RETINAL PATHOPHYSIOLOGY IN INFANTS AND ADULTS

视网膜色素变性代表了一个主要的，无法治愈的原因， 视力丧失在美国 在早期阶段，疾病 主要涉及视杆细胞介导的视杆细胞外节（ROS） 变短变得杂乱无章 Naka-Rushton分析 全视野视网膜电图（ERG）将用于评估 来自光夹带的视杆ERG参数的日变化 代表视网膜炎的每种主要遗传类型的患者 色素沉着症和锥杆变性患者。 率 恢复视网膜电图参数后，昼夜光触发 阈值增加将用于确定ROS盘是否 在这些患者中合成可能减少或减慢。 的日 视杆细胞ERG的变化也将用于评估调节性视网膜电图。 控制感光细胞更新的机制。 尝试将 用于改变椎间盘脱落和椎间盘合成之间的平衡 通过控制每天的光照周期 很少有人知道的自然过程杆损失视网膜炎 色素沉着，尽管事实上，夜盲症和提高 适应黑暗的阈值通常是第一个症状 与这种疾病。 视杆ERG、视杆视野和视杆 每年进行瞳孔测量，以确定 每种遗传类型的患者的杆丢失率和模式 视网膜色素变性 现有证据表明， 基于杆的功能的进展速度将比 已经建立了基于锥的功能。 评估能力 年轻视网膜炎患者的治疗干预 色素沉着与措施，显示快速进展，应加强 试图改变这种潜在致盲的自然历史， 疾病 大多数白内障发生在老年患者身上，他们也最有可能 患有老年性黄斑病变 焦点视网膜电图测量 将在治疗前后从患者身上获取黄斑功能的信息 白内障手术，以确定这项措施的效用， 检测具有中膜混浊的眼睛中的黄斑病变。 准确 黄斑病变的检测是重要的， 临床医生可以对白内障手术做出明智的决定 并预测术后视力潜力。 前瞻性研究 的单侧黄斑裂孔患者将识别患者 存在双侧成孔的风险，帮助识别风险因素 黄斑裂孔的形成，并导致合理的干预， 防止对侧眼黄斑裂孔形成。

Retinitis pigmentosa represents a major, untreatable cause of visual loss in the United States. In early stages, the disease involves primarily rod-mediated vision as rod outer segments (ROS) become shortened and disorganized. The Naka-Rushton analysis of the full-field electroretinogram (ERG) will be used to evaluate diurnal variations in rod ERG parameters from light-entrained patients representing each major genetic type of retinitis pigmentosa and patients with cone-rod degeneration. The rate of recovery of ERG parameters following the diurnal light-triggered threshold increase will be used to determine whether ROS disc synthesis may be reduced or slowed in these patients. The diurnal variation in the rod ERG will also be used to evaluate regulatory mechanisms controlling photoreceptor renewal. Attempts will be made to alter the balance between disc shedding and disc synthesis in patients by manipulating the daily light cycles. Little is known about the natural course of rod loss in retinitis pigmentosa, despite the fact that night-blindness and elevated dark-adapted thresholds are typically the first symptoms associated with this disease. Rod ERGs, rod visual fields and rod pupillometry will be evaluated yearly in order to determine the rate and pattern of rod loss in patients with each genetic type of retinitis pigmentosa. Available evidence suggests that the rate of progression will be faster for rod-based function than that already established for cone-based function. The ability to assess therapeutic intervention in young patients with retinitis pigmentosa with measures that show rapid progression should enhance attempts to alter the natural history of this potentially blinding disease. Most cataracts occur in older patients who are also most likely to have age-related maculopathy. Focal electroretinographic measures of macular function will be obtained from patients before and after cataract surgery to determine the utility of this measure for detecting maculopathy in eyes with media opacities. Accurate detection of maculopathy is important so that both patient and clinician can make informed decisions concerning cataract surgery and anticipate post-surgical visual potential. Prospective studies of patients with unilateral macular holes will identify patients at risk for bilateral hole formation, help identify risk factors for macular hole formation, and lead to rational intervention to prevent macular hole formation in the fellow eye.