期刊
RETINA-THE JOURNAL OF RETINAL AND VITREOUS DISEASES
卷 38, 期 12, 页码 2401-2414出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/IAE.0000000000001871
关键词
fundus autofluorescence; retinal diseases; retinitis pigmentosa; RPGR
资金
- National Institute for Health Research Biomedical Research Centre at Moorfields Eye Hospital National Health Service Foundation Trust
- UCL Institute of Ophthalmology (United Kingdom)
- Fight For Sight (United Kingdom)
- Moorfields Eye Hospital Special Trustees (United Kingdom)
- Moorfields Eye Charity (United Kingdom)
- Foundation Fighting Blindness (USA)
- Retinitis Pigmentosa Fighting Blindness (United Kingdom)
- Wellcome Trust [099173/Z/12/Z]
- FFB Career Development Award
Purpose: Quantitative analysis of hyperautofluorescent rings and progression in subjects with retinitis pigmentosa associated with retinitis pigmentosa GTPase regulator (RPGR) gene mutations. Methods: Prospective observational study of 46 subjects. Ring area, horizontal and vertical diameter measurements taken from outer and inner ring borders. Intraobserver repeatability, baseline measurements, progression rates, interocular symmetry, and association with age and genotype were investigated. Results: Baseline ring area was 11.8 +/- 13.4 mm(2) and 11.4 +/- 13.2 mm(2) for right and left eyes, respectively, with very strong interocular correlation (r = 0.9398; P < 0.0001). Ring area constriction was 1.5 +/- 2.0 mm(2)/year and 1.3 +/- 1.9 mm(2)/year for right and left eyes, respectively, with very strong interocular correlation (r = 0.878, P < 0.0001). Baseline ring area and constriction rate correlated negatively with age (r = 20.767; P < 0.0001 and r = 20.644, P < 0.0001, respectively). Constriction rate correlated strongly with baseline area (r = 0.850, P < 0.0001). Age, but not genotype, exerted a significant effect on constriction rates (P < 0.0001), with greatest rates of progression seen in younger subjects. An exponential decline overall was found. Conclusion: This study provides disease-specific baseline values and progression rates together with a repeatability assessment of fundus autofluorescence metrics. Our findings can guide future treatment trials and contribute to the clinical care of patients with RPGR-associated retinitis pigmentosa.
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