LAUSR

BACKGROUND Liver transplantation (LT) is an excellent treatment option for patients with biliary atresia (BA) who fail portoenterostomy surgery. LT is also increasingly performed in patients with metabolic liver diseases. This study compared the outcomes in pediatric patients who underwent LT for metabolic liver diseases and BA. BASIC PROCEDURES Data from 237 pediatric patients who underwent primary LT at Seoul National University Hospital from 1988 to 2015, including 33 with metabolic liver diseases and 135 with BA, were retrospectively analyzed. MAIN FINDINGS Compared with children with BA, children with metabolic liver diseases were significantly older at the time of LT (121.3 vs. 37.3 months; P < 0.001), and had lower Child-Pugh (7.1 vs. 8.4; P = 0.010) and Pediatric End-stage Liver Disease (6.5 vs. 12.8; P = 0.042) scores. Overall survival rates were similar (87.8% vs. 90.8%; P = 0.402), but hepatic artery (HA) complications were significantly more frequent in children with metabolic liver diseases (12.1% vs. 1.5%; P = 0.014). PRINCIPAL CONCLUSION Despite similar overall survival, children with metabolic liver diseases had a higher rate of HA complications. TYPE OF SUBMISSION Original article, Case control study, Retrospective. EVIDENCE LEVEL III.