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一名患有Usher综合征患者的非肉芽肿性前葡萄膜炎。

Nongranulomatous anterior uveitis in a patient with Usher syndrome.

作者信息

Alzuhairy Sultan Abdulaziz S, Alfawaz Abdullah

机构信息

Ophthalmology Department, Faculty of Medicine, Qassem University, Buridah, Saudi Arabia.

Anterior Segment and Uveitis Division, Ophthalmology Department, Faculty of Medicine, King Saud University, Riyadh, Saudi Arabia.

出版信息

Saudi J Ophthalmol. 2013 Oct;27(4):295-8. doi: 10.1016/j.sjopt.2013.06.001. Epub 2013 Jun 19.

DOI:10.1016/j.sjopt.2013.06.001
PMID:24371428
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3841258/
Abstract

A 34-year-old female with Usher syndrome, but no family history of similar illness, presented with complaints of vision reduction, redness, and photophobia. Biomicroscopic examination showed mildly injected conjunctivae bilateral, small, round keratic precipitates; bilateral +2 cells with no flare reaction in the anterior chamber; and bilateral posterior subcapsular cataracts. No associated posterior synechiae, angle neovascularization, or iris changes were detected; normal intraocular pressures were obtained. Fundus examination demonstrated waxy pallor of both optic nerves, marked vasoconstriction in retinal vessels, and retinal bone spicule pigment formation, with a normal macula. Electroretinography confirmed the diagnosis of retinitis pigmentosa, optical coherent tomography was normal and otolaryngology consultation was conducted. To our knowledge, an association between Usher syndrome and bilateral nongranulomatous anterior uveitis has not been previously reported, and our purpose is to report this association.

摘要

一名34岁患有尤塞综合征的女性,但无类似疾病家族史,主诉视力下降、眼红和畏光。生物显微镜检查显示双侧结膜轻度充血,有小的圆形角膜后沉着物;双侧前房有+2细胞且无房水闪辉反应;双侧后囊下白内障。未检测到相关的虹膜后粘连、房角新生血管或虹膜改变;眼压正常。眼底检查显示双侧视神经蜡样苍白,视网膜血管明显收缩,视网膜有骨针样色素形成,黄斑正常。视网膜电图确诊为色素性视网膜炎,光学相干断层扫描正常,并进行了耳鼻喉科会诊。据我们所知,尤塞综合征与双侧非肉芽肿性前葡萄膜炎之间的关联此前尚未见报道,我们的目的是报告这种关联。

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本文引用的文献

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Clin Ophthalmol. 2011;5:557-9. doi: 10.2147/OPTH.S19525. Epub 2011 Apr 29.
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Usher syndrome associated with Fuchs' heterochromic uveitis.伴 Fuchs 异色性虹膜睫状体炎的先天性耳聋综合征。
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EB-virus transformed human lymphocytes from uveitis and retinitis pigmentosa patients secrete antibodies to retinal antigens.
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