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

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.