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类科茨病性视网膜色素变性:三例报告

Coats-like retinitis pigmentosa: Reports of three cases.

作者信息

Kan Emrah, Yilmaz Turgut, Aydemir Orhan, Güler Mete, Kurt Jülide

机构信息

Department of Ophthalmology, Firat University School of Medicine, Elaziğ, Turkey.

出版信息

Clin Ophthalmol. 2007 Jun;1(2):193-8.

Abstract

PURPOSE

Describing the ophthalmic findings of an exudative vasculopathy called as Coats-like retinitis pigmentosa on three patients. The etiology of the Coats-like retinitis pigmentosa is obscure. The principal theories have been discussed in this article.

METHODS

Three observational case series have been discussed. Complete ophthalmic examinations and color fundus photos, visual field, and fluorescein angiography have been performed.

RESULTS

We have identified 3 patients who have some typical clinical features of Coats-like retinitis pigmentosa; peripheral serous retinal detachment, telangiectasia, prominent lipid deposition, pigmentary changes in peripheral retina, and loss of vision. None of the three patients had positive family history. All of the patients have had symptoms of nyctalopia, decreased central vision, and two of them have had constriction of visual field. All of the patients have had cataracts and two of them underwent cataract surgery. Fundus examination and fluorescein angiography of patients revealed typical retinitis pigmentosa with Coats-type changes in bilateral inferiotemporal quadrants.

CONCLUSION

A better understanding of clinical features and genetic etiology of Coats-type retinitis pigmentosa will aid diagnosis and development of new therapies. If sufficient conditions arise, genetic factors that influence the expression of CRB1 mutations in Coats-like retinitis pigmentosa should be detected.

摘要

目的

描述3例被称为类科茨病性视网膜色素变性的渗出性血管病变的眼科检查结果。类科茨病性视网膜色素变性的病因尚不清楚。本文讨论了主要的理论。

方法

讨论了3个观察性病例系列。进行了全面的眼科检查、彩色眼底照片、视野检查和荧光素血管造影。

结果

我们确定了3例具有类科茨病性视网膜色素变性一些典型临床特征的患者;周边浆液性视网膜脱离、毛细血管扩张、显著的脂质沉积、周边视网膜色素改变和视力丧失。这3例患者均无阳性家族史。所有患者均有夜盲症状、中心视力下降,其中2例有视野缩窄。所有患者均患有白内障,其中2例接受了白内障手术。患者的眼底检查和荧光素血管造影显示典型的视网膜色素变性,双侧颞下象限有科茨病样改变。

结论

更好地了解科茨病样视网膜色素变性的临床特征和遗传病因将有助于诊断和开发新的治疗方法。如果有足够的条件,应该检测影响类科茨病性视网膜色素变性中CRB1突变表达的遗传因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5f39/2704518/7158a8b884b2/opth-1-193f1.jpg

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