No

Antidote

Adjunctive therapy to prevent/treat hyperammonemia in patients with urea cycle enzymopathy involving partial or complete deficiencies of carbamoyl-phosphate synthetase, ornithine transcarbamoylase or argininosuccinate synthetase

C

Diluting Ucephan® with an acidic solution can result in precipitation of the drug

Infants and Children: Oral: 2.5 mL (250 mg sodium benzoate and 250 mg sodium phenylacetate)/kg/day divided 3-6 times/day; total daily dose should not exceed 100 mL

Dilute each dose in 4-8 oz of infant formula or milk and administer with meals

Must be diluted before use; due to its lingering odor, exercise care in mixing to minimize contact with skin and clothing

Monitor serum electrolytes, blood ammonia

Solution: Sodium phenylacetate 100 mg and sodium benzoate 100 mg per mL (100 mL)

Brusilow SW, Danney M, Waber LJ, et al, "Treatment of Episodic Hyperammonemia in Children With Inborn Errors of Urea Synthesis," N Engl J Med, 1984, 310(25):1630-4.

Gutteridge C and Kuhn RJ, "Compatibility of 10% Sodium Benzoate Plus 10% Sodium Phenylacetate With Various Flavored Vehicles," Am J Hosp Pharm, 1994, 51(19):2508, 2510.