Long-term outcome following pediatric liver transplantation for metabolic disorders

Stevenson T, Millan MT, Wayman K, Berquist WE, Sarwal M, Johnston EE, Esquivel CO, Enns GM. Long‐term outcome following pediatric liver transplantation for metabolic disorders. Pediatr Transplant 2010:14:268–275. © 2009 John Wiley & Sons, A/S. : In order to determine long‐term outcome, including survival, growth and development, following liver transplantation in children with metabolic disorders, we retrospectively reviewed charts of 54 children with metabolic disorders evaluated from 1989–2005 for presenting symptoms, transplantation timing and indications, survival, metabolic parameters, growth, and development. Thirty‐three patients underwent liver transplantation (12 received combined liver–kidney transplants) at a median age of 21 months. At a median follow‐up of 3.6 yr, patient survival was 100%, and liver and kidney allograft survival was 92%, and 100%, respectively. For the group as a whole, weight Z scores improved and body mass index at follow‐up was in the normal range. Two yr post‐transplantation, psychomotor development improved significantly (p