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淀粉样肾病的肾脏受累模式:与糖尿病肾病的比较。

Renal involvement patterns of amyloid nephropathy: a comparison with diabetic nephropathy.

作者信息

Nakamoto Y, Hamanaka S, Akihama T, Miura A B, Uesaka Y

出版信息

Clin Nephrol. 1984 Oct;22(4):188-94.

PMID:6509804
Abstract

Thirty kidney specimens obtained from 26 patients with intrarenal amyloid deposition were evaluated. The deposited amyloid belonged to AL protein in 15 cases and AA protein in 11. The intrarenal distribution was examined. Intraglomerular nodular pattern showed a wider extraglomerular distribution of amyloid with a florid accumulation along the vasa recta in the outer medulla, whereas intraglomerular diffuse pattern had amyloid deposition confined mainly to a narrow range surrounding the glomeruli. However, no essential difference was appreciated between AL and AA proteins in the intrarenal distribution, although the former more often disclosed the diffuse pattern. There were many morphological expressions resembling diabetic nephropathy; in addition to the diffuse and nodular pictures, capsular drop-like deposits along Bowman's capsule, simultaneous deposition in the afferent and efferent arterioles, glomerular microaneurysm of early stage, and accumulation along the tubular basement membrane were observed in amyloid nephropathy. Thus, the spicule formation in renal amyloidosis and the diffuse thickening of glomerular and tubular basement membranes in the diabetic emerged as the respective specific lesion. The implicated pathogenesis of diabetic nephropathy was also discussed.

摘要

对从26例肾内淀粉样蛋白沉积患者获取的30份肾脏标本进行了评估。15例中沉积的淀粉样蛋白属于AL蛋白,11例属于AA蛋白。检查了肾内分布情况。肾小球内结节样模式显示淀粉样蛋白在肾小球外分布更广,在外髓质沿直小血管有大量积聚,而肾小球内弥漫性模式的淀粉样蛋白沉积主要局限于肾小球周围的狭窄区域。然而,尽管AL蛋白更常表现为弥漫性模式,但在肾内分布方面,AL蛋白和AA蛋白之间未发现本质差异。有许多形态学表现类似于糖尿病肾病;除了弥漫性和结节样表现外,在淀粉样肾病中还观察到沿鲍曼囊的包膜滴状沉积物、入球小动脉和出球小动脉同时沉积、早期肾小球微动脉瘤以及沿肾小管基底膜的积聚。因此,肾淀粉样变性中的针状形成以及糖尿病中肾小球和肾小管基底膜的弥漫性增厚分别成为各自的特异性病变。还讨论了糖尿病肾病的相关发病机制。

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