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[肾病患儿的玻连蛋白——1. 儿童IgA肾病中玻连蛋白和C5b-9的免疫荧光研究]

[Vitronectin in children with renal disease--1. Immunofluorescence study of vitronectin and C5b-9 in childhood IgA nephropathy].

作者信息

Takahashi T, Inaba S, Okada T

机构信息

Department of Pediatrics, Faculty of Medicine, Toyama Medical & Pharmaceutical University, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 1995 Apr;37(4):213-23.

PMID:7541480
Abstract

Renal biopsy specimens from 54 children (33 boys, 21 girls) with IgA nephropathy were investigated by immunofluorescence microscopy to determine the localization of vitronectin and C5b-9. vitronectin and C5b-9 deposits were found within the glomeruli of all cases and the intensity of vitronectin and C5b-9 deposits was as follows: vitronectin: (3+) 17 cases, (2+) 21 cases, (1+) 16 cases and C5b-9: (3+) 28 cases, (2+) 18 cases, (1+) 8 cases. In 19 cases and C5b-9 deposits were shown to be more predominant than vitronectin (Group C) and the same or less intense in 35 cases (Group V). In 33 cases, vitronectin deposits were demonstrated in the mesangial area only (M type), while in 21 cases deposits were presented both the mesangial area and the capillary wall (MC type). Linear staining of vitronectin was noted in Bowman's basement membrane and in the tubular basement membrane. Granular staining of vitronectin was observed in Bowman's basement membrane (29 cases) and tubular basement membrane (46 cases). M type C5b-9 deposits were demonstrated in 34 cases and MC type in 20 cases. C5b-9 deposits were observed in Bowman's basement membrane (19 cases) and in tubular basement membrane (37 cases). Intensely stained vitronectin was found in the sclerotic glomeruli and segmental sclerotic portion of the tissues. In serial biopsy, the intensity of both vitronectin and C5b-9 deposits became less intense. It was found that many patients of Group C had heavy proteinuria (P < 0.05) and that nephrotic patients were more frequent in Group C than in Group V (p < 0.05). In the light microscopic examination, most cases with intense C5b-9 deposits had mesangial proliferation (p < 0.10) and an increase in mesangial matrix (p < 0.10). Mesangial sclerosis index was correlated with the intensity of vitronectin deposits (p < 0.05). These findings suggest that vitronectin and the terminal complement system may play a critical role as a mediator of immunological tissue injury, especially mesangial sclerotic lesion in childhood IgA nephropathy.

摘要

对54例IgA肾病患儿(33例男孩,21例女孩)的肾活检标本进行免疫荧光显微镜检查,以确定玻连蛋白和C5b - 9的定位。在所有病例的肾小球内均发现玻连蛋白和C5b - 9沉积,玻连蛋白和C5b - 9沉积强度如下:玻连蛋白:(3 +)17例,(2 +)21例,(1 +)16例;C5b - 9:(3 +)28例,(2 +)18例,(1 +)8例。19例中C5b - 9沉积比玻连蛋白更显著(C组),35例中两者相同或C5b - 9沉积强度较弱(V组)。33例中,玻连蛋白沉积仅见于系膜区(M型),21例中沉积见于系膜区和毛细血管壁(MC型)。在鲍曼囊基底膜和肾小管基底膜可见玻连蛋白线性染色。在鲍曼囊基底膜(29例)和肾小管基底膜(46例)观察到玻连蛋白颗粒状染色。34例显示为M型C5b - 9沉积,20例为MC型。在鲍曼囊基底膜(19例)和肾小管基底膜(37例)观察到C5b - 9沉积。在硬化的肾小球和组织的节段性硬化部分发现玻连蛋白染色强烈。在系列活检中,玻连蛋白和C5b - 9沉积强度均减弱。发现C组许多患者有大量蛋白尿(P < 0.05),且C组肾病患者比V组更常见(p < 0.05)。在光镜检查中,大多数C5b - 9沉积强烈的病例有系膜增生(p < 0.10)和系膜基质增加(p < 0.10)。系膜硬化指数与玻连蛋白沉积强度相关(p < 0.05)。这些发现表明,玻连蛋白和终末补体系统可能作为免疫性组织损伤的介质发挥关键作用,尤其是在儿童IgA肾病的系膜硬化病变中。

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