7 years' experience with hepatic transplantation in children

Between the 7th of June 1985 and december 1991, 71 liver transplants (LTx) were performed in 60 patients (pts) with ages ranging from 8 months to 14 years and weight between 4 and 38 kg. Sixty-two percent were cholestatic diseases (32 biliary atresia, 3 sclerosing cholangitis and 2 biliary paucity), 25% metabolic hepatic-based diseases (6 glycogen storage disease, 4 Byler disease, 3 tyrosinaemia and 2 alpha-1-antitrypsin deficiency) and 13% miscellaneous diagnosis (2 post-hepatitic cirrhosis, 2 autoimmune hepatitis, 2 fulminant hepatitis and one case of cholesteryl ester storage disease and one case of Budd-Chiari disease). Primary non-function was observed in 4 cases (5.5), severe bacterial infection in 4 pts. (5.5%), severe viral infection in 2 (3%), arterial thrombosis in 2 (3%), arterial rupture in 1 (1.5%), recurrence in 3 cases (5%) and chronic rejection in two (3%). Two patients were in III and IV coma respectively prior to surgery. Intraoperative mortality was nil. Total mortality was 19 cases, which represents an actuarial survival rate of 70%. Our philosophy has always been to offer this treatment to all patients candidates for LTx. In some end-stage situation it is very difficult to objectively assess their possibilities and when necessary refuse the only opportunity the patient has. Nevertheless, results are satisfactory. In conclusion: 1. LTx in children has been confirmed in our programme over 7 years as efficient treatment for end-stage liver diseases. 2. Advances in the therapeutic arsenal and increased experience have notably improved the progress of these patients.(ABSTRACT TRUNCATED AT 250 WORDS)