Details

Autor(en) / Beteiligte

• Jolly, Jasleen K
• Groppe, Markus
• Birks, Jacqueline
• Downes, Susan M
• MacLaren, Robert E

Titel

Functional Defects in Color Vision in Patients With Choroideremia

Ist Teil von

• American journal of ophthalmology, 2015-10, Vol.160 (4), p.822-831.e3

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2015

Link zum Volltext

Quelle

Elsevier ScienceDirect Journals Complete

Beschreibungen/Notizen

• Purpose To characterize defects in color vision in patients with choroideremia. Design Prospective cohort study. Methods Thirty patients with choroideremia (41 eyes) and 10 age-matched male controls (19 eyes) with visual acuity of ≥6/36 attending outpatient clinics in Oxford Eye Hospital underwent color vision testing with the Farnsworth-Munsell 100 hue test, visual acuity testing, and autofluorescence imaging. To exclude changes caused by degeneration of the fovea, a subgroup of 14 patients with a visual acuity ≥6/6 was analyzed. Calculated color vision total error scores were compared between the groups and related to a range of factors using a random-effects model. Results Mean color vision total error scores were 120 (95% confidence interval [CI] 92, 156) in the ≥6/6 choroideremia group, 206 (95% CI 161, 266) in the <6/6 visual acuity choroideremia group, and 47 (95% CI 32, 69) in the control group. Covariate analysis showed a significant difference in color vision total error score between the groups ( P < .001 between each group). Conclusions Patients with choroideremia have a functional defect in color vision compared with age-matched controls. The color vision defect deteriorates as the degeneration encroaches on the fovea. The presence of an early functional defect in color vision provides a useful biomarker against which to assess successful gene transfer in gene therapy trials.