Infant Data
Results
INDICATIONS
Adjunctive treatment of acute hyperammonemia in neonates with urea cycle disorders. Arginine hydrochloride should be used concomitantly with sodium phenylacetate/sodium benzoate. Hemodialysis is the primary treatment of acute hyperammonemia during the early management period. Caloric supplementation, dietary protein restriction, and other ammonia lowering therapies should also be considered during acute hyperammonemic episodes.
CONTRAINDICATIONS/PRECAUTIONS
Caution advised for use in patients with congestive heart failure, severe renal impairment, or other clinical conditions involving sodium retention with edema; product contains 30.5 mg of sodium per mL. Extravasation may lead to tissue necrosis; administration through central line required
ADVERSE EFFECTS
The most common adverse effects include vomiting (9%), hyperglycemia (7%), and hypokalemia (7%). Vomiting and lethargy can occur with higher than recommended doses. Hypotension seen more frequently in patients 30 days of age and less. Potentially lifethreatening toxicity can occur with doses greater than 750 mg/kg per day.
ADMINISTRATION
- Must be administered through a central line.
- For loading and maintenance doses, dilute sodium phenylacetate/sodium benzoate and arginine in 25 to 35 mL/kg of D10W prior to administration.
- Infuse the loading dose over 90 to 120 minutes.
MONITORING
Measure plasma ammonia levels every hour during dialysis until levels stabilize to less than 200 to 300 micromoles/L. Capillary blood should not be used for monitoring ammonia levels. Monitor blood glucose, electrolytes (especially potassium), and acid-base status closely during the acute phase (eg, every 4 hours). Toxicity due to ammonia scavenging drugs presents as ketoacidosis. An anion gap that is greater than 15 mEq/L or has increased by greater than 6 mEq/L from baseline may indicate drug accumulation. Monitor amino acids daily to assess the effectiveness of citrulline/arginine replacement and glutamine removal. Assess AST and ALT levels. Evaluate neurological status, Glasgow Coma Scale, respiratory status, CT or MRI or fundoscopic evidence of cerebral edema, and/or of gray matter and white matter damage to assess patient response to treatment. Monitor infusion site closely during infusion for signs of extravasation