Correction of Phenylketonuria after Liver Transplantation in a Child with Cirrhosis

To the Editor: Liver transplantation has been performed in a few children with inborn errors of metabolism that do not cause liver failure but may be responsible for severe disease of extrahepatic organs (e.g., Crigler-Najjar syndrome type I). Liver transplantation has restored the correct metabolic pathway in such patients¹. We report on the correction of the metabolic abnormalities of phenylketonuria in a 10-year-old boy from Italy. The child required orthotopic liver transplantation because of concomitant, unrelated end-stage chronic liver disease. Phenylketonuria had been diagnosed in the neonatal period; it was immediately treated by restriction of dietary phenylalanine. Satisfactory control…