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非淀粉样变性纤维性肾小球病、免疫触须样肾小球病及丝状肾小球病的鉴别诊断

Nonamyloidotic fibrillary glomerulopathy, immunotactoid glomerulopathy, and the differential diagnosis of filamentous glomerulopathies.

作者信息

Devaney K, Sabnis S G, Antonovych T T

机构信息

Department of Genitourinary Pathology, Armed Forces Institute of Pathology, Washington, DC.

出版信息

Mod Pathol. 1991 Jan;4(1):36-45.

PMID:2020659
Abstract

Kidney biopsies from 12 patients between the ages of 10 and 63 yr were diagnosed as nonamyloidotic fibrillary glomerulopathy (NAFG) or immunotactoid glomerulopathy (IG) on the basis of the electron microscopic finding of filamentous or tubular material within the glomerular capillaries and mesangium. Six patients were male and six were female. Eleven presented with nephrotic syndrome and one with acute renal failure. Eight were hypertensive, and four of these patients had gross or microscopic hematuria as well. Biopsies from 11 patients were Congo red negative; one was weakly positive. By light microscopy, the predominant glomerular change was thickening of the capillary basement membrane with or without widening of the mesangium; these changes were suggestive of membranous glomerulonephritis. Immunofluorescent studies performed in four of the cases were positive for immunoglobulin G (IgG). Immunoperoxidase staining for beta 2-microglobulin was negative in four patients. Ultrastructurally, filaments or tubules were identified in the glomerular capillary basement membrane and/or mesangium in each patient. The filaments in NAFG, IG, amyloidosis, and other paraprotein deposits can be differentiated by size, arrangement, and location of filamentous material.

摘要

对年龄在10至63岁之间的12名患者的肾活检组织进行检查,基于在肾小球毛细血管和系膜内发现丝状或管状物质的电镜检查结果,诊断为非淀粉样纤维性肾小球病(NAFG)或免疫触须样肾小球病(IG)。6例为男性,6例为女性。11例表现为肾病综合征,1例表现为急性肾衰竭。8例有高血压,其中4例患者还伴有肉眼血尿或镜下血尿。11例患者的活检组织刚果红染色阴性;1例弱阳性。光镜下,肾小球的主要变化是毛细血管基底膜增厚,伴或不伴系膜增宽;这些变化提示膜性肾小球肾炎。4例病例进行的免疫荧光研究显示免疫球蛋白G(IgG)阳性。4例患者β2微球蛋白免疫过氧化物酶染色阴性。超微结构上,在每位患者的肾小球毛细血管基底膜和/或系膜中均发现了细丝或小管。NAFG、IG、淀粉样变性和其他副蛋白沉积物中的细丝可通过丝状物质的大小、排列和位置进行区分。

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Nonamyloidotic fibrillary glomerulopathy, immunotactoid glomerulopathy, and the differential diagnosis of filamentous glomerulopathies.非淀粉样变性纤维性肾小球病、免疫触须样肾小球病及丝状肾小球病的鉴别诊断
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