does not increase w expiration - give BBs to improve angina sx, if refractory then give verapamil / diltiazem - amlodipine, spironolactone, nitrates, ACEI/ARB c/i by decreasing preload and SVR --> worsen LVOT

Answer 36

6. Aortic stenosis - ejection click then systolic crescendo-decrescendo ejection murmur at R 2nd intercostal space radiating to carotids; if severe --> pulsus parvus et tardus, soft and single S2, late peaking murmur - decreased with increased afterload - handgrip (along with HOCM and MVP) - supravalvular aortic stenosis also possible, same murmur but different BP in UE + palpable thrill - syncope, angina, dyspnea - if symptomatic --> needs valve replacement 7. Aortic regurg - blowing diastolic decrescendo diastolic murmur best heard at left lower sternal border (bw 3-4th intercostal spaces) radiating to apex *if murmur is due to aortic root dilatation - heard best on R sternal border* - increased when leaning forward in full expiration - head bobbing, water hammer "bounding" pulses and wide pulse pressure, nail bed pulsations, leg >> arm BP - pulses bisferiens (biphasic pulse), also seen in HOCM and large PDA 8. Mitral stenosis - opening snap then rumbling diastolic murmur heard best at the apex (worse murmur heard earlier in cycle) - increase in LAP transmitted back --> increased pulmonary artery pressure - MCC rheumatic fever - often in younger immigrants - biggest RF for afib (MC arrhythmia in hyperthyroid) - a/w dysphagia hoarseness 2/2 LA enlargement 9. Mitral regurg - blowing holosystolic high-pitched murmur, loudest at apex and radiates towards axilla - p/w CHF sx of fatigue, exertional dyspnea - murmur increased with leg raise squatting handgrip 10. MVP - systolic crescendo murmur with mid-systolic click, earlier onset (more severe) with Valsalva and standing (like HOCM), later with squatting and handgrip - MVP syndrome - nonspecific sx eg atypical CP, SOB, palpitations, anxiety, panic attack --> can give BBs - a/w Marfan, Ehlers-Danlos 11. Tricuspid regurg - holosystolic plateau-shaped murmur at lower sternal border, loudest on inspiration a/w fluid overload CHF signs eg JVD, hepatomegaly, edema

Answer 37

Meningitis 1. LP A. Bacterial (MCC Strep pneumo, Neisseria, Staph aureus if recent NSG, GBS if <1 month old) - cell count in 1000s with neutrophils and elevated opening pressure - HIGH protein (>250), LOW glucose (<40), positive gram stain and cx B. Viral - cell count in 100s with lymphocytes - high protein (<150), NORMAL glucose (40-70) C. TB - cell count in 100s with lymphocytes - HIGH protein (>250), VERY LOW glucose (<10) D. Fungal (eg cryptococcus - presents over weeks), Lyme, Rickettsia - cell count in 100s with lymphocytes - protein can be high, glucose can be low - more subacute presentation 2. Do head CT and give abx before LP if seizures, focal neuro abnormalities, papilledema, confusion 3. Treatment - ceftriaxone + vancomycin + steroids (dexamethasone) if lots of neutrophils on LP to prevent complications of H flu in kids (deafness) or S pneumo in adults (death) + ampicillin to cover listeria if neonates or >50, immunocompromised eg HIV, alcoholics, pregnant, or steroid use 4A. Encephalitis (eg HSV) - confusion instead of headache, photophobia - MCC is HSV - do head CT, PCR of CSF, and start acyclovir (foscarnet if resistant) B. Abscess - MCC are S aureus and Viridans direct spread from otitis media, sinusitis or hematogenous spread from endocarditis --> fever + focal neuro signs eg hemiparesis, Babinski - cerberitis on MRI --> single ring-enhancing lesion with central necrosis - dx - CT guided aspiration or surgical biopsy for Gram stain and culture - tx - metro, cef, vanc + aspiration

Answer 38

Light's criteria - if 1+ are true --> exudative - pleural / serum protein > 0.5 - pleural / serum LDH > 0.6 - pleural fluid LDH > 2/3 ULN for serum Normal pleural fluid pH = 7.6 Transudative - 7.4-7.55 Exudative - 7.3-7.45 Empyema, tumor <7.3 Transudative - fluid moves from microvasculature into pleural space; <3 protein, >60 nl glucose A. atelectasis - decreased intrapleural pressure B. CHF, cirrhotic venous obstruction - elevated capillary hydrostatic pressure C. hypoalbuminemia (nephrotic syndrome) - decreased capillary oncotic pressure D. also peritoneal dialysis Exudative - due to increased capillary permeability (if infectious/inflammatory), thoracic duct obstruction/increased interstitial oncotic pressure (if chylothorax); >3 protein, <60 glucose A. TB (lymphocytosis, v elevated protein >4 and LDH B. PNA --> paraneumonic effusion - sterile exudative effusion, pH > 7.2, glucose >60, WBC <50K; tx - abx --> complicated parapneumonic effusion - bacteria invade effusion, pH <7.2, low glucose <60, WBC >50K with neutrophils; tx - abx + drainage --> empyema - same as complicated effusion but with frank pus on paracentesis or (+) Gram stain C. malignancy (abnormal cytology, pH <7.2, glucose <60, high LDH ; MCC breast and lung ca and lymphoma) D. chylothorax (milky white, increased TGs; MCC malignancy and trauma) E. Others - PE, asbestos, sarcoid

Answer 39

Pharyngitis A. Causes - MCC is viruses (EBV, HSV, rhino, parainfluenza, adeno), GABHS, GCC, Candida B. Clinical - viral more likely to have cough, coryza, rhinorrhea - Centor criteria for GABHS - 1 point for each: Cough absent Exudate on tonsils Nodes (tender anterior cervical LAD) Temperature >100.4F OR - young <14 add one point, OR old >45 subtract 1 point C. Mgmt *Centor criteria is for adults, not reliable in preadolescents <12 yo! Do rapid antigen test, if (-) throat cx, then treat <1 - reassurance that it is viral, analgesia, salt water gargle, lozenges 2-3 - rapid strep, then throat culture >4 - no rapid strep necessary, empiric tx - PCN, ammo, or erythro to prevent ARF (will not present PSGN) tender cervical LAD and splenomegaly - do monospot for CMV

Answer 40

1. Bilious vomiting - NPO, NGT, IVF, and abdominal X-ray: - if free air (necrotizing enterocolitis - pneumatosis intestinalis), hematemesis, unstable VS --> ex lap - dilated loops of bowel --> do contrast enema --> transition zone is Hirschsprung a/w Down; microcolon is meconium ileus a/w Cystic fibrosis - NGT in duodenum --> do UGI series --> ligament of trietz on R side and corkscrew --> malrotation / midgut volvulus - double bubble sign --> duodenal atresia a/w Down syndrome and annular pancreas 2A. Vascular ring - anomalous branch of aortic arch circles trachea and esophagus --> biphasic stridor and feeding difficulties, improves with neck extension - dx via barium swallow, MRI with angiography B. Laryngomalacia - laxity of supraglottic structures --> inspiratory stridor worse then supine, with feeding or URI that improves with lying prone - dx with direct or fiber-optic laryngoscopy, spontaneous resolution by 18 mos

Answer 41

Colo - start at 50 - 75 yo q10 with colonoscopy, q5 with flex sig, q1 with FOBT 1. FHx - start earlier - age 40 or 10 years before age of dx q5yrs 2. IBD - start 8-10 years after dx (12-15 if dz only in L colon) q1-3 yrs 3. FAP - start age 10-12 and repeat annually 4. Lynch - start age 21 and repeat q1-2 yrs - -- 1. Gastric outlet obstruction: - causes - malignancy, PUD, Crohns, strictures / pyloric stenosis 2/2 caustic agents, bezoars - postprandial n/v, abdominal pain; abdominal succussion splash to test for gastric outlet syndrome - green tinge to vomit = bilious --> SBO (can present with shock eg hypotension, tachycardia, fever, diffuse abdominal pain - which is not seen with GOO) 2. Ogilvie syndrome - colonic pseudo-obstruction in M >60, presents 3-7 days postop - causes - infection eg PNA, cardiac eg MI CHF, abd/ortho sx, neuro eg MS Alzheimer Parkinson - obstipation (no flatus or BM), abd pain, distension, n/v - dx - CT A/P showing colonic dilatation without obstruction (contrast visualized throughout colon) - tx - neostigmine 3. Paralytic ileus - causes - trauma, abd surgery; a/w hypokalemia - n/v, abd distension, obstipation (no flatus or stool), hypoactive BS (vs hyperactive tinkling BS in SBO) - dx - X-ray shows dilated gas-filled bowel loops with no transition point

Answer 42

1. Exercise ECG - Mechanism - increased cardiac contractility --> increased HR and BP - c/i in pts with LBBB, pacemaker, unable to reach target HR (arthritis, amputation) *can consider exercise thallium (nuclear isotope uptake) or exercise echo (wall motion abnormalities) 2. Pharm with adenosine or dipyramidole - in patients who cannot get to target HR - mechanism - dilate coronary arteries --> increased blood flow in nonobstructed >> obstructed --> reduction in radioactive isotope taken up by myocardial cells supplied by stenotic arteries - c/i in reactive airway disease (asthma, COPD), pts already on dipyramidole or theophylline, hypotension 3. Dobutamine stress echo - mechanism - B1 agonist that increases cardiac contractility and 02 consumption --> wall motion abnormalities on echo - c/i in tachyarrhythmias

Answer 43

Cirrhosis - do not need liver biopsy (gold standard) if clinical findings suggest cirrhosis + cause eg ETOH use A. compensated - nonspecific sx (nausea, fatigue) or asx --> US q6 mos for liver cancer screening + EGD for varices screening B. decompensated - jaundice, pruritus, UGIB, ascites, AMS or asterixis 2/2 hepatic encephalopathy - triggers - drugs (sedative, narcotic), hypovolemia (diarrhea), hypokalemia, increased nitrogen (GI bleed), infection (PNA SBP UTI), TIPS procedure, increased ammonia (protein rich diet) - mgmt: correct precipitating cause - -- ascites - lasix, paracentesis - -- encephalopathy - lactulose + rifamixin to decrease ammonia - -- variceal bleed - octreotide, ppx with propranolol or ligation C. Vaccinations - influenza yearly - Td q10 with one Tdap - PPSV23 before 65 (also for CAD, COPD, DM2, tobacco), then PPSV13 and 23 at age 65 - HAV 2 doses (for cirrhosis, and also IVDU, MSM) - HBV 3 doses

Answer 44

1. Goodpasture (type of RPGN) A. Diagnosis - Type II HSN - anti-GBM and alveolar BM Abs; linear deposits on bx B. Clinical - hematuria, hemoptysis C. Tx - emergent plasmapheresis, steroids 2. PSGN Post strep glomerulonephritis A. Dx - type III HSN - anti-DNAse Ab and ASO titers and low complement C3 B. Clinical - 2-4 weeks after GABHS pharyngitis or impetigo with cola colored urine, HTN, periorbital edema - more common in children, can lead to CKD in adults C. Tx - resolves spontaneously 3. IgA nephropathy A. Dx - MCC glomerulonephritis in USA; most accurate is renal biopsy B. Clinical - w/in 5 days of URI (synpharyngitic) with recurrent gross hematuria, nl complement levels - more common in young men, a/w Henoch-Schonlein C. Tx - no tx; severe proteinuria - steroids, ACEIs 4. Alport A. Dx - mutation in type IV collagen --> splitting of BM B. Clinical - can't pee, can't see, can't hear a bee C. Tx - None 5. Lupus nephritis A. Dx - tram track immune complex deposits and mesangial hypercellularity (MPGN, also a/w Hep B and C), wire looping of capillaries (DPGN) B. Clinical - nephritis and CAD (eg MI) common causes of death in SLE; often presents as nephrotic + nephritic concurrently

Answer 45

Nephrotic syndrome - >3.5g /day - edema (albumin loss), HLD, infections (Ig loss), renal vein thrombosis (antithrombin loss), Vit D deficiency, microcytic anemia (transferrin loss) - diagnose via urine albumin/creatinine ratio, gold standard is renal biopsy - tx - glucocorticoids and cyclophosphamide, ACEI/ARB to control proteinuria, diuretics for edema, statins for HLD 1. FSGS - MCC in adults esp blacks and Hispanics; IVDU, HIV/AIDS, massive obesity - regions of mesangial sclerosis and BM collapse 2. Membranous nephropathy (ie membranous glomeulonephritis) - 2nd MCC; a/w NSAIDs, Hep B and Hep C, breast or lung cancer, or lupus (nephrotic presentation of SLE) - thickened BM and subepithelial spikes - a/w renal vein thrombosis 3. MCD - primary idiopathic in kids, in adults - secondary, a/w NSAID use and Hodgkin lymphoma - in kids, responds to corticosteroids 4. Diabetes - MCC ESRD in USA - (w/in first year)glomerular hyperfiltration --> (w/in 5 years) thickening and nonenzymatic glycosylation of BM, mesangial expansion, hyalinolysis of afferent/efferent renal arterioles -->(w/in 10 yrs) microalbuminuria, nodular glomerulosclerosis with Kimmelstiel-Wilson nodules 5. Amyloid - MM MCC AL amyloidosis, RA MCC AA amyloidosis; enlarged kidneys on PE and apple-green birefringence on Congo red staining - tx - treat underlying, then melphalan and prednisone

Answer 46

Hemorrhage - progressive confusion and lethargy over several hours, first focal neuro deficits then ICP (n/v, HA); MCC is HTN MC sites of hypertensive hemorrhage: basal ganglia 50% (putamen), cerebellar nuclei, thalamus, pons, cerebral cortex 1. Basal ganglia (caudate/putamen, nucleus accumbens, subthalamic nucleus, substantia nigra) - contralateral hemiparesis and hemisensory loss eg dysarthria / clumsy hand (affects internal capsule), homonymous hemianopsia, conjugate gaze deviation (eyes deviate towards hemiparesis and away from lesion) - absence of cortical signs eg aphasia, neglect, hemianopia or seizure or AMS - can lead to uncal herniation (fixed dilated pupil, extensor posturing) 2. Cerebellum - NO hemiparesis but facial weakness, nystagmus and ataxia, occipital headache and neck stiffness 3. Thalamus - c/l hemiparesis and hemisensory loss, nonreactive miotic pupils and upgaze palsy, eyes deviate towards lesion --> leads to thalamic pain syndrome (severe paroxysmal burning pain over affected area after a stroke with allodynia) 4. Pons - miotic reactive pupils, coma and total paralysis 5. Cerebral cortex - c/l hemiparesis (if frontal lobe), c/l hemisensory loss (if parietal lobe), homonymous hemianopsia (if occipital lobe), eyes deviate away from hemiparesis and towards the lesion Adults > 60 - MCC spontaneous lobar hemorrhage is amyloid angiopathy, a/w Alzheimer Kids - MCC intracranial hemorrhage is AVM

Answer 47

1. MCA (most common) - c/l hemiparalysis and sensory loss of face and upper limb, conjugate eye deviation towards side of infarct (away from deficits), homonymous heminanopsia - aphasia if dominant - hemineglect if nondominant 2. ACA - c/l paralysis and sensory loss in lower limbs, abulia (lack of will), emotional disturbances, urinary incontinence 3. Wallenberg (lateral medulla 2/2 PICA occlusion) - dsyphagia, hoarseness, decreased gag reflex + ipsi Horner's + decreased pain and temp from ipsi face and contra body+ ataxia, dysmetria + vomiting, vertigo, nystagmus 4. Lateral pontine syndrome (lateral pons 2/2 AICA occlusion) - paralysis of face + decreased lacrimation, sensation anterior 2/3 tongue, salivation + decreased pain and temp from ipsi face and contra body + ataxia, dysmetria + vomiting, vertigo, nystagmus 5. Medial medullary syndrome (2/2 ASA occlusion) - contralateral paralysis and loss of proprioception/vibration of upper and lower limbs and tongue deviates ipsilaterally 6. PCA - contralateral hemianopsia with ocular sparing - alexia without agraphia if dominant 7. Lacunar - lipohyalinolysis of lenticulostriate vessels --> deep subcortical structure - pure motorhemiparesis, pure sensory, dysarthria - clumsy hand; no cortical symptoms

Answer 48

1. Polymyalgia rheumatica - seen in adults >50 yo A. Cause - unknown B. Diagnosis - increased inflammatory markers (ESR > 40, CRP, thrombocytosis, normocytic anemia), rapid response to prednisone; normal CPK and aldolase C. Clinical - bilateral shoulder or hip stiffness (limited ROM) and aching for >1 month duration, no localized tenderness or motor weakness - systemic sx (Fatigue, weight loss, low-grade fever, decreased appetite, depression) - associated with giant cell / temporal arteritis (u/l HA, jaw claudication, dx via bx, c/b aortic aneurysm) D. Mgmt - responds rapidly to low-dose prednisone (15mg) PO in few days, but taper over 1 year or so 2. Fibromyalgia A. Cause - unknown B. Diagnosis - 3+ months symptoms on widespread pain index, all normal lab markers C. Clinical - women with widespread pain worse after exercise, tenderness at trigger points eg mid-trapezius - fatigue, "fibro fog" D. Mgmt - exercise regimen, sleep hygiene, TCAs 3. Myositities A. Cause - anti-Jo1, SRP, Mi2 Ab B. Diagnosis - (+) ANA, high CK aldolase, muscle biopsy shows endomysial (poly) or perimysial (derm) infiltrate C. Clinical - progressive symmetrical proximal muscle weakness; can also involve esophagus --> dysphagia, regurgitation - dermatomyositis - above + Gottron papules, heliotrope rash (eyelids), shawl sign; a/w increased risk occult malignancy D. Mgmt - steroids then mtx IVIG, hydroxychloroquine for skin lesions

Answer 49

4. Scleroderma A. Cause - autoimmune noninflammatory vasculopathy and collagen deposition w fibrosis of skin and visceral organs (puffy taut skin w/out wrinkles) B. Diagnosis - ANA (+), ESR normal i. CREST - anti-Centromere ii. diffuse - Anti-Scl70 (anti-topoisomerase) Ab C. Clinical i. CREST - Calcinosis, Raynaud, Esophageal dysmotility (low LES resting pressure on manometry), Sclerodactyly, Telangiectasia ii. Diffuse - CREST + visceral involvement: - renal - renal crisis with HTN + MAHA - GI - GERD, SIBO --> toxic megacolon - pulm (MCC death) - pulm HTN 2/2 arterial intimal hyperplasia --> RVH and heave; interstitial lung disease with decreased lung volumes, DLCO D. Mgmt - symptomatic only eg PPIs (GERD), CCBs (Raynaud), cyclophosphamide (pulm fibrosis), sildenafil bosentan iloprost (pulm HTN), ACEI (renal crisis), methotrexate *nephrogenic systemic sclerosis can present similarly but is post-gadolinium (Eg MRI) in pts with CKD --> 30% mortality, do skin bx

Answer 50

All vasculitides present with fever, malaise/fatigue, weight loss, myalgias 1. Wegener granulomatosis ie granulomatosis with polyangiitis A. Dx - c-ANCA, do lung bx, normal complement C3 B. Clinical - necrotizing vasculitis --> URI (sinusitis, otitis media), LRI (hemoptysis, cough, CXR - lung nodules with cavitation), renal (hematuria, pauci immune RPGN - no C3/Ig deposition) + cutaneous eg pyoderma gangrenosum, urticaria, purpura C. Tx - prednisone, mtx, cyclophosphamide 2. Microscopic polyangiitis (type of RPGN) A. Dx - p-ANCA, normal complement B. Clinical - similar to Wegener but NO nasopharyngeal (sinusitis) C. Tx - prednisone, cyclophosphamide 3. Churg Strauss ie eosinophilic granulomatosis with polyangiitis A. Dx - p-ANCA, eosinophilia B. Clinical - asthma, palpable purpura, foot/wrist drop 4. Polyarteritis nodosa A. Dx - Type III HSN in young adults, a/w Hep B; fibrinoid necrosis of vessel walls B. Clinical - HTN, abd pain 2/2 chronic mesenteric ischemia, LE ulcers, mononeuropathy multiplex (eg asymmetric wrist or foot drop), tender subQ nodules, but NO lung sx C. Tx -corticosteroids and cyclophosphamides 5. Behcet syndrome - in young adults of Turkish, Middle Eastern, Asian descent A. Dx - Nonspecific vasculitis on biopsy B. Clinical - uveitis/blindness, recurrent painful oral aphthous and genital ulcers, skin lesions eg erythema nodosum, thrombosis - pathergy - exaggerated ulcerated response to minor trauma eg needle stick C. Tx - corticosteroids, cyclophosphamide, colchicine 6. Henoch Schonlein purpura - IgA mediated leukocytoclastic vasculitis that occurs in kids post URI: - arthralgias - abd pain / intussusception - palpable purpura - IgA nephropathy (hematuria) * need to have normal platelet and coag studies. Tx - supportive (hydration + NSAIDs); systemic glucocorticoids if severe. good prognosis * other vasculitides are Kawasaki, Beurgers, Takayasu arteritis, and temporal arteritis

Answer 51

Hyponatremia - Na <135; remember to add 2 for every 100 increase in glucose over 100 (cause of hypertonic) Hypotonic hyponatremia (serum Osm <280) 1. Hypovolemic - high RAAS and ADH activity; tx - NS - UNa < 40 (kidney trying to reabsorb Na) --> nonrenal salt losses eg vomiting, diarrhea, dehydration - UNa > 40 --> renal salt losses eg diuretics, Addison 2. Hypervolemic; tx - diuresis, fluid restriction - UNa < 40 --> CHF (low CO --> RAAS + ADH; Na level correlates to CHF severity), cirrhosis, nephrotic syndrome - UNa > 40 --> renal failure 3. Euvolemic; tx - fluid restriction; give hypertonic 3% saline if Na <120 --> correct 8 or less in first 24 hours to avoid osmotic demyelination, can also give demeclocycline - Uosm <100 and UNa >40 --> psychogenic polydipsia, beer potomania (low serum Osm, low urine Osm) - Uosm > 100 and UNa > 40 --> SIADH, hypothyroid (low serum Osm, high urine Osm) * SIADH causes - pulm eg PNA TB; CNS eg stroke TBI tumor meningitis; paraneoplastic with SCLC; meds eg carbamazepine, SSRIs, NSAIDs, sulfas, cyclophosphamide

Answer 52

Hypernatremia - Na > 145 Normal serum osmolality - 275-295 1. Hypovolemic; tx - free water replacement - Uosm >600 or UNa <20 --> insensible losses eg GI, burns - Uosm 300- 600 or UNa >20 --> hyperglycemia, mannitol 2. Hypervolemic - primary hyperaldo (HTN, high Na low K) or Cushing syndrome - hypertonic saline or sodium bicarb 3. Euvolemic - - Uosm <300 --> do water deprivation --> hypodipsia if corrects (urine becomes more concentrated) - then give vasopressin - -- corrects > 50% --> central DI; tx - desmopressin or vaptans - -- does not correct --> nephrogenic DI (can be caused by foscarnet, lithium, demeclocycline); tx - amiloride, HCTZ, indomethacin / NSAIDs DI - high serum Osm, low urine Osm, high Na SIADH - low serum Osm, high urine Osm, low Na

Answer 53

1. Aspergillosus - - invasive - triad of fever, pleuritic CP, and hemoptysis in immunocompromised eg neutropenics, glucocorticoid use, HIV - chronic pulmonary aspergillosis - >3 mos of cough, weight loss + aspergilloma in pre-existing cavity + positive IgG serology - CXR - nodular lesions with surrounding ground-glass opacities - dx - positive cell wall markers eg galactomann, beta-D-glucan - tx - voriconazole, capsofungin; resect aspergilloma = 2. PJP - also subacute pulm sx eg dyspnea, nonproductive cough, low grade fever + hypoxia - CXR - b/l, diffuse, interstitial infiltrates - tx - TMP-SMX and add corticosteroids if Sa02 <92%, Pa02 <70, or A-a gradient >35; give pentamidine if c/i to bactrim 3. Histoplasma - in Mississippi and Ohio River Valley, can lead to disseminated disease in HIV <100 - CXR - lung calcifications / apical cavitary lesions, reticulonodular infiltrates, non-caseating granulomas, and mediastinal/ hilar adenopathy (similar to TB and sarcoid) - a/w erythema nodosum, HSM, LAD - labs - transaminitis, pancytopenia, increased LDH and ferritin - dx - urine Histo antigen - tx - IV amphotericin B and oral itraconazole for maintenance 4. Blasto - in similar areas but more in Midwest, can lead to PNA with verrucous crusted skin lesions and lytic bone lesions, prostate dissemination in immuncompromised 5. Coccidioides also a/w erythema nodosum or erythema multiforme, in desert southwest, leads to CAP / San Joaquin Valley fever

Answer 54

CHF - nl EF = 55%; MCC death is arrhthymia 1. HFrEF (systolic dysfunction) - decreased contractility -> EF < 40% - MCC is CAD (MI), also dilated cardiomyopathy (ETOH, myocarditis, Chagas, thyroid, peripartum, beriberi, doxorubicin leading to myocyte necrosis and replacement with fibrous tissue), valvular disease, congenital heart disease, chronic hypertension - mortality benefit: ACEI/ARBs, BBs (metoprolol, carvedilol, bisoprolol), spironolactone / eplerenone, hydralazine + nitrates (in blacks and when ACEI/ARB c/i eg in CKD), implantable defibrillator AICD (ischemic CAD and EF <35%), biventricular pacemaker (dilated cardiomyopathy with EF <35% and QRS >120) - symptomatic: diuretics, digoxin (reduces hospitalizations) 2. HFpEF (diastolic dysfunction) - decreased compliance and relaxation --> nl EF - MCC is hypertrophy eg LVH 2/2 HTN, also hypertrophic cardiomyopathy, amyloidosis sarcoidosis, hemachromatosis, scleroderma - medications: ACEI/ARB, diuretics (role of BBs not established) 3. High output HF - cardiac output exceeds metabolic demand - due to severe anemia, thyrotoxicosis, paget, beriberi, AV fistulas - pulsatile tinnitus, bounding pulses, laterally displaced PMI, S3 gallop - supportive, treat underlying condition 4. Acute pulmonary edema - fluid accumulates in lungs rapidly -life threatening! - MCC due to acute decompensated heart failure 2/2 CHF (triggered by diet, infection, anemia) but can also be due to severe HTN, renal artery stensois, CKD w fluid overload - mgmt - goal is preload reduction --> upright position, Lasix, morphine, 02, nitrates to vasodilate IF BP is stable, norepi if BP is unstable *BB c/I bc worsen HF sx*

Answer 55

1. Hereditary spherocytosis (AD) - hereditary RBC membrane defect (in ankyrin) - presentation - hemolytic anemia, jaundice, splenomegaly with increased MCHC, RDW, negative Coombs - dx - acidified glycerol lysis and eosin-5-maleimide binding tests - tx - folic acid, blood transfusions, splenectomy - c/b pigment gallstones, aplastic crisis w Parvo B19 infections 2. RBC enzyme deficiency - G6PD deficiency (XLR) MCC - triggers - dapsone, quinidine, primaquine, macrobid, sulfas, fava beans - most accurate test is G6PD level 1-2 months after ep 3. pyruvate kinase - chronic hemolytic anemia in newborn, extravascular hemolysis 2/2 rigid RBCs --> HSM, skin ulcers, pigmented gallstones; high retic and (-) Coombs 4. PNH - acquired mutation with increased complement RBC lysis; Coombs (-) hemolytic anemia with episodic AM hematuria, pancytopenia, venous thrombosis (mesenteric/hepatic veins); CD 55/59 (-) RBCs on flow cytometry - tx - prednisone, bone marrow transplant, eculizumab 5. Fanconi anemia - inherited bone marrow failure 2/2 DNA repair defect presents 4-12 yo with pancytopenia, short stature, cafe au lait spots, thumb/radial defects - tx - stem cell transplant 6. Diamond-Blackfan anemia - congenital pure RBC aplasia (nl plts, WBC) presents in 1st year w macrocytic anemia, a/w limb abnormalities eg triphalyngeal thumbs, cleft palate; tx - steroids, RBC transfusions 7. Autoimmune hemolysis - direct Coombs (+) A. Warm - glucocorticoid eg prednisone, then splenectomy B. Cold - rituximab antiCD20 Ab +/- fludarabine

Answer 56

1. cholesterol emboli syndrome- seen following vascular procedure eg cardiac cath --> systemic eboli A. Clinical - derm - livedo reticularis, gangrene, blue toes - renal - AKI that lasts >2 weeks (contrast nephropathy resolves w/in 1 week) - CNS - amaurosis fugax, stroke - ocular - Hollenhorst plaques - GI - n/v, abd pain 2/2 intestinal ischemia, pancreatitis B. Diagnosis - increased Cr, low C3, eosinophilia - skin/renal bx shows perivascular inflammation with eosinophils - start on statins 2. Leriche - aortoiliac occlusion 1. b/l hip, thigh, butt claudication 2. absent or diminished femoral pulses 2/2 chronic ischemia 3. impotence (ED)

Answer 57

1. pemphigus vulgaris - Type II HSN anti-desmoglein Ab -- netlike IgG in epidermis with tombstone cells along basal layer and intraepidermal cleavage and acantholysis - flaccid bullae Nikolsky (+), mucosal erosions - tx - systemic steroids (prednisone) --> azathioprine or mycophenolate 2. bullous pemphigoid - Type II HSN anti-hemidesmosome Ab -- linear IgG and C3 deposits at BM epidermal-dermal junction (subepidermal cleavage with no acantholysis) - tense pruritic bullae in flexural surfaces, groin, axilla; no oral lesions - tx - high potency topical glucocorticoid eg clobetasol 3. scarlet fever - rash most pronounced in skin folds and desquamates at hands/foot; also a/w with tender LAD, tonsillar exudates (strep pharyngitis), fever, strawberry tongue; tx - penicillin 4. staph scalded skin syndrome - occurs in infancy (rare >5 yo) - diffuse erythema --> superficial flaccid bullae and then extensive skin exfoliation + fever, irritability, and (+) Nikolsky sign + multiorgan (AKI, hypotension); tx - naf or vanc 5. erythema toxicorum neonatum - asymptomatic, scattered erythematous papules and pustules containing eosinophils, spare palms and soles; tx - none, resolves spontaneously w/in 2 weeks

Answer 58

1A. Anaphylactic - rapid onset angioedema, urticaria, hypotension, respiratory distress - due to anti-IgA Ab (IgA deficiency) --> wash blood B. Allergic - urticaria, flushing, angioedema, pruritus w/in 2-3 hrs - Type I HSN - recipient IgE against donor plasma proteins 2. Acute hemolytic - fever, flank pain, hemoglobinuria, DIC w/in 1 hour; can have hypotension, tachypnea - due to ABO incompatibility (Type II HSN); direct Coombs positive, pink plasma 3. Febrile nonhemolytic - most common type; fever/chills but NO hemodynamic compromise w/in 1-6 hours (but can occur up to 24 hrs) - Type II HSN - host Ab against donor HLA antigens (requires prior exposure); OR cytokine accumulation from WBCs during blood storage --> leukoreduce blood 4. TRALI (Transfusion related acute lung injury) - respiratory distress, noncardiogenic pulm edema (normal - low PCWP) w/in 6 hours of exposure to plasma-containing products (MC platelets) - diffuse bilateral alveolar and interstitial infiltrates on CXR - due to donor anti-leukocyte Ab against host HLA antigens 5. Delayed hemolytic - mild fever and hemolytic anemia w/in 2-10 days - direct Coombs (+), new Ab screen (+) - due to anamnestic host Ab response against previously generated donor RBC alloantigens that escaped cross-matching detection 6. GvHD - Type IV HSN - grafted T cells against host cells --> maculopapular rash, jaundice, diarrhea, HSM - in bone marrow and liver transplants

Answer 59

1. Chromium - impaired glucose control in diabetics 2. Copper - brittle hair, ataxia / sx of B12 deficiency, depigmentation, sideroblastic anemia (also B6 deficiency), osteoporosis 3. Selenium - thyroid dysfunction, cardiomyopathy 4. Zinc - acrodermatitis enteropathica - alopecia, pustular skin rash (perioral), impaired wound healing, impaired taste, hypogonadism 5. Niacin (B3) deficiency - pellagra = diarrhea (plus glossitis), dermatitis (resembles sunburn), dementia, death 6. Riboflavin (B2) deficiency - angular cheilosis, glossitis, stomatitis (oral edema/erythema), seborrheic dermatitis (erythematous scaly patches) 7. Pyridoxine (B6) deficiency - cheilosis, glossitis, stomatitis, confusion/depression, dermatitis, stomatitis, sideroblastic anemia, elevated homocysteine --> VTE 8. Magnesium - fatigue, muscle cramps, arrhythmias low magnesium stimulates PTH but super low Mg inhibits PTH --> decreased Calcium low magnesium leads to excess renal K loss --> refractory hypokalemia; think about this in alcoholics give Mag to abort torsades but can even give with prolonged QT and normal Mg levels (other option is BB); also used for hypokalemia, hypocalcemia, and eclampsia (antidote is calcium gluconate) - Mag is renally excreted; hyperMag in CKD 9. Vitamin C - scurvy - punctuate hemorrhage / coiled corkscrew hair, mucosal bleeding, peridontal dz / gingivitis, delayed wound healing

Answer 60

Acromegaly - tumor effect - visual field defects, HA - MSK/skin - coarse facial features, skin tags, hyperhidrosis - cardio - mitral/aortic regurg, diastolic HTN, LVH on EKG (MCC death is cardiomegaly --> CHF) - pulm - sleep apnea - endocrine - high blood glucose PTH TGs; low libido and galactorrhea Workup 1. IGF1 level 2. if elevated --> oral glucose suppression test (should suppress GH secretion) 3. if GH increases --> MRI brain A. if normal --> evaluate for ectopic GH secreting tumor B. if pituitary mass (prolactinoma) - 95% cases - asx and <10 mm --> observe - asx OR >10 mm --> dopamine agonists which inhibit GH (cabergoline, bromocriptine) or somatostatin analog (octreotide) --> if that fails, then transphenoidal resection - >3 cm --> resection

Answer 61

1. Central cord - hyperextension (fall, whiplash) in elderly patients with preexisting degenerative changes eg cervical spondylosis (chronic neck pain, hypertrophic vertebral bodies, sclerotic facet joints, bony spurs) - bilateral motor >> sensory loss - UE >> LE - distal >> proximal - bladder dysfunction - vibration/proprioception/light touch (DCLMS) unaffected 2. Anterior cord - due to occlusion of anterior spinal artery ASA or flexion injury (disc retropulsion 2/2 accident) --> bilateral spastic motor paresis (LCT) + loss of pain/temp distal to injury (LST) 1-2 levels below injury bc of decussation - vibration/proprioception/light touch (DCLMS) unaffected 3. Posterior cord - due to MS, vertebral artery dissection --> bilateral loss of vibration / proprioception 4. Brown-Sequard - due to cord hemisection --> ipsilateral loss of motor, vibration/proprioception + contralateral loss of pain / temp 1-2 levels below the injury

Answer 62

A. CAP = Strep pneumo - empiric tx - (outpatient) azithro or doxy or levo (inpatient) levo or cef+azithro B. HAP = Gram (-) rods eg E coli, pseudomonas - empiric tx - vanc + zosyn C. VAP = Aerobic Gram (-) rods eg Pseudomonas, E coli and Gram (+) cocci eg Staph, strep - empiric tx - vanc + zosyn + gent and change after getting Gram stain and culture results 1. neonates (0-6 weeks) - GBS, E coli 2. children (6 weeks - 18 years) - RSV (<1yr), Parainfluenza croup (2-5 yrs), Mycoplasma, Chlamydia, Strep pneumo 3. adults (18-40 years) - Myco, Chlamydia, Strep pneumo 4. adults up until 65 years - Myco, Strep pneumo, H flu, anaerobes, viruses 5. elderly >65 years - Strep pneumo, viruses, anaerobes, H flu 6. CF - MCC PNA <20 yo is S. aureus, MCC > 20 yo is Pseudomonas 7. COPD - H flu, Moraxella, Strep pneumo 8. Postviral - S aureus (MRSA necrotizing PNA with multilobar cavitary infiltrates), Strep pneumo (cavitary lung lesions, affects elderly), H flu

Answer 63

Fat embolism: a/w long bone fractures, occurs 1-3 days after event 1. respiratory distress - hypoxemia Pa02 <60, bilateral fluffy infiltrates on CXR 2. petechial rash - neck, chest, axilla 3. neurologic dysfunction eg AMS, seizures ARDS: inflammatory injury to alveolar-capillary barrier - causes - sepsis / shock, pancreatitis, PNA, aspiration, uremia, trauma, amniotic fluid embolism (hypotension, LOC, DIC), drug OD - endothelial damage --> pro-inflammatory cytokines released --> increased alveolar permeability --> diffuse alveolar damage, hyaline membrane, noncardiogenic pulm edema (nl R-sided pressure and PCWP) --> fibrosis - hypoxemia with Pa02 / Fi02 < 300 mmHg - w/in one week after event with sudden onset SOB, fever, diffuse crackles - bilateral diffuse hazy opacities/densities on CXR with perihilar fullness - ventilate with low TV (6ml/kg) to and high PEEP to keep Fi02 <50% (plateau pressure = elastic pressure + PEEP, should be <30 cm water) --> goal is to prevent alveolar distension, keep Sa02 >88%, Pa02 55-80mmHg

Answer 64

MS - autoimmune inflammatory demyelination of CNS axons in brain and spinal cord (oligodendrocytes, which produce myelin around multiple axons) - clinical - deficits in time and space - optic neuritis (monocular vision loss with painful eye movement and afferent pupillary defect), b/l INO (demyelination of MLF) - cerebellar (intention tremor, ataxia, nystagmus) - transverse myelitis (motor/sensory loss below level of lesion with bowel/bladder incontinence) - can present with b/l trigeminal neuralgia - CSF - normal opening pressure cell count and total protein (increased % T cells), but high IgG, increased oligoclonal bands, myelin basic protein - T2 MRI shows periventricular plaques with well-circumscribed white matter lesions (destruction of axons) - flare treated with corticosteroids eg IV methylprednisolone, if refractory then IVIG or plasmapheresis - chronic maintenance therapy with IFNbeta or glatiramer or natalizumab, baclofen for spasticity

Answer 65

1. Intermittent - 2 or fewer SABA use / week and nighttime awakening / month, normal FEV1/FVC --> SABA prn 2. Mild persistent - >2 but not daily SABA use / week and 3-4 nighttime awakenings / month --> low-dose ICS 3. Moderate persistent - daily SABA use and weekly nighttime awakenings --> low-dose ICS + LABA or medium-dose ICS 4. Severe persistent - multiple daily SABA use and nightly nighttime awakenings --> medium-dose ICS + LABA symptomatic mgmt: if condition remains well controlled for 3 mos, consider step down and vice versa

Answer 66

Hypokalemia <3.5 - PR prolongation, QT prolongation, T wave flattening --> U wave, can lead to supraventricular and ventricular ectopic rhythms; a/w hypomagnesiumia Hyperkalemia EKG changes: 5.5-7 - shortened PR, peaked T wave 7-8 - loss of p wave, ST elevation, peaked T wave 8+ - widened QRS in sine wave pattern, AV nodal blocks, fascicle and BBBs --> vfib, PEA, or asystole - drugs - BBs, ACEI/ARB, eplerenon/spironolactone, digitalis, cyclosporine, heparin, NSAIDs, succinylcholine, trimethoprin - acute therapy (calcium gluconate, insulin + glucose) for K>6.5 or EKG changes metabolic alkalosis 1. saline responsive - urine Cl <20 eg vomiting, diuretics, laxative abuse, volume depletion 2. saline unresponsive - urine Cl >20 eg primary hyperaldo, Cushing, severe hypokalemia <2, Bartter/Gitelman syndromes

Answer 67

1. Cushing (obesity, facial plethora, bruising) --> late night salivary cortisol, low dose dexamethasone suppression test, and 24 hr urine cortisol --> if 2/3 (+) for high cortisol - -> measure plasma ACTH to differentiate ACTH independent ie low ACTH (exogenous use - suppressed by low dose dexa, adrenal adenoma - low ACTH and DHEAS) vs ACTH dependent (Cushing disease ie pituitary adenoma, ectopic) - -> then do high dose dexa suppression test to differentiate pituitary adenoma (high DHEAS, will be suppressed by high dose) vs ectopic (will not) - can give metyrapone (inhibitor of cortisol synthesis) while waiting for definitive sx tx 2. Pheo (pressure, pain, perspirations, pallor, palpitations) --> plasma free metanephrines is best initial test - confirm with 24 hr urine collection for metanephrines, VMA 3. Addison ie primary adrenal insufficiency (low cortisol and aldosterone and androgens --> hypotension, increased skin pigmentation, low Na, high K, low glucose) - -> high dose ACTH stim test (no response), plasma ACTH (elevated), 8 am serum cortisol (which should be when cortisol is at its peak but it will be low) - MCC Is autoimmune adrenalitis, also TB HIV or mets - tx - hydrocortisone (glucocorticoid), fludrocortisone (mineralocorticoid)

Answer 68

VIPoma (pancreatic cholera) - watery secretory tea-colored odorless diarrhea, muscle cramps, lethargy, flushing, n/v - labs - hypokalemia (increased intestinal secretion), hypercalcemia (increased bone resorption), hyperglycemia (increased glycogenolysis), stool osmol gap <50 - can be associated with hyperPTH as part of MEN1 - dx -VIP > 75, CT A/P shows tumor usually in pancreatic tail, can metastasize to liver by diagnosis - tx - IVF, octreotide, hepatic resection Carcinoid - neuroendocrine tumor in small bowel that secrete serotonin - clinical - see clinical signs only with mets to liver --> episodic flushing, secretory diarrhea (low stool osmotic gap <50), bronchospasm, R sided valve failure eg tricuspid regurg; niacin deficiency (pellagra) - dx - increased 5HIAA in urine, increased serum chromogranin A - tx - octreotide, resection Zollinger-Ellison - gastrinoma (parietal cell hyperplasia) of pancreas or duodenum - clinical - multiple ulcers in duodenum/ jejunum, dyspepsia, steatorrhea (2/2 inactivation of pancreatic enzymes); also a/w MEN1 (look for hypercalcemia) - dx - positive secretin test, gastrin >1000 with acidic pH <4 - do CT, then endoscopy to exclude metastatic dz

Answer 69

1. Adenomyosis - endometrial glands and stroma accumulate within myometrium; affects multips >40 yo w prior uterine sx eg C-section --> new-onset dysmenorrhea --> dull chronic pelvic pain, heavy menstrual bleeding, and symmetrically enlarged boggy tender uterus on PE - dx - clinical, do pelvic US, confirm via pathology after sx - tx -OCPs, levonorgesterol IUD, then hysterectomy 2. Fibroids - heavy menstrual bleeding; firm nontender irregularly-shaped uterus on PE; can affect fertility, lead to miscarriage - dx - US - tx - observe if asymptomatic, OCP or surgery if symptomatic 3. Endometriosis - in women 25-35 to with dysmenorrhea (pain peaks right before menses), dyschezia, dyspareunia; posterior cul-de-sac nodularity and adnexal masses (endometriomas), and fixed immobile uterus; common cause of infertility 2/2 adhesions - dx - laparoscopy - tx -asx endometriosis does not need to be treated; if sx (dysmenorrhea, dyspareunia, dyschezia) -- OCPs, NSAIDs, IUD (not copper), leuprolide (suppresses estrogen --> leads to hot flashes); definitive is surgical resection, hysterectomy 4. PCOS - obesity --> increased insulin resistance --> decreased SHBG --> increased free androgen levels - aromatase in fat tissue converts peripheral androgens to estrogen --> high frequency GnRH levels at hypothalamus --> increased LH:FSH at pituitary --> no LH surge --> anovulation - need 2 of 3: hirsutism, anovulatory cycles / oligmenorrhea, and cysts on US - tx - clomiphene (ovulation induction - restores pulsatile GnRH for LH surge; letrozole if BMI >30), OCPs, metformin, weight loss

Answer 70

1. malignant hyperthermia (AD) - uncontrolled efflux of calcium from sarcoplasmic reticulum; a/w halothane and dantrolene; hyperthermia, hyperkalemia, tachycardia, muscle rigidity; tx - IV dantrolene (antagonizes Ryanodine receptors --> inhibits Ca2+ release) 2. thyroid storm - from acute illness (trauma, infection), childbirth, postop, iodine contrast; hyperthermia 104-106F, tachycardia+ HTN + arrhythmias eg afib, AMS, warm and moist skin with tremor, lid lag, goiter, n/v/diarrhea; tx - propranolol, PTU, hydrocortisone - MOA - inotropy (increased cardiac contractility) and chronotropy (tachycardia) --> systolic HTN and increased pulse pressure, decreased SVR 3. NMS - usually w/in 2 weeks of starting antipsychotic; fever>104F, confusion/AMS, generalized muscle rigidity, autonomic instability (HTN, tachycardia, diaphoresis); labs - high WBC and CK; tx - dantrolene, bromocriptine, lorazepam 4. Serotonin syndrome - autonomic instability (HTN, tachy, diaphoresis), AMS, hyperreflexia and myoclonus, increased BS; tx - cyproheptadine - drugs that can cause serotonin syndrome: MDMA ie ecstasy - also causes hyponatremia cocaine bath salts - also cause agitation, psychosis opioids LSD

Answer 71

1. Pityriasis rosea- antihistamines, spontaneous resolution 2. Rosacea - topical metronidazole for papulopustular and ocular types, brimonidine (alpha2agonist) for facial erythema/flushing/telangiectasias (triggered by ETOH, stress, heat); other type is phymatous 3. Seborrheic dermatitis - if head selenium sulfide if face topical antifungal or corticosteroid 4. Dermatitis herpetiformis (on elbows, knees, back, butt) - oral dapsone 5. SJS/TEN - IVIG 6. Vitiligo - topical corticosteroids 7. Keloid - intralesional corticosteroids 8. Actinic keratosis - fluorouracil cream 9. Acne - first topical retinoid + benzoyl peroxide (preferred for inflammatory) or salicylic acid (preferred for comedonal) --> then topical abx (erythromycin, clindamycin) --> then oral abx (minocycline) --> then oral isoretinoin (for nodular, cystic acne) 10. Eczema - topical corticosteroids, tacrolimus, antihistamines, UV light 11. Psoriasis - high potency topical steroids eg clobetasol, triamcinolone, Vit D (calcipotriene), coal tar ointments, tacrolimus, TNF inhibitors, UV light 12. Lichen sclerosis - high potency topical steroid eg clobetasol but do punch biopsy to r/o cancer

Answer 72

1. Li-Fraumeni - SBLA (sarcoma, breast, leukemia, adrenals) 2. Osler-Weber-Rendu (AD) - telangiectasias, recurrent epistaxis, AVM, GIB, hematuria 3. Ataxia telangiectasia - cerebellar degeneration (Ataxia), spider angiomas (telangiectasias), IgA deficiency 2/2 failure to repair dsDNA breaks; increased AFP - high risk for cancer 4. McCune Albright - triad of polyostotic fibrous dysplasia, autonomous endocrine dysfunction, and café au lait spots 5. Felty: RA, neutropenia (ANC<1500), splenomegaly 6. Caplan: RA, pneumoconiosis, lung nodules

Answer 73

7. Juvenile RA (ie juvenile idiopathic arthritis) - typically occurs in female toddlers - high, spiking quotidian fevers >104F with evanescent salmon-colored macular rash on chest and abdomen + polyarticular joint pain with morning stiffness; also splenomegaly, pericardial effusion - anemia, high WBC and ferritin and platelets - do periodic ophtho exams for uveitis 8. Plummer Vinson - IDA, esophageal webs, dysphagia 9. Peutz-Jeghers - hamartomatous colonic polyps (increased GI cancer risk eg CRC, pancreatic ca) and hyperpigementation of hands/feet/genitalia 10. Gardner - colon cancer + osteomas + desmoid and other soft tissue tumors 11. Turcot - colon cancer + CNS malignancy 12. Shy-Drager (multiple system atrophy) - parkinsonism + autonomic dysfunction + widespread neuro signs

Answer 74

Non-glomerular hematuria - gross>>microscopic hematuria, can also be due to kidney stones, cancer, cystitis - UA - blood but no protein, normal RBCs (not dysmorphic or casts) 1. ATN - acute azotemia with increased FeNa, occurs 7-10 days after exposure - causes - hypotensive (shock, sepsis) or nephrotoxic injury eg aminoglycosides, lead, cisplatin, contrast - dark urine with muddy brown casts 2. AIN - microscopic hematuria and pyuria (eosinophils) with elevated creatinine +/- proteinuria - clinical - fever, rash, hematuria, CVA tenderness - causes - pee (diuretics), pain free (NSAIDs), penicillins/cephs, PPIs, rifamPin 3. Renal papillary necrosis - - causes - NSAID --> NSAIDs, Sickle cell, Analgesic abuse (tylenol), Infection (pyelo), DM2 - painless gross hematuria that resolves spontaneously 4. Crystal-induced AKI - <7 days of starting drug - causes - IV acyclovir, sulfas, mtx, ethylene glycol, protease inhibitors, uric acid (Via tumor lysis syndrome) - UA shows hematuria, pyuria, crystals (which block the renal tubules and cause direct toxicity) - tx- IVF

Answer 75

1. spinal epidural abscess - fever, back pain, neuro findings (bowel/bladder, paralysis) --> do MRI to dx + blood cx; tx - vanc + ceftri and aspiration/decompression (steroids make it worse!) * can have suprapubic mass --> distended bladder 2/2 urinary retention - MCC is S aureus from distant infection eg cellulitis, or spinal procedure, IVDU 2. spondylolisthesis - vertebral body slips forward, MCC back pain in <25 yo, a/w gymnastics swimming weight lifting; step-off on PE and increased pain with hyperextension, can have neuro sx eg incontinence 3. Osteo - exquisite focal tenderness on percussion + paravertebral muscle spasm + decreased ROM; fever, high CRP ESR plts; do blood cultures and X-rays (can be normal) --> MRI --> CT aspiration and culture biopsy - MCC is S aureus, RFs - IVDU, sickle cell, immunosuppresion other back pain workup - MRI - sensory/motor deficits, cauda equina syndrome / cord compression, spinal epidural abscess, osteomyelitis - X-ray - suspected malignancy eg MM or prostate mets, ankylosing spondylitis, osteoporosis/compression fracture

Answer 76

1. Chronic lymphocytic leukemia - in adults (MC) - elevated WBC --> painful LAD --> HSM --> fatigue and anemia --> thrombocytopenia - smudge cells on peripheral smear - most accurate test - flow cytometry - clonality of mature B cells - tx - fludarabine (purine analog) and rituximab (anti-CD20 Ab) if symptomatic; cyclophosphamide if refractory - secondary malignancy --> Richter transformation into DLBCL 2. Acute lymphocytic leukemia - MC in kids - bone pain, LAD, and pancytopenia - B-cell ALL in kids with fever, infections, bleeding, HSM - T-cell ALL in teenagers with mediastinal mass (infiltrates thymus) - best initial test - smear showing increased lymphoblasts; most accurate test - bone marrow bx >25% blasts - tx - need intrathecal chemo w mtx bc spreads to CNS and testes 3. Chronic myelogenous leukemia - due to BCR-ABL fusion on Philadelphia chromosome - in older adults - WBC >100K, all neutrophils with increased % precursors, basophilia on smear - LAP low (compared to high LAP in leukemoid reaction caused by mobilization of immature leukocytes from bone marrow 2/2 severe infection) - clinical - fatigue, night sweats, aquagenic pruritus (2/2 histamine release from basophils), splenomegaly; can transform into ALL/AML in blast crisis - tx - imatinib, only cure is stem cell transplant 4. Acute myelogenous leukemia - in older adults; a/w Down syndrome - precursor is Myelodysplastic syndrome (asx pancytopenia on CBC, RFs hx of prior chemo/radiation w dysplastic cells on smear) - high WBC but low mature; low RBC and platelets - best initial test - >20% blasts (APL is myeloperoxidase (+) and has Auer rods) on smear; most accurate test - flow cytometry - clinical - fatigue, LAD, HSM, fever, swollen gums; APL can present with DIC - tx - ATRA for APL 5. Hairy cell - clonal B cell neoplasm in middle-aged men with massive splenomegaly (early satiety), pancytopenia (fatigue, infections, bruising) - dry tap 2/2 bone marrow fibrosis, smear shows hairy cells - dx - bone marrow bx + flow cytometry; tx - cladribine

Answer 77

6. NHL - proliferation of lymphocytes in lymph nodes and spleen (similar to CLL except those WBC are circulating) - painless LAD + B sx - types - Burkitt (a/w EBV), DLBCL (MC in adults), follicular, mantle cell, primary CNS lymphoma - pancytopenia, elevated LDH (worse prognosis) - best initial test - excisional biopsy (NOT needle aspiration) also CT scans, bone marrow biopsy - tx - CHOP (cyclo, adriamycin, vincristine, prednisone) + rituximab; relapses treated with radiation - increased risk of another primary malignancy eg breast thyroid lung ca 2/2 radiation, also constrictive pericarditis (--> R HF), premature CAD 7. Hodgkin lymphoma - same presentation and tests as NHL except HD presents more locally / less advanced, Reed Sternberg cells on pathology - tx - ABVD (adria, bleo, vinblast, dacarbazine)

Answer 78

1. IUGR - <10th percentile weight - symmetric - onset in 1st trimester, due to infections (toxo, CMV) or chromosomal abnormalities, global growth lag - asymmetric - onset in 2/3rd trimesters, due to maternal malnutrition or uteroplacental insufficiency 2/2 HTN or DM2 or tobacco, "head sparing" growth lag - do weekly BPP, serial umbilical artery Dopplers, and serial growth US 2. IUFD = fetal death @ 20+ weeks; will not hear fetal heart tones on doppler/NST --> do abdominal US to confirm lack of cardiac activity < 24 weeks --> D and E or vaginal delivery >24+ weeks --> vaginal delivery; retention of fetus can lead to coagulopathy (screen via fibrinogen) evaluation: fetal autopsy and karyoptype, examination of placenta and cord; Kleihauer-Betke test for fetomaternal hemorrhage, APL-Ab and coag studies 3. Cerclage indications - if hx preterm labor or painless 2nd trimester losses - IM progesterone (maintains uterine quiescence) starting 2nd trimester + serial TVUS until 24 weeks (during 2nd trimester) --> if cervix <2.5 cm --> cerclage if 14-24 weeks - if no hx - do screening TVUS b/w 16-24 weeks to determine cervical length - -> if <2cm --> vaginal progesterone from 16 to 36 weeks or cerclage (?) - -> if >2 cm --> normal prenatal care

Answer 79

4. PPROM - <34 weeks - abx and steroids and observe; if signs of infection or fetal compromise --> Mg (if <32) and delivery - 34-37 weeks - abx (amp, erythro) + steroids + delivery 5. Preterm labor - RFs - bacterial vaginosis, multis, HTN, prior hx, cervical insufficiency A. >34 weeks - manage expectantly but can consider betamethasone, penicillin if GBS (+) from culture 35-37 weeks, prior delivery, prior GBS UTI OR unknown status + <37 weeks, intrapartum fever, or ROM >18 hrs B. 32- 34 weeks - betamethasone, penicillin, tocolytics (extend gestation by 48 hrs so you can give 2 doses steroids) - if ctx but no cervical change -- do fetal fibronectin test (high NPV) C. <32 weeks - betamethasone, penicillin, tocolytics, mag sulfate (Ca2+ competitive antagonist, membrane stabilizer) for fetal neuroprotection; side effects - hyporeflexia, flushing, HA, diplopia, respiratory depression; reverse with calcium gluconate Tocolytics - relax myometrium A. Indomethacin - inhibits cyclooxygenase, 1st line for <32 weeks - maternal - gastritis, platelet dysfunction - fetal - closure of ductus arteriosus, olighydramnios B. Nifedipine - 1st line for 32-34 weeks - maternal - tachycardia/hypotension, nausea, flushing, HA C. Terbutaline - short-term inpatient use - maternal - arrhythmias, hypotension, hyperglycemia (c/i in DM), pulm edema 6. Preeclampsia - PEC w/out severe features - delivery at 37+ weeks - PEC w/ severe features (>160/110 x2, plt <100K, incr LFTs, Cr>1.1, visual/cerebral sx, pulm edema) - delivery at 34+ weeks - magnesium sulfate - anti-HTN - IV hydralazine, IV labetolol (c/i if bradycardic), oral nifedipine

Answer 80

1. Heparin - osteoporosis, hyperkalemia 2/2 hypoaldosteronism, HIT 2. LMWH (enoxaparin, preferred in pregnancy), fondaparinaux, rivaroxaban c/I in CKD (GFR <30) -- use unfractionated heparin instead * if no c/i, rivaroxaban preferred to warfarin for DVT/PE 3. Halothane - acute liver failure acute liver failure: cannot have cirrhosis or underlying liver dz - elevated LFTs - hepatic encephalopathy eg confusion, AMS, asterixis - synthetic liver dysfunction - INR >1.5 MCC Is tylenol OD, also viral Hep A B D E, ischemia (shock liver, Budd-Chiari (hepatic vein thrombosis)), autoimmune hepatitis, Wilson - liver transplant if Cr >3.4, PT >100, grade III/IV encephalopathy 4. Etomidate - inhibits 11B hydroxylase --> adrenal insufficiency in elderly, septic 5. NO - inactivates B12 --> can lead to peripheral neuropathy in those with pre-existing B12 deficiency 6. Propofol - myocardial depression --> severe hypotension, c/i in HFrEF 7. Succinylcholine - binds to postsynaptic Ach receptors --> influx of Na and efflux of K --> delayed skeletal muscle repolarization --> paralysis - can lead to arrhythmias 2/2 hyperkalemia esp with comorbid conditions eg rhabdo, burns, muscle atrophy, denervation (GBS, stroke); also malignant hyperthermia - in these cases use nondepolarizing agents eg rocuronium 8. Venlafaxine- HTN (Dose-dependent) 9. Amantadine - livedo reticularis 10. Non-dihydropyridine (verapamil, diltiazem) - AV block --> worsening of bradyarrhythmias and HF, constipation; verapamil - increases prolactin levels 11. Dihydropyridine (-dipines) - peripheral edema arteriolar dilation --> (increased capillary hydrostatic pressure), HA flushing dizziness, gingival hyperplasia 12. BBs - bronchoconstriction, fatigue, depression/psychosis, weight gain, sexual dysfunction, exacerbates MG, Raynaud

Answer 81

Precocious puberty - devlpt of 2 sex characteristics in girls <8 and boys <9 Bone age -- normal --> premature thelarche or adrenarche -- delayed (2+ SDs) --> check basal LH A. high basal LH --> central precocious puberty eg pituitary microadenoma, idiopathic precocious puberty B. low basal LH --> GnRH stim test --> low LH (peripheral), can do pelvic or abd US eg CAH, testicular tumor * McCune Albright is cause of GnRH independent peripheral precocious puberty with HIGH FSH/LH also high TSH, GH, ACTH --> high LH (central) -- DKA and HHS mgmt: (DKA - lipolysis of fat stores --> FFA broken down in liver into ketones) - IVF - high flow 0.9% NS, add 5% dextrose when glucose <200 - continuous IV insulin, switch to SQ if: able to eat, glucose <200, AG<12, and HC03>15; overlap with IV by 2 hrs - add IV K if <5.2, hold insulin if K <3.3 - add bicarb if pH <6.9 - add phosphate if <1, respiratory depression, cardiac dysfunction and monitor Ca - best markers for resolution of DKA are serum anion gap and direct assay of betahydroxybutyrate (predominant ketone in DKA)

Answer 82

1. diverticulitis - CT shows stranding, colonic thickening - uncomplicated - PO abx (cipro or any flouroquinolone + metronidazole), bowel rest, observation in outpatient - uncomplicated but elderly, immunosuppressed, comorbid conditions eg DM2, fever/highWBC --> inpatient IV abx - complicated - abscess, perf, fistula, obstruction - -- abscess <3 cm - observe - -- abscess >5 cm - CT-guided perc drainage, then lap for surgical drainage debridement - -- fistula, obstruction, recurrent attacks, perforation - sigmoid resection 2. toxic megacolon - abd X-ray showing dilated >6 cm, air-fluid levels, haustra that do not extend across lumen and 3+ of following: fever, pulse>120, WBC>10.5, anemia tx - IV corticosteroids (Esp if IBD-related), NGT, IVF IV abx and bowel rest - if peritoneal signs (Rebound/guarding) --> ex lap

Answer 83

Ovarian 1. dysgerminoma - equivalent to male seminoma; in adolescents; secrete LDH (some secrete bhCG) 2. yolk sac - MC tumor in male infants; aggressive - acute onset pelvic pain, in ovaries or testis; AFP 3. serous cystadenocarcinoma - MC ovarian neoplasm (type of epithelial ovarian carcinoma); pelvic pain, bloating, constipation; increased Ca125 and psamomma bodies (also seen in papillary thyroid cancer); do US (solid mass with septations), Ca125 levels, then exlap 4. granulosa cell tumor - MC malignant stromal tumor; Call Exner bodies; granulosa cells convert testosterone to estradiol via aromatase and secrete inhibin (blocks FSH) --> high estradiol and inhibin levels--> precocious puberty, endometrial hyperplasia, postmenopausal bleeding 5. embryonal carcinoma - occurs in young women; hCG and AFP 6. Krukenberg tumor - metastatic gastric cancer to ovary Testicular Germ cell tumors - 95 % - dx - ultrasound then staging (CT) - tx - radical orchiectomy with lymph node dissection and chemotherapy 1. Seminoma - malignant, most common testicular tumor, high beta-hCG and ALP - radiosensitive (chemo), good prognosis 2. Nonseminomatous (mixed) germ cell tumor - high beta-hCG and AFP - anterior mediastinal mass suggests primary mediastinal germ cell tumor - confirm with bx, do testicular US to differentiate primary mediastinal vs metastatic from testicles *also include teratoma 3. Non-germ cell - 5% - leydig cell --> gynecomastia - sertoli cell - testicular lymphoma - metastatic lymphoma to testes

Answer 84

Incontinence 1. Stress (urethral hypermobility >30deg) - kegel, pessary, urethral sling (surgery and pessary equally efficacious in pelvic organ prolapse) 2. Urge (detrusor instability) - bladder training, oxybutynin (antimuscarinic), mirabegron (beta agonist for people who cannot take anticholinergics) 3. Overflow - eg diabetic neuropathy (impaired detrusor contractility), MCC in men is bladder outflow obstruction via BPH (do renal US if AKI to assess for hydronephrosis, if cath is needed) - tamsulosin (alpha1 antagonist), finasteride (5alphaR inhibitor, takes longer to work); increased postvoid residual volume - >150mL in women, >50mL in men; tx- cholinergic agonist eg bethanochol, intermittent self-cath Workup for UTI: - uncomplicated cystitis - macrobid, bactrim, or fosfomycin; urine culture only if 1st treatment fails - complicated cystitis - DM2, pregnancy, CKD, catheter, immunosuppression, hospital acquired - do urine culture, cipro 5-14 days or amp/gent for more severe - pyelo - do urine culture, outpatient cipro or levo; inpatient IV levo + amp VCUG: newborns <1 month, children <2 yrs with recurrent UTI, children <2 yrs with UTI from organism other than E. coli renal and bladder US: <24 mos with first febrile UTI, recurrent febrile UTIs any age, no response to abx

Answer 85

Anterior (LAD) - V1-V6 Lateral (LCX) - STEMI in I, avL, V5, V6; depression in II III avF Inferior (RCA) - II, III, avF *hypotension, bradycardia, AV block Posterior (RCA or LCX) - depression in V1-V3; (LCX) STEMI in I and avL; (RCA) ST depression in I and aVL Inferior (RCA) - V4R-V6R AAA - RFs are age >60, smoking (highest RF for aneurysm rupture!), FHx, white, atherosclerosis - repair indications: >5.5cm, >0.5cm / 6 mos or >1.0 cm / 1 year expansion, sx incl abd back or flank pain - repair complication - bowel ischemia to L and sigmoid colons after loss of IMA during graft placement--> abd pain, bloody diarrhea, fever, WBC count - aortoenteric fistula is late complication (months-years) when duodenum erodes through --> either anemia + FOBT OR massive GI bleed - yearly low-dose CT chest lung ca screening in patients 55-80 yo with 30+ pack-year hx and <15 years since quitting - one time AAA US in men 65-75 yo who have ever smoked

Answer 86

1. Kidney stones - colicky flank pain radiating to groin - dx: CTA/P w/out contrast, then US - MC type is calcium oxalate; calcium phosphate a/w primary hyperPTH and RTA, cysteine a/w COLA, struvite a/w pH>8 and urease bacteria, uric acid are radiolucent and a/w breakdown of purines (eg tumor lysis) or acidic pH - mgmt: hydrate, decrease salt and oxalate, increase potassium citrate (both to alkalinize urine and bc citrate is a stone inhibitor, to prevent both calcium and uric acid stones), normal calcium in diet; HCTZ for recurrent calcium stones 2. post transplant complications: < 1 week - hyperacute rejection 2/2 ABO mismatch <1 month - bacterial infection --> sepsis (Fever, hypotension, leukocytosis) 2/2 postop or hospital <3 months - acute rejection - fever, RUQ pain, LFTs, leukocytosis, HTN, decreased urine output 2/2 prior sensitization to donor alloantigens (Type IV HSN) --> treat with 3-5 day course of high dose IV steroids <6 months - post transplant opportunistic infections - PCP (ppx with TMPSMX; causes PNA) and CMV (ppx with valgancyclovir; causes pneumonitis, hepatitis, gastroenteritis) >6 months - immunosuppressants at maintenance levels, main risk is community-acquired pathogens

Answer 87

Cyanotic heart lesions (most babies have RAD bc PDA shunts blood towards RV 1. AV canal defects (ASD, VSD) - a/w Down, increased markings on CXR 2/2 excess pulm blood flow * these are acyanotic bc L--> R flow, unless Eisenmenger 2. Ebstein anomaly - a/w maternal lithium use for bipolar; atrialization of RV; tall P waves and RAD on EKG, cardiomegaly on CXR 3. Tetralogy of Fallot - *MCC cyanotic lesion* RVOT, RVH, VSD, overriding aorta; RAD on EKG, boot shaped heart on CXR (upturning of cardiac apex 2/2 RVH) - VSD holosystolic murmur + harsh systolic ejection murmur at left upper sternal border from pulmonic stenosis - tet spells and cyanosis with crying, feeding 4. TAVR - severe cyanosis and respiratory distress; RVH and RAD on EKG, snowman sign on CXR 2/2 enlarged supracardiac and SVC veins 5. Truncus arteriosus - single S2 + systolic ejection murmur with loud ejection click at left border; a/w DiGeorge, EKG nl, cardiomegaly and increased markings on CXR 2/2 increased pulm blood flow - severe dyspnea, frequent respiratory infections, bounding peripheral pulses - sequelae is pulm htn 6. Tricuspid atresia - single S2 + VSD murmur; LAD (only congenital cyanotic defect with LAD!) and peaked p waves on EKG, decreased pulmonary markings on CXR 2/2 minimal pulm blood flow 7. Transposition of great arteries - single S2 +/- VSD; "egg on string" heart (narrowed mediastinum) on CXR - neonatal cyanosis and tachypnea but babies appear relatively comfortable

Answer 88

pregnancy changes: - renal: increased renal blood flow --> increased GFR, decreased BUN/Cr, increased proteinuria; increased risk UTIs 2/2 urinary stasis - heme: hypercoagulable state - increased fibrinogen, decreased Protein C and S; dilutional anemia --> decreased Hb, also decreased plts; anemic if <10.5 in 1st and 3rd tri and <11 in 2nd - cardio: decreased SVR, increased SV and HR --> increased CO, peripheral edema; BP low-normal and can have systolic ejection murmur - pulm: dyspnea 2/2 increased TV and minute vent via progesterone --> chronic respiratory alkalosis with metabolic compensation; decreased FRC 2/2 diaphragm - GI: hepatic enzymes eg LFTs, Tbili remain in normal range although ALP can be elevated; if LFTs high --> think about HELLP - endo: increased insulin resistance 2/2 human placental lactogen; dietary modifications then insulin if fasting glucose >95, 1hr postprandial >140 (Glucola @ 24-28 wks >140 and 3-hour >140 to dx) - -- estrogen stimulates increase in TBG, bHCG alpha unit stimulates TSHR --> increased total T4, unchanged fT4, and decreased TSH --> increase thyroxine dose when pregnant - lactation suppresses ovulation via PRL suppression of GnRH for 6 months, 50% women resume ovulation after 6 mos vs Sheehan - inability to lactate (low PRL, TSH) - ENT: pyogenic granulomas on anterior nasal septum - cause nosebleeds HTN risks - postpartum hemorrhage, gestational DM, placental abruption, IUGR, oligohydramnios, preterm delivery DM risks - - first trimester - NTD, small left colon, caudal regression / sirenomelia, congenital heart defects - later on - macrosomia (4500+ g offer C-section), polyhydramnios, polycythemia, hypocalcemia, hypoglycemia, NRDS in preterm babies (insulin delays pneumocyte maturation --> surfactant deficiency --> hypoinflation, decreased compliance, atelectasis with air bronchograms and white out lung on CXR), TTN (tachypnea w no hypoxia, fluid in fissures) PEC risks - IUGR, placental abruption, DIC, eclampsia, hepatic rupture

Answer 89

Neonatal conjunctivitis - Chemical <24 hrs due to silver nitrate; tx - eye lubricant - Gonococcal - 2-5 days - profuse purulent d/c, swelling, corneal ulceration; tx - IM cefotaxime - Chlamydial - 5-14 days - watery d/c, mild swelling; tx - oral erythromycin (watch out for pyloric stenosis) Perianal dermatoses: 1. Contact - MCC, spares creases/skinfolds - topical barrier ointment 2. Candida - 2nd MCC , beefy red rash that involves skinfolds and has satellite lesions - topical antifungal eg nystatin - culture shows pseudohyphae w budding yeast 3. Strep - in school age kids, sharply demarcated erythema and dyschezia, anal fissures - needs oral abx eg PCN, ammo 4. Tinea corporis (ringworm) - annular lesions with raised border and central clearing, caused by dermatophyte - septated hyphae on KOH prep - topical antifungal

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