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Distinguishing features of the major intrahepatic bile duct disorders
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Distinguishing features of the major intrahepatic bile duct disorders

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Distinguishing features of the major intrahepatic bile duct disorders

Distinguishing features of the major intrahepatic bile duct disorders

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  • 1. Robbins Cotran_Pathology_8ed Yapa Wijeratne M/07/189 Distinguishing Features of the Major Intrahepatic Bile Duct Disorders Secondary Biliary Cirrhosis Primary Billiary Cirrhosis Primary Sclerosing Cholangitis Etiology Extrahepatic bile duct obstruction: biliary atresia, gallstones, stricture, carcinoma of pancreatic head Possibly autoimmune Unknown , possibly autoimmune; 50% to 70% associated with IBD Sex predilection None Female to male, 6 : 1 Female to male, 1 : 2 Symptoms and signs Pruritus, jaundice, malaise, dark urine, light stools, hepatosplenomegaly Onset insidious Laboratory findings Conjugated hyperbilirubinemia, increased serum alkaline phosphatase, bile acids, cholesterol ↑ serum IgM autoantibodies (especially M2 form of anti-mitochondrial antibody) ↑ serum IgM, hypergammaglobulinemia Important pathologic findings before cirrhosis develops 1. Prominent bile stasis in bile ducts, 2. bile ductular proliferation with surrounding neutrophils, 3. portal tract edema Dense lymphocytic infiltrate in portal tracts with granulomatous destruction of bile ducts 1. Periductal portal tracts fibrosis, 2. segmental stenosis of extrahepatic and 3. intrahepatic bile ducts