Gyrate Atrophy of the Choroid and Retina
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).
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