Suzuki S, Maruyama Y, Nakamura T, Ueno M, Nishi S, Ooshima A, Isemura M, Arakawa M
Department of Medicine II, Niigata University School of Medicine, Japan.
Nephron. 1994;66(4):462-9. doi: 10.1159/000187865.
A 35-year-old male patient clinically characterized by massive proteinuria and hypertension without evidence of systemic diseases is reported. Histological investigation of renal biopsy specimens revealed extensive nodular formations in the mesangial areas in every glomerulus. Light-microscopic examination did not allow discrimination between the glomerular changes found in these specimens and the nodular glomerulosclerosis described in patients with diabetes mellitus. Electron-microscopic examination confirmed the presence of massive, nodular, mesangial expansions consisting of finely fibrillar substances without electron-dense deposits and circumferential mesangial interposition. Immunofluorescent examination showed deposition of IgG, C3, fibrinogen and kappa and lambda light chains in mesangial areas, peripheral capillary loops and a part of the nodules. Furthermore, collagen types IV, V, VI and laminin were detected in the nodules. Amyloid was not observed in these nodules. This diagnosis has not been made, and the mechanism of this nodular glomerulosclerosis remains unknown.
报告了一名35岁男性患者，临床表现为大量蛋白尿和高血压，无全身性疾病证据。肾活检标本的组织学检查显示每个肾小球的系膜区有广泛的结节形成。光镜检查无法区分这些标本中发现的肾小球变化与糖尿病患者中描述的结节性肾小球硬化。电镜检查证实存在由细纤维状物质组成的大量、结节状、系膜扩张，无电子致密沉积物和系膜周围插入。免疫荧光检查显示IgG、C3、纤维蛋白原以及κ和λ轻链在系膜区、外周毛细血管袢和部分结节中沉积。此外，在结节中检测到IV型、V型、VI型胶原和层粘连蛋白。这些结节中未观察到淀粉样物质。尚未做出此诊断，这种结节性肾小球硬化的机制仍然未知。
