Carglumic Acid: uses, dosing, side effects & brands
Carglumic Acid is a carbamoyl phosphate synthetase 1 activator sold in the U.S. under one brand, for hyperammonemia. Below: what the FDA label says, every product that contains it, what the pills look like, and its recall record.
Key facts
- Drug class
- Carbamoyl Phosphate Synthetase 1 Activator
- Treats
- Hyperammonemia
- Available as
- Tablet
- Sold as
- Carbaglu
- Prescription?
- Prescription only
- Generic available?
- Not in our catalog
How carglumic acid is dosed
From the FDA label for Carbaglu (application NDA022562). Other carglumic acid products — different forms, different strengths — are dosed differently. Follow the label for the one you were prescribed.
Acute Hyperammonemia due to NAGS deficiency ( 2.2 ) The recommended dosage in adult and pediatric patients is 100 mg/kg to 250 mg/kg orally daily. Divide the daily dosage into 2 to 4 doses. Chronic Hyperammonemia due to NAGS deficiency ( 2.2 ) The recommended dosage in adult and pediatric patients is 10 mg/kg to 100 mg/kg orally daily. Divide the daily dosage into 2 to 4 doses. Therapeutic Monitoring for NAGS Deficiency ( 2.2 ) Closely monitor plasma ammonia and titrate dosage to maintain the ammonia level within normal range for the patient's age, taking into consideration their clinical condition. Acute Hyperammonemia due to PA or MMA ( 2.3 ) The recommended dosage in adult and pediatric patients is: 150 mg/kg orally daily for patients less than or equal to 15 kg 3.3 g/m 2 orally daily for patients greater than 15 kg Divide the daily dosage into 2 doses. Continue treatment until ammonia level is less than 50 micromol/L and for a maximum duration of 7 days. Patients with Renal Impairment ( 2.4 ) See Full Prescribing Information for Instructions on Dosage Adjustment. Preparation and Administration ( 2.5 ) Disperse CARBAGLU tablets in water. Do not swallow whole or crushed. Take immediately before meals or feedings. For additional instructions on preparation and administration orally or through a nasogastric tube or gastrostomy tube, see Full Prescribing Information. 2.1…
Carglumic Acid side effects
NAGS deficiency : Most common adverse reactions (≥13%) are vomiting, abdominal pain, pyrexia, tonsillitis, anemia, diarrhea, ear infection, infections, nasopharyngitis, hemoglobin decreased, and headache. ( 6.1 ) PA and MMA : Most common adverse reactions (≥5%) are neutropenia, anemia, vomiting, electrolyte imbalance, decreased appetite, hypoglycemia, lethargy/stupor, encephalopathy and pancreatitis/lipase increased. ( 6.1 ) To report SUSPECTED ADVERSE REACTIONS, contact Recordati Rare Diseases Inc. at 1-888-575-8344, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch. 6.1 Clinical Trials Experience Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice. Acute and Chronic Hyperammonemia due to NAGS Deficiency In a retrospective case series of 23 NAGS deficiency patients treated with CARBAGLU, 17 of the 23 patients reported an adverse reaction. The most common adverse reactions (occurring in ≥ 13% of patients) were vomiting, abdominal pain, pyrexia, tonsillitis, anemia, diarrhea, ear infection, infections, nasopharyngitis, hemoglobin decreased, and headache. Table 1 summarizes adverse reactions occurring in 2 or more patients treated with CARBAGLU. Table…
Who shouldn’t take carglumic acid
None None. ( 4 )
Every carglumic acid product we track (1)
Same active ingredient — different manufacturer, form, price and FDA recall record. That last one is what our independent score measures.
Only one: Carbaglu.
What carglumic acid pills look like
Imprint codes, colour and shape from the FDA’s labelling data. Match the imprint on your pill — or search any imprint.
| Imprint | Strength | Colour | Shape | Maker |
|---|---|---|---|---|
| N;120 | 200 mg | white | capsule | — |
| C | 200 mg | white | rectangle | — |
Can you crush or split carglumic acid?
At least one carglumic acidproduct is labelled to be swallowed whole — crushing an extended-release tablet releases the whole day’s dose at once. Which applies depends on the form you were given.
What each carglumic acid label says about crushing, splitting and chewingHow long carglumic acid keeps
The date on a sealed pack is not the only one: some carglumic acid labels start a second clock once the product is opened or mixed, as short as 90 days.
Does carglumic acid expire? The in-use limits and storage rules from its labelsWhat people report to the FDA about carglumic acid
The FDA Adverse Event Reporting System (FAERS) collects reports from patients and clinicians. It holds 82 reports naming carglumic acid, and the FDA flagged 94% of those reports as serious. The effects reported most often — leaving out reports about overdose, misuse or the condition being treated, which dominate the raw list for common medicines:
- vomiting17 reports
- hospitalisation7 reports
- hyperammonaemia7 reports
- hyperammonaemic encephalopathy6 reports
- rebound effect6 reports
- tremor5 reports
- abdominal discomfort4 reports
- ammonia increased4 reports
Read these as a signal, not a rate. A report does not mean carglumic acid caused the effect — anyone can file one, and many describe people taking several medicines for several conditions. Crucially there is no denominator: FAERS does not record how many people took the drug, so these counts cannot be turned into “X% of patients” — a bigger number often just means a more widely used or more talked-about drug. Duplicates exist, and publicity drives reporting. For what is actually established, read the FDA label section above.
Source: openFDA drug/event (FAERS), retrieved July 25, 2026.
Related calculators
Frequently asked questions
What is carglumic acid?
CARBAGLU tablets for oral suspension contain 200 mg of carglumic acid. Carglumic acid, the active substance, is a carbamoyl phosphate synthetase 1 (CPS 1) activator and is soluble in boiling water, slightly soluble in cold water, and practically insoluble in organic solvents.
What kind of drug is carglumic acid?
The FDA classifies carglumic acid as a carbamoyl phosphate synthetase 1 activator. If you are checking whether it is safe to combine with something else, the class is what matters — two drugs from the same class usually should not be stacked.
Can you take carglumic acid with other medicines?
It depends on the medicine. We check it against the FDA labels rather than guessing: our interaction checker searches each drug's own label for the other and quotes what it says, naming the section it came from. Run carglumic acid against whatever else you take — and remember that a label not naming a drug is not the same as that combination being safe.
What forms does carglumic acid come in?
Across the brands we track, carglumic acid is currently marketed as tablet, per the FDA's National Drug Code Directory. Each form is dosed differently — follow the label for the exact product you were prescribed.
Is there a generic carglumic acid?
We do not currently list a generic-labelled carglumic acid product. That does not always mean none exists — it means none appears under a generic name in the FDA data we track. Ask your pharmacist.
Cite this page
- APA
- pharmaranks. (2026, July 24). Carglumic Acid: uses, dosing, side effects & brands. https://pharmaranks.com/drugs/carglumic-acid
- MLA
- “Carglumic Acid: uses, dosing, side effects & brands.” pharmaranks, 24 July 2026, https://pharmaranks.com/drugs/carglumic-acid.
We summarise public FDA and NIH sources — for a clinical claim, cite the primary source we link to as well.
Sources: FDA openFDA drug label, National Drug Code Directory, and Enforcement (recall) database. This page reproduces public FDA data and is not medical advice. Dosing is set by your prescriber.
Read the full FDA label for carglumic acid on DailyMed (NIH) ↗.