Buphenyl (sodium phenylbutyrate)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Sodium phenylbutyrate is an ammonia detoxicant.
Sodium phenylbutyrate is used as adjunctive therapy for the chronic management of hyperammonemia in patients with urea cycle disorders.
Sodium phenylbutyrate is a prodrug that is rapidly metabolized to phenylacetate, an active metabolite. Phenylacetate is conjugated with glutamine in the liver and kidneys to form phenylacetylglutamine, a nitrogen waste product that is excreted by the kidneys and provides an alternative to urea for the excretion of waste nitrogen. The nitrogen content of phenylacetylglutamine is identical to that of urea (i.e., both contain 2 moles of nitrogen).
Glutamine used in these reactions is replaced by synthesis, thereby reducing the nitrogen pool and attenuating the risk of ammonia- and glutamine-induced neurotoxicity in patients with deficiencies of urea cycle enzymes. (For further information on the role of ammonia detoxicants in the management of urea cycle disorders, see Description in Sodium Phenylacetate and Sodium Benzoate 40:10.)
Although sodium phenylbutyrate lacks the extremely disagreeable odor of sodium phenylacetate, it has a strong, musty odor, and has a strong unpleasant salty taste.
USES: This medication is used along with diet changes for long-term treatment of a certain inherited disorder (urea cycle disorder). Sodium phenylbutyrate helps remove a certain chemical (ammonia) from the body. Too much ammonia in the body can cause brain damage and sometimes death.
This medication should not be used to treat sudden, severely high levels of ammonia in the body. Get medical help right away if you have sudden symptoms of too much ammonia (such as trouble thinking, nausea, vomiting, irritability, trouble speaking, trouble walking, fainting).
Generic name: sodium phenylbutyrate
Pronunciation: (SOE-dee-um FEN-il-BUE-ti-rate)
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Buphenyl
Indications
The following indications are listed for Buphenyl:
- Carbamoyl Phosphate Synthetase Deficiency
- Citrullinemia
- Ornithine Carbamoyltransferase Deficiency
Also known as: Argininosuccinate Synthetase Deficiency, Argininosuccinic Acid Synthetase Deficiency, Caps Deficiency, Carbamoyl-Phosphate Synthetase Deficiency, Cps Deficiency, Hyperammonemia Due To Ornithine Transcarbamylase Deficiency, Hyperammonemic Syndrome, Ornithine Transcarbamoylase Deficiency, Ornithine Transcarbamylase Deficiency.
Dosing
Dosage of sodium phenylbutyrate is expressed in terms of the salt. Dosage of sodium phenylbutyrate is based on body weight in neonates, infants, and children weighing less than 20 kg and on body surface area in pediatric patients weighing at least 20 kg and in adults. Sodium phenylbutyrate tablets are used in pediatric patients weighing more than 20 kg and in adults. When the teaspoon provided by the manufacturer is used to measure sodium phenylbutyrate oral powder, each level teaspoonful of the powder provides 3 g of sodium phenylbutyrate. When the tablespoon provided by the manufacturer is used, each level tablespoonful of the powder provides 8.6 g of sodium phenylbutyrate.
For the chronic management of hyperammonemia in patients with urea cycle disorders, the usual oral dosage of sodium phenylbutyrate powder in neonates, infants and children weighing less than 20 kg is 450-600 mg/kg daily, given in 3-6 equally divided doses. In children weighing 20 kg or more and in adults, the usual oral dosage of sodium phenylbutyrate powder or tablets is 9.9-13 g/m2 daily, given in 3-6 equally divided doses. Safety and efficacy of dosages exceeding 20 g daily have not been established.
Sodium phenylbutyrate is administered orally. Sodium phenylbutyrate powder also may be administered via a gastrostomy or nasogastric tube. Although the effect of food on the drug has not been determined, sodium phenylbutyrate should be administered with meals or feedings. Sodium phenylbutyrate powder should be lightly shaken before use and then mixed with solid or liquid food before administration. Although sodium phenylbutyrate is very soluble in water and will dissolve when the powder is added to a liquid, the excipients in the commercially available formulation will not dissolve.
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Drug interactions
There is no enhanced messaging for this drug.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
Known hypersensitivity to sodium phenylbutyrate or any ingredient in the formulation. Treatment of acute hyperammonemia which is a medical emergency.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
There are no moderate contraindications for this drug.
Adverse reactions
Adverse effects reported in 3% or more of patients receiving sodium phenylbutyrate include amenorrhea or menstrual dysfunction, decreased appetite, body odor, dysgeusia, acidosis, hypoalbuminemia, alkalosis and hyperchloremia, anemia, hypophosphatemia, increased concentrations of alkaline phosphatase and aminotransferase, leukopenia, leukocytosis, and thrombocytopenia.
There are no severe adverse reactions for this drug.
There are no less severe adverse reactions for this drug.
Precautions
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Use of sodium phenylbutyrate tablets in neonates, infants and children weighing less than 20 kg is not recommended.
Category C. (See Users Guide)
Not known whether sodium phenylbutyrate is distributed into milk. Caution is advised if the drug is administered in nursing women.
No enhanced messaging available.
ICD codes
No ICD codes are available for this drug.
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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