Pediatric liver transplantation for metabolic liver disease: report of 42 cases

Objective To Analyze the clinical outcomes of pediatric liver transplantation (LT) for liver-based metabolic disorders. Methods We conducted a retrospective analysis on 42 pediatric patients with liver-based metabolic disorders from June 2013 to March 2017, and analyzed the pediatric end stage liver disease model (PELD), growth and development, type of transplant, postoperative complications and prognosis of patients. Results There were 42 children with liver-based metabolic disorders (15.56%) out of all the 270 children who underwent LT.The median age was 51.0 months (range, 3.4-160.9 months). Of the 42 children, 19 received living donor liver transplantation (LDLT), 18 cases received deceased donor liver transplantation (DDLT) and 5 cases received domino liver transplantation.1-, 2- and 3-year cumulative survival rate of 42 recipients was 97.7%, 93.6% and 93.6%, and that of the grafts was 95.3%, 91.4% and 91.4%, respectively.As compared with the 194 children with biliary atresia who underwent LT, significant difference was found in PELD and weight Z-score between the two groups. Conclusion Liver transplantation is a valuable option for children with metabolic disorders, and it has gained a better prognosis. Key words: Liver transplantation; Child; Metabolic disease