Outcomes of adult native liver survivors with biliary atresia: the current situation in Japan

Purpose: This study aimed to elucidate the difficulties faced by adult native liver survivors with biliary atresia (BA) in Japan. Methods: A single-center, retrospective, observational study of 57 adult patients with BA was conducted. The clinical course of BA was compared between native liver survivors and non-survivors who reached adulthood. Indications and outcomes of liver transplantation (LT) among non-survivors were assessed. Results: A significantly larger portion of non-survivors (n=10) met the criteria for LT (p <0.001) and received treatment for portal hypertension after reaching 20 years of age (p <0.01) compared with the survivors. Causes of death included liver cirrhosis (n=8), graft failure of living donor liver transplantation (LDLT) (n=1), and hepatocarcinoma (n=1). Two of the non-survivors who died of liver cirrhosis had no indication for LT because of alcohol dependence and uncontrolled infection. An appropriate donor candidate could not be found for the five patients who opted for LDLT. All six patients waitlisted for deceased donor liver transplantation (DDLT) died after a median waiting period of 17 months. Conclusion: Adult BA patients in Japan have limited options for LT, mainly owing to low donor candidate availability for LDLT and a low prevalence of DDLT.