Sustained oral lysine supplementation in ornithine delta-aminotransferase deficiency.
Oral lysine administration to three patients with B6-nonresponsive gyrate atrophy reduced plasma ornithine concentrations by 21-31% within 1-2 days. No further reduction was noted with time.
Elpeleg
N
N
Metabolic Disease Unit, Shaare-Zedek Medical Center, Jerusalem, Israel. Elpeleg@cc.huji.ac.il
Korman
S H
SH
D000293:Adolescent; D000328:Adult; D015799:Gyrate Atrophy / Q000209:etiology; D006801:Humans; D008239:Lysine / Q000008:administration & dosage* / Q000097:blood / Q000627:therapeutic use; D009952:Ornithine / Q000097:blood; D009953:Ornithine-Oxo-Acid Transaminase / Q000172:deficiency*
D009952:Ornithine; D009953:Ornithine-Oxo-Acid Transaminase; D008239:Lysine