Carglumic acid

drugbox
IUPAC_name = N-Carbamoyl-L-glutamic Acid
CAS_number = 1188-38-1
ATC_prefix = A16
ATC_suffix = AA05

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C=6 | H=10 | N=2 | O=5 | Na=1
molecular_weight = 190.2 g/mol
bioavailability = 30%
metabolism = Partial
protein_bound = Undetermined
elimination_half-life = 4.3 to 9.5 hours
excretion = Fecal (60%) and renal (9%, unchanged)
licence_EU = Carbaglu
pregnancy_category = unknown
routes_of_administration = Oral

Carglumic acid is an orphan drug, marketed by Orphan Europe under the trade name Carbaglu. 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.

References

*cite journal | author = Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M | title = Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate | journal = J Pediatr | volume = 145 | issue = 4 | pages = 552–4 | year = 2004 | pmid = 15480384 | doi = 10.1016/j.jpeds.2004.06.047

*cite journal | author = Elpeleg O, Shaag A, Ben-Shalom E, Schmid T, Bachmann C | title = N-acetylglutamate synthase deficiency and the treatment of hyperammonemic encephalopathy | journal = Ann Neurol | volume = 52 | issue = 6 | pages = 845–9 | year = 2002 | pmid = 12447942 | doi = 10.1002/ana.10406