Japanese Biliary Atresia Registry.

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Clinical Data Management

Background:

• Biliary atresia (BA) is a severe neonatal liver disease requiring timely surgical intervention.
• The Japanese Biliary Atresia Registry (JBAR) was established to centralize and analyze BA patient data.
• Long-term outcomes and treatment efficacy require comprehensive, multi-institutional data collection.

Purpose of the Study:

• To establish and maintain a comprehensive database of biliary atresia patients in Japan.
• To track the long-term outcomes of patients with biliary atresia, including survival rates.
• To evaluate the impact of surgical interventions, such as Kasai portoenterostomy and liver transplantation, on patient survival.

Main Methods:

• The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS).
• Data collection involved initial, liver transplantation, and follow-up questionnaires sent to pediatric surgeons across 123 participating hospitals.
• A total of 3160 patients were registered between 1989 and 2015, with ongoing follow-up for up to 30 years.

Main Results:

• Between 1989 and 2015, 3160 patients with biliary atresia were registered in JBAR.
• Of these, 1236 patients underwent liver transplantation.
• The 20-year overall and native-liver survival rates were 89% and 49%, respectively.

Conclusions:

• The surgical management of biliary atresia has shown marked improvement.
• The combined approach of Kasai portoenterostomy and liver transplantation has significantly enhanced patient outcomes.
• The JBAR provides a valuable, large-scale Japanese database for ongoing research into biliary atresia.