Advances in prognostic biomarkers for biliary atresia: Current insights and future directions.

Abstract

Biliary atresia (BA) is a progressive, fibrosing cholangiopathy of infancy characterized by inflammatory obstruction of the bile ducts, ultimately leading to end-stage liver disease if untreated. Early diagnosis and timely surgical intervention via hepatoportoenterostomy (HPE) are critical for improving outcomes; however, prognostication remains challenging due to heterogeneous responses to surgery and variable clinical trajectories. This review provides a comprehensive synthesis of current research on prognostic biomarkers in BA, encompassing clinical indicators, routine laboratory parameters, novel serum biomarkers, histopathologic features, hepatic gene expression profiles, imaging modalities, and both in vitro and computational prognostic modeling systems. While traditional clinical factors, such as age at HPE and postoperative serum bilirubin levels, continue to serve as important predictors of outcome, they lack sufficient discriminatory power for individualized risk stratification. Recent advances have identified emerging biomarkers, including inflammatory cytokines, immune activation markers, and indicators of fibrosis and extracellular matrix remodeling, which show potential in correlating with disease progression and native liver survival. Imaging modalities such as ultrasound elastography have also demonstrated promise in noninvasively assessing liver stiffness and predicting clinical outcomes. Furthermore, the identification of hepatic gene expression signatures and multigene prognostic classifiers offers new avenues for precision risk assessment. However, most of these advancements have not translated into clinical practice due to small sample sizes and limited external validation. Future research efforts must focus on large-scale, multicenter studies to validate findings and establish robust, integrative prognostic models that can inform clinical decision-making and facilitate personalized therapeutic strategies in BA.