Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Stenbackinkatu 11 PO Box 281, 00029, HUS, Finland.
Department of Pediatric Gastroenterology, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki, Stenbäckinkatu 9/PO BOX 347, 00029, HUS, Finland.
Best Pract Res Clin Gastroenterol. 2022 Feb-Mar;56-57:101764. doi: 10.1016/j.bpg.2021.101764. Epub 2021 Sep 4.
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.
经皮肝门空肠吻合术（PE）作为治疗胆道闭锁（BA）的一线手术已有 50 多年的历史。目前，接近一半的 BA 患者在其自身肝脏的支持下能存活超过 10 年，其中大多数在没有进行肝移植（LT）的情况下进入成年期。尽管通过 PE 可使血清胆红素恢复正常，但胆管反应和门脉纤维化仍存在于自身肝脏中。慢性胆管病进展为肝硬化、门静脉高压症并发症、复发性胆管炎或肝胆肿瘤，这些都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健康受损、神经运动发育和生活质量下降。只有少数高质量的试验可提供 PE 术后辅助药物治疗或疾病并发症管理的循证指导。更好地了解将肝损伤与临床结果联系起来的病理生理机制，对于开发准确的随访工具和旨在改善自身肝功能和生存的新疗法至关重要。
