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.
Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan. nagasa-hirono@k2.dion.ne.jp
Hironori
H
Nagasaka
Haruki
H
Komatsu
Toshihiro
T
Ohura
Tsuyoshi
T
Sogo
Ayano
A
Inui
Tohru
T
Yorifuji
Kei
K
Murayama
Masaki
M
Takayanagi
Hideaki
H
Kikuta
Kunihiko
K
Kobayashi
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