Carglumic acid

Concise Prescribing Info
Hyperammonaemia in patient w/ NAG synthase deficiency.
Dosage/Direction for Use
Adult : PO Initial: 100-250 mg/kg/day as 2-4 divided doses immediately prior to meals, adjusted thereafter. Maintenance: 10-100 mg/kg/day.
Dosage Details
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.
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

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Store between 2-8°C.
ATC Classification
A16AA05 - carglumic acid ; Belongs to the class of amino acids and derivatives products. Used in treatment of alimentary tract and metabolism problems.
Disclaimer: This information is independently developed by MIMS based on Carglumic acid from various references and is provided for your reference only. Therapeutic uses, prescribing information and product availability may vary between countries. Please refer to MIMS Product Monographs for specific and locally approved prescribing information. Although great effort has been made to ensure content accuracy, MIMS shall not be held responsible or liable for any claims or damages arising from the use or misuse of the information contained herein, its contents or omissions, or otherwise. Copyright © 2020 MIMS. All rights reserved. Powered by
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