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。 Objective: To investigate the renal pathological features of female Fabry disease and provide the basis for clinical diagnosis. Methods: The renal biopsy was performed in 1 female patient with persistent proteinuria at 30 years old. The specimens were observed under light microscope and electron microscope. Results: The ultrastructure of renal biopsy showed that the cytoplasm of glomerular epithelial cells contained a large number of osmiophilic lamellar bodies, which were round and consisted of concentric dense layers with a diameter of 0.5-3 μm and a small lamella The lamellar interval of the body is about 4-5 nm. CONCLUSION: The presence of osmiophilic lamellar bodies in the glomerular visceral cells is a characteristic morphological change of Fabry’s disease.