摘要
目的总结儿童复发性移植肾肾病的临床特点及诊治经过,为早期诊断和治疗提供经验。方法回顾性分析总结2014年1月至2024年10月于中山大学附属第一医院诊治的年龄≤18岁免疫性肾小球疾病肾移植术后复发患儿的临床及病理资料。结果18例复发性移植肾肾病患儿中男性11例、女性7例。诊断构成:移植肾局灶节段性肾小球硬化(FSGS)9例,移植肾IgA肾病(IgAN)5例,移植肾抗中性粒细胞胞质抗体相关肾炎(AAGN)2例,移植肾IgA血管炎相关性肾炎(IgAVN)2例。9例移植肾FSGS患儿,经治疗后4例达到完全缓解,术后随访3个月~7年,6例肾功能正常,5例尿蛋白持续未转阴(其中2例移植肾失功、1例移植肾功能不全、2例肾功能正常)。5例IgAN患儿,2例表现为大量蛋白尿,分别于术后2、7年进展至终末期肾病。2例移植后IgAVN均对糖皮质激素治疗有反应,1例经利妥昔单抗治疗后症状消失,1例经历2次复发后维持部分缓解,术后2年9个月、3年肾功能均正常;2例移植后AAGN治疗后完全缓解、肾功能正常。结论儿童常见的复发性移植肾肾病包括FSGS、IgAN、AAGN和IgAVN。积极肾活检协助诊断及有效干预可取得较好的疗效。
Objective To summarize the clinical and pathological features,diagnosis,and treatment of recurrent allograft nephropathy in children,and to provide experience for early diagnosis and treatment.Methods The clinical and pathological data of children≤18 years old with recurrent immune glomerular disease after kidney transplantation diagnosed and treated in the First Affiliated Hospital of Sun Yat-sen University from January 2014 to October 2024 were retrospectively analyzed and summarized.Results There were 11 males and 7 females.Of the 18 patients,9 cases of focal segmental glomerulosclerosis(FSGS),5 cases of IgA nephropathy(IgAN),2 cases of anti-neutrophil cytoplasmic antibody associated glomerulonephritis(AAGN),and 2 cases of IgA vasculitis associated glomerulonephritis(IgAVN).Among the 9 cases of FSGS,4 cases achieved complete remission after treatment,6 cases had normal renal function 3 months to 7 years after operation,and 5 cases had no proteinuria.Among them,2 cases had graft failure,1 case had graft insufficiency,and 2 cases had normal renal function.Of the 5 IgAN patients,2 cases showed massive proteinuria and entered ESRD at 2 and 7 years after operation.Two cases of IgAVN responded to glucocorticoid therapy.One case was treated with rituximab and the symptoms disappeared.The other case experienced two relapses and maintained partial remission.Two patients with AAGN achieved complete remission with normal renal function after treatment.Conclusions The common recurrent allograft nephropathy in children includes FSGS,IgAN,AAGN,and IgAVN.Active renal biopsy and effective intervention can achieve good curative effects.
作者
姜梦婕
黄淑娟
裴瑜馨
容丽萍
许园园
林知朗
丘原全
刘龙山
蒋小云
陈丽植
Jiang Mengjie;Huang Shujuan;Pei Yuxin;Rong Liping;Xu Yuanyuan;Lin Zhilang;Qiu Yuanquan;Liu Longshan;Jiang Xiaoyun;Chen Lizhi(Department of Pediatric Nephrology and Rheumatology,2Organ Transplant Center,the First Affiliated Hospital,Sun Yat-sen University,Guangzhou 510080,China)
出处
《中华移植杂志(电子版)》
2025年第1期16-21,共6页
Chinese Journal of Transplantation(Electronic Edition)
基金
国家重点研发计划(2022YFC2705104)。
关键词
肾移植
复发
儿童
临床特点
治疗
Kidney transplantation
Relapse
Child
Clinical feature
Therapy
