Long-term N-carbamylglutamate treatment of hyperammonemia in patients with classic organic acidemias

Kıykım E., Oguz O., Duman Ç., Zübarioğlu T., Cansever M. Ş., Aktuğlu Zeybek A. Ç.

MOLECULAR GENETICS AND METABOLISM REPORTS, cilt.26, 2021 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 26
  • Basım Tarihi: 2021
  • Doi Numarası: 10.1016/j.ymgmr.2021.100715
  • Dergi Adı: MOLECULAR GENETICS AND METABOLISM REPORTS
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED)
  • Anahtar Kelimeler: Hyperammonemia, Methylmalonic acidemia, N-Carbamylglutamate, Organic acidurias, Propionic acidemia
  • İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet

Özet

Background: Classic organic acidurias (OAs) usually characterized by recurrent episodes of acidemia, ketonuria, and hyperammonemia leading to coma and even death if left untreated. Acute hyperammonemia episodes can be treated effectively with N-carbamylglutamate (NCG). The effect of the long-term efficacy of N-carbamylglutamate is little known.