Fishman G A, Ward L M, Rusin M M
Department of Ophthalmology, University of Illinois, College of Medicine, Chicago 60612.
Retina. 1990;10(2):102-4. doi: 10.1097/00006982-199004000-00002.
Ten patients with Best's macular dystrophy were examined with vitreous fluorophotometry and results were compared with a normal population. Seventeen of the 20 affected eyes demonstrated an intact blood-retinal barrier with normal inward permeability of fluorescein dye. Despite diffuse functional impairment of the retinal pigment epithelium (RPE), determined by electro-oculography, as well as accumulation of a lipofuscin or lipofuscin-like substance within virtually all RPE cells, the blood-retinal barrier function of these cells remained intact as determined by clinical fluorophotometry. Similar findings have previously been noted in another hereditary retinal disorder (fundus flavimaculatus) in which a lipofuscin-like substance also accumulates diffusely within RPE cells.
对10例Best黄斑营养不良患者进行了玻璃体荧光光度测定，并将结果与正常人群进行比较。20只患眼中的17只显示血视网膜屏障完整，荧光素染料的内向通透性正常。尽管通过眼电图测定视网膜色素上皮（RPE）存在弥漫性功能损害，并且几乎所有RPE细胞内都有脂褐素或脂褐素样物质积聚，但临床荧光光度测定显示这些细胞的血视网膜屏障功能仍保持完整。先前在另一种遗传性视网膜疾病（黄斑黄点症）中也注意到类似的发现，其中脂褐素样物质也在RPE细胞内弥漫性积聚。
