Multicenter Study on Early Predictors of Biliary Atresia Outcomes

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OBJECTIVES: To compare liver biochemistry, aspartate aminotransferase-to-platelet ratio index (APRI) and patient characteristics for prediction of biliary atresia (BA) outcomes at time of portoenterostomy. METHODS: This was a retrospective observational study. Seven tertiary centers participated. Predictors of clearance of jaundice (COJ, bilirubin <20 μmol/L 6 months after portoenterostomy) and native liver survival (NLS, recorded at transplantation, death, or latest follow-up with NL) were analyzed with logistic regression and Cox regression. Cytomegalovirus (CMV) DNA detection in serum/urine or IgM antibodies were defined as CMV-associated BA. RESULTS: Out of 630 patients, 58 % achieved COJ. CMV-positive patients were older at portoenterostomy (74 vs 52 days, p < 0.001) whereas BA splenic malformation (BASM) patients were younger (median 47 vs 56 days, p = 0.001) and had equal COJ rates (56 %) compared to others. Absence of cirrhosis (p = 0.001) and CMV infection (p = 0.001), age <60 days at portoenterostomy (p < 0.001), and lower AST (p = 0.007-0.037) and APRI (p = 0.016) predicted COJ. While these variables also predicted NLS, in multivariable regression only COJ (hazard ratio, HR 15, p < 0.001), BASM (HR 1.9, p < 0.001), and portoenterostomy age >60 days (HR 1.3, p = 0.044) remained significant predictors of transplantation/death. Among COJ patients, only BASM predicted transplantation/death (HR 2.4, p = 0.002). CONCLUSIONS: APRI is not superior to AST in the prediction of BA outcomes. However, in COJ patients, both are unrelated to NLS. Despite younger age at portoenterostomy and similar COJ rates compared to isolated BA, BASM strongly predicts shorter NLS. COJ is possible in CMV positive patients who, after achieving COJ, have similar NLS compared to other patients.

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