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家族性淀粉样多神经病的多模态视网膜成像

Multimodal retinal imaging of familial amyloid polyneuropathy.

作者信息

Latasiewicz Marta, Sala-Puigdollers Anna, Gonzalez-Ventosa Andrea, Milla Elena, Adan Civera Alfredo

机构信息

Department of Ophthalmology, Hospital Clinic of Barcelona, Barcelona, Spain.

Faculty of Medicine, University of Barcelona, Barcelona, Spain.

出版信息

Ophthalmic Genet. 2019 Oct;40(5):407-420. doi: 10.1080/13816810.2019.1666413. Epub 2019 Oct 2.

Abstract

: Retinal amyloid angiopathy is a sight-threatening complication of familial amyloid polyneuropathy (FAP) caused by pathological deposition of transthyretin. The purpose of this report is to present ocular findings in patients with FAP using a combination of novel non-invasive retinal imaging techniques, including first time published images of optical coherence tomography angiography (OCT-A) in FAP.: Observational cross-sectional study of retinal images in patients with FAP using: fundus ultra wide-field photography (UWF); autofluorescence (AF); optical coherence tomography (OCT); and, OCT-A. Fifteen eyes of eight patients with FAP from a tertiary center were included. A descriptive analysis of obtained images and clinical data was performed.: Amyloid vitreous and retinal deposits were easily identified using OCT scans, AF, and UWF images, especially in the red-free modality. OCT-A allowed quality reconstruction of posterior pole vasculature, foveal avascular zone, and areas of ischemia.: Different modalities of currently available non-invasive retinal imaging techniques, including OCT-A scans described for the first time in FAP, are safe and useful in detecting and analyzing retinal amyloidosis. Retinopathy in FAP in the studied group was more frequent than previously reported.

摘要

视网膜淀粉样血管病是由转甲状腺素蛋白的病理性沉积引起的家族性淀粉样多神经病(FAP)的一种威胁视力的并发症。本报告的目的是使用多种新型非侵入性视网膜成像技术,展示FAP患者的眼部表现,包括首次发表的FAP患者光学相干断层扫描血管造影(OCT-A)图像。:对FAP患者的视网膜图像进行观察性横断面研究,使用:眼底超广角摄影(UWF);自发荧光(AF);光学相干断层扫描(OCT);以及OCT-A。纳入了来自三级中心的8例FAP患者的15只眼睛。对获得的图像和临床数据进行了描述性分析。:使用OCT扫描、AF和UWF图像,尤其是在无赤光模式下,淀粉样玻璃体和视网膜沉积物很容易被识别。OCT-A能够对后极部血管系统、黄斑无血管区和缺血区域进行高质量重建。:目前可用的非侵入性视网膜成像技术的不同模式,包括首次在FAP中描述的OCT-A扫描,在检测和分析视网膜淀粉样变性方面是安全且有用的。研究组中FAP的视网膜病变比之前报道的更为常见。

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