Gyrate atrophy of the choroid and retina: further experience with long-term reduction of ornithine levels in children.

Abstract

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.

• Authors: Muriel I Kaiser-Kupfer, Rafael C Caruso, David Valle
• Journal: Archives of ophthalmology (Chicago, Ill. : 1960) (Arch Ophthalmol) Vol. 120 Issue 2 Pg. 146-53 (Feb 2002) ISSN: 0003-9950 [Print] United States
• PMID: 11831916 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Arginine
• Ornithine

Topics

• Adult
• Arginine (administration & dosage)
• Choroid Diseases (blood, diet therapy, physiopathology)
• Diet, Protein-Restricted
• Disease Progression
• Electroretinography
• Female
• Fundus Oculi
• Gyrate Atrophy (blood, diet therapy, physiopathology)
• Humans
• Male
• Nuclear Family
• Ornithine (blood)
• Pedigree
• Photography
• Retinal Diseases (blood, diet therapy, physiopathology)
• Visual Acuity
• Visual Fields