Pediatric liver transplantation for urea cycle disorders and organic acidemias: United Network for Organ Sharing data for 2002‐2012

摘要: Decision making concerning liver transplantation is unique for children with urea cycle disorders (UCDs) and organic acidemias (OAs) because of their immediate high priority on the waiting list, which not related to severity disease. There are limited national outcome data recommendations about UCDs or OAs can be based. This study was a retrospective analysis United Network Organ Sharing recipients who underwent at an age < 18 years in 2002-2012. Repeat transplants were excluded. Among pediatric transplants, 5.4% liver-only UCDs/OAs. The proportion UCDs/OAs increased from 4.3% 2002-2005 7.4% 2010-2012 (P < 0.001). Ninety-six percent deceased donor 59% these patients <2 age. Graft survival improved as age transplant (P = 0.04). Within 5 after transplantation, graft rate 78% children < 2 old 88% children ≥ 2 (P = 0.06). Vascular thrombosis caused 44% losses, 65% losses occurred old. Patient also increased: 5-year patient 99% ≥2 (P = 0.006). At last-follow-up (54 ± 34.4 months), more likely have cognitive motor delays than other indications. Cognitive associated metabolic disorders, but they predicted by weight transplant, sex, ethnicity, type (split versus whole), hospitalization univariate multivariate analyses. In conclusion, most occur early childhood. Further research benefits needed younger may increase posttransplant morbidity. Liver Transpl 20:89-99, 2014. © 2013 AASLD.