Liver transplantation for Alagille's syndrome

Arch Surg. 1993 Mar;128(3):337-9. doi: 10.1001/archsurg.1993.01420150093017.


Twenty-three children with Alagille's syndrome and end-stage liver disease underwent liver transplantation with cyclosporine and low-dose steroid immunosuppression. Two to 9 years (mean, 4.4 years) after surgery, 13 (57%) of the children were still alive, with normal liver function. Three of the fatalities were due to cardiovascular failure secondary to associated cardiopulmonary disease. Mortality was higher among patients who had more severe cardiac disease and patients who had previously undergone a Kasai procedure. Although it has a higher than average risk, liver transplantation can be efficacious in patients with Alagille's syndrome and end-stage liver disease.

Publication types

  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Alagille Syndrome / pathology
  • Alagille Syndrome / physiopathology
  • Alagille Syndrome / surgery*
  • Child
  • Child, Preschool
  • Cholestasis / pathology
  • Female
  • Follow-Up Studies
  • Heart Diseases / pathology
  • Humans
  • Infant
  • Jaundice / pathology
  • Liver Cirrhosis / pathology
  • Liver Transplantation* / adverse effects
  • Liver Transplantation* / methods
  • Male
  • Prognosis
  • Reoperation
  • Retrospective Studies
  • Survival Rate
  • Thrombosis / etiology