Tuesday, 19 August 2014

Biliary cirrhosis

1) PRIMARY BILLIARY CIRRHOSIS
• inflammatory autoimmune disease
• characterized by nonsuppurative, inflammatory destruction of medium-sized intrahepatic bile ducts
• mostly middle-aged women ( 40-50 yrs age group)
• gender predilection--- Female to male ;6:1
• symptoms and sign---- Pruritus, jaundice, malaise, dark urine, light stools, hepatosplenomegaly
• Laboratory findings-- Conjugated hyperbilirubinemia, increased serum alkaline phosphatase, bile acids, cholesterol; elevated serum IgM autoantibodies (especially M2 form of anti- mitochondrial antibody target the E2 component of the pyrurate dehydrogenase complex (PDC-E2))
• Histology--- Dense lymphocytic infiltrate in portal tracts with granulomatous destruction of bile ducts.
• Risk—micronodular cirrhosis; hepatocellular carcinoma 
• Most common cause of death --- liver cell failure > variceal bleeding
2)SECONDARY BILIARY CIRRHOSIS
• Etiology--- Extrahepatic bile duct obstruction (most common cause of obstruction in adults is extrahepatic cholelithiasis or gallstones) biliary atresia, gallstones, stricture, carcinoma of pancreatic head
• Sex predilection--- male and female equally affected
• Symptoms and signs--- Pruritus, jaundice, malaise, dark urine, light stools, hepatosplenomegaly
• Laboratory findings-- Conjugated hyperbilirubinemia, increased serum alkaline phosphatase, bile acids, cholesterol
• Histology--- Prominent bile stasis in bile ducts, bile ductular proliferation with surrounding neutrophils, portal tract edema

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