Quantitative Fundus Autofluorescence in Retinal Disorders

视网膜疾病中的定量眼底自发荧光

基本信息

批准号：
10358501
负责人：
Janet Ruthe Sparrow
金额：
$ 39.29万
依托单位：
COLUMBIA UNIVERSITY HEALTH SCIENCES
依托单位国家：
美国
项目类别：
财政年份：
2014
资助国家：
美国
起止时间：
2014-06-01 至 2023-12-31
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/10358501
关键词：
Abbreviations Acute Address Adverse effects Age Age related macular degeneration Alleles Analysis of Variance Cells Characteristics Choroideremia Clinical Confidence Intervals Dark Adaptation Data Diagnosis Disease Disease Progression Ethnic Origin Eye Female Fluorescence Funding Fundus Gender Genetic Genotype Glyoxal Human Hydroxychloroquine Image Individual Inferior Influentials Investigation Lasers Legal Blindness Lesion Light Link Lipofuscin Liquid Chromatography Measures Medical Genetics Melanins Membrane Methods Monitor Mosaicism Multimodal Imaging Mutation Natural History Normal Range Nuclear Ophthalmoscopy Optical Coherence Tomography Outcome Measure Participant Pathogenicity Patients Pattern Performance Phenotype Photoreceptors Predisposition Process Progressive Disease Public Health Pyruvaldehyde Rab escort protein Retina Retinal Degeneration Retinal Diseases Retinitis Pigmentosa Retinol dehydrogenase Scanning Serous Signal Transduction Source Spatial Distribution Spectrometry, Mass, Electrospray Ionization Standardization Stargardt&apos s disease Structure of retinal pigment epithelium Testing Therapeutic Thick Toxic effect Visual Acuity Work aged clinical diagnosis confocal imaging disorder of macula of retina fundus imaging genetic testing interest macula mutation carrier retinal imaging therapy outcome

项目摘要

Description: Project Summary and Relevance The inherent autofluorescence (AF) of the fundus originates from RPE bisretinoid lipofuscin. While RPE lipofuscin is amassed even in healthy eyes, imaging of fundus AF by confocal scanning laser ophthalmoscopy (cSLO) has shown that the patterns and intensities of AF deviate from normal in several retinal disorders. Accordingly, we previously demonstrated that a standardized approach to quantifying short wavelength fundus AF (qAF) can assist in the diagnosis of retinal disease, in the monitoring of disease progression and in the assessment of therapeutic outcomes. To enable these investigations we have gathered normative qAF data from a large number of participants with healthy eye status (aged 6-60) so as to establish ranges of qAF values with respect to age, gender and ethnicity. Going forward we will use these normal values to determine whether bisretinoid lipofuscin accumulation, a cellular burden that distinguishes RPE cells, confers susceptibility to the adverse effects of HCQ on retina and whether qAF levels in inferior macula can serve as an early sign of HCQ toxicity (Specific Aim 1). We will also determine why female carriers of choroideremia, present with reduced RPE lipofuscin measured as qAF (Specific Aim 2). Using the qAF approach we will test for a relationship between the loss of the ellipsoid zone in SD-OCT images and changes in short wavelength fundus autofluorescence (SW-AF) intensities in patients diagnosed with central serous chorioretinopathy (Specific Aim 3). Homozygous mutations in ABCA4 are well known to confer elevated qAF although the spatial distribution of the fluorescence intensity is not uniform. By multi-modal imaging we will elucidate the underlying structural basis of this topographic distribution (Specific Aim 4). In summary, the studies proposed in this application will examine the contribution that the lipofuscin of retina makes to the onset and progression of several retinal diseases and will demonstrate that quantitation of fundus AF facilitates the diagnosis and monitoring of some retinal disorders.

描述：项目摘要和相关性眼底固有的自发荧光 (AF) 源自 RPE 双维甲酸脂褐素。而RPE 即使在健康的眼睛中，脂褐素也会积聚，通过共焦扫描激光检眼镜对眼底 AF 进行成像 (cSLO) 表明，在几种视网膜疾病中，AF 的模式和强度偏离正常。因此，我们之前证明了量化短波长眼底的标准化方法 AF (qAF) 可协助诊断视网膜疾病、监测疾病进展以及评估治疗结果。为了进行这些调查，我们从大量参与者那里收集了规范的 qAF 数据健康的眼睛状态（6-60 岁），以确定与年龄、性别和年龄相关的 qAF 值范围种族。展望未来，我们将使用这些正常值来确定双维A酸脂褐素是否积累是区分 RPE 细胞的细胞负担，使人容易受到以下不良影响的影响： HCQ 对视网膜的影响以及下黄斑中的 qAF 水平是否可以作为 HCQ 毒性的早期征兆（具体目标1）。我们还将确定为什么女性无脉络膜血症携带者会出现 RPE 脂褐质减少测量为 qAF（具体目标 2）。使用 qAF 方法，我们将测试损失之间的关系 SD-OCT 图像中的椭球区和短波长眼底自发荧光 (SW-AF) 的变化诊断为中心性浆液性脉络膜视网膜病变的患者的强度（具体目标 3）。纯合突变众所周知，ABCA4 会导致 qAF 升高，尽管荧光强度的空间分布不统一。通过多模态成像，我们将阐明这种地形的潜在结构基础分布（具体目标 4）。总之，本申请中提出的研究将检验视网膜脂褐质的贡献导致多种视网膜疾病的发生和进展，并将证明眼底的定量 AF 有助于某些视网膜疾病的诊断和监测。