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Gyrate Atrophy of the Choroid and Retina

2002; American Medical Association; Volume: 120; Issue: 2 Linguagem: Inglês

10.1001/archopht.120.2.146

1538-3601

Muriel I. Kaiser‐Kupfer, Rafael C. Caruso, David Solá-Del Valle,

Glaucoma and retinal disorders

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.Natural history study of 2 pairs of siblings with gyrate atrophy treated with an arginine-restricted diet.Fundus photography and electrophysical and psychophysical retinal function tests.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.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.