RETINAL PATHOPHYSIOLOGY IN INFANTS & ADULTS

婴儿视网膜病理生理学

• 批准号: 3260158
• 负责人: DAVID G BIRCH
• 金额: $ 7.46万
• 依托单位: RETINA FOUNDATION OF THE SOUTHWEST
• 依托单位国家: 美国
• 财政年份: 1984
• 资助国家: 美国
• 起止时间: 1984-12-01 至 1992-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/3260158
• 关键词: adult human (21+); autosomal recessive trait; cataract surgery; circadian rhythms; cone cell; congenital vision disorder; diagnosis quality /standard; disease /disorder prevention /control; disease /disorder proneness /risk; electroretinography; histopathology; human subject; infant human (0-1 year); longitudinal human study; night blindness; psychophysics; pupillography; retina; retina degeneration; retinitis pigmentosa; rod cell; visual fields; visual perception; visual photoreceptor; visual threshold

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.

色素性视网膜炎是导致视网膜色素变性的一个主要的、无法治愈的原因。 在美国视力丧失。 在疾病的早期阶段， 主要涉及视杆细胞介导的视觉，如视杆细胞外节（ROS） 变得缩短且杂乱无章。 Naka-Rushton 分析 全视野视网膜电图（ERG）将用于评估 光夹带引起的视杆 ERG 参数的日变化 代表视网膜炎每种主要遗传类型的患者 色素性病变和锥杆变性患者。 的比率 昼夜光触发后 ERG 参数的恢复 阈值增加将用于确定ROS盘是否 这些患者的合成可能会减少或减慢。 昼夜 杆 ERG 的变化也将用于评估监管 控制光感受器更新的机制。 尝试将是 改变椎间盘脱落和椎间盘合成之间的平衡 通过操纵每日光周期来治疗患者。 关于视网膜炎中视杆细胞丢失的自然过程知之甚少 色素变性，尽管事实上夜盲症和升高 暗适应阈值通常是相关的第一个症状 患有这种疾病。 视杆细胞ERG、视杆细胞视野和视杆细胞 每年都会评估瞳孔测量以确定 每种遗传类型患者的杆丢失率和模式 视网膜色素变性。 现有证据表明，该比率 基于杆的功能的进展会比基于杆的功能更快 已经建立了基于锥体的功能。 评估能力 年轻视网膜炎患者的治疗干预 色素性病变的测量显示快速进展应该增强 试图改变这种可能致盲的自然历史 疾病。 大多数白内障发生在老年患者身上，他们也最有可能发生白内障 患有年龄相关性黄斑病。 局灶视网膜电图测量 将在治疗前和治疗后从患者处获得黄斑功能的数据 白内障手术以确定该措施的效用 检测有介质混浊的眼睛中的黄斑病变。 准确的 黄斑病变的检测非常重要，因此患者和 临床医生可以就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 单侧黄斑裂孔患者将识别患者 有双侧孔形成的风险，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的形成。