DRUG SUPPLIED DOSE COMMENTS
CARGLUMIC ACID (Paeds)
(Carbaglu, N-carbamyl-L-glutamatic acid)

Mechanism of action:

Synthetic analogue of N-acetylglutamate


Ref: 44, 61, 118



Last update: 2024-03-18		 Dispersible Tablet: 200 mg
 Pediatric:

N-acetylglutamate synthase (NAGS) Deficiency
Acute treatment:  100 mg/kg PO/NG once as bolus dose, then 25 - 62.5 mg/kg PO/NG Q6H
Maintenance dose: 3.3 - 33.3 mg/kg PO TID

Propionic Acidemia and Methylmalonic Acidemia
Acute treatment: 75 mg/kg PO/NG Q12H for up to 7 days
(Dose range 100-250 mg/kg/day divided in 2 to 4 doses)

**Each tablet must be dispersed in a minimum of 2.5 mL of water and taken immediately		 May be used for patients with undiagnosed metabolic disorder

Taken before meals or feeding.

May cause vomiting (25%).

Suspension will have a slightly acidic taste.

Unopened bottles stored in refrigerator. Once opened, stable for 1 month at room temperature.

For patients with NAGS deficiency, doses may be higher.


Standard Prescription:

carglumic acid    mg PO__(frequency) (    mg/kg/day)