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| Fabry病肾病临床病理分析 | |
| 引用本文： | 张明辉,刘艳辉,史伟,罗东兰,骆新兰,姚军,庄恒国. Fabry病肾病临床病理分析[J]. 临床与实验病理学杂志, 2010, 26(2) |
| 作者姓名： | 张明辉 刘艳辉 史伟 罗东兰 骆新兰 姚军 庄恒国 |
| 作者单位： | 1. 广东省医学科学院/广东省人民医院病理医学部病理科,广州,510080 2. 广东省医学科学院/广东省人民医院肾内科,广州,510080 |
| 摘 要： | 目的阐明具有肾脏损害的Fabry病的临床病理特点。方法回顾性分析3例具有肾损害的Fabry病患者的临床表现,并对其肾活检组织进行免疫荧光、光镜及超微结构观察。结果3例患者均出现血尿、蛋白尿;光镜下肾小球足细胞体积增大,空泡变性,肾小管上皮也可出现上述改变;电镜下肾小球足细胞胞质内可见大量嗜锇性髓鞘样包涵体—圆形或卵圆形,明暗相间呈板层状,大小为0.3～10μm,外有单位膜包绕。结论具有肾脏损害的Fabry病患者临床上可出现蛋白尿、血尿,晚期常发展为肾功能衰竭。肾小球足细胞胞质内出现嗜锇性髓鞘样包涵体是Fabry病肾病特征性的形态学改变。 |
| 关 键 词： | Fabry病 α-半乳糖苷酶A 肾活检 超微结构 |
Renal involvement in Fabry disease:a clinicopathological analysis of 3 cases |
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| ZHANG Ming-hui,LIU Yan-hui,SHI Wei,LUO Dong-lan,LUO Xin-lan,YAO Jun,ZHUANG Heng-guo. Renal involvement in Fabry disease:a clinicopathological analysis of 3 cases[J]. Chinese Journal of Clinical and Experimental Pathology, 2010, 26(2) | |
| Authors: | ZHANG Ming-hui LIU Yan-hui SHI Wei LUO Dong-lan LUO Xin-lan YAO Jun ZHUANG Heng-guo |
| Affiliation: | ZHANG Ming-hui1,LIU Yan-hui1,SHI Wei2,LUO Dong-lan1,LUO Xin-lan1,YAO Jun1,ZHUANG Heng-guo1 |
| Abstract: | Purpose To investigate the clinical and pathological characteristics of renal involvement in Fabry disease.Methods Clinical manifestations of three patients with renal involvement in Fabry disease were analyzed retrospectively.Light,electron microscopy and immunofluorescence stain were performed in renal tissue by biopsy.Results Three patients had proteinuria and hematuria.On light microscopy,the podocytes were enlarged and vacuolated,giving a honeycomb appearance to the glomeruli.Similar changes were seen ... |
| Keywords: | Fabry disease alpha-galactosidase A renal biopsy ultrastructure |
| 本文献已被 CNKI 万方数据 等数据库收录！ | |