Katafuchi R, Taguchi T, Takebayashi S, Harada T
Clin Nephrol. 1984 Jul;22(1):1-8.
To obtain a better understanding of the mechanism of proteinuria in amyloidosis we investigated the ultrastructural distribution and pattern of arrangement of glomerular amyloid fibrils in 15 patients. Clinical data were correlated with the ultrastructural features and with the morphometric indices obtained from composite electron micrographs of whole glomeruli. The epithelial side of amyloid deposits showed spicules (discrete pointed bundles of fibrils) and apparent fraying which we termed tufts (ill-defined and diffuse expansion of fibrils). The extent of mesangial amyloid deposition and total GBM deposition did not correlate with proteinuria. Current data suggest the importance of partial detachment of epithelial cells in pathogenesis of proteinuria, which in turn correlates with distortion of amyloid fibrils, spicules and tufts.
为了更好地理解淀粉样变性中蛋白尿的机制，我们研究了15例患者肾小球淀粉样纤维的超微结构分布和排列模式。临床数据与超微结构特征以及从整个肾小球的复合电子显微照片获得的形态计量指标相关。淀粉样沉积物的上皮侧显示出针状体（离散的尖状纤维束）和明显的磨损，我们称之为簇（纤维的不明确和弥漫性扩张）。系膜淀粉样沉积程度和肾小球基底膜总沉积与蛋白尿无关。目前的数据表明上皮细胞部分脱离在蛋白尿发病机制中的重要性，这反过来又与淀粉样纤维、针状体和簇的变形相关。
