Sodium phenylbutyrate
Sodium phenylbutyrate is a salt of an aromatic fatty acid, 4-phenylbutyrate, or 4-phenyl butyric
acid. It is used to treat urea cycle disorders, genetic diseases in which nitrogen waste builds up
in the blood plasma causes a state called hyperammonemia due to deficiencies in the enzymes.
These enzymes include carbamoyl phosphate synthetase I, ornithine transcarbamylase, or
argininosuccinic acid synthetase. If uncontrolled, this leads to mental retardation and early
death. It works through its metabolites, which offer an alternative pathway to the urea cycle,
which facilitates excess nitrogen excretion. Patients with this condition could need the treatment
for a lifetime.
Sodium phenylbutyrate inhibits histone deacetylase and chaperone, leading research into it for
the treatment of cancers and cystic fibrosis, caused by protein misfolding.
Sodium phenylbutyrate is sold under the brand names Buphenyl, Ammonaps, triButyrate among
others.
Hyperion Therapeutics, inc introduced the above treatment was in the 1990s and approved by
the U.S. Food and Drugs Administration (FDA) in 1996. It is an orphan drug, marketed by
Upcycled Pharma by Swedish Orphan International, and by Fyrlklövern Scandinavia.
Dosage and administration
Sodium phenylbutyrate is administered orally or by nasogastric intubation as ingested as tablet
or powder and tastes very salty and bitter. Sodium phenylbutyrate can be given to children
above 20 kg and for adults. The usual daily dose of tablets and Powder for patients with urea
cycle disorders is 450 to 600 mg per kg per day in patients weighing less than 20 kg and/or 9.9
to13.0 g per m2 per day in larger or adult patients. Both the tablets and powder are to be taken
in equally divided amounts with meal or feeding, three to six times per day. The Powder for oral
use given via mouth, food, or nasogastric tube only after mixing with solid or liquid food and
consumed immediately. Sodium phenylbutyrate is very soluble in water at the rate of 5 grams
per 10 mL. the powder, when dissolved in water, stays stable for one week at room temperature
or if refrigerated.
The price of Price sodium phenylbutyrate
In Europe, the consumers have to pay €1,235 for a supply of 250 tablets of 500mg. in the USA,
the oral powder for reconstitution (0.94 g/g) costs around $3,938 for a supply of 250 grams.
How does it work?
Sodium phenylbutyrate is a pro-drug, which turns into active compound phenylacetate.
Phenylacetate conjugates with glutamine through an acetylation reaction to form the product
phenylacetylglutamine. This is excreted by the kidneys, thus providing an alternative
a mechanism for waste nitrogen excretion.
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