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儿童肾病综合征中非硬化性肾小球的超微结构

Ultrastructure of the non-sclerotic glomeruli in childhood nephrotic syndrome.

作者信息

Yoshikawa N, Cameron A H, White R H

出版信息

J Pathol. 1982 Feb;136(2):133-47. doi: 10.1002/path.1711360207.

Abstract

An electron-microscopic (EM) study of the non-sclerotic glomeruli was made in 96 children with the nephrotic syndrome in whom light microscopy had shown minimal change, focal global glomerulosclerosis or segmental glomerulosclerosis. EM showed a variety of alterations. Foot process fusion, duplication and crenation of the lamina densa, and granular and lucent expansion of lamina rara interna were noted in almost all patients in all three groups. Localised ulceration of the podocytes was noted in some patients in each group. There was no difference in the mean thickness of the glomerular basal lamina but there was an increase with age in minimal change. Curious extracellular curved striated bodies, clusters of electron-dense, round microparticles and microfilaments were found in all three, but most frequently in segmental glomerular sclerosis. Electron-dense deposits were seen in all but one of the patients with segmental glomerular sclerosis and usually involved the capillary wall. They were seen in one third of those with minimal change and focal glomerular sclerosis but rarely in the capillary wall. There were no specific features which distinguished segmental glomerular sclerosis although the various types of deposits were more extensive and more frequent than in minimal change and focal glomerular sclerosis. These observations are consistent with common pathogenetic factors operating at different intensities in segmental glomerular sclerosis, focal glomerular sclerosis and minimal change.

摘要

对96例肾病综合征患儿的非硬化性肾小球进行了电子显微镜(EM)研究,这些患儿的光学显微镜检查显示为微小病变、局灶性球性肾小球硬化或节段性肾小球硬化。电子显微镜显示出多种改变。在所有三组的几乎所有患者中均观察到足突融合、致密层重复和皱缩,以及内疏松层颗粒状和透亮性扩张。每组中的一些患者存在足细胞局部溃疡。肾小球基底膜的平均厚度无差异,但微小病变患者随年龄增加而增厚。在所有三组中均发现了奇怪的细胞外弯曲条纹体、电子致密圆形微粒簇和微丝,但在节段性肾小球硬化中最为常见。除1例节段性肾小球硬化患者外,其余患者均可见电子致密沉积物,且通常累及毛细血管壁。在三分之一的微小病变和局灶性肾小球硬化患者中可见电子致密沉积物,但很少累及毛细血管壁。尽管与微小病变和局灶性肾小球硬化相比,节段性肾小球硬化中各种类型的沉积物更广泛、更频繁,但没有区分节段性肾小球硬化的特异性特征。这些观察结果与在节段性肾小球硬化、局灶性肾小球硬化和微小病变中以不同强度起作用的共同致病因素一致。

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