The Liver Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, UK.
Eur J Pediatr. 2010 Apr;169(4):395-402. doi: 10.1007/s00431-009-1125-7. Epub 2009 Dec 18.
Biliary atresia is a rare, serious and challenging disease in newborn children. Its aetiology remains unknown. Optimal management at specialist centres with resultant better overall outcomes is achieved through a multidisciplinary team approach. The Kasai portoenterostomy performed early in life remains the only surgical repair procedure. Two thirds of patients will clear their jaundice after a Kasai procedure, but only about one third will retain their livers after the first decade of life. Failure of this procedure leaves liver transplantation as the only chance for survival, and this disease is the commonest indication for liver transplantation in children. With modern medical care and refinements in surgical techniques, survival after either or both of these procedures is about 90%. Early referral to specialist centres and long-term specialist care remains the key to successful treatment of this condition.
先天性胆道闭锁是一种罕见的、严重且具有挑战性的新生儿疾病。其病因尚不清楚。在专科中心通过多学科团队的方法进行最佳管理，可实现更好的整体治疗效果。在生命早期进行的 Kasai 门腔分流术仍然是唯一的手术修复方法。三分之二的患者在 Kasai 手术后黄疸会消退，但只有约三分之一的患者在生命的第一个十年后仍能保留肝脏。如果该手术失败，肝移植是唯一的生存机会，这种疾病是儿童肝移植最常见的适应证。通过现代医疗护理和手术技术的改进，这两种手术的存活率约为 90%。早期向专科中心转诊和长期专科护理仍然是成功治疗这种疾病的关键。
