The clinical features and outcomes of acute-on-chronic liver failure in biliary atresia patients on a waiting list for liver transplantation.

Abstract

Purpose: Acute-on-chronic liver failure (ACLF) in adults has an extremely poor prognosis. However, reports on the epidemiology and outcomes of ACLF in biliary atresia (ACLF-BA) are scarce.

Methods: Among 123 BA cases deemed eligible for liver transplantation (LT), we retrospectively evaluated them according to the Japanese ACLF diagnostic criteria established in 2018 and categorized them into ACLF-BA and non-ACLF-BA groups. We also retrospectively reviewed the liver and extrahepatic organ failures during the waitlist period in both groups.

Results: Fourteen patients (11.4%) were categorized as ACLF-BA. The liver function in ACLF-BA dramatically worsened both in the Child-Pugh and MELD scores in the short term after ACLF onset. Seven cases (50%) in ACLF-BA were categorized as grade 3 with more than three extrahepatic organ failures. The waitlist mortality rate for deceased donors in ACLF-BA was as high as 50%. However, the post-LT patient survival rate in ACLF-BA was 90.9%, and not statistically significantly different compared to non-ACLF-BA.

Conclusion: Although ACLF-BA awaiting LT carries an increased waitlist mortality, the post-LT patient survival was comparable to recipients without ACLF. The type and number of extrahepatic organ failures could correlate with patient mortality. The prioritization of organ allocation for ACLF-BA warrants further discussion.