Portoenterostomy (PE) has remained as the generally accepted first line surgical treatment for biliary atresia (BA) for over 50 years. Currently, close to half of BA patients survive beyond 10 years with their native livers, and most of them reach adulthood without liver transplantation (LT). Despite normalization of serum bilirubin by PE, ductular reaction and portal fibrosis persist in the native liver. The chronic cholangiopathy progresses to cirrhosis, complications of portal hypertension, recurrent cholangitis or hepatobiliary tumors necessitating LT later in life. Other common related health problems include impaired bone health, neuromotor development and quality of life. Only few high-quality trials are available for evidence-based guidance of post-PE adjuvant medical therapy or management of the disease complications. Better understanding of the pathophysiological mechanisms connecting native liver injury to clinical outcomes is critical for development of accurate follow-up tools and novel therapies designed to improve native liver function and survival.

50 多年来，门肠造口术 (PE) 一直是公认的胆道闭锁 (BA) 一线手术治疗方法。目前，接近一半的 BA 患者在其原生肝脏的情况下存活超过 10 年，其中大多数在没有肝移植 (LT) 的情况下达到成年。尽管 PE 使血清胆红素正常化，导管反应和门静脉纤维化仍然存在于天然肝脏中。慢性胆管病进展为肝硬化、门静脉高压并发症、复发性胆管炎或肝胆肿瘤，需要在以后的生活中进行 LT。其他常见的相关健康问题包括骨骼健康受损、神经运动发育和生活质量受损。只有少数高质量的试验可用于 PE 后辅助药物治疗或疾病并发症管理的循证指导。