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目的：探讨视网膜色素变性的病理改变及其发病机制。方法：对１例常染色体显性遗传性视网膜色素变性患者的视网膜进行组织病理和超微结构观察。结果：视网膜各层组织均有变性改变和结构紊乱，并有区域性差异，后极部变性较周边部为重。视网膜色素上皮细胞病变与感光细胞病变程度密切相关，但后者似较前者为重。感光细胞的超微结构有明显变性改变，尤以外节变性、线粒体变性、胞浆内脂褐素沉着为突出。结论：超微结构变化提示感光细胞能量代谢系统和（或）自噬系统功能障碍。
Objective: To investigate the pathological changes of retinitis pigmentosa and its pathogenesis. Methods: The retina of one patient with autosomal dominant retinitis pigmentosa was observed histopathologically and ultrastructurally. Results: All the retina layers had degenerative changes and structural disorders, with regional differences. The posterior pole degeneration was more serious than the peripheral ones. Retinal pigment epithelial cells and photoreceptor cell disease is closely related to the extent, but the latter seems more important than the former. The ultrastructure of photoreceptor cells have obvious degeneration changes, especially in the degeneration, mitochondrial degeneration, lipofuscin calm cytoplasm prominent. CONCLUSION: The ultrastructural changes suggest photoreceptor cell energy metabolism and / or autophagy dysfunction.