The frequency and outcome of biliary atresia in the UK and Ireland
The frequency and outcome of biliary atresia in the UK and Ireland
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DOI：
10.1016/s0140-6736(99)03492-3
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发表时间：
2000-01-01
期刊：
影响因子：
168.9
通讯作者：
Kelly, DA
中科院分区：
文献类型：
--
作者：
McKiernan, PJ;Baker, AJ;Kelly, DA
背景胆道闭锁是一种婴儿期闭塞性胆管病，如果不治疗是致命的。外科治疗，加塞门肠吻合术，可以恢复胆汁流动和清除黄疸，如果成功，实现90%的10年生存率与天然肝脏。在英国和爱尔兰的胆道闭锁的儿童2年队列的结果进行了评估，以找到目前的频率，影响结果的因素，和中期需要肝transplantation.Methods的情况下，诊断1993年3月，1995年2月，由儿科医生通知英国儿科监测单位通过每月报告系统。确诊病例进行了后续的邮寄问卷通知儿科医生。结果93例确诊，1/16 700活产的频率，在15个外科中心的91名儿童中进行了初次手术，清除黄疸的早期成功率为55%。各中心根据案件量进行分组; A组每年有5例以上案件，B组每年少于5例。A组中心的早期成功率较高，比值比为2.02（95% CI 0.86-4.73），但未达到统计学显著性。41例手术未能清除黄疸的患儿中，9例（22%）死亡，30例（73%）接受了肝移植。A组中心的非肝移植生存率和总生存率均显著较高，率比为0.48（95%CI 0.27-0.86）和0.32（0.11-0.94）。A组中心的5年无移植生存率为61.3%，B组中心为13.7%。精算5年总生存率为91.2%，在A组和75%，在组B，一旦中心的大小被考虑在内，没有其他因素，包括手术时的年龄，是预测生存没有移植或总survival.Interpretation胆道闭锁的儿童的结果是相关的手术中心，他们有他们的主要手术的病例。胆道闭锁的儿童应在外科中心进行管理，每年病例数超过5例。
Background Biliary atresia is an obliterative cholangiopathy of infancy that is fatal if untreated, Surgical treatment, the Kasai portoenterostomy, may restore bile flow and clear jaundice, and, if successful, achieve a 10-year survival of 90% with a native liver. The outcome of a 2-year cohort of children with biliary atresia in the UK and Ireland was assessed to find the current frequency, the factors influencing outcome, and the medium-term need for liver transplantation.Methods Cases diagnosed between March, 1993, and February, 1995, were notified by paediatricians to the British Paediatric Surveillance Unit via a monthly reporting system. Confirmed cases were followed up by postal questionnaires to notifying paediatricians.Findings 93 cases were confirmed, a frequency of 1/16 700 livebirths, Primary surgery was done in 91 children in 15 surgical centres with an early success rate for clearing jaundice of 55% overall. Centres were grouped according to caseload; group A had more than 5 cases/year and group B fewer than 5 cases/year. Early success was higher in group-A centres, odds ratio 2.02 (95% CI 0.86-4.73), but this did not reach statistical significance. Of 41 children in whom surgery was unsuccessful in clearing jaundice 9 (22%) died and 30 (73%) underwent liver transplantation. Survival without liver transplantation and overall survival were both significantly greater in group-A centres, rate ratios 0.48 (95% CI 0.27-0.86) and 0.32 (0.11-0.94). Actuarial 5-year survival without transplantation was 61.3% in group-A centres and 13.7% in group-B centres. Actuarial 5-year overall survival was 91.2% in group A and 75% in group B, Once centre size was taken into account, no other factor, including age at surgery, was predictive of survival without transplantation or overall survival.Interpretation The outcome of children with biliary atresia is related to the caseload of the surgical centre where they have their primary surgery. Children with biliary atresia should be managed in surgical centres with a caseload of more than five cases annually.