|See warnings and precautions below.|
| RAVICTI™ (glycerol phenylbutyrate) oral liquid
Initial U.S. Approval: 1996
RAVICTI (glycerol phenylbutyrate) is a clear, colorless to pale yellow oral liquid. It is insoluble in water and most organic solvents, and it is soluble in dimethylsulfoxide (DMSO) and >65% acetonitrile.
Glycerol phenylbutyrate is a nitrogen-binding agent. It is a triglyceride containing 3 molecules of PBA linked to a glycerol backbone, the chemical name of which is benzenebutanoic acid, 1′, 1′ ‘ –(1,2,3-propanetriyl) ester with a molecular weight of 530.67.
| Mechanism of Action:
UCDs are inherited deficiencies of enzymes or transporters necessary for the synthesis of urea from ammonia (NH3, NH4+). Absence of these enzymes or transporters results in the accumulation of toxic levels of ammonia in the blood and brain of affected patients. RAVICTI is a triglyceride containing 3 molecules of phenylbutyrate (PBA). PAA, the major metabolite of PBA, is the active moiety of RAVICTI. PAA conjugates with glutamine (which contains 2 molecules of nitrogen) via acetylation in the liver and kidneys to form PAGN, which is excreted by the kidneys. On a molar basis, PAGN, like urea, contains 2 moles of nitrogen and provides an alternate vehicle for waste nitrogen excretion.
| INDICATIONS AND USAGE:
RAVICTI is indicated for use as a nitrogen-binding agent for chronic management of adult and pediatric patients 2 years of age with urea cycle disorders (UCDs) that cannot be managed by dietary protein restriction and/or amino acid supplementation alone. RAVICTI must be used with dietary protein restriction and, in some cases, dietary supplements (e.g., essential amino acids, arginine, citrulline, protein-free calorie supplements).
Limitations of Use:
Safety and efficacy for treatment of N-acetylglutamate synthase (NAGS) deficiency has not been established.
The use of RAVICTI in patients <2 months of age is contraindicated
WARNINGS AND PRECAUTIONS:
| ADVERSE REACTIONS
Most common adverse reactions in 10% of patients are diarrhea, flatulence, and headache.
To report SUSPECTED ADVERSE REACTIONS, contact Hyperion Therapeutics at 1-855-823-7878 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
Probenecid: May affect renal excretion of metabolites of RAVICTI, including PAGN and PAA.
USE IN SPECIFIC POPULATIONS
Nursing Mothers: Discontinue nursing or discontinue the drug.
See PACKAGE INSERT for PATIENT COUNSELING INFORMATION and Medication Guide.
| DOSAGE AND ADMINISTRATION:
RAVICTI should be prescribed by a physician experienced in management of UCDs.
Instruct patients to take with food and to administer directly into mouth via oral syringe or dosing cup.
Maximum daily dosage is 17.5 mL (19 g).
Switching From Sodium Phenylbutyrate to RAVICTI:
Initial Dosage in Phenylbutyrate-Naïve Patients:
For patients with some residual enzyme activity who are not adequately controlled with dietary restriction, recommended starting dose is 4.5 mL/m2/day.
Take into account patient’s estimated urea synthetic capacity, dietary protein intake, and diet adherence.
Dosage Modifications in Patients With Hepatic Impairment:
| Package insert data: [Accessed: Jan 2014].
Issued: February 2013
© Hyperion Therapeutics, Inc.
RAVICTI is a trademark of Hyperion Therapeutics, Inc.
National Institutes of Health, U.S. National Library of Medicine, DailyMed Database.
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