Step 2 C

Question 1

Rapidly increasing pulse that collapses suddenly as arterial pressure decreases rapidly in late systole and diastole; can be palpated at wrist or femoral arteries

Answer 1

Corrigan’s pulse (Water-Hammer pulse)

Seen with Aortic Regurgitation

Question 2

Low-pitched diastolic rumble due to competing flow anterograde from LA and retrograde from the aorta.

Answer 2

Austin-Flint murmur

Seen with Aortic Regurgitation

Question 3

Opening snap followed by low-pitched diastolic rumble and pre-systolic accentuation.

S2 followed by opening snap.

Murmur followed by loud S1

Answer 3

Mitral stenosis

Question 4

Harsh crescendo-decrescendo systolic murmur radiating to carotid arteries; decreases with valsalva

Soft S2, may also be single since aortic component may be delayed and merge into P2

S4

Parvus et tardus - diminished and delayed carotid upstrokes

Answer 4

Aortic stenosis

Question 5

De musset’s sign
Muller’s sign
Duroziez’s sign
Quincke sign

Answer 5

De mussets: Head bobbing (rhythmical jerking of head)
Mullers: Uvula bobs
Duroziez’s sign: pistol-shot sound heard over femoral arteries.
Quincke: capillary pulsations in nail bed, more visible when pressure is applied

Seen with aortic regurg

Question 6

Holosystolic murmur radiating to axilla, diminished S1, widening of S2, S3 gallop, loud/palpable P2, midsystolic click

Answer 6

Mitral Regurgitation

Question 7

Blowing holosystolic murmur intensified with inspiration, reduced during expiration/valsalva

Answer 7

Tricuspid regurg

Question 8

Midsystolic/late systolic click. Mid to late systolic murmur
Murmur increases with standing/valsalva
Murmur decreases with squatting

Answer 8

Mitral valve prolapse

Question 9

Lereiche’s syndrome

Answer 9

Atheromatous occlusion of distal aorta just above bifurcation causing bilateral claudication, impotence, and absent/diminished femoral pulses

Question 10

Ankle Brachial Index

Answer 10

Ratio of systolic BP at the ankle to systolic BP at the arm
Normal: 0.9-1.3
>1.3 is due to noncompressible vessels and indicates severe disease.
Claudication ABI

Question 11

Common causes of ARDS

Answer 11

AAAA R DDDD SSS
Aspiration
Acute pancreatitis
Air/Amniotic embolism
Radiation
Drug overdose
Diffuse lung disease
DIC 
Drowning
Shock
Sepsis
Smoke inhalation

Question 12

Characteristics of ARDS (3)

Answer 12

Refractory hypoxemia
Decreased lung compliance
Pulmonary edema

Question 13

Lab findings in ARDS
ABG
Swan-Ganz catheter
PaO2:FiO2

Answer 13

ABG - Respiratory alkalosis, ↓O2 (dt impairment of O2 transfer from pulmonary capillaries by pulmonary edema), ↓ CO2 (dt hyperventilation)

Swan-Ganz catheter - wedge pressure

Question 14

What type of primary lung cancer is seen with:
Hypercalcemia
Dermatomyositis

Answer 14

Squamous cell

Question 15

What type of primary lung cancer is seen with: 
DIC
Thrombophlebitis
Microangiopathic hemolytic andmiea
Dermatomyositis

Answer 15

Adenocarcinoma

Question 16

What types of paraneoplastic syndromes are seen with small cell carcinoma (9)

Answer 16

Cushing syndrome
SIADH
Ectopic growth hormone and ACTH secretion
Peripheral neuropathy
Subacute cerebellar degeneration
Lambert-Eaton syndrome (similar presentation to myasthenia gravis)
Subacute sensory neuropathy
Limbic encephalitis
Dermatomyositis

Question 17

What type of primary lung cancer is seen with:
Gynecomastia
Dermatomyositis

Answer 17

Large cell carcinoma

Question 18

> 50yo
Progressive exercise intolerance, dyspnea, dry crackles, JVD, tachypnea, digital clubbing
PFT: normal FEV1/FVC, ↓FVC, ↓ TLC, ↓compliance
Bronchioalveolar lavage shows ↑PMNs
CXR: reticulonodular pattern with “honeycomb” lung
CT: “ground glass” appearance

Disease?
Treatment?

Answer 18

Disease: Idiopathic pulmonary fibrosis

Treatment: Corticosteroids + Azathioprine/cyclophosphamide
Lung transplant frequently indicated

Question 19

Blacks > whites, females > males, 10-40yo
Cough, malaise, wt loss, dyspnea, knee/ankle arthritis, CP, fever, erythema nodosum, LAD, vision loss, cranial nerve palsy
↑ serum ACE, ↑ Ca, ↑ urine Ca, ↑ alk phos, ↓WBC, ↑ESR
PFT: ↓FVC and ↓DLCO
CXR: Bilateral hilar LAD, pulmonary infiltrates (ground glass appearance)

Disease?
Treatment?

Answer 19

Disease: Sarcoidosis
Treatment: occasionally self-limited, corticosteroids if chronic, cytotoxic drugs used with failure of steroids, lung transplant only if severe

Question 20

Restrictive lung disease + h/o working with insulation, construction, demolition, building maintenance, automobiles

XR: multinodular opacities with predeliction to lower lobes, pleural effusions, blurring of heart/diaphragm
CT: linear pleural/parenchymal fibrosis

Answer 20

Asbestosis

Increased risk of malignant mesothelioma and lung CA

Synergistic effect with tobacco

Question 21

Restrictive lung disease + h/o mining, pottery making, sandblasting, cutting granite

XR: Small apical nodular opacities with prediliction for upper lobes, hilar adenopathy

Answer 21

Silicosis

Increased risk of TB infection
Progressive fibrosis

Question 22

Restrictive lung disease + h/o coal mining

XR: Small apical nodular opacities

Answer 22

Coal worker disease - inhalation of coal dust, which contains carbon and silica
Complication: Progressive fibrosis

Question 23

Pulmonary edema, diffuse granuloma formation + h/o working with electronics, ceramics, tools, die manufacturing.

XR: Diffuse infiltrates, hilar adenopathy

Answer 23

Berylliosis
Diagnostic blood test: beryllium lymphocyte proliferation test

Increased risk of lung CA, may need chronic corticosteroid treatment to maintain respiratory function

Question 24

Hemoptysis, dyspnea, recent respiratory infection

Labs: + anti-GBM Ab
PFT: restrictive pattern with increased DLCO (due to presence of Hgb in alveoli)
UA: Proteinuria and granular casts
Renal biopsy: Crescentic glomerulonephritis and IgG deposition along glomerular capillaries
CXR: bilateral alveolar infiltation.

Disease?
Treatment?

Answer 24

Goodpasture syndrome: progressive AI disease of lungs/kidneys caused by anti-glomerular basement membrane (Anti-GBM) Ab, characterized by intra-alveolar hemorrhage and glomerulonephritis

Tx: Plasmapheresis to remove autoAb, corticosteroids, immunosuppressants

Question 25

Hemoptysis, dyspnea, myalgias, chronic sinusitis, ulcerations of nasopharynx, fever, mild hematuria, CNS (hearing loss, sensory neuropathy, CN dysfunction), ophthalmologic (conjunctivitis, proptosis, corneal ulceration, diplopia), cardiac (arrhythmias) Labs: + c-ANCA Lung biopsy: noncaseating granulomas Renal biopsy: vasculitis process Disease? Treatment?

Answer 25

Granulomatosis with polyangiitis (Wegener's) - rare disease with granulomatous inflammation and necrosis of lung and other organ systems. Caused by systemic vasculitis that mainly affects lung/kidney, causing formation of noncaseating granulomas and destruction of lung parenchyma Treatment: cytotoxic therapy (cyclophosphamide), corticosteroids Rapidly fatal if untreated

Question 26

4 stages of sarcoidosis

Answer 26

XR: Stage I: bilateral hilar adenopathy (highest rate of remission) Stage II: hilar adenopathy + parenchymal infiltrates Stage III: diffuse parenchymal infiltrates without hilar adenopathy (least favorable prognosis) Stage IV: pulmonary fibrosis + honeycombing and fibrocystic parenchymal changes

Question 27

H/o Asthma + pulmonary infiltrates, rash, eosinophilia, skin/muscle/nerve lesions. Significant blood eosinophilia + pANCA

Answer 27

Churg-strauss syndrome Tx: systemic glucocorticoids

Question 28

H/o drinking surface water Greasy foul-smelling diarrhea, abdominal pain, malaise Disease? Treatment?

Answer 28

Giardia lamblia - cysts and trophozoites seen in stool Tx: Metronidazole, hydration

Question 29

H/o drinking water in areas of poor sanitation Mild to severe bloddy diarrhea, abdominal pain Disease? Treatment?

Answer 29

Entamoeba histolytica - cysts and trophozoites seen in stool | Tx: Metronidazole, paromomycin

Question 30

H/o contaminated food/water. More common in immunocompromised patients Watery diarrhea, abdominal pain, malaise Disease? Treatment?

Answer 30

Cryptosporidium parvum - acid-fast stain of stool shows parasites Tx: Control immune suppression, nitazoxanide

Question 31

H/o eating undercooked pork Fever, myalgias, periorbital edema, eosinophilia Disease? Treatment?

Answer 31

Trichinella spiralis Treatment: Albendazole, mebendazole if CNS/Cardiac symptoms

Question 32

H/o eating undercooked pork. Nausea, abdominal pain. Disease? Treatment? Complication?

Answer 32

``` Intestinal taeniasis (T. solium) - disease from ingestion of CYSTS Tx: Praziquantel ``` Complication: Ingestion of eggs from feces of human with intestinal taeniasis → Cysticercosis; cysts in muscles, subQ tissues, eyes, extraocular muscls, brain (neurocysticercosis). May cause seizures. Tx: Albendazole + corticosteroids for neurocysticercosis

Question 33

Gradually progressive dysphagia of solids and liquids, regurgitation, cough, aspiration, heartburn, weight loss from poor intake. Manometry shows ↑ LES pressure, incomplete LES relaxation and decreased peristalsis Barium swallow shows tapering at LES Disease? Treatment? Complications?

Answer 33

Achalasia: impaired peristalsis and ↓ LES relaxation due to intramural neuron dysfunciton. Idiopathic - most commonly affects persons 25-60yo. Secondary causes include Chagas disease, neoplasm, scleroderma Tx: nitrates and CCBs relax LES but are rarely used bc of simultaneous cardiac effects; pneumatic dilation, botox injections or myotomy to relieve obstruction Complications: myotomy can cause GERD

Question 34

Chest pain and dysphagia. Manometry shows nonperistaltic, uncoordinated esophageal contractions Barium swallow shows "corkscrew" pattern Disease? Treatment?

Answer 34

Diffuse esophageal spasm - neuromuscular disorder in which nonperistaltic contractions of the esophagus occur Tx: CCBs, nitrates or TCAs to reduce chest pain and dysphagia

Question 35

Type A vs Type B Chronic Gastritis

Answer 35

Type A: Fundus, Auto-Ab for parietal cells → ↓ gastric acid and gastrin. Associated with pernicious anemia, achlorhydria, thyroditis Type B: 90% of cases, Antrum, Assoc with H. Pylori infection, ↑ gastric acid, associated with PUD and gastric cancer

Question 36

Gastric ulcer vs Duodenal ulcer

Answer 36

Gastric ulcer: >50yo, H. pylori/NSAID suers, pain worsens soon after eating, normal/low gastric acid level, high gastrin level Duodenal ulcer: younger, H. pylori infection, 75% of cases, improvement in pain 2-4h after eating with later worsening, high gastric acid level, normal gastrin level

Question 37

4 types of gastric CA

Answer 37

Ulcerating - resembles ulcers seen w PUD Polypoid - large, intraluminal neoplasms Superficial spreading - mucosal and submucosal layers only, BEST prognosis Linitis plastica: ALL layers involved, decreased stomach elasticity, POOR prognosis *** most commonly adenocarcinoma. squamous is rare and caused by invasion from esophagus

Question 38

h/o weight loss, anorexia, early satiety, vomiting, dysphagia, epigastric pain, L supraclavicular/periumbilical lymph node. ↑ CEA, ↑ 2-glucuronidase in gastric secretions, anemia Radiology: barium swallow may show mass or thickened "leather bottle" stomach Disease? Treatment? Complications?

Answer 38

Gastric CA Tx: Subtotal gastrectomy for lesions in distal 1/3 of stomach, total gastrectomy for lesions in middle/upper stomach or invasive lesions; adjuvant chemo and radiation Complications: Early detection has high cure (70%) but poor prognosis in later detection (

Question 39

H/o diarrhea, steatorrhea, wt loss, bloating. +/- depression, anxiety, arthralgias. Assoc w down syndrome. Assoc w dermatitis herpetiformis. Labs: + Antiendomysial and antigliadin Ab in serum. Biopsy shows blunting of duodenal and jejunal villi. Disease? Treatment?

Answer 39

Disease: Celiac disease Tx: Removal of gluten from diet, refractory disease may require corticosteroids

Question 40

H/o diarrhea, steatorrhea, wt loss, bloating. +/- depression, anxiety, arthralgias. History of living in tropical area Labs: No antiendomysial or anti-TTG Ab. Disease? Treatment?

Answer 40

Tropical sprue | Tx: Folic acid replacement and tetracycline. Removal of gluten has no effect

Question 41

H/o wt loss, joint pain, abdominal pain, diarrhea, dementia, cough, bloating, steatorrhea; fever, vision abnormalities, LAD, new heart murmur; severe wasting in late disease. Jejunal biopsy shows foamy macrophages on periodic acid-schiff stain and villous atrophy. Disease? Treatment?

Answer 41

Whipple disease due to infection with Trophyerma whippelii and likely immune deficiency (unknown if innate to host or caused by infection); multiple organs involved. Risk factors: white males with European ancestry Tx: TMP-SMX or ceftriaxone for 12 months. High mortality if untreated

Question 42

Rome III criteria for diagnosis of IBD

Answer 42

Recurrent abdominal pain/discomfort for at least 3 days/mo, associated with 2+ of the following: Improvement of pain with defecation Change in frequency of stool Change in form/appearance of stool

Question 43

``` Chron's Disease: Site of involvement Symptoms PE Extraintestinal manifestations Labs Radiology Tx Complications ```

Answer 43

Chron's Disease: Site of involvement - entire GIT with skip lesions, Distal ileum most commonly involved, ALL LAYERS of bowel wall Symptoms - abd pain, wt loss, WATERY diarrhea PE - fever, RLQ mass, perianal fissures and fistulas, oral ulcers Extraintestinal manifestations - Arthritis, ankylosing psondylitis, uveitis, nephrolithiasis Labs - + ASCA, rare pANCA, biopsy is diagnostic Radiology - ulcers, strictures, "cobblestoning", fissures, bowel edema Tx - Mesalamine, broad spectrum abx, corticosteroids, immunosuppressives, surgical tx for severe areas/fistulas/strictures Complications - abscess, fistula, fissure, malabsorption

Question 44

``` Ulcerative Colitis: Site of involvement Symptoms PE Extraintestinal manifestations Labs Radiology Tx Complications ```

Answer 44

Ulcerative colitis: Site of involvement - continuous beginning at rectum, extends as far as distal ileum. Only mucosa and submucosa affected. Symptoms - abd pain, urgency, BLOODY diarrhea, tenesmus, n/v, wt loss PE - fever, orthostatic hypotension, tachycardia, gross blood on rectal exam Extraintestinal manifestations - arthritis, uveitis, ankylosing psondylitis, primary sclerosing cholangitis, erythema nodosum, pyoderma gangrenosum Labs - +pANCA, rare + ASCA, biopsy diagnostic Radiology - pseudopolyps, "lead pipe" colon w/o haustra and colon shortening Tx - mesalamine, supplemental iron, corticosteroids, immunosuppressives, total colectomy is CURATIVE Complications - significantly increased risk of COLON CA, hemorrhage, toxic megacolon, bowel obstruction

Question 45

``` Small bowel obstruction: Causes Symptoms PE Radiology Tx ```

Answer 45

Small bowel obstruction: Causes -ADHESIONS, incarcerated HERNIA, neoplasm, intussuception, volvulus, chron's, congenital stricture Symptoms - abd pain, vomiting, distention, obstipation PE - visible peristaltic waves, high-pitched bowel sounds vs absence of bowel sounds, fever Radiology - XR; LADDER-LIKE dilated loops of bowel, air-fluid levels Tx - NPO, maintain hydration, nasogastric decompression, possible surgery

Question 46

``` Large bowel obstruction: Causes Symptoms PE Radiology Tx ```

Answer 46

Large bowel obstruction: Causes - NEOPLASM, diverticulitis, volvulus, congenital stricture Symptoms - pain, distension, nausea, LATE FECULENT VOMITING PE - tenderness, palpable mass, high-pitched bowel sounds vs absence Radiology - XR; distention proximal to obstruction Tx - NPO, hydration, colonoscopy may relieve obstruction, possible surgery

Question 47

H/o abdominal distention and pain, vomiting, obstipation, palpable abd mass. Abdominal XR: "Double bubble". Barium enema: "birds beak". Diagnosis? Treatment?

Answer 47

Volvulus - rotation of bowel creating obstruction and possible ischemia; most common at cecum and sigmoid colon. Commonly elderly and infants. Tx: possibly self-limited. Colonoscopic decompression of sigmoid volvulus; surgical repair or resection may be required in cecal volvulus or failed colonoscopic detorsion.

Question 48

H/o abd pain, flushing, diarrhea, bronchoconstriction, tricuspid/pulmonary valve disease Labs: ↑ 5-hydroxyindolacetic acid (5-HIAA) i n urine, ↑ serum serotonin Disease? Treatment?

Answer 48

Carcinoid tumor - most commonly in bronchopulmonary tree, ileum, rectum, appendix. Tumor arising from neuroectodermal cells that function as amine precursor uptake and decarboxylation (APUD) cells. * Carcinoid syndrome (flushing, diarrhea, bronchoconstriction, tricuspid/pulmonary valvular disease) is caused by serotonin secretion by tumor and is only seen with liver mets or extra-gastrointestinal involvement. Tx: Tumors 2cm have high risk of metastases and require extensive resection. Metastatic disease treated with IFN-α, octreotide, and embolization

Question 49

Familial colon tumor syndromes: Hundreds of polyps in colon; near certain development of malignant neoplasm

Answer 49

Familial adenomatous polyposis (FAP) - caused by mutation in adenomatous polyposis coli (APC) gene - Autosomal dominant - 100% risk of colorectal CA by 3rd or 4th decade of life. - Colon always involved, duodenum in 90% of cases. prophylactic subtotal colectomy reocmmended

Question 50

Familial colon tumor syndromes: Hundreds of polyps in colon; near-certain development of malignant neoplasm; + osteomas, dental abnormalities, benign soft tissue tumors, desmoid tumors, sebaceous cysts

Answer 50

Gardner syndrome - caused by mutation in adenomatous polyposis coli (APC) gene - Risk of colorectal cancer is 100% by age 40

Question 51

Familial colon tumor syndromes: Many colonic adenomas with high malignant potential; comorbid malignant CNS tumors (cerebellar medulloblastoma or glioblastoma multiforme)

Answer 51

Turcot syndrome - caused by mutation in adenomatous polyposis coli (APC) gene - Autosomal recessive

Question 52

Familial colon tumor syndromes: Hamartomatous polyps of colon, small bowel, and stomach that frequently are source of GI bleeding; slightly increased risk of malignancy later in life

Answer 52

Juvenile polyposis

Question 53

Familial colon tumor syndromes: Polyps are hamartomas with low risk of malignancy; mucocutaneous pigmentation of mouth/hands/genitals/palmar surfaces

Answer 53

Peutz-Jeghers syndrome

Question 54

Familial colon tumor syndromes: Multiple genetic mutations; CA arises from normal-appearing mucosa; neoplasms tend to form in proximal colon

Answer 54

Hereditary nonpolyposis colorectal cancer (HNPCC) Lynch syndrome I: early onset colorectal CA, absence of antecedent multiple polyposis Lynch syndrome II: all features of Lync I + increased # and early occurrence of other cancers

Question 55

Duke classification system for staging and corresponding prognosis of colorectal cancer

Answer 55

Class A (TNM I) - tumor confined to bowel wall, 90% cure rate Class B (TNM II) - penetration into colonic serosa or perirectal fat, 80% cure rate Class C (TNM III) - lymph node involvement,

Question 56

Causes of acute pancreatitis (12)

Answer 56

PANCREATITIS ``` hyperParathyroid (→ hyperCa) Alcohol Neoplasm Cholelithiasis Rx (medications) ERCP Abdominal surgery hyperTriglycerides Infection (mumps) Trauma Idiopathic Scorpion bite ```

Question 57

Ranson criteria for determining prognosis during acute pancreatitis

Answer 57

``` At admission: GA LAW: Glucose > 200 AST >250 LDH >350 Age >55 Wbc >16 ``` ``` At 48h: Calvin & HOBBS Ca 10% PaO2 5 Base deficit >4 Sequestration of fluid >6L ```

Question 58

Whipple procedure and Childs operation

Answer 58

aka pancreaticoduodenectomy Removal of the antrum (distal) stomach, duodenum, proximal jejunum, head of pancreas, CBD, and gallbladder, lymph nodes. Reconstruction (Child's operation) consists of attaching pancreas to the jejunum (pancreaticojejunostomy), attaching hepatic duct to jejunum (hepaticojejunostomy) to allow digestive juices and bile to flow into the GIT, and attaching stomach to jejunum (gastrojejunostomy) to allow food to pass through. * head of pancreas and duodenum share same blood supply (gastroduodenal artery), therefore both must be removed if the blood supply is severed.

Question 59

H/o headache, visual changes, confusion, weakness, mood instability, palpitations, diaphoresis Labs: ↑ fasting insulin, spontaneous hypoglycemia, ↑ C-peptide Disease? Treatment?

Answer 59

Insulinoma - insulin-secreting β-islet cell tumor causing hypoglycemia * * Whipple's triad: sx of hypoglycemia while fasting, hypoglycemia, improvement in sx with carbohydrate load. * * almost always solitary, multiple insulinomas may be seen in MEN1 Tx: Surgical resection; diazoxide or octreotide may relieve symptoms in non-resectable disease

Question 60

H/o abdominal pain, diarrhea, wt loss, mental status changes, exfoliating rash (necrolytic migratory erythema), diabetes Labs: hyperglycemia Diagnosis? Treatment?

Answer 60

Glucagonoma - glucagon-secreting α-cell tumor causing hyperglycemia Tx: Surgical resection if localized; octreotide, IFN-α, chemotherapy and embolization in metastatic disease Frequently malignant, poor prognosis

Question 61

H/o watery diarrhea, weakness, n/v, abdominal pain Labs: stool osmolality suggests secretory cause Diagnosis? Treatment?

Answer 61

VIPoma - vasoactive intestinal peptide (VIP)-producing tumor of non-β-islet cells Tx: Surgical resection for localized tumors; corticosteroids, chemo, octreotide, embolization in metastatic disease

Question 62

h/o fatigue, pruritus, arthralgias, jaundice, xanthomas, skin hyperpigmentation, hepatosplenomegaly Labs: ↑ alk phos, ↑ GGT, normal AST/ALT, ↑ cholesterol, ↑ bili (total and direct), + ANA, +antimitochondrial Ab Diagnosis? Treatment?

Answer 62

Primary biliary cirrhosis - AI disease with intrahepatic bile duct destruction leading to accumulation of cholesterol, bile acids, bilirubin. Risk factors: RA, sjogren syndrome, scleroderma, FEMALE > male Tx: Ursodeoxycholic acid improves liver function and reduces symptoms; colchicines or methotrexate can be added if severe, liver transplant in progressive disease

Question 63

H/o fatigue, pruritus, RUQ pain, fever, night sweats, jaundice, xanthomas. Labs: ↑ alk phos, ↑ GGT, normal AST/ALT, ↑ cholesterol, ↑ bilirubin (total and direct), possible +pANCA ERCP shows stricturing and irregularity of extra and intrahepatic bile ducts ("beads on a string") Diagnosis? Treatment?

Answer 63

Primary sclerosing cholangitis - progressive destruction of intrahepatic and extrahepatic bile ducts leading to fibrosis and cirrhosis Risk factors: Ulcerative colitis, MALE > female

Question 64

Causes of unconjugated hyperbilirubinemia (7) - Excess production (3) - Reduced uptake/Impaired conjugation (4)

Answer 64

Excess production: - Hemolytic andemia - Disorders of erythropoiesis - Internal hemorrhage resoprtion Reduced uptake/Impaired conjugation: - Physiologic jaundice of the newborn - Deficiency of glucuronosyltransferase (gilbert dz, crigler-najjar syndrome) - Hepatocellular disease (cirrhosis, hepatitis) - Drug induced (sulfonamides, PCN, rifampin, radiocontrast agents)

Question 65

Causes of conjugated hyperbilirubinemia (7) - Decreased excretion (3) - Extrahepatic obstruction (4)

Answer 65

Decreased excretion: - Impaired bilirubin transport (Dubin-Johnson syndrome, Rotor syndrome) - Hepatocellular disease (cirrhosis, hepatitis) - Drug induced (i.e. OCPs) Extrahepatic obstruction: - Intrahepatic bile duct disease (PBC, PSC) - Choledocholithiasis - Pancreatic/biliary CA - Biliary atresia

Question 66

Causes of portal hypertension: - Prehepatic (1) - Intrahepatic (4) - Posthepatic (3)

Answer 66

Prehepatic - Portal vein thrombosis Intrahepatic - Cirrhosis - Schistosomiasis - Parenchymal disease - Granulomatous disease Posthepatic - R sided heart failure - Hepatic vein thrombosis - Budd chiari syndrome (hepatic vein thrombosis secondary to hypercoagulability)

Question 67

H/o loss of coordination, dysphagia, jaundice, tremor, green-brown rings in cornea, hepatomegaly, psych disturbances (depression, neuroses, personality changes) Labs: ↓ serum ceruloplasmin, ↑ urinary copper, slightly ↑ AST/ALT Diagnosis? Treatment? Complications?

Answer 67

Wilson disease - AR disorder of impaired copper secretion, primarily in young adults Tx: Trientine or penicillamine for copper chelation, lifelong zinc for maintenance therapy, dietary copper restriction (no organ meats, shellfish, chocolate, nuts, mushrooms), supplementary vitamin B6. Liver transplant may be needed in cases of liver failure Complications: Fulminant liver failure, cirrhosis

Question 68

H/o RUQ pain, wt loss, malaise, anorexia, diarrhea, dyspnea, jaundice, hepatomegaly, liver bruit, ascites Labs: slightly ↑ AST/ALT, ↑ alk phos, ↑ bili (total and direct), ↑ α-fetoprotein Diagnosis? Risk factors? Treatment? Complications?

Answer 68

``` Hepatocellular carcinoma (hepatoma) ** paraneoplastic syndromes: hypoglycemia, excessive RBC production, refractory watery diarrhea, hyperCa, variable skin lesions ``` Risk factors: HBV/HCV infection, cirrhosis, hemochromatosis, excessive consumption of aflatoxin from Aspergillus-infected food, schistosomiasis. Treatment: surgical resection of small tumors and chemo, transplant may be an option for limited disease. Radiofrequency ablation and chemoembolization are options for unresectable tumors. Complications: poor prognosis, portal vein obstruction, Budd-chiari syndrome, liver failure

Question 69

Differential diagnosis of ascites (9)

Answer 69

- Cirrhosis, portal HTN - CHF - Chronic renal disease - Massive fluid overload - Tuberculosis peritonitis - Malignancy - Hypoalbuminemia - Peripheral vasodilation secondary to endotoxin-induced release of nitrous oxide → ↑ renin secretion → secondary hyperaldosteronism - Impaired liver inactivation of aldosterone

Question 70

Complications of liver failure (11)

Answer 70

``` AC, 9H Ascites Coagulopathy Hypoalbuminemia portal HTN Hyperammonemia Hepatic encephalopathy Hepatorenal syndrome Hypoglycemia Hyperbilirubinemia/jaundice Hyperestrinism (spider angiomas, palmar erythema, gynecomastia, testicular atrophy) Hepatocellular carcinoma ```

Question 71

Most common type of benign liver tumor

Answer 71

Cavernous hemangiomas - ↑ in size w pregnancy or OCPs - Complications (rare unless very large): rupture with hemorrhage, obstructive jaundice, coagulopathy, CHF due to large AV shunt, gastric outlet obstruction - Most don't require treatment, consider resection if symptomatic or high risk of rupture

Question 72

Types of hepatocellular carcinoma

Answer 72

1. Non-fibrolamellar (most common) - usually assoc with HBV/HCV or cirrhosis. Usually unresectable with very short survival time (months) 2. Fibrolamellar - NOT assoc with HBV/HCV or cirrhosis, more often resectable, relatively longer survival time, most commonly in adolescents and young adults

Question 73

Causes of ↑ AST/ALT (8) | ABCDEFGHI

Answer 73

``` Autoimmune hepatitis hepatitis B hepatitis C Drugs/toxins Ethanol Fatty liver (triglyceridemia) Growths (tumors) Hemodynamic disorders (i.e. CHF) Iron (hemochromatosis, Copper (Wilson's disease) or AAT deficiency ```

Question 74

Mallory-Weiss syndrome vs Boerhaave's syndrome

Answer 74

Mallory-Weiss -- mucosal tear at the GE junction Boerhaave's -- transmural tear causing esophageal perforation

Question 75

Extraintestinal manifestations of IBD (8)

Answer 75

- Eye lesions - episcleritis, anterior uveitis - Skin lesions - erythema nodosum (chron's), pyoderma gangrenosum (UC) - Arthritis (most common) - migratory monoarticular arthritis, ankylosing spondylitis (UC), sacroilitis - Thromboembolic-hypercoaguable state - DVT, PE, CVA - Idiopathic trhombocytopenic purpura - Osteoporosis - Gallstones (Chron's with ileal involvement) - Sclerosing cholangitis (UC)

Question 76

Which lipid-lowering agent matches? Side effect: facial flushing

Answer 76

Niacin

Question 77

Which lipid-lowering agent matches? Side effect: elevated LFTs, myositis

Answer 77

Statins, Fibrates

Question 78

Which lipid-lowering agent matches? Side effect: GI discomfort, bad taste

Answer 78

Bile acid binding resins

Question 79

Which lipid-lowering agent matches? Best effect on HDL

Answer 79

Niacin

Question 80

Which lipid-lowering agent matches? Best effect on triglycerides

Answer 80

Fibrates

Question 81

Which lipid-lowering agent matches? Best effect on LDL/cholesterol

Answer 81

Statins

Question 82

Which lipid-lowering agent matches? Binds C.diff toxin

Answer 82

Cholestyramine

Question 83

AHA/ACC guidelines for which patients should be on statin (4)

Answer 83

Clinical ASCVD Type 2 DM and age 40-75 LDL >190 ASCVD risk >7.5%

Question 84

Most appropriate treatment for elevated homocysteine

Answer 84

Vitamin B12 Vitamin B6 Folic acid

Question 85

Medications that negatively affects lipid measurements? (5)

Answer 85

``` Oral estrogens Thiazides β-blockers Protease inhibitors Atypical antipsychotics (i.e. Clozapine, olanzapine) ```

Question 86

MAP ↓ SVR ↑ HR ↑ PCWP ↓

Answer 86

Hypovolemic shock due to ↓ intravascular volume. Causes: hemorrhage/trauma, burns, GI loss (prolonged vomiting/diarrhea) Treatment: address underlying cause, IVF

Question 87

``` MAP ↓ SVR ↑ HR variable PCWP ↑ PCWP after fluid challenge: ↑↑ ```

Answer 87

Cardiogenic shock due to pump failure. Causes: Acute MI, dilated cardiomyopathy, arrhythmia, ruptured septum, papillary muscle rupture Treatment: Dobutamine, intra-aortic balloon pump. ** be careful with fluids to avoid pulmonary edema

Question 88

``` MAP ↓ SVR ↑ HR ↑ PCWP ↓/normal PCWP after fluid challenge: unchanged or ↑ ```

Answer 88

Obstructive shock due to extracardiac causes of pump failure → ↓ venous return and ↓ preload. Causes: tension PTX, hemothorax, pulmonary embolism. Treatment: Treat underlying cause

Question 89

``` MAP ↓ SVR ↑ HR ↑ PCWP ↑ PCWP after fluid challenge: ↑ ```

Answer 89

Obstructive shock due to extracardiac causes of pump failure ↓ venous return and ↓ preload Causes: cardiac tamponade Treatment: Pericardiocentesis

Question 90

``` MAP ↓ SVR ↓ HR ↑ PCWP normal PCWP after fluid challenge: ↑ ```

Answer 90

Septic (distributive) shock from peripheral vasodilation due to infection or due to severe type I hypersensitivity reaction Treatment: Septic - IVF, norepinephrine, antibiotics Anaphylactic - Epinephrine, IVF, airway management, antihistamines, H2 blocker

Question 91

``` MAP ↓ SVR ↓ HR ↓ PCWP normal PCWP after fluid challenge: ↑ ```

Answer 91

Neurogenic (distributive) shock from peripheral vasodilation and bradycardia due to autonomic dysregulation. Consequence of CNS injury. Treatment: IVF, vasopressors, atropine for bradycardia

Question 92

β1 agonist - increased HR and contractility Pure inotrope Reflex dilation Used in cardiogenic shock

Answer 92

Dobutamine

Question 93

α1 agonist - vasoconstriction β1 agonist - ↑ HR and ↑ CO Side effect: peripheral ischemia, mesenteric ischemia, renal insufficiency Used with septic shock

Answer 93

Norepinephrine

Question 94

α1 α2 β1 β2 agonist Low dose - β1 increases CO High dose α1 vasoconstriction. Used with anaphylactic shock, second line in septic shock

Answer 94

Epinephrine

Question 95

Weak vasoconstrictor | Used 2nd line for septic shock or anaphylactic shock

Answer 95

Vasopressin (Anti-diuretic hormone)

Question 96

α1 agonist - vasoconstriction Causes ↑ SVR and ↑ afterload without ↑ contractility → ↓ SV and ↓ CO Used 2nd line for septic shock

Answer 96

Phenylephrine

Question 97

Low dose: β1 agonist High dose: α1 agonist (weaker than norepinephrine) 2nd line for cardiogenic shock. Adjuvant pressor

Answer 97

Dopamine

Question 98

Diagnosis of acute rheumatic fever (JONES PEACE)

Answer 98

Requires recent strep infection + 2 major criteria or 1 major and 2 minor. ``` Major: (JONES) J - Joints - polyarthritis O - Heart; pancarditis N - Nodules; subQ or on extensor surfaces E - Erythema marginatum S - Syndenham chorea ``` ``` Minor: (PEACE) P - Previous rheumatic fever E - ECG with PR prolongation A- Arthralgias C - CRP and ESR elevation E - Elevated temperature ```

Question 99

Duke criteria for bacterial endocarditis

Answer 99

Requires 2 major, 1 major + 3 minor, or 5 minor criteria ``` Major: + serial blood cultures + vegetations/abscess on echo New valvular regurg murmur Blood culture positive for Coxiella brunetii ``` Minor: Predisposing condition/IVDU Temp >38.0 C Vascular phenomenon: arterial emboli, conjunctival hemorrhages, Janeway lesions Immuno phenomenon: glomerulonephritis, Osler nodes, Roth spots + cultures not meeting requirements for major criteria Serologic evidence of infection w/o + culture

Question 100

Empiric antibiotic for infective endocarditis

Answer 100

Vancomycin - covers staph, strep, and enterococci

Question 101

Which drugs block transmission through the AV node? (3)

Answer 101

β-blocker Non-dihydropyridine calcium channel blcoker Digoxin

Question 102

HA, temple tenderness, jaw claudication, vision loss Granuloma Elderly women Associated with polymyalgia rheumatica Disease? Best 1st test? Treatment?

Answer 102

Giant cell arteritis (temporal arteritis) Best 1st test: ESR Tx: Prednisone - high dose 1-2mo, followed by taper up to 12mo. Low dose ASA to reduce risk of vision loss/stroke from vessel occlusion. Vitamin D and Ca to reduce risk of osteoporosis from prolonged high-dose corticosteroid use

Question 103

Malaise, vertigo, syncope, fever, ↓ carotid/limb pulses. Asian women 10-40yo Involves aortic arch Disease? Treatment?

Answer 103

Takayasu arteritis Tx: corticosteroids, immunosuppressive agents, bypass grafting for obstructed vessels

Question 104

Diagnostic criteria for Kawasaki disease (mucocutaneous lymph node syndrome) (CRASH) Treatment??

Answer 104

Fever >104F at least 5 days + 4/5 of the following: Conjunctivitis (bilateral, non-exudative, painless) Rash on the trunk Adenopathy of cervical lymph nodes Strawberry tongue and diffuse erythema of mucous membranes Hands and Feet edema with induration/erythema/desquamation *** Coronary artery aneurysms can occur w/in weeks of the illness Treatment: IVIG (w/in 10days of onset), HIGH DOSE ASA until 48h after fever resolution, followed by low-dose ASA until platelets and ESR return to normal (usually 6w)

Question 105

Fever, HTN, hematuria, anemia, neurpathy, wt loss, joint pain, palpable purpura/ulcers on skin. Associated with Hep B/C ANCA negative Disease? Treatment?

Answer 105

Polyarteritis nodosa - inflammation of sm/med arteries → ischemia Commonly affects Kidneys, GIT **** Spares lungs Tx: Corticosteroids, immunosuppressive agents

Question 106

``` Child with recent URI + Palpable purpura of lower extremities Arthritis/arthralgias Abdominal pain due to intestinal hemorrhage Renal disease due to IgA deposition ``` Disease? Treatment

Answer 106

Henoch-Schonlein purpura - IgA complex-mediated vasculitis affecting arterioles, capillaries, and venules Treatment - frequently self-limited; use corticosteroids for severe GI symptoms

Question 107

Melzer's Triad: Palpable purpura, peripheral neuropathy/weakness, arthralgias. Associated with Hep C Polyclonal IgG and IgM antibodies Associated with renal disease

Answer 107

Mixed cryoglobulinemia syndrome

Question 108

Disease of lung, upper airway, kidneys. | + cANCA

Answer 108

Granulomatosis with polyangiitis (Wegener's)

Question 109

Asthmatic symptoms, fatigue, malaise, mononeuropathy (pain/paresthesia/weakness), erythematous/papular rash +pANCA

Answer 109

Eosiophilic granulomatosis with polyangiitis (Churg-strauss syndrome)

Question 110

K-sparing diuretics (4)

Answer 110

Spironolactone Eplerenone Amiloride Triamterene

Question 111

Caucasian with foul smelling chronic diarrhea + iron deficiency anemia Disease? Treatment?

Answer 111

Whipple disease Tx: TMP-SMX

Question 112

Recent immigrant from the Dominican Republic with foul-smelling chronic diarrhea + macrocytic anemia

Answer 112

Tropical sprue

Question 113

Chronic diarrhea + arthralgias and ataxia

Answer 113

Celiac dz

Question 114

Chronic diarrhea + itchy grouped vesicles on the elbows and knees

Answer 114

Celiac dz

Question 115

Chronic diarrhea + stool sudan stain Normal D-xylose test

Answer 115

Pancreatic disease

Question 116

Vomiting w/in several hours of eating fried rice, diarrhea later Diagnosis? Treatment?

Answer 116

Bacillus cereus Tx: Self-limited; hydration

Question 117

H/o eating poultry Bloody diarrhea, abdominal pain; +/- reactive arthritis Guillian-barre syndrome 2-3 w later. Diagnosis? Treatment?

Answer 117

Campylobacter jejuni 2nd most common foodborne bacterial GI infection Tx: Hydration, generally self-limited. Erythromycin

Question 118

H/o eating honey or home-canned foods. Nausea, vomiting, diarrhea, flaccid paralysis. Neuro symptoms start with cranial neuropathy → bilateral symmetric descending weakness. Diagnosis? Treatment?

Answer 118

Clostridium botulinum Tx: Botulism antitoxin (not given to infants); self-limited

Question 119

Watery or bloody diarrhea, pseudomembranous colitis. H/o recent abx use (esp. clindamycin) Diagnosis? Treatment?

Answer 119

Clostridium difficile Tx: Metronidazole, PO Vancomycin

Question 120

Traveler's diarrhea Watery diarrhea, vomiting, fever Diagnosis? Treatment?

Answer 120

Enterotoxigenic E. coli (ETEC) Tx: Hydration; self limited. May be treated with fluoroquinolone

Question 121

Bloody diarrhea, vomiting, fever, abdominal pain. Risk of hemolytic uremic syndrome. H/o of eating ground beef with indirect fecal contamination. Diagnosis? Treatment?

Answer 121

Enterohemorrhagic E. coli (EHEC) O157:H7 Tx: hydration; self-limited; abx may actually worsen symptoms due to toxin release

Question 122

Vomiting w/in several hours of eating room-temperature food; diarrhea later. Diagnosis? Treatment?

Answer 122

Staphylococcus aureus. Disease caused by consuming preformed toxin. Tx: self-limited; hydration

Question 123

Nausea, abdominal pain, bloody diarrhea, fever, vomiting. H/o eating eggs, poultry, milk, fresh produce. Diagnosis? Treatment?

Answer 123

Salmonella species. Most common foodborne bacterial GI infection. Tx: Hydration; self limited. Treat immunocompromised patients with fluoroquinolone (cipro/levofloxacin)

Question 124

Fever, nausea, vomiting, SEVERE bloody diarrhea, abdominal pain. Risk of HUS H/o consuming contaminated food/water. Increased risk in overcrowded areas. Disease? Treatment?

Answer 124

Shigella species "Bacterial dysentery" Tx: Hydration; cipro/TMP-SMX in severe cases

Question 125

Copious watery ("rice water") diarrhea, signs of dehydration, severe electrolyte imbalance. H/o consuming seafood, contaminated water. Disease? Treatment?

Answer 125

Vibrio cholerae Tx: Hydration; tetracycline/doxycycline decreases disease length

Question 126

Abdominal pain, watery diarrhea w/in 24h of eating seafood (esp oysters). Disease? Treatment?

Answer 126

Vibrio parahaemolyticus Tx: Hydration, self-limited

Question 127

Abdominal pain, bloody diarrhea, RLQ pain (pseudoappendicitis), fever. H/o eating pork/fresh produce or exposure to pet feces. Diagnosis? Treatment?

Answer 127

Yersinia enterocolitica Tx: Hydration; self-limited

Question 128

Greasy, foul-smelling diarrhea; abdominal pain, malaise. Cysts/trophozoites seen in stool. H/o of consumption of surface water (usually in wilderness or other countries) Diagnosis? Treatment?

Answer 128

Giardia lamblia Tx: hydration, metronidazole

Question 129

Mild-severe blood diarrhea, abdominal pin. Cysts and trophozoites seen in stool sample. H/o consumption of water in poor areas of sanitation. Diagnosis? Treatment?

Answer 129

Entamoeba histolytica "Amoebic dysentery" Tx: Metronidazole, paromomycin

Question 130

Watery diarrhea, abdominal pain, malaise; acid-fast stain of stool shows parasites. H/o consuming contaminated food/water or immunocompromised patient. Diagnosis? Treatment?

Answer 130

Cryptosporidium parvum. Tx: manage immunosuppression. Nitazoxanide

Question 131

Fever, myalgias, periorbital eosinophilia. H/o consumption of undercooked pork. Diagnosis? Treatment?

Answer 131

Trichinella spiralis Tx: albendazole, mebendazole if CNS/Cardiac symptoms

Question 132

Nausea abdominal pain. H/o consuming undercooked pork Diagnosis? Treatment?

Answer 132

Taenia solium (Intestinal taeniasis) due to ingestion of cysts Tx: Praziquantel

Question 133

Cysts in muscles, subcutaneous tissues, eyes, extraocular muscles, brain. +/- seizures. Diagnosis? Treatment?

Answer 133

Taenia solium Neurocysticercosis due to fecal/oral transmission of eggs from feces of human with intestinal taeniasis Tx: Albendazole + corticosteroids for CNS sx

Question 134

Which heart sounds are considered benign when there is no evidence of disease? (4)

Answer 134

Split S1 Split S2 on inspiration S3 Early quiet systolic murmur

Question 135

Tumor marker for pancreatic CA

Answer 135

CA 19-9

Question 136

Peptic Ulcer + diarrhea Fasting gastrin level >1000 What radiographic studies? Treatment?

Answer 136

Zollinger ellison syndrome CT, MRI, octreotide scan (aka somatostatin receptor scintigraphy, SRS), endoscopic ultrasound Treatment: Resection if localized, High dose PPI to suppress acid secretion, octreotide

Question 137

Hypoglycemia, elevated fasting insulin level, elevated C-peptide Diagnosis? Treatment?

Answer 137

Insulinoma Tx: surgical resection or diazoxide/octreotide to suppress insulin secretion

Question 138

Hyperglycemia, refractory diabetes, painful and pruritic migratory rash on face and perineum Diagnosis? Treatment?

Answer 138

Glucagonoma Treatment: octreotide to suppress glucagon secretion. Frequently malignant at diagnosis with poor prognosis. FYI: Painful and pruritic migratory rash on face and perineum = necrolytic migratory erythema. Very specific for glucagonoma

Question 139

Most common presentation of VIPoma? | Treatment?

Answer 139

Watery diarrhea Tx: hydration and electrolyte replacement, octreotide to suppress VIP secretion.

Question 140

Which bacterial GI infection is most likely associated with diarrhea and pseudoappendicitis?

Answer 140

Yersinia enterocolitica

Question 141

Female with: Fatigue Pruritus that starts during pregnancy but is not relieved postpartum → excoriations. Skin changes: hyperpigmentation, xerosis, dermatographism Xanthomas and xanthelasmas Steatorrhea Diagnosis? Pathophysiology? Important lab findings? Treatment?

Answer 141

Primary biliary cholangitis (cirrhosis) Pathophysiology: autoimmune destruction of intrahepatic bile ductules → cholestasis → cirrhosis (late) Frequently associated with AI diseases, esp sjogren syndrome, CREST syndrome, RA Labs: ↑ alk phos, ↑ bili (in late disease), ↑ cholesterol, + anti-mitochondrial Ab (>95%, hallmark finding of PBC), + ANA (70%). Treatment: Ursodeoxycholic acid. liver transplant in late disease

Question 142

Male with fatigue, pruritus, excoriations, jaundice, hepatomegaly H/o of ulcerative colitis ``` Diagnosis? Pathophysiology? Important lab findings? Treatment? Complications? ```

Answer 142

Primary sclerosing cholangitis Pathophysiology: progressive inflammation, fibrosis, sclerosis of intra and extra hepatic bile ducts Strongly associated with ulcerative colitis Labs: ↑ alk phos +/- ↑ bilirubin ANA negative pANCA positive. ERCP shows irregular biliary stricturing and dilations ("beads on a string") Tx: no effective medical therapy; liver transplant Complications: ↑ risk of cholangiocarcinoma

Question 143

MOA of lactulose

Answer 143

Non-absorbed sugar that lowers colonic pH, preventing ammonia reabsorption of the GI tract and facilitating fecal ammonia excretion Used for hepatic encephalopathy seen with cirrhosis

Question 144

MOA of Rifaximin

Answer 144

Abx that kills enteric bacteria that generate ammonia Used for hepatic encephalopathy seen with cirrhosis

Question 145

What physical exam findings may be seen in the hands of patients with cirrhosis? (4)

Answer 145

Palmar erythema due to increased estrogen (not metabolized due to liver failure) Digital clubbing Asterixis Dupuytren's Contracture

Question 146

Acute: RUQ pain, hepatomegaly, rapid development of jaundice and ascites Subacute/chronic: gradual development of ascites, LE edema, cirrhosis, and portal HTN over a few months. Eventual: liver failure and hepatic encephalopathy Diagnosis? Treatment?

Answer 146

Budd-Chiari syndrome - diagnosed by ultrasound (best initial test) and hepatic venography (most sensitive and specific) Treatment: Thrombolytics, diuretics, anticoagulation, angioplasty, shunt

Question 147

Causes of Ascites + SAAG (serum-ascites-albumin-gradient) >1.1 (5)

Answer 147

Always due to portal hypertension (low albumin in ascites fluid). ``` Causes: Cirrhosis Alcoholic hepatitis Heart failure/constrictive pericarditis Massive hepatic metastases Budd-chiari syndrome ```

Question 148

Causes of Ascites + SAAG (serum-ascites-albumin-gradient)

Answer 148

Ascites is NOT due to portal HTN. ``` Causes High albumin in ascites - Peritoneal carcinomatosis - Peritoneal tuberculosis - Pancreatitis - Serositis ``` Low serum albumin -Nephrotic syndrome

Question 149

Pt with ascites, fever, AMS, diarrhea, +/- abdominal tenderness. SAAG >1.1. Ascites neutrophil count > 250 Low ascites glucose High ascites LDH Diagnosis? Treatment?

Answer 149

Spontaneous bacterial peritonitis - infection of ascitic fluid without surgically treatable intra-abdominal source (i.e. perforation) Treatment: Cefotaxime or ceftriaxone for at least 5 days to cover for gut bacteria, staph, and strep. IV Albumin - maintains plasma volume → preserves renal function → reduces renal impairment and mortality

Question 150

Paraneoplastic syndromes seen with hepatocellular carcinoma (5)

Answer 150

Polycythemia - due to secretion of EPO Hypercalcemia - due to secretion of PTH-related peptide Watery diarrhea - due to secretion of VIP and other factors Hypoglycemia - due to high metabolic needs Skin lesions

Question 151

4 causes of polycythemia as a paraneoplastic syndrome

Answer 151

POTENTIALLY REALLY HIGH HCT Pheochromocytoma Renal cell carcinoma Hepatocellular carcinoma Hemangioblastoma

Question 152

Seronegative spondyloarthropathies (4)

Answer 152

``` PAIR Psoriatic arthritis Ankylosing spondylitis IBD-associated arthritis Reactive arthritis ``` *** all share arthritis, + HLAB27, and anterior uveitis

Question 153

+ ANA (95%) + Anti-dsDNA (60%) + Anti-sm or Anti-smith Ab False + RPR or VDRL

Answer 153

SLE

Question 154

+ anti-histone Ab | + ANA

Answer 154

Drug-induced lupus

Question 155

Common causes of drug-induced lupus (5)

Answer 155

SHIPP ``` Sulfa drugs Hydralazine INH Phenytoin Procainamide ```

Question 156

+ RF (75%) + ACPA (Anti-citrullinated peptide Ab) + ANA (

Answer 156

RA

Question 157

+ ANA | + Anti-Jo-1

Answer 157

Polymyositis or Dermatomyositis

Question 158

+ Anti-Scl-70 ANA aka Anti-DNA topoisomerase

Answer 158

Scleroderma

Question 159

+ Anticentromere Ab

Answer 159

CREST syndrome

Question 160

+ Anti-RNP U1 ANA

Answer 160

Mixed connective tissue disease

Question 161

+ Anti-Ro (Anti-SSA) | + Anti-La (Anti-SSB)

Answer 161

Sjogren Syndrome

Question 162

What primary cancers frequently metastasize to the bones? (6)

Answer 162

"Permanently Relocated Tumors Like Bones" ``` Prostate Renal cell cancer Testes and Thyroid Lung Breast ```

Question 163

``` Most common malignant primary bone tumor Teen boys or adults > 65 Rb gene mutation Prior chemo/radiation Commonly distal femur/proximal tibia Codman's triangle due to elevation of periosteum on XR Sunburst pattern on XR ```

Answer 163

Osteosarcoma

Question 164

``` Multinucleated giant cells ("osteoclastoma") Young adults (20-30yo) Distal femur/proximal tibia Benign but locally destructive "Soap bubble" appearance on XR ```

Answer 164

Giant Cell Tumor

Question 165

Most common benign bone tumor | Males

Answer 165

Osteochondroma Treatment: Surgical excision only for soft tissue irritation or severe bone deformity

Question 166

Blow to abducted, externally rotated, extended arm (blocking a basketball shot) PE: prominent acromion, arm held in external rotation and slight abduction. Diagnosis? Complication?

Answer 166

Anterior shoulder dislocation | Axillary artery and nerve are at risk

Question 167

Blow to internally rotated, adducted shoulder (i.e. seizure/electrocution). PE: arm held in internal rotation and adduction, unable to externally rotate.

Answer 167

Posterior shoulder dislocation

Question 168

What nerve injury? Patient asked to extend all fingers 4th and 5th digits are hyperextended with IP joints held in flexion

Answer 168

Ulnar claw (ulnar nerve) - innervates 3rd and 4th lumbricals, interosseous, adductor pollicis

Question 169

What nerve injury? Loss of thenar muscles and inability to abduct the thumb.

Answer 169

Ape hand - median nerve due to injury of the elbow

Question 170

What nerve injury? Patient asked to flex all fingers, 2nd and 3rd digits remain extended.

Answer 170

Hand of benediction (Pope's blessing) - median nerve injury above the elbow.

Question 171

What nerve injury? Inability to extend the wrist - wrist drop

Answer 171

"Saturday night palsy" - radial nerve injury due to compression against the spiral groove of the humerus

Question 172

Fall on outstretched hand, posterior displacement of distal radius, forearm profile looks like a dinner fork. +/- ulnar fracture. Type of fracture? Treatment?

Answer 172

Colles fracture Tx: Closed reduction, long arm cast (cover joints above and below), possible surgery ** most common wrist fracture, more likely in osteoporotic bone

Question 173

Fall on flexed wrist, distal radius is anteriorly (volarly) displaced. Type of fracture? Treatment?

Answer 173

Smith fracture | Tx: Closed reduction, casting, possible surgery

Question 174

Fall on radially deviated out stretched hand. What fracture are you worried for? Complications? Treatment?

Answer 174

Scaphoid fracture (snuffbox tenderness). * Increased risk of AVN * Not seen on XR for 1-2 weeks after injury Most common carpal bone fracture Tx: Thumb spica cast, possible surgery

Question 175

5th metacarpal neck fracture following punching hard object or surface with strong force. Type of fracture? Treatment? Complications?

Answer 175

Boxer's fracture Tx: Closed reduction, ulnar gutter splint, possible surgical pinning. Complication: "fight bite" - open wounds from teeth will need surgical exploration to r/o tendon involvement. *abx treatment

Question 176

Fall on outstretched arm with arm hyperpronated. Fracture of proximal 1/3 of ulna with dislocation of radial head. Name of fracture? Treatment?

Answer 176

Monteggia fracture | Tx: Closed reduction of radial head, surgical repair of ulna

Question 177

Fracture of distal shaft of radius and dislocation of distal radius ulnar joint (DRUJ) Name of fracture? Treatment?

Answer 177

Galeazzi fracture | Tx: Surgical repair, cast forearm in supination to maintain reduction of DRUJ

Question 178

Complication of tibia fracture?

Answer 178

Compartment syndrome

Question 179

Most common mechanism of injury to the ankle

Answer 179

Supination and external rotation

Question 180

What nerve is injured? Scapular winging

Answer 180

Long thoracic

Question 181

What nerve is injured? Unable to wipe bottom

Answer 181

Thoracodorsal

Question 182

What nerve is injured? Loss of shoulder abduction

Answer 182

Axillary

Question 183

What nerve is injured? Loss of elbow flexion and forearm supination

Answer 183

Musculocutaneous

Question 184

What nerve is injured? Trouble initiating shoulder abduction

Answer 184

Suprascapular

Question 185

What nerve is injured? Unable to abduct arm beyond 10 degrees

Answer 185

Axillary

Question 186

What nerve is injured? Unable to raise arm above horizontal

Answer 186

Long thoracic and spinal accessory

Question 187

What nerve is most at risk? Fracture of shaft of humerus

Answer 187

Radial

Question 188

What nerve is most at risk? Fracture of surgical neck of humerus

Answer 188

Axillary

Question 189

What nerve is most at risk? Supracondylar humerus fracture

Answer 189

Median

Question 190

What nerve is most at risk? Fracture of medial epicondyle

Answer 190

Ulnar

Question 191

What nerve is most at risk? Anterior shoulder dislocation

Answer 191

Axillary

Question 192

What nerve is most at risk? Injury to carpal tunnel

Answer 192

Median

Question 193

Arm extended and adducted with pronated forearm. Site of injury? Mechanism of injury?

Answer 193

AKA Waiter's tip Superior trunk of the brachial plexus due to hyperadduction of the arm causing widening of the humeral-glenoid gap.

Question 194

Weak finger adduction, poor 4th/5th finger flexion, clawed 4th/5th fingers from lumbrical weakness. Site of injury? Mechanism of injury?

Answer 194

Ulnar nerve of brachial plexus due to epiphyseal separation of medial epicondyle of humerus

Question 195

Inability to extend wrist or fingers; loss of sensation from dorsal hand. Site of injury? Mechanism of injury?

Answer 195

Posterior cord or radial nerve of brachial plexus due to mid-humerus fracture causing nerve impingement or tear

Question 196

Impaired shoulder abduction or elevation. Site of injury? Mechanism of injury?

Answer 196

Deltoid paralysis due to axillary nerve damage. Anterior shoulder dislocation causes axillary nerve impingement or stretching

Question 197

Claw hand, poor wrist and hand function +/- horner syndrome. Site of injury? Mechanism of injury?

Answer 197

Klumpke palsy due to damage of posterior or medial cords. caused by hyperabduction of arm placing excess tension on lower cords and nearby sympathetic chain.

Question 198

Causes of microcytic anemia (MCV

Answer 198

``` Iron deficiency Lead poisoning Sideroblastic anemia Thalassemia Anemia of chronic disease (late in disease) ```

Question 199

Causes of normocytic anemia (MCV 80-100fL) (3)

Answer 199

Anemia of chronic disease (early in disease) Hemolysis Hemorrhage

Question 200

Causes of macrocytic anemia (MCV >100fL) (4)

Answer 200

Folate deficiency B12 deficiency Liver disease Alcohol abuse

Question 201

Pt presents with: fatigue, SOB/exercise intolerance, tachycardia, conjunctival pallor, ice pica, angular stomatitis, nail spooning. ``` Labs: ↓ Hgb and Hct ↓ MCV ↓ MCH and MCHC ↓ serum iron ↓ ferritin ↑ transferrin ↓ % transferrin saturation. ``` Diagnosis? Etiologies: (4) Treatment?

Answer 201

Microcytic anemia due to iron deficiency. Etiologies: Chronic blood loss (most common), inadequate iron intake, poor iron absorption, pregnancy Tx: Fe supplement, determine underlying cause

Question 202

Pt presents with: fatigue, dyspnea, pallor, abdominal pain and constipation, headache, irritability, short-term memory loss, peripheral neuropathy (i.e. foot drop/wrist drop), gingival lines. ``` Labs: ↓ Hgb and Hct ↓ MCV Peripheral smear: Basophilic stippling Normal iron studies (serum iron, transferrin, transferrin saturation, ferritin) ``` Diagnosis? Treatment?

Answer 202

Microcytic anemia due to lead poisoning. Treatment: Adults - EDTA Children - add dimercaprol

Question 203

Pt presents with fatigue, dyspnea, pallor. Labs: ↓ Hgb and Hct ↓ MCV Peripheral smear: microcytic, hypochromic anemia Bone marrow biopsy: ringed sideroblasts (nucleated RBC precursors with iron granules encircling the nucleus) Diagnosis? Etiologies? Treatment?

Answer 203

Sideroblastic anemia - defective heme synthesis. Inherited - genetic defect in heme synthesis pathway Acquired - chronic alcohol abuse, INH Treatment: Inherited - Vitamin B6 supplement (pyridoxine) Acquired - address underlying cause. RBC Transfusions, EPO administration. Chelation (i.e. deferoxamine) or phlebotomy PRN iron overload

Question 204

What causes the O2 dissociation curve to shift to the left? (5)

Answer 204

Shift to the left = higher affinity of Hgb for O2 at given PO2 → ↓ O2 delivery to tissues. Causes: Alkalosis, ↓ temperature, ↓ CO2, ↓ 2,3 BPG, Hgb F

Question 205

What causes the O2 dissociation curve to shift to the right? (6)

Answer 205

Shift to the right = lower affinity of Hgb for O2 at given PO2 → ↑ O2 delivery to tissues Causes: Acidosis, ↑ body temperature, high altitude, exercise, ↑ CO2, ↑ 2,3 BPG

Question 206

Microcytic anemia Normal iron studies Peripheral smear shows target cells Diagnosis? Treatment?

Answer 206

α or β thalassemia May or may not be symptomatic If symptomatic, may require transfusion Significant # of transfusions → iron overload → requires iron chelation (deferoxamine)

Question 207

Acidosis, hypoxia, or dehydration → episodes of acute pain - chest pain, stroke, dactylitis, osteonecrosis of humeral/femoral head, priapism. XR: "hair-on-end" appearance Diagnosis? Treatment?

Answer 207

Sickle cell crisis Diagnosis: peripheral smear shows sickling of RBCs, Hgb electrophoresis shows Hgb S and increased Hgb F. Hair-on-end XR due to marrow hyperplasia. Tx: IVF, supplemental O2, opioids Folic acid supplement to optimize bone marrow production of RBCs Hydroxyurea to increased Hgb F production Prophylactic PCN until age 5 to prevent pneumococcal infection

Question 208

What immunizations are required in sickle cell patients, especially those who have had autosplenectomy? (4)

Answer 208

``` PLEASE HIT MY INFECTIONS Pneumococcal Hib Meningococcal annual Influenza ```

Question 209

H/o fatigue, dyspnea, tachycardia, tachypnea, pallor/jaundice Labs: ↓ Hgb and Hct, normal MCV, ↑ indirect bili, ↑ LDH, ↓ serum haptoglobin, ↑ reticulocytes Peripheral smear: Burr cells, Schistocytes Direct Coombs: + Diagnosis? Pathology? Treatment?

Answer 209

Drug-induced hemolytic anemia. Drugs bind to RBC membrane → oxidative destruction → induce production of anti-drug Ab → formation of immune complexes that fix complement or induce anti-Rh antibodies. Common causes: recent PCN, methyldopa, quinidine, cephalosporins, NSAIDs Tx: Stop offending agent

Question 210

H/o fatigue, dyspnea, tachycardia, tachypnea, pallor/jaundice Labs: ↓ Hgb and Hct, normal MCV, ↑ indirect bili, ↑ LDH, ↓ serum haptoglobin, ↑ reticulocytes Peripheral smear: spherocytes or RBC agglutination Direct Coombs: + Diagnosis? Pathology? Treatment?

Answer 210

Immune-medicated hemolytic anemia. Anti-RBC Ab, autoimmune disease, or drug-induced. Warm-reacting Ab (IgG) or cold-reacting Ab (IgM). Cold agglutinins - form immune complexes that are snowflake shaped. Occur regularly with EBV, mycoplasma, and some malignancies. Circulation to a cold extremity → IgM binds RBC Ag → complement fixation → RBC lysis Tx: Warm agglutinin disease - corticosteroids. Cold agglutinin disease - avoid cold exposure. Drug-induced - stop offending agent. Splenectomy may be needed in persistent cases.

Question 211

Possible causes for warm-agglutinin hemolytic anemia (4)

Answer 211

Viral - HIV, EBV Lupus Malignancy - CLL, non-hodgkin's lymphoma Congenital immune abnormalities

Question 212

Direct vs indirect coombs test

Answer 212

Direct - prepared anti-human Ab added to patient RBC's to detect presence of immunoglobulins already present on RBCs Indirect - patient's serum incubated with normal RBCs to detect presence of circulating Ab. Positive when Ab to foreign RBCs are present

Question 213

H/o fatigue, dyspnea, tachycardia, tachypnea, pallor/jaundice Labs: ↓ Hgb and Hct, normal MCV, ↑ indirect bili, ↑ LDH, ↓ serum haptoglobin, ↑ reticulocytes Peripheral smear: Schistocytes Direct Coombs: negative Diagnosis? Pathology? Treatment?

Answer 213

Mechanical hemolytic anemia due to prosthetic heart valve, HTN, coagulation disorder RBCs broken by force or turbulent flow Treat underlying cause

Question 214

H/o fatigue, dyspnea, tachycardia, tachypnea, pallor/jaundice + HEPATOSPLENOMEGALY Labs: ↓ Hgb and Hct, normal MCV, ↑ indirect bili, ↑ LDH, ↓ serum haptoglobin, ↑ reticulocytes Peripheral smear: Spherocytes - no pale, central clearing Direct Coombs: negative Diagnosis? Pathology? Treatment?

Answer 214

Hereditary spherocytosis - genetic defect of RBC membranes resulting in spherical RBCs ** RBC's are smaller in size, however ↑ reticulocytes makes MCV normal. Tx: Splenectomy → howell-jolly bodies

Question 215

H/o fatigue, dyspnea, tachycardia, tachypnea, pallor/jaundice, fatigue, dark urine, back/abdominal pain Labs: ↓ Hgb and Hct, normal MCV, ↑ indirect bili, ↑ LDH, ↓ serum haptoglobin, ↑ reticulocytes Peripheral smear: Degmacytes ("bite" cells), heinz bodies Direct Coombs: negative Diagnosis? Pathology? Treatment?

Answer 215

G6PD deficiency - required enzyme to repair oxidative damage to RBCs. Tx: avoid oxidants

Question 216

Causes of hemolysis due to G6PD deficiency (7)

Answer 216

Spleen Purges Nasty Inclusion From Damaged Cells ``` Sulfonamides Primaquine Nitrofurantoin INH Fava beans Dapsone Chloroquine ```

Question 217

Urticaria DURING blood transfusion. Cause? Treatment?

Answer 217

Caused by IgE Ab against soluble Ag in donor plasma. Tx: Diphenhydramine; may continue transfusion

Question 218

Shock/hypotension, angioedema, respiratory failure SECONDS TO MINUTES after starting blood transfusion. Cause? Treatment?

Answer 218

Anaphylactic reaction Possibly due to anti-IgA IgG Ab in patients with IgA deficiency. Tx: Epinephrine, IVF, O2

Question 219

Fever, chills, malaise starting 1-6 HOURS after blood transfusion. Cause? Treatment?

Answer 219

Nonhemolytic febrile reaction. Due to cytokines produced by stored donor cells. Tx: APAP to control fever

Question 220

Hemolysis, fever, chills, tachycardia, tachypnea, hypotension onset DURING blood transfusion Cause? Treatment?

Answer 220

Acute hemolytic reaction due to ABO incompatibility Tx: Aggressive supportive care

Question 221

Mild fever, hemolysis, ↑ direct bilirubin, onset 2-10 days after blood transfusion. Cause? Treatment?

Answer 221

Delayed hemolytic reaction Caused by Anti-Kidd or Anti-D (Rh) Ab. No therapy needed

Question 222

Possible causes of Vitamin B12 deficiency (7)

Answer 222

1. Pernicious anemia - Auto Ab against gastric parietal cells or intrinsic factor (IF) 2. Chronic gastritis → impaired IF production and gastric acid secretion 3. Malabsorption (i.e. Chron's, pancreatic dz) 4. GI bypass/resection → bacterial overgrowth in blind loop of jejunum 5. Resection of terminal ileum (site of absorption of IF-B12 complex) 6. D. latum infection (fish tapeworm) 7. Inadequate B12 intake (strict vegans)

Question 223

History of fatigue, pallor, dyspnea, loss of vibration/proprioception, weakness/paresthesias/ataxia in lower extremities, dementia and memory loss, atrophic glossitis. Labs: ↑ MCV, ↑methylmalonic acid (MMA), ↑ homocysteine. Diagnosis? Treatment?

Answer 223

Megaloblastic anemia due to Vitamin B12 deficiency Tx: IM B12 shots, oral supplements, or nasal spray

Question 224

History of pallor, dyspnea, fatigue, atrophic glossitis. Labs: ↑ MCV, normal methylmalonic acid (MMA), ↑ homocysteine. Diagnosis? Causes? Definitive lab test? Management?

Answer 224

Megaloblastic anemia due to Folate deficiency Caused by inadequate folate intake, most commonly alcoholics or poor nutrition Definitive lab test: ↓ RBC folate level (shows folate levels over period of time, vs serum folate level) Management: PO folate supplements *** always evaluate for concurrent B12 deficiency, because correcting folate level will not change neuro symptoms.

Question 225

H/o easy bruising, mucosal bleeding, skin bleeding, menorrhagia Labs: ↑ PTT, ↑ bleeding time, normal platelet count, ↓ ristocetin cofact activity Diagnosis? Management?

Answer 225

von Willebrand disease Normal vWF: released by injured vascular endothelial cells → enables platelet adhesion and aggregation and stabilizes factor VIII Management: DDAVP (induces vWF release from endothelial cells) is first line for acute bleeding. vWF concentrate for severe/refractory bleeding or before surgery OCPs for menorrhagia Avoid ASA and other anti-platelet drugs

Question 226

H/o hemarthrosis, hematuria, GI bleed, intracranial hemorrhage (rare), mucosal bleeding, epistaxis, bleeding following minor trauma Labs: ↑ PTT, normal PT/bleeding time/platelet count Diagnosis? Management?

Answer 226

Hemophilia A or B Management: Hemophilia A: give factor VIII, or DDAVP will increase factor VIII levels Hemophilia B: give factor IX

Question 227

H/o easy bruising, mucosal bleeding, melena, hematuria. Possible intracranial hemorrhages in newborns. Labs: ↑ PT/INR, normal PTT (↑ if disease is severe) Diagnosis? Management?

Answer 227

Vitamin K deficiency Management: Vitamin K (oral or IM) -- IM given to all newborns due to insufficient bacterial colonization of the GI tract and insufficient vitamin K in breast milk Treat acutebleeding with FFP

Question 228

Causes for DIC (6)

Answer 228

``` STOP Making Thrombi Sepsis Trauma OB complications (i.e. amniotic fluid embolism) Pancreatitis Malignancy Transfusion ```

Question 229

Inherited hypercoaguable states? (5)

Answer 229

``` Factor V Leiden (most common, unable to inactivate Protein C) Prothrombin 20210 gene mutation Antithrombin deficiency Protein C deficiency Protein S deficiency ```

Question 230

Child age 2-5 with Trisomy 21 presenting with bone pain, fever, LAD, fatigue, recurrent infections. Labs: ↑ lymphoblasts that are PAS+ and TdT+. Anemia and thrombocytopenia. Diagnosis?

Answer 230

Acute lymphocytic leukemia (ALL)

Question 231

Adult male, approx 65yo presenting with fatigue, easy bruising, recurrent infections. Labs: pancytopenia, ↑ myeloblasts that are myeloperoxidase + and have auer rods. Diagnosis?

Answer 231

Acute myeloid leukemia (AML)

Question 232

Adult approx 50yo with history of exposure to radiation. Presents with fatigue, splenomegaly, bleeding, anemia, thrombocytosis (however platelets are defective), leukocytosis. Diagnosis? Treatment?

Answer 232

Chronic myeloid leukemia (CML) - due to t(9;22), bcr-abl (Philadelphia chromosome) - Constitutively active tyrosine kinase in tumor cells Risk of blast crisis (smear has >20% blasts, with high mortality). Tx: Hematopoietic cell (bone marrow) transplant. Tyrosine kinase inhibitor (imatinib, dasatinib, nilotinib) → triggers apoptosis

Question 233

Elderly adult (usually asymptomatic), may present with fever, night sweats, fatigue, LAD, HSM. Labs: SEVERE leukocytosis (50-100K), "smudge cells" seen on peripheral smear. Diagnosis? Management?

Answer 233

Chronic lymphocytic leukemia (CLL) Management: Periodic monitoring Chemo/radiation as needed ** Very indolent leukemia, and because patients are usually very old and often asymptomatic, treatment is not necessary

Question 234

H/o normocytic anemia, bone pain, back pain, pathologic fractures, constipation, abdominal pain, encephalopathy, polyuria, dehydration, renal failure. Diagnosis? What important labs do you order? Management?

Answer 234

Multiple myeloma Labs: SPEP - look for monoclonal Ab spike ("M spike") UPEP - look for bence jones proteins Skeletal survey - look for lytic lesions due to local osteolytic factors * Renal disease: light chain cast nephropathy aka "Myeloma kidney" * Hypercalcemia due to lytic bone lesions cause: constipation, abdominal pain, encephalopathy, polyuria, dehydration Management: Chemotherapy (lenalidomide) Bone marrow transplant

Question 235

Large binucleated cells with prominent nucleoli and clearing around the cell, among background of reactive lymphocytes and granulocytes

Answer 235

Reed-sternberg cells seen with hodgkin lymphoma

Question 236

Most common subtype of hodgkin lymphoma

Answer 236

Nodular sclerosis

Question 237

Most common subtype of non-hodgkin lymphoma

Answer 237

Diffuse large B cell lymphoma

Question 238

Most common subtype of non-hodgkin lymphoma in children

Answer 238

Lymphoblastic lymphoma

Question 239

Non-hodgkin lymphoma with t(14;18) and "cleaved cells"

Answer 239

Follicular lymphoma

Question 240

Non-hodgkin lymphoma with t(8;14), "starry sky" appearance

Answer 240

Burkitt lymphoma (think 8urki14) 3 forms: Sporadic Endemic (EBV in Africa) Immunodeficiency-associated (HIV)

Question 241

Non-hodgkin lymphoma where malignant cells are identical to those in CLL, but confined to a tumor mass

Answer 241

Small lymphocytic lymphoma

Question 242

Visual disturances (blurred vision, amaurosis fugax, scintillating scotoma, opthalmic migraine) Thrombosis (CVA, MI, DVT, PE, Budd-chiari, superficial thrombophlebitis) Erythromelalgia - burnng pain in hands/feet with erythema/pallor/cyanosis Pruritus (esp after warm bath) Facial plethora Hepatosplenomegaly Diagnosis? Management?

Answer 242

Polycythemia vera Management: Phlebotomy to keep HCT 70yo, prior thrombosis, Platelets >1500K, hx of CV risk factors) ASA daily to prevent thrombosis

Question 243

What artery supplies medial and superior surfaces of the frontal lobes of the brain?

Answer 243

Anterior cerebral artery (ACA)

Question 244

What artery supplies lateral surfaces and temporal lobes of the brain?

Answer 244

Middle cerebral artery (MCA)

Question 245

What artery supplies the inferior surfaces and occipital lobes of the brain?

Answer 245

Posterior cerebral artery (PCA)

Question 246

What artery supplies the midbrain and brainstem (pons)?

Answer 246

Basilar artery

Question 247

What artery supplies the brainstem (pons) and parts of the cerebellum?

Answer 247

Anterior inferior cerebellar artery (AICA)

Question 248

What artery supplies the brainstem (medulla) and parts of the cerebellum?

Answer 248

Posterior inferior cerebellar artery (PICA)

Question 249

Spastic (UMN) and flaccid (LMN) paralysis. Diagnosis? Tracts affected?

Answer 249

Amyotrophic lateral sclerosis Affects corticospinal tract, ventral horn

Question 250

Flaccid paralysis Diagnosis? Tracts affected?

Answer 250

Poliomyelitis Ventral horn

Question 251

Impaired proprioception, pain Diagnosis? Tracts affected?

Answer 251

Tabes dorsalis (tertiary syphilis) Dorsal columns

Question 252

Bilateral loss of pain and temp 1 level below lesion Bilateral spastic paresis below lesion Bilateral flaccid paralysis at level of lesion Diagnosis? Tracts affected?

Answer 252

Spinal artery syndrome CST, STT, ventral horn, lateral gray matter *** Spinal artery supplies all of the spinal cord except dorsal columns

Question 253

Bilateral loss of vibration and discrimination Bilateral spastic paresis affecting legs before arms Diagnosis? Tracts affected?

Answer 253

Vitamin B12 deficiency Dorsal columns, CST

Question 254

Bilateral loss of pain and temp 1 level below lesion Bilateral flaccid paralysis at level of lesion Diagnosis? Tracts affected?

Answer 254

Syringomyelia Ventral horn, ventral white commissure

Question 255

Ipsilateral loss of vibration/discrimination below lesion Ipsilateral spastic paresis below lesion Ipsilateral flaccid paralysis at level of lesion Contralateral loss of pain and temp below lesion Diagnosis? Tracts affected?

Answer 255

Brown-Sequard syndrome ALL tracts on 1 side of cord *** STT decussates immediately

Question 256

What nerve innervates muscles of mastication?

Answer 256

CN V (Trigeminal)

Question 257

What nerve innervates sensation to anterior 2/3 of tongue?

Answer 257

CN VII (Facial)

Question 258

What nerve innervates muscles of facial expression?

Answer 258

CN VII (Facial)

Question 259

What nerve innervates stapedius muscle?

Answer 259

CN VII (Facial)

Question 260

What nerve innervates lacrimal, submandibular, and sublingual glands?

Answer 260

CN VII (Facial)

Question 261

What nerve innervates sensation to posterior 1/3 of tongue?

Answer 261

CN IX (Glossopharyngeal)

Question 262

What nerve innervates parotid gland?

Answer 262

CN IX (Glossopharyngeal)

Question 263

What nerve innervates motor to tongue?

Answer 263

CN XII (Hypoglossal)

Question 264

Motor/sensory deficits of lower extremities and trunk. What area of the brain? Supplied by what artery?

Answer 264

Medial cortex (frontal and parietal lobes) Anterior cerebral artery

Question 265

Motor/sensory deficits of face and upper extremity, aphasia. What area of the brain? Supplied by what artery?

Answer 265

Lateral cortex (parietal and temporal lobes) Middle cerebral artery (MCA)

Question 266

Impaired vision What area of the brain? Supplied by what artery?

Answer 266

Occipital lobe Posterior cerebral artery (PCA)

Question 267

Patient understands language but can't generate language - "nonfluent". Affects both spoken and written language). + face and arm hemiparesis, loss of oral coordination. Good comprehension. What type of aphasia? Where is the lesion?

Answer 267

Broca aphasia/Expressive aphasia Inferior frontal gyrus, dorsolateral frontal cortex, anterior parietal cortex.

Question 268

Patient can generate words/sentences but can't understand what is said to them; language generated is often incomprehensible -- word substitutions, meaningless words, meaningless phrases, poor comprehension, "word salad". What type of aphasia? Where is the lesion?

Answer 268

Wernicke aphasia/Receptive aphasia Posterior superior temporal gyrus, inferior parietal lobe

Question 269

Patient has fluent speech, frequent attempts to correct words, word-finding pauses, can't repeat what is said to them. Language comprehension and generation are intact. What type of aphasia? Where is the lesion?

Answer 269

Conduction aphasia Supramarginal gyrus, angular gyrus

Question 270

Non-fluent speech + poor comprehension + poor repetition. Limb ataxia. What type of aphasia? Where is the lesion?

Answer 270

Global aphasia Large infarcts of left cerebral hemisphere

Question 271

Loss of pain/temp on contralateral side of body. Loss of pain/temp on ipsilateral side of the face Cerebellar defects (i.e. ataxia, past-pointing) Where is the lesion? What artery supplies this area?

Answer 271

Wallenberg syndrome Lesion of the posterior inferior cerebellar artery, supplying the inferior cerebellum and lateral medulla.

Question 272

Contralateral hemispatial neglect. Where is the lesion?

Answer 272

Non-dominant parietal lobe (usually the right)

Question 273

Gerstmann syndrome -- Agraphia, acalculia, finger agnosia Where is the lesion?

Answer 273

Dominant parietal lobe (usually the left)

Question 274

Personality changes. Where is the lesion?

Answer 274

Frontal lobe

Question 275

Kluver Bucy syndrome -- disinhibition, loss of fear, hyperorality/hyperphagia, hypersexuality Where is the lesion?

Answer 275

Bilateral amygdalae

Question 276

Hemiballismus Where is the lesion?

Answer 276

Contralateral subthalamic nucleus

Question 277

Most common organisms for bacterial meningitis in patient

Answer 277

E. coli GBS Listeria Ampicillin + Gentamycin +/- Cefotaxime

Question 278

Most common organisms for bacterial meningitis in patient 1-3mo of age? (4) Empiric abx for these organisms?

Answer 278

GBS E. coli Streptococcus pneumoniae Neisseria meningitidis 3rd gen cephalosporin (cefotaxime/ceftriaxone) + Vancomycin

Question 279

Most common organisms for bacterial meningitis in patient 3mo-50yo? (2) Empiric abx for these organisms?

Answer 279

Streptococcus pneumoniae Neisseria meningitidis 3rd gen cephalosporin (cefotaxime/ceftriaxone) + Vancomycin

Question 280

Most common organisms for bacterial meningitis in patient >50yo? (3) Empiric abx for these organisms?

Answer 280

Streptococcus pneumoniae Listeria Neisseria meningitidis Ampicillin + 3rd gen cephalosporin (cefotaxime/ceftriaxone) Vancomycin

Question 281

What antibiotic prophylaxis do you give to close contacts of patients with Neisseria meningitidis meningitis? (3)

Answer 281

Ciprofloxacin Rifampin Ceftriaxone

Question 282

Pt with meningeal signs + history of head trauma, clear fluid draining from nose (CSF leak). Most likely organism? Treatment?

Answer 282

Meningitis due to streptococcus pneumoniae Tx: 3rd gen cephalosporin (cefotaxime/ceftriaxone), Vancomycin

Question 283

Pt with meningeal signs + recent sinus/ear infection. Most likely organism? Treatment?

Answer 283

Meningitis due to streptococcus pneumoniae Tx: 3rd gen cephalosporin (cefotaxime/ceftriaxone), Vancomycin

Question 284

Pt with meningeal signs + recent neuro surgery -- especially one where hardware (i.e. shunt) was placed Most likely organisms (3)? Empiric treatment?

Answer 284

Coag-negative staph S. aureus GNR (i.e. pseudomonas) Empiric treatment: Vancomycin, cefepime/meropenem

Question 285

When do you give dexamethasone as part of treatment for meningitis (3)?

Answer 285

``` Child with Hib meningitis Strep pneumo meningitis TB meningitis *** Only give if 1st dose is before or during first dose of antibiotics. Contraindicated if ```

Question 286

Meningeal signs in patient with h/o severe immunocompromise (i.e. HIV) Most likely organism? How do you diagnose? Treatment?

Answer 286

Cryptococcal meningitis Rapid Ag detection in CSF, or india ink stain Tx: Amphotericin B + Flucytosine x2w followed by Fluconazole x8w

Question 287

Meningeal signs, CSF stain shows gram-positive diplococci

Answer 287

S. pneumo

Question 288

Meningeal signs, CSF stain shows gram-negative diplococci

Answer 288

Neisseria meningitidis

Question 289

Meningeal signs, CSF stain shows small gram-negative coccobacilli

Answer 289

Haemophilus influenzae

Question 290

Meningeal signs, CSF shows gram-positive rods

Answer 290

Listeria

Question 291

Decreased movement, postural instability, festinating gait, pill rolling tremor, cogwheel rigidity, mask-like facies, autonomic dysfunction, orthostatic hypotension, cognitive dysfunction, dementia, depression, apathy. Diagnosis? Pathophysiology? Most common treatment?

Answer 291

Parkinson disease Degeneration of dopaminergic neurons in substantia nigra → ↓ DA with relative ↑ Ach +/- Lewy bodies: eosinophilic inclusions of α-synuclein and ubiquitin Most common tx: Carbidopa/Levodopa

Question 292

MAO-B inhibitor used in early parkinsons for neuroprotective effects

Answer 292

Seligiline

Question 293

COMT inhibitors to potentiate levodopa in parkinsons

Answer 293

Entacapone, tolcapone

Question 294

DA agonists (3) used with parkinsons

Answer 294

Bromocriptine (ergot) Non-ergot D3 stimulators: pramipexole, ropinirole, rotigotine (transdermal) Apomorphine (subQ) - for sudden akinetic episodes

Question 295

Anticholinergics used for tremor in parkinsons

Answer 295

Trihexyphenidyl, benztropine

Question 296

``` CAG repeat on chromosome Cuatro (4) aCetylCholine and GABA are decreased Choreiform movements Cognitive decline Caudate nucleus atrophy on MRI age Cuarenta (40) ``` What disease?

Answer 296

Huntington disease

Question 297

Asymmetric limb weakness in hands/fingers/shoulders/lower extremity (foot drop)/pelvic girdle +/- bulbar dysfunction (dysarthria/dysphagia) Progressizes to spastic paralysis, slowed rapid alternating movements, inappropriate laughgin/crying, weakness, gait disorder, flaccid paralysis. Diagnosis? Definitive diagnosis? Treatment?

Answer 297

Amyotrophic lateral sclerosis (ALS) Testing: EMG shows widespread acute and chronic muscular denervation and reinnervation Tx: Riluzole - slows progression of ALS and prolongs survival by decreasing glutamate-induced excitotoxicity

Question 298

30yoF: Neck flexion → electrical shock-like tingling radiating down back and into extremities Also complaining of diplopia, ptosis, urinary incontinence, eye pain Diagnosis? Gold standard for diagnosis? CSF findings? Treatment (acute and maintenance)

Answer 298

MS Lhermitte's sign (neck flexion causes shock-like tingling radiating down back/extremities) Gold standard: MRI of brain/orbits/spine shows demyelinated white matter lesions CSF shows oligoclonal bands Acute tx: high-dose glucocorticoids (methylprednisolone 500-1000mg daily x5d) Maintenance (↓ risk of exacerbation): IFNβ, glatiramer, natalizumab (better efficacy but worse side effects), dimethyl fumarate, teriflunomide

Question 299

Acute eye pain worse with movement, central vision loss Afferent pupillary defect (Marcus-Gunn pupil) - no constriction of either pupil when light is shone into the affected eye; both pupils constrict when light is shone into the unaffected eye Diagnosis?

Answer 299

Unilateral optic neuritis seen with MS

Question 300

Patient can direct both eyes forward Patient can direct both eyes to the right When patient looks to the left, they cannot adduct right eye. Diagnosis? Where is the lesion?

Answer 300

Internuclear opthalmoplegia seen with MS Damage to right medial longitudinal fasciculus (MLF)

Question 301

H/o cervical spine injury (months to years ago) Gradual loss of pain and temp 1-3 segments below lesion UE weakness, flaccid paralysis, atrophy of hand muscles Decreased DTRs Muscle fasciculations Diagnosis? Treatment?

Answer 301

Syringomyelia - cystic degeneration of the spinal cord leading to expansion of the cavity compressing adjacent neural tissue. Loss of pain and temp due to compression of STT in the anterior white commissure UE symptoms due to compression of LMN in the anterior horns Tx: surgical decompression and shunting

Question 302

Treatment for alzheimer disease (4)

Answer 302

``` Cholinesterase inhibitors - Donepezil - Galantamine - Rivastigmine NMDA receptor blocker - Memantine ```

Question 303

Dementia + parkinsonian features, visual hallucinations, episodes of syncope/repeated falls

Answer 303

Lewy Body Dementia

Question 304

Dementia + inappropriate social behavior or progressive aphasia

Answer 304

Frontotemporal dementia (pick disease)

Question 305

Pt presenting with cognitive impairment, urinary incontinence, gait disturbance Diagnosis? Evaluation? Treatment?

Answer 305

Normal pressure hydrocephalus (NPH): inflammation and fibrosis of the arachnoid granulations → impaired reabsorption of CSF → excess CSF builds up in the ventricles. Presentation: WACKY, WET, WOBBLY Evaluation: MRI shows dilation of cerebral ventricles. CSF pressure is not elevated Tx: shunt

Question 306

What measurements (3) indicate a positive tilt test? What does it suggest?

Answer 306

When standing, change in: ↑ HR ≥ 20bpm ↓ SBP ≥ 20 mmHg ↓ DBP ≥ 10 mmHg Suggests orthostatic syncope

Question 307

In an intact brainstem, the patient's eye should move in which direction with ice water infusion into an ear canal?

Answer 307

Eyes move towards the ear, nystagmus away from the ear

Question 308

What stage of sleep? Beta waves (high frequency, low amplitude). Full motor tone.

Answer 308

Awake

Question 309

What stage of sleep? Alpha waves (high frequency low amplitude)

Answer 309

Awake, relaxed, drifting off to sleep

Question 310

What stage of sleep? Theta waves (high frequency, low amplitude)

Answer 310

``` Stage N1 (non-REM) Light sleep ```

Question 311

What stage of sleep? Sleep spindles and K complexes (decreased frequency, increased amplitude) Bruxism

Answer 311

Stage N2 (non-REM)

Question 312

What stage of sleep? Delta waves (low frequency, high amplitude) Sleepwalking Night terrors Enuresis

Answer 312

Stage N3 (non-REM)

Question 313

What stage of sleep? Beta waves (high frequency, low amplitude) Loss of motor tone Erections Night mares

Answer 313

REM

Question 314

Excessive daytime sleepiness, hallusinations and cataplexy. Polysomnography shows short sleep latency. Diagnosis? Treatment?

Answer 314

Narcolepsy - sleep goes from awake to REM Tx: Regular sleep schedule (includes naps) Meds to improve daytime sleepiness: modafinil, armodafinil, amphetamines Meds to reduce REM: venlafaxine, fluoxetine, atomoxetine Severe symptoms: sodium oxybate

Question 315

Effect of benzos on sleep

Answer 315

Binds to GABA receptors ↑ N2 ↓ N3 and REM

Question 316

First line treatment for restless leg syndrome

Answer 316

Pramipexole and ropinirole (DA agonists)

Question 317

Most common brain tumors in adults (4). Most common location?

Answer 317

``` "MGM Studios" Metastasis Glioblastoma (form of astrocytoma) Meningioma Schwannoma ``` Most common location: supratentorial

Question 318

Adult brain tumor Irregular mass with necrotic center, surrounded by edema. Located in cerebral hemisphere. Diagnosis? Treatment? Prognosis?

Answer 318

Glioblastoma (most common primary brain tumor in adults) Tx: resection, chemo, radiation. Very poor prognosis (6mo-1y)

Question 319

Adult brain tumor. Slow growing tumor located near the surface of the brain. Often asymptomatic. Diagnosis? Treatment? Prognosis?

Answer 319

Meningioma (2nd most common primary brain tumor in adults) Tx: resection. Good prognosis

Question 320

Adult brain tumor. Presentation: tinnitus and hearing loss. Diagnosis? Treatment?

Answer 320

Schwannoma - localizes to CN VIII Tx: resection

Question 321

Most common primary pediatric brain tumors (3) Most common location

Answer 321

"Animal kingdom, Magic kingdom, Epcot center" Astrocytoma Medulloblastoma Ependymoma Most commonly infratentorial

Question 322

Child brain tumor. Slow growing, located in posterior fossa Diagnosis? Treatment?

Answer 322

Pilocytic astrocytoma - most common primary brain tumor in children Tx: Resection

Question 323

Child brain tumor Presenting with headache and ataxic gait. Compression of the 4th ventricle → non-communicating hydrocephalus. Diagnosis? Treatment?

Answer 323

Medulloblastoma - 2nd most common primary brain tumor in children. Cerebellar tumor. Malignant Tx: resection, chemo, radiation.

Question 324

``` Presentation: Uniformly hyperpigmented macules Axillary/inguinal freckling Hamartomas of iris Benign peripheral nerve sheath tumors Vision loss, color vision problems, abnl papillar function, proptosis ``` Diagnosis? Treatment?

Answer 324

Neurofibromatosis type 1 - "Von Recklinghausen disease" Defect of NF1 gene on c17. Cafe au lait spots - uniform hyperpigmented macules Lisch nodules - hamartomas of iris Neurofibromas - benign peripheral nerve sheath tumors (increased risk for malignancy) Optic pathway gliomas - vision changes Tx: supportive

Question 325

Bilateral tinnitus, hearing loss, balance dysfunction Most likely diagnosis?

Answer 325

Neurofibromatosis type 2 - intracranial meningiomas and spinal tumors 90-95% of patients have bilateral schwannoma

Question 326

H/o muscle weakness worsening throughout the day, ptosis, diplopia, dysarthria, dysphagia, dyspnea. Diagnosis? What tests do you want to do? Treatments?

Answer 326

Myasthenia gravis - AI disorder with Ab against ACh receptors. Tests: Test for Ach-receptor Ab Tensilon test - administer Edrophonium (Ach-esterase inhibitor) → improves symptoms. *** Always order Chest CT to evaluate for thymoma Treatment: Ach-esterase inhibitors (neostigmine, pyridostigmine) Immunosuppressants (prednisone, azathioprine) Plasmapheresis IVIG Thymectomy

Question 327

H/o muscle weakness that improves with use. Diagnosis? Common association? Treatment?

Answer 327

Lambert-Eaton syndrome - AI disorder with Ab against presynaptic voltage-gated Ca channels ** 60% will have small cell lung CA (paraneoplastic syndrome) ``` Tx: Treat underlying CA ACh-esterase inhibitors (pyridostigmine) Immunosuppressants Plasmapheresis ```

Question 328

H/o symmetric muscle weakness beginning in distal legs with associated absent/depressed DTRs that ascends → facial muscle weakness, autonomic dysfunction, respiratory paralysis. Often follows recent viral illness (diarrhea) ** no sensory deficits Diagnosis? Important labs? Treatment? Prognosis?

Answer 328

Guillain-Barré syndrome aka acute inflammatory demyelinating polyradiculopathy Labs: CSF: albuminocytologic dissociation - ↑ protein with normal cell counts, normal glucose, normal pressure. Tx: Hospitalization for respiratory failure Plasmapheresis, IVIG * NO glucocorticoids Prognosis: 80-90% by 1 year 5-10% with prolonged disease

Question 329

Fixed oscillation of hands or head What type of movement disorder? Treatment?

Answer 329

Essential tremor Tx: βblocker (propanolol), benzos (alprazolam, clonazepam), primidone, thalamotomy, deep brain stimulation * tremor improves with alcohol use

Question 330

Dance-like movements What type of movement disorder? Common associations? (5)

Answer 330

Chorea Associated with hyperthyroidism, huntington disease, lupus, levodopa, rheumatic fever

Question 331

Snake-like movements What type of movement disorder? Common associations? (4)

Answer 331

Athetosis Associated with cerebral palsy, encephalopathy, huntington disease, wilson disease

Question 332

Sustained contractions of proximal limbs and trunk What type of movement disorder? Common associations? (6) Treatment?

Answer 332

Dystonia Associated with neuroleptic drugs, Wilson disease, Parkinson disease, cerebral palsy, Huntington disease, encephalitis Treatment: Levodopa, carbidopa, botulinum toxin

Question 333

Flinging movements of the extremities Diagnosis? Treatment?

Answer 333

Hemmiballismus associated with stroke of the subthalamic nucleus Treatment: haloperidol

Question 334

Involuntary movements - blinking, grimacing, grunting, sniffing, throat clearing What type of movement disorder? Common associations? (3) Treatment?

Answer 334

Tics Associated with Tourette syndrome, OCD, ADHD Treatment: Fluphenazine, pimozide, tetrabenazine

Question 335

What light reflexes will be seen if the right optic nerve is damaged distal to the pretectal nucleus?

Answer 335

Afferent defect No constriction of either L or R pupil when light is shone in R eye Both pupils constrict if light is shown in the L eye

Question 336

What light reflexes will be seen if the right oculomotor nerve is damaged?

Answer 336

Efferent defect R pupil will not respond to light shone in either R or L eye L pupil will constrict when light is shone in either eye

Question 337

Left anopsia. Where is the lesion?

Answer 337

Left optic nerve

Question 338

Bitemporal hemianopia Where is the lesion?

Answer 338

Optic chiasm

Question 339

Right homonymous hemianopia Where is the lesion?

Answer 339

Left optic tract

Question 340

Right upper quadrantic anopia Where is the lesion?

Answer 340

Left temporal lobe

Question 341

Right lower quadrantic anopia Where is the lesion?

Answer 341

Left parietal lobe

Question 342

Right hemianopia with macular sparing Where is the lesion?

Answer 342

Left visual cortex

Question 343

Blurred vision at all distances Cause? Treatment?

Answer 343

Astigmatism - warped/asymmetrical cornea Tx: corrective lenses

Question 344

Blurred distance vision aka nearsightedness Cause? Treatment?

Answer 344

Myopia - axial length of the eye is too LONG for the refractive power of the cornea and lens Tx: corrective lenses or laser surgery

Question 345

Blurred near vision aka farsightedness Cause? Treatment?

Answer 345

Hyperopia - axial length of the eye is too SHORT for the refractive power of the cornea and lens Tx: corrective lenses or laser surgery

Question 346

Bilateral (but not symmetrical), painless progressive decrease in vision manifested by difficulty driving at night, reading road signs, reading fine print. Common presentation: patient who was previously farsighted slowly becomes nearsighted Diagnosis? Treatment?

Answer 346

Cataracts - opacification of lens due to build up of epithelial cells Tx: surgical removal of opacified lens and replacement with synthetic lens

Question 347

Gradual loss of central vision with age Diagnosis? Management?

Answer 347

Dry (atrophic) age related macular degeneration - cellular debris called "drusen" accumulates on the retina Tx: Smoking cessation Anti-oxidant supplements (i.e. beta-carotene, vitamin C, lutein, selenium, zinc)

Question 348

Sudden central vision loss with rapid progression, happens in old age

Answer 348

Wet (exudative) age related macular degeneration - neovascularization → hemorrhage → sudden vision loss Tx: Injection of anti-VEGF drugs (i.e. ranibizumab) into vitreous humor

Question 349

Painless, sudden vision loss with flashing lights and floaters, "curtain pulled over the eye" Fundoscopic exam: wrinkled, pale area visible Diagnosis? Treatment?

Answer 349

Retinal detachment Tx: Laser photocoagulation Cryotherapy Surgical reattachment

Question 350

Acute, painless vision loss. Fundoscopic exam shows pale retina with cherry red spot. Diagnosis? Treatment?

Answer 350

Central retinal artery occlusion (CRAO) ``` Tx: Thrombolytics Acetazolamide or mannitol to ↓ IOP Anterior chamber paracentesis (removal of aqueous humor with a needle) Hyperbaric oxygen ```

Question 351

Gradual, painless vision loss Fundoscopic exam shows retinal edema, retinal hemorrhages, venous dilation, cotton wool spots Diagnosis? Treatment?

Answer 351

Central retinal vein occlusion (CRVO) Tx: VEGF inhibitors Laser photocoagulation

Question 352

Sudden onset of unilateral eye pain with redness and tearing, frontal HA, nausea and vomiting. Blurry vision with colored halos encircling light sources. Exam: hazy cornea, pupil is fixed and mid-dilated. Eye feels "rock hard". Diagnosis? Treatment?

Answer 352

Acute angle-closure (narrow-angle) glaucoma - lens pushes anteriorly against iris, blocking flow of aqueous humor through the pupil → ↑ IOP ``` Tx: IMMEDIATE REFERRAL to ophthalmologist for laser peripheral iridotomy Eye drops (given 1 min apart): - Timolol - Apraclonidine - Pilocarpine PO Acetazolamide If refractory, IV mannitol ```

Question 353

Gradual loss of peripheral vision, usually bilateral. Fundoscopic exam shows optic cup:disc ratio >50%. Diagnosis? Treatment?

Answer 353

Open-angle glaucoma - impermeability of trabecular meshwork → ↓ resorption of aqueous humor → gradual ↑ in IOP Tx: - Prostaglandins (latanoprost) to ↑ outflow of aqueous humor - βblockers (timolol) to inhibit aqueous humor production - α2 agonist (apraclonidine, brimonidine) to ↓ aqueous humor production and increase outflow - Cholinergic agonist (pilocarpine) to cause miosis and open trabecular meshwork to increase outflow - Topical acetazolamide to reduce aqueous humor production * Laser trabeculoplasty to improve outflow

Question 354

Eye redness, swelling, pain, proptosis, pain with extraocular movements, opthalmoplegia, diplopia Diagnosis? Complications?

Answer 354

Orbital cellulitis 10% will have permanent vision loss 1-2% mortality

Question 355

Inflammation of internal meibomian sebaceous glands → eyelid swelling Diagnosis? Treatment?

Answer 355

Chalazion Usually self-limited but can be treated with surgical excision and/or intralesional steroid injection

Question 356

Infection of external sebaceous glands of Zeiss or Mol → tender, red swelling at the eyelid margin Diagnosis? Treatment?

Answer 356

Hordeolum (stye) Warm compress 3-4x/d for 10-15min Abx ointment q3h if not resolved in 48h +/- I&D

Question 357

What is the most likely cause of conjunctivitis appearing in the 1st 24h of life?

Answer 357

Chemical conjunctivitis due to abx prophylaxis for N. gonorrhoea conjunctivitis

Question 358

Night blindness or complete blindness Xeropthalmia (dry eye) Bitot spots (areas of abnl squamous cell proliferation and keratinization of the conjunctiva) Diagnosis?

Answer 358

Vitamin A Deficiency

Question 359

Child with recent infection with S. pyogenes presenting with HTN, edema, brown urine. EM shows subepithelial humps. Diagnosis? What other tests? Treatment?

Answer 359

Post-streptococcal glomerulonephritis Order ASO titer, Anti-DNAse B Tx: supportive. Treat edema and HTN

Question 360

40yoF with proteinuria, + ANA, + Anti-dsDNA Ab. Light microscopy of kidney: endothelial deposits causing "wire loops" Diagnosis? Treatment?

Answer 360

Lupus nephritis - can be mesangial, membranous, focal nephritis, diffuse nephritis Tx: glucocorticoids, ACE inhibitor, Statin, Immunosuppressant (cyclophosphamide or mycophenolate mofetil)

Question 361

Pt presenting with microscopic hematuria, pulmonary nodules/infiltrates, saddle nose, sinus disease, palate granulomas/ulcers, c-ANCA positive. Diagnosis? Treatment?

Answer 361

Granulomatosis with polyangiitis (GPA) (Wegener's) Tx: glucocorticoids, cyclophosphamide

Question 362

Pt presenting with proteinuria and hematuria, cough/hemoptysis, pulmonary infiltrates. Immunofluorescence: linear deposits on glomerular basement membrane. Diagnosis? Treatment?

Answer 362

Goodpasture syndrome Tx: glucocorticoid, plasmapheresis

Question 363

Pt with hx of cataracts, high-frequency hearing loss presenting with nephritis. EM shows splitting of the basement membrane. Diagnosis? Treatment?

Answer 363

Alport syndrome (hereditary nephritis) - X-linked defect in type IV collagen synthesis → defective basement membranes Tx: ACE, renal transplant

Question 364

Pt presenting with microscopic hematuria and proteinuria, p-ANCA positive. Scarce immune complex deposits. Diagnosis? Treatment?

Answer 364

Pauci-immune rapidly progressive glomerulonephritis (RPGN) Tx: rapid progression to ESRD (weeks to months), glucocorticoid, cyclophosphamide

Question 365

Child with proteinuria, hypoalbuminemia, edema (periorbital, sacral, scrotal), HTN. EM: effacement of podocyte foot processes. Diagnosis? Treatment?

Answer 365

Minimal change disease Tx: Glucocorticoids

Question 366

Adult with proteinuria, hypoalbuminemia, edema. Dz affects

Answer 366

Focal segmental glomerular sclerosis (FSGS) ↑ prevalence in HIV patients, African Americans, Latinos. Tx: Glucocorticoid, ACE, Statin, +/- cyclosporine

Question 367

Proteinuria, hypoalbuminemia, edema. EM: subendothelial "humps" of IgG along glomerular basement membrane with mesangial cell proliferation and mesangial immune deposits. Diagnosis? Common disease associations? Treatment?

Answer 367

Membranoproliferative glomerulonephritis (MPGN) Common disease associations: SLE, subacute bacterial endocarditis, Hep C Tx: Treat underlying cause, glucocorticoids, +/- cyclophosphamide

Question 368

Proteinura, hypoalbuminemia, edema. EM: thickening of basement membrane with "spike and dome" appearance. Diagnosis? Common disease associations? Treatment?

Answer 368

Membranous nephropathy Common disease associations: SLE, Hep B Tx: ACE, Statin, +/- cyclophosphamide with steroid

Question 369

Proteinuria, hypoalbumninema, edema. EM shows thickening of glomerular basement membrane, acellular eosinophilic nodules in the mesangium, expansion of the mesangium. Diagnosis? Treatment?

Answer 369

Diabetic nephropathy Acellular eosinophilic nodules = kimmelstiel-wilson nodules Tx: Aggressive glycemic control, Aggressive BP control, ACE/ARB

Question 370

Proteinuria, hypoalbuminemia, edema. Expansion of mesangial matrix. Congo red stain shows apple-green birefringence. Diagnosis? Causes? Treatment?

Answer 370

Renal amyloidosis May be due to : plasma cell dyscrasia (Ig light chains), chronic inflammatory diseases (i.e. RA, spondyloarthropathy, IBD), hemodialysis, heritable amyloidosis Tx: Melphalan, hematopoietic cell transplant

Question 371

What acid base disturbance would you see with: Vomiting

Answer 371

Metabolic alkalosis

Question 372

What acid base disturbance would you see with: Diuretics

Answer 372

Metabolic alkalosis

Question 373

What acid base disturbance would you see with: Cushing disease

Answer 373

Metabolic alkalosis

Question 374

What acid base disturbance would you see with: Hyperaldosteronism

Answer 374

Metabolic alkalosis

Question 375

What acid base disturbance would you see with: Hyperventilation

Answer 375

Respiratory alkalosis

Question 376

What acid base disturbance would you see with: High altitude

Answer 376

Respiratory Alkalosis

Question 377

What acid base disturbance would you see with: Asthma

Answer 377

Respiratory alkalosis

Question 378

What acid base disturbance would you see with: Aspirin toxicity (early)

Answer 378

Respiratory alkalosis

Question 379

What acid base disturbance would you see with: PE

Answer 379

Respiratory alkalosis

Question 380

What acid base disturbance would you see with: COPD

Answer 380

Respiratory acidosis

Question 381

What acid base disturbance would you see with: Neuromuscular disease

Answer 381

Respiratory acidosis

Question 382

What acid base disturbance would you see with: Opioid overdose

Answer 382

Respiratory acidosis (respiratory depression)

Question 383

What acid base disturbance would you see with: Diarrhea

Answer 383

Non anion gap metabolic acidosis

Question 384

What acid base disturbance would you see with: Hyperaldosteronism

Answer 384

Non anion gap metabolic acidosis

Question 385

What acid base disturbance would you see with: TPN administration

Answer 385

Non anion gap metabolic acidosis

Question 386

34yoF 2 weeks s/p thyroidectomy for hyperthyroidism. Presenting with complaint of hoarseness - she is unable to project her voice in a loud environment and has difficulty speaking for long periods of time. What diagnostic test do you want to order? Damaged muscle? Damaged nerve?

Answer 386

Fiberoptic laryngoscopy Posterior cricoarythenoid muscle - the only muscle that opens the vocal cords. Damage to recurrent laryngeal nerve ** RLN also supples digastric, omohyoid, thyroarytenoid muscle, but they do not directly open the vocal cords.

Question 387

Precocious puberty in female with: | ↑ LH, ↑ estrogen

Answer 387

Central precocious puberty

Question 388

Precocious puberty in female with: | ↓LH, ↑ estrogen

Answer 388

Peripheral precocious puberty

Question 389

Female with precocious puberty with: ↑ DHEA-S Potential causes?

Answer 389

Congenital adrenal hyperplasia or adrenal neoplasm

Question 390

What non-hormonal options can be used in treatment of menopausal hot flashes? (4)

Answer 390

Desvelafaxine - only one that is FDA approved; also works as antidepressant Venlafaxine - good for depression, anxiety, fatigue, isolation. Clonidine - also good for BP. SE: dry mouth, constipation, drowsiness Gabapentin - good choice with insomnia, restless leg syndrome, seizure disorder, neuropathy, chronic pain

Question 391

Female pt presenting with weight gain, HA, abdominal/pelvic pain, abdominal bloating, change in bowel habits, food cravings, mood lability, depression, fatigue, irritability, breast tenderness, edema, acne. Diagnosis? Treatment?

Answer 391

PMS/PMDD Tx: NSAIDs, exercise, relaxation, SSRIs (can be used just during luteal phase for several days or continuously), GnRH agonists

Question 392

Female presenting with pelvic pain 2-7d prior to menses and lasting throughout menses, dyspareunia, constipation, diarrhea, bowel pain, and infertility. Exam: tenderness of vaginal fornix, posterior cul-de-sac or rectovaginal septum. Lateral displacement of cervix. Palpable tender nodules in posterior cul-de-sac, uterosacral ligaments and rectovaginal septum. Pain with movement of uterus. Uterus fixed in retroverted position. ↑CA-125 Diagnosis? Definitive diagnosis? Treatment?

Answer 392

Endometriosis Definitive diagnosis: direct visualization on laparoscopy Tx: NSAIDs, OCPs, GnRH agonists, Danazol, ablation of lesions during laparoscopy hysterectomy with salpingo-oophorectomy

Question 393

Abnormal uterine bleeding plus: + β-hCG, + intrauterine pregnancy, closed os

Answer 393

Threatened abortion

Question 394

Abnormal uterine bleeding plus: enlarged uterus + menometrorrhagia for months

Answer 394

Fibroids, adenomyosis

Question 395

Abnormal uterine bleeding plus: Bleeding associated with severe menstrual pain

Answer 395

Endometriosis, adenomyosis

Question 396

Abnormal uterine bleeding plus: Perimenopausal/postmenopausal

Answer 396

Endometrial hyperplasia/neoplasm

Question 397

Abnormal uterine bleeding plus: Started with menarche

Answer 397

vonWillebrand Disease

Question 398

Abnormal uterine bleeding plus: + β-hCG + severe pain, no intrauterine pregnancy seen on ultrasound

Answer 398

Ectopic pregnancy

Question 399

Abnormal uterine bleeding plus: after intercourse, no pain, normal sized uterus

Answer 399

Cervical lesion/polyp

Question 400

Abnormal uterine bleeding plus: Weight gain, fatigue, constipation

Answer 400

Hypothyroidism

Question 401

Abnormal uterine bleeding plus: Bitemporal hemianopsia

Answer 401

Pituitary adenoma/prolactinoma

Question 402

Abnormal uterine bleeding plus: obesity, hirsutism, acne, insulin resistance

Answer 402

PCOS

Question 403

Most common cause of primary amenorrhea given: parent went through puberty late with subsequent normal growth/development

Answer 403

Constitutional delay

Question 404

Most common cause of primary amenorrhea given: excessive exercise, stress and/or weight loss

Answer 404

Hypothalamic amenorrhea

Question 405

Most common cause of primary amenorrhea given: short stature, webbed neck, low hairline

Answer 405

Turner syndrome

Question 406

Most common cause of primary amenorrhea given: Breast development + absent uterus + no pubic/axillary hair

Answer 406

Androgen insensitivity syndrome

Question 407

Most common cause of primary amenorrhea given: Breast development + absent uterus + pubic and axillary hair

Answer 407

Mullerian agenesis

Question 408

Most common cause of primary amenorrhea given: Cyclic pain, bluish vaginal bulge on exam

Answer 408

Imperforate hymen

Question 409

Most common cause of primary amenorrhea given: Anosmia; no secondary sexual characteristics

Answer 409

Kallmann syndrome

Question 410

Most common cause of secondary amenorrhea

Answer 410

Pregnancy

Question 411

Most common cause of secondary amenorrhea given: Hirsutism, insulin resistance, "string of pearls" on ultrasound

Answer 411

PCOS

Question 412

Most common cause of secondary amenorrhea given: Clitoromegaly, voice deepening, ↑ muscle mass

Answer 412

↑ androgens

Question 413

Most common cause of secondary amenorrhea given: Fatigue, cold intolerance, weight gain, constipation, delayed relaxation of DTRs

Answer 413

Hypothyroidism

Question 414

Most common cause of secondary amenorrhea given: Anxiety, palpitations, tremor, heat intolerance, weight loss, diarrhea

Answer 414

Hyperthyroidism

Question 415

Most common cause of secondary amenorrhea given: Bitemporal hemianopsia + galactorrhea

Answer 415

Prolactinoma

Question 416

Most common cause of secondary amenorrhea given: Patient with hx of schizophrenia + galactorrhea

Answer 416

Antipsychotic side effect

Question 417

Most common cause of secondary amenorrhea given: Hot flashes, vaginal dryness

Answer 417

Menopause, primary ovarian insufficiency

Question 418

Most common cause of secondary amenorrhea given: H/o postpartum hemorrhage

Answer 418

Sheehan syndrome

Question 419

Most common cause of secondary amenorrhea given: H/o D&C for septic abortion

Answer 419

Asherman syndrome

Question 420

``` Female patient with h/o menstrual irregularities Lab findings: ↑ LH (LH:FSH ratio > 2:1) ↑ androgens ↑ insulin and insulin resistance ↑ estrogens ↓ sex hormone binding globulin ``` Diagnosis? Increased risk of what? Treatment?

Answer 420

PCOS Increased risk of endometrial hyperplasia/neoplasm due to increased amount of unopposed estrogen Tx: - Diet and exercise - OCPs - Cyclic progestins alone - protect against endometrial hyperplasia but will not help with menstrual irregularities - Spironolactone - add if additional tx of hirsutism is needed - Clomiphene - ovulation induction in patients desiring pregnancy - Metformin - 2nd line, restores ovulatory cycles in 50% of women

Question 421

Infertility + h/o gonorrhea or chlamydia Cause of infertility? Next step?

Answer 421

PID Hysterosalpingogram

Question 422

Infertility + h/o severe dysmenorrhea, dyspareunia, pelvic pain Cause of infertility? Next step?

Answer 422

Endometriosis Laparoscopy

Question 423

Infertility + h/o pelvic irradiation Cause of infertility? Next step?

Answer 423

Primary ovarian insufficiency Check FSH

Question 424

Infertility + h/o tubal surgery Cause of infertility? Next step?

Answer 424

Tubal obstruction Hysterosalpingogram

Question 425

Infertility + h/o enlarged uterus with irregular contours Cause of infertility? Next step?

Answer 425

Fibroids Ultrasound

Question 426

Infertility + h/o D&C for septic abortion Cause of infertility? Next step?

Answer 426

Asherman syndrome Hysteroscopy

Question 427

Female presenting with thin, white/gray discharge with fishy odor. Vaginal pH > 4.5. Clue cells present. + whiff test. H/o smoking, douching, sexual activity Diagnosis? Most likely organism? Treatment?

Answer 427

Bacterial vaginosis due to Gardenerella vaginalis Tx: Metronidazole, Clindamycin

Question 428

Female presenting with greenish frothy vaginal discharge, irritation, dysuria, strawberry cervix, vaginal pH >4.5. + nucleic acid amplification test for organism. Diagnosis? Treatment?

Answer 428

Trichomoniasis due to Trichomonas vaginalis. Tx: Metronidazole *also treat partner

Question 429

Female presenting with vulvar/vaginal itching and irritation, thick white cottage cheese-like vaginal discharge, pH normal (3.5-4.5). H/o recent abx use, DM, immunosuppression, ↑ estrogen levels. Diagnosis? Treatment?

Answer 429

Vulvovaginal candidiasis Tx: PO Fluconazole, topical azole

Question 430

Menstruating female presenting with fever, hypotension, vomiting, diarrhea, ↑ BUN/creatinine, confusion, altered mental status. Macular rash of palms and soles that is desquamating. Diagnosis? Most likely organism? Treatment?

Answer 430

Toxic shock syndrome due to Staphylococcus aureus production of exotoxin TSST-1. Tx: remove tampon, supportive care (IVF, pressors), Clindamycin + Vancomycin

Question 431

Female with h/o purulent vaginal discharge, spotting, postcoital bleeding, urinary sx, erythematous and friable cervix. Possible positive gram stain. Nucleic acid amplification test is positive for organism. Diagnosis? Most likely organisms? Treatment?

Answer 431

Cervicitis Most common: N. gonorrhoeae, C. trachomatis, T. vaginalis, HSV Tx: Chlamydia - Azithromycin x1 dose or Doxycycline x7d. Gonorrhea - Ceftriaxone x1 dose PLUS Azithromycin x1 dose (covers for chlamydia but also treats gonorrhea that is resistant to ceftriaxone).

Question 432

Female with flesh-colored, "cauliflower-like" papules in genital region that turn white with application of 5% of acetic acid. Diagnosis? Most likely organisms? Treatment (physical, chemical, and immunologic)

Answer 432

Genital warts (condyloma acuminata) HPV types 6 and 11 cause majority of genital warts Types 16 and 18 cause majority of cervical cancers Tx: Physical - excision, laser, cryotherapy Chemical - podophyllin, trichloroacetic acid, 5-FU Immunologic - Imiquod, IFNα

Question 433

Pt presenting with multiple vesicles on erythematous base on genitals, clusters of painful/shallow ulcers, first outbreak accompanied by systemic sx (fever/malaise/myalgias), dysuria, LAD Tzanck smear: Multinucleated giant cells Diagnosis? Treatment?

Answer 433

Genital herpes due to HSV 1/2 Tx: Acyclovir, famciclovir, valacyclovir

Question 434

Initially: single papule → chancre (painless, clean-based ulcer with heaped up border; heals spontaneously) Later: fever, malaise, LAD, rash on palms and soles, condyloma lata Diagnosis? Treatment? Complications?

Answer 434

Syphilis due to Treponema pallidium Tertiary syphilis - Gummas (granulomatous lesions in skin/bones/internal organs), CV (aneurysms of ascending aorta, aortic valve insufficiency) Neurosyphilis - general paresis, tabes dorsalis, argyll-robertson pupil Tx: PCN G, doxycycline, tetracycline

Question 435

Erythematous papule → pustule → painful ulcer with purulent base. + painful inguinal LAD, bubo formation Gram stain: GNR in school of fish pattern Diagnosis? Organism? Treatment?

Answer 435

Chancroid due to Haemophilus ducreyi. Tx: Ceftriaxone, Azithromycin

Question 436

Painless ulcer on genitals that resolves spontaneously → unilateral inguinal LAD → bubo formation with "groove sign", proctocolitis Diagnosis? Organism? Treatment?

Answer 436

Lymphogranuloma venereum Chlamydia trachomatis L1-L3 serovars Tx: Doxycycline

Question 437

Painful genital nodule(s) → shallow painless ulcer(s) with beefy-red base. Wright-giemsa stain shows intracellular GNR Diagnosis? Organism? Treatment?

Answer 437

Granuloma inguinale (Donovanosis) Klebsiella granulomatis Tx: Azithromycin

Question 438

LSIL What is the next step in management? - 30yo

Answer 438

30: HPV testing; if + colposcopy, if negative repeat in 1y

Question 439

HSIL Next step in management?

Answer 439

Colposcopy (or LEEP if >25yo)

Question 440

ASCUS What is the next step in management? - 25yo

Answer 440

25yo: HPV testing, colposcopy if positive

Question 441

ASC-H (atypical squamous cells can't exclude HSIL) What is the next step in management?

Answer 441

Colpscopy

Question 442

Atypical glandular cells (ACG) on pap test What is the next step in management?

Answer 442

Endocervical sampling, colposcopy. + endometrial biopsy if >25yo

Question 443

Most likely cause of adnexal mass if: woman of reproductive age

Answer 443

Physiologic ovarian cyst

Question 444

Most likely cause of adnexal mass if: Dysmenorrhea, dyspareunia, pelvic pain

Answer 444

Endometrioma

Question 445

Most likely cause of adnexal mass if: Fever, vaginal discharge, cervical motion tenderness

Answer 445

Tubo-ovarian abscess

Question 446

Most likely cause of adnexal mass if: Abdominal pain, vaginal bleeding, + pregnancy test

Answer 446

Ectopic

Question 447

Most likely cause of adnexal mass if: Otherwise normal pregnancy

Answer 447

Corpus luteum cyst

Question 448

Postmenopausal woman with abdominal pain, early satiety, ascites. ↑CA-125. Diagnosis? Prognosis?

Answer 448

Epithelial ovarian tumor (serous, mucinous) Poor prognosis

Question 449

Adolescent female with abdominal pain/mass PLUS: - ↑ LDH (what type is this?) - ↑ AFP (what types are this?) - ↑ AFP and ↑ hCG (what type is this?) Prognosis?

Answer 449

Germ cell tumor of the ovary - ↑ LDH: dysgerminoma - ↑ AFP: yolk sac or teratoma - ↑ AFP and ↑ hCG: embryonal Prognosis: excellent

Question 450

Female with precocious puberty and abnormal uterine bleeding. ↑ Estrogen ↑ Inhibin Diagnosis? Prognosis?

Answer 450

Sex cord-stromal tumor of ovary Granulosa cell tumor Good prognosis

Question 451

Female with virilization ↑ Testosterone What type of ovarian tumor? Prognosis?

Answer 451

Sex cord-stromal tumor of ovary Sertoli-Leydig cell tumor Good prognosis

Question 452

Drugs known to caused gynecomastia (7)

Answer 452

``` STACKED Spironolactone TCH Alcohol (chronic use) Cimetidine Ketoconazole Estrogens Digoxin ```

Question 453

Female with h/o DM, smoker presenting with painful breast mass, large area of redness/warmth/tenderness, purulent drainage from the nipple, fever. Diagnosis? Treatment?

Answer 453

Breast abscess Tx: I and D Abx: Dicloxacillin, cephalexin, or augmentin. TMP-SMX if you suspect MRSA. Metronidazole if you suspect anaerobes (foul smelling pus)

Question 454

35-50yo F with multiple painful bilateral breast masses that ↑ in size and pain before menstruation, regress after menstruation. Diagnosis? Treatment?

Answer 454

Fibrocystic changes Reduce intake of caffeine and fat OCPs will decrease stimulation of the breast

Question 455

F

Answer 455

Fibroadenoma - most common benign breast tumor in women under 30yo. US can distinguish solid mass from cyst. FNA, core biopsy or excisional biopsy are optional. Diagnostic mammogram if pt is >35yo Tx: May not be required Surgical excision or cryotherapy if large or symptomatic

Question 456

Female with serous ("straw colored") nipple discharge +/- streaked with blood. Diagnosis? Management?

Answer 456

Intraductal papilloma - benign lesion of ductal tissue; rare malignant transformation Tx: surgical excision to evaluate for cancer

Question 457

50yoF Large, bulky tumor with "leaf-like" projections seen on biopsy Diagnosis? Management?

Answer 457

Phyllodes tumor - generally benign, may occasionally become malignant Management: close monitoring Resection if size begins causing symptoms

Question 458

Patient presenting with polydipsia, polyuria, signs of dehydration. Labs: low urine osmolality with large urine volume, hypernatremia ADH administration followed by water deprivation → ↑ urine osmolality Diagnosis? Treatment?

Answer 458

Central diabetes insipidus - failure of posterior pituitary to secrete ADH; can result from idiopathic causes, cerebral trauma, pituitary tumors, hypoxic encephalopathy, or anorexia nervosa Treatment: desmopressin (DDAVP) given as ADH analog

Question 459

Patient presenting with polydipsia, polyuria, signs of dehydration. Labs: low urine osmolality with large urine volume, hypernatremia ADH administration followed by water deprivation → no change in urine osmolality Diagnosis? Treatment?

Answer 459

Nephrogenic diabetes insipidus - kidneys do not respond to ADH; can result from hereditary renal disease, Lithium toxicity, hypercalcemia, hypokalemia Tx: salt restriction, ↑H2O intake, thiazide diuretics to reduce fluid loss (causes mild hypovolemia through activity at DCT to increase water absorption at PCT); treat underlying condition (stop medications, treat tumor/renal disease)

Question 460

Breast mass +/- nipple discharge Biopsy: malignant cells in ducts without stromal invasion, possible calcifications, unifocal. Diagnosis? Management?

Answer 460

DCIS ** This mass has higher risk of subsequent invasive CA than LCIS Tx: Lumpectomy +/- radiation; consider mastectomy in high-risk individuals

Question 461

Breast mass found as incidental finding, asymptomatic. Biopsy: malignant cells in lobules without stromal invasion, multifocal. Diagnosis? Management?

Answer 461

LCIS **Lower risk of invasive CA than DCIS, however has higher risk of contralateral malignancy. Tx: Observation + Tamoxifen to ↓ risk of invasion ** consider prophylactic bilateral mastectomy

Question 462

Firm, palpable breast mass, skin dimpling, nipple retraction, peau d'orange, nipple discharge. Biopsy: malignant cells in ducts with stromal invasion and microcalcifications; fibrotic response in surrounding tissue. Diagnosis? Treatment?

Answer 462

Invasive ductal carcinoma - most common form of invasive breast cancer ``` Tx: Early focal disease: lumpectomy Multifocal: mastectomy Metastatic disease/large tumor >1cm: chemotherapy/hormone treatment Sentinel lymph node biopsy ```

Question 463

Firm, palpable breast mass, skin dimpling, nipple retraction, peau d'orange, nipple discharge. Biopsy: malignant cells in breast lobules with insidious infiltration, less fibrous response, bilateral and multifocal. ER+/PR+ Diagnosis? Treatment?

Answer 463

Invasive lobular carcinoma ``` Tx: Early focal disease: lumpectomy Multifocal: mastectomy Metastatic disease/large tumor >1cm: chemotherapy/hormone treatment Sentinel lymph node biopsy ```

Question 464

Scaly, eczematous or ulcerated lesion on the nipple and areola; may be preceded by pain/burning/itching. Diagnosis?

Answer 464

Paget disease of the breast - malignant adenoCA cells infiltrate epithelium of nipple and areola; indicates carcinoma (usually ductal) in deeper breast parenchyma

Question 465

Congenital infections: Mom has h/o exposure to cat feces, ingestion of undercooked meat Infant has chorioretinitis, diffuse intracranial calcifications, hydrocephalus, microcephaly. Risk of spontaneous abortion Diagnosis? Treatment?

Answer 465

Congenital toxoplasmosis Tx: Spiramycin Pyrimethamine + sulfadiazine + folinic acid

Question 466

Congenital infections: Mom has mono-like symptoms Infant has sensorineural hearing loss, periventricular intracranial calcifications, microcephaly, intrauterine growth restriction, MR, vision abnormalities, hydrocephalus, seizures, hepatosplenomegaly Diagnosis? Treatment?

Answer 466

Cytomegalovirus - most common congenital infection. Tx: No tx if infection develops during pregnancy Ganciclovir may decrease effects in neonates Good hygiene reduces risk of transmission

Question 467

Congenital infections: Mom has mild fever and rash, starting in the face and then spreading to trunk/extremities. Infant has sensorineural hearing loss, cataracts, PDA, blueberry muffin rash, hepatitis, intrauterine growth restriction. Increased risk of spontaneous abortion. Diagnosis? Treatment?

Answer 467

Congenital rubella No treatment if infection develops during pregnancy. No proved benefit from rubella immune globulin. Mom should be immunized before attempting to become pregnant. ** Immunization contraindicated during pregnancy.

Question 468

Congenital infections: Mom has fever and "slapped cheek" rash, arthritis. Infant has severe anemia, cardiac failure, hydrops fetalis. Diagnosis? Treatment?

Answer 468

Parvovirus B19 Tx: Serial ultrasounds to monitor fetal growth Monitor hemoglobin by percutaneous umbilical blood sampling; intrauterine transfusion for severe anemia

Question 469

Congenital infections: Mom with flu-like illness, h/o eating deli meats Infant with granulomatosis infantiseptica (rash, widespread internal abscesses), stillbirth Diagnosis? Treatment?

Answer 469

Congenital listeriosis Tx: Ampicillin

Question 470

Congenital infections: Fetus with scarring of skin in dermatomal pattern, CNS abnormalities, eye abnormalities (chorioretinitis), limb hypoplasia, encephalitis, pneumonia, intrauterine growth restriction. Neonate may have disseminated disease if transmitted during delivery Diagnosis? Treatment?

Answer 470

Congenital varicella-zoster virus Tx: Maternal exposure: Varicella immune globulin Maternal infection: Acyclovir Maternal infection @ time of delivery: Acyclovir + Varicella immune globulin to infant Neonatal infection: Acyclovir ** vaccine contraindicated during pregnancy

Question 471

Congenital infections: Neonate with vesicular skin rash, conjunctivitis, meningoencephalitis, disseminated disease (sepsis, hepatitis, pneumonia). Intrauterine growth restriction, high risk of spontaneous abortion/neonatal death Diagnosis? How can disease be prevented?

Answer 471

Herpes simplex virus From 36w-delivery, suppressive therapy with Acyclovir At time of delivery, if active genital lesions or prodromal symptoms (burning/itching/pain) are present, perform C-section Acyclovir may be beneficial in neonates

Question 472

30yoF with amenorrhea, vaginal bleeding, pelvic pain/pressure, nausea and vomiting, elevated BP. Labs show + β hCG with significantly elevated quant US: "snowstorm" appearance, no evidence of intrauterine pregnancy. Diagnosis? Treatment? Complications/risks?

Answer 472

Hydatidiform mole - Partial mole (2 sperm fertilize 1 egg, karyotype 69 XXX/XXY/XYY) - Complete mole (1 or 2 sperm fertilize empty egg, karyotype 46 XX/XY) Treatment: D and C *** Must follow β-hCG to zero Wait at least 6mo before attempting to conceive Complications/risks Partial (1-5%) and Complete (15-20%) increase risk for malignant gestational trophoblastic disease (aka choriocarcinoma)

Question 473

30yoF presenting with brown, bloody discharge, shortness of breath, cough, hemoptysis. Hx of recent pregnancy. Diagnosis? Management?

Answer 473

Malignant gestational trophoblastic disease aka Choriocarcinoma May arise from complete mole, spontaneous abortion, ectopic pregnancy, or normal pregnancy Most commonly metastasizes to the lung (also vagina, brain, liver, ect). Management: Chemotherapy (very responsive to Methotrexate) +/- surgery Follow hCG to zero Wait at least 1y before attempting pregnancy

Question 474

30yoF with h/o uterine surgery presents with painless vaginal bleeding late in pregnancy. Most likely diagnosis? Next step?

Answer 474

Placenta previa Ultrasound ** Pelvic exam could cause unintentional perforation of possible previa

Question 475

30yo pregnant female presenting with spontaneous rupture of membranes. Shortly after, bleeding and non-reassuring fetal heart tones indicating hypoxia. Diagnosis? Treatment/

Answer 475

Vasa previa -- unprotected fetal vessels overlie cervical os Rupture of membranes → compression/laceration of fetal vessels → fetal hypoxia/hemorrhage/exsanguination. Tx: Emergent c-section

Question 476

30yo pregnant female with sudden-onset painful vaginal bleeding late in pregnancy, contractions, evidence of fetal distress on fetal heart rate monitor. Diagnosis? Complications? Treatment/

Answer 476

Placental abruption (abruptio placentae) Complications - potential for DIC Tx: Emergent c-section

Question 477

Female with short stature, HTN, protruding ears, neck webbing, low occipital hairline. Presents with primary amenorrhea. Diagnosis? Karyotype?

Answer 477

Turner syndrome 45, XO

Question 478

Male with testicular atrophy, tall/thin body, gynecomastia, mild MR, psychosocial adjustment abnormalities. Diagnosis? Karyotype?

Answer 478

Klinefelter syndrome 47, XXY

Question 479

Male with tall body (>6ft), significant acne, mild MR Diagnosis?

Answer 479

47, XYY

Question 480

Female with increased incidence of mental retardation, menstrual abnormalities, 80th percentile for height and 25th percentile for head circumference. Diagnosis?

Answer 480

47, XXX

Question 481

Pt with MR, protruding tongue, flat nose, small hears, decreased hearing and vision, transverse palmar crease, increased space between 1st and 2nd toes, heart murmur. Diagnosis? What conditions is the patient at increased risk for?

Answer 481

Down syndrome Trisomy 21 Increased risk for cervical spine instability, duodenal atresia and other GI abnormalities, Alzheimer disease, ALL, cardiac defects

Question 482

Pt with severe mental retardation, small mouth, malposition of limbs, rocker-bottom feet, overlapping fingers on grasp, cardiac defect, GI abnormalities. Diagnosis? Prognosis?

Answer 482

Edwards syndrome Trisomy 18 Frequently fatal within 1st year

Question 483

Pt with cleft lip and palate, cardiac defects, CNS defects, severe MR, rounded nose, polydactyly. Diagnosis? Prognosis?

Answer 483

Patau syndrome Trisomy 18 Frequently fatal w/in first year

Question 484

Pt with high pitched cat like cary, small head, low birth weight, MR, failure to thrive. Diagnosis?

Answer 484

Cri du chat syndrome

Question 485

Pt with overeating, obesity, decreased muscular tone in infancy, mental retardation, small hands and feet. Diagnosis? Deletion of what? Complications?

Answer 485

Prader-Willi syndrome 15q11-15q13 (deletion of Paternal allele) Obesity related complications can decrease lifespan.

Question 486

Pt with puppetlike movement, happy mood, unprovoked laughter, MR, seizures. Diagnosis? Deletion of what?

Answer 486

Angelman syndrome | 15q11-15q13 (deletion of maternal allele)

Question 487

Short, upturned nose; long philtrum, wide mouth, short stature, MR, cheerful/friendly personality, supravalvular stenosis. Diagnosis?

Answer 487

Williams syndrome | * elfin facies

Question 488

Male with large face and prominent jaw, large ears, hand/foot abnormalities, macroorchidism, MR, hyperactivity, possible seizures. Diagnosis?

Answer 488

Fragile X Syndrome

Question 489

Newborn presenting with tachypnea, respiratory distress, poor feeding. PE: continuous murmur at L 2nd intercostal, wide pulse pressure, bounding pulses. XR: cardiomegaly. Next step? Diagnosis? Risk factors? Treatment?

Answer 489

Next step: Echocardiogram - will show defect plus enlarged LA and LV Diagnosis: PDA ``` Risk factors: Female > Male Prematurity Congenital rubella infection Trisomy 21 ``` Treatment: Indomethacin for premature newborns Surgical ligation

Question 490

Child presenting with shortness of breath, failure to thrive, cyanosis. Exam: fixed splitting of S2, midsystolic pulmonary flow murmur Next step? Diagnosis? Treatment?

Answer 490

Echocardiogram - will show defect, enlarged RA and RV Diagnosis: ASD Tx: Small - monitor Large/heart failure - surgical repair

Question 491

Infant presenting with tachypnea, poor feeding, failure to thrive. Exam with holosystolic murmur along LLSB. Diagnosis? Treatment?

Answer 491

VSD - most common congenital heart defect Tx: Diuretic +/- inotropic for heart failure symptoms Large defects require surgical repair

Question 492

Newborn presents with bilious vomiting, lethargy, poor feeding, diarrhea, hematochezia, abdominal distention, abdominal tenderness. Labs: hyponatremia, metabolic acidosis Radiology: pneumatosis intestinalis, free air. Diagnosis? Treatment?

Answer 492

Necrotizing enterocolitis ``` Tx: TPN IV broad-spectrum abx NG suction Surgical resection of affected bowel ```

Question 493

Child with sudden intermittent abdominal pain lasting

Answer 493

Intussusception Risk factors: Meckel diverticulum, Henoch-schonlein purpura, adenovirus, cystic fibrosis. Tx: Barium enema Surgery

Question 494

Five "2's" of meckel diverticulum

Answer 494

``` 2 inches long 2 feet from ileocecal valve 2% of the population First 2 years of life 2 types of epithelium - pancreatic, gastric ```

Question 495

Causes of physiologic jaudice

Answer 495

Hemolysis of fetal RBC's ↓ activity of UDPGT in infants Breast milk jaundice - possibly causes ↑ intestinal absorption of bilirubin

Question 496

3mo-3yo child with nasal congestion, cough, inspiratory stridor. XR: narrowed trachea - "steeple sign" Diagnosis? Most likely organism? Treatment?

Answer 496

Croup Parainfluenza virus type 1 > type 2 ``` Tx: Humidifier, shower steam, cool night air Mild dz - dexamethasone x1 dose Moderate - keep pt calm, dexamethasone, nebulized epi, humidified oxygen Severe - intubation ```

Question 497

Child with acute-onset fever, sore throat, dysphagia, drooling, respiratory distress, "tripoding". CBC: leukocytosis with bandemia. XR: "thumb print" sign Diagnosis? Organism? Treatment?

Answer 497

Acute epiglottitis Haemophilus influenza type B Tx: Minimize stress/anxiety Intubation or tracheostomy asap Culture and sensitivity from blood and epiglottis surface Empiric IV Abx - Vanco or clinda + ceftriaxone or cefotaxime

Question 498

Child with 7-10 days of mild URI symptoms followed by 2-8 weeks of paroxysms of cough, worse at night and associated with post-tussive emesis and exhaustion. Diagnosis? Treatment?

Answer 498

Pertussis Tx: - Azithromycin x5d or TMP-SMX x14d - Azithromycin for close contacts - Isolation from school/daycare until after 5d of abx or 3 weeks after onset of symptoms - Hospitalization if: resp distress, pneumonia, inability to feed, cyanosis/apnea (+/- cough), seizures

Question 499

Infant with 1-3 days of rhinorrhea, mild cough, mild fever, poor feeding, cough, tachypnea, expiratory wheezing, crackles, nasal flaring, retractions, grunting. XR: Hyperinflation, patchy perihilar infiltrates Diagnosis? Organism? Treatment? Prevention?

Answer 499

RSV bronchiolitis Tx: Supportive care - IVF, O2 STEROIDS NOT EFFECTIVE ABX and RIBAVIRIN NOT USED Px: Palivizumab is used in premature infants to decrease risk of hospitalization

Question 500

Child with 2-3 days of fever, malaise, anorexia, cough, coryza, conjunctivitis. After 1-2 days, develops white-gray spots with red base on buccal mucosa. 2 days later develops erythematous maculopapular rash starting at the head and then spreading to the feet, lasting 4-5 days, resolves head down. Diagnosis? Treatment?

Answer 500

Measles (rubeola) Supportive therapy (antipyretics, fluids) Monitor/treat bacterial superinfections (i.e. pneumonia, otitis media) Vitamin A

Question 501

Child with low-grade fever, anorexia for 1-5 days. Then develops suboccipital/posterior cervical LAD. Then develops erythematous, tender, maculopapular rash that starts at the face, then generalizes. Rash persists x5d and does not darken. Pt's mom develops polyarthritis. Diagnosis?

Answer 501

German measles (Rubella)

Question 502

Child with sore throat, fever, HA, cough, coryza, and diarrhea x2-5 days. Then develops slapped-cheek rash that spreads to trunk and extremities. Rash resolves in 2 weeks. Diagnosis? Sickle cell risks? Pregnancy risks?

Answer 502

Parvovirus B19 aka erythema infectiosum aka 5th disease Sickle cell patients - aplastic crisis Pregnancy - hydrops fetalis and intrauterine fetal death

Question 503

Chid with sudden high fever (>102) for 3-4 days but acting normally. Develops rash that appears when fever dissipates and starts on the trunk, spreading over the entire body, lasting 24h. Associated erythematous papules on soft palate and uvula, mild cervical LAD, edematous eyelids, bulging anterior fontanelle in infants. Diagnosis? Treatment?

Answer 503

Roseola infantum (HHV6) (6th disease) Tx: Antipyretics Supportive

Question 504

Child with 4-5 days of periodic fever, apthous ulcers, pharyngitis, and adenitis. Occurs every 28 days for 4-8 years. Diagnosis? Treatment?

Answer 504

PFAPA syndrome. Tx: Self-limited Glucocorticoids relieve symptoms in matter of hours Tonsillectomy may be helpful

Question 505

Child presenting with short stature, hypopigmented spots and cafe-au-lait spots, abnormality of thumbs, micro/hydrocephaly, developmental delay. Labs: Pancytopenia ↑ alphafetoprotein. Bone marrow biopsy - hypocellularity, increased chromosomal breakage in lymphocytes. Diagnosis? Treatment? Complications?

Answer 505

Fanconi anemia Tx: hematopoietic cell transplantation Complications: death from bone marrow failure, increased risk of CA (leukemia, head/neck cancer)

Question 506

Child presenting with craniofacial abnormalities, opthalmologic abnormalities, neck anomalies, cardiac anomalies, thumb anomaly, GU malformations, pre and post-natal growth failure. Labs: Macrocytic anemia, ↓ reticulocytes, normal WBC, normal platelets. Bone marrow biopsy: normal cellularity but decreased/absent erythroid precursors. Diagnosis? Treatment?

Answer 506

Diamond-blackfan anemia Tx: Corticosteroids, blood transfusion

Question 507

Child with abdominal distension and pain, wt loss, malaise, bone pain, diarrhea, abdominal mass, HTN

Answer 507

Neuroblastoma - most common adrenal tumor in children (vs pheochromocytoma in adults) Tx: surgery

Question 508

Pt recently bit by a spider, complaining of muscle pain and spasm, diaphoresis, autonomic stimulation. Diagnosis? Treatment?

Answer 508

Black widow spider bite Latrotoxin: a neurotoxin that causes ACh release Tx: mild skin reaction resolve in

Question 509

Pt recently bit by spider, complaining of local ulceration and skin necrosis at bite site. Diagnosis? Treatment?

Answer 509

Brown recluse spider bite Tx: If ulcerated, wound care with dressing changes and debridement. If signs of infection/cellulitis/abscess, oral erythromycin Consider dapsone to possibly reduce extent of local necrosis due to leukocyte inhibitory properties (rule out G6PDD prior due to risk of hemolytic anemia)

Question 510

Drug toxicity: Mild - hypokalemia, hyperglycemia, vomiting Severe - seizure, hypotension, cardiac tachyarrhythmias What is the drug? Treatment?

Answer 510

Theyophylline * narrow therapeutic index (10-20mg/L) ``` Tx: Supportive measures - IVF, potassium ACLS protocol for SVT Benzos for seizures GI decontamination Hemodialysis ```

Question 511

Drug toxicity: Bradycardia, hypotension, hypoglycemia, pulmonary edema Drug? Treatment?

Answer 511

β-blockers and calcium channel blcokers ``` Tx: IVF Atropine to ↑ HR Glucagon Calcium Insulin and glucose ```

Question 512

Toxicity: Hyperpyrexia, decreased secretions, cutaneous flushing, cycloplegia and mydriasis, disorientation, constipation, urinary retention, tachycardia, ↓/absent bowel sounds. Drug? Treatment?

Answer 512

Anticholinergics Tx: Physostigmine

Question 513

Drug toxicity: Fatigue, blurred vision, change in color vision, anoreexia, n/v/d, abdominal pain, headache, dizziness, confusion, delirium ECG: prolonged PR, "scooping" ST segments, bradycardia, PVCs common +/- atrial tachycardia with AV block Labs: Hyperkalemia Drug? Treatment?

Answer 513

Digoxin toxicity ``` Tx: Digoxin Ab fragments if: - Life-threatening arrhythmia - Hyperkalemia with K>5 - End organ dysfunction Activated charcoal Atropine for bradycardia ```

Question 514

Pt presenting with hyperthermia, mixed respiratory alkalosis and metabolic acidosis, tachypnea, nausea, vomiting, dehydration, altered mental status, tinnitus. Diagnosis? Treatment?

Answer 514

ASA Overdose Tx: Charcoal, dialysis, sodium bicarbonate

Question 515

Pt presents with diarrhea, urination, miosis, bronchospasm, bradycardia, emesis and excitation of skeletal muscle, lacrimation, sweating, salivation. Diagnosis? Treatment?

Answer 515

Organophosphate poisoning Tx: Atropine (anticholinergic) Pralidoxime (reactivates acetylcholinesterase)

Question 516

Tx for iron overdose

Answer 516

deferoxamine, gastric lavage

Question 517

Tx for lead poisoning (peds and adults)

Answer 517

Peds with moderate toxicity - succimer Peds with severe toxicity - dimercaprol and calcium disodium edetate Adults - succimer

Question 518

Tx for mercury poisoning

Answer 518

Dimercaprol

Question 519

Tx for arsenic poisoning

Answer 519

Dimercaprol, succimer, penicillamine

Question 520

Pt found in domestic fire. Presenting with tachycardia and hypertension → bradycardia and hypotension, headache, confusion, seizure, coma, flushing, almond-scented breath. Diagnosis? Treatment?

Answer 520

Cyanide poisoning Tx: - Sodium thiosulfate - sulfur donor that facilitates the conversion of cyanide to thiocyanate, which is renally excreted - Hydroxocobalamin - binds cyanide to form cyanocobalamin (less toxic, excreted in urine) - Amyl nitrate and sodium nitrate - induces methemoglobin which binds cyanide to form cyanomethemoglobin.

Question 521

Pt >40yo Progressive exercise intolerance, dyspnea, dry crackles, JVD, tachypnea, possible digital clubbing. ↓ lung volume Normal FEV1/FVC XR: honeycomb lung CT: ground glass appearance Bronchioalveolar lavage shows ↑ PMNs Diagnosis? Treatment?

Answer 521

Idiopathic pulmonary fibrosis - Ineffective repair of alveolar epithelial cells injured by smoking, pollutants, microaspirations Tx: pirfenidone, nintedanib, sildenafil, cortiosteroids, antbiotics, lung transplant

Question 522

30yo black F complaining of cough, malaise, weight loss, dyspnea, arthritis (knees and ankles), chest pain, fever, tender red nodules on shins and arms, ALD, vision loss, cranial nerve palsies ``` ↑ ACE ↑ Ca ↑ urine Ca ↑ alk phos ↑ ESR ↓ WBC ↓ FVC and DLCO ``` CXR: bilateral hilar LAD and pulmonary infiltrates (ground glass appearance) Diagnosis? Treatment?

Answer 522

Sarcoidosis Tx: occasionally self-resolving; corticosteroids in chronic cases; cytotoxic drugs can be used with failure of steroids; lung transplant is rarely required

Question 523

Pt presenting with hemoptysis, dyspnea, recent respiratory infection PFT shows restrictive pattern but increased DLCO. UA shows proteinuria and granular casts. Renal biopsy shows crescentic glomerulonephritis and IgG deposition along glomerular capillaries. CXR shows bilateral alveolar infiltration Diagnosis? Treatment?

Answer 523

Goodpasture syndrome - progressive AI disease of lungs and kidneys caused by antiglomerular basement membrane (anti-GBM) Ab and characterized by intra-alveolar hemorrhage and glomerulonephritis

Question 524

Pt presenting with hemoptysis, dyspnea, myalgias, chronic sinusitis; ulcerations of nasopharynx, fever; mild hematuria, hearing loss, sensory neuropathy, CN dysfunction, conjunctivitis, corneal ulceration, arrhythmia Lung biopsy shows noncaseating granulomas Renal biopsy detects vasculitic process Diagnosis? Treatment?

Answer 524

Granulomatosis with polyangiitis (Wegeners) - rare disease with granulomatous inflammation and necrosis of lung and other organ systems + c-ANCA Tx: cytotoxic therapy (cyclophosphamide), corticosteroids - Rapidly fatal if left untreated

Question 525

↑PTH ↑ serum calcium ↓ serum phosphate Normal Vitamin D Diagnosis? Treatment?

Answer 525

Primary hyperparathyroidism Tx: surgical resection if single adenoma, resect 3 1/2 glands if there is 4-gland hyperplasia. Treat hypercalcemia with IVF and bisphosphonates. ``` If surgery is contraindicated/refused: Cinacalcet Avoid thiazides and lithium Adequate hydration Bisphosphonates ```

Question 526

↑ PTH ↓/NL serum calcium ↓ serum phosphate ↓ Vitamin D Diagnosis?

Answer 526

Rickets

Question 527

↑ PTH ↓/NL serum calcium ↑ serum phosphate ↓ 1,25-OH Vitamin D Diagnosis?

Answer 527

Secondary hyperparathyroidism from renal disease - kidneys unable to respond to PTH to increase excretion of phosphate and convert vitamin D

Question 528

Pt with short stature, seizures, poor mental development, hypocalcemia ↑ PTH ↓ serum calcium ↑ serum phosphate Normal 1,25-OH Vitamin D Diagnosis?

Answer 528

Pseudohypoparathyroidism - hypocalcemia resulting from tissue non-responsiveness to PTH; associated with developmental and skeletal abnormalities. (Albright hereditary osteodystrophy) * Administration of PTH causes no change in serum or urine Ca

Question 529

Cushingoid features ↑ Aldosterone ↓ Renin activity ↑ plasma aldosterone concentration : plasma renin activity ratio Diagnosis? Causes? Treatment?

Answer 529

Primary hyperaldosteronism (Conn syndrome) Causes: adrenal adenoma vs bilateral hyperplasia Tx: Spironolactone remove adenoma

Question 530

Cushingoid features ↑ Aldosterone ↑ Renin activity ↓ plasma aldosterone concentration : plasma renin activity ratio Diagnosis? Causes? Treatment?

Answer 530

Secondary hyperaldosteronism - ↑ RAAS due to ↓ renal perfusion Causes: renal artery stenosis, left-sided heart failure, nephrotic syndrome, cirrhosis Tx: spironolactone, treat underlying cause

Question 531

Tan pt presenting with weakness, fatigue, anorexia, wt loss, n/v, myalgias/arthralgias, decreased libido, mild psychosis, hypotension. Labs: ↓ Na, ↑K, eosinophilia, ↓ cortisol, ↑ ACTH Diagnosis? Treatment? Complications?

Answer 531

Primary adrenal insufficiency - AI destruction of adrenal cortices caused by AI disease, infection, hemorrhage; can occur with other endocrine processes Tx: glucocorticoid replacement (hydrocortisone, dexamethasone, prednisone), mineralocorticoid replacement (fludrocortisone) ** stress doses of steroids when needed Complications: adrenal/addisonian crisis - weakness, fever, AMS, vascular collapse caused by stress and increased need for cortisol; treat with IV glucose and hydrocortisone/vasopressors

Question 532

Tan pt presenting with weakness, fatigue, anorexia, wt loss, n/v, myalgias/arthralgias, decreased libido, mild psychosis, hypotension. Labs: ↓ Na, ↑K, eosinophilia, ↓ cortisol, ↓ ACTH Diagnosis?

Answer 532

Secondary or tertiary adrenal insufficiency Secondary - due to insufficient ACTH production by the pituitary Tertiary - due to insufficient CRH secretion by hypothalamus, usually due to chronic corticosteroid use

Question 533

Man with ambiguous genitalia Woman with amenorrhea HTN Labs: ↓ Na, ↑K, ↓ androgens Diagnosis? Treatment?

Answer 533

Congenital adrenal insufficiency 17-α-hydroxylase deficiency - deoxycorticosterone overproduction, cortisol/androgen/estrogen deficiency tx: cortisol replacement to achieve ACTH suppression Most children raised as females Estrogen-progesterone replacement for genotypic females at puberty Genotypic males may have reconstructive surgery of genitals

Question 534

``` Female infant with ambiguous genitalia Woman with virilization Man with macrogenitosomia and precocious puberty Dehydration Hypotension ``` Labs: ↓ Na, ↑K, ↑ androgens Diagnosis? Treatment?

Answer 534

Congenital adrenal insufficiency 21-α-hydroxylase deficiency - Partial deficiency of enzyme resulting in excess androstenedione and insufficient cortisol/aldosterone Tx: cortisol replacement therapy for ACTH suppression; fludrocortisone for mineralocorticoid replacement, reconstructive genital surgery

Question 535

``` Female infant with ambiguous genitalia Woman with virilization Man with macrogenitosomia and precocious puberty Dehydration Hypertension ``` Labs: ↑ androgens, ↑ deoxycorticosterone, ↑ deoxycortisol Diagnosis? Treatment?

Answer 535

Congenital adrenal insufficiency 11-β-hydroxylase deficiency - enzyme deficiency resulting in excess deoxycorticosterone, deoxycortisol, and androgens and insufficient cortisol and aldosterone Tx: cortisol replacement (hydrocortisone or dexamethasone) for ACTH suppression; anti-HTN may be required for persistent HTN

Question 536

Sudden palpitations, CP, diaphoresis, HA, anxiety, tachycardia, HTN Labs: ↑ 24h urinary catecholamines and metanephrines; ↑ plasma free metanephrines, ↑ 24h urinary vanillylmandelic acid Diagnosis? Treatment?

Answer 536

Pheochromocytoma - adrenal medulla tumor that secretes epinephrine and norepinephrine, leading to stimulation of sympathetic nervous system; rarely extra-adrenal in location Tx: surgical resection; α (phenoxybenzamine) and β blockers used before and during surgery to control BP; α must be given first before β to avoid hypertensive crisis from unopposed α

Question 537

Hypercalcemia, peptic ulcers, acromegaly, galactorrhea

Answer 537

MEN I Parathyroid adenoma Pancreas (islet cell) or GI endocrine tumor Pituitary adenoma

Question 538

Hypercalcemia, increased calcitonin, increased serum/urine catecholamines

Answer 538

MEN IIa Medullary thyroid cancer Parathyroid hyperplasia Pheochromocytoma

Question 539

Hypercalcemia, increased calcitonin, marfanoid body habitus, mucosal nodules

Answer 539

MEN IIb Mucosal neuromas Medullary thyroid cancer Pheochromocytoma

Question 540

Pt presenting with headache, neck stiffness, fever, mental status changes. CD4

Answer 540

Croptococcal meningitis Amphotericin B, fluconazole

Question 541

Pt presenting with vision loss, esophagitis, diarrhea. CD4

Answer 541

Cytomegalovirus Ganciclovir, foscarnet, valganciclovir

Question 542

Pt presenting with fatigue, weight loss, fever, diarrhea, abdominal pain, LAD, HSM. CD4

Answer 542

Mycobacterium avium complex (MAC) Clarithromycin, azithromycin, rifabutin, rifampin

Question 543

Pt presenting with cough, fever, dyspnea. CD4

Answer 543

Coccidioidomycosis Fluconazole, itraconazole, or amphotericin B

Question 544

Pt with gradual onset of nonproductive cough, dyspnea on exertion, fever CD4

Answer 544

Pneumocystis jirovecii pneumonia TMP-SMX, corticosteroids

Question 545

Pt presenting with abdominal pain, GI bleeding, skin lesions, dyspnea, meningitis, adrenal insufficiency. CD4

Answer 545

Histoplasmosis Long-term amphotericin B or itraconazole

Question 546

Pt with headache, confusion, possible focal neuro symptoms. CD4

Answer 546

Cerebral toxoplasmosis Pyrimethamine, sulfadiazine, clindamycin

Question 547

Abacavir MOA? Adverse effects?

Answer 547

NRTI Inhibits production of viral genome, prevents incorporation of viral DNA into host genome through reverse transcriptase inhibition.. SE of all NRTI's: lactic acidosis, lipodysptrophy, pancreatitis Specific for abacavir: hypertensitivity reaction

Question 548

Zidovudine MOA? Adverse effects?

Answer 548

NRTI Inhibits production of viral genome, prevents incorporation of viral DNA into host genome through reverse transcriptase inhibition.. SE of all NRTI's: lactic acidosis, lipodysptrophy, pancreatitis Specific for zidovudine: bone marrow toxicity

Question 549

Efavirenz MOA? Adverse effects?

Answer 549

NNRTI Inhibits reverse transcriptase activity to prevent viral replication. Rash Neuropsych effects (depression, nightmares) *** Teratogenic

Question 550

MOA and common side effects of protease inhibitors?

Answer 550

Interferes with viral replication to cause production of non-functional viruses SE: hypertriglyceridemia, hyperglycemia, GI toxicity, hyperbilirubinemia

Question 551

MOA and common side effects of integrase inhibitors?

Answer 551

Inhibit final step in integration of viral DNA into host DNA SE: Neutropenia, pancreatitis, hepatotoxicity, hyperglycemia

Question 552

Enfuvirtide MOA? Side effects?

Answer 552

Fusion inhibitor Binds to gp41, inhibits viral ability to fuse with CD4 membrane and enter cell. SE: hypersensitivity rxn at injection site, bacterial pneumonia ** must be injected and very expensive, only used in resistant disease

Question 553

Congenital heart disease, tetany, abnormal facial structure. H/o recurrent viral, protozoal, and fungal infections. ↓ serum calcium. Diagnosis? Treatment?

Answer 553

DiGeorge syndrome - T cell deficiency - chromosomal deletion in 22q11 resulting in thymic and parathyroid hypoplasia as well as congenital heart disease. Tx: Ca, Vitamin D, thymic transplant, bone marrow transplant, surgical correction of heart abnormalities; IVIG or abx prophylaxis

Question 554

H/o persistent infections of skin/nails/mucous membranes by Candida albicans. Possible ↓ IgG Frequently associated adrenal pathology. Diagnosis?

Answer 554

Chronic mucocutaneous candidiasis - T cell deficiency

Question 555

Boy with recurrent bacterial infections after 6mo of age. Diagnosis?

Answer 555

X-linked agammaglobulinemia B cell deficiency Defective tyrosine kinase gene Low levels of all immunoglobulins

Question 556

Recurrent sinus, lung, and gastrointestinal infections, associated with atopy and asthma. Possible anaphylaxis to blood transfusions and blood products. Diagnosis?

Answer 556

IgA deficiency due to abnormal immune globulin production by B cells

Question 557

Severe recurrent infections - chronic mucocutaneous candidiasis - fatal/recurrent RSV, VZV, HSV, measles, flu, parainfluenza - PCP pneumonia Chronic diarrhea Failure to thrive Diagnosis?

Answer 557

Severe combined immunodeficiency syndrome - absent T cells and abnormal Ab function resulting in severe immune compromise. Frequently fatal at an early age. Tx: bone marrow transplant, IVIG, Abx

Question 558

Recurrent sinus/lung infections Poor smooth pursuit of moving target with eyes Telangiectasis of face Radiation sensitivity Increased risk for lymphoma and acute leukemia Elevated AFP

Answer 558

Ataxia-telangiectasia

Question 559

Thrombocytopenia and purpura Easy bleeding Eczema Recurrent pyogenic infections due to encapsulated bacteria and opportunistic pathogens Diagnosis? Genetic analysis? Tx?

Answer 559

Wiskott-Aldrich syndrome - X-linked disorder of immune development. Genetic analysis detects abnormal WASP gene Tx: Splenectomy, Abx prophylaxis IVIG, bone marrow transplant

Question 560

Cutaneous, pulmonary, perirectal abscess formation Chronic LAD Nitroblue tetrazolium (NBT) test: no yellow to blue/black oxidation Diagnosis? Treatment?

Answer 560

Chronic granulomatous disease - lack of NADPH oxidase → phagoctes can't destroy catalase positive microbes. Especially susceptible to S. aureus, aspergillus Tx: prophylactic TMP/SMX and itraconazole. IFNɣ can be helpful

Question 561

Eczema Recurrent COLD S. aureus abscesses (no inflammatory response) Coarse facial features - broad nose, prominent forehead ("frontal bossing"), deep set eyes, "doughy" skin Retained primary teeth resulting in 2 rows of teeth. Diagnosis?

Answer 561

Hyper-IgE syndrome (Job syndrome) Mutation in the gene for STAT3 signaling protein leading to impaired differentiation of Th17 cells and impaired recruitment of neutrophils. High levels of IgE and eosinophils

Question 562

Partial albinism Recurrent respiratory tract and skin infections Neuro disorders (i.e. peripheral neuropathy, ataxia, seizures) Smear: giant cytoplasmic granules in PMNs

Answer 562

Chediak-Higashi syndrome Defective LYST gene (lysosomal transport) Defective phagocyte lysosomes → giant cytoplasmic granules in PMNs

Question 563

``` Recurrent bacterial infections of upper respiratory tract and skin Delayed separation of umbilical cord Short stature Abnormal facies Cognitive impairments ↑ serum neutrophils ```

Answer 563

Leukocyte adhesion deficiency - inability of neutrophils to leave circulation due to abnormal leukocyte integrins (type 1) or abnormal E-selectin (type 2). Defective chemotactic response of neutrophils upon stimulation.

Question 564

Adverse effect of cyclosporine

Answer 564

nephrotoxicity, androgenic effects, HTN

Question 565

Adverse effect of azathioprine. What medication can not be taken with azathioprine?

Answer 565

Leukopenia Azathioprine is metabolized by xanthine oxidase, therefore it can NOT be taken with Allopurinol

Question 566

Adverse effect of tacrolimus

Answer 566

Nephrotoxicity, neurotoxicity

Question 567

Adverse effect of Muromonab

Answer 567

1 time cytokine release (fever, bronchospasm), leukopenia

Question 568

Adverse effect of Rapamycin

Answer 568

Thrombocytopenia, hyperlipidemia

Question 569

Adverse effect of Mycophenolic acid

Answer 569

Leukopenia, GI toxicity, lymphoma

Question 570

Adverse effect of Hydroxychloroquine

Answer 570

Visual disturbance

Question 571

``` Hypophosphatemia Hypokalemia Hypomagnesemia CHF and arrhythmias Rhabdomyolysis Delirium Seizures ```

Answer 571

Refeeding syndrome - sudden shift from fat metabolism to carbohydrate metabolism

Question 572

40yoM comes to physician with 6mo h/o fatigue, weakness, and weight loss. Wife thinks his skin has gotten darker over this period. BP is 100/60. Labs show hyponatremia and hyperkalemia. Diagnosis? Treatment?

Answer 572

Adrenal insufficiency Tx: replace glucocorticoids with hydrocortisone/prednisone/dexamethasone, replace mineralocorticoids with fludrocortisone

Question 573

Middle aged/elderly pt with painful, fragile blisters in oropharynx, chest, face, perineal region. Blisters rupture easily and erosions are common. Skin biopsy: separating of epidermal cells (acantholysis) with intact basement membrane. Immunofluorescence demonstrates antiepidermal antibodies. Diagnosis? Treatment? Prognosis?

Answer 573

Pemphigus vulgaris - AI disorder characterized by autoantibodies to adhesion molecules in the epidermis ** almost always has oral lesions Tx: high dose systemic corticosteroids. Azathioprine or mycophenolate mofetil (steroid reducing adjuvant) Abx for secondary infections Prognosis: fatal if left untreated, mortality 5% even with treatment.

Question 574

>60yo patient with widespread blistering (esp on flexor surfaces and perineal region), pruritus, +/- erosions from blister rupture. Immunofluorescence shows antibasement membrane antibodies. Diagnosis? Treatment?

Answer 574

Bullous pemphigoid - AI disorder characterized by autoantibodies to epidermal basement membrane Tx: topical steroids (clobetasol) Oral steroids (prednisone) if topical steroids aren't possible (i.e. oral lesions) Chronic management - mycophenolate mofetil, azathioprine, methotrexate

Question 575

Pt with h/o chronic blistering lesions on sun-exposed skin (esp dorsa of hands, forearms, neck, face, ears, feet), hyperpigmented skin, facial hypertrichosis. Multiple scars from old blisters. Labs: ↑AST/ALT, ↑ total plasma porphyrin, ↑ urine porphyrins, Diagnosis? Risk factors? Treatment?

Answer 575

Porphyria cutanea tarda - deficiency of hepatic uroporphyrinogen decarboxylase, an enzyme involved in heme metabolism. Risk factors: alcoholism, hep C, iron overload, estrogen use, smoking Tx: Periodic phlebotomy, low-dose chloroquine/hydroxychloroquine, sunscreen use, avoid triggers (alcohol/estrogen/tobacco/iron supplements)

Question 576

Erythematous papule with rough, yellow-brone scales

Answer 576

Actinic keratosis - precancerous skin lesion that can progress to SCC Tx: Topical 5-FU or imiquimod, cryotherapy Complications: 0.1%/y risk of progression to SCC. ** 60% of SCC arises from actinic keratosis.

Question 577

Painless, erythematous papule with scaling or keratinized growth in sun-exposed area. Lesions may bleed, ulcerate, or be painful. Bx shows anaplastic epidermial cells extending to dermis. Diagnosis? Risk factors? Treatment? Complications?

Answer 577

Squamous cell carcinoma Risks - sun exposure (UVB), actinic keratosis, arsenic exposure, fair complexion, radiation. Tx: surgical excision, Mohs excision (serial shallow excisions w histologic analysis to minimize cosmetic damage) may be performed for lesions of the face, radiation for large lesions. Complications: progresses slowly, but can be large by the time it is diagnosed if in a poorly visualized area. 5-10% metastasize

Question 578

Pearly papule with telangiectasias. Bx shows basophilic-staining basal epidermal cells arranged in palisades. Diagnosis? Treatment? Complications?

Answer 578

Basal cell carcinoma - most common type of skin cancer Tx: surgical excision, Mohs excision, radiation, cryotherapy Complications: metastasize in

Question 579

Painless pigmented lesion with recent changes in appearance. Irregular borders, multiple colors, large/rapidly growing Bx shows atypical melanocytes and possible invasion into the dermis. Diagnosis? Subtypes? (4) Treatment? Complications?

Answer 579

Melanoma Types - Superficial spreading; most common - Nodular - grows vertically and rapidly invasive - Acral lentiginous - Lentigo maligna - long lasting in-situ stage Tx: surgical excision - 0.5cm margin if in situ - 1cm margin if 2mm thick +/- lymph node dissection Chemo and radiation if metastatic Complications - aggressive; lesions can have metastasized by time of discovery (most common - lung, brain, GIT) ** thickness >0.76mm associated with increased risk of metastasis

Question 580

20-30yo with sharply demarcated patches of complete depigmentation with hyperpigmented borders, most common at acral areas and around body orifices. Skin is of normal texture. Diagnosis? Comorbidities? Treatment?

Answer 580

Vitiligo - loss of melanocytes. Comorbidities: AI disorders: graves dz, autoimmune thyroiditis, pernicious anemia, type 1 DM, primary adrenal insufficiency, hypopituitarism, alopecia areata, AI hepatitis Tx: Minimize tanning of normal skin Makeup/dyes Topical corticosteroids (1st line) Tacrolimus or pimecrolimus (calcineurin inhibitors) Psoralens (topical/oral) + PUVA or UVB light Surgical mini grafting Hydroquinone for depigmentation of normal skin to match regions of vitiligo

Question 581

Purple red lesion on face that does not regress with age

Answer 581

port wine stain

Question 582

Infant with bright red lesion that regresses over months-years. Diagnosis? Treatment?

Answer 582

Infantile (strawberry) hemangioma Tx: * Uncomplicated will resolve w/in first 2 years of life Complicated - oral propanolol, systemic glucocorticoids, vincristine, interferon alpha

Question 583

Benign, small red papule that appears on skin with age

Answer 583

Cherry hemangioma

Question 584

Bright red papule with radiating blanching vessels

Answer 584

Spider angioma - think liver cirrhosis

Question 585

Blue, compressible mass that does not regress

Answer 585

Cavernous hemangioma

Question 586

Red pink nodule on a child that is often confused with melanoma

Answer 586

Spitz nevus

Question 587

Asymptomatic, inflammatory, non-scarring areas of complete hair loss, may be precipitated by stress. Diagnosis? What labs should you order? Treatment?

Answer 587

Alopecia areata Obtain RPR, CBC, BMP, ESR, TSH, ANA (r/o pernicious anemia, chronic active hepatitis, thyroid disease, SLE, addison's disease) Regrowth after 1st attack: 30% by 6mo, 50% by 1y, 80% by 5y 10-30% will no regrow 5% progress to total hair loss ``` Tx: Intralesional steroid injections Topical corticosteroids (less effective, less penetration into scalp) Topical minoxidil Topical immunotherapy Topical anthralin Oral corticosteroids ```

Question 588

Telogen effluvium

Answer 588

Diffuse stress-related hair loss

Question 589

Another name for: | Ascorbic acid

Answer 589

Vitamin C

Question 590

Another name for: | Cholecalciferol

Answer 590

Vitamin D

Question 591

Another name for: | Cyanocobalamin

Answer 591

Vitamin B12

Question 592

Another name for: | Folic acid

Answer 592

Vitamin B9

Question 593

Another name for: | Niacin

Answer 593

Vitamin B3

Question 594

Another name for: | Pyridoxine

Answer 594

Vitamin B6

Question 595

Another name for: | Thiamine

Answer 595

Vitamin B1

Question 596

Another name for: | Retinol

Answer 596

Vitamin A

Question 597

Another name for: | Riboflavin

Answer 597

Vitamin B2

Question 598

What vitamin deficiency matches this description: | Increased RBC fragility

Answer 598

Vitamin E

Question 599

What vitamin deficiency matches this description: | Dermatitis, cheilosis, glossitis

Answer 599

Vitamins B2, B3, B9, B12

Question 600

What vitamin deficiency matches this description: | Peripheral neuropathy, angular cheilosis, glossitis

Answer 600

Vitamin B12

Question 601

What vitamin deficiency matches this description: | Hemorrhagic disease

Answer 601

Vitamin K

Question 602

What vitamin deficiency matches this description: | Neural tube defects

Answer 602

Vitamin B9 (folic acid)

Question 603

What vitamin deficiency matches this description: | Dermatitis, diarrhea, dementia

Answer 603

Vitamin B3 (niacin)

Question 604

What vitamin deficiency matches this description: | Megaloblastic anemia

Answer 604

Vitamins B9 (folic acid) and B12

Question 605

What vitamin deficiency matches this description: | Pernicious anemia

Answer 605

Vitamin B12

Question 606

What vitamin deficiency matches this description: | Bitot's spots, keratomalacia, xeropthalmia

Answer 606

Vitamin A

Question 607

What vitamin deficiency matches this description: | Osteomalacia and rickets

Answer 607

Vitamin D

Question 608

What vitamin deficiency matches this description: | Opthalmoplegia, unsteady gait, confusion

Answer 608

Vitamin B1 (thiamine)

Question 609

What vitamin deficiency matches this description: | Alcoholic with anterograde amnesia and confabulation

Answer 609

Vitamin B1 (thiamine)

Question 610

What vitamin deficiency matches this description: | Loose teeth, poor wound healing, bleeding/bruising

Answer 610

Vitamin C

Question 611

What vitamin is associated with: Treatment of acne and psoriasis

Answer 611

Vitamin A Vitamin D can be used with psoriasis only

Question 612

What vitamin is associated with: Involved in hydroxylation of prolyl residues in the formation of collagen

Answer 612

Vitamin C

Question 613

What vitamin is associated with: | Requires intrinsic factor for absorption

Answer 613

Vitamin B12

Question 614

What vitamin is associated with: | Deficiency may result from kidney disease

Answer 614

Vitamin D

Question 615

What vitamin is associated with: | Given prophylactically to newborns

Answer 615

Vitamin K

Question 616

What vitamin is associated with: | Can be used to elevated HDL and lower LDL

Answer 616

Vitamin B3 (niacin)

Question 617

What vitamin is associated with: | Given to patients taking isoniazid to prevent peripheral neuropathy

Answer 617

Vitamin B6 (pyridoxine)

Question 618

What vitamin is associated with: | Contains cobalt

Answer 618

Vitamin B12 (cyanocobalamin)