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KG Locke, GE Fish, DH Wheaton, DG Birch, DR Hoffman; Fundus Appearance in X-linked Retinitis Pigmentosa (XLRP) Over a 4-Year-Interval . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1171.
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Purpose: Patients with XLRP have been followed in a double-blind, clinical trial to assess the degree to which docosahexaenoic acid (DHA) supplementation can elevate blood lipid DHA levels and influence progression of disease. This study evaluates yearly fundus photographs as an index of progression. Methods: Yearly fundus photos (7 standard views + nasal view) were taken of both eyes with a 60° camera in 44 patients with XLRP (age: 16 ± 9 yrs). Slides from initial visit (year 0) and year 4 were arranged for dual projection with eye and retinal view matched. Year 0 and year 4 were randomly switched between the two projectors. All authors, with exception of KGL, were masked and required to identify year 4 slides. Each examiner independently graded the change in vessel caliber and pigmentation on a scale from 0 (no change) to 3 (major change with multiple areas of new pigment). Results: Year 4 photographs were correctly identified in 42 of 44 (95%) patients. A change in vessel caliber and/or increased pigmentary abnormalities were noted in 37 of 44 (84%) patients, and 16 of 44 (36%) received a score of 3 (major change). The median score over 4 years of follow-up was 2.2. There was a significant (Spearman R = 0.39; p < 0.01) correlation between age and change in fundus appearance. Conclusion: Fundus photography over a 4-year-interval reflects the progression of retinal degeneration in the majority of patients with XLRP. Contrary to many functional measures, changes over 4 years in fundus appearance are most evident in the older individuals. Clear change over a relatively short time interval makes fundus appearance a potentially valuable ancillary measure for clinical trials in XLRP.
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