Hartley Jane L, Davenport Mark, Kelly Deirdre A
Liver Unit, Birmingham Children's Hospital NHS Trust, Birmingham, UK.
Lancet. 2009 Nov 14;374(9702):1704-13. doi: 10.1016/S0140-6736(09)60946-6.
Biliary atresia is a rare disease of infancy, which has changed within 30 years from being fatal to being a disorder for which effective palliative surgery or curative liver transplantation, or both, are available. Good outcomes for infants depend on early referral and timely Kasai portoenterostomy, and thus a high index of suspicion is needed for investigation of infants with persistent jaundice. In centres with much experience of treating this disorder, up to 60% of children will achieve biliary drainage after Kasai portoenterostomy and will have serum bilirubin within the normal range within 6 months. 80% of children who attain satisfactory biliary drainage will reach adolescence with a good quality of life without undergoing liver transplantation. Although much is known about management of biliary atresia, many aspects are poorly understood, including its pathogenesis. Several hypotheses exist, implicating genetic predisposition and dysregulation of immunity, but the cause is probably multifactorial, with obliterative extrahepatic cholangiopathy as the common endpoint. Researchers are focused on identification of relevant genetic and immune factors and understanding serum and hepatic factors that drive liver fibrosis after Kasai portoenterostomy. These factors might become therapeutic targets to halt the inevitable development of cirrhosis and need for liver transplantation.
胆道闭锁是一种罕见的婴儿疾病，在30年内已从致命疾病转变为一种可通过有效的姑息性手术或根治性肝移植（或两者皆有）进行治疗的疾病。婴儿的良好预后取决于早期转诊和及时进行肝门空肠吻合术，因此对于持续性黄疸婴儿的检查需要高度怀疑指数。在治疗这种疾病经验丰富的中心，高达60%的儿童在肝门空肠吻合术后可实现胆汁引流，并在6个月内血清胆红素处于正常范围。80%实现满意胆汁引流的儿童在不进行肝移植的情况下可健康成长至青春期。尽管对胆道闭锁的管理已有很多了解，但包括其发病机制在内的许多方面仍知之甚少。存在几种假说，涉及遗传易感性和免疫失调，但病因可能是多因素的，以肝外胆管闭锁为共同终点。研究人员专注于识别相关的遗传和免疫因素，并了解肝门空肠吻合术后驱动肝纤维化的血清和肝脏因素。这些因素可能成为阻止肝硬化不可避免发展和避免肝移植需求的治疗靶点。
