This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (6)  • Contact me when this article is cited  Related Content  • Related article  • Similar articles in this journal  Topic Collections  • Ophthalmology, Other  • Articles for Residents  • Alert me on articles by topic  Social Bookmarking   What's this?

Further Experience With Long-term Reduction of Ornithine Levels in Children

Muriel I. Kaiser-Kupfer, MD; Rafael C. Caruso, MD; David Valle, MD

Arch Ophthalmol. 2002;120:146-153.

Objective  To determine whether the long-term reduction of plasma ornithine levels by way of an arginine-restricted diet in patients with gyrate atrophy will slow the progression of this chorioretinal degeneration.

Design  Natural history study of 2 pairs of siblings with gyrate atrophy treated with an arginine-restricted diet.

Main Outcome Measures  Fundus photography and electrophysical and psychophysical retinal function tests.

Results  After 16 to 17 years of receiving an arginine-restricted diet, the younger sibling in each pair, who was prescribed the diet at an earlier age than the older sibling, demonstrated a slower progression of lesions compared with the older sibling.

Conclusions  If started at an early age, long-term substantial reduction of plasma ornithine levels may appreciably slow the progression of the chorioretinal lesions and, to a lesser extent, the progressive loss of retinal function in patients with gyrate atrophy.

From the National Eye Institute, National Institutes of Health, Bethesda, Md (Drs Kaiser-Kupfer and Caruso); and the McKusick-Nathans Institute of Genetic Medicine and Howard Hughes Medical Institutes, Johns Hopkins University School of Medicine, Baltimore, Md (Dr Valle).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

RELATED ARTICLE

Archives of Ophthalmology Reader's Choice: Continuing Medical Education
Arch Ophthalmol. 2002;120(2):243.
FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Myopathy with tubular aggregates and gyrate atrophy of the choroid and retina due to hyperornithinaemia
Fleury et al.
J. Neurol. Neurosurg. Psychiatry 2007;78:656-657.
FULL TEXT  

Cytotoxic Effect of Spermine on Retinal Pigment Epithelial Cells
Kaneko et al.
IOVS 2007;48:455-463.
ABSTRACT | FULL TEXT  

Use of an Arginine-Restricted Diet to Slow Progression of Visual Loss in Patients With Gyrate Atrophy
Kaiser-Kupfer et al.
Arch Ophthalmol 2004;122:982-984.
ABSTRACT | FULL TEXT