儿童特发性膜性肾病128例临床病理特征及预后分析  

Clinicopathological features and prognosis of 128 children with idiopathic membranous nephropathy

作　　者：刘俊梅[1] 史佩佩[1] 贾莉敏[1] 曹璐 张花婷 王琴[1] 张建江[1] Liu Junmei;Shi Peipei;Jia Limin;Cao Lu;Zhang Huating;Wang Qin;Zhang Jianjiang(Department of Pediatrics,the First Affiliated Hospital of Zhengzhou University,Clinical Center of Pediatric Nephrology of Henan Province,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院儿科,河南省儿童肾脏病临床诊疗中心,郑州450052

出　　处：《中华实用儿科临床杂志》2023年第6期452-456,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：河南省高等学校重点科研项目(21A320070);河南省医学科技攻关计划联合共建项目(LHGJ20210280)。

摘　　要：目的分析儿童特发性膜性肾病(IMN)的临床病理特征及预后,并探讨预后影响因素。方法回顾性分析2012年1月至2019年12月郑州大学第一附属医院住院的128例IMN患儿的临床、病理资料,根据病理表现将膜性肾病(MN)患儿分为2组:A组(典型MN组),B组(不典型MN组),比较2组临床病理特征。总结不同治疗方案及疗效,分析预后及其影响因素。随访主要终点事件为发生终末期肾病(ESRD),次要终点事件为发生肾功能不全。按照患儿至随访终点时间是否发生终点事件将其分为达终点事件组和未达终点事件组。采用Kaplan-Meier生存曲线法进行生存分析。采用Cox比例风险模型法分析IMN患儿肾脏不良预后的影响因素。结果1.共纳入128例患儿,中位发病年龄13.0(10.3,15.0)岁;发病年龄高峰为12~16岁(占68.8%);男女发病比例1.13∶1.00。119例(93.0%)表现为大量蛋白尿,其中合并血尿103例(80.5%);4例(3.1%)为单纯血尿;5例(3.9%)为非肾病水平蛋白尿。A组29例(22.7%),B组99例(77.3%)。2.B组血三酰甘油水平[2.1(1.5,3.0)mmol/L]高于A组[1.7(1.1,2.5)mmol/L](P<0.05),B组血高密度脂蛋白[1.5(1.1,1.8)mmol/L]、血白蛋白[22.0(17.0,27.3)g/L]、血补体C3[(1.1±0.2)g/L]水平均低于A组[1.8(1.4,2.1)mmol/L,25.5(21.0,32.5)g/L,(1.2±0.2)g/L](均P<0.05)。3.共91例IMN患儿住院及复诊资料完整,中位随访时间87.0(49.0,104.5)个月,其中5例(5.5%)进展为ESRD(其中3例接受肾移植),9例(9.9%)发生次要终点事件。5年和10年ESRD的累积肾脏生存率分别为96.2%、92.9%;5年和10年的次要终点的累积肾脏生存率分别为95.2%、84.8%。4.Kaplan-Meier生存分析显示,A组和B组患儿累积肾脏生存率差异无统计学意义(P>0.05)。多因素Cox回归分析显示,肾小管萎缩/间质纤维化为IMN患儿肾功能不全的独立危险因素(HR=0.102,95%CI:0.011~0.940,P<0.05)。结论儿童IMN临床表现主要为大量蛋白尿合并血尿,病理表现以不典型MN为主;肾小管萎�Objective To analyze the clinicopathological features and prognosis of idiopathic membranous nephropathy(IMN)in children,and to investigate the factors influencing their prognosis.Methods The clinical and pathological data of 128 children with IMN hospitalized in the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2019 were retrospectively analyzed.They were divided into 2 groups according to the pathological manifestations:group A[typical membranous nephropathy(MN)group]and group B(atypical MN group),and the clinicopathological characteristics of the 2 groups were compared.Different treatment regimens and their efficacy were summarized,and the prognosis and its influencing factors were analyzed.The primary endpoint event at follow-up was the occurrence of end stage renal disease(ESRD),and the secondary endpoint event was the occurrence of renal insufficiency.Children with IMN were further divided into endpoint event group and non-endpoint event group according to the presence or absence of endpoint events at the last follow-up.Survival analysis was performed using the Kaplan-Meier survival curve method.The Cox proportional risk model method was used to analyze the factors influencing the prognosis of poor kidney outcomes in children with IMN.Results(1)A total of 128 children were included,with the male-to-female ratio of 1.13∶1.00.The median age of onset and peak age of onset were 13.0(10.3,15.0)years,and 12-16 years(68.8%),respectively.Massive proteinuria was detected in 119 cases(93.0%),including 103 cases(80.5%)with massive proteinuria and hematuria,4 cases(3.1%)with simple hematuria,and 5 cases(3.9%)with non-renal proteinuria.There were 29 cases(22.7%)in group A and 99 cases(77.3%)in group B.(2)Blood triacylglycerol level was significantly higher in group B than that of group A[2.1(1.5,3.0)mmol/L vs.1.7(1.1,2.5)mmol/L],while high-density lipoprotein[1.5(1.1,1.8)mmol/L vs.1.8(1.4,2.1)mmol/L],serum albumin[22.0(17.0,27.3)g/L vs.25.5(21.0,32.5)g/L]and complement C3[(1.1±0.2)g/

关 键 词：特发性膜性肾病 儿童 免疫抑制剂 预后 

分 类 号：R726.9[医药卫生—儿科]