BACKGROUND:Biliary atresia(BA) is a major cause of chronic cholestasis,a fatal disorder in infants.This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation(LDLT) in comparison with the traditional first-line treatment,the Kasai procedure.METHODS:We assessed 28 children with BA at age of less than two years(3-21.3 months) who had undergone LDLT in two hospitals in Southwest China during the period of 2008-2011.Eighteen children who had had primary LDLT were included in a primary LDLT group,and ten children who had undergone the Kasai operation in a pre-Kasai group.All patients were followed up after discharge from the hospital.The records of the BA patients and donors were reviewed.RESULTS:The time of follow-up ranged 12-44.5 months with a median of 31 months.The 30-day and 1-year survival rates were 85.7% and 78.6%,respectively.There was no significant difference in the 30-day or 1-year survival between the two groups(83.3% vs 90% and 77.8% vs 80%,P>0.05).The main cause of death was hepatic artery thrombosis.There were more patients with complications who required intensive medical care or re-operation in the pre-Kasai group(8,80%) than in the primary LDLT group(9,50%)(P=0.226).But no significant differences were observed in operating time(9.3 vs 8.9 hours,P=0.77),intraoperative blood loss(208.6 vs 197.0 mL,P=0.84) and blood transfusion(105.6 vs 100.0 mL,P=0.91) between the two groups.The durations of ICU and hospital stay in the primary LDLT group and pre-Kasai group were 180.4 vs 157.7 hours(P=0.18) and 27 vs 29 days(P=0.29),respectively.CONCLUSIONS:Primary LDLT is a safe and efficient management for young pediatric patients with BA.Compared with the outcome of LDLT for patients receiving a previous Kasai operation,a similar survival rate and a low rate of re-operation and intensive medical care for patients with BA can be obtained. BACKGROUND: Biliary atresia (BA) is a major cause of chronic cholestasis, a fatal disorder in infants. This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation (LDLT) in comparison with the traditional first-line treatment, the Kasai procedure. METHODS: We assessed 28 children with BA at age of less than two years (3-21.3 months) who had undergone LDLT in two hospitals in Southwest China during the period of 2008-2011. Eighteen children who had had primary LDLT were included in a primary LDLT group, and ten children who had undergone the Kasai operation in a pre-Kasai group. All patients were followed up after discharge from the hospital. Records of the BA patients and donors were reported .RESULTS: The time of follow-up ranged 12-44.5 months with a median of 31 months. The 30-day and 1-year survival rates were 85.7% and 78.6% respectively. There was no significant difference in the 30-day or 1-year survival between the two groups (83.3% vs 90% and 77.8% vs 80%, P> 0.05) .The main cause of death was hepatic artery thrombosis.There were more patients with complications who required intensive medical care or re-operation in the pre-Kasai group (8,80%) than in the primary LDLT group (P = 0.226). No significant differences were observed in operating time (9.3 vs 8.9 hours, P = 0.77), intraoperative blood loss (208.6 vs 197.0 mL, P = 0.84) and blood transfusion (105.6 vs 100.0 mL, P = 0.91) between the two groups. Duration of ICU and hospital stay in the primary LDLT group and pre-Kasai group were 180.4 vs 157.7 hours (P = 0.18) and 27 vs 29 days (P = 0.29), respectively. CONCLUSIONS: Primary LDLT is a safe and efficient management for young pediatric patients with BA. Compared with the outcome of LDLT for patients receiving a previous Kasai operation, a similar survival rate and a low rate of re- operation and intensive medical care for patients with BA can be obtained.