Nitric oxide synthesis in ornithine transcarbamylase deficiency: possible involvement of low no synthesis in clinical manifestations of urea cycle defect.
Nitric oxide (NO) levels in plasma and urine were determined in 5 girls with ornithine transcarbamylase deficiency (OTCD) of late-onset type, who often developed migraine-like headache or vomiting. The patients were found to have low NO synthesis, suggesting that the low NO synthesis contributes to the clinical manifestations of urea cycle defect.
Nagasaka
Hironori
H
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan. nagasa-hirono@k2.dion.ne.jp
Komatsu
Haruki
H
Ohura
Toshihiro
T
Sogo
Tsuyoshi
T
Inui
Ayano
A
Yorifuji
Tohru
T
Murayama
Kei
K
Takayanagi
Masaki
M
Kikuta
Hideaki
H
Kobayashi
Kunihiko
K
D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008659:Metabolic Diseases / Q000175:diagnosis / Q000378:metabolism*; D009569:Nitric Oxide / Q000096:biosynthesis* / Q000097:blood / Q000652:urine; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000378:metabolism*; D014508:Urea / Q000378:metabolism*
D009569:Nitric Oxide; D014508:Urea