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IgM相关性原发性弥漫性系膜增生性肾小球肾炎

IgM associated primary diffuse mesangial proliferative glomerulonephritis.

作者信息

Lawler W, Williams G, Tarpey P, Mallick N P

出版信息

J Clin Pathol. 1980 Nov;33(11):1029-38. doi: 10.1136/jcp.33.11.1029.

Abstract

Twenty-three cases of IgM associated primary diffuse mesangial proliferative glomerulonephritis are presented. In 18, IgM was the sole localising host immunoglobulin, and it was the predominant globulin in five; C3 was also present in 18. Light microscopy revealed variable diffuse and global mesangial proliferation in all cases, with additional focal global sclerosis in 16, focal segmental sclerosis in 15, and small capsular crescents in seven. Material for electron microscopy was available from 19 patients; in 13, occasional intramesangial electron dense deposits were identified, and in 18 there were irregular, rather ill defined areas of increased electron density in mesangial regions. Clinically, 14 patients presented with the nephrotic syndrome, and nine had asymptomatic proteinuria. During follow-up, only 10 patients showed no change in renal function or improved; the remainder showed increasing hypertension and/or renal function deterioration and four developed end stage renal failure. It is suggested that IgM associated mesangial proliferative glomerulonephritis should be considered as a distinct clinicoimmunopathological entity.

摘要

本文报告了23例IgM相关性原发性弥漫性系膜增生性肾小球肾炎。18例中,IgM是唯一定位的宿主免疫球蛋白,5例中它是主要球蛋白;18例中也存在C3。光镜检查显示所有病例均有不同程度的弥漫性和全球性系膜增生,16例伴有局灶性全球性硬化,15例伴有局灶节段性硬化,7例伴有小新月体。19例患者有电子显微镜检查材料;13例中偶尔发现系膜内电子致密沉积物,18例系膜区域有不规则、界限不清的电子密度增加区域。临床上,14例患者表现为肾病综合征,9例有无症状蛋白尿。随访期间,只有10例患者肾功能无变化或改善;其余患者血压升高和/或肾功能恶化,4例发展为终末期肾衰竭。提示IgM相关性系膜增生性肾小球肾炎应被视为一种独特的临床免疫病理实体。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cb45/1146333/a0f73dcc6d4a/jclinpath00471-0014-a.jpg

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