Carglumic Acid

(rINN)
Carglumic Acid Chemical formula
Synonyms: Acide Carglumique; Ácido carglúmico; Acidum Carglumicum; Carglutamic Acid; Karglumik Asit. N-Carbamoyl
Cyrillic synonym: Карглумовая Кислота.

💊 Chemical information

Chemical formula: C6H10N2O5 = 190.2.
CAS — 1188-38-1.
ATC — A16AA05.
ATC Vet — QA16AA05.

💊 Profile

Carglumic acid is used for the treatment of hyperammonaemia in patients with N-acetylglutamate synthase deficiency. The initial daily dose ranges from 100 to 250 mg/kg, adjusted thereafter to maintain normal plasma levels of ammonia. Individual responsiveness to carglumic acid should be tested before starting longterm therapy; daily maintenance doses range from 10 to 100 mg/kg. The total daily dose should preferably be taken as 2 to 4 divided doses before food.
1. Bélanger-Quintana A, et al. Hyperammonaemia as a cause of psychosis in an adolescent. Eur J Pediatr 2003; 162: 773–5
2. Caldovic L, et al. Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate. J Pediatr 2004; 145: 552–4
3. Gebhardt B, et al. N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia. J Inherit Metab Dis 2005; 28: 241–4
4. Guffon N, et al. Neonatal hyperammonemia: the N-carbamoylL-glutamic acid test. J Pediatr 2005; 147: 260–2
5. Jones S, et al. N-Carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia. J Inherit Metab Dis 2008. Available at: doi: 10.1007/s10545-008-0777-1

💊 Preparations

Proprietary Preparations

Belg.: Carbaglu; Cz.: Carbaglu; Denm.: Carbaglu; Fr.: Carbaglu; Ger.: Carbaglu; Ital.: Carbaglu; Neth.: Carbaglu; Pol.: Carbaglu; Port.: Carbaglu; Spain: Carbaglu; UK: Carbaglu.
Published February 07, 2019.