Abstract：
This study aimed to report our experience with the use of sirolimus 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 sirolimus 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 postsirolimus 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 sirolimus treatment owing to nephrotic proteinuria. In conclusion, sirolimus may be a safe and effective treatment for chronic and steroid-resistant rejection and may improve allograft rejection-related fibrosis and ductal damage.
摘要：
这项研究旨在报告我们的经验与使用西罗莫司在小儿肝移植患者慢性排斥反应或类固醇抵抗排斥与肝纤维化，专注于它们的组织学进化。所有在2003年7月至2022年7月期间接受西罗莫司治疗的慢性导管性排斥反应或皮质抗性排斥反应的儿科肝移植受者均纳入研究。纳入研究的所有9名患者在西罗莫司后1个月内均显示肝酶和胆汁淤积参数改善。在36个月时，在7/9（77.7％）患者中观察到纤维化分期降低。除一名患者外，所有患者在12个月时的排斥活动指数和导管减少均有所改善。由于肾病性蛋白尿，一名患者不得不停止西罗莫司治疗。总之,西罗莫司可能是一种安全有效的治疗慢性和类固醇耐药的排斥反应，并可能改善同种异体移植排斥反应相关的纤维化和导管损伤。
