儿童肾移植术后局灶性节段性肾小球硬化六例诊治报道并文献复习  

Diagnosis and treatment of focal segmentalglomerulosclerosis after kidney transplantation in children

作　　者：聂蔚健 傅茜[1] 李军[1] 吴成林[1] 邓荣海[1] 干茜茜 陈文芳[2] 陈丽植[3] 莫樱[3] 蒋小云[3] 刘龙山[1] 王长希[1] Nie Weijian;Fu Qian;Li Jun;Wu Chenglin;Deng Ronghai;Gan Xixi;Chen Wenfang;Chen Lizhi;Mo Ying;Jiang Xiaoyun;Liu Longshan;Wang Changxi(Organ Transplant Center,First Affiliated Hospital,Sun Yat-sen University,Guangzhou 510080,China;Department of Pathology,First Affiliated Hospital,Sun Yat-sen University,Guangzhou 510080,China;Department of Pediatrics,First Affiliated Hospital,Sun Yat-sen University,Guangzhou 510080,China)

机构地区：[1]中山大学附属第一医院器官移植中心,广州510080 [2]中山大学附属第一医院病理科,广州510080 [3]中山大学附属第一医院儿科,广州510080

出　　处：《中华器官移植杂志》2020年第2期70-74,共5页Chinese Journal of Organ Transplantation

基　　金：国家自然科学基金(81870511、81670680、81700655);广东省自然科学基金(2018A030313016、2018A030313855);广东省科技计划项目(2014B020212006、2015B020226002);广东省器官捐献与移植免疫重点实验室(2013A061401007、2017B030314018);广州市科技计划项目(201803040011、201903010058);器官移植国际合作基地建设(2015B050501002)。

摘　　要：目的:探讨儿童移植肾后FSGS的诊断与治疗。方法:总结2015年至2019年共6例儿童移植肾FSGS的临床特征。6例受者术后4~49 d内发生大量蛋白尿(3.2~13 g/24 h),使用了以甲泼尼龙联合血浆置换和(或)利妥昔单抗为核心的治疗方案,辅以ACEI/ARB类药物控制尿蛋白。5例维持他克莫司,1例改为环孢素A冲击治疗后重新换回他克莫司。结果:4例受者经治疗后达到完全缓解,1例部分缓解。6例术后随访11个月至4年,5例血肌酐在正常水平,1例在术后15个月因肺部感染死亡。结论:儿童肾移植后发生FSGS,及时予甲泼尼龙冲击,联合使用血浆置换、利妥昔单抗,并辅以ACEI/ARB等药物,可取得良好疗效。Objective To explore the diagnosis and treatment of focal segmental glomerulosclerosis(FSGS)post-kidney transplantation in children.Methods Clinical data were retrospectively analyzed for 6 FSGS children after transplantation from 2015 to 2019.Massive proteinuria(3.2-13 g/24 h)occurred at 4 days-49 days post-transplantation.For proteinuria,glucocorticoid plus therapeutic plasma exchange and/or rituximab were provided with supplemental ACEI/ARB drugs.Five cases received tacrolimus as maintenance therapy while another case had cyclosporin A as an initial intensive therapy and switched to tacrolimus.Results Four cases achieved complete remission after therapy.One recipient showed partial remission.During a follow up period of 11 months to 4 years,serum creatinine remained normal and stable in five cases while one died from severe pulmonary infection.Conclusions Once FSGS occurs post-transplantation,prompt treatment of pulse glucocorticoid plus therapeutic plasma exchange and/or rituximab with supplemental ACEI/ARB drugs may yield favorable outcomes.

关 键 词：肾移植 儿童 局灶性节段性肾小球硬化 糖皮质激素 血浆置换 利妥昔单抗 

分 类 号：R726.9[医药卫生—儿科]