X连锁视网膜色素变性携带者。血视网膜屏障通透性的荧光光度测定。

Carriers of X-linked retinitis pigmentosa. A fluorophotometric determination of the permeability of the blood-retinal barrier.

作者信息

Larsen M, Krogsaa B, Warburg M, Sørensen S A, Lund-Andersen H

出版信息

Ophthalmic Paediatr Genet. 1986 Mar;7(1):21-8. doi: 10.3109/13816818609058038.

Abstract

The blood-retinal barrier permeability to fluorescein was studied in a single family with X-linked retinitis pigmentosa by an elaborate vitreous fluorophotometric method. Affected males showed a ten times increase of permeability compared with normals. In a group of seven obligate carriers the mean permeability was significantly increased by 45% compared with the mean permeability in a group of normals. Five of the seven carriers had permeabilities higher than the normal mean permeability + 2 SD, while two carriers had a permeability within the normal mean +/- 2 SD. Thus, this study supported that carriers of X-linked retinitis pigmentosa show deterioration of the blood-retinal barrier. However, the phenomenon is not present in all carriers and the permeability cannot be used as a completely safe indicator of the carrier state.

摘要

采用精细的玻璃体荧光光度法，对一个患有X连锁视网膜色素变性的家系进行了血视网膜屏障对荧光素通透性的研究。与正常人相比，患病男性的通透性增加了10倍。在一组7名肯定携带者中，其平均通透性比正常人群组的平均通透性显著增加了45%。7名携带者中有5人的通透性高于正常平均通透性+2个标准差，而有2名携带者的通透性在正常平均通透性±2个标准差范围内。因此，本研究支持X连锁视网膜色素变性携带者存在血视网膜屏障的损害。然而，这种现象并非在所有携带者中都存在，通透性不能作为携带者状态的完全可靠指标。