CARGLUMIC ACID

(Carbaglu, N-carbamyl-L-glutamatic acid)

Standard Prescription

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

Dosages

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

Mechanism of Action

Synthetic analogue of N-acetylglutamate

Forms Supplied

Dispersible Tablet: 200 mg

Comments

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.

References

44, 61, 118

Last Edited

2024-03-18 03:58:58