Fuchs' corneal dystrophy.

Fuchs' corneal dystrophy.
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DOI:
10.1586/eop.10.8
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发表时间:
2010-04
影响因子:
0.7
通讯作者:
Gottsch JD
Gottsch JD
中科院分区:
其他
文献类型:
--
作者:
Eghrari AO;Gottsch JD

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Fuchs角膜营养不良症(FCD)是一种进行性遗传性角膜疾病,一个世纪前由奥地利眼科医生Ernst Fuchs首次描述。患者通常出现在生命的第五到六十年,早上视力模糊,持续时间随着疾病的进展而增加。主要是角膜后部的一种情况,其特征包括形成被称为“点滴”的后弹力膜的局灶性异常,内皮细胞密度降低,以及以角膜水肿和上皮性大泡的形成为表现的终末期疾病。在我们对疾病的遗传和病理生理机制的理解方面的最新进展,以及新的成像方式和较小侵入性外科手术的应用,为改善FCD患者的预后提供了新的机会。
Fuchs’ corneal dystrophy (FCD) is a progressive, hereditary disease of the cornea first described a century ago by the Austrian ophthalmologist Ernst Fuchs. Patients often present in the fifth to sixth decade of life with blurry morning vision that increases in duration as the disease progresses. Primarily a condition of the posterior cornea, characteristic features include the formation of focal excrescences of Descemet membrane termed ‘guttae’, loss of endothelial cell density and end-stage disease manifested by corneal edema and the formation of epithelial bullae. Recent advances in our understanding of the genetic and pathophysiological mechanisms of the disease, as well as the application of new imaging modalities and less invasive surgical procedures, present new opportunities for improved outcomes among patients with FCD.