Davenport Mark
Department of Paediatric Surgery, Kings College Hospital, London, UK.
Semin Pediatr Surg. 2005 Feb;14(1):42-8. doi: 10.1053/j.sempedsurg.2004.10.024.
Biliary atresia (BA) remains a devastating disease of infants. It is still a disease of largely unknown etiology although many hypotheses such as an aberrant early bile duct development, perinatal viral infection, aberrant immune response, and abnormalities of bile acids have all been suggested as possibly etiologically important. Although recent studies, using the techniques of molecular biology and immunohistochemistry, have improved the understanding of some of the inflammatory elements of BA, there is a lack of understanding of how many such disparate elements interact and relate. Clinically, the management in the majority of cases should consist of a primary portoenterostomy (Kasai procedure) to try and restore bile flow and alleviate jaundice. Transplantation should be reserved for those who develop chronic liver disease and its attendant complications. Recent series would suggest that over 50% of infants in large centers will be able to clear their jaundice and therefore have a reasonable expectation of long-term survival with a good quality-of-life.
胆道闭锁（BA）仍是一种严重威胁婴儿的疾病。尽管有许多假说，如早期胆管发育异常、围产期病毒感染、异常免疫反应和胆汁酸异常等都被认为可能在病因学上具有重要意义，但它仍然是一种病因很大程度上不明的疾病。尽管最近利用分子生物学和免疫组织化学技术的研究增进了对BA某些炎症因素的理解，但对于众多如此不同的因素如何相互作用和关联仍缺乏了解。临床上，大多数病例的治疗应包括初次肝门空肠吻合术（葛西手术），以尝试恢复胆汁流动并减轻黄疸。肝移植应保留给那些发展为慢性肝病及其相关并发症的患者。最近的系列研究表明，大型中心超过50%的婴儿能够消退黄疸，因此对长期生存且生活质量良好有合理的期望。
