Archives of ophthalmology (Chicago, Ill. : 1960) · 2002
Gyrate atrophy of the choroid and retina: further experience with long-term reduction of ornithine levels in children.
Клинический случайСтатьяПоддержка исследования: вне правительства СШАПоддержка службы здравоохранения СШАНазвание и аннотация: Перевод пока не подготовлен
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Kaiser-Kupfer Muriel I, Caruso Rafael C, Valle David
Аннотация
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.
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.