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Drug Name:


Generic Name and Formulations:
Glycerol phenylbutyrate 1.1g/mL (delivers 1.02g/mL of phenylbutyrate); liquid.

Horizon Pharma

Therapeutic Use:

Indications for RAVICTI:

Chronic management of patients ≥2 months of age 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.

Adults and Children:

<2 months: contraindicated. Take with food or formula. Administer dose via oral syringe or dosing cup; may give by NG or gastrostomy tube: see full labeling. 2 months to <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) X 0.86 or total daily dose of sodium phenylbutyrate powder (g) x 0.81. Moderate-to-severe hepatic impairment: start at the lower end of dosing range. Dose adjustment and monitoring: see full labeling.


Children <2 months of age.


Monitor for signs/symptoms of 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.

Pharmacological Class:

Nitrogen-binding agent.


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).

Adverse Reactions:

Diarrhea, flatulence, headache, nausea, vomiting, decreased appetite, hyperammonemia, dizziness, fatigue, upper abdominal pain, rash. Also peds: neutropenia, pyrexia, hypophagia, cough, nasal congestion, rhinorrhea, papule.


Enroll women exposed to Ravicti during pregnancy by calling (855) 823-2595.

Generic Availability:


How Supplied:

Liq (25mL)—1, 4

Data provided by MPR.

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