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Olpruva Generic Name & Formulations
Legal Class
General Description
Pharmacological Class
How Supplied
Kit—1 (pellets + suspending agent)
Manufacturer
Generic Availability
NO
Mechanism of Action
Sodium phenylbutyrate is a pro-drug and is metabolized to phenylacetate. Phenylacetate is a metabolically active compound that conjugates with glutamine via acetylation to form phenylacetylglutamine. Phenylacetylglutamine is excreted by the kidneys, hence providing an alternate vehicle for waste nitrogen excretion.
Olpruva Indications
Indications
Adjunct in the chronic management of urea cycle disorders involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase, or argininosuccinic acid synthetase.
Limitations of Use
Not for treatment of acute hyperammonemia.
Olpruva Dosage and Administration
Adults and Children
See full labeling. Take with food; drink entire susp within 5mins after stirring. Do not give via gastrostomy or NG tubes. ≥20kg and BSA ≥1.2m2: 9.9–13g/m2/day in 3–6 doses; max 20g/day. Use in combination with dietary protein restriction and/or amino acid supplementation (eg, essential amino acids, arginine, citrulline, protein-free calorie supplements). Hepatic impairment, elderly: start at the lower end of dosing range.
Olpruva Contraindications
Not Applicable
Olpruva Boxed Warnings
Not Applicable
Olpruva Warnings/Precautions
Warnings/Precautions
Risk for neurotoxicity (phenylacetate metabolite exposure). Obtain plasma levels of phenylacetate and phenylacetylglutamine. Consider reducing dose if neurologic symptoms (eg, vomiting, nausea, headache, somnolence, confusion) occur in absence of high ammonia levels or other incurrent illness. Monitor serum potassium; initiate treatment when necessary. Conditions associated with edema (eg, heart failure, cirrhosis, nephrosis): consider sodium content. Discontinue if new-onset edema develops or worsening edema during treatment. Renal impairment: monitor plasma ammonia levels at initiation. Hepatic impairment. Elderly. Pregnancy. Nursing mothers.
Olpruva Pharmacokinetics
Distribution
Apparent volume of distribution of phenylbutyrate: 7.2 L (under fasted conditions).
Elimination
Renal. Half-life: 0.5 hours (phenylbutyrate); 1.2 hours (phenylacetate).
Olpruva Interactions
Interactions
May be potentiated by probenecid. Corticosteroids may increase plasma ammonia levels. Hyperammonemia possible with haloperidol and valproic acid.
Olpruva Adverse Reactions
Adverse Reactions
Menstrual dysfunction, decreased appetite, body odor, bad taste, taste aversion; neurotoxicity.
Olpruva Clinical Trials
See Literature
Olpruva Note
Not Applicable
Olpruva Patient Counseling
See Literature
