西罗莫司治疗小儿肝移植中的慢性和类固醇耐药的异体移植排斥相关纤维化。

IF 2.4 3区 医学 Q3 GASTROENTEROLOGY & HEPATOLOGY
Jesús Quintero Bernabeu, Javier Juamperez Goñi, Maria Mercadal Hally, Cristina Padrós Fornieles, Juan Ortega López, Mauricio Larrarte King, José A Molino Gahete, María T Salcedo Allende, Ernest Hidalgo Llompart, Itxarone Bilbao Aguirre, Ramon Charco Torra
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引用次数: 0

摘要

本研究旨在报告西罗莫司在慢性排斥反应或类固醇耐受性排斥反应合并肝纤维化的小儿肝移植患者中的应用经验,重点关注其组织学演变。2003年7月至2022年7月期间,所有因慢性导管开放性排斥反应或皮质类固醇耐受性排斥反应而接受西罗莫司标签外治疗的小儿肝移植受者均被纳入研究。研究中的9名患者在使用西罗莫司1个月后,肝酶和胆汁淤积指标均有所改善。在 36 个月时,7/9(77.7%)名患者的肝纤维化程度有所减轻。除一名患者外,所有患者的排斥反应活动指数和导管减少症在 12 个月时都有所改善。一名患者因出现肾病性蛋白尿而不得不停止西罗莫司治疗。总之,西罗莫司可能是治疗慢性和类固醇耐药排斥反应的一种安全有效的方法,并可改善异体移植排斥反应相关的纤维化和导管损伤。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Sirolimus to treat chronic and steroid-resistant allograft rejection-related fibrosis in pediatric liver transplantation.

This study aimed to report our experience with the use of Sirolimus (SRL) in pediatric liver transplant patients with chronic rejection or steroid-resistant rejection with hepatic fibrosis, focusing on their histological evolution. All pediatric liver transplant recipients who received off-label treatment with SRL for chronic ductopenic rejection or cortico-resistant rejection between July 2003 and July 2022 were included in the study. All nine patients included in the study showed improvement in liver enzymes and cholestasis parameters as soon as 1-month after post-SRL introduction. A decrease in fibrosis stage was observed in 7/9 (77.7%) patients at 36 months. All but one patient experienced an improvement in the Rejection Activity Index and ductopenia at 12 months. A single patient had to discontinue SRL treatment owing to nephrotic proteinuria. In conclusion, SRL may be a safe and effective treatment for chronic and steroid-resistant rejection and may improve allograft rejection-related fibrosis and ductal damage.

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来源期刊
CiteScore
5.30
自引率
13.80%
发文量
467
审稿时长
3-6 weeks
期刊介绍: ​The Journal of Pediatric Gastroenterology and Nutrition (JPGN) provides a forum for original papers and reviews dealing with pediatric gastroenterology and nutrition, including normal and abnormal functions of the alimentary tract and its associated organs, including the salivary glands, pancreas, gallbladder, and liver. Particular emphasis is on development and its relation to infant and childhood nutrition.
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