RAVICTI glycerol phenylbutyrate liquid

Země: Spojené státy

Jazyk: angličtina

Zdroj: NLM (National Library of Medicine)

Aktivní složka:

GLYCEROL PHENYLBUTYRATE (UNII: ZH6F1VCV7B) (GLYCEROL PHENYLBUTYRATE - UNII:ZH6F1VCV7B)

Dostupné s:

Hyperion Therapeutics, Inc.

INN (Mezinárodní Name):

GLYCEROL PHENYLBUTYRATE

Složení:

GLYCEROL PHENYLBUTYRATE 1.1 g in 1 mL

Druh předpisu:

PRESCRIPTION DRUG

Stav Autorizace:

New Drug Application

Charakteristika produktu

RAVICTI- GLYCEROL PHENYLBUTYRATE LIQUID
HYPERION THERAPEUTICS, INC.
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HIGHLIGHTS OF PRESCRIBING INFORMATION
THESE HIGHLIGHTS DO NOT INCLUDE ALL THE INFORMATION NEEDED TO USE
RAVICTI SAFELY AND EFFECTIVELY. SEE FULL PRESCRIBING INFORMATION FOR
RAVICTI.
RAVICTI™ (GLYCEROL PHENYLBUTYRATE) ORAL LIQUID
INITIAL U.S. APPROVAL: 1996
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). (1)
Limitations of Use:
RAVICTI is not indicated for treatment of acute hyperammonemia in
patients with UCDs. (1)
Safety and efficacy for treatment of _N_-acetylglutamate synthase
(NAGS) deficiency has not been established. (1)
The use of RAVICTI in patients <2 months of age is contraindicated (4)
DOSAGE AND ADMINISTRATION
RAVICTI should be prescribed by a physician experienced in management
of UCDs. (2.1)
Instruct patients to take with food and to administer directly into
mouth via oral syringe or dosing cup. (2.1)
Total daily dosage is given in 3 equally divided dosages, rounded up
to nearest 0.5 mL. (2.1)
Maximum daily dosage is 17.5 mL (19 g). (2.1)
Must be used with dietary protein restriction. (2.1)
Switching From Sodium Phenylbutyrate to RAVICTI:
Daily dosage of RAVICTI (mL) = daily dosage of sodium phenylbutyrate
(g) x 0.86. (2.2)
Initial Dosage in Phenylbutyrate-Naïve Patients:
Recommended dosage range is 4.5 to 11.2 mL/m /day (5 to 12.4 g/m
/day). (2.3)
For patients with some residual enzyme activity who are not adequately
controlled with dietary restriction,
recommended starting dose is 4.5 mL/m /day. (2.3)
Take into account patient's estimated urea synthetic capacity, dietary
protein intake, and diet adherence. (2.3)