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[早期肾淀粉样变性的临床病理特征]

[The clinicopathological features of early renal amyloidosis].

作者信息

Wang Su-xia, Zou Wan-zhong, Wang Mei, E Jie, Wang Shu-he, Tang Xiu-ying

机构信息

Department of Electron Microscopy, Peking University First Hospital, Beijing 100034, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2003 Apr;32(2):120-3.

Abstract

OBJECTIVE

To investigate the clinicopathological manifestations of early renal amyloidosis (AL) and its diagnostic criteria.

METHODS

Fifteen cases with early renal amyloidosis admitted from 1994 to 2001 were collected from the hospital, and their clinical and pathological features were reviewed. Of them, the initial diagnoses were not made by depending findings from the light microscopy (LM) and immunofluorescense (IF), but confirmed by electron microscopy (EM) afterwards. Immuno-electron microscopy (IEM) were applied for amyloidosis typing.

RESULTS

Most patients of early renal AL were in the middle to old age. Nephrotic syndrome was the most prominent symptoms and signs accompanying with rare microscopic hematuria and hypertension. Most of them had a normal renal function. Pathological examinations of renal biopsies using LM and IF showed mild mesangial proliferation and mild thickening of glomerular basement membrane (GBM). Immunoglobulins and complements were negative or only scanty in certain cases, but in all cases there was a light chain protein deposition homogeously. There were 4 cases of minimal change glomerulopathy, 5 cases of mild mesangial proliferative glomerulonephritis, 5 cases of stage I membranous nephropathy, and 1 case of cast nephropathy diagnosed with LM. The amyloid fibrils (diameter 8 - 10 nm) were randomly distributed in the mesangium, along GBM and at the arteriolar wall under EM. Additionally, Congo red staining was positive. IEM demonstrated that amyloid fibrils labeled with colloid gold was combined with a kind of light chain protein which was confirmed as the light chain type of AL.

CONCLUSIONS

The diagnosis of early renal AL was occasionally neglected by depending only findings of LM and LF. However, special amyloid fibrils can be detected using EM. EM observation is an indispensable technique for the diagnosis of early renal AL and the typing of AL may further be determined by using IEM.

摘要

目的

探讨早期肾淀粉样变性(AL型)的临床病理表现及其诊断标准。

方法

收集1994年至2001年收治的15例早期肾淀粉样变性患者,回顾其临床和病理特征。其中,最初诊断并非依赖光镜(LM)和免疫荧光(IF)检查结果,而是随后经电子显微镜(EM)确诊。采用免疫电子显微镜(IEM)进行淀粉样变性分型。

结果

早期肾AL型患者多为中老年人。肾病综合征是最突出的症状和体征,伴有罕见的镜下血尿和高血压。大多数患者肾功能正常。肾活检的LM和IF病理检查显示轻度系膜增生和肾小球基底膜(GBM)轻度增厚。免疫球蛋白和补体在某些病例中呈阴性或仅少量存在,但所有病例均有轻链蛋白均匀沉积。经LM诊断为微小病变性肾小球病4例,轻度系膜增生性肾小球肾炎5例,Ⅰ期膜性肾病5例,管型肾病1例。EM下淀粉样纤维(直径8 - 10 nm)随机分布于系膜、沿GBM及小动脉壁。此外,刚果红染色呈阳性。IEM显示胶体金标记的淀粉样纤维与一种轻链蛋白结合,证实为AL型轻链。

结论

仅依靠LM和LF的检查结果,早期肾AL型的诊断偶尔会被忽视。然而,使用EM可检测到特殊的淀粉样纤维。EM观察是早期肾AL型诊断不可或缺的技术,且使用IEM可进一步确定AL的类型。

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