[Stargardt病与黄斑眼底]。

P François, P Turut, B Puech, J C Hache
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引用次数: 0

摘要

从他们的62个个人观察和文献病例的研究中,作者证明,在Stargardt病和黄斑眼底黄斑病变的眼科透视和功能方面是严格相同的。它们的遗传也是相同的,通常是常染色体和隐性遗传,更罕见的是显性遗传。黄斑状病变位于黄斑周围或外周区域可能共存于同一家族,并且肯定对应于一种独特基因的不同表达形式。作者讨论了这两种情感和其他青少年黄斑变性所带来的病理性问题。他们的结论如下:同一疾病可能有三种不同的形式:—纯Stargardt病;——黄斑周围冠状Stargardt病;——伴有外周黄斑眼底的Stargardt病。
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[Stargardt's disease and fundus flavimaculatus].

From their sixty two personnal observations and a study of literature cases, the authors demonstrate that the ophthalmoscopic fluoroscopic and functionnal aspects of macular lesions are strictly identical in Stargardt disease and in Fundus Flavimaculatus. Their transmission is also identical, generally autosomal and recessive, more rarely dominant. Flavimaculate lesions situated in perimacular or inperipheric area may coexist in the same family, and certainly correspond to variable forms of expressivity of a unique gene. The authors discuss the nosologic problems brought by these two affections and other juvenile macular degenerations. Their conclusions are as follows: The same disease may present three different forms: -- Pure Stargardt disease; -- Stargardt disease with perimacular flavimaculate crown; -- Stargardt disease with peripheric Fundus Flavimaculatus.

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