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去铁胺诱导的阴性视网膜电图反应。

Deferoxamine-induced electronegative ERG responses.

作者信息

Jauregui Ruben, Park Karen Sophia, Bassuk Alexander G, Mahajan Vinit B, Tsang Stephen H

机构信息

Department of Ophthalmology, Columbia University, New York, NY, USA.

Jonas Children's Vision Care and Bernard & Shirlee Brown Glaucoma Laboratory, New York, NY, USA.

出版信息

Doc Ophthalmol. 2018 Aug;137(1):15-23. doi: 10.1007/s10633-018-9640-3. Epub 2018 May 16.

Abstract

PURPOSE

To report a case of deferoxamine-induced retinopathy characterized by electroretinography (ERG), optical coherence tomography angiography (OCT-A), and other multimodal imaging.

METHODS

This is an observational case report of one patient. Full-field ERG was performed. OCT-A, spectral-domain optical coherence tomography (SD-OCT), color fundus photography, and fundus autofluorescence were used to characterize the retinopathy induced by deferoxamine use.

RESULTS

A 64-year-old man with a history of β-thalassemia intermedia presented with worsening visual acuity, nyctalopia, and electronegative ERG. OCT-A revealed atrophy of the choriocapillaris in areas of hypoautofluorescence, corresponding to regions of retinal atrophy. SD-OCT showed disruption of the ellipsoid zone, granular hyperreflective deposits within the retinal pigment epithelium, thinning of the retinal layers, and extensive choroidal sclerosis and atrophy of the retinal pigment epithelium.

CONCLUSION

Deferoxamine-induced retinopathy can manifest with electronegative maximal ERG responses, and OCT-A can be used to detect deferoxamine toxicity.

摘要

目的

报告一例以视网膜电图(ERG)、光学相干断层扫描血管造影(OCT - A)及其他多模态成像为特征的去铁胺诱导性视网膜病变病例。

方法

这是一例对一名患者的观察性病例报告。进行了全视野ERG检查。使用OCT - A、光谱域光学相干断层扫描(SD - OCT)、彩色眼底照相和眼底自发荧光来描述因使用去铁胺引起的视网膜病变。

结果

一名64岁的中间型β地中海贫血病史男性患者,出现视力下降、夜盲和ERG阴性。OCT - A显示低自发荧光区域的脉络膜毛细血管萎缩,对应视网膜萎缩区域。SD - OCT显示椭圆体带破坏、视网膜色素上皮内颗粒状高反射沉积物、视网膜各层变薄以及广泛的脉络膜硬化和视网膜色素上皮萎缩。

结论

去铁胺诱导性视网膜病变可表现为ERG最大反应阴性,且OCT - A可用于检测去铁胺毒性。

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