Microperimetry in Three Inherited Retinal Disorders

Microperimetry in Three Inherited Retinal Disorders
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DOI:
10.1080/08820538.2019.1622025
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发表时间:
2019-06-01
影响因子:
1.7
通讯作者:
Fulton, Anne B.
Fulton, Anne B.
中科院分区:
医学4区
文献类型:
--
作者:
Bagdonaite-Bejarano, Laura;Hansen, Ronald M.;Fulton, Anne B.

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目的:微视野检查(MP)用于评估中央视网膜介导的视觉敏感性。因此,MP 表现是基因治疗试验的候选结果衡量标准。在此,我们回顾了三种已启动基因治疗试验的遗传性视网膜疾病的 MP 结果:无脉络膜血症、Stargardt 病和 X 连锁青少年视网膜劈裂症。这些疾病中的每一种通常出现在儿童时期,并且每种疾病对中央视网膜都有不同的影响。结果和结果:我们的审查表明,微视野测定法在上述每种情况下都是可行的。 MP 敏感度图因条件而异,与三种条件中每种条件的已知影响一致。然而,这三种疾病中的每一种在注视稳定性和敏感性丧失模式方面都存在相当大的变异性。结论:微视野测量是监测个体患者中央视网膜功能的一个有价值的工具,特别是与 OCT、自发荧光和敏锐度等其他方式结合使用,因此可能有助于评估基因治疗的疗效。 MP 参数的可变性引起了在样本量可能较小的治疗试验中应用 MP 作为结果测量时的一些注意事项。尽管如此,我们怀疑 MP 将继续在未来的基因治疗试验中占有一席之地。
Objective: Microperimetry (MP) is used to assess visual sensitivity mediated by the central retina. As such, MP performance is a candidate outcome measure for gene therapy trials. Herein, we review MP results in three inherited retinal disorders for which gene therapy trials have been initiated-choroideremia, Stargardt disease, and X-linked juvenile retinoschisis. Each of these disorders typically presents in childhood and each has distinct effects on the central retina. Outcomes and Results: Our review indicates that microperimetry is feasible in each of these conditions. The MP sensitivity maps vary among conditions consistent with known effects of each of the three conditions. There is, however, within each of the three disorders considerable variability in fixation stability and in the pattern of sensitivity loss. Conclusions: Microperimetry is a valuable tool for monitoring functional aspects of central retina in an individual patient, especially in combination with other modalities such as OCT, autofluorescence, and acuity and thus may contribute to evaluating the efficacy of gene treatments. Variability of the MP parameters raises some cautions in application of MP as an outcome measure in treatment trials that may have small sample sizes. Nonetheless, we suspect that MP will continue to have a rightful place in future gene therapy trials.