May 2005
Volume 46, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2005
CRB1– Associated Retinal Degeneration: Macular Pigment and Lutein Supplementation
Author Affiliations & Notes
  • T.S. Aleman
    Department of Ophthalmology, Scheie Eye Institute, Philadelphia, PA
  • E.A. M. Windsor
    Department of Ophthalmology, Scheie Eye Institute, Philadelphia, PA
  • S.B. Schwartz
    Department of Ophthalmology, Scheie Eye Institute, Philadelphia, PA
  • A. Sumaroka
    Department of Ophthalmology, Scheie Eye Institute, Philadelphia, PA
  • A.Y. Cheung
    Department of Ophthalmology, Scheie Eye Institute, Philadelphia, PA
  • E.E. Smilko
    Department of Ophthalmology, Scheie Eye Institute, Philadelphia, PA
  • A.V. Cideciyan
    Department of Ophthalmology, Scheie Eye Institute, Philadelphia, PA
  • S.G. Jacobson
    Department of Ophthalmology, Scheie Eye Institute, Philadelphia, PA
  • Footnotes
    Commercial Relationships  T.S. Aleman, None; E.A.M. Windsor, None; S.B. Schwartz, None; A. Sumaroka, None; A.Y. Cheung, None; E.E. Smilko, None; A.V. Cideciyan, None; S.G. Jacobson, None.
  • Footnotes
    Support  NIH EY0– 13385, –13729, –13203, FFB, Macula Vision Res Fndn, Macular Disease Fndn.
Investigative Ophthalmology & Visual Science May 2005, Vol.46, 556. doi:
  • Views
  • Share
  • Tools
    • Alerts
      ×
      This feature is available to authenticated users only.
      Sign In or Create an Account ×
    • Get Citation

      T.S. Aleman, E.A. M. Windsor, S.B. Schwartz, A. Sumaroka, A.Y. Cheung, E.E. Smilko, A.V. Cideciyan, S.G. Jacobson; CRB1– Associated Retinal Degeneration: Macular Pigment and Lutein Supplementation . Invest. Ophthalmol. Vis. Sci. 2005;46(13):556.

      Download citation file:


      © ARVO (1962-2015); The Authors (2016-present)

      ×
  • Supplements
Abstract

Abstract: : Purpose: Crumbs homologue 1 (CRB1) mutations are associated with thick, coarsely–laminated retina and severe visual loss. A subset of patients can have relatively preserved central vision. Murine and Drosophila evidence suggests a detrimental effect of light on CRB1–associated retinal degeneration. Seeking means to prevent central visual loss in these patients, we studied their macular pigment (MP) and vision and the response to oral lutein supplementation. Methods: Patients with CRB1 mutations (n=2) and relatively preserved visual acuities (at least 20/50) were included. Both subjects (in the second decade of life) had stable foveal fixation. MP optical density was measured with heterochromatic flicker photometry. MP, serum carotenoids, visual acuity, foveal sensitivity and retinal thickness (by optical coherence tomography, OCT) were quantified at two baseline visits (separated by <1 month) and following supplementation with lutein at 20 mg/day for 6 months. Results: Patients had preserved foveal architecture by OCT but the surrounding retina was abnormally thick and coarsely–laminated. MP density at baseline was within normal limits in one patient and reduced in the second. Foveal thickness was related to MP levels. After supplementation, both patients showed increases in serum lutein and MP density. MP change with supplementation was gradual and varied in magnitude between individuals. Central vision remained unchanged after 6 months of supplementation. Conclusions: MP density was measurable in two patients with CRB1–associated retinal degeneration and could be augmented by supplemental lutein. Central vision was unchanged after 6 months of supplementation. Long–term influences of lutein supplementation on the natural history of this retinal degeneration warrant study.

Keywords: retinal degenerations: hereditary • degenerations/dystrophies • macular pigment 
×
×

This PDF is available to Subscribers Only

Sign in or purchase a subscription to access this content. ×

You must be signed into an individual account to use this feature.

×