Alternate Name(s):
Sodium Benzoate/Sodium Phenylacetate
Classification:
Urea Cycle Disorder (UCD) Treatment Agent
Original Date:
Sept 2022
Indications:
- Acute treatment of hyperammonemia associated with urea cycle disorders
Administration:
-
IV Intermittent Infusion: central line
Infusion time: 90 minutes -
IV Continuous Infusion: central line
Dosage:
- Loading: 250 mg/kg/dose IV as a loading dose. Give IV over 90 minutes
- Maintenance: Followed by 250 mg/kg/day as a continuous IV infusion
Side Effects:
- IV infiltration may cause necrosis and phlebitis
- Nausea, vomiting (especially with loading dose)
- Hypotension
- Metabolic acidosis, hyperglycemia, hypokalemia, hypocalcemia, hyperammonemia, hypernatremia
- Seizure, cerebral edema, agitation
Parameters to Monitor:
-
Monitor plasma ammonia levels, neurological status, blood gases
-
Electrolytes and glucose
-
LFTs and renal function (use with caution in renal or hepatic impairment)
Reconstitution and Stability:
CHEO
- Available from pharmacy as a 10 mg/mL solution in D10W, with or without Arginine HCL* added.
*see monograph for Arginine HCL
Compatibility:
- Solutions Compatible: D10W ONLY
- Additive/Above Cassette Compatible: arginine HCL, sodium bicarbonate
- Y-site Compatibility: arginine HCL, sodium bicarbonate
Notes:
-
For 250 mg/kg Ammonul® and 600 mg/kg Arginine HCL dosing, use Ammonul® 10 mg/mL with Arginine HCL 24 mg/mL in D10W.
- Fluid overload: Use with caution in patients at risk for fluid overload (eg, heart failure, severe renal impairment) or sodium retention edema; contains a significant amount of sodium (Ammonul® vials of 100 mg/mL contains 0.52 mmol of sodium/mL)
- Caution in neonates: displaces bilirubin from protein-binding site
- Enhanced potassium excretion may occur with treatment of hyperammonemia, monitor serum potassium concentration closely.
References:
- Lexi-Comp Online Database, accessed Sept 2022
- Ammonul Product Monograph