Vimalesvaran Sunitha, Jagadisan Barath, Dhawan Anil
Department of Paediatric Gastroenterology, Hepatology and Nutrition, King's College Hospital, London, UK.
Pediatr Res. 2025 Jul 22. doi: 10.1038/s41390-025-04034-9.
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.
胆道闭锁（BA）是一种进行性胆管病，是儿童肝移植的主要原因。虽然其病因尚不清楚，但发育异常、病毒感染和免疫失调等因素已被牵连其中。早期的Kasai肝门空肠吻合术（KPE）是恢复胆汁流动的标准外科干预措施，血清胆红素正常化是关键的成功指标。即使在成功进行KPE后，进行性纤维化仍然是一个主要挑战。最近的研究集中在确定BA预后的生物标志物，从胆管炎和门静脉高压的指标到黄疸清除和自体肝存活的预测指标。Taylor等人的研究探讨了预测BA患者KPE后胆汁引流的早期免疫特征。他们的研究结果显示，单核细胞样巨噬细胞（MLM）增加和粒细胞-巨噬细胞集落刺激因子（GM-CSF）水平升高与KPE后胆汁流动改善相关。作者认为，GM-CSF驱动的巨噬细胞极化可能增强抗炎反应，可能影响纤维化进展和长期肝功能。虽然这些发现为BA中免疫介导的机制提供了有价值的见解，但我们表明BA中生物标志物的临床应用仍存在模糊性。仍需要进行研究来验证这些预后生物标志物并改善这些患者的风险分层。
