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与克劳-深泽综合征相关的肾小球病变。

Glomerular lesions associated with the Crow-Fukase syndrome.

作者信息

Sano M, Terasaki T, Koyama A, Narita M, Tojo S

出版信息

Virchows Arch A Pathol Anat Histopathol. 1986;409(1):3-9. doi: 10.1007/BF00705402.

Abstract

Three cases of the Crow-Fukase syndrome without radiographic changes of multiple myeloma are reported, with special reference to the glomerular changes seen. Proteinuria was detected in one case, although decreased renal function was observed in all (GFR: 41.0, 62.0, 74.1 ml/min respectively) at the time of renal biopsy. Glomerular changes were similar in all three cases. The main characteristic changes were mesangial proliferation and thickening of the glomerular capillary walls. Pictures by light microscopy were therefore similar to that of MPGN. On electron microscopy, the thickened capillary walls showed circumferential mesangial interposition and the subendothelial zone was electron-lucent and contained small dense granules or flocculent deposits. By immunofluorescent microscopy, no immunoglobulins, complement components or light chain were detected in the glomeruli except in one case.

摘要

报告了3例无多发性骨髓瘤影像学改变的Crow-Fukase综合征病例,并特别提及所观察到的肾小球变化。1例检测到蛋白尿,尽管在肾活检时所有病例均观察到肾功能下降(肾小球滤过率分别为41.0、62.0、74.1 ml/min)。所有3例的肾小球变化相似。主要特征性变化为系膜增生和肾小球毛细血管壁增厚。因此,光镜下的表现与膜增生性肾小球肾炎相似。电镜下,增厚的毛细血管壁可见系膜沿周边插入,内皮下区域电子密度减低,含有小的致密颗粒或絮状沉积物。免疫荧光显微镜检查显示,除1例病例外,肾小球内未检测到免疫球蛋白、补体成分或轻链。

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