Functional defects in color vision in patients with choroideremia
<br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Purpose: </strong>To characterize defects in color vision in patients with choroideremia.</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Design: </s...
| Main Authors: | , , , , |
|---|---|
| Format: | Journal article |
| Language: | English |
| Published: |
Elsevier
2015
|
| _version_ | 1797073835757928448 |
|---|---|
| author | Jolly, J Groppe, M Birks, J Downes, S MacLaren, R |
| author_facet | Jolly, J Groppe, M Birks, J Downes, S MacLaren, R |
| author_sort | Jolly, J |
| collection | OXFORD |
| description | <br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Purpose: </strong>To characterize defects in color vision in patients with choroideremia.</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Design: </strong>Prospective cohort study.</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Methods: </strong>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.</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Results: </strong>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).</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Conclusions: </strong>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.</br> |
| first_indexed | 2024-03-06T23:27:44Z |
| format | Journal article |
| id | oxford-uuid:6af964c4-3395-4b9b-bb67-33038d7e575b |
| institution | University of Oxford |
| language | English |
| last_indexed | 2024-03-06T23:27:44Z |
| publishDate | 2015 |
| publisher | Elsevier |
| record_format | dspace |
| spelling | oxford-uuid:6af964c4-3395-4b9b-bb67-33038d7e575b2022-03-26T19:00:48ZFunctional defects in color vision in patients with choroideremiaJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:6af964c4-3395-4b9b-bb67-33038d7e575bEnglishSymplectic Elements at OxfordElsevier2015Jolly, JGroppe, MBirks, JDownes, SMacLaren, R<br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Purpose: </strong>To characterize defects in color vision in patients with choroideremia.</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Design: </strong>Prospective cohort study.</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Methods: </strong>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.</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Results: </strong>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).</br><br xmlns:etd="http://www.ouls.ox.ac.uk/ora/modsextensions"><strong>Conclusions: </strong>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.</br> |
| spellingShingle | Jolly, J Groppe, M Birks, J Downes, S MacLaren, R Functional defects in color vision in patients with choroideremia |
| title | Functional defects in color vision in patients with choroideremia |
| title_full | Functional defects in color vision in patients with choroideremia |
| title_fullStr | Functional defects in color vision in patients with choroideremia |
| title_full_unstemmed | Functional defects in color vision in patients with choroideremia |
| title_short | Functional defects in color vision in patients with choroideremia |
| title_sort | functional defects in color vision in patients with choroideremia |
| work_keys_str_mv | AT jollyj functionaldefectsincolorvisioninpatientswithchoroideremia AT groppem functionaldefectsincolorvisioninpatientswithchoroideremia AT birksj functionaldefectsincolorvisioninpatientswithchoroideremia AT downess functionaldefectsincolorvisioninpatientswithchoroideremia AT maclarenr functionaldefectsincolorvisioninpatientswithchoroideremia |