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引用本文的文献

1
Phototoxicity avoidance is a potential therapeutic approach for retinal dystrophy caused by EYS dysfunction.避免光毒性是治疗由 EYS 功能障碍引起的视网膜营养不良的一种潜在治疗方法。
JCI Insight. 2024 Apr 22;9(8):e174179. doi: 10.1172/jci.insight.174179.

评估视网膜色素变性中受视网膜血管遮蔽的外核层。

Evaluation of outer nuclear layer overshadowed by retinal vessels in retinitis pigmentosa.

机构信息

Department of Ophthalmology and Visual Sciences, Kyoto University Graduate School of Medicine, Sakyo-ku, Kyoto, Japan.

出版信息

Eye (Lond). 2022 May;36(5):1042-1049. doi: 10.1038/s41433-021-01578-y. Epub 2021 May 11.

DOI:10.1038/s41433-021-01578-y
PMID:33976398
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9046433/
Abstract

OBJECTIVES

We investigated in vivo light-induced photoreceptor damage in retinitis pigmentosa (RP) using spectral-domain optical coherence tomography (SD-OCT) images.

METHODS

We retrospectively reviewed patients with genetic diagnosis of EYS-associated RP. The outer nuclear layer (ONL) thickness under retinal vessels was measured on SD-OCT vertical scans. As a control, we measured adjacent ONL thickness 100 μm superior and inferior from the vessel. Same measurements were performed in healthy subjects. We calculated the ratio of ONL thickness under vessel divided by the average of adjacent ONL thickness and defined as ONL preservation ratio. In patients with RP, the length of ellipsoid zone (EZ) from the fovea was also measured with SD-OCT vertical scans.

RESULTS

Thirty EYS-associated RP patients and 25 healthy subjects were included. In both groups, ONL thickness overshadowed by retinal vessels was not significantly different from that of adjacent area. However, ONL preservation ratio of RP was larger than that of healthy control in both superior and inferior retina (1.03 vs 0.97; p < 0.01, 1.15 vs 0.95; p < 0.01, respectively). In RP, ONL preservation ratio was significantly larger in the inferior retina than superior retina (p < 0.01). Furthermore, in RP patients, the EZ length from the fovea was always shorter in the inferior than superior retina and there was a significant difference (p < 0.01).

CONCLUSIONS

Patients with EYS-associated RP exhibited inferior-dominant photoreceptor death and the relative ONL preservation under retinal vessels. These results suggest that longitudinal environment light exposure may be correlated with the photoreceptor death.

摘要

目的

我们使用频域光相干断层扫描(SD-OCT)图像研究色素性视网膜炎(RP)中的光诱导感光器损伤。

方法

我们回顾性分析了具有 EYS 相关 RP 基因诊断的患者。在 SD-OCT 垂直扫描上测量视网膜血管下的外核层(ONL)厚度。作为对照,我们测量血管上下 100μm 处相邻 ONL 厚度。对健康受试者进行相同的测量。我们计算了血管下 ONL 厚度与相邻 ONL 厚度平均值的比值,并定义为 ONL 保留率。在 RP 患者中,还使用 SD-OCT 垂直扫描测量从黄斑中心凹开始的椭圆体区(EZ)的长度。

结果

共纳入 30 名 EYS 相关 RP 患者和 25 名健康受试者。在两组中,视网膜血管下的 ONL 厚度与相邻区域无显著差异。然而,RP 患者的上、下视网膜的 ONL 保留率均高于健康对照组(1.03 比 0.97;p<0.01,1.15 比 0.95;p<0.01,分别)。在 RP 中,下视网膜的 ONL 保留率明显大于上视网膜(p<0.01)。此外,在 RP 患者中,黄斑中心凹的 EZ 长度总是在下视网膜比上视网膜短,且存在显著差异(p<0.01)。

结论

EYS 相关 RP 患者表现为下优势感光器死亡和视网膜血管下相对的 ONL 保留。这些结果表明,纵向环境光暴露可能与感光器死亡有关。