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C. Brue, A. Giovannini, S. Salvolini, C. Mariotti; Resonance Raman Spectroscopic Measurement of Macular Carotenoids in Best’s Disease. Invest. Ophthalmol. Vis. Sci. 2009;50(13):1718.
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Best’s disease (BD) is an autosomal dominantly inherited disorder with variable penetrance, that primarly affects the RPE. The retinopathy is caracterized by the variable deposition of yellowish material, attributed to lipofucsin in the retinal pigment epithelium (RPE). The phenotypic appearance varies with the stage of the disease.Light-adsorbing macular carotenoids (MCs),i.e. lutein and zeaxantin, are efficient free radical scavengers in human retina and might play a role in the wellness of the RPE in BD. The present study aimed at verifying whether MCs levels correlate with the stages of progression of the disease and if they can be predictive of subsequent visual outcome.
We examined retrospectively 30 consecutive eyes of 30 patients of 30-50 years old (mean age 45.6 years) that were divided in four matched groups: i) control (negative for BD, n=6); and ii) BD group (n=6) in the vitelliform stage, (n=6) in the pseudoypopion stage, (n=6) in the scrambled-egg stage and (n=6) in the atrophic stage. All patients underwent fluorescein angiography, visual field and the electrooculogram. The levels of MCs were measured by a resonance Raman spectroscope in all of the subjects. Both the eyes of each subject were monitored and triplicate measurements were performed at each time. Moreover, pupil dilatation, instrument self-adjustment and appropriate eyeglasses correction were used.
MCs levels, measured as Raman counts, were 1990 (250), 1730 (205), 1800 (300), 1600 (270), 1370 (220) in the control, vitelliform, pseudoypopion, scrambled-egg and atrophic group respectively. The difference among the groups was statistically significant ( p<0.05).
A role of MCs in the pathogenesis and stages of Best disease is proposed herein and future investigations are warranted to further clarify these aspects and to test their possible clinical use in the treatment of this pathology.
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