Buphenyl Powder

  • Inborn errors of metabolism

Buphenyl Powder Generic Name & Formulations

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


Buphenyl Powder 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


Acute hyperammonemia.

Buphenyl Powder Boxed Warnings

Not Applicable

Buphenyl Powder 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


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