Hepatoma in familial cholestatic cirrhosis of childhood: its occurrence in twin brothers.
Obstructive jaundice, pruritus, and malabsorption developed in twin brothers in infancy. Early liver biopsy specimens showed intracellular and canalicular cholestasis with normal bile ducts. By the age of 3 years, both had cirrhosis and portal hypertension. Each died during the teen years from hepatocellular carcinoma. These brothers represent the tenth reported family with familial cholestatic cirrhosis, and they are the first patients with this syndrome in whom hepatoma developed.
D000293:Adolescent; D006528:Carcinoma, Hepatocellular / Q000235:genetics* / Q000473:pathology; D002779:Cholestasis / Q000235:genetics* / Q000473:pathology; D004200:Diseases in Twins; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000235:genetics* / Q000473:pathology; D008113:Liver Neoplasms / Q000235:genetics* / Q000473:pathology; D008297:Male