OLPRUVA (sodium phenylbutyrate) (Sodium Phenylbutyrate KIT)

OLPRUVA (sodium phenylbutyrate) is an FDA-approved orphan drug developed by Zevra Therapeutics, Inc. for the treatment of urea cycle disorders (UCDs). It is an oral medication that helps reduce ammonia levels in patients with these rare metabolic disorders by providing an alternative pathway for nitrogen excretion.

Pack Size

90 BOX in 1 CARTON (72542-200-09) / 1 KIT in 1 BOX (72542-200-02) * 1 FOR SUSPENSION in 1 PACKET

Manufacturer / TM Owner

Zevra Therapeutics, Inc.

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Indications & Clinical Uses

OLPRUVA is indicated for the treatment of urea cycle disorders (UCDs), a group of rare genetic metabolic disorders characterized by deficiencies in enzymes of the urea cycle, leading to hyperammonemia (elevated blood ammonia levels). The drug is used in pediatric and adult patients with UCDs to reduce ammonia concentrations and prevent hyperammonemic crises, which can cause severe neurological complications, encephalopathy, and developmental delays. Sodium phenylbutyrate works by providing an alternative pathway for nitrogen disposal; it is metabolized to phenylacetate, which conjugates with glutamine via the enzyme phenylacetyl-CoA:glutamine-N-acetyltransferase to form phenylacetylglutamine, which is then excreted in the urine. This mechanism effectively removes excess nitrogen from the body, bypassing the defective urea cycle and reducing toxic ammonia accumulation. OLPRUVA is particularly beneficial for patients with various UCD subtypes, including ornithine transcarbamylase (OTC) deficiency, carbamoyl phosphate synthetase deficiency, and other enzymatic defects in the urea cycle pathway.

Side Effects

Common side effects of OLPRUVA include gastrointestinal disturbances such as nausea, vomiting, abdominal pain, and diarrhea. Taste abnormalities and decreased appetite may also occur. Serious adverse events include hyperchloremic metabolic acidosis, hypokalemia (low potassium levels), and hypophosphatemia (low phosphate levels). Patients require regular monitoring of serum ammonia levels, electrolytes (sodium, potassium, chloride, phosphate), acid-base status, and renal function. Baseline and periodic assessment of liver function and nutritional status is essential. Blood ammonia levels should be monitored regularly to ensure therapeutic efficacy and prevent hyperammonemic episodes.

Precautions & Warnings

OLPRUVA is contraindicated in patients with known hypersensitivity to sodium phenylbutyrate or any component of the formulation. Use with caution in patients with renal impairment, as reduced renal function may affect drug elimination and increase risk of adverse effects. Hepatic impairment may require dose adjustment. Pregnancy: limited data available; use only if benefits outweigh risks. Pediatric patients require careful dose adjustment based on body weight and ammonia levels. Patients with hypokalemia, hyponatremia, or acid-base disturbances require close monitoring. Concomitant use with other ammonia-lowering agents should be coordinated carefully. Regular assessment of nutritional status is important, as protein restriction may be necessary in UCD management.

Avoid Interactions

OLPRUVA may interact with medications affecting renal function or electrolyte balance. Concurrent use with other nitrogen-scavenging agents (such as glycerol phenylbutyrate or lactulose) requires careful coordination and monitoring. NSAIDs and ACE inhibitors may increase risk of renal complications and electrolyte abnormalities when used with OLPRUVA. Corticosteroids may increase protein catabolism and ammonia production. Patients should avoid high-protein supplements unless specifically prescribed as part of their UCD management plan. Alcohol consumption should be minimized due to potential effects on liver function and ammonia metabolism.

Frequently Asked Questions

OLPRUVA is used to treat urea cycle disorders (UCDs), rare genetic metabolic conditions where the body cannot properly process ammonia. By reducing ammonia levels, OLPRUVA helps prevent hyperammonemic crises and associated neurological complications in both pediatric and adult patients with UCDs.

OLPRUVA is administered orally as a powder that is mixed with food or liquid. The dosage is individualized based on patient weight, ammonia levels, and clinical response. Patients should follow their healthcare provider's specific dosing instructions carefully.

Common side effects include nausea, vomiting, abdominal discomfort, diarrhea, and taste abnormalities. Most side effects are mild to moderate; however, patients should report persistent or severe symptoms to their healthcare provider immediately.

OLPRUVA is manufactured by Zevra Therapeutics, Inc., a biopharmaceutical company specializing in rare metabolic disorders.

Yes, OLPRUVA has been designated and approved as an orphan drug by the FDA. This designation recognizes its use in treating urea cycle disorders, which are rare diseases affecting a limited patient population.

Published by

GNH India Pharmaceuticals Limited

Licensed pharmaceutical supplier

Last updated: 17 Jun 2026

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