Liver transplantation for young adult patients with post-Kasai portoenterostomy biliary atresia: Analysis of Korean Organ Transplantation Registry database

Abstract

Background: Some young adults with native liver after Kasai portoenterostomy (KPE) for biliary atresia (BA) have a long survival period. However, a considerable number of them finally require liver transplantation (LT) due to various BA-associated complications. This study aimed to analyze the outcomes of LT in adult patients with post-KPE BA using Korea Organ Transplantation Registry (KOTRY) database. Methods: This study was a retrospective analysis of multi-center data using KOTRY database, including 14 adult patients who underwent LT in six institutions during a 7-year period from January 2014 to December 2020. Results: All 14 patients underwent KPE at around 1 year of age. The mean ages at LT were 25.0±5.9 years. The mean model for end-stage liver disease score was 13.5±4.6. The main reasons for LT were advanced liver cirrhosis in nine and intractable cholangitis in five. All patients received living-donor LT. The mean graft-to-recipient body weight ratio was 1.21%±0.31%. All 14 patients are currently alive during the mean follow-up period of 32.3±17.1 months. Adult BA patients were being cared for by pediatric gastroenterologists in 3 institutions, by adult gastroenterologists in 1 institution, and undetermined in 2 institutions. Conclusion: Young adult patients with BA are a unique group of patients requiring specialist care during the transition from pediatric to adult services. Living donor LT has been the main type of LT for these patients because of donor shortage in Korea. Similar to our previous single-center study, in the present study, LT outcomes in young adult BA patients were excellent. Therefore, LT should be considered in patients showing serious BA-associated complications.