Quantitative Fundus Autofluorescence in Retinal Disorders

描述：项目摘要和相关性 眼底固有的自体荧光(AF)来源于RPE双维A酸脂褐素。而RPE 即使在健康的眼睛中也有脂褐素积聚。共焦扫描激光眼底镜对眼底房颤的成像 (CSLO)的研究表明，在一些视网膜疾病中，房颤的模式和强度偏离了正常。 因此，我们以前证明了一种量化短波长眼底的标准化方法 房颤(QAF)有助于视网膜疾病的诊断、病情进展的监测和治疗。 对治疗结果的评估。 为了进行这些调查，我们从大量参与者那里收集了标准化的QAF数据 健康的眼睛状况(6-60岁)，以建立年龄、性别和年龄相关的QAF值范围 种族问题。展望未来，我们将使用这些正常值来确定双维A酸脂褐素 堆积，一种区别于RPE细胞的细胞负担，使人对 HCQ对视网膜的影响以及黄斑下部的QAF水平是否可以作为HCQ中毒的早期迹象(特异性 目标1)。我们还将确定为什么女性脉络膜血症携带者RPE脂褐素降低 以QAF(特定目标2)衡量。使用QAF方法，我们将测试损失与 SD-OCT图像中的椭球区与眼底短波自体荧光的变化 诊断为中心性浆液性脉络膜视网膜病变患者的强度(特定目标3)。纯合突变 在ABCA4中，众所周知，尽管荧光强度的空间分布增加了QAF 是不统一的。通过多模式成像，我们将阐明这一地形的潜在结构基础 分配(具体目标4)。 总之，在本申请中提出的研究将检查视网膜的脂褐素的贡献 与几种视网膜疾病的发生和发展有关，并将证明眼底定量 房颤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.