Article Text

Download PDFPDF

Severe familial intrahepatic cholestasis
  1. M. Odièvre,
  2. M. Gautier,
  3. M. Hadchouel,
  4. D. Alagille


    Five cases of intrahepatic cholestasis are reported in 4 families in which 7 other children have had the same disease. Cholestasis, beginning before the age of one year, is notable for the absence of lipid retention, evolving by attacks brought on by intercurrent infections with gradual development of fibrosis and fatal cirrhosis before the age of 15. Extrahepatic and interlobular bile ducts are undamaged.

    The disease is sometimes accompanied by biliary and pancreatic abnormalities, perhaps due to an abnormality in the biliary acid metabolism.

    Statistics from

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.