【摘要】 目的:探讨肾淀粉样变性的病理特点,为临床诊断提供依据。方法:对2例肾病综合征患者行经皮肾穿刺活检术,所取组织分别进行组织学、免疫组织化学以及电镜检查。结果:光镜下观察结果:肾小球系膜区呈结节样改变,系膜区及内皮下见大量HE淡染的均质无细胞性物质沉积,间质小动脉管壁增厚,管壁内亦见HE淡染的均质沉积。刚果红及高锰酸钾刚果红染色均显示:肾小球系膜区、间质、间质动脉管壁阳性。免疫组织化学检测结果:2例IgG(±)、IgM(±)、IgA(-);1例C3c(-)、C4c(-)、C1q(-),1例C3c(±)、C4c(-)、C1q(-)。电镜下观察结果:肾小球系膜区无分支、紊乱排列的细纤维状结构。结论:淀粉样物质沉积于肾小球系膜区、间质及间质小动脉,使肾小球系膜区增宽,间质小动脉管壁增厚,刚果红染色阳性,高锰酸钾刚果红抵抗是肾淀粉样变性典型的病理特点。更多还原

【Abstract】 Objective: To investigate the pathological features of renal amyloidosis（AL） and provide diagnostic basis for AL via analyzing clinical cases.Methods: Renal biopsy was applied to 2 patients with nephrotic syndrome and routine stains such as haematoxylin and eosin（HE）,Congo red stain,KMnO₄ Congo red stain and immunohistochemistry were prepared for specimen.Electron microscopy was also used to observation.Results: Light microscopy results showed that there were nodal alterations in glomerular mesangium region and plenty of irregular hypochromatic HE deposits were seen in the mesangium and sub-endothelium region.Arteriole wall thickening and HE deposits were observed in the tubulointerstitium,Congo-red stains proved to be positive in the mesangium,interstitium and interstitial arterioles and further KMnO₄ Congo Red was still positive.Immunohistology results showed: 2 patients IgG（±）,IgM（±）,IgA（-）,1 patient C3c（-）,C4c（-）,C1q（-）,another patient C3c（±）,C4c（-）,C1q（-）.Characteristic fibrillary deposits of amyloid were found on electron microscopy.Conclusions: Our findings suggest（assertive） pathological changes in amyloidosis should include amyloid substance deposits in the mesangium,interstitium and interstitial arterioles,which result in the mesangial widening with arterial wall hypertrophy.A positive Congo red stain and a further KMnO₄ Congo red resistance are essential for early diagnosis of amyloidosis.更多还原