학술논문
Baseline Visual Field Findings in the RUSH2A Study: Associated Factors and Correlation With Other Measures of Disease Severity
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article
Author
Duncan, Jacque L; Liang, Wendi; Maguire, Maureen G; Audo, Isabelle; Ayala, Allison R; Birch, David G; Carroll, Joseph; Cheetham, Janet K; Degli Esposti, Simona; Durham, Todd A; Erker, Laura; Farsiu, Sina; Ferris, Frederick L; Heon, Elise; Hufnagel, Robert B; Iannaccone, Alessandro; Jaffe, Glenn J; Kay, Christine N; Michaelides, Michel; Pennesi, Mark E; Sahel, José-Alain; Group, Foundation Fighting Blindness Consortium Investigator
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Abstract
PurposeTo report baseline visual fields in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study.DesignCross-sectional study within a natural history study.MethodsSetting: multicenter, international.Study populationUsher 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 proceduresRepeatability 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 (VTOT) was assessed with general linear models. Associations between VTOT and other functional and morphologic measures were assessed using Spearman correlation coefficients and t tests.Main outcome measuresVTOT (SP) and III4e isopter area (KP).ResultsUSH2 participants had more severe visual field loss than ARRP participants (P < .001, adjusting for disease duration, age of enrollment). Mean VTOT 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 VTOT (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).ConclusionsUSH2 participants had more visual field loss than participants with USH2A-related ARRP, adjusting for duration of disease and age of enrollment. VTOT 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.