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继发性系统性淀粉样变A合并原发性肾小球肾炎及系统性疾病

[Secondary systemic amyloidosis A combined with primary glomerulonephritis and systemic diseases].

作者信息

Bogov B, Kiperova B, Dzherasi R, Andreev E, Minkova V

出版信息

Vutr Boles. 1999;31(1):19-22.

Abstract

Amyloidosis is characterized by organic dysfunction as a result of deposition of amyloid substance in the walls of the small blood vessels and extracellularly in different organs. The involvement of the kidneys in systemic amyloidoses AL and AA has irreversible evolution to renal failure. The object of the study was to determine the prevalence of the secondary (reactive) systemic amyloidosis AA in combination with primary glomerulonephritis (PGN) and lupus nephropathy (SLE) and to create diagnostic approach for its early detection. The prevalence of amyloidosis among the renal biopsies in the Department of Nephrology by the Chair of Internal Diseases for the period 1981-1988, retrospectively, is 4% (in 11 out of 268 biopsies). For the period 1989-1996, prospectively, by directed quest, amyloid was found in 35 out of 269 renal biopsies (11%). For differentiation of AA preliminary treatment of the histologic material with KMnO4 was used. In 20 cases amyloidosis appeared as independent finding in the renal tissue, while in 15 it was combined with histologic picture of immune nephropathies: in 11 with primary glomerulonephritis (7% out of 155 PGN) and in 4 with systemic lupus erythematodes (11% out of 31 SLE). The combination of PGN with AA was almost always associated with chronic infections. It was most often observed in diffuse membranous GN or FSGSH. Our studies demonstrate increased prevalence of amyloidosis among the renal biopsies during the last years, which could be due to directed quest, but it could be a real fact, too. We suggest staining for amyloid in all renal biopsies, as well as its directed quest in mucosae of the gastro-intestinal tract and by aspiration of the abdominal subcutaneous fatty tissue in the patients with primary GN or systemic diseases.

摘要

淀粉样变性的特征是由于淀粉样物质沉积在小血管壁和不同器官的细胞外而导致器官功能障碍。系统性淀粉样变性AL和AA累及肾脏会不可逆转地发展为肾衰竭。本研究的目的是确定继发性(反应性)系统性淀粉样变性AA与原发性肾小球肾炎(PGN)和狼疮性肾炎(SLE)合并存在的患病率,并创建早期检测的诊断方法。回顾性研究1981 - 1988年期间内科疾病教研室肾脏病科肾活检中淀粉样变性的患病率为4%(268例活检中有11例)。前瞻性研究1989 - 1996年期间,通过定向询问,在269例肾活检中有35例发现淀粉样物质(11%)。为鉴别AA,使用KMnO4对组织学材料进行预处理。在20例中,淀粉样变性在肾组织中表现为独立发现,而在15例中,它与免疫性肾病的组织学表现合并存在:11例与原发性肾小球肾炎合并(155例PGN中的7%),4例与系统性红斑狼疮合并(31例SLE中的11%)。PGN与AA的合并几乎总是与慢性感染有关。最常见于弥漫性膜性肾小球肾炎或FSGSH。我们的研究表明,近年来肾活检中淀粉样变性的患病率有所增加,这可能是由于定向询问,但也可能是一个实际情况。我们建议对所有肾活检进行淀粉样物质染色,并对原发性GN或全身性疾病患者的胃肠道黏膜和通过抽吸腹部皮下脂肪组织进行定向询问。

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