Infant Data
CPS = carbamyl phosphate synthetase; OTC = ornithine transcarbamylase; NAGS = N-acetyl glutamate synthase; ASS = argininosuccinic acid synthetase; ASL = argininosuccinic acid lyase
Results
INDICATIONS
Adjunctive treatment of acute hyperammonemia in neonates with urea cycle disorders. Sodium phenylacetate/sodium benzoate should be used concomitantly with arginine hydrochloride. Hemodialysis is the primary treatment of acute hyperammonemia during the early management period.
CONTRAINDICATIONS
Arginine hydrochloride contains 47.5 mEq of chloride in 100 mL. Hyperchloremic metabolic acidosis has been reported in 2 pediatric patients receiving excessive arginine. Extravasation can cause tissue necrosis. Arginine is a nitric oxide precursor. Excessive arginine accumulation can result in nitric oxide overproduction with potential for vasodilation and hypotension.
ADMINISTRATION
For treatment of acute hyperammonemia, must be administered through a central line. For loading and maintenance doses, dilute arginine and sodium phenylacetate/sodium benzoate in 25 to 35 mL/kg of D10W prior to administration.
MONITORING
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 electrolytes and acid-base status closely during the acute phase (eg, every 4 hours). Monitor amino acids daily to assess the effectiveness of citrulline/arginine replacement and glutamine removal.