2012-9-27 Disorders and Drugs Inat can cau epatic Fibrosis Hepatic Fibrosis Copper storage diseases (eg, wilson's disease) Self-limited, acute liver injury (eg, acute viral hepatitis A) even architecture and hence does not cause fibrosis, despite o when fulminant, does not necessarily distort the scaffoldin In its initial stages, hepatic fibrosis can regress if the rouisomal disorders (eg, Zellweger syndrome) hronic or repeated injury, fibrosis becomes permanent Pathogenesis of Liver Cirrhosis Histopathologic Classification micronodular uniformly small nodules(< 3 mm in diameter) and regular ands of connective tissu fibrous septa formation macronodular nodules that vary in size(3 mm to 5 cm in diameter) Hepatitis B, C: Hem matosis. Wilsons disease mixed macro and micronodular micronodular and macronodular cirrhosis A1-AT deficiency, Wilson's disease; Hepatitis B rna surface of a normal liver The color is DO to 1600 grams2012-9-27 2  Certain storage diseases and inborn errors of metabolism α 1-Antitrypsin deficiency Copper storage diseases (eg, Wilson's disease) Fructosemia Galactosemia Glycogen storage diseases (especially types III, IV, VI, IX, and X) Iron-overload syndromes (hemochromatosis) Lipid abnormalities (eg, Gaucher's disease) Peroxisomal disorders (eg, Zellweger syndrome) Tyrosinemia  Congenital hepatic fibrosis  Others Cystic fibrosis Graft-versus-host disease Jejunoileal bypass Sarcoidosis Disorders and Drugs That Can Cause Hepatic Fibrosis  Self-limited, acute liver injury (eg, acute viral hepatitis A), even when fulminant, does not necessarily distort the scaffolding architecture and hence does not cause fibrosis, despite loss of hepatocytes.  In its initial stages, hepatic fibrosis can regress if the cause is reversible (e.g., with viral clearance). After months or years of chronic or repeated injury, fibrosis becomes permanent.  Fibrosis develops even more rapidly in mechanical biliary obstruction. Disorders and Drugs That Can Cause Hepatic Fibrosis Etiology Liver function Injury, Portal hypertension Diffuse, chronic liver injury Formation of diffuse fibrous septa regenerative nodules formation Hepato-cellular necrosis, collapse of hepatic lobules Complications Upper GI Bleeding, Hepatic coma, infections, Hepatocellular carcinoma; Functional renal failure Pathogenesis of Liver Cirrhosis FIBROSIS CIRRHOSIS Histopathologic Classification  micronodular uniformly small nodules (< 3 mm in diameter) and regular bands of connective tissue Alcoholic, stasis  macronodular nodules that vary in size (3 mm to 5 cm in diameter) Hepatitis B, C; Hemochromatosis, Wilson’s disease  mixed macro and micronodular (incomplete septal cirrhosis) combines elements of micronodular and macronodular cirrhosis A1-AT deficiency, Wilson’s disease; Hepatitis B