البلد: الولايات المتحدة
اللغة: الإنجليزية
المصدر: NLM (National Library of Medicine)
SODIUM PHENYLBUTYRATE (UNII: NT6K61736T) (PHENYLBUTYRIC ACID - UNII:7WY7YBI87E)
Horizon Therapeutics USA, Inc.
SODIUM PHENYLBUTYRATE
SODIUM PHENYLBUTYRATE 500 mg
ORAL
PRESCRIPTION DRUG
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
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.
New Drug Application
BUPHENYL- SODIUM PHENYLBUTYRATE TABLET BUPHENYL- SODIUM PHENYLBUTYRATE POWDER HORIZON THERAPEUTICS USA, INC. ---------- 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 اقرأ الوثيقة كاملة