【摘要】 目的:探讨女性Fabry病的肾脏病理特点,为临床诊断提供依据。方法:对1例30岁有持续蛋白尿的女性患者行肾活检,标本在光镜和电镜下进行观察。结果:肾活检的超微结构显示肾小球上皮细胞的胞浆中含有大量的嗜锇板层小体,呈圆形,由同心的致密层组成,直径约0．5-3μm,板层小体的板层间隔周期约4-5 nm。结论:肾小球脏层细胞内出现嗜锇板层小体是Fabry病特征性形态学改变。更多还原

【Abstract】 Objective: To study the renal pathology features of a female with Fabry’s diseas. Methods: A 30 year-old women presented with persistent proteinuria for which renal biopy was performed. The specimen was observed by lightnd electron microscopy. Results: Ultrastructural study of the renal biopsy demonstrated that the scattered epithelial cells had the present of foam under the light microscopy and in the glo meruli containing numerous laminated bodies under the electron microscopy. They are round and composed of concentric whorls of dense layers. The diameter is about 0. 5-10 μm. The periodicity of the membrane structure of the laminated bodies is estimated to be 4-5 nm. Conclusion: The diagnostic morphology of Fabry’s disease is osmiophilic myelinic body in the cytoplasm of viscral cytes.更多还原