PHEBURANE- sodium phenylbutyrate pellet PHEBURANE- sodium phenylbutyrate kit

Land: Bandaríkin

Tungumál: enska

Heimild: NLM (National Library of Medicine)

Virkt innihaldsefni:

SODIUM PHENYLBUTYRATE (UNII: NT6K61736T) (PHENYLBUTYRIC ACID - UNII:7WY7YBI87E)

Fáanlegur frá:

Medunik USA

Stjórnsýsluleið:

ORAL

Gerð lyfseðils:

PRESCRIPTION DRUG

Ábendingar:

PHEBURANE is indicated as adjunctive therapy to standard of care, which includes dietary management, for the chronic management of adult and pediatric patients with urea cycle disorders (UCDs), involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC) or argininosuccinic acid synthetase (AS). Limitations of Use Episodes of acute hyperammonemia may occur in patients while on PHEBURANE. PHEBURANE is not indicated for the treatment of acute hyperammonemia, which can be a life-threatening medical emergency that requires rapid acting interventions to reduce plasma ammonia levels. None Risk Summary Available data with sodium phenylbutyrate use in pregnant women are insufficient to identify a drug associated risk of major birth defects, miscarriage or adverse maternal or fetal outcomes. Animal reproduction studies have not been conducted with sodium phenylbutyrate. Based on published animal data, phenylacetate may be neurotoxic to the developing brain (see Data) . There are serio

Vörulýsing:

PHEBURANE (sodium phenylbutyrate) oral pellets consists of white to off-white pellets and is available in a child-resistant high-density polyethylene (HDPE) bottle with a desiccant in the cap. Each bottle contains 84 g of sodium phenylbutyrate (equivalent to 74 g of phenylbutyrate) in 174 g of oral pellets (NDC 71770-200-10). Each gram of pellets contains 483 mg of sodium phenylbutyrate (equivalent to 423 mg of phenylbutyrate). A calibrated measuring spoon that dispenses up to 3 g of sodium phenylbutyrate in increments of 0.25 g is provided in the packaging. Store PHEBURANE at 20°C to 25°C (68°F to 77°F); excursions permitted between 15°C and 30°C (59°F and 86°F) [see USP Controlled Room Temperature]. Discard any remaining PHEBURANE 45 days after first opening of the bottle.

Leyfisstaða:

New Drug Application

Vara einkenni

PHEBURANE- SODIUM PHENYLBUTYRATE PELLET
PHEBURANE- SODIUM PHENYLBUTYRATE
MEDUNIK USA
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HIGHLIGHTS OF PRESCRIBING INFORMATION
THESE HIGHLIGHTS DO NOT INCLUDE ALL THE INFORMATION NEEDED TO USE
PHEBURANE SAFELY AND
EFFECTIVELY. SEE FULL PRESCRIBING INFORMATION FOR PHEBURANE.
PHEBURANE (SODIUM PHENYLBUTYRATE) ORAL PELLETS
INITIAL U.S. APPROVAL: 1996
INDICATIONS AND USAGE
PHEBURANE is a nitrogen-binding agent indicated as adjunctive therapy
to standard of care, which
includes dietary management, for the chronic management of adult and
pediatric patients with urea cycle
disorders (UCDs), involving deficiencies of carbamylphosphate
synthetase (CPS), ornithine
transcarbamylase (OTC) or argininosuccinic acid synthetase (AS). (1)
Limitations of Use
PHEBURANE is not indicated for the treatment of acute hyperammonemia.
(1)
DOSAGE AND ADMINISTRATION
PHEBURANE treatment should be supervised by a healthcare provider
experienced in the treatment of
UCDs. For administration, see full prescribing information. (2.1, 2.4)
The recommended dosage measured as sodium phenylbutyrate is:
Patients weighing < 20 kg: 450–600 mg/kg/day of sodium
phenylbutyrate orally. (2.1)
Patients weighing ≥ 20 kg: 9.9–13.0 g/m /day of sodium
phenylbutyrate orally. (2.1)
Monitor plasma ammonia levels to determine the need for dosage
adjustment. (2.2)
Monitor patients for potential neurotoxicity. (2.2)
For patients with hepatic impairment, start at the lower end of the
recommended dosing range. (2.3)
DOSAGE FORMS AND STRENGTHS
Oral pellets: 84 g of sodium phenylbutyrate per bottle. (3)
CONTRAINDICATIONS
None. (4)
WARNINGS AND PRECAUTIONS
Neurotoxicity of Phenylacetate: Increased exposure to phenylacetate,
the major metabolite of
PHEBURANE, may be associated with neurotoxicity in patients with UCDs.
Consider reducing the dose if
neurotoxicity symptoms are present. (5.1)
Hypokalemia: Renal excretion of phenylacetylglutamine may induce
urinary loss of potassium. Monitor
serum potassium during therapy and initiate appropriate treatment when
necess