title: Liver transplantation in children with metabolic diseases: the studies of pediatric liver transplantation experience.
abstract: 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.
authors.affiliation: Department of Pediatrics, Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY 10029, USA.
authors.forename: Ronen
authors.initials: R
authors.lastname: Arnon
authors.forename: Nanda
authors.initials: N
authors.lastname: Kerkar
authors.forename: Michael K
authors.initials: MK
authors.lastname: Davis
authors.forename: Ravinder
authors.initials: R
authors.lastname: Anand
authors.forename: Wanrong
authors.initials: W
authors.lastname: Yin
authors.forename: Regino P
authors.initials: RP
authors.lastname: González-Peralta
mesh_terms: D000293:Adolescent; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D006085:Graft Survival; D006499:Hepatic Artery; D006801:Humans; D053208:Kaplan-Meier Estimate; D007674:Kidney Diseases / Q000453:epidemiology; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008659:Metabolic Diseases / Q000401:mortality / Q000601:surgery*; D015999:Multivariate Analysis; D011169:Portal Vein; D011183:Postoperative Complications / Q000453:epidemiology; D012042:Registries; D006435:Renal Dialysis; D012086:Reoperation / Q000706:statistics & numerical data; D012189:Retrospective Studies; D012307:Risk Factors; D013927:Thrombosis / Q000453:epidemiology; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D020246:Venous Thrombosis / Q000453:epidemiology