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. 2024 Oct 9;14:23629. doi: 10.1038/s41598-024-74088-y

Table 4.

Retinal structure and function at the preferred retinal locus in participants with Stargardt disease.

Participant Eye PRL distance from fovea (°) Average PRL sensitivity [SD] (dB) 63% BCEA (deg2) SW-FAF NI-FAF SD-OCT
ELM EZ RPE/Bruch’s
S01 OD 3.11 1.5 [2.6] 7.00 Hypo-AF N/A Absent Absent Absent
OS 0.01 17.0 [2.2] 1.20 Hypo-AF N/A Present Present Present
S02 OD 0.04 2.0 [3.5] 0.30 Hypo-AF N/A Absent Absent Present
OS 0.03 1.0 [1.7] 0.20 Hypo-AF N/A Absent Absent Present
S03 OD 0.04 0.3 [0.4] 1.60 Hypo-AF N/A Absent Absent Present
OS 0.03 5.3 [6.9] 1.30 Normal N/A Present Present Present
S04 OD 0.03 26.0 [0] 0.50 Normal Hypo-AF Present Present Present
OS 0.09 24.5 [6.1] 0.60 Normal Hypo-AF Present Present Present
S05 OD 4.42 21.8 [2.4] 0.80 Hypo-AF N/A Absent Absent Present
OS 1.97 10.5 [10.5] 3.60 Hypo-AF N/A Absent Absent Present
S06 OD 0.02 3.8 [6.5] 0.70 Hypo-AF Hypo-AF Absent Absent Present
OS 1.91 13.5 [13.5] 1.40 Hypo-AF Hypo-AF Present Present Present
S07 OD 4.13 23.0 [1.4] 4.70 Normal N/A Present Present Present
OS 2.94 21.0 [2.8] 3.10 Hypo-AF N/A Present Present Present
S08 OD 8.06 20.0 [2.2] 5.50 Hypo-AF N/A Present Present Present
OS 8.66 20.5 [3.0] 5.70 Hypo-AF N/A N/A N/A N/A
S09 OD 2.56 20.0 [7.1] 1.80 Hypo-AF N/A Present Present Present
OS 0.30 16.0 [4.2] 1.20 Hypo-AF N/A Present Absent Present
S10 OD 5.30 19.0 [1.0] 4.30 Normal Normal Present Present Present
OS 5.90 19.5 [2.6] 3.80 Normal Normal Present Present Present
S11 OD 21.35 13.0 [3.7] 14.90 Hypo-AF Hypo-AF Present Absent Present
OS 16.81 N/A 6.40 Hypo-AF Hypo-AF Present Absent Present
S12 OD 5.53 17.3 [10.1] 4.50 Normal Normal Present Present Present
OS 5.96 31.5 [4.5] 4.90 Normal Normal Present Present Present
S13 OD 10.30 22.0 [3.7] 7.60 Normal Normal Present Present Present
OS 9.64 23.5 [1.7] 6.20 Hyper-AF Hyper-AF Present Present Present
S14 OD 4.79 0.5 [0.9] 7.60 Hypo-AF Hypo-AF Absent Absent Present
OS 6.62 14.0 [5.1] 3.10 Hypo-AF Hypo-AF Absent Absent Present
S15 OD 9.06 15.0 [7.1] 1.50 Normal Normal Present Present Present
OS 8.26 18.5 [4.3] 3.40 Hypo-AF Hypo-AF Present Present Present
S16 OD 10.12 17.0 [1.0] 10.00 Hypo-AF Hypo-AF Present Present Present
OS 8.36 16.5 [0.9] 12.20 Hyper-AF Hypo-AF N/A N/A N/A

AF autofluorescence, ELM external limiting membrane, EZ ellipsoid zone, NI-FAF near-infrared fundus autofluorescence, OD right eye, OS left eye, PRL preferred retinal locus, RPE retinal pigment epithelium, SD-OCT spectral-domain optical coherence tomography, SW-FAF short-wavelength fundus autofluorescence. The median PRL eccentricity was 4.60° from the foveal centre (IQR 7.45°, range 0.09°–21.35°), median 63% BCEA was 3.5 deg2 (IQR 4.6 deg2, range 0.2 deg2–14.9 deg2), and median PRL sensitivity was 17.0 dB (IQR 9.0 dB, range 0.3–31.5 dB). Spearman’s correlation coefficients between participants’ right and left eyes were 0.90 (p < 0.001) for PRL eccentricity, 0.69 (p = 0.003) for 63% BCEA, 0.89 (p < 0.001) for retinal sensitivity, and 0.62 (p = 0.013) for PRL sensitivity.