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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