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儿童IgA肾病节段性簇状病变的结构——三维分析表明节段性病变的发展

[Structure of segmental tuft lesions in childhood IgA nephropathy--three dimensional analysis indicates development of segmental lesions].

作者信息

Akioka Y

机构信息

Department of Pediatric Nephrology, Tokyo Women's Medical College, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 1994 Sep;36(9):973-81.

PMID:7967181
Abstract

The mechanisms responsible for the formation and development of segmental lesions of IgA nephropathy in children were studied by analysis of three dimensional reconstruction. Forty-eight segmental lesions from 15 cases (diffuse proliferative glomerulonephritis (DPGN) in 13 cases, focal glomerulonephritis (FGN) in 2 cases) were examined by light microscopy by analysis of serial sections (in average 26 sections/glomerulus). In tuft, three types of tuft lesions were defined by their chronisity: 1) endocapillary proliferation including exudative changes, 2) mesangial hypercellularity, 3) deposition of mesangial matrix with sclerosis. Extra-capillary lesions, namely crescent were also defined by their chronisity: 1) cellular, 2) fibrocellular, 3) fibrous. The structural relation in each tuft and endocapillary lesions were observed. Endocapillary proliferative lesions of tufts were closely associated with cellular crescent of extra-capillary lesions. In fibrocellular and fibrous crescents, the frequency of endocapillary proliferation in tufts were reduced, while the association of mesangial proliferation and sclerosis were increased. Nevertheless, endocapillary proliferation of tufts were still observed in 33% of fibrous crescent. We concluded that segmental lesions were originated by endocapillary tuft lesions leading to cellular crescents. The multifocal and repeated attack of endocapillary proliferative lesions within segmental sclerosis promotes further development of glomerular sclerosis in IgA nephropathy in children.

摘要

通过三维重建分析,研究了儿童IgA肾病节段性病变形成和发展的机制。对15例患者的48个节段性病变(13例弥漫性增生性肾小球肾炎(DPGN),2例局灶性肾小球肾炎(FGN))进行了光镜检查,通过连续切片分析(平均每个肾小球26个切片)。在肾小球毛细血管襻中,根据病变的慢性程度定义了三种类型的襻病变:1)包括渗出性改变的毛细血管内增生,2)系膜细胞增多,3)伴有硬化的系膜基质沉积。毛细血管外病变,即新月体,也根据其慢性程度进行了定义:1)细胞性,2)纤维细胞性,3)纤维性。观察了每个肾小球襻和毛细血管内病变的结构关系。肾小球襻的毛细血管内增生性病变与毛细血管外病变的细胞性新月体密切相关。在纤维细胞性和纤维性新月体中,肾小球襻中毛细血管内增生的频率降低,而系膜增生和硬化的相关性增加。然而,在33%的纤维性新月体中仍观察到肾小球襻的毛细血管内增生。我们得出结论,节段性病变起源于毛细血管内肾小球襻病变,导致细胞性新月体形成。节段性硬化内毛细血管内增生性病变的多灶性和反复攻击促进了儿童IgA肾病肾小球硬化的进一步发展。

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