Japanese Biliary Atresia Registry

Abstract

The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS). JBAR employed an initial questionnaire, a questionnaire for liver transplantation, and a follow-up questionnaire. The questionnaire has been sent to the pediatric surgeons of JBAS member institutions and the hospitals in which council members of the Japanese Society of Pediatric Surgeons are working. One hundred and twenty-three hospitals have been included in JBAR between 1989 and 2015. Each patient is to be followed up for 30 years. Between 1989 and 2015, 3160 patients have been registered in JBAR. Among them, 1236 patients underwent liver transplantation. Follow-up questionnaire is also going on. 20-year overall and native-liver survival rates were 89 and 49%, respectively. The surgical outcome of BA has markedly improved owing to the co-operation of Kasai portoenterostomy and liver transplantation. Japanese big database of BA is now available.