title: Long-term outcome following pediatric liver transplantation for metabolic disorders.
abstract: 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 < 0.01), but mental skills did not; however, both indices were in the low-normal range of development. When compared to patients with biliary atresia, children with metabolic disorders showed significantly lower mental developmental scores at one and two yr post-transplantation (p < 0.05), but psychomotor developmental scores were not significantly different. We conclude that, in patients with metabolic disorders meeting indications for transplantation, liver transplantation or combined liver-kidney transplantation (for those with accompanying renal failure) is associated with excellent long-term survival, improved growth, and improved psychomotor development.
authors: Department of Surgery, Stanford University, Stanford, CA, USA.
authors: Terrell
authors: T
authors: Stevenson
authors: Maria T
authors: MT
authors: Millan
authors: Karen
authors: K
authors: Wayman
authors: William E
authors: WE
authors: Berquist
authors: Minnie
authors: M
authors: Sarwal
authors: Emily E
authors: EE
authors: Johnston
authors: Carlos O
authors: CO
authors: Esquivel
authors: Gregory M
authors: GM
authors: Enns
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016030:Kidney Transplantation; D016031:Liver Transplantation; D008297:Male; D008659:Metabolic Diseases / Q000150:complications / Q000601:surgery*; D012189:Retrospective Studies; D013997:Time Factors