来自中西部儿科手术联盟（MWPSC）的真实世界多机构数据，以评估延迟进行肝门空肠吻合术对胆道闭锁患者胆汁引流的影响。

Real-world Multi-institutional Data From the Midwest Pediatric Surgery Consortium (MWPSC) to Assess the Effect of Delayed Kasai Procedure on Biliary Drainage in Patients With Biliary Atresia.

作者信息

Farooqui Zishaan A, Hartman Stephen J, Stetson Alyssa E, Schepers Emily J, Speck Karen E, Gadepalli Samir K, Van Arendonk Kyle J, Georgeades Christina, Lal Dave R, Deans Katherine J, Minneci Peter C, Apfeld Jordan C, Saito Jacqueline M, Mak Grace Z, Slidell Mark B, Lemoine Caroline, Superina Riccardo, Wright Tiffany N, Downard Cynthia D, Devara Lekha P, Hirschl Ronald B, Landman Matthew P, Leys Charles M, Markel Troy A, Rymeski Beth, Mullapudi Bhargave, Tiao Gregory M

机构信息

Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229, USA.

出版信息

J Pediatr Surg. 2025 May;60(5):162250. doi: 10.1016/j.jpedsurg.2025.162250. Epub 2025 Feb 18.

DOI:10.1016/j.jpedsurg.2025.162250

PMID:40023993

Abstract

PURPOSE

Early Kasai portoenterostomy (KPE) for infants with biliary atresia (BA) increases the chance of transplant-free survival (TFS). However, early timing of KPE is not consistently achieved in the United States. Clearance of jaundice at three months is predictive of TFS. Among a cohort of patients with BA, we investigated institutional variability in the initiation of hyperbilirubinemia evaluation and operative timing to identify factors associated with successful jaundice clearance.

METHODS

A multi-institutional, retrospective study was performed at eleven U.S. tertiary children's hospitals. Infants diagnosed with BA between 10/1/2015-10/1/2020 were identified. Age at initiation of diagnostic workup and age at KPE were collected. Adjusted multivariable logistic regression was used to determine factors associated with direct bilirubin normalization at three months following KPE.

RESULTS

In 161 infants, the median age at initiation of jaundice evaluation was 35 days (IQR 8-60). Among 148 patients who underwent KPE, median age at surgery was 53 days (IQR 37.3-67.5). Each 10-day increase in age at KPE was associated with a 18.8 % decrease in odds of normalizing bilirubin at three months (OR 0.81, 95 % CI 0.66-0.99), with infants who underwent KPE ≤50 days significantly more likely to normalize bilirubin (OR 2.6, CI 1.1-6.1) compared to KPE >50 days. There was significant variation among institutions in the time from initiation of workup to KPE (range 0-24.5 days, p = 0.02) and the odds of patients normalizing direct bilirubin at three months (range 0.04-0.89, p = 0.044).

CONCLUSION

Our results confirmed that increasing age at KPE decreases the odds of clearing bilirubin at three months post-KPE. We identified significant institutional variability in the time from workup to KPE that may have impacted the likelihood of successful biliary drainage.

LEVEL OF EVIDENCE

IV (Well-designed case-control or cohort study).

摘要

目的

对于患有胆道闭锁（BA）的婴儿，早期进行Kasai肝门空肠吻合术（KPE）可增加无移植生存（TFS）的机会。然而，在美国，KPE的早期时机并非总能实现。三个月时黄疸消退可预测TFS。在一组BA患者中，我们调查了高胆红素血症评估起始和手术时机方面的机构差异，以确定与黄疸成功消退相关的因素。

方法

在美国11家三级儿童医院进行了一项多机构回顾性研究。确定了2015年10月1日至2020年10月1日期间诊断为BA的婴儿。收集诊断检查起始时的年龄和KPE时的年龄。采用校正后的多变量逻辑回归来确定与KPE后三个月直接胆红素正常化相关的因素。

结果

在161名婴儿中，黄疸评估起始的中位年龄为35天（四分位间距8 - 60天）。在148例接受KPE的患者中，手术的中位年龄为53天（四分位间距37.3 - 67.5天）。KPE时年龄每增加10天，三个月时胆红素正常化的几率降低18.8%（比值比0.81，95%置信区间0.66 - 0.99），与KPE>50天的婴儿相比，KPE≤50天的婴儿胆红素正常化的可能性显著更高（比值比2.6，置信区间1.1 - 6.1）。各机构从检查起始到KPE的时间（范围0 - 24.5天，p = 0.02）以及患者三个月时直接胆红素正常化的几率（范围0.04 - 0.89，p = 0.044）存在显著差异。