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Pronunciation |
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(SOW
dee um fen il AS e tate & SOW
dee um BENZ oh ate) |
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U.S. Brand
Names |
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Ucephan® |
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Generic
Available |
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No |
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Pharmacological Index |
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Antidote |
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Use |
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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 |
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Pregnancy Risk
Factor |
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C |
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Stability |
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Diluting Ucephan® with an acidic solution can result in
precipitation of the drug |
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Usual Dosage |
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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 |
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Dietary
Considerations |
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Dilute each dose in 4-8 oz of infant formula or milk and administer with
meals |
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Patient
Information |
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Must be diluted before use; due to its lingering odor, exercise care in
mixing to minimize contact with skin and clothing |
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Nursing
Implications |
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Monitor serum electrolytes, blood ammonia |
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Dosage Forms |
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Solution: Sodium phenylacetate 100 mg and sodium benzoate 100 mg per mL (100
mL) |
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References |
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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.
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