视网膜色素变性视网膜的组织病理和超微结构观察

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、胞浆内脂褐索沉着为突出。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。 （中华眼底病杂志，1997，13：24-26）

OBSERVATION ON THE RETINAL HISTOPATHOLOGY AND 1TS ULTRASTRUCTURE IN A PATIENT WITH RETINITIS PIGMENTOSA.

PURPOSE:To study the retinal pathologic changes and pathogenesis of retinitis pigmentosa(RP).METHODS:The retina from a patient with autosomal dominant RP was examined by light and electron microscopy.RESULTS:Degeneration and structure disturbance almost involved in every layer of retina and were accompanied by regional differences:Posterior region was more than periphery one in severity.Degeneration of retinal pigment epithelium(RPE)closely correlated to that of the photoreceptor.The ultrastructure of the retina showed extensive and severe degeneration in the photoreceptors,parti cularly in outer segments and mitochondrias.Lipofuscin granules were accumulated in the cytoplasm.CONCLUSIONS:These changes suggested that self energizing system and self engulfing system of the photoreceptors were disfunctioned.