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Tobias Duncker, Jonathan P. Greenberg, Theodore Smith, Stephen H. Tsang, François C. Delori; Quantitative Autofluorescence (qAF) in Best Vitelliform Macular Dystrophy. Invest. Ophthalmol. Vis. Sci. 2012;53(14):2683.
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To compare quantitative measurements obtained from fundus autofluorescence images in Best disease to normal controls.
10 patients with electrooculography confirmed Best disease (5-57 years, mean 31±21 years) were studied, including 4 subclinical cases. Autofluorescence (AF) images were acquired with the Spectralis SLO-OCT (Heidelberg Eng.) modified by insertion of an internal AF reference to account for variable laser power and detector sensitivity [Delori et al, IOVS, 2011]. Maximum qAF within the central 30° field was measured in 1 eye per patient, both for normal appearing background retina and, if present, within the macular lesion (Figure 1). The results were compared to a fixed temporal region in normal controls (1 eye per subject), which generally represented the highest qAF levels in this group.
The background retina from 8 out of 10 patients, including 2 out of 4 subclinical cases, showed qAF values significantly above the 95%CI of age similar controls (Figure 2). Macular lesions always exhibited the highest qAF readings within the 30° field, with up to 7 times higher qAF levels than maximum background retina.
The background retina in Best patients shows high maximum qAF levels compared to age similar controls. This suggests increased lipofuscin accumulation outside the visible macular lesions. qAF may enhance our understanding of Best disease, and aid in the identification of subclinical cases.
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