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USMLE2 Medicine 06
GI
| Question | Answer |
|---|---|
| how to best dx UC? how to best dx Crohn's? | both with colonoscopy with bx |
| "string sign" on small bowel follow through | Crohn's or colon CA. pt drinks contrast --> xrays of distal duodenum/duodenojejunal junction to the ileocecal valve. |
| how to tx acute exacerbations of UC? CD? | high- dose steroids (budesonide) |
| CD or UC associated with palpable and painful abd masses? | CD. Transmural granulomas in the bowel wall --> inflammed --> stick together --> form mass. |
| skip lesions | CD (anywhere from mouth to anus) |
| large bowel involvement only - UC or CD? | UC |
| fistula formation - UC or CD? | CD |
| oral and perianal involvement | CD |
| more likely to cause bloody diarrhea - UC or CD | UC (with mucus) |
| What is UC/CD relationship with CA? | IBD will lead to colon CA after 8 -10 years of colon involvement. If CD does not involve the colon, then it will not lead to CA. |
| Ca oxalate kidney stones and cholesterol gallstones associated with UC or CD? | CD. Kidney stones 2/2 fat malabsorption --> low Ca + increased absorption of oxalate --> kidney stone formation |
| anti-Saccharomyces cerevisiae Ab's (ASCA) | CD. AS-Crohn's |
| cANCA associated with what? | UC and Wegener's |
| prothrombin time in Crohn's | prolonged because of Vit K malabsorption |
| Mesalamine (3 drugs) | anti-inflammatory (5-ASA) for IBD. PANtasa - released in upper and lower bowel - used in CD. AS(S)acol - released in colon - used in UC. Rowasa (Row around the Rectum) - used only for rectal disease. |
| side effects of sulfa drugs | Sulfa is a HLR IN pts. Hemolysis, Leukopenia, Rash, Interstitial Nephritis (allergic) |
| IBD drugs | 6-SAM. 6-mercaptopurine, Steroids for flares, Azathioprine, Mesalamine derivatives |
| what are azathioprine and 6-mercaptopurine associated with | drug-induced pancreatitis |
| drugs to use in CD with perianal disease | cipro and metronidazole |
| drug to use in CD if have fistulae or refractory disease | infliximab |
| most common side effects of infliximab | arthralgias |
| Surg in IBD - better for UC or CD? | curative in UC. Not effective in CD and will recur at site of anastomosis. |
| which has more extra-intestinal manifestations? UC or CD? | CD |
| which has more likely to have arthritis? UC or CD? | CD |
| perianal disease - UC or CD? | CD |
| mucosal cobblestoning | Crohn's |
| fistulas, fissures, strictures | Crohn's |
| lead pipe colon on barium enema | UC |
| which form of IBD has risk for megacolon with perforation? | UC |
| transmural lesions | Crohn's |
| granulomas | Crohn's |
| Crypt abscesses | UC |
| what is the most important thing when assessing diarrhea | is pt hypovolemic? (hypotension, orthostasis) |
| what to do if see bloody diarrhea | abx (also, if fever) |
| w/u of diarrhea | CBC, chem10, Stool (O&P, cx, WBC's) |
| most common causes of infectious diarrhea | campylobacter and salmonella (esp in sickle cell and achlorhydria) |
| refried Chinese food | b.cereus (spores, vomiting 1-6 h later, never blood) |
| infectious diarrhea in reactive arthritis or Guillian Barre | campylobacter |
| diarrhea in HIV+ pt CD4<100 (2 organisms) | Cryptosporidia or Isospora |
| pathogenesis of e. coli 0157:H7 | hamburger meat --> Shiga toxin --> HUS once organism dies --> abx contraindicated (because don't want to kill them if they're in there!) |
| what is contraindicated in diarrhea caused by e. coli 0157:H7 | abx and plt transfusions |
| unfiltered water in mountains | Giardia (never blood, +fullness, bloating, gas) |
| chicken eggs, dairy products | salmonella |
| vomiting, diarrhea, flushing, wheezing minutes after eating tuna, mahi mahi, mackerel | Scombroid. Org invade fish --> histamine released into fish --> eating dish give you huge dose of histamine |
| raw shellfish, mussels, oysters, clams | vibrio parahaemolyticus |
| raw shellfish in pts with liver disease or d/o iron metabolism | vibrio vulnificans |
| associated with development of skin bullae | vibrio vulnificans |
| child in day care, no WBCs or blood on stool studies | viral |
| dairy products, eggs, salads --> mostly n/v 1-6 h after eating | S. aureus |
| grouper, red snapper, barracuda --> 2-6 hours get diarrhea and neuro sx's (paresthesia, weakness, reversal of heat/cold | Ciguiatera-toxin |
| when are fecal leukocytes positive? | invasion of the intestinal mucosa --> bloody diarrhea (dysentery) |
| name all the organisms that can cause invasive bloody diarrhea | S Sh C VP VV Y E. VV is the VP of Camp ShEY (chez) Surrender. Vibrio vulnificans, Vibrio parahaemolyticus, Campylobacter, Shigella, E.coli, Yersina, Salmonella |
| how to dx Cryptosporidiosis | modified acid-fast stool |
| how to dx Giardia | ELISA stool antigen |
| best empiric tx for infectious diarrhea | cipro +/- metronidazole |
| how to tx scromboid poisoning? | anti-histamine diphenhydramine |
| how to tx giardia | metronidazole/tinidazole |
| how to tx Cryptosporidiosis | paromomycin, raise CD4 >100 with antiretrovirals |
| abx most likely to cause C. diff diarrhea | clindamycin, but really, any abx |
| how to dx C.diff | stool c diff toxin |
| how to tx c.diff colitis | oral metronidazole. oral vanc if refractory (can't use this IV because doesn't get through bowel wall). IV metronidazole if NPO. |
| how to dx lactose intolerance | usu tx empirically but -- inc stool osmolality and inc osmolar gap (measured stool osmolality is greater than would be expected just by the Na and K --> extra osmoles from lactose. DDx (also panc insuff, celiac, bacterial overgrowth) |
| IBS | irritable bowel syndrome, inc peristaltic contractions |
| abd pain, diarrhea/constipation, woman, h/o childhood abuse | IBS |
| Dx IBS | Rome criteria (at least 3 months). 1. Pain relieved by BM or change in bowel habit (developing diarrhea makes pain go away), 2. fewer sx's at night, 3. diarrhea alternating with constipation |
| What sx's DON'T you have in IBS? | fever, wt loss, anorexia, anemia |
| Tx for IBS | high fiber diet, anti-diarrheal agents (loperamide, alosetron), antispasmodics (hyoscyamine, belladonna), TCAs (anti-depressant, analgesic for neuropathic pain), tegaserod (for constipation) |
| Carcinoid syndrome | tumor of the neuroendocrine system that releases serotonin. in GI (appendix, ileum, rectum) and lung (rare in bronchus). metastatic. |
| when does a pt get sx's of carcinoid syndrome? | 1. when tumor is in bronchi --> serotonin directly into systemic circulation. 2. when liver is overwhelmed and can't metabolize all the serotonin anymore (late in dz) |
| Sx's of carcinoid syndrome | Carcinoid has a FHx of DTR's. Flushing, HypoTN, Diarrhea, Tachy, Rash (niacin def) |
| what deficiency in Carcinoid and why? | Serotonin and niacin both from tryptophan. Serotonin overproduction uses up tryptophan --> niacin deficiency. |
| endocardial fibrosis | carcinoid (constant exposure of R heart to serotonin) |
| cardiac effects of carcinoid syndrome | RH endocardial fibrosis, tricuspid insuff, pulmonic stenosis |
| Dx of carcinoid syndrome | Urinary 5-HIAA level |
| Tx of carcinoid | octreotide (somatostain analog, control diarrhea). if localized tumor --> resection (most often possible with bronchial) |
| Causes of fat malabsorption: | all have steatorrhea and wt loss: celiac, pancreatitis, tropical sprue, Whipple disease, giardia |
| why do pts with malabsorption also have bleeding abnormalities and hypocalcemia? | ADEK malabsorption, esp vit D (Ca) and vit K. |
| malabsorption where causes Fe deficiency? | duodenum |
| Fe def anemia is what kind of anemia? | Microcytic |
| malabsorption where causes B12 deficiency? | terminal ileum |
| most common presentation of celiac disease | Fe deficiency anemia |
| dermatitis herpetiformis | celiac (skin rash on extensor surfaces) |
| severe UC attack | Ulcerative colitis: definition of a severe attack A STATE: 1. Anemia less than 10g/dl, 2. Stool frequency greater than 6 stools/day with blood, 3. T >37.5, 4. Alb < 30g/L 4. Tachycardia greater than 90bpm, 5. ESR greater than 30mm/hr |
| Causes of pancreatitis | GET SMASHED. Gallstones, EtOH, Trauma, Steroids, Mumps (paramyxovirus, other viruses - EBV/CMV, Autoimmune (PAN, SLE), Scorpion/Snake, HLP/HyperCa/Hypotherm, ERCP, Drugs (SAND - 6-mercaptopurine/sulfonamide, Azathioprine, NSAIDs, diuretics - furosemide) |
| Indications for surg in IBD | IBD: surgery indications "I CHOP": Infection Carcinoma Haemorrhage Obstruction Perforation |
| extra-intestinal manifestations of IBD | IBD: extraintestinal manifestations A PIE SAC: Aphthous ulcers Pyoderma gangrenosum Iritis Erythema nodosum Sclerosing cholangitis Arthritis Clubbing of fingertips |
| Complications of UC | Ulcerative colitis: complications "PAST Colitis": Pyoderma gangrenosum, Ankylosing spondylitis, Sclerosing pericholangitis, Toxic megacolon, Colon carcinoma |
| Crohn's sx's | CHRISTMAS: Cobblestones, High temperature, Reduced lumen, Intestinal fistulae, Skip lesions, Transmural (all layers, may ulcerate), Malabsorption, Abdominal pain, Submucosal fibrosis |
| Whipple Disease | infectious dz caused by Tropheryma whipplei. David DHALMO has Whipple's. Dementia/Diarrhea, Hyperpigmentation, Arthritis, LAD/fatty deposits in lymph nodes of the intestine, Malabsorption, Oculomasticatory myorhythmia. Also Fever. |
| Tropical sprue | malabsorption disease commonly found in the tropical regions, marked with abnormal flattening of the villi and inflammation of the lining of the small intestine; not autoimmune like celiac is. |
| oculomasticatory myorhythmia | pendular vergence oscillations of the eyes and synchronous contractions of the masticatory but not palatal muscles) is pathognomonic of Whipple disease |
| anti-gliadin | Celiac's |
| anti-endomysial | Celiac's |
| anti-transglutaminase | Celiac's |
| how to diagnose Celiac's and what do you find? | small bowel bx, find flattening of villi |
| Why does chronic pancreatitis cause VitB12 deficiency? | B12 requires proteases from the pancreas to be cleaved from its carrier in the duodenum so that it can attach to IF. only when B12 is attached to IF can it be absorbed in the terminal ileum. |
| Why are Vit K and Ca malabsorbed in Celiac's | Vit K is a fat-soluble vitamin that is absorbed in the terminal ileum, which is damaged in Celiac's. Ca requires Vit D, which is also a fat-soluble vitamin. Also, when fat is not absorbed, it binds to Ca. |
| how is tropical sprue diagnosed? | bowel wall bx showing the organism that caused it |
| Most sensitive test for Whipple Disease | PCR of bowel bx showing Tropheryma whippelli |
| bx that shows foamy macrophages that are PAS + | Whipple Disease |
| Tx of chronic pancreatitis | oral enzyme replacement |
| tx for tropical sprue | bactrim (SMZ/TMP) or doxy x 6mo |
| tx for Whipple disease | bactrim (SMZ/TMP) or doxy or CTX x 1yr |
| Diverticulosis | outpocketings of the colonic mucosa and submucosa through weaknesses of muscle layers in the colon wall. more common in sigmoid colon - a common place for increased pressure. |
| Who gets diverticulosis | >40 yo, increasing with age |
| What causes diverticulosis? | lack of fiber --> decreased bulk in stool --> increased intracolonic P --> outpocketing of colon |
| colicky LLQ pain, 60yo man, painless bloody stool | diverticulosis |
| Dx of diverticulosis | colonoscopy |
| diverticula more common where in the bowel? Bleeding more commonly from where? | L -- diverticula more common, R -- bleeding more common because of thinner mucosa |
| diverticulitis | infection in one of the diverticula (entrance to diverticula blocked by something like corn or nut) |
| fever, LLQ tenderness, increased WBC, h/o diverticulosis | consider diverticulitis |
| how to dx diverticulitis | CT |
| what studies are contraindicated in diverticulitis? | endoscopy and barium since danger of perforation |
| tx for diverticulitis | abx (cipro and metronidazole) |
| tx for diverticulosis | more fiber in diet |
| how do TCA's and CCB's affect bowel movements | cause constipation |
| risk factors for colon CA | fam hx (most are sporadic), smoking, high fat and high red meat diet |
| which side of colon is affected if pt comes in with heme-positive brown stool and chronic anemia | R |
| which side of the colon is affected if pt comes in with narrowing of stool caliber or obstruction? | L or sigmoid |
| what bacteria is associated with colon CA | S bovis, clostridium septicum |
| pt comes in with endocarditis from Strep bovis. what should be considered? | w/u for colon CA (also for clostridium septicum) |
| dx colon ca | colonoscopy with bx |
| resectable vs non-resectable colon CA | resectable if localized and not touching outermost layer (serosa) or nodes |
| Tx for colon CA | resect if not spread beyond muscularis layers (before the serosa); chemo if spread (use 5FU); if only 1 liver met then resect. |
| screening for colon CA | FOBT qyr; sigmoidoscopy q5yr; colonoscopy q10yr. starting at age 50. |
| what causes false + FOBT? false -? | Fe, red meat, poultry, ASA, NSAIDs. False - caused by Vit C. |
| What happens if a polyp is found on colonoscopy? | repeat colonoscopy q3-5yr |
| when should screening begin for someone with fam h/o colon CA? | at 40, or 10 years earlier than the youngest age of diagnosis in family member, whichever comes first |
| Lynch Syndrome | Hereditary Non-polyposis - HN. 3 family members in at least 2 generations with colon CA, at least 1 dx <50. |
| Cancers associated with Lynch Sydrome | Hereditary Non-polyposis. Colon CA by definition, but also Ovarian and Endometrial. Lynch Amy COE! |
| CA screening for people with family h/o Lynch syndrome | colonoscopy q yr starting at age 25 |
| Familial Adenomatous Polyposis gene | APC - adenomatous polyposis coli |
| what is the chance that a pt will develop colon CA if they have the APC gene? | 100% chance of adenoma by age 35 (usu polyps by age 25), and colon CA by age 50. |
| CA screening for people with family h/o Familial Adenomatous Polyposis (FAP) | flex sig q1-2yr starting at age 12!!! |
| Prophylaxis for Familial Adenomatous Polyposis | as soon as polyp found, perform COLECTOMY and make NEW RECTUM from terminal ileum! |
| Juvenile Polyposis Syndrome (JPS) - how much risk for colon CA? | 10% |
| Difference in polyps found in Familial Adenomatous Polyposis vs. Juvenile Polyposis Syndrome | Familial Adenomatous Polyposis polyps are adenomas. Juvenile Polyposis Syndrome polyps are hamartomas (much less risk of developing into CA) |
| rectal bleeding in childhood | Juvenile Polyposis Syndrome or Cowden Syndrome |
| Cowden Syndrome | genetic polyposis syndrome with harmartoma polyps, only slightly inc risk of colon CA compared to general population |
| Gardner Syndrome | Colon CA + soft tissue tumors (osteomas, lipomas, cysts, fibrosarcomas) |
| mandible osteoma | Gardner Syndrome - do colonoscopy looking for colon CA! |
| how to dx Gardner Syndrome | colonoscopy |
| Peutz Jehgers Syndrome | harmatomatous polyps in bowels with hyperpigmented melanotic spots on lips, buccal mucosa, and skin. |
| Harmartomatous polyps | Peutz Jehgers, Cowden Syndrome, Juvenile Polyposis Syndrome. PJ had to use GPS (JPS) to get to her C/S to deliver her baby Hamma (Hannah). |
| Peutz Jehgers Syndrome and risk of colon CA | only slightly more than general population |
| abd pain due to obstruction or intussusception, plus hyperpigmentation on lips and skin | Peutz Jehgers Syndrome |
| xray finds osteoma. what would you do? | Gardner Syndrome - do colonoscopy looking for colon CA! |
| Turcot Syndrome | Colon CA with CNS malignancy. Turban made of colon wrapped about the head (CNS). |
| what is the most important step when pt presents with significant GIB? | fluid resuscitation |
| pt has active GIB + elevated PT. what should pt get? | fluid resuscitation PLUS FFP |
| Pt has active GIB and plt <50K. what should you do? | fluid resuscitation PLUS plt transfusion |
| pt with active GIB and h/o cirrhosis. what should you do? | fluid resuscitation PLUS octreotide (decreases portal htn) |
| pt with GIB and gastritis or h/o ulcer. What should to start? | fluid resuscitation PLUS PPI (not H2 blocker) |
| T or F: 80% of GIB will spontaneously resolve with supportive therapy, regardless of etiology | true |
| dx GIB | colonoscopy |
| pt with GIB and esophageal varices. What should to start? | IVF and octreotide to lower portal htn |
| if supportive therapy does not work for bleeding esophageal varices, then what? | banding via emergency endoscopy or sclerotherapy or TIPS (transjugular intrahepatic portosystemic shunting - connects portal vein to hepatic veins so that blood bypasses liver and decrease PHTN) |
| complication of TIPS procedure | worsening of hepatic encephalopathy |
| pt has portal HTN and esophageal varices. what drug should they be on? | propranolol - decreases frequency of bleeding |
| most common causes of UGIB | bleeding proximal to the ligament of Treitz. Ddx: ulcer, gastritis, Mallory Weiss, esophagitis and gastric CA. UGMEC. Upper GastroEnterology Can Make the diagnosis. |
| UGIB with h/o AAA repair in last year | think: aortic-enteric fistula |
| DDx for LGIB | diverticulosis, AVM, hemorroids, CA, IBD. CHADI has LGIB |
| red blood in stool vs. black stool or melena | red blood- LGIB vs. black stool or melena - UGIB |
| melena indicates how much blood has been lost? | at least 100mL |
| orthostasis - in terms of HR | >10 rise in HR when pt lying to standing/sitting |
| orthostasis in terms of BP | >20 pt drop in SBP from lying to standing/sitting |
| what does orthostasis indicate in terms of volume | 15-20% blood loss or decrease in intravascular volume |
| HR > 100/min or SBP <100 indicates what? | >30% blood loss |
| how to detect low volume GIB? | nuclear bleeding scan |
| two most common causes of endoscopy | EtOH and gallstones |
| what causes the pathology in pancreatitis? | premature activation of trypsinogen into trypsin while still in the pancreas --> autodigestion of pancreas |
| midepigastric pain that radiates through to the back, tenderness, n/v | pancreatitis |
| Cullen sign | blue discoloration around the umbilicus --> hemoperitoneum |
| Turner's sign | Must "turn" to flanks to see Turner's. Blue-ish purple discoloration around the flanks --> tissue catabolism of Hb --> bleed |
| which is better in pancreatitis? amylase or lipase? | lipase more specific to the pancreas |
| Best test for pancreatitis | CT |
| Ranson criteria for pancreatitis: at admission | "GA LAW": Glucose >200, AST >250, LDH >350, Age >55 y.o., WBC >16000 |
| Ranson criteria for pancreatitis: initial 48 hours | "C & HOBBS" (Calvin and Hobbes): Calcium < 8, Hct drop > 10%, Oxygen < 60 mm, BUN > 5, Base deficit > 4, Sequestration of fluid > 6L |
| best test to see biliary and pancreatic ductal pathology | ERCP |
| test to predict severity of pancreatitis | Urinary trypsinogen activation peptide |
| tx for pancreatitis. what if there's pancreatic necrosis? necrosis and fever? necrosis and infection? | bowel rest, pain meds, IVF. If necrosis on CT scan: abx (imipenum). If necrosis and persistent fever: percutaneous needle bx. If infection + necrosis, then surgical debridement. |
| when do pancreatic pseudocysts occur? | 2-4 weeks after episode of pancreatitis. |
| When do you need to drain a pancreatic pseudocyst? | pain, fistula, rupture, expanding (when cyst is >5cm) |
| most common cause of cirrhosis in US | EtOH |
| most common reason to need a liver transplant | HepC |
| Causes of cirrhosis | Cirrhosis is caused by a HEPatitis WASH. HepC, EtPH, Primary biliary cirrhosis, Wilson’s disease, A-1-anti-trypsin deficiency, Sclerosing cholangitis, Hemochromatosis. |
| physical manifestations of cirrhosis | ascited, peripheral edema, spider angiomata, palmar erythema, asterixis, encephalopathy, jaundice & pruritis |
| where are clotting factors made? | all clotting factors are made in the liver except F8 and vWF (these are made in EC's) |
| how to diagnose SBP? | spontaneous bacterial peritonitis if WBC>500/mm3, neutrophils >250/mm3 on paracentesis |
| tx SBP | cefotaxime or CTX PLUS albumin (decreases risk of hepato-renal syndrome) |
| SAAG and its interpretation of ascites fluid | serum ascites albumin gradient. the diff btwn the two is usually >>1 because serum albumin is way greater than albumin in ascetic fluid. if SAAG > 1.1, PHTN or cirrhosis is usu cause of ascites. If SAAG <1.1, albumin in ascites is high --> CA or infxn |
| what is a complication of cirrhosis? | hepatocellular CA |
| what is the diuretic most effective in cirrhosis? why? | spironolactone. cirrhotic pts have intravascular volume depletion --> high aldosterone (keeps Na, H2O, excretes K H). Spironolactone blocks aldosterone. |
| how to treat hepatic encephalopathy. how does the drug work? | neomycin or lactulose. nonabsorbed disaccharide that bacteria metab in colon --> makes colon more acidic --> NH3 to NH4+ --> NH4+ isn't absorbed well --> excretion of ammonia |
| is it effective to give Vit K to cirrhotic pts with coag problems? | no because the liver isn't able to synth clotting factors |
| what is primary biliary cirrhosis? | idiopathic autoimmune d/o. damage of the small biliary ducts in the liver--> bile builds up --> damage to liver |
| who gets primary biliary cirrhosis? | middle aged women |
| how often do we see high bili leves in pt with primary biliary cirrhosis? | only if the disease is very very advanced |
| primary biliary cirrhosis is associated with what other autoimmune diseases? | Sjogren, RA, and scleroderma |
| middle aged woman with fatigue, pruritis, with elevated alkPhos/GGTP, nl LFT's, and osteoporosis | primary biliary cirrhosis |
| what is the most specific blood test for primary biliary cirrhosis? | anti-mitochondrial Ab |
| tx for primary biliary cirrhosis | no real tx. UV light for pruritis, liver txplt for late stage disease |
| primary sclerosing cholangitis | progressive inflammation and scarring of the bile ducts of the liver (2/2 autoimmunity) --> impedes flow of bile to the gut --> cirrhosis/failure/CA. definitive treatment is liver transplantation. |
| anti-mitochondrial Ab | primary biliary cirrhosis. Mity-mouse - PB and Chocolate |
| primary sclerosing cholangitis is associated with what disease | IBD, most likely UC |
| most specific test for primary sclerosing cholangitis | ERCP or transhepatic cholangiogram. this is the only chronic liver disease in which liver bx is not most accurate test |
| primary sclerosing cholangitis tx | no cure. could try cholestyramine (bile-acid-binding resin), UV light for pruritis, liver txnsplnt for late stage. |
| hemochromatosis | over-absorption of Fe in duodenum --> Fe buildup in other tissues --> chronic hepatic inflammation/fibrosis (cirrhosis) |
| hemochromatosis associated with what CA? | hepatocellular CA |
| what cardiac condition associated with hemochromatosis? | restrictive cardiomyopathy |
| what infections are associated with hemochromatosis | Vibrio vulnificans and yersinia because of their affinity for Fe. VV and Yersinia love pumping iron! |
| Screening test for hemochromatosis. What is best test? | Screen is Fe blood level. Dx is liver bx. |
| Tx of hemochromatosis | phlebotomy to remove large amounts of Fe from body. deferoxamine in pts who can't undergo phlebotomy. |
| pt presenting with choreoathetoid movements and psychosis. what should you do next? | do slit lamp exam --> kayser-fleischer rings --> Wilson's disease |
| Wilson disease genetics | autosomal recessive |
| Wilson disease | can't excrete Cu, inc absorption of Cu thru intestines; Cu buildup makes one feel BLE. Brain, Liver, Eye. in brain (basal ganglia - choreoathetosis), liver, cornea (Kayser Fleischer rings). |
| kidney problems in Wilson disease | Cu deposition in kidney --> Fanconi Syndrome (fanCuKidni). Loss of HCO3 in Fanconi will lead to type 2 proximal renal tubular acidosis. |
| Fanconi Syndrome | disease of the proximal renal tubules of the kidney in which glucose, amino acids, uric acid, phosphate and bicarbonate are passed into the urine, instead of being reabsorbed |
| Tx for Wilson disease | penicillamine - chelator. liver txplnt is curative. |
| a-1-antitrypsin deficiency genetics | autosomal recessive |
| cirrhosis and emphysema in a young person, never smoker | a-1-antitrypsin deficiency |
| dx a-1-antitrypsin deficiency | blood level of enzyme |
| tx for a-1-antitrypsin deficiency | none. enzyme replacement and don't smoke |
| Dx Hep B and Hep C | Hep B - HepB surface antigen (HBsAg) persists >6mo. HepC - Ab to Hep C + elevated viral load by PCR. Most accurate for both is liver bx. |
| Tx for HepB | interferon, lamivudine, or adefovir |
| Tx for Hep C | pegylated interferon AND ribavarin |