Carglumic acid

Generic Medicine Info
Indications and Dosage
Hyperammonaemia in patients with N-acetylglutamate synthase deficiency
Adult: Initially, 100-250 mg/kg daily given as 2-4 divided doses immediately prior to meals, adjusted thereafter to maintain normal ammonia plasma levels. Maintenance: 10-100 mg/kg daily.
Special Precautions
Adverse Reactions
Nervous: Headache.
CV: Bradycardia.
GI: Abdominal pain, tonsillitis, vomiting, diarrhoea.
Resp: Nasopharngitis.
Haematologic: Anaemia.
Otic: Ear infection.
Dermatologic: Rash, sweating.
Immunologic: Infection.
Others: Pyrexia.
Patient Counseling Information
This drug may cause protein catabolism, complete dietary protein restriction for 24-48 hr and caloric supplementation is recommended.
Monitoring Parameters
Monitor amino acids and ammonia plasma levels, neurologic status, haematologic, cardiac, hepatic, and renal functions.
Description: Carglumic acid is a synthetic structural analogue of N-acetylglutamate (NAG). It is used to treat patients w/ deficiency in N-acetylglutamate synthase (NAGS), a mitochondrial enzyme which produces NAG to activate carbamoyl phosphate synthetase (CPS). Thus, it restores the activity of the urea cycle, reducing plasma ammonia concentrations to normal levels.
Absorption: Time to peak plasma concentration: 3 hr.
Distribution: Volume of distribution: Approx 2,657 L.
Metabolism: Metabolised by intestinal flora to CO2.
Excretion: Via faeces (≤60% as unchanged drug), urine (9% as unchanged drug), and lungs (as CO2). Elimination half-life: 4.3-9.5 hr.
Chemical Structure

Chemical Structure Image
Carglumic acid

Source: National Center for Biotechnology Information. PubChem Database. Carglumic acid, CID=121396, (accessed on Jan. 21, 2020)

Store between 2-8°C.
MIMS Class
Antidotes & Detoxifying Agents
ATC Classification
A16AA05 - carglumic acid ; Belongs to the class of amino acids and derivatives products. Used in treatment of alimentary tract and metabolism problems.
Anon. Carglumic Acid. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. Accessed 17/11/2016.

Buckingham R (ed). Carglumic Acid. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. Accessed 17/11/2016.

Carbaglu Tablet (Orphan Europe, SARL). DailyMed. Source: U.S. National Library of Medicine. Accessed 17/11/2016.

Joint Formulary Committee. Carglumic Acid. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. Accessed 17/11/2016.

McEvoy GK, Snow EK, Miller J et al (eds). Carglumic Acid. AHFS Drug Information (AHFS DI) [online]. American Society of Health-System Pharmacists (ASHP). Accessed 17/11/2016.

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