DOSAGE and ADMINISTRATION

  • Administration must be through a central line. Administration through a peripheral line may cause burns.
    AMMONUL (sodium phenylacetate and sodium benzoate) Injection 10%/10% is a sterile, concentrated solution intended for intravenous (IV) administration only after dilution with sterile Dextrose Injection, 10%.
  • AMMONUL is administered via a bolus IV dose over a 90 to 120 minute period. A maintenance infusion of the same dose should be administered over 24 hours or until the patient is no longer hyperammonemic or until the patient can tolerate oral nutrition and medications.
  • Intravenous arginine is an essential component of therapy for patients with CPS, OTC, ASS, and ASL deficiency.
Patient Population Components of Infusion Solution
AMMONUL must be diluted with 10% sterile dextrose injections at ≥ 25 mL/kg before administration. 		Dosage Provided
AMMONUL Arginine HCl Injection, 10% Sodium Phenylacetate Sodium Benzoate Arginine HCl

CPS and OTC Deficiency (0 to 20 kg)
Dose Loading: over 90 to 120 minutes

Maintenance: over 24 hours		 2.5 mL/kg 2.0 mL/kg 250 mg/kg 250 mg/kg 200 mg/kg

ASS and ASL Deficiency (0 to 20 kg)
Dose Loading: over 90 to 120 minutes

Maintenance: over 24 hours		 2.5 mL/kg 6.0 mL/kg 250 mg/kg 250 mg/kg 600 mg/kg

CPS and OTC Deficiency (>20 kg)
Dose Loading: over 90 to 120 minutes

Maintenance: over 24 hours		 55 mL/m2 2.0 mL/kg 5.5 g/m2 5.5 g/m2 200 mg/kg

ASS and ASL Deficiency (>20 kg)
Dose Loading: over 90 to 120 min.

Maintenance: over 24 hours.		 55 mL/m2 6.0 mL/kg 5.5 g/m2 5.5 g/m2 600 mg/kg
  • Because of prolonged plasma levels achieved by phenylacetate in pharmacokinetic studies, repeat loading doses should not be administered.
  • Dialysis is recommended for those patients who fail to have a significant reduction in plasma ammonia levels within four to eight hours after receiving AMMONUL.
  • AMMONUL can be continued during dialysis as it suppresses the production of ammonia from catabolism of endogenous proteins and dialysis eliminates the ammonia and ammonia conjugates.
  • Treatment of hyperammonemia also requires caloric supplementation and restriction of dietary protein.
Please see prescribing information for complete dosage and administration instructions.

Important Safety Information for AMMONUL Injection 10% / 10%

  • IMPORTANT: Filtration required. Due to the possibility that particulate matter may impact the safe use of this product and to ensure optimal patient care, you are instructed to use the Millex® Durapore GV 33 mm Sterile Syringe Filter (0.22 μm) during the admixture process when injecting AMMONUL into the 10% dextrose IV bag. Note that particulate matter may not be readily seen on visual inspection, so this filter must be employed in all cases regardless of whether particulate matter is seen in the vial.
  • The most common adverse reactions are vomiting (9%), hyperglycemia (7%), hypokalemia (7%), convulsions (6%), and mental impairment (6%).
  • Dilute AMMONUL with sterile 10% Dextrose Injection (D10W) before administration. Use a central venous catheter line; use of a peripheral line may cause burns.
  • Monitor patients for prolonged exposure to elevated plasma ammonia levels as this can rapidly result in injury to the brain or death. Promptly use any therapies necessary to reduce plasma ammonia levels.
  • Monitor plasma potassium levels carefully and treat as necessary.
  • Use caution if AMMONUL is administered to patients with congestive heart failure, severe renal insufficiency, or with a condition in which there is sodium retention with edema.
  • Monitor the infusion site for possible tissue infiltration since extravasation of AMMONUL into the perivenous tissues during high flow bolus infusion may lead to skin necrosis, especially in infants.
  • Do not administer repeat loading doses because of prolonged plasma levels achieved by phenylacetate in pharmacokinetic studies.
  • Monitor patient blood chemistry profiles and perform frequent blood pH and pCO2 measurements because AMMONUL may cause side effects typically associated with salicyclate overdose such as hyperventilation and metabolic acidosis.
  • Administration of valproic acid to patients with urea cycle disorders may exacerbate their condition and antagonize the efficacy of AMMONUL.

Click here for Full Prescribing Information for AMMONUL