Quantitative Fundus Autofluorescence in Retinal Disorders

描述：项目总结和相关性 眼底的固有自发荧光（AF）来源于RPE双视黄脂褐素。当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检查眼底AF的成像 （cSLO）已经表明，AF的模式和强度在几种视网膜疾病中偏离正常。 因此，我们先前证明了量化短波长眼底的标准化方法， AF（qAF）可以辅助视网膜疾病的诊断、疾病进展的监测和视网膜病变的诊断。 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性qAF数据， 健康眼睛状态（6-60岁），以便建立关于年龄、性别和 种族今后，我们将使用这些正常值，以确定是否bisretinoid脂褐素 累积，一种区分RPE细胞的细胞负荷，赋予对以下不利影响的易感性： 视网膜上的HCQ和下黄斑中的qAF水平是否可以作为HCQ毒性的早期迹象（特异性 目标1）。我们还将确定为什么女性无脉络膜携带者，目前减少视网膜色素上皮脂褐素 QAF（Specific Aim 2）。使用qAF方法，我们将测试 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中的强度（特定目标3）。纯合突变 在ABCA 4中的荧光强度的空间分布是众所周知的，以赋予升高的qAF， 并不统一。通过多模态成像，我们将阐明这种地形的潜在结构基础 具体目标（4）。 总之，本申请中提出的研究将检查视网膜脂褐素对视网膜细胞凋亡的贡献。 使几种视网膜疾病的发病和进展，并将证明，眼底定量 AF有助于某些视网膜疾病的诊断和监测。

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.