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：项目摘要和相关性底面的固有自荧光（AF）起源于RPE双倍脂蛋白。而RPE 脂肪霉素即使在健康的眼睛中也会积聚，通过共聚焦扫描激光眼镜检查对Feldus AF的成像（CSLO）表明，在几种视网膜疾病中，AF的模式和强度偏离了正常情况。因此，我们先前证明了量化短波长底面的标准化方法 AF（QAF）可以帮助诊断视网膜疾病，监测疾病进展和评估治疗结果。为了实现这些调查，我们收集了来自大量参与者的规范性QAF数据健康的眼睛状况（6-60岁），以建立QAF值的范围种族。展望未来，我们将使用这些正常值来确定双素脂肪蛋白是否积累是区分RPE细胞的细胞负担 HCQ在视网膜上以及下黄斑中的QAF水平是否可以作为HCQ毒性的早期迹象（特定目标1）。我们还将确定为什么脉络膜血症的女性载体（rpe脂肪霉素降低）测量为QAF（特定目标2）。使用QAF方法，我们将测试损失之间的关系 SD-OCT图像中的椭圆形区域以及短波长眼底自动荧光（SW-AF）的变化诊断患有中央浆膜性脑炎病的患者的强度（特定目标3）。纯合突变在ABCA4中，尽管荧光强度的空间分布不统一。通过多模式成像，我们将阐明该地形的基本结构基础分布（特定目标4）。总而言之，本申请中提出的研究将研究视网膜脂蛋白的贡献使几种视网膜疾病的发作和进展 AF促进了某些视网膜疾病的诊断和监测。