Baseline Visual Field Findings in the RUSH2A Study: Associated Factors and Correlation With Other Measures of Disease Severity.,American Journal of Ophthalmology

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Baseline Visual Field Findings in the RUSH2A Study: Associated Factors and Correlation With Other Measures of Disease Severity.
American Journal of Ophthalmology ( IF 4.2 ) Pub Date : 2020-05-22 , DOI: 10.1016/j.ajo.2020.05.024
Jacque L Duncan ₁ , Wendi Liang ₂ , Maureen G Maguire ₃ , Isabelle Audo ₄ , Allison R Ayala ₂ , David G Birch ₅ , Joseph Carroll ₆ , Janet K Cheetham ₇ , Simona Degli Esposti ₈ , Todd A Durham ₇ , Laura Erker ₉ , Sina Farsiu ₁₀ , Frederick L Ferris ₁₁ , Elise Heon ₁₂ , Robert B Hufnagel ₁₃ , Alessandro Iannaccone ₁₀ , Glenn J Jaffe ₁₀ , Christine N Kay ₁₄ , Michel Michaelides ₈ , Mark E Pennesi ₉ , José-Alain Sahel ₁₅ ,

Affiliation

University of California, San Francisco, San Francisco, CA, USA.
Jaeb Center for Health Research, Tampa, FL, USA.
University of Pennsylvania, Philadelphia, PA, USA.
Institut de la Vision, Sorbonne Université, INSERM, CNRS, Paris, France; Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, INSERM-DGOS CIC1423, Paris, France.
Retina Foundation of the Southwest, Dallas, TX, USA.
Medical College of Wisconsin Eye Institute, Milwaukee, WI, USA.
Foundation Fighting Blindness, Columbia, MD, USA.
Moorfields Eye Hospital and UCL Institute of Ophthalmology, London, United Kingdom.
Casey Eye Institute-Oregon Health & Science University, Portland, OR, USA.
Department of Ophthalmology, Duke University, Durham, NC, USA.
Ophthalmic Research Consultants, Waxhaw, NC, USA.
Departments of Ophthalmology and Vision Sciences, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario, Canada.
National Eye Institute, Bethesda, MD, USA.
Vitreoretinal Associates, Gainesville, FL, USA.
Institut de la Vision, Sorbonne Université, INSERM, CNRS, Paris, France; Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, INSERM-DGOS CIC1423, Paris, France; Department of Ophthalmology, The University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.

Purpose

To report baseline visual fields in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study.

Design

Cross-sectional study within a natural history study.

Methods

Setting: multicenter, international. Study population: Usher syndrome type 2 (USH2) (n = 80) or autosomal recessive nonsyndromic retinitis pigmentosa (ARRP) (n = 47) associated with biallelic disease-causing sequence variants in USH2A. Observation procedures: Repeatability of full-field static perimetry (SP) and between-eye symmetry of kinetic perimetry (KP) were evaluated with intraclass correlation coefficients (ICCs). The association of demographic and clinical characteristics with total hill of vision (V_TOT) was assessed with general linear models. Associations between V_TOT and other functional and morphologic measures were assessed using Spearman correlation coefficients and t tests. Main outcome measures: V_TOT (SP) and III4e isopter area (KP).

Results

USH2 participants had more severe visual field loss than ARRP participants (P < .001, adjusting for disease duration, age of enrollment). Mean V_TOT measures among 3 repeat tests were 32.7 ± 24.1, 31.2 ± 23.4, and 31.7 ± 23.9 decibel-steradians (intraclass correlation coefficient [ICC] = 0.96). Better VA, greater photopic ERG 30-Hz flicker amplitudes, higher mean microperimetry sensitivity, higher central subfield thickness, absence of macular cysts, and higher III4e seeing area were associated with higher V_TOT (all r > .48; P < .05). Mean III4e isopter areas for left (4561 ± 4426 squared degrees) and right eyes (4215 ± 4300 squared degrees) were concordant (ICC = 0.94).

Conclusions

USH2 participants had more visual field loss than participants with USH2A-related ARRP, adjusting for duration of disease and age of enrollment. V_TOT was repeatable and correlated with other functional and structural metrics, suggesting it may be a good summary measure of disease severity in patients with USH2A-related retinal degeneration.

中文翻译：

RUSH2A 研究中的基线视野发现：相关因素和与其他疾病严重程度测量的相关性。

目的

报告USH2A相关视网膜变性 (RUSH2A) 研究进展率的基线视野。

设计

自然史研究中的横断面研究。

方法

环境：多中心、国际化。研究人群：与 USH2A 中双等位基因致病序列变异相关的 Usher 综合征 2 型 ( USH2 ) (n = 80) 或常染色体隐性遗传非综合征性色素性视网膜炎 (ARRP) (n = 47) 。观察程序：用组内相关系数 (ICCs) 评估全场静态视野 (SP) 的重复性和动态视野 (KP) 的眼间对称性。使用一般线性模型评估人口统计学和临床特征与总视野 (V _{TOT ) 的关联。}使用 Spearman 相关系数和t检验评估V _TOT与其他功能和形态测量之间的关联。主要结果测量：V _TOT(SP) 和 III4e 等视点区域 (KP)。

结果

USH2 参与者的视野丧失比 ARRP 参与者更严重（P < .001，调整了疾病持续时间、入组年龄）。3 次重复测试中的平均 V _TOT测量值为 32.7 ± 24.1、31.2 ± 23.4 和 31.7 ± 23.9 分贝球面度（组内相关系数 [ICC] = 0.96）。更好的 VA、更大的明视 ERG 30-Hz 闪烁幅度、更高的平均显微视野灵敏度、更高的中央亚视野厚度、没有黄斑囊肿和更高的 III4e 视野与更高的 V _TOT相关（所有r > .48；P < .05） . 左眼（4561 ± 4426 平方度）和右眼（4215 ± 4300 平方度）的平均 III4e 等视点面积一致（ICC = 0.94）。