Progressive glomerulopathy with unusual deposits of striated structures: a new disease entity?
Progressive glomerulopathy with unusual deposits of striated structures: a new disease entity?
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DOI：
10.1093/ndt/gfq037
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发表时间：
2010-06-01
影响因子：
6.1
通讯作者：
Sawada, Ken-ichi
中科院分区：
文献类型：
--
作者：
Ohtani, Hiroshi;Wakui, Hideki;Sawada, Ken-ichi
一名68岁的男子出现蛋白尿和肾功能不全。肾活检显示系膜增生和双轮廓几乎所有的肾小球。淀粉样蛋白刚果红染色阴性。免疫荧光显微镜显示没有免疫球蛋白沉积。电子显微镜显示不寻常的沉积物的条纹状结构，主要是在血管内皮下的空间和系膜。这些沉积物含有规则堆叠的直电子致密带。还观察到微生物学样沉积物。患者对类固醇治疗无反应，并发展为终末期肾病。排除所有已知的非淀粉样蛋白非免疫球蛋白衍生的有组织肾小球沉积物疾病。进行性肾小球病在我们的病人可能是一个新的疾病实体。
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.