Liver transplantation for Alagille's syndrome
A. G. Tzakis, J. Reyes, K. Tepetes, V. Tzoracoleftherakis, S. Todo and T. E. Starzl
Department of Surgery, University Health Center of Pittsburgh, University of Pittsburgh, PA.
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.
