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Indications and Dosage
Oral
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.
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Contraindications
Lactation.
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Special Precautions
Pregnancy.
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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. |
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Patient Counseling Information
This drug may cause protein catabolism, complete dietary protein restriction for 24-48 hr and caloric supplementation is recommended.
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Monitoring Parameters
Monitor amino acids and ammonia plasma levels, neurologic status, haematologic, cardiac, hepatic, and renal functions.
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Action
Description:
Mechanism of Action: 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. Pharmacokinetics: 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. |
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Chemical Structure
 Source: National Center for Biotechnology Information. PubChem Database. Carglumic acid, CID=121396, https://pubchem.ncbi.nlm.nih.gov/compound/Carglumic-acid (accessed on Jan. 21, 2020) |
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Storage
Store between 2-8°C.
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MIMS Class
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ATC Classification
A16AA05 - carglumic acid ; Belongs to the class of amino acids and derivatives products. Used in treatment of alimentary tract and metabolism problems.
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References
Anon. Carglumic Acid. Lexicomp Online. Hudson, Ohio. Wolters Kluwer Clinical Drug Information, Inc. https://online.lexi.com. Accessed 17/11/2016. Buckingham R (ed). Carglumic Acid. Martindale: The Complete Drug Reference [online]. London. Pharmaceutical Press. https://www.medicinescomplete.com. Accessed 17/11/2016. Carbaglu Tablet (Orphan Europe, SARL). DailyMed. Source: U.S. National Library of Medicine. https://dailymed.nlm.nih.gov/dailymed/. Accessed 17/11/2016. Joint Formulary Committee. Carglumic Acid. British National Formulary [online]. London. BMJ Group and Pharmaceutical Press. https://www.medicinescomplete.com. 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). https://www.medicinescomplete.com. Accessed 17/11/2016.
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