2 型神经元类钙化脂褐质沉着症（CLN2）荧光素血管造影的周边视网膜发现。

IF 1.2 4区医学 Q3 OPHTHALMOLOGY

Journal of Aapos Pub Date : 2024-04-01 DOI:10.1016/j.jaapos.2024.103830

David L. Rogers MD , Emily De Los Reyes MD , Thomas A. Mendel MD, PhD , Brian Caprul , Sarah Podlasiak RN , Catherine O. Jordan MD

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引用次数: 0

摘要

根据韦尔-康奈尔眼科严重程度评分（WCOSS）分期系统，2 型神经元类脂膜炎（CLN2）患者视网膜上的外周血管变化被描述为疾病晚期的血管衰减。我们描述了在 WCOSS 分级为 2 期的患者中通过荧光素血管造影发现的孤立、轻度、伴有外周动脉脱落的外周血管炎。我们认为，这种血管炎代表了疾病的早期血管变性阶段，会导致疾病晚期出现血管衰减。

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Peripheral retinal finding on fluorescein angiography in neuronal ceroid lipofuscinosis type 2 (CLN2)

Classically, peripheral vascular changes in the retina in patients with neuronal ceroid lipofuscinosis type 2 (CLN2) are described as vascular attenuation seen in the late stages of disease on the Weill Connell Ophthalmic Severity Score (WCOSS) staging system. We describe isolated, mild, peripheral vasculitis with peripheral arteriolar dropout identified by fluorescein angiography in patients with a WCOSS grade of stage 2. We believe this vasculitis represents an early vasodegenerative phase of disease that leads to the vascular attenuation seen in later stages of the disease.

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来源期刊

Journal of Aapos 医学-小儿科

CiteScore

2.40

自引率

12.50%

发文量

159

审稿时长

55 days

期刊介绍： Journal of AAPOS presents expert information on children''s eye diseases and on strabismus as it affects all age groups. Major articles by leading experts in the field cover clinical and investigative studies, treatments, case reports, surgical techniques, descriptions of instrumentation, current concept reviews, and new diagnostic techniques. The Journal is the official publication of the American Association for Pediatric Ophthalmology and Strabismus.