Term
| Nonalcoholic Fatty Liver Disease (NAFLD) |
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Definition
| hepatic steatosis without alcohol consumption, inflammation, steatohepatitis, oxidative stress is cause, AST/ALT ratio less than one. Mallory bodies |
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Term
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Definition
| excessive accumulation of body iron. homozygous-recessive disorder. micronodular cirrhosis, diabetes mellitus, skin pigmentation. Genes: HFE (adult), hepcidin HAMP deficiency, hemojuvelin HJV, transferrin receptor 2 TfR2 |
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Term
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Definition
| secondary hemochromatosis, often from blood transfusions |
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Term
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Definition
| autosomal recessive, ATP7B, impaired copper excretion and failure to incorporate copper into ceruloplasmin, hepatic steatosis, vacuolated nuclei, mallory bodies, rhodamine stain, Kayser-Fleischer rings |
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Term
| alpha one-antitrypsin deficiency |
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Definition
| PiZZ protein mutation is most commonly significant, defect in ER to golgi migration. round-to-oval cytoplasmic globular inclusions in hepatocytes. PAS-positive globules |
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Term
| Secondary Biliary Cirrhosis |
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Definition
| extrahepatic cholelithiasis or malignancies obstruct, may cause ascending cholangitis |
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Term
| Primary Biliary Cirrhosis |
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Definition
| autoimmune disease, nonsuppurative inflammatory destruction of medium-sized intrahepativ bile ducts, antimitochondrial antibodies, hyperpigmentation due to melanin deposition, inflammatory arthropathy, eyelid xanthelasmas |
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Term
| Primary Sclerosing Cholangitis |
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Definition
| inflamation and obliterative fibrosis of bile ducts with dilation of preserved segments. beading of contrast, males, onion-skin fibrosis, solid, cordlike fibrous scar replaces ducts. Elevated alkaline phosphatase, risk of cholangiocarcinoma |
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Term
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Definition
| culsters of modestly dilated bile ducts, bile duct hemartomas |
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Term
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Definition
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Term
| Congenital Hepatic fibrosis |
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Definition
| broad bands of collagenous tissue, embryonic form of the biliary tree, portal hypertension |
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Term
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Definition
| Paucity of bile ducts, arteriohepatic dysplasia, cholestasis, pulmonary artery stenosis, butterfly-like vertebral arch defects, embryotoxon eye defect, peculiar hypertelic facies. Genes: Jagged1, Notch receptors |
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Term
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Definition
| larger ducts of biliary tree are segmentally dilated, associated with polycystic kidney disease |
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