BUPHENYL- sodium phenylbutyrate tablet BUPHENYL- sodium phenylbutyrate powder

Nchi: Marekani

Lugha: Kiingereza

Chanzo: NLM (National Library of Medicine)

Viambatanisho vya kazi:

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

Inapatikana kutoka:

Horizon Therapeutics USA, Inc.

INN (Jina la Kimataifa):

SODIUM PHENYLBUTYRATE

Tungo:

SODIUM PHENYLBUTYRATE 500 mg

Njia ya uendeshaji:

ORAL

Dawa ya aina:

PRESCRIPTION DRUG

Matibabu dalili:

BUPHENYL is indicated as adjunctive therapy in the chronic management of patients with urea cycle disorders involving deficiencies of carbamoyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS). It is indicated in all patients with neonatal-onset deficiency (complete enzymatic deficiency, presenting within the first 28 days of life). It is also indicated in patients with late-onset disease (partial enzymatic deficiency, presenting after the first month of life) who have a history of hyperammonemic encephalopathy. It is important that the diagnosis be made early and treatment initiated immediately to improve survival. Any episode of acute hyperammonemia should be treated as a life-threatening emergency. BUPHENYL must be combined with dietary protein restriction and, in some cases, essential amino acid supplementation. (See Nutritional Supplementation subsection of the DOSAGE AND ADMINISTRATION section.) Previously, neonatal-onset disease was almost universal

Bidhaa muhtasari:

BUPHENYL Tablets are available in 250 cc bottles which contain 250 sodium phenylbutyrate tablets (NDC 75987-060-08). The bottles are equipped with child-resistant caps. Each tablet is off-white, oval, and embossed with "UCY 500". Each tablet contains 500 mg of sodium phenylbutyrate. STORE AT ROOM TEMPERATURE 15°C–30°C (59°F–86°F). AFTER OPENING, KEEP BOTTLE TIGHTLY CLOSED. BUPHENYL Powder is available in 500 cc bottles, which hold 266 grams of powder, containing 250 grams of sodium phenylbutyrate (NDC 75987-070-09). The bottles are equipped with child-resistant caps. Measurers are provided. Each level teaspoon (enclosed) dispenses 3.2 grams of powder and 3.0 grams of sodium phenylbutyrate. Each level tablespoon (enclosed) dispenses 9.1 grams of powder and 8.6 grams of sodium phenylbutyrate. STORE AT ROOM TEMPERATURE 15ºC–30ºC (59ºF–86ºF). AFTER OPENING, KEEP BOTTLE TIGHTLY CLOSED.

Idhini hali ya:

New Drug Application

Tabia za bidhaa

BUPHENYL- SODIUM PHENYLBUTYRATE TABLET
BUPHENYL- SODIUM PHENYLBUTYRATE POWDER
HORIZON THERAPEUTICS USA, INC.
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BUPHENYL (SODIUM PHENYLBUTYRATE) TABLETS
BUPHENYL (SODIUM PHENYLBUTYRATE) POWDER
[bu'fen-əl]
(SODIUM PHENYLBUTYRATE)
Rx Only
DESCRIPTION
BUPHENYL (sodium phenylbutyrate) Tablets for oral administration and
BUPHENYL
(sodium phenylbutyrate) Powder for oral, nasogastric, or gastrostomy
tube
administration contain sodium phenylbutyrate. Sodium phenylbutyrate is
an off-white
crystalline substance which is soluble in water and has a strong salty
taste. Sodium
phenylbutyrate also is freely soluble in methanol and practically
insoluble in acetone and
diethyl ether. It is known chemically as 4-phenylbutyric acid, sodium
salt with a molecular
weight of 186 and the molecular formula C
H
O Na.
Chemical Structure:
Each tablet of BUPHENYL contains 500 mg of sodium phenylbutyrate and
the inactive
ingredients microcrystalline cellulose NF, magnesium stearate NF, and
colloidal silicon
dioxide NF.
Each gram of BUPHENYL Powder contains 0.94 grams of sodium
phenylbutyrate and
the inactive ingredients calcium stearate NF, and colloidal silicon
dioxide NF.
CLINICAL PHARMACOLOGY
Sodium phenylbutyrate is a pro-drug and is rapidly metabolized to
phenylacetate.
Phenylacetate is a metabolically active compound that conjugates with
glutamine via
acetylation to form phenylacetylglutamine. Phenylacetylglutamine then
is excreted by the
kidneys. On a molar basis, it is comparable to urea (each containing
two moles of
nitrogen). Therefore, phenylacetylglutamine provides an alternate
vehicle for waste
nitrogen excretion.
PHARMACOKINETICS
GENERAL
®
®
10
11
2
Pharmacokinetic studies have not been conducted in the primary patient
population
(neonates, infants, and children), but pharmacokinetic data were
obtained from normal
adult subjects.
ABSORPTION
Peak plasma levels of phenylbutyrate occur within 1 hour after a
single dose of 5 grams
of sodium phenylbutyrate tablet with a C
of 218 µg/mL under fasting conditions;
peak plasma