• Inborn errors of metabolism

Ravicti Generic Name & Formulations

General Description

Glycerol phenylbutyrate 1.1g/mL (delivers 1.02g/mL of phenylbutyrate); liquid.

Pharmacological Class

Nitrogen-binding agent.

How Supplied

Liq (25mL)—1, 4


Generic Availability


Ravicti Indications


Chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone.

Limitations of Use

Not for treatment of acute hyperammonemia in patients with UCDs. Safety and efficacy for the treatment of N-acetylglutamate synthase (NAGS) deficiency has not been established.

Ravicti Dosage and Administration

Adults and Children

Take with food or formula. Administer dose via oral syringe; may give by NG or gastrostomy tube: see full labeling. <2yrs: give in 3 or more equally divided doses, rounded to nearest 0.1mL. ≥2yrs: give in 3 equally divided doses, rounded to nearest 0.5mL. Max 17.5mL (19g) daily. Must be used with dietary protein restriction and, in some cases, dietary supplements (eg, essential amino acids, arginine, citrulline, protein-free calorie supplements). Phenylbutyrate-naïve: 4.5–11.2mL/m2/day (5–12.4g/m2/day); patients with some residual enzyme activity not adequately controlled with protein restrictions: 4.5mL/m2/day. To determine starting dose in treatment-naïve patients: consider the patient’s residual urea synthetic capacity, dietary protein requirements, and diet adherence. Switching from sodium phenylbutyrate: give dose that contains the same amount of phenylbutyric acid; convert as follows: Total daily dose of Ravicti (mL) = total daily dose of sodium phenylbutyrate tablets (g) × 0.86 or total daily dose of sodium phenylbutyrate powder (g) × 0.81. Moderate to severe hepatic impairment: start at the lower end of dosing range. Dose adjustment and monitoring: see full labeling.

Ravicti Contraindications

Not Applicable

Ravicti Boxed Warnings

Not Applicable

Ravicti Warnings/Precautions


Risk for neurotoxicity; reduce dose if occurs. Pancreatic insufficiency or intestinal malabsorption: closely monitor ammonia levels. Hepatic or renal impairment: monitor closely. Pregnancy. Nursing mothers: not recommended.

Ravicti Pharmacokinetics

See Literature

Ravicti Interactions


Concomitant corticosteroids, valproic acid, or haloperidol may increase plasma ammonia levels; monitor closely. May be potentiated by probenecid. Antagonizes midazolam; monitor. May antagonize CYP3A4 substrates with narrow therapeutic index (eg, alfentanil, quinidine, cyclosporine).

Ravicti Adverse Reactions

Adverse Reactions

Diarrhea, flatulence, headache, nausea, vomiting, decreased appetite, dizziness, fatigue, abdominal pain; hyperammonemia. In children: also rash, neutropenia, constipation, pyrexia, hypophagia, cough, nasal congestion, rhinorrhea, papule.

Ravicti Clinical Trials

See Literature

Ravicti Note


Enroll women exposed to Ravicti during pregnancy by calling (866) 479-6742.

Ravicti Patient Counseling

See Literature