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胆道闭锁的序贯治疗策略:葛西肝门肠吻合术-肝移植:一项单中心回顾性比较队列研究。

Sequential treatment strategy for biliary atresia: Kasai hepatoportoenterostomy-liver transplantation: a single-center retrospective comparative cohort study.

作者信息

Xie Zhenyu, Chen Yang, Peng Yun, Luo Chengkun, Xiang Bo, Yang Jiayin, Song Jiulin, Jin Shuguang

机构信息

Department of Pediatric Surgery, West China Hospital, Sichuan University, Chengdu, 610041, China.

Department of Liver Transplantation Center, West China Hospital, Sichuan University, Chengdu, 610041, China.

出版信息

BMC Pediatr. 2025 Aug 4;25(1):594. doi: 10.1186/s12887-025-05959-0.

DOI:10.1186/s12887-025-05959-0
PMID:40759930
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12320277/
Abstract

OBJECTIVE

To analyze and compare the efficacy of two treatment strategies for biliary atresia (BA) sequential treatment of Kasai hepatoportoenterostomy-liver transplantation and primary liver transplantation.

METHODS

A retrospective analysis was conducted on the clinical data of 300 patients with BA who underwent LT in our center. Among these patients, 225 underwent Kasai hepatoportoenterostomy - liver transplantation sequential treatment (Kasai-LT group), while 75 received primary liver transplantation (pLT group). Data on demographic characteristics, perioperative conditions, postoperative recovery, and complications were collected to analyze the efficacy, complication rates, and survival outcomes of the two treatment approaches.

RESULTS

After propensity score matching analysis, there were no differences in postoperative liver function recovery between two groups. However, the Kasai-LT group had lower APRI, lower γ-GT level, and lower PELD scores when liver transplantation. The Kasai-LT group had shorter operative time, shorter PICU stay, shorter hospital stay with lower hospitalization cost. The incidence of hepatic artery complications and thoracoabdominal infections after LT was significantly higher in the pLT group compared to the Kasai-LT group. The 5-year recipient cumulative survival rate was 94.40% in the Kasai-LT group compared to 88.10% in the pLT group. Furthermore, the 5-year cumulative graft survival rate was 89.30% in the Kasai-LT group and 86.80% in the pLT group.

CONCLUSION

The treatment of BA should follow Kasai-LT sequential treatment combined with individualized treatment plans to achieve better pre-transplantation general condition and reduce the incidence of postoperative complications.

摘要

目的

分析和比较两种治疗策略对胆道闭锁(BA)的疗效,即Kasai肝门肠吻合术 - 肝移植序贯治疗与原位肝移植。

方法

对在本中心接受肝移植的300例BA患者的临床资料进行回顾性分析。其中,225例行Kasai肝门肠吻合术 - 肝移植序贯治疗(Kasai - LT组),75例接受原位肝移植(pLT组)。收集人口统计学特征、围手术期情况、术后恢复及并发症等数据,以分析两种治疗方法的疗效、并发症发生率和生存结局。

结果

倾向评分匹配分析后,两组术后肝功能恢复情况无差异。然而,Kasai - LT组在肝移植时APRI较低、γ - GT水平较低、PELD评分较低。Kasai - LT组手术时间较短、PICU住院时间较短、住院时间较短且住院费用较低。与Kasai - LT组相比,pLT组肝移植后肝动脉并发症和胸腹感染的发生率显著更高。Kasai - LT组5年受者累积生存率为94.40%,pLT组为88.10%。此外,Kasai - LT组5年移植肝累积生存率为89.30%,pLT组为86.80%。

结论

BA的治疗应采用Kasai - LT序贯治疗并结合个体化治疗方案,以获得更好的移植前一般状况并降低术后并发症的发生率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43d0/12320277/8a61e3d35308/12887_2025_5959_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43d0/12320277/826d27fd8379/12887_2025_5959_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43d0/12320277/8a61e3d35308/12887_2025_5959_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43d0/12320277/826d27fd8379/12887_2025_5959_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43d0/12320277/8a61e3d35308/12887_2025_5959_Fig2_HTML.jpg

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本文引用的文献

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Biliary atresia.先天性胆道闭锁。
Nat Rev Dis Primers. 2024 Jul 11;10(1):47. doi: 10.1038/s41572-024-00533-x.
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Establishment and validation of a predictive model of immune tolerance after pediatric liver transplantation: a multicenter cohort study.小儿肝移植后免疫耐受预测模型的建立与验证:一项多中心队列研究
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Primary Liver Transplant in Biliary Atresia: The Case for and Against.胆道闭锁的原位肝移植:支持与反对的理由
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Long-term outcomes of liver transplantation for biliary atresia and results of policy changes: over 20 years of follow-up experience.胆道闭锁肝移植的长期预后及政策变化结果:20多年的随访经验
Front Pediatr. 2024 Mar 1;11:1242009. doi: 10.3389/fped.2023.1242009. eCollection 2023.
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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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A novel prediction tool based on shear wave elastography, gallbladder ultrasound, and serum biomarkers for the early diagnosis of biliary atresia in infants younger than 60 days old.一种基于剪切波弹性成像、胆囊超声和血清生物标志物的新型预测工具,用于60日龄以下婴儿胆道闭锁的早期诊断。
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[Clinical efficacy of split liver transplantation in the treatment of children with biliary atresia].劈离式肝移植治疗小儿胆道闭锁的临床疗效
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