Gilbe syndrome, true all except

Gilbe's Syndrome This is the commonest of the familial, genetically-determined diseases of the liver affecting 2-5% of the population. Gilbe's syndrome is characterised by mild, benign, unconjugated hyperbilirubinaemia (serum bilirubin 1-5 mg/dl) which is not due to haemolysis. The condition is inherited as an autosomal dominant character. The defect in bilirubin metabolism is complex and appears to be reduced activity of UDP-glucuronosyl transferase with decreased conjugation, or an impaired hepatic uptake of bilirubin. The jaundice is usually mild and intermittent. MORPHOLOGIC FEATURES: There are no morphologic abnormalities in the liver except some increased lipofuscin pigment in centrilobular hepatocytes. The prognosis of patients with Gilbe's syndrome is excellent, though chronic jaundice persists throughout life. TEXTBOOK OF PATHOLOGY 6TH EDITION HARSH MOHAN PAGE NO:600