Country: Stati Uniti
Lingwa: Ingliż
Sors: NLM (National Library of Medicine)
SODIUM PHENYLBUTYRATE (UNII: NT6K61736T) (PHENYLBUTYRIC ACID - UNII:7WY7YBI87E)
Par Pharmaceutical, Inc.
SODIUM PHENYLBUTYRATE
SODIUM PHENYLBUTYRATE 0.94 g in 1 g
ORAL
PRESCRIPTION DRUG
Sodium phenylbutyrate powder is indicated as adjunctive therapy in the chronic management of patients with urea cycle disorders involving deficiencies of carbamylphosphate 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. Sodium phenylbutyrate powder 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, neona
Sodium phenylbutyrate powder is available in 500 cc bottles, which hold 266 grams of powder, containing 250 grams of sodium phenylbutyrate (NDC 49884-006-04). The bottles are equipped with child-resistant caps. Measurers are provided. A teaspoon and tablespoon come with each bottle. A yellow color teaspoon and blue color tablespoon are enclosed with this product. Each level yellow teaspoon (enclosed) dispenses 3.2 grams of powder and 3 grams of sodium phenylbutyrate. Each level blue tablespoon (enclosed) dispenses 9.1 grams of powder and 8.6 grams of sodium phenylbutyrate. Store at 20º to 25ºC (68° to 77°F); [See USP Controlled Room Temperature]; Excursions permitted to 15° to 30°C (59° to 86°F). After Opening, Keep Bottle Tightly Closed.
Abbreviated New Drug Application
SODIUM PHENYLBUTYRATE- SODIUM PHENYLBUTYRATE POWDER PAR PHARMACEUTICAL, INC. ---------- SODIUM PHENYLBUTYRATE POWDER RX ONLY DESCRIPTION Sodium phenylbutyrate powder nasogastric, or gastrostomy tube administration contain sodium phenylbutyrate. Sodium phenylbutyrate powder 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 gram of sodium phenylbutyrate powder contains 0.94 grams of sodium phenylbutyrate and the inactive ingredients calcium stearate and colloidal silicon dioxide. 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: Pharmacokinetic studies have not been conducted in the primary patient population (neonates, infants, and children), but pharmacokinetic data were obtained from normal adult subjects. ABS ORPTION: Peak plasma levels of phenylbutyrate occur within 1 hour after a single dose of 5 grams of sodium phenylbutyrate powder with a C of 195 mcg/mL under fasting conditions. The effect of food on phenylbutyrate's absorption is unknown. DIS POS ITION: The overall disposition of sodium phenylbutyrate and its metabolites has not been characterized fully. However, the drug is known to be metabolized to phenylacetate and subsequently to phenylacetylglutamine. Following oral administration of 5 grams of the powder, measurable plasma levels of phenylbutyrate and phenylacetate Aqra d-dokument sħiħ