Clinical reference
Metabolic / Urea Cycle · N-acetylglutamate analogue · Neonatology, Metabolic / Genetics
Carglumic acid is a synthetic analogue of N-acetylglutamate for hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency and as adjunct in acute hyperammonemia in propionic acidemia (PA) or methylmalonic acidemia (MMA). NAGS acute: 100–250 mg/kg/day PO/NG divided 2–4 doses; maintenance 10–100 mg/kg/day. PA/MMA acute (≤15 kg): 150 mg/kg/day in 2…