Liver transplantation in children with metabolic diseases: the studies of pediatric liver transplantation experience.

Publication Type
Journal Article
Year of Publication
Arnon, Ronen; Kerkar, Nanda; Davis, Michael K; Anand, Ravinder; Yin, Wanrong; González-Peralta, Regino P; SPLIT Research Group
Pediatr Transplant
Date Published
2010 Sep 01
Adolescent; Cause of Death; Child; Child, Preschool; Female; Graft Survival; Hepatic Artery; Humans; Kaplan-Meier Estimate; Kidney Diseases; liver transplantation; Male; Metabolic Diseases; Multivariate Analysis; Portal Vein; Postoperative Complications; Registries; Renal Dialysis; Reoperation; Retrospective Studies; Risk Factors; Thrombosis; Transplantation, Homologous; Treatment Outcome; Venous Thrombosis

Metabolic diseases are the second largest indication for LT in children after BA. There are limited data on the long-term post-transplant outcome in this unique group of patients. Therefore, our aim was to assess post-liver transplant outcomes and to evaluate risk factors for mortality and graft loss in children with metabolic disorders in comparison to those with non-metabolic diagnoses. We reviewed all patients enrolled in the SPLIT registry. Between 1995 and 2008, 446 of 2997 (14.9%) children enrolled in SPLIT underwent liver transplant for metabolic diseases. One-yr and five-yr patient survival for children with metabolic diseases was 94.6% and 88.9% and for those with other diseases 90.7% and 86.1% (log-rank p = 0.05), respectively. One-yr and five-yr graft survival for children with metabolic disorders was 90.8% and 83.8%, and for those with other diseases 85.4% and 78.0% (log-rank p = 0.005), respectively. Children with metabolic diseases were less likely to experience gastrointestinal complications (5.6% vs. 10.7%, p = 0.001), portal vein thrombosis (2.9% vs. 5.2%, p = 0.04), and reoperations within 30 days post-transplant (33.4% vs. 37.8%, p = 0.05) than those with other indications. In conclusion, children who underwent liver transplant for metabolic disease had similarly excellent patient survival as, and better graft survival than, those who received a liver allograft for other indications.