重复肾活检诊断为原发性局灶节段性肾小球硬化症患儿的临床病理表现  

Clinical and pathological features in children diagnosed with primary focal segmental glomerulosclerosis after repeated renal biopsy

作　　者：张志强 彭映潮 贾丽丽 高春林 夏正坤 Zhang Zhiqiang;Peng Yingchao;Jia Lili;Gao Chunlin;Xia Zhengkun(Department of Pediatrics,Jinling Hospital,Nanjing 210016,China)

机构地区：[1]东部战区总医院儿科,南京210016

出　　处：《中华肾脏病杂志》2022年第8期657-663,共7页Chinese Journal of Nephrology

基　　金：东部战区总医院2021年度院内课题青年课题(YYQN2021093)。

摘　　要：目的探讨重复肾活检诊断为原发性局灶节段性肾小球硬化症(focal segmental glomerulosclerosis,FSGS)患儿的临床病理特点。方法回顾性收集2000年1月1日至2020年12月31日在东部战区总医院经重复肾活检诊断为原发性FSGS患儿的临床病理资料,总结和分析患儿临床表现特征、两次肾活检病理类型及治疗反应。结果共34例原发性FSGS患儿入选本研究,男性22例,女性12例,首次肾活检中位年龄14岁(1~18岁),重复肾活检中位间隔时间6个月(1~151个月)。31例表现为肾病综合征,其中22例伴有镜下血尿,4例血肌酐升高;余3例中,2例表现为血尿、蛋白尿,1例表现为蛋白尿。首次肾活检时有16例患儿诊断为微小病变性肾小球病,14例诊断为系膜增生性肾小球肾炎,2例诊断为IgA肾病,2例诊断为IgM肾病。34例患儿均表现为对激素和免疫抑制剂治疗反应差。首次肾活检部分患儿肾脏病理表现为球囊粘连(2/34)、袢足细胞附着减少(2/34)、外周袢向尿极延伸(2/34)、肾小管反流(4/34)、毛细血管腔内血栓形成(2/34)及IgM沉积(12/34)。结论FSGS发病初期临床病理表现不典型,易漏诊,对激素和免疫抑制剂治疗反应差。肾脏病理表现为球囊粘连、袢足细胞附着减少、外周袢向尿极延伸、肾小管反流、毛细血管腔内血栓形成及IgM沉积的患儿需加强随访,必要时行重复肾活检,明确FSGS诊断。Objective To analyze the clinical and pathological characteristics in children diagnosed with primary focal segmental glomerulosclerosis(FSGS)after repeated renal biopsy.Methods The clinicopathological data of children who ever experienced renal biopsy in Jinling Hospital from January 1,2000 to December 31,2020 were retrospectively reviewed.Clinical manifestations,pathological characteristics and treatment responses were analyzed.Results Of the 34 enrolled patients,there were 22 males and 12 females.The median age of the first renal biopsy was 14 years old(1-18 years old),and the median interval between repeat renal biopsy and first renal biopsy was 6 months(1-151 months).Thirty⁃one showed nephrotic syndrome,of which 22 had microscopic hematuria,and 4 had elevated serum creatinine.Among the other 3 patients,2 had hematuria and proteinuria,and 1 had proteinuria.In the first renal biopsy,16 cases were diagnosed as minimal change disease,14 cases were diagnosed as mesangial proliferative glomerulonephritis,2 cases were diagnosed as IgA nephropathy,and 2 cases were diagnosed as IgM nephropathy.All 34 children showed poor responses to hormone and immunosuppressive therapies.The pathological features of the first renal biopsy in some patients were adhesion(2/34),decreased loop podocyte attachment(2/34),peripheral loop extension to the urinary pole(2/34),renal tubular reflux(4/34),capillary thrombosis(2/34)and IgM deposition(12/34).Conclusions The initial diagnosis of FSGS is difficult,and the lesions are atypical and easily misdiagnosed.The patients have poor responses to hormone and immunosuppressive therapies.For patients with the pathological changes of adhesion,decreased loop podocyte attachment,peripheral loop extension to the urinary pole,renal tubular reflux,capillary thrombosis and IgM deposition,follow⁃up is required,and if necessary,repeat renal biopsy needs be performed to determine whether it is FSGS.

关 键 词：肾小球硬化症 局灶节段性 儿童 肾病综合征 肾脏病理 

分 类 号：R726.9[医药卫生—儿科]