• Inborn errors of metabolism

Carbaglu Generic Name & Formulations

General Description

Carglumic acid 200mg (functionally scored); tabs for oral susp.

Pharmacological Class

Carbamoyl phosphate synthetase 1 (CPS 1) activator.

How Supplied

Tabs—5, 60

Generic Availability


Carbaglu Indications


Adjunctive therapy to standard of care for the treatment of acute hyperammonemia due to N-acetylglutamate synthase (NAGS) deficiency, propionic acidemia (PA) or methylmalonic acidemia (MMA). Maintenance therapy for the treatment of chronic hyperammonemia due to NAGS deficiency.

Carbaglu Dosage and Administration

Adults and Children

Do not swallow whole or crush tabs. Dissolve each tab with at least 2.5mL of water; stir and mix. Swallow mixture orally or via an oral syringe immediately before meals; may administer via NG tube or G-tube. NAGS deficiency (acute hyperammonemia): 100–250mg/kg/day; divided into 2–4 doses rounded to the nearest 100mg; administer with other ammonia lowering therapies (eg, alternate pathway meds, hemodialysis, protein restriction); (chronic hyperammonemia): 10–100mg/kg/day; divided into 2–4 doses rounded to the nearest 100mg; may need other ammonia lowering therapies and protein restriction based on plasma ammonia levels. PA or MMA (acute hyperammonemia): (≤15kg): 150mg/kg/day; (>15kg): 3.3g/m2/day; divided into 2 equal doses (given 12hrs apart) rounded up to the next multiple of 50mg; continue until ammonia level <50micromol/L and max duration of 7 days; administer with other ammonia lowering therapies (eg, IV glucose, insulin, L-carnitine, protein restriction, dialysis). Moderate or severe renal impairment: adjust dosage; see full labeling.

Carbaglu Contraindications

Not Applicable

Carbaglu Boxed Warnings

Not Applicable

Carbaglu Warnings/Precautions


Monitor plasma ammonia levels closely. Moderate or severe renal impairment. Pregnancy. Nursing mothers.

Carbaglu Pharmacokinetics

See Literature

Carbaglu Interactions

Carbaglu Adverse Reactions

Adverse Reactions

Vomiting, anemia; NAGS: also abdominal pain, pyrexia, tonsillitis, diarrhea, ear infection, infections, nasopharyngitis, hemoglobin decreased, headache; PA and MMA: also neutropenia, electrolyte imbalance, decreased appetite, hypoglycemia, lethargy/stupor, encephalopathy, pancreatitis/lipase increased.

Carbaglu Clinical Trials

See Literature

Carbaglu Note

Not Applicable

Carbaglu Patient Counseling

See Literature