Prognostic biomarkers of biliary atresia-are we there yet?

Abstract

Biliary atresia (BA) is a progressive cholangiopathy and the leading cause of pediatric liver transplantation. While its etiology remains unclear, factors such as developmental anomalies, viral infections, and immune dysregulation have been implicated. Early Kasai portoenterostomy (KPE) is the standard surgical intervention to restore bile flow, with serum bilirubin normalization serving as a key success indicator. Even after successful KPE, progressive fibrosis remains a major challenge. Recent research has focused on identifying biomarkers for BA prognosis, ranging from indicators of cholangitis and portal hypertension to predictors of jaundice clearance and native liver survival. The study by Taylor et al. explores early immune signatures predicting post-KPE biliary drainage in BA. Their findings revealed that increased monocyte-like macrophages (MLM) and elevated granulocyte-macrophage colony-stimulating factor (GM-CSF) levels correlated with improved bile flow post-KPE. The authors suggest that GM-CSF-driven macrophage polarization may enhance an anti-inflammatory response, potentially influencing fibrosis progression and long-term liver function. While these findings provide valuable insight into immune-mediated mechanisms in BA, we show there remains ambiguity around the clinical utility of biomarkers in BA. Studies are still needed to validate these prognostic biomarkers and improve risk stratification in these patients.