Diagnostic approach to patients with cholestatic jaundice.

Abstract

Conjugated hyperbilirubinemia due to any form of hepatobiliary disease is essentially the result of impairment in bile formation and/or bile flow, a condi tion known as cholestasis. Cholestatic jaundice is of ten accompanied by a broad spectrum of laboratory, clinical, and histological abnormalities. Laboratory abnormalities include increased serum levels of alkaline phosphatase and gamma-glutamyltransferase (GGT), and variable elevation of bilirubin, serum copper, ceruloplasmin, cholesterol, lipoprotein X, and serum bile acids, as well as of prothrombin time, which is corrected by vitamin K supplementation. T h e r e i s m i n i m a l o r n o e l e v a t i o n o f aminotransferases. Clinically, pruritus, fatigue, xanthomas, back pain from osteoporosis, pale stools, or even steatorrhea may be present, with evidence of fat-soluble vitamin deficiency. Histologically, conjugated hyperbilirubinemia is characterized by bile plugs (bilirubinostasis), feathery degeneration of hepatocytes (cholate stasis) , small-bile-duct destruction, perich olangitis, portal edema, bile lakes and infarcts (typically with extrahepatic obstruction), and finally, biliary cirrhosis.