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Science 5 December 1980:
Vol. 210. no. 4474, pp. 1128 - 1131
DOI: 10.1126/science.7444439
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Articles

Science, Vol 210, Issue 4474, 1128-1131
Copyright © 1980 by American Association for the Advancement of Science

articles

Gyrate atrophy of the choroid and retina: improved visual function following reduction of plasma ornithine by diet

MI Kaiser-Kupfer, FM de Monasterio, D Valle, M Walser, and S Brusilow

In a patient with gyrate atrophy of the choroid and retina, an arginine-deficient diet has reduced plasma ornithine concentration fivefold during the past 20 months. Subjective improvement in her visual function was noted approximately 15 months after institution of her diet. This has been documented by improvements in the electroretinogram, dark-adaptation, and color vision. The improvement involves rod and, to a lesser extent, cone function. The results, although preliminary and limited to a single patient, suggest that reduction of plasma ornithine with a low arginine diet is beneficial in this disease.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Gyrate Atrophy of the Choroid and Retina: Further Experience With Long-term Reduction of Ornithine Levels in Children.
M. I. Kaiser-Kupfer, R. C. Caruso, and D. Valle (2002)
Arch Ophthalmol 120, 146-153
   Abstract »    Full Text »    PDF »
Correction of ornithine accumulation prevents retinal degeneration in a mouse model of gyrate atrophy of the choroid and retina.
T. Wang, G. Steel, A. H. Milam, and D. Valle (2000)
PNAS 97, 1224-1229
   Abstract »    Full Text »    PDF »
Ophthalmology in the post-genomic era.
G C M BLACK, M E BOULTON, P N BISHOP, and D McLEOD (1999)
Br J Ophthalmol 83, 1215-1218
   Full Text »
Gyrate Atrophy of the Choroid and Retina: Long-term Reduction of Ornithine Slows Retinal Degeneration.
M. I. Kaiser-Kupfer, R. C. Caruso, and D. Valle (1991)
Arch Ophthalmol 109, 1539-1548
   Abstract »    PDF »
Retinal dystrophy: development retarded by galactose feeding in spontaneously hypertensive rats.
R. Frank, R. Keirn, G. Keirn, M. Mancini, and J. Khoury (1986)
Science 231, 376-378
   Abstract »    PDF »


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