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伴有系膜IgM沉积的肾小球病:64例儿童的长期随访

Glomerulopathy with mesangial IgM deposits: long-term follow up of 64 children.

作者信息

Zeis P M, Kavazarakis E, Nakopoulou L, Moustaki M, Messaritaki A, Zeis M P, Nicolaidou P

机构信息

Second Department of Pediatric, University of Athens, Greece.

出版信息

Pediatr Int. 2001 Jun;43(3):287-92. doi: 10.1046/j.1442-200x.2001.01396.x.

Abstract

BACKGROUND

The aim of the present study was to investigate to what extent IgM nephropathy in children with minimal change nephrotic syndrome (MCNS) and diffuse mesangial hypercellularity (DMH) evolves to focal segmental glomerulosclerosis (FSGS).

METHODS

Tissues from renal biopsies were examined by light microscopy (LM), immunofluorescence (IF) and, in four cases, by electron microscopy (EM). From a total of 352 nephrotic children, 121 had renal biopsy results as steroid dependent or resistant. A diagnostic renal biopsy was also performed in 331 children with non-nephrotic proteinuria and/or hematuria. A second renal biopsy was performed in 16 children whose renal function was impaired during the follow up. The clinical course of IgM-positive children was compared with that of IgM-negative children.

RESULTS

Of the 121 nephrotic children with renal biopsy, 85 were MCNS. Twenty were IF positive mainly for IgM, six of whom (30%) presented evolution to FSGS, while of the remaining 65 IF-negative children, only three (4.6%) presented evolution to FSGS. Of the total 331 children with non-nephrotic proteinuria and/or hematuria, 139 were diagnosed as IgA--IgG nephropathy, 44 had positive IF for IgM and 148 were IF negative. Of the 44 children IF positive for IgM, seven (15.9%) presented evolution to FSGS, while none of the 148 IF-negative children presented evolution to FSGS. The follow-up time for all children ranged from 1 to 14 years.

CONCLUSIONS

Of IgM nephropathy patients with MCNS and DMH, a significant percentage develop impaired renal function, due to the evolution of FSGS, as revealed by repeat biopsy during long-term follow up.

摘要

背景

本研究旨在调查微小病变肾病综合征(MCNS)合并弥漫性系膜细胞增多(DMH)的儿童中,IgM肾病发展为局灶节段性肾小球硬化(FSGS)的程度。

方法

对肾活检组织进行光学显微镜(LM)、免疫荧光(IF)检查,4例进行了电子显微镜(EM)检查。在总共352例肾病儿童中,121例肾活检结果为激素依赖或抵抗。对331例非肾病性蛋白尿和/或血尿患儿也进行了诊断性肾活检。对16例随访期间肾功能受损的患儿进行了第二次肾活检。比较了IgM阳性患儿与IgM阴性患儿的临床病程。

结果

在121例进行肾活检的肾病患儿中,85例为MCNS。20例IF主要为IgM阳性,其中6例(30%)发展为FSGS,而其余65例IF阴性患儿中,只有3例(4.6%)发展为FSGS。在总共331例非肾病性蛋白尿和/或血尿患儿中,139例被诊断为IgA-IgG肾病,44例IF为IgM阳性,148例IF阴性。在44例IF为IgM阳性的患儿中,7例(15.9%)发展为FSGS,而148例IF阴性患儿中无一例发展为FSGS。所有患儿的随访时间为1至14年。

结论

长期随访期间重复活检显示,MCNS和DMH的IgM肾病患者中,有相当比例因FSGS的发展而出现肾功能受损。

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