A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000
A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000
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DOI：
10.1016/j.jpeds.2005.12.054
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发表时间：
2006-04-01
影响因子：
5.1
通讯作者：
Sokol, Ronald J.
中科院分区：
文献类型：
--
作者：
Shneider, Benjamin L.;Brown, Morton B.;Sokol, Ronald J.
目的确定预后因素和最佳的方法来诊断和治疗胆道闭锁，肝移植的主要指征在children.Study design一项回顾性研究进行了所有的儿童谁接受肝门肠道吻合术（HPE）胆道闭锁1997年和2000年之间在9个中心在美国。结果在24个月的年龄与人口统计学和临床parameters.Results共104名儿童进行HPE; 25%的先天性异常，和结果是更糟糕的胆道闭锁脾畸形综合征。诊断和临床方法各不相同，但具体方法似乎与结果无关。转诊时的平均年龄为53天。HPE的平均年龄是61岁。天在24个月大时，58名儿童存活于其自体肝脏，42名接受了肝移植（37名存活，5名死亡），4名未接受移植而死亡。Kaplan-Meier生存分析显示HPE后3个月总胆红素水平< 2 mg/dL的儿童生存率显著提高（84% vs 16%; P <0.0001）。HPE后早期随访的总胆红素对结局具有高度预测性。HPE后努力改善胆汁流量可能会改善胆道闭锁儿童的结局。
Objective To determine the prognostic factors and optimal approaches to the diagnosis and management of biliary atresia, the leading indication for liver transplantation in children.Study design A retrospective study was performed of all children who underwent hepatoportoenterostoiny (HPE) for biliary atresia between 1997 and 2000 at 9 centers in the United States. Outcome at age 24 months was correlated with demographic and clinical parameters.Results A total of 104 children underwent HPE; 25% had congenital anomalies, and outcome was worse in those with biliary atresia splenic malformation syndrome. Diagnostic and clinical approaches varied, although specific approaches did not appear to correlate with outcome. The average age at referral was 53 days. and the average age at HPE was 61. days. At age 24 months, 58 children were alive with their native liver, 42 had undergone liver transplantation (37 alive, 5 dead), and 4 had died without undergoing transplantation. Kaplan-Meier analysis of survival without liver transplantation revealed markedly improved survival in children with total bilirubin level < 2 mg/dL at 3 months after HPE (84% vs 16%; P < .0001).Conclusions Outcome in the study centers was equivalent to that reported in other countries. Total bilirubin in early follow-up after HPE was highly predictive of outcome. Efforts to improve bile flow after HPE may lead to improved outcome in children with biliary atresia.