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血红蛋白S/O:一种罕见血红蛋白病的视网膜表现

Hemoglobin S/O: Retinal Manifestations of a Rare Hemoglobinopathy.

作者信息

Sanders Riley, Ly Victoria, Ahmad Kinza, Swift Jesse, Sallam Ahmed, Uwaydat Sami

机构信息

Harvey and Bernice Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

College of Medicine, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

出版信息

Case Rep Ophthalmol. 2020 May 27;11(2):189-195. doi: 10.1159/000507879. eCollection 2020 May-Aug.

Abstract

Hemoglobin S/O (Hgb S/O) disease is a rare hemoglobinopathy which presents similarly to sickle cell retinopathy, with only three prior reports that describe associated retinal findings. In this report, we present ophthalmic examination findings in 2 patients with Hgb S/O. One patient exhibited peripheral ischemia and sunburst lesions without neovascular disease, and the other patient developed proliferative retinopathy of both eyes and multiple posterior-pole branch retinal artery occlusions in one eye. To our knowledge, this is the first case of retinal arterial occlusive disease in Hgb S/O, and the first report of fundus autofluorescence and OCT angiography in Hgb/O retinopathy.

摘要

血红蛋白S/O(Hgb S/O)病是一种罕见的血红蛋白病,其表现与镰状细胞视网膜病变相似,此前仅有三篇报告描述了相关的视网膜表现。在本报告中,我们展示了2例Hgb S/O患者的眼科检查结果。1例患者表现为周边缺血和日光放射状病变,无新生血管疾病,另1例患者双眼发生增殖性视网膜病变,其中1只眼出现多条后极部视网膜分支动脉阻塞。据我们所知,这是Hgb S/O病中视网膜动脉闭塞性疾病的首例,也是Hgb/O视网膜病变中眼底自发荧光和光学相干断层扫描血管造影的首例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/326d/7315179/b2bd2bc4d946/cop-0011-0189-g01.jpg

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