UWorld 1 Flashcards

Question

Cirrhosis, neuropsych symptoms, and brownish/gray-green rings in eye in young adult. What disease? Background info.

Answer 1

Hepatolenticular Degeneration (WIlson Disease) Kayser-Fleisher Rings are the eye rings - granular copper deposits in cornea Rare, autosomal recessive disease most often identified in younger people ages 5-40. The genetic mutations involved hinder Cu metabolism by reducing the formation and secretion of ceruloplasmin and by decreasing the secretion of Cu into the biliary system. Cu is pro-oxidant, and as it accumulates in greater quantities within the liver, it causes damage to hepatic tissue through the generation of free radicals. Eventually, Cu leaks from injured hepatocytes into the blood to be deposited in various tissues, including the basal ganglia (hepatolenticular degeneration) and cornea

Answer 2

ASx liver function abnormalities, chronic hepatitis, fulminant hepatitis, portal HTN, or macronodular cirrhosis. Neuropsych - Parkinson-like tremor, rigidity, ataxia, slurred speech, drooling, personality changes, depression, paranoia, catatonia. Wilson's is also associated with Fanconi Anemia, hemolytic anemia, and neuropathy Gold standard for dx is Liver Bx that shows hepatic copper greater than 250 dry weight. More commonly, dx is confirmed by low serum ceruloplasmin (particularly less than 20) in conjunction with increased urinary copper excretion or Kayser-Fleishcer rings

Answer 3

Must be adhered to for the patient's lifetime and focuses on removing accumulated copper in the tissues and preventing re-accumulation First line meds - copper chelators like D-penicillamine or trientine Oral Zinc is also recommended as it prevents copper absorption Liver transplant may be only option of those with fulminant hepatitis or decompensated liver disease that does not respond to pharm

Answer 4

Color 1) Bloody - trauma, malignancy, TB (rarely) 2) Milky - Chylous, pancreatic 3) Turbid - Possible infection 4) Straw color - likely more benign causes Neutrophils 1) Less than 250 - no peritonitis 2) 250 and up - Peritonitis (secondary or spontaneous bacterial) Total protein 1) 2.5 and up (high protein ascites) - CHF, constrictive pancreatitis, peritoneal carcinomatosis, TB, Budd-Chiari Syndrome, Fungal (coccidiomycosis) 2) Less than 2.5 - Cirrhosis, nephrotic syndrome SAAG 1) 1.1 and up (indicates portal HTN) - cardiac ascites, cirrhosis, Budd-Chiari 2) Less than 1.1 (absence of portal HTN) - TB, peritoneal carcinomatosis, pancreatic ascites, nephrotic syndrome

Answer 5

Serum-to-Ascites Albumin Gradient. Subtract the peritoneal albumin from the serum albumin Above 1.1 - possible portal HTN etiology, therefore the ascites may be due to increased hydrostatic pressure within hepatic capillary beds Less than 1.1 - Increased capillary permeability (eg malignant ascites, pancreatitis, nephrotic, TB)

Answer 6

1) General - Bulky, foul smelling, floating stools 2) Fat and protein - Loss of muscle mass, loss of subq fat, fatigue 3) Iron - Pallor (anemia), fatigue 4) Calcium and Vit D - Bone pain (osteomalacia), fracture (osteoporosis) 5) Vit K - Easy bruising 6) Vit A - Hyperkeratosis

Answer 7

IgA anti-tissue transglutaminase and IgA anti-endomysial antibodies BUT, many patients with bx-confirmed (villous atrophy) disease will have negative antibody results since IgA deficiency is fairly common in Celiac If suspicious for Celiac is high but IgA serology is negative, measure total IgA (or get IgG based serology)

Answer 8

Uncommon disorder producing chronic watery diarrhea. Colon is frequently involved, but colonoscopy shows normal mucosa. Bx shows mucosal subepithelial collagen deposition

Answer 9

Epi 1) Age 20-50 2) 80% Sporadic/20% MEN1 Clinical 1) Multiple and refractory peptic ulcers 2) Ulcers distal to duodenum 3) Chronic diarrhea Dx 1) Markedly elevated serum gastrin (more than 1000) in the presence of normal gastric acid (pH less than 4) Workup 1) Endoscopy 2) CT/MRI and somatostatin receptor scintigraphy for tumor localization

Answer 10

Gastrinoma is usually in pancreas or duodenum Dx is suggested by marked elevation in serum gastrin (above 1000) in presence of acidic gastric pH (less than 4). Endoscopy reveals ulcers usually (90%) and sometimes will ID a primary duodenal gastronoma CT, MRI and somatostatin receptor scintigraphy can be used to ID pancreatic tumors and metastatic disease If gastrinoma is confirmed, patients should be screened for MEN with assays for PTH, ionized Ca, and Prolactin

Answer 11

Patients with compensated cirrhosis typically are either ASx or complain of vague symptoms (anorexia, weakness, fatigue) Those with decompensated cirrhosis may present with jaundice, pruritus, upper GI bleeding, abdominal distention due to ascites, or confusion due to hepatic encephalopathy

Answer 12

Esophageal varices. They can occur in half of patients.

Answer 13

Liver biopsy, though clinical impression and history is usually enough

Answer 14

Early Primary biliary cholangitis (formerly primary biliary cirrhosis) Chronic, progressive liver disease characterized by cholestasis with autoimmune destruction of intrahepatic bile ducts Most common in middle aged women and has insidious onset. Pruritus and fatigue are usually first symptoms. As it progresses, jaundice, steatorrhea, HSM, eyelid xanthelasma, portal HTN, and osteopenia may develop Drug of choice - ursodeoxycholic acid. Less effective in advanced disease that may require liver transplant.

Answer 15

used in a number of cholestatic disorders. It is a hydrophilic bile acid that decreases biliary injury by the more hydrophobic endogenous bile acids. Also increases biliary secretion and may have additional anti-inflammatory effects. It delays progression in PBC and may improve symptoms and possibly survival. Start it in PBC as soon as dx is made, even if patient is asymptomatic Liver transplant is definitive treatment but only indicated in those with severe liver damage or cirrhosis

Answer 16

Focal nodular hyperplasia 1) Associated with anomalous arteries 2) Arterial flow and central scar on imaging Hepatic adenoma 1) Women on long term OCPs 2) Possible hemorrhage or malignant transformation Regenerative nodules 1) Acute or chronic liver injury (cirrhosis) HCC 1) Systemic symptoms 2) Chronic hepatitis or cirrhosis 3) Elevated AFP Liver mets 1) Single/multiple lesions 2) Known extrahepatic malignancy

Answer 17

It's benign! US shows well-demarcated, hyperechoic lesions Contrast CT can show early peripheral enhancement. Needle bx is not recommended for suspected hepatic adenoma due to risk of bleeding. Surgical excision is preferred. Long term complications are progressive growth, rupture, and malignant transformation

Answer 18

Characterized by rapidly enlarging liver mass, often with satellite lesions. It usually occurs in patients with cirrhosis or chronic hepatitis (esp B)

Answer 19

Caused by echinococcus tapeworm. In USA, most cases are seen in immigrants, although occasional cases may be seen in individuals from the southwest who have been exposed to sheep and dogs It causes cystic not solid liver lesions

Answer 20

Definition 1) Hepatic steatosis on imaging or biopsy 2) Exclusion of significant alcohol use 3) Exclusion of other causes of fatty liver Clinical 1) Mostly ASx 2) Metabolic Syndrome 3) With or without steatohepatitis (AST/ALT less than 1) 4) Hyperechoic texture on US Tx 1) Diet and exercise 2) Consider bariatric surgery if BMI is at least 35

Answer 21

Middle aged, obese, with features of the metabolic syndrome

Answer 22

Can range from bland steatosis to inflammation and necrosis (steatohepatitis) to fibrosis and cirrhosis Can resemble alcohol-induced fatty liver (macrovesicular fat deposition, peripheral displacement of nuclei) but occurs in patients with minimal or no alcohol history

Answer 23

Can be due to increased transport of free fatty acids from adipose tissue to the liver, decreased oxidation of FFA in the liver, or decreased clearance of FFA from the liver (due to decreased VLDL production) Frequently related to peripheral insulin resistance leading to increased peripheral lipolysis, triglyceride synthesis, and hepatic uptake of fatty acids Hepatic FFA increases oxidative stress and production of proinflammatory cytokines (tumor necrosis factory alpha)

Answer 24

Characterized by the development of severe hemorrhagic erosive lesions after exposure to gastric mucosa to various injurious agents or after a substantial reduction in blood flow Aspirin decreases the protective prostaglandin production. In addition, aspirin and alcohol cause direct mucosal injury Acute mucosal injury decreases the normal protective barriers (decreased levels of secreted mucins and bicarb and decreased integrity of the epithelium), thus permitting acid and other luminal substances (proteases and bile acids) to penetrate into the lamina propria, thereby causing additional injury to vasculature and subsequent hemorrhage. Patients may manifest with hematemesis and abdominal pain ***NSAIDs and aspirin cause acute erosive gastritis and upper GI bleeding. Alcohol can make it worse. It is NOT the platelet dysfunction that NSAIDs cause. It is direct injury.

Answer 25

1) Chronic alcohol use 2) Gallstones 3) Hyperlipidemia (types 1, 4, and 5) 4) Drugs (didanosine, azathioprine, valproic acid) 5) Infections (CMV, Legionella, Aspergillus) 6) Trauma 7) Iatrogenic (post-ERCP)

Answer 26

Diagnosis requires 2 of the following 1) Acute epigastric abd pain often radiating to the back 2) Increased amylase, lipase (more than 3 times normal limit) 3) Abdominal imaging showing focal or diffuse pancreatic enlargement with heterogeneous enhancement with IV contrast (CT) or diffusely enlarged and hypoechoic pancreas (US) Other findings 1) n/v, leukocytosis 2) Severe disease with possible abdominal tenderness, fever, tachypnea, hypoxemia and hypotension 3) ALT level above 150 - biliary pancreatitis

Answer 27

1) Pleural effusion 2) Ileus 3) Pancreatic pseudocyst/abscess/necrosis 4) ARDS

Answer 28

Early in pancreatitis, the pancreas synthesizes digestive enzymes but cannot secrete them. These enzymes leak out of the acinar cells into the systemic circulation. Amylase rises within 6-12h of symptom onset and may remain elevated for 3-5 days. Lipase rises within 4-8h of symptom onset but remains elevated longer than amylase (8-14d) As a result, lipase is more useful and sensitive than amylase for dx (esp in alcoholics and patients presenting later in disease course)

Answer 29

If diagnosis is unclear, or patient does not improve with conservative management (to ID infection or necrosis) Once diagnosis is made, US can be done to find cause (gallstones) Early ERCP for suspected biliary pancreatitis may decrease morbidity and mortality. ERCP is also useful for evaluating patients with recurrent pancreatitis or draining pancreatic pseudocysts

Answer 30

Low serum Fe and ferritin with high TIBC

Answer 31

Should be considered to be from GI blood loss (polyps, cancer, angiodysplasia) until proven otherwise A positive FOBT without lab values is enough to guide management to colonoscopy and endoscopy A negative FOBT is not enough to rule out occult GI bleed. Elderly patients with IDA should get colonoscopy and endoscopy despite a single negative FOBT, esp if no other obvious source of chronic blood loss is identified.

Answer 32

Radioisotope scan that is useful in determining the source of active bleeding but not diagnostic in absence of active bleed Typically used in acute (NOT chronic) GI blood loss when source of bleeding remains unidentified on colonoscopy and endoscopy

Answer 33

More common 1) Chronic hepatitis B or C infections - IV drug use, blood transfusions, multiple sexual contacts 2) Alcoholic liver disease - excessive alcohol intake 3) Nonalcoholic fatty liver disease - Diabetes, obesity and/or metabolic syndrome 4) Hemochromatosis - FHx of cirrhosis; history of diabetes, bronze skin, high transferrin saturation Less common 1) Autoimmune - FHx of cirrhosis, history of other coexisting autoimmune disorders (thyroiditis), anti-smooth muscle/liver kidney microsomal type 1 antibodies 2) PBC - more common in women, fatigue, pruritus, elevated alk phos, positive antimitochondrial antibody 3) PSC - Associated with IBD 4) Alpha 1 antitrypsin deficiency - coexisting lung involvement. FHx of cirrhosis 5) Cardiac cirrhosis - coexisting R-sided HF 6) Meds - Methotrexate, isoniazid 7) Wilson Disease - FHx of cirrhosis at young age, KF rings in eyes

Answer 34

Alcohol or hepatitis or NAFLD. Pick one of these 3 if the question is about "most likely" in USA

Answer 35

Etiology 1) Sudden increase in abdominal pressure (forceful retching, blunt abdominal trauma) 2) Mucosal tear in esophagus or stomach (submucosal arterial or venous plexus bleeding) 3) Risk - hiatal hernia, alcoholism*** Clinical 1) Vomiting, retching 2) Hematemesis 3) Epigastric pain Dx 1) Longitudinal laceration on endoscopy near GEJ Tx 1) Most heal spontaneously 2) Endoscopy therapy for persistent bleeding (electrocoagulation or local injection of epinephrine)

Answer 36

Typically presents with total or segmental nonobstructive colonic dilation, severe bloody diarrhea and systemic findings (fever, tachy) IBD patients are at the highest risk of developing it early in the disease, within 3 years of dx. Other causes of toxic megacolon: 1) Ischemic colitis 2) Volvulus 3) Diverticulitis 4) Infections (C Dif) 5) Obstructive colon cancer (less common) Dx is confirmed on plain abdominal XR and at least of the following: 1) Fever above 38C 2) Pulse over 120 3) WBC over 10,500 4) Anemia May also see volume depletion, AMS, hypotension, lyte issues. Plain XR shows dilated Right or Transverse Colon (greater than 6cm), possible multiple air-fluid levels, and thick haustral markings that do not extend across the entire lumen Tx - it's an emergency! Can cause perforation. Give IV fluids, broad spectrum ABX, NG decompression, and bowel rest. IV steroids are preferred for treating IBD-induced toxic megacolon Emergency surgery (subtotal colectomy with end ileostomy as procedure of choice) may be needed if it does not resolve

Answer 37

monosccharide that can be absorbed in the proximal small intestine WITHOUT degradation by pancreatic or brush border enzymes It is then excreted in the urine. In the D-xylose test, patient is given an oral dose with subsequent assay of urine and venous blood. Patients with proximal intestine mucosal disease (Celiac) cannot absorb D-xylose in the intestine, and urinary and venous D-xylose levels will be low. Fecal levels will be high. Patients with malabsorption due to enzyme deficiencies (chronic pancreatitis) will have normal absorption of D-xylose.

Answer 38

False positive is decreased urinary excretion of xylose even though they have normal mucosal absorption. Can be seen in patients with delayed gastric emptying or impaired glomerular filtration.

Answer 39

Characterized by alterations in the flora of the SI, usually due to abnormal intestinal anatomy or motility, A false positive D-xylose test can be seen in SIBO due to bacterial fermentation of the ingested d-xylose dose before it can be absorbed. Treatment with Rifaximin will improve this condition (if D-xylose is still positive on rifaximin, the prob is most likely Celiac)

Answer 40

Causes diarrhea, abdominal pain, and flatulence after consuming milk or milk-containing products. Normal D-xylose.

Answer 41

Presence of proteins and fatty acids in the duodenum acts as a stimulus for release of CCK, which in turn stimulates the contraction of the gallbladder. In patients on TPN or prolonged fasting, the normal stimulus for CCK release and GB contraction is absent. This leads to biliary stasis and promotes the formation of bile sludge and gallstones. Small bowel (ileal) resection (or Crohn's) also contributes to formation of gallstones via DECREASED enterohepatic circulation of bile acids that results in altered hepatic bile composition, which becomes supersaturated with cholesterol and promotes gallstone formation

Answer 42

Estrogen-induced increase in cholesterol secretion. Estrogen causes an increase in cholesterol secretion and progesterone causes a reduction in bile acid secretion, causing increased cholesterol saturation of bile. Progesterone also slows GB emptying and facilitates formation of cholesterol gallstones.

Answer 43

Pathophys - Uncoordinated, simultaneous contractions of esophageal body Symptoms - intermittent Chest pain, dysphagia for solids and liquids Dx - Esophagram shows "corkscrew pattern." Manometry - intermittent peristalsis, multiple simultaneous contractions Tx - CCBs. Alternative is nitrates or TCAs Endoscopy is often normal.

Answer 44

Angina due to coronary artery spasm. Typical symptoms include episodic chest pain at rest. Patients may have normal ECG findings btw attacks, with variable ST abnormalities during acute events. Dysphagia rules this out.

Answer 45

Functional disorder of esophagus characterized by sensation of foreign body in throat. Often worse when swallowing saliva and is frequently associated with anxiety. Pain, dysphagia, dysphonia or systemic symptoms are NOT TYPICAL and would suggest something else.

Answer 46

Occurs in setting of hypotension (septic shock, heart failure) and manifests as acute, massive increases in AST and ALT with milder associated increases in total bili and alk phos. In patients who survive the inciting condition, liver enzymes typically return to normal within a few weeks. Look for a patient who comes in in shock and develops massive liver enzyme elevations like a day later.

Answer 47

Should raise suspicion for C dif, even without diarrhea

Answer 48

Recent hospitalization, advanced age, antibiotic use (fluoroquinolones, penicillins, cephalosporins, clindamycin)

Answer 49

Common in patients with longstanding indwelling urinary catheters Actual symptomatic fungal UTIs are rare. In cases of uncomplicated symptomatic fungal UTIs, antifungals arent needed.

Answer 50

empiric metronidazole while awaiting stool studies

Answer 51

GI malignancies like colorectal or pancreatic cancers since venous drainage is through the portal system directly to liver Lung, breast, skin also spread to liver often. Liver is common site for mets bc of its dual blood supply (systemic and portal) and hepatic sinusoidal fenestrations allow for easier met deposition

Answer 52

1) Supportive care (volume repletion [albumin] if needed, restraints if agitated, correct lytes) 2) Adequate nutrition without protein-restricted diet unless patient is unable to tolerate protein intake 3) Treat precipitating cause (GI bleed, hypovolemia, infection, lyte abnormalities) 4) Lower serum ammonia. 4a) Lactulose orally or enema if unable to take oral 4b) Rifaximin orally if no improvement in 48h with lactulose or unable to take lactulose

Answer 53

Hypokalemia increases renal ammonia production. Metabolic alkalosis (from intravascular volume depletion, poor oral intake) stimulates conversion of ammonium (can't cross BBB) to ammonia (can cross BBB). This is why patients with HE and hypoK require intravascular volume repletion (albumin) and K repletion. Disaccharides (lactulose or lacitol) convert ammonia to ammonium (ammonia trap).

Answer 54

Shingles - consider it when patients have regional pain and no conclusive evidence of disease in the local internal organs

Answer 55

Can occur at any age. Most common after age 50. Risk goes up with age. Frequently triggered by severe physical stress (cancer tx) or immunosuppressed states, but many cases are spontaneous Reactivation of varicella zoster in DRG where it was dormant since last chickenpox infection Pain may precede rash by several days

Answer 56

Patients may develop persistent hypersensitivity of afferent pain fibers after shingles infection.

Answer 57

Antivirals (acyclovir, valacyclovir, famciclovir) in first few days of outbreak can shorten the duration of symptoms and decrease the risk of post-herpetic neuralgia

Answer 58

Type 1 1) Primary hyperparathyroidism (more than 90%) 2) Enteropancreatic tumors (60-70%) 3) Pituitary tumors (10-20%) Type 2A 1) MTC (More than 90%) 2) Pheochromocytoma (40-50%) 3) Parathyroid hyperplasia (10-20%) Type 2B 1) MTC 2) Pheo 3) Other - mucosal and intestinal neuromas, marfanoid habitus

Answer 59

Abdominal pain, microcytic anemia, positive FOBT, and hepatomegaly with a hard edge Moderate abnormalities in LFTs (high alk phos with mild elevations in AST and ALT) - this makes a liver injury less likely (more likely to be infiltrative or cholestatic in the case described) Pleural effusion on Left (hepatic hydrothorax from cirrhosis is on the right usually)

Answer 60

Liver is often shrunken and edge is not palpable. AST usually greater than ALT by factor of 1.5-2

Answer 61

Presents with difficulty initiating swallowing due to inability to properly transfer food from mouth to pharynx Etiologies include stroke, advanced dementia, oropharyngeal malignancy, neuromuscular disorders (MG)

Answer 62

Coughing, choking, nasal regurgitation on swallowing. Aspiration pneumonia and weight loss** (look for recurrent RLL pneumonias)

Answer 63

Videofluoroscopic modified barium swallow

Answer 64

FHx of adenomatous polyps or CRC - 1st degree relative at age younger than 60 1) Colonoscopy at age 40 OR 10 years before the age of dx in the relative (whichever comes first) 2) Repeat every 3-5y IBD - UC or Crohn 1) Begin 8 years post diagnosis (12-15 years if disease only in L colon) 2) Colonoscopy with biopsies every 1-2 years Familial adenomatous polyposis 1) Begin at age 10-12 2) Colonoscopy every year Hereditary nonpolyposis CRC (Lynch Syndrome) 1) Begin at age 20-25 2) Colonoscopy every 1-2 years

Answer 65

Gallstones without symptoms - No tx needed in most patients (only 20% of ASx gallstones will develop symptoms within 15y) - exception is patients at higher risk of developing gallbladder cancer (like if they have a porcelain gallbladder) Gallstones with typical biliary colic symptoms - elective lap chole, possible ursodeoxycholic acid in poor surgical candidates Complicated gallstone disease (acute cholecystitis, choledocholithiasis, gallstone pancreatitis) - chole within 72h

Answer 66

Etiology 1) ***Alcohol use (even prolonged use of socially acceptable amounts) 2) Cystic fibrosis (common in kids) 3) Ductal obstruction (malignancy, stones) 4) Autoimmune Clinical presentation 1) Chronic epigastric pain with intermittent pain-free intervals 2) Malabsorption - steatorrhea, weight loss (from reduced exocrine function like less amylase, protease, lipase) 3) Diabetes mellitus Lab results/imaging 1) Amylase/lipase can be normal and nondiagnositc 2) CT scan or MRCP can show calcifications, dilated ducts and enlarged pancreas Tx 1) Pain management 2) Alcohol and smoking cessation 3) Frequent, small meals 4) Pancreatic enzyme supplements

Answer 67

Can present with dull abdominal pain (usually after eating) and unintentional weight loss due to avoidance of food. Mood cases due to atherosclerosis Dx with mesenteric angiogram

Answer 68

Patient presents with dysphagia to solids and liquids and a dilated esophagus with smooth tapering of distal esophagus. Primary achalasia is loss of peristalsis in distal esophagus with lack of LES relaxation. Pseudo is due to esophageal cancer (narrowing of distal esophagus NOT due to denervation) So what clues point you to pseudo? 1) Tobacco and alcohol use are major risk factors for esophageal cancer 2) Significant weight loss 3) rapid symptom onset (less than 6 months) 4) Presentation at age greater than 60 Moral of the story: In all patients with suspected achalasia, get an endoscopy to rule out malignancy In primary achalasia, it is usually easy to pass scope through LES and scope will show normal mucosa and dilated esophagus If endoscopy shows malignancy, do CT for staging

Answer 69

Low surgical risk - Lap myotomy, pneumatic balloon dilatation High surgical risk - BoTox, nitrates, CCBs ONLY consider treatment after malignancy is excluded by endoscopy

Answer 70

Subtypes 1) Adeno - distal esophagus, arises from Barret esophagus 2) SCC - anywhere in esophagus Risk 1) Acid reflux, obesity, also smoking (adeno) 2) Smoking, alcohol, caustic injury (SCC) Symptoms 1) CP 2) Weight loss 3) Dysphagia (solids) Dx 1) Endoscopy with bx 2) CT (PET/CT) for staging

Answer 71

Young, low risk patients with undetermined esophageal symptoms may start with Barium esophagram Those over 55 OR who have alarm symptoms (weight loss, gross or occult bleeding, early satiety) will usually go straight to endoscopy