Table 3.
The results of the data analysis on patients with cone/cone-dystrophy (CDs/CRDs) included in the study.
| RPGRORF15 | |
|---|---|
| Male, No./Total No. (%) | 36/36 (100) |
| Age of Onset | n = 18 |
| 1st decade | 4 |
| 2nd decade | 9 |
| 3rd decade | 4 |
| ≥4th decade | 1 |
| High Myopia, No./Total No. (%) | 14/33 (42.42) |
| Cataract and/or previous cataract surgery in at least one eye, No./Total No. (%) | 4/33 (12.12) |
| ǂ CD/CRD, No/No | 15/21 |
| Age at last visit, mean ± SD, y | n = 36; 43.97 ± 11.24 |
| Light perception or no light perception in at least one eye, No./total No. (%) |
1/35 (2.86) |
| § BCVA at last visit, LogMar, Mean ± SD [Snellen Equivalent] | n = 35; 0.97 ± 0.71 [20/200] |
| Follow-up BCVA, No | n = 35 [range: 0–19 y] |
| 0 y | 11 |
| 1–5 y | 14 |
| 6–10 y | 9 |
| >10 y | 1 |
| Estimation of BCVA decline | n = 24 |
| Annual rate, LogMAR/year ± SD | 0.04 ± 0.06 |
| Mean regression slope ± SD | 0.04 ± 0.08 |
| Mean regression intercept ± SD | −0.90 ± 3.34 |
| Binocular normal color vision, No./Total No. (%) | 1/32 (3.12) |
| Bilateral undetectable ff-ERG, No./Total No. (%) | 5/36 (13.89) |
| SW-FAF Phenotype type | n = 35 |
| Group 1 | 6 |
| Group 2 | 21 |
| Group 3 | 8 |
| Peripapillary sparing, No./Total No. (%) | 10/35 (28.57) |
| Estimation of central hyperAF ring enlargement | n = 19 |
| Horizontal diameter, µm/y | 42.88 ± 48.98 |
| Slope | 43.16 ± 47.81 |
| Intercept | 228.60 ± 1689.24 |
| Vertical diameter, µm/y | 39.44 ± 40.90 |
| Slope | 39.36 ± 40.57 |
| Intercept | −24.16 ± 1658.86 |
| CRT, µm, mean ± SD | n = 35; 148.97 ± 27.17 |
| Unilateral or bilateral ERM, No./Total No. (%) | 2/35 (2.78) |
| Unilateral or bilateral iHRF, No./Total No. (%) | 15/35 (42.86) |
No.—Number; SD—Standard deviation; BCVA—Best corrected visual acuity; VF—Visual field; iHRF—Intraretinal hyper-reflective foci; ERM—Epiretinal membrane; ff-ERG—Full field electroretinogram; SW-FAF—Short-wavelength fundus autofluorescence; OCT—Optical coherence tomography; CRT—Central retinal thickness. ǂ—Definition of CDs and CRDs was based on the ff-ERG; §—For BCVA calculation, patients with or without light perception were excluded as this is not possible to convert to LogMar. Counting fingers and hand motions were converted to 2 and 3 LogMar, respectively [24].