- Hyperammonemia, acute (urea cycle disorders)
- IV Intermittent Infusion. Give IV over 90 minutes. Central line preferred
- IV Continuous Infusion. Maximum Infusion rate: 1 g/kg/hr. Central line preferred
Urea Cycle Disorders:
Argininosuccinate synthetase (ASS, Citrullinemia) or Argininosuccinate lyase (ASL) deficiency:
- Loading: 600 mg/kg IV as a loading dose. Give IV over 90 minutes
- Maintenance: Followed by 600 mg/kg/day as a continuous IV infusion
Carbamyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC) deficiency:
- Loading: 200 mg/kg IV as a loading dose. Give IV over 90 minutes
- Maintenance: Followed by 200 mg/kg/day as a continuous IV infusion
- IV infiltration may cause necrosis and phlebitis
- Flushing, headache, nausea and vomiting with rapid infusion
- Hyperglycemia, metabolic acidosis, hyperchloremia
- Potassium levels: more commonly hyperkalemia is experienced, however, hypokalemia is possible in some (follow levels closely)
- Arginine may result in overproduction of nitric oxide, which may result in hypotension and vasodilation.
- Monitor acid-base status (arterial or capillary blood gases), serum electrolytes, BUN, glucose, ammonia every 4 hours in the acute phase
CHEO
- Available from pharmacy, most often as a combo product with Ammonul®* (Sodium Phenylacetate and Sodium Benzoate).
- Often prepared as Ammonul® 10 mg/mL with Arginine HCL 24 mg/mL in D10W
*see monograph for Ammonul®
- Solutions Compatible: NS, D10W
- Additives/Above Cassette Compatible: Ammonul®, sodium bicarbonate
- Y-site Compatible: Ammonul®, sodium bicarbonate
- Arginine 250 mg/mL contains 0.475 mmol/mL of chloride
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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.
- Lexi-Comp Online Database, accessed Sept 2022
- Arginine HCL Injection Product Monograph