SODIUM PHENYLBUTYRATE powder

Country: United States

Language: English

Source: NLM (National Library of Medicine)

Active ingredient:

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

Available from:

Sigmapharm Laboratories, LLC

INN (International Name):

SODIUM PHENYLBUTYRATE

Composition:

SODIUM PHENYLBUTYRATE 0.94 g in 1 g

Administration route:

ORAL

Prescription type:

PRESCRIPTION DRUG

Therapeutic indications:

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 t

Product summary:

Sodium Phenylbutyrate Powder is available in 500 cc bottles, which hold 266 grams of powder, containing 250 grams of sodium Phenylbutyrate (NDC 42794-086-14). The bottles are equipped with child-resistant caps. Measurers are provided. 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 20ºC TO 25ºC (68ºF TO 77ºF) [SEE USP CONTROLLED ROOM TEMPERATURE]. AFTER OPENING, KEEP BOTTLE TIGHTLY CLOSED. Manufactured by: Sigmapharm Laboratories, LLC Bensalem, PA 19020 NDC 42794-086-14 bottle containing 250 grams of sodium phenylbutyrate powder. OS086-04 REV.0817

Authorization status:

Abbreviated New Drug Application

Summary of Product characteristics

SODIUM PHENYLBUTYRATE- SODIUM PHENYLBUTYRATE POWDER
SIGMAPHARM LABORATORIES, LLC
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SODIUM PHENYLBUTYRATE POWDER (SO-DEE-UM FEN-ILL-BYOU-TIH-RATE) RX ONLY
DESCRIPTION
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 gram of Sodium Phenylbutyrate 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:
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 powder with a C
of 195 µg/mL under fasting conditions.
10
11
2
max
The effect of food on phenylbutyrate's absorption is unknown.
DISPOSITION:
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. However, the drug is known to
be metabolized
to phenylacetat