Howard E R, Davenport M
Department of Paediatric Surgery, King's College Hospital, London, UK.
Tohoku J Exp Med. 1997 Jan;181(1):75-83. doi: 10.1620/tjem.181.75.
European studies of biliary atresia have suggested that the aetiology is heterogeneous. Histological studies of the liver and biliary remnants excised at portoenterostomy have failed to identify any prognostic features except for the size of bile ductules in the porta hepatis. Most of the major series have confirmed that there is a relationship between age at portoenterostomy and clearance of jaundice which has been achieved in more than 67% of infants under 10 weeks of age. Cholangitis reduced survival and bleeding from esophageal varices has occurred in more than 19% of long-term survivors. The 5-year jaundice-free survival rate after portoenterostomy is 37% and the 10 year rate is 18%. It is predicted that these survival rates will improve. Orthotopic transplantation now results in long-term survival in 70% of patients who fail the portoenterostomy operation and long-term survival is now achieved in a majority of children born with biliary atresia.
欧洲关于胆道闭锁的研究表明，其病因具有异质性。对在肝门空肠吻合术中切除的肝脏和胆道残余组织进行的组织学研究，除了肝门部胆小管的大小外，未能发现任何预后特征。大多数主要系列研究证实，肝门空肠吻合术时的年龄与黄疸消退之间存在关联，在10周龄以下的婴儿中，超过67%实现了黄疸消退。胆管炎会降低生存率，超过19%的长期存活者出现过食管静脉曲张出血。肝门空肠吻合术后5年无黄疸生存率为37%，10年生存率为18%。预计这些生存率将会提高。目前，原位移植使70%肝门空肠吻合术失败的患者获得长期生存，并且大多数患有胆道闭锁的患儿现在也能实现长期生存。
