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Buphenyl Powder Generic Name & Formulations
Legal Class
Rx
General Description
Sodium phenylbutyrate 940mg/gram; contains sodium 11.7g/100g of pwd.
Pharmacological Class
Phenylacetate prodrug.
See Also
How Supplied
Tabs—250; Pwd—266g
Manufacturer
Buphenyl Powder Indications
Indications
Adjunct in the chronic management of urea cycle disorders involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase, including neonatal-onset deficiency and late-onset disease with a history of hyperammonemic encephalopathy.
Buphenyl Powder Dosage and Administration
Adults and Children
<20kg: use powder. Take in equally divided doses with each meal or feeding. Mix powder with food or liquid. <20kg: 450–600mg/kg/day. ≥20kg: 9.9–13g/m2/day. Max 20g/day.
Buphenyl Powder Contraindications
Contraindications
Acute hyperammonemia.
Buphenyl Powder Boxed Warnings
Not Applicable
Buphenyl Powder Warnings/Precautions
Warnings/Precautions
Restrict dietary protein, may need to supplement with essential amino acids. CHF. Sodium retention with edema. Renal or hepatic insufficiency. Inborn errors of beta oxidation. Monitor serum levels of phenylbutyrate and metabolites, phenylacetate and phenylacetylglutamine. Plasma levels of ammonia, arginine, branched-chain amino acids, serum proteins: maintain within normal levels; plasma glutamine: maintain at <1000μmol/L. Elderly. Pregnancy (Cat.C). Nursing mothers.
Buphenyl Powder Pharmacokinetics
See Literature
Buphenyl Powder Interactions
Interactions
May be potentiated by probenecid. Corticosteroids may increase plasma ammonia levels. Hyperammonemia possible with haloperidol and valproic acid.
Buphenyl Powder Adverse Reactions
Adverse Reactions
Amenorrhea/menstrual dysfunction, acidosis, hypoalbuminemia, anemia, alkalosis, hyperchloremia, hypophosphatemia, increased alkaline phosphatase, increased liver transaminases, leukopenia, leukocytosis, decreased appetite, body odor, bad taste, taste aversion, others.
Buphenyl Powder Clinical Trials
See Literature
Buphenyl Powder Note
Not Applicable
Buphenyl Powder Patient Counseling
See Literature
