Sorsby's fundus dystrophy. A clinical study.
Sorsby's fundus dystrophy. A clinical study.
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DOI：
10.1016/s0161-6420(89)32654-6
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发表时间：
1989-12
期刊：
影响因子：
13.7
通讯作者：
P. Polkinghorne;M. R. Capon;T A Berninger;A. Lyness;Kulwant S. Sehmi;A. Bird
中科院分区：
文献类型：
--
作者：
P. Polkinghorne;M. R. Capon;T A Berninger;A. Lyness;Kulwant S. Sehmi;A. Bird
对一个已知患有索尔斯比眼底营养不良的家庭进行了一项调查。对 50 名成员进行了审查，发现 14 名成员受到影响。索斯比的许多原始结论都得到了证实，包括遗传模式和视力丧失的年龄。在病程早期，布鲁赫膜上就存在黄色物质。然而，最早的表型标记是脉络膜毛细血管的延迟充盈。随着时间的推移，脉络膜灌注异常变得更加严重并离心扩展。中心视力丧失通常是由于外视网膜和脉络膜萎缩所致。视网膜下新生血管形成罕见。这种营养不良与年龄相关性黄斑疾病之间的同源性强调了该家族临床发现的重要性。
A survey was undertaken of a family known to have Sorsby's fundus dystrophy. Fifty members were reviewed, and 14 were found to be affected. Many of Sorsby's original conclusions were confirmed, including the pattern of inheritance and age of visual loss. Yellow material was present at the level of Bruch's membrane early in the course of the disease. However, the earliest phenotypic marker was delayed filling of the choriocapillaris. Abnormalities of choroidal perfusion became more profound and extended centrifugally with time. The loss of central vision was commonly due to atrophy of the outer retina and choroid. Subretinal neovascularization was a rare occurrence. The homology between this dystrophy and age-related macular disease underlines the importance of the clinical findings in this family.