[Skip to Content]
[Skip to Content Landing]
Article
March 1993

Liver Transplantation for Alagille's Syndrome

Author Affiliations

From the Department of Surgery, University Health Center of Pittsburgh (Pa), University of Pittsburgh (Drs Tzakis, Reyes, Tepetes, Tzoracoleftherakis, Todo, and Starzl), and the Veterans Affairs Medical Center, Pittsburgh (Dr Starzl).

Arch Surg. 1993;128(3):337-339. doi:10.1001/archsurg.1993.01420150093017
Abstract

• 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.

(Arch Surg. 1993;128:337-339)

×