PELD and BALF Scores Demonstrate Similar Reliability for Predicting Acute on Chronic Liver Failure and Death in Biliary Atresia Patients: ACG Fellow Award Presidential Poster

Abstract

Introduction: Biliary atresia (BA) is a destructive fibrosing obliterative cholangiopathy and is the most common indication for liver transplant (LT) in young children. The greatest risk of death while waiting for LT is the development of acute-on-chronic liver failure (ACLF). The pediatric end-stage liver disease (PELD) scoring system, which takes into account age, total bilirubin, albumin, INR, and nutritional status, is used to prioritize BA patients listed for LT. Recently the bilary atresia liver fibrosis (BALF) score was introduced. Taking into account serum total bilirubin, γ-glutamyltransferase, serum albumin, and age, this novel system has been validated for estimating the extent of hepatic fibrosis in BA. Because the extent of liver fibrosis correlates with hepatic reserve, the BALF score may have clinical utility. Our purpose was to compare the predictive reliability of the BALF and PELD scoring systems for the development of ACLF in BA patients listed for LT. Methods: Medical records of 89 patients with BA listed for LT at Texas Children’s Hospital between 2001 and 2013 were reviewed. The ACLF group was comprised of patients who met UNOS criteria for status IB listing. Listing PELD and BALF scores of BA patients who developed ACLF and those who did not develop ACLF were compared. All statistical analyses were performed using R (version 2.15.2, 2012). Statistical significance was determined at P≤0.05. Results: Eighty-nine BA patients were listed for LT between 2001 and 2013. Nineteen developed ACLF, of whom 10 died. Univariate logistic regression analysis demonstrated a significant positive correlation between PELD and BALF scores at listing and the later development of ACLF (Wald p<0.01). ROC plots demonstrated similar diagnostic accuracy for BALF and PELD. Conclusion: Both PELD and BALF scores at the time of listing for LT demonstrated a comparable diagnostic accuracy for predicting ACLF. Although inherently limited by sample size and a retrospective approach, this study supports the continued use of PELD as a primary method of assessing clinical risk in BA patients listed for LT. However, the BALF scoring system appears to demonstrate a similar degree of reliability for predicting ACLF. Accordingly, further study of the BALF scoring system is warranted.Table 1: Predictive Characteristics*of Listing Adjusted BALF**and PELD Scores for the Later Development of ACLF