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评估Kasai肝门空肠吻合术在90天以上龄期胆道闭锁患儿中的作用。

Evaluating the role of Kasai portoenterostomy in biliary atresia older than 90 days.

作者信息

Tian Yu, Lian Haoran, Ye Mao, Shao Yifeng, Geng Yuanyuan, Chen Zhen, Chen Shuai

机构信息

Department of Pediatric Surgery, Capital Center for Children's Health, Capital Medical University, Capital Institute of Pediatrics, Beijing, 100020, China.

Precision Regenerative Medicine Research Centre, Medical Science Division, and State Key Laboratory of Quality Research in Chinese Medicine, Macau University of Science and Technology, Macao, 999078, China.

出版信息

Pediatr Surg Int. 2025 Sep 4;41(1):285. doi: 10.1007/s00383-025-06184-7.

DOI:10.1007/s00383-025-06184-7
PMID:40908322
Abstract

PURPOSE

Biliary atresia (BA) patients presenting after 90 days of age face contentious treatment decisions between Kasai portoenterostomy and direct liver transplantation. This study evaluated outcomes of Kasai portoenterostomy in older BA patients to inform therapeutic decision-making.

METHODS

A retrospective multicenter study analyzed 32 BA patients who underwent Kasai portoenterostomy beyond 90 days of age. Primary outcomes included jaundice clearance (total bilirubin < 20 μmol/L) and two-year native liver survival. Patients were stratified by jaundice clearance status for comparative analysis.

RESULTS

The median surgical age was 110 days (IQR: 98-119 days). Twenty patients (62.5%) achieved jaundice clearance, while 12 (37.5%) failed to clear jaundice. All patients achieving jaundice clearance survived with their native Liver at two years, with only one requiring transplantation in the fourth postoperative year. Conversely, none of the non-clearance patients achieved two-year native Liver survival, with 11 undergoing transplantation for progressive cholestasis at a median of 5 months postoperatively and one death after declined transplantation. Surgical age was the most significant predictor of success, with ROC analysis yielding an optimal cutoff of 109 days (AUC: 0.779), while histological fibrosis stage also showed significant association with outcomes".

CONCLUSION

Kasai portoenterostomy remains clinically meaningful in selected older BA patients, with 109 days representing a critical surgical threshold. This approach is particularly valuable in resource-limited settings where liver transplantation availability is constrained.

摘要

目的

90日龄后出现的胆道闭锁(BA)患者在Kasai肝门肠吻合术和直接肝移植之间面临有争议的治疗决策。本研究评估了大龄BA患者行Kasai肝门肠吻合术的结局,以为治疗决策提供依据。

方法

一项回顾性多中心研究分析了32例90日龄后接受Kasai肝门肠吻合术的BA患者。主要结局包括黄疸清除(总胆红素<20μmol/L)和两年自体肝生存率。根据黄疸清除状态对患者进行分层以进行比较分析。

结果

手术时的中位年龄为110天(四分位间距:98 - 119天)。20例患者(62.5%)实现了黄疸清除,而12例(37.5%)未能清除黄疸。所有实现黄疸清除的患者在两年时自体肝均存活,只有1例在术后第四年需要进行移植。相反,未清除黄疸的患者中无一例实现两年自体肝存活,11例因进行性胆汁淤积在术后中位时间5个月时接受了移植,1例在拒绝移植后死亡。手术年龄是成功的最显著预测因素,ROC分析得出最佳临界值为109天(曲线下面积:0.779),而组织学纤维化阶段也与结局显示出显著相关性。

结论

Kasai肝门肠吻合术在部分大龄BA患者中仍具有临床意义,109天是一个关键的手术阈值。在肝移植资源有限的情况下,这种方法尤其有价值。

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Serum matrix metalloproteinase-7 for discriminating biliary atresia: a diagnostic accuracy and validation study.血清基质金属蛋白酶-7鉴别先天性胆道闭锁:一项诊断准确性和验证研究。
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Biliary drainage surgery before or after 3 months of life versus primary liver transplantation in children with biliary atresia: comparative cohort study.胆道闭锁患儿在 3 个月前或后行胆道引流术与行肝移植术的对比:队列研究。
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Identification of Early Clinical and Histological Factors Predictive of Kasai Portoenterostomy Failure.预测葛西肝门空肠吻合术失败的早期临床和组织学因素的识别
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Primary vs. salvage liver transplantation for biliary atresia: A retrospective cohort study.先天性胆道闭锁的肝移植:一项回顾性队列研究。
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Sequential Treatment of Biliary Atresia With Kasai Hepatoportoenterostomy and Liver Transplantation: Benefits, Risks, and Outcome in 393 Children.采用葛西肝门肠吻合术和肝移植序贯治疗胆道闭锁:393例儿童的获益、风险及结局
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Impact of Age of Patient and Experience of Surgeon on the Outcome after Kasai Portoenterostomy: Can We Delay the Surgery?患者年龄和外科医生经验对 Kasai 门腔分流术预后的影响:我们能否延迟手术?
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