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83 Cards in this Set
- Front
- Back
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What is non-alcoholic fatty liver disease?
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looks like alcoholic liver disease in ppl who are not heavy drinkers
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What is non-alcoholic fatty liver disease associated with?
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obesity, dislipidemia, hyperinsulinemia, DMII
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What is non-alcholic fatty liver disease like?
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mainly asymptomatic
70% of chronic hepatitis 30% cirrhosis (crypotgenic) |
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How do you treat non-alcoholic fatty liver disease?
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diet & exercise
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What is hemochromatosis?
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excessive accumulation of iron in liver
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What causes hemochromatosis?
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genetic defect where lack the major excretory pathway for iron excretion
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What does hemochromatosis cause?
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cirrhosis
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What gene is mutated in hemochromatosis?
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HFE
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What does mutations in HFE cause?
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inabiiltiy to down regulate iron uptake protiens leading to excessive life-long excessive absorbtion of iron
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What are some features of fully developed hemochromatosis?
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micronodular cirrhosis
diabetes skin pigmentation (bronze) |
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What sex gets hemochromatosis more and why?
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men cause womens menstration aids in the loss of iron
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How is iron directly toxic to host cells?
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lipid peroxidation
stimulation of collagen formation reactive oxygen species interaction |
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What is wilson's disease?
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accumulation of copper in liver, brain & eyes
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What causes wilson's disease?
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defective bilinary excretion
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What accounts for 95% of ciruclating bound copper?
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ceruloplasmin
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What are the mainfestations of wilson's disease?
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liver disease, neuropsychic changes, Kayser-Fleisher rings in eyes
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What is a kayser-fleisher ring?
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green/brown deposits of copper in the cornea
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What is the most commonly diagnosed genetic liver disease in infants?
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a1-antitrypsin deficiency
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What are the diseases caused by a1-antitrypsin defiecncy?
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emphysema & liver disease
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What do you see histologically in the liver of kids with a1-antitrypsin deficiency?
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misfolded protien in vesicles of hepatocytes
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What are clinical features of a1-antitrypsin deficiency?
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neonatal hepatitis w cholestatic jaundice
cirrhosis later in life hepatocellular carcinoma |
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What causes neonatal cholestasis?
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prolonged unconjugated hyperbilirubinemia
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What is the clinical presentation of neonatal cholestasis?
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jaundice, dark urine, light stools, hepatomegaly
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What are light stools called?
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acholic
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What are some major causes of neonatal cholestasis?
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bile duct obstsruction
a1-antitrypsin deficiency idiopathic |
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What percent of neonatal cholestasis is idiopathic?
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50%
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What are some other causes of neonatal cholestasis?
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neonatal infection
toxins metabolic diseases other... |
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What is the cause of secondary bilinary cirrhosis?
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obstruction of extrahepatic biliary tree
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What are the most common causes of secondary bilinary cirrhosis in adults?
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1. extrahepatic cholelithiasis
2. malignancys of bilinary tree @ head of pancreas 3. strictures from surgery |
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What are the most common causes of secondary bilinary cirrhosis in children?
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1. biliary atresia
2. cystic fibrosisd 3. choledochal cysts |
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What can prolonged cholestais lead to?
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inflammation, periportal fibrosis, hepatic scarring, cirrhosis
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What do you see in lab findings for secondary bilinary cirrhosis?
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increased: conjugated bilirubin, ALK phos, bile acids, cholesterol, GGT, 5'NT
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What are important findings before cirrhosis in secondary bilinary cirrhosis?
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bile stasis, bile duct proliferation with PMNs and edema
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What is primary biliary cirrhosis?
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nonsppurative, inflammatory destriction of medium sized intrahepatic bile ducts
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What do you get with primary bilinary cirrhosis?
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insidious onset with prurtis, jaudice appears late, hepatomegaly, anthomas due to cholesterol retention and cirrhosis
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Who gets primary bilinary cirrhosis?
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women 6:1
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What labs do you get with primary bilinary cirrhosis?
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increased: serum ALK, cholesterol, conjugated bilirubin, AMA, GGT, 5'NT
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What is the pathogenesis of primary bilinary cirrhosis?
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autoimmune
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what are important findings before cirrhosis in primary bilinary cirrhosis?
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dense lymphocytic portal inflammation wiht granulomatous inflamation of bile ducts
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What is primary sclerosing cholangitis?
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inflammation & obliterative fibrosis of intrahepatic & extrahepatic bile ducts
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What coexists in 70% of pts with primary sclerosing cholangitis?
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IBD
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Who gets primary sclerosing cholangitis?
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males in the 3-5th decade
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what is the pathogenesis of primary sclerosing cholangitis?
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inflammatory cytokines or ischemia
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What are the clinical features of primary sclerosing cholangitis?
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progressive fatigue, pruritis, jaundice
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What labs do you have with primary sclerosing cholangitis?
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persistenly elevated ALK
AMA in <10% |
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What are you at risk for with primary sclerosing cholangitis?
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cholangiocarcinoma (bile duct cancer)
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Describe liver infarcts
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typically subcapsular
rare due to dual blood supply |
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What is budd-chiari syndrome?
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obstruction of >2 hepatic veins due to thrombosis
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What do you see clincally wiht Budd-Chiari Syndrome?
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liver enlargement, asciteis, RUQ pain due to increased hepatic BP secondary to obstruction
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What is veno-occlusice liver disease?
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obliteration of hepatic vein radicles
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When does veno-occlusive liver disease occur?
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weeks following BM transplant in 25% of pts
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What does this post BM transplant veno-occulusive disease cause?
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sponto recovery
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What is the clincal diagnosis of veno-occlusive disease?
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hepatomegaly, ascites, weight gain, jaundice
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What is the pathogenesis of veno-occlusive disease?
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toxic injury to sinusoidal epthithelium
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What are some hepatic diseases associated with pregnancy?
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pre-eclampsia
eclampsia HELLP syndrome complications |
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What is pre-eclampsia?
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maternal HTN, prteinuria, peripherial edema, coagularion abnormalities, DIC
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What is eclampsia?
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pre-eclampsia progresses with hyper-reflexia & convulsions
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What is HELLP syndrome?
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Hemolysis, elevated liver enzymes, low platelets, pre-eclampsia
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What are some clinical manifestations of liver nodules & tumors?
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epigastric fullness & discomfort
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Who get nodular hyperplasia?
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young to middle aged females
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What is focal nodular hyperplasia?
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encapsulated scar contains large arteries - fibromuscular hyperplasia
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What is nodular regenrateive hyperplasia?
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entire liver affected with nodules, in abscence of fibrosis
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What is nodular regenreative hyperplasia associated with?
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portal HTN related to renal transplantation, BM transplant & vascular conditions
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What are 2 benign neoplasms of the liver?
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cavernous hemagioma
liver cell adenoma |
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What is cavernous hemagioma?
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most commonly subcapsular
biopsy may cause bleeding |
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Who gets liver cell adenoma?
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in young females using oral conraceptives, regress with stopping meds
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What is the clinical significanc of liver cell adenoma?
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tendency to rupture (PG)
mistaken for hepatocellular CA may harbor hepatocellular CA |
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What do you see histologially with liver cell adenoma?
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irregular cords of hepatocytes with vasuclar supply but no portal tracts
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What is a liver cell adenoma grossly?
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pendulous mass
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What are some metastatic liver tumors?
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metastatiic
hepatoblastoma angiosarcoma |
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Where do mets come from?
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breast, GI, lung
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What is hepatblastoma?
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MC malignant liver tumor of young kids that is fatal if not resected within 1 year
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What are the 2 variants of hepatoblastoma?
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eptihelial type - resemble normal liver
mixed epithelia & mesnchymal - foci of osteoid, cartilage or muscle |
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What is angiosarcoma assoicated with?
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exposure to vivyl chloride, arsenic or thorotrast
- long latency period |
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What are key etiological associations with hepatocellular carcinoma?
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viral hep
alcoholism food conatminants (aflotoxin) tyroinemia hereditary hemochromatosis |
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What is a variant of hepatocellular carcinoma?
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fibromellar
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Who does fibrolamellar hepatocellular carcinoma occur in?
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young 20-40
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What is fibrolamellar hepatocellular carcinoma not associated with?
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HBV or cirrhosis
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What is the prognosis of fibrolamellar hepatocellular carcinoma?
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better
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What do you see histologically with fibrolamellar hepatocellular carcinoma?
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cords of hepatocytes sperated by dense bundles of collagen
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What is cholangiocarcinoma?
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malignancy of bilinary tree areising within or outside the liver
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What are the risk factors for cholangiocarcinoma?
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sclerosing cholangitis, Caroli disease, choledochal cysts & exposure to thorotrast
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How is cholangiocarcinoma detected?
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obstruction or hepatic mass
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