Department of Nephrology and Dialysis, Akita Kumiai General Hospital, Akita, Japan.
Nephrol Dial Transplant. 2010 Jun;25(6):2016-9. doi: 10.1093/ndt/gfq037. Epub 2010 Feb 10.
A 68-year-old man developed proteinuria and renal insufficiency. A renal biopsy showed mesangial proliferation and double contour in almost all glomeruli. Congo red staining for amyloid was negative. Immunofluorescence microscopy revealed no deposition of immunoglobulins. Electron microscopy showed unusual deposits of striated structures mainly in the subendothelial space and the mesangium. These deposits contained regularly stacked straight electron-dense bands. Microfilament-like deposits were also observed. The patient did not respond to steroid therapy and developed end-stage renal disease. All known disease entities with non-amyloid non-immunoglobulin-derived organized glomerular deposits were excluded. Progressive glomerulopathy in our patient might be a new disease entity.
一位 68 岁男性出现蛋白尿和肾功能不全。肾活检显示几乎所有肾小球均有系膜增生和双轨征。刚果红染色检查淀粉样物质为阴性。免疫荧光显微镜检查未发现免疫球蛋白沉积。电子显微镜检查显示在肾小球的内皮下和系膜区有特殊的条纹状结构沉积。这些沉积物含有规则排列的电子致密带。也观察到微丝样沉积物。该患者对类固醇治疗无反应，并发展为终末期肾病。所有已知的伴有非淀粉样、非免疫球蛋白来源的有组织的肾小球沉积的疾病实体均被排除。我们的患者的进行性肾小球病可能是一种新的疾病实体。
