Arch Surg 1993 Mar;128(3):337-9

Liver transplantation for Alagille's syndrome

Tzakis AG, Reyes J, Tepetes K, Tzoracoleftherakis V, Todo S, Starzl TE

Department of Surgery, University Health Center of Pittsburgh, University of
Pittsburgh, PA

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