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 16.4 1区化学 Q1 CHEMISTRY, MULTIDISCIPLINARY

Accounts of Chemical Research 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

Accounts of Chemical Research 化学-化学综合

CiteScore

31.40

自引率

1.10%

发文量

312

审稿时长

2 months

期刊介绍： Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance. Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.