Biliary atresia: The King's College Hospital experience (1974-1995)

Biliary atresia: The King's College Hospital experience (1974-1995)
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DOI:
10.1016/s0022-3468(97)90611-4
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发表时间:
1997-03-01
影响因子:
2.4
通讯作者:
Howard, ER
Howard, ER
中科院分区:
医学3区
文献类型:
--
作者:
Davenport, M;Kerkar, N;Howard, ER

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本文分析了1973年1月至1995年12月间338例胆道闭锁患儿的生存经验。所有的婴儿都在一个英国中心接受了初次手术。这些婴儿根据出生年份分为三组;第1组70年代38例;组2(20世纪80年代,n = 182),组3(20世纪90年代,n = 118),第1组的数据不完整且经过选择,因此与其余组的比较受到限制,然而,所有自1980年以来接受治疗的婴儿均接受了门肠吻合术或肝管空肠吻合术,并被纳入。结果如下:在整个队列中,有89例死亡(26%),79例儿童(23%)接受了肝移植,170例儿童(50%)在末次随访时存活。第2组的5年和10年精算生存率分别为50%和41%,第3组的5年精算生存率为60%。总的来说,57名儿童在胆道闭锁手术后存活了10年。手术年龄从第1组的中位数77天逐渐下降,第2组的中位数69天下降到第3组的中位数56天(P <0.0001)。然而,对于自1980年以来接受治疗的婴儿(n = 200),5岁时的结局在年龄组(< 40天,41至60天,61至99天,大于或等于100天; P > .1)之间没有显著差异。结论:门肠吻合术是治疗40%~ 50%婴儿胆道闭锁的一种有效的长期手术。其余的50%-60%需要移植,大多数在2岁以内,尽管也有超过5岁和10岁的持续需求,手术年龄作为门肠吻合术后生存率的预测因素作用有限,当然不应该用于指导主要治疗。版权所有(C)1997 W.B.桑德斯公司
The survival experience of 338 infants born with biliary atresia between January 1973 and December 1995 was analyzed. All the infants had their initial surgery at a single UK centre. These infants were divided into three groups based on year of birth; group 1 (1970s, n = 38); group 2 (1980s, n = 182), and group 3 (1990s, n = 118), The data from group 1 were incomplete and selected, and comparisons with the remaining groups were therefore restricted, However, all infants who had been treated since 1980 underwent portoenterostomy or hepaticojejunostomy and were included. Results: In the whole cohort there were 89 deaths (26%), 79 children (23%) who underwent liver transplantation and 170 children (50%) who were alive at last follow-up, The 5- and 10-year actuarial survival for group 2 was 50% and 41%, respectively and the 5-year actuarial survival for group 3 was 60%. Overall, 57 children have survived to 10 years after surgery for biliary atresia. There has been a progressive fall in the age at surgery from a median of 77 days in group 1, through 69 days in group 2 to 56 days in group 3 (P < .0001). However, there was no significant difference in outcome to 5 years between the age cohorts (< 40 days, 41 to 60 days, 61 to 99 days, and greater than or equal to 100 days; P > .1) for the infants treated since 1980 (n = 200). Conclusions: Portoenterostomy is an effective long-term procedure for biliary atresia in about 40% to 50% of infants. The remaining 50% to 60% will require transplantation mostly within 2 years of age, although there is also a continuing need beyond 5 and 10 years, The age at surgery has limited usefulness as a predictor of survival after portoenterostomy and certainly should not be used to dictate primary treatment. Copyright (C) 1997 by W.B. Saunders Company.