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Carglumic Acid

Medically reviewed by Last updated on May 20, 2020.


(kar GLU mik AS id)

Index Terms

  • N-Carbamoyl-L-Glutamic Acid
  • N-Carbamylglutamate

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Tablet, Oral:

Carbaglu: 200 mg [scored]

Brand Names: U.S.

  • Carbaglu

Pharmacologic Category

  • Antidote
  • Metabolic Alkalosis Agent
  • Urea Cycle Disorder (UCD) Treatment Agent


N-acetylglutamate synthase (NAGS) is a mitochondrial enzyme which produces N-acetylglutamate (NAG). NAG is a required allosteric activator of the hepatic mitochondrial enzyme, carbamoyl phosphate synthetase 1 (CPS 1), which converts ammonia into urea in the first step of the urea cycle. In NAGS-deficient patients, carglumic acid serves as a replacement for NAG.


Via intestinal flora to carbon dioxide.


Feces (≤60% as unchanged drug); urine (9% as unchanged drug).

Time to Peak

Median: 3 hours (range: 2 to 4 hours).

Half-Life Elimination

25 hours.

Use: Labeled Indications

Hyperammonemia: Adjunctive treatment of acute hyperammonemia and maintenance therapy of chronic hyperammonemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS) in adult and pediatric patients


There are no contraindications listed in the manufacturer’s labeling.

Canadian labeling: Additional contraindications (not in US labeling): Hypersensitivity to carglumic acid or any component of the formulation; breastfeeding

Dosing: Adult

Acute hyperammonemia: Oral: 100 to 250 mg/kg/day given in 2 to 4 divided doses (rounded to the nearest 100 mg); titrate to age-appropriate plasma ammonia levels and clinical symptoms. Concomitant adjunctive ammonia-lowering therapy recommended.

Chronic hyperammonemia: Oral: 10 to 100 mg/kg/day given in 2 to 4 divided doses (rounded to the nearest 100 mg); titrate to age-appropriate normal plasma ammonia levels.

Dosing: Pediatric

Acute hyperammonemia: Infants, Children, and Adolescents: Oral: 100 to 250 mg/kg/day in 2 or 4 divided doses; round to the nearest 100 mg (ie, one-half of a Carbaglu tablet); titrate to age-appropriate plasma ammonia concentrations and clinical condition considerations (including nutritional requirements, protein intake, growth parameters, etc); concomitant ammonia-lowering therapy recommended.

Chronic hyperammonemia: Infants, Children, and Adolescents: Oral: Usual dose: 10 to 100 mg/kg/day in 2 or 4 divided doses; titrate to age-appropriate plasma ammonia concentrations and clinical condition considerations (including nutritional requirements, protein intake, growth parameters, etc).


Note: The resultant concentration of a 200 mg tablet dispersed in a minimum of 2.5 mL is 80 mg/mL.

Oral: Disperse each 200 mg tablet or 1/2 tablet (ie, 100 mg) in ~2.5 mL of water (no other foods/liquids); stir and administer immediately.

NG tube: Disperse each 200 mg tablet or 1/2 tablet (ie, 100 mg) in water (~2.5 mL per tablet); stir and draw mixture up in a catheter-tip syringe; administer immediately.


Administer immediately prior to meals or feedings. Disperse in water prior to administration; should not be mixed with any other foods or liquids other than water.

Oral: Tablets should not be crushed or swallowed whole. After dispersion in water, administer immediately. Tablets do not dissolve completely, and some particles may remain; rinse container with water and administer rinse immediately; repeat as needed until no tablet pieces are left.

NG tube: After dispersion of tablets in water, immediately administer through a NG tube (tablets do not dissolve completely; some particles may remain). Flush immediately with 1 to 2 mL additional water to clear the tube; repeat as needed until no pieces of tablet are left in syringe or NG tube.


Before opening, store in original container refrigerated at 2°C to 8°C (36°F to 46°F). After opening, do not refrigerate; store at 15°C to 30°C (59°F to 86°F). Protect from moisture. Discard 1 month after opening.

Drug Interactions

There are no known significant interactions.

Adverse Reactions

The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.


Central nervous system: Headache (13%)

Gastrointestinal: Vomiting (26%), abdominal pain (17%), diarrhea (13%)

Hematologic & oncologic: Anemia (13%), decreased hemoglobin (13%)

Infection: Infection (13%)

Otic: Otic infection (13%)

Respiratory: Tonsillitis (17%), nasopharyngitis (13%)

Miscellaneous: Fever (17%)

1% to 10%:

Central nervous system: Drowsiness (9%)

Dermatologic: Hyperhidrosis (9%), skin rash (9%)

Endocrine & metabolic: Weight loss (9%)

Gastrointestinal: Anorexia (9%), dysgeusia (9%)

Infection: Influenza (9%)

Neuromuscular & skeletal: Weakness (9%)

Respiratory: Pneumonia (9%)

<1%, postmarketing, and/or case reports: Acidosis, acquired blood coagulation disorder, brain damage, brain disease, central nervous system dysfunction, cerebral edema, coma, decreased white blood cell count, developmental delay (motor development), eating disorder, eosinophilia, epilepsy, hyperammonemia, hyponatremia, increased intracranial pressure, increased liver enzymes, lactic acidosis, lethargy, low serum ferritin, otitis media, respiratory failure, seizure, sepsis, thrombocytopenia


Other warnings/precautions:

• Experienced physician: Acute symptomatic hyperammonemia is a life-threatening emergency; management of hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency should be done in coordination with those experienced in the management of metabolic disorders.

• Nutritional management: Since hyperammonemia is the result of protein catabolism, complete protein restriction is recommended to be maintained for 24-48 hours and caloric supplementation should be maximized to reverse catabolism and nitrogen turnover.

Monitoring Parameters

Plasma ammonia; monitor for physical signs/symptoms of hyperammonemia (eg, lethargy, ataxia, confusion, vomiting, seizures, and memory impairment).

Pregnancy Considerations

Untreated maternal N-acetylglutamate synthase (NAGS) deficiency can trigger a hyperammonemic crisis leading to neurological impairment, coma, and possibly death. Information specific to the use of carglumic acid in pregnancy has not been located (Wilcox 2018).

Data collection to monitor pregnancy and infant outcomes following exposure to carglumic acid is ongoing. Health care providers are encouraged to enroll females exposed to carglumic acid during pregnancy in the pregnancy pharmacovigilance program (1-888-575-8344).

Patient Education

What is this drug used for?

• It is used to treat high ammonia levels in some people.

All drugs may cause side effects. However, many people have no side effects or only have minor side effects. Call your doctor or get medical help if any of these side effects or any other side effects bother you or do not go away:

• Headache

• Diarrhea

• Abdominal pain

• Change in taste

• Sore throat

• Stuffy nose

• Weight loss

• Lack of appetite

• Vomiting

• Nausea

WARNING/CAUTION: Even though it may be rare, some people may have very bad and sometimes deadly side effects when taking a drug. Tell your doctor or get medical help right away if you have any of the following signs or symptoms that may be related to a very bad side effect:

• Infection

• Fatigue

• Sweating a lot

• Ear pain

• Severe loss of strength and energy

• Signs of an allergic reaction, like rash; hives; itching; red, swollen, blistered, or peeling skin with or without fever; wheezing; tightness in the chest or throat; trouble breathing, swallowing, or talking; unusual hoarseness; or swelling of the mouth, face, lips, tongue, or throat.

Note: This is not a comprehensive list of all side effects. Talk to your doctor if you have questions.

Consumer Information Use and Disclaimer: This information should not be used to decide whether or not to take this medicine or any other medicine. Only the healthcare provider has the knowledge and training to decide which medicines are right for a specific patient. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a limited summary of general information about the medicine’s uses from the patient education leaflet and is not intended to be comprehensive. This limited summary does NOT include all information available about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to this medicine. This information is not intended to provide medical advice, diagnosis or treatment and does not replace information you receive from the healthcare provider. For a more detailed summary of information about the risks and benefits of using this medicine, please speak with your healthcare provider and review the entire patient education leaflet.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.