Liver transplantation in children

Abstract

We report on a 12 year-old female with criggler-najjar syndrome type I. She showed signs of kernicterus like relative decrease in IQ and speech disturbance. Total bilirubin was 29 and direct bilirubin 1 mg/dl. The first child of the parents, a male newborn, died because of high hyperbilirubinemia. One 10 year-old son is normal, and a 3 year-old daughter is also congenitally icteric. The liver of a 16-year old boy who died in automobile accident was transplanted to her soon after his brain death. The immune system was depressed by methylprednisolone, I.V cyclosporine, and cell cept. In an 18-months follow-up, she had normal liver and renal function as well as normal CBC. 14 months after transplantation, she had her first menstruation.