Yamada Shunsuke, Masutani Kohsuke, Katafuchi Ritsuko, Fujigaki Yoshihide, Muranaka Yoshinori, Tsuruya Kazuhiko, Iida Mitsuo
Department of Medicine and Clinical Science, Kyushu University, Higashi-ku, Fukuoka, 812-8582, Japan.
Clin Exp Nephrol. 2008 Dec;12(6):509-12. doi: 10.1007/s10157-008-0088-8. Epub 2008 Nov 1.
A 42-year-old woman was admitted to Kyushu University hospital because of 6 months' history of bilateral leg edema. Upon admission, ascites and pleural effusion as well as systemic edema were noted. Laboratory tests revealed hypoalbuminemia of 1.5 g/dl and massive proteinuria of 10 g/day. She was diagnosed with nephrotic syndrome. Renal biopsy revealed diffuse thickening of the glomerular basement membrane (GBM) and a crescent-like extracapillary lesion with segmental sclerosis in four of 11 glomeruli. Immunoglobulins and complements were negative by immunofluorescence examination. Therefore, we diagnosed this as focal segmental glomerulosclerosis (FSGS) rather than membranous nephropathy. Using an electron microscope, we observed a thickening of the GBM with numerous intramembranous vesicle-like microstructures and an infolding of the podocyte into the GBM. Since the microstructures were partly demarcated by a unit membrane and some of them were located very closely to the infolded podocyte, we speculated that the microstructures were derived from the podocyte. The unique electron microscopic finding of our case is a disease entity rather than a reactive phenomenon.
一名42岁女性因双侧腿部水肿6个月入住九州大学医院。入院时,发现有腹水、胸腔积液以及全身性水肿。实验室检查显示血清白蛋白水平低至1.5g/dl,蛋白尿大量增加,达每日10g。她被诊断为肾病综合征。肾活检显示肾小球基底膜(GBM)弥漫性增厚,11个肾小球中有4个出现类似新月体的球外病变伴节段性硬化。免疫荧光检查显示免疫球蛋白和补体均为阴性。因此,我们将此诊断为局灶节段性肾小球硬化(FSGS)而非膜性肾病。通过电子显微镜观察,我们发现GBM增厚,伴有大量膜内囊泡样微结构,足细胞向GBM内折叠。由于这些微结构部分由单位膜分隔,其中一些与折叠的足细胞位置非常接近,我们推测这些微结构源自足细胞。我们病例独特的电子显微镜发现是一种疾病实体而非反应性现象。