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成人免疫球蛋白M肾病:一项临床病理研究。

Immunoglobulin M nephropathy in adults: a clinicopathological study.

作者信息

Mubarak Muhammed, Naqvi Rubina, Kazi Javed, Shakeel Shaheera

机构信息

Histopathology Department, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.

出版信息

Iran J Kidney Dis. 2013 May 21;7(3):214-9.

PMID:23689154
Abstract

INTRODUCTION

There is no data specifically on the clinical and immunopathologic features of Immunoglobulin M nephropathy (IgMN) in adults with kidney diseases in Pakistan.

MATERIALS AND METHODS

We retrospectively reviewed our adult native renal biopsy records from May 2001 to April 2010 and identified 57 cases out of a total of 1,753 records labeled as IgMN on final histopathological analysis. Among these, 41 cases were included in the present analysis. Their relevant data items were collected from the case files and biopsy reports.

RESULTS

The mean age of this cohort was 30.21 ± 10.12 years. The male-female ratio was 1.15:1. The most common presentation was idiopathic nephrotic syndrome. Hematuria and hypertension at presentation were noted in 24 (58.5%) and 10 (24.4%) patients, respectively. The most common morphologic change was glomerular mesangial cell proliferation, found in 28 biopsies (68.3%). Mesangial matrix expansion was noted in 16 (39%). Minor glomerular alterations were noted in 5 cases (12.2%) and focal segmental glomerulosclerosis in 4 (9.8%). Immunofluorescence microscopy showed diffuse mesangial positivity of IgM in all specimens. Subdominant IgA was noted in 6 cases (14.6%). Complements C3 and C1q were found in 28 (68.3%) and 21 (51.2%) patients, respectively.

CONCLUSIONS

Our results show that IgMN is not very common in adults. Its clinicopathological spectrum is similar to that described from the neighboring countries, showing a spectrum of morphologic changes ranging from minor changes to focal segmental glomerulosclerosis.

摘要

引言

在巴基斯坦患有肾脏疾病的成年人中,尚无关于免疫球蛋白M肾病(IgMN)临床和免疫病理特征的具体数据。

材料与方法

我们回顾性分析了2001年5月至2010年4月期间成人原发性肾活检记录,在总共1753份记录中,经最终组织病理学分析确定有57例被标记为IgMN。其中,41例纳入本分析。从病例档案和活检报告中收集了他们的相关数据项。

结果

该队列的平均年龄为30.21±10.12岁。男女比例为1.15:1。最常见的表现是特发性肾病综合征。分别有24例(58.5%)和10例(24.4%)患者在就诊时出现血尿和高血压。最常见的形态学改变是肾小球系膜细胞增生,在28份活检标本中发现(68.3%)。16例(39%)出现系膜基质扩张。5例(12.2%)有轻微肾小球改变,4例(9.8%)有局灶节段性肾小球硬化。免疫荧光显微镜检查显示所有标本中IgM呈弥漫性系膜阳性。6例(14.6%)有亚优势IgA。分别在28例(68.3%)和21例(51.2%)患者中发现补体C3和C1q。

结论

我们的数据表明,IgMN在成年人中并不常见。其临床病理谱与邻国描述的相似,显示出从轻微改变到局灶节段性肾小球硬化的一系列形态学变化。

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