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.
OBJECTIVE: To determine whether the long-term reduction of plasma ornithine levels by way of an arginine-restricted diet in patients with gyrateatrophy will slow the progression of this chorioretinal degeneration. DESIGN: Natural history study of 2 pairs of siblings with gyrateatrophy 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 gyrateatrophy.
Authors: M Fleury; R Barbier; F Ziegler; M Mohr; O Caron; H Dollfus; C Tranchant; J M Warter Journal: J Neurol Neurosurg Psychiatry Date: 2006-11-06 Impact factor: 10.154
Authors: Guillermo Salcedo-Villanueva; Miguel Paciuc-Beja; Cristina Villanueva-Mendoza; Mariana Harasawa; Jesse M Smith; Raul Velez-Montoya; Jeffrey L Olson; Scott C Oliver; Naresh Mandava; Hugo Quiroz-Mercado Journal: Int Ophthalmol Date: 2015-05-26 Impact factor: 2.031
Authors: R Santinelli; C Costagliola; C Tolone; A D'Aloia; A D'Avanzo; F Prisco; L Perrone; E Miraglia del Giudice Journal: J Inherit Metab Dis Date: 2004 Impact factor: 4.982