Pediatric Liver Transplantation Under Therapy With Cyclosporin-A and Steroids.

D URING the period 5/9/81 to 5/9/82. 27 consecutive liver transplantations were performed on 23 pediatric patients. Ages ranged from 7.5 months to 18 years. There were II males and 12 females. Four patients were adolescents (12-18 years). 6 patients were school age (5-10 years). and 13 were infants to preschools (7.5 months-4.5 years). Follow-up time ranged from 3.5 months to 15.5 months. Survival stands at 70% (16/23). while 61 % (14/23) can be described as in excellent condition. Orthotopic liver transplantation (OL T) has. since its inception, I been a procedure with high mortality and morbidity. Steady improvement in survival statistics have. however. occurred during the 20-year history of the procedure. Improvements in surgical technique. patient selection. and immunosuppres-sion have moved OL T into the realm of practical therapeutics. Pediatric OL T. while always enjoying slightly better survival statistics than its adult counterpart. 2 has had added morbidity without correlate in the nonpedialric population. The profound effect of high doses of steroids on the child, which leads not only to growth failure but also to Cushingoid changes. has prompted ~ome to question the use of trans-plantation in children 3 at all. We report improved survival statistics for pediatric OL T under cyclosporin-A (CyA) and low-dose steroids. as well as preliminary data suggesting normal growth velocity post-transplantation in these patients. All the patients listed in Table I represent I-year experience with liver transplantation dating from 5/9/81 to 5/9/82. Nineteen patients received I graft and while 3 patients had 2 grafts, and I patient had a total of 3 grafts. Biliary atresia with a failed allempt at restoration of bile drainage by portoenterostomy represented the most frequent indication for liver transplantation in children. Following biliary atresia. are a variety of inborn errors of metabolism (alpha-I-antitrypsin [A,A T] deficiency being by far the most frequent of the inborn errors), biliary hypoplasia, chronic active hepatitis. neonatal hepatitis. secondary biliary cirrhosis. Jnd familial intrahepatic cho-lestasis. Patients who were transplanted were initially placed on a transplant candidacy list follOWing an in-hospital medical evaluation. The evaluation was undertaken to (I) verify acceptable pre transplantation anatomy (generally requiring demonstration of a patent extrahepatic portal vein), (2) review the patient's diagnosis (accomplished by a review of medical records, biopsy slides. and occasionally other further testing), and (3) an attempt at gaugeing the medical urgency of transplantation. Transplantation recipients were chosen from the candidacy list based on the size …