Kelly Deirdre A, Davenport Mark
The Liver Unit, Birmingham Children's Hospital NHS Trust, Birmingham, UK.
Arch Dis Child. 2007 Dec;92(12):1132-5. doi: 10.1136/adc.2006.101451. Epub 2007 Sep 18.
Extra-hepatic biliary atresia occurs in approximately 1:15,000 live births leading to about 50 new cases/year in the UK. Presentation is with prolonged jaundice, usually in a term baby who develops signs of obstructive jaundice. Management has been improved by public and professional education to encourage early referral and diagnosis to facilitate initial surgery before 8 weeks of age. Surgical management is complementary and includes an attempt to restore biliary flow (the Kasai portoenterostomy) and liver transplantation if necessary. Medical management consists of antibiotics, ursodeoxycholic acid to encourage bile flow, fat soluble vitamin supplementation and nutritional support. Centralising surgery to specialised centres has improved survival of this potentially fatal disease to over 90% in the UK. Over half of infants undergoing portoenterostomy will clear the jaundice and have a greater than 80% chance of a good quality of life, reaching adolescence without transplantation. For those children developing intractable complications of cirrhosis and portal hypertension, liver transplantation provides a 90% chance of achieving normal life.
肝外胆道闭锁的发病率约为1/15000活产儿，在英国每年约有50例新发病例。其表现为持续性黄疸，通常发生在足月儿身上，并出现梗阻性黄疸的体征。通过公众教育和专业教育，鼓励早期转诊和诊断，以促进在8周龄前进行初次手术，从而改善了治疗效果。手术治疗是综合性的，包括尝试恢复胆汁流动（Kasai肝门空肠吻合术），必要时进行肝移植。药物治疗包括使用抗生素、熊去氧胆酸以促进胆汁流动、补充脂溶性维生素以及营养支持。将手术集中在专业中心进行，已使这种潜在致命疾病在英国的存活率提高到90%以上。超过一半接受肝门空肠吻合术的婴儿黄疸会消退，且有超过80%的机会拥有良好的生活质量，无需移植即可进入青春期。对于那些出现肝硬化和门静脉高压难治性并发症的儿童，肝移植使其有90%的机会过上正常生活。
