Carbaglu Generic Name & Formulations
Legal Class
Rx
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
Manufacturer
Generic Availability
YES
Mechanism of Action
Carglumic acid is a synthetic structural analogue of N-acetylglutamate (NAG) that acts as a CPS1 activator, improves or restores the function of the urea cycle, and facilitates ammonia detoxification and urea production.
Carbaglu Indications
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
Warnings/Precautions
Monitor plasma ammonia levels closely. Moderate or severe renal impairment. Pregnancy. Nursing mothers.
Carbaglu Pharmacokinetics
Absorption
Absolute bioavailability: ~10%.
Elimination
Fecal, renal.
Carbaglu Interactions
Not Applicable
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
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