Mieli-Vergani G, Howard E R, Portman B, Mowat A P
Department of Child Health, King's College School of Medicine, Denmark Hill, London.
Lancet. 1989 Feb 25;1(8635):421-3. doi: 10.1016/s0140-6736(89)90012-3.
To assess whether clinicopathological features other than the age at operation influence prognosis after surgery for extrahepatic biliary atresia (EHBA) and to determine whether the age at referral has fallen since a previous survey, 50 consecutive cases with EHBA referred between February, 1985, and December, 1987, were reviewed. Liver or spleen size, liver function tests, or histological appearance of liver biopsy specimen before surgery were not predictive of outcome. The jaundice cleared up in 12 of 14 children operated on by age 8 weeks, but in only 13 of 36 operated on later. In 41 referral was delayed. All 25 children in whom surgery was successful are alive and well, while 13 of 25 with unsuccessful surgery have died, at a median age of 1 year. To improve the prognosis of infants with EHBA parents and health staff need a better awareness of the early clinical features of EHBA and of the necessity for prompt referral. Liver disease should be suspected in any infant jaundiced after 14 days of age.
为评估肝外胆管闭锁（EHBA）手术后除手术年龄外的临床病理特征是否影响预后，并确定自上次调查以来转诊年龄是否有所下降，我们回顾了1985年2月至1987年12月期间连续转诊的50例EHBA病例。术前肝脏或脾脏大小、肝功能检查或肝活检标本的组织学表现均不能预测预后。14名8周龄前接受手术的儿童中有12名黄疸消退，但36名年龄较大才接受手术的儿童中只有13名黄疸消退。41例转诊延迟。所有25例手术成功的儿童均存活且状况良好，而25例手术失败的儿童中有13例死亡，中位年龄为1岁。为改善EHBA婴儿的预后，家长和医护人员需要更好地认识EHBA的早期临床特征以及及时转诊的必要性。任何14日龄后出现黄疸的婴儿都应怀疑患有肝病。
