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C.I. Schupp, C. Gerth, E. Olano-Martin, B.M. Morrissey, C.E. Cross, J.S. Werner; Low Macular Pigment but Normal Visual Function in Adult Cystic Fibrosis Patients . Invest. Ophthalmol. Vis. Sci. 2003;44(13):488.
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Purpose: To study the impact of poor carotenoid absorption on vision in a population of adult cystic fibrosis patients by assessing the amount of macular pigment, serum levels of lutein and zeaxanthin as well as three aspects of visual function. Subjects and methods: 10 cystic fibrosis patients (age 31.5 ± 3.1 y) and ten control subjects (age 32.2 ± 3.4 y) were matched for age, sex and ethnicity. All received a clinical eye examination. Macular pigment optical density was measured by heterochromatic flicker photometry from 15 min to 2 deg retinal eccentricity, and a non-fasting blood sample was drawn for HPLC analysis of lutein and zeaxanthin. Functional tests included photopic contrast sensitivity (spatial frequencies from 0.55 to 18 cpd), color discrimination (the Cambridge Colour Test), and the multifocal electroretinogram (mfERG). Results: Cystic fibrosis patients showed significantly lower serum carotenoid levels and macular pigment optical density than the control group. We found a significant positive correlation (r (L) = 0.74, r (Z) = 0.78) between serum concentration of the two xanthophylls and macular pigment optical density for both experimental groups. We did not find any significant differences between patients and controls for photopic contrast sensitivity or color discrimination along protan, deutan and tritan confusion lines. The mfERG revealed normal response densities in all patients and delayed implicit time N1 and P1 in 2 of 9 patients using the concentric ring analysis (one recording was excluded from the analysis due to low signal-to-noise ratio). Conclusion: Low macular pigment optical densities are consistent with the decreased level of serum lutein and zeaxanthin in the tested CF patients and reflect their complex fat malabsorption syndrome. Despite their generally poor health, CF patients enjoy surprisingly good ocular health and visual function.
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