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What is Sodium Phenylbutyrate
Phenylbutyric acidThe Generic name of this drug
Treatment SummaryButyric acid phenyl ester is a type of monocarboxylic acid used to inhibit histone deacetylase. It has been found to have anticancer activity, inhibiting cell proliferation, invasion, and migration, as well as inducing apoptosis in glioma cells. Additionally, butyric acid phenyl ester inhibits protein isoprenylation, decreases plasma glutamine, increases production of foetal hemoglobin, and affects the activation of hPPARγ.
Buphenylis the brand name
Sodium Phenylbutyrate Overview & Background
Brand Name
Generic Name
First FDA Approval
How many FDA approvals?
Buphenyl
Phenylbutyric acid
1996
20
Effectiveness
How Sodium Phenylbutyrate Affects PatientsPhenylbutyric acid is used to treat patients with urea cycle disorders, which involve high levels of ammonia and glutamine in the blood. It helps the body get rid of extra nitrogen waste. In the intestines, phenylbutyric acid has been shown to reduce inflammation and regulate fluid flow. It may also have anti-cancer properties and could be used to prevent or treat certain types of cancer. It may work by acting as an ammonia scavenger, chemical chaperone, and histone deacetylase inhibitor.
How Sodium Phenylbutyrate works in the bodySodium phenylbutyrate is a form of phenylbutyric acid used as a drug in the body. It is quickly converted into phenylacetate, which then combines with glutamine to form phenylacetylglutamine. This compound is then passed by the kidneys and helps remove nitrogen from the body in a similar way to urea.
When to interrupt dosage
The measure of Sodium Phenylbutyrate is contingent upon the diagnosed condition, including Ornithine Transcarbamylase Deficiency, Citrullinemia and Enzymes. The quantity of dosage fluctuates as per the technique of delivery (e.g. Powder, for suspension - Oral or Oral) featured in the table below.Condition
Dosage
Administration
Amyotrophic Lateral Sclerosis
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Protein Restriction
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Late Onset Disorders
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Hyperammonemia
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
neonatal-onset deficiency
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Disorder of the Urea Cycle
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Carbamyl Phosphate
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Enzymes
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Citrullinemia
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Ornithine Transcarbamylase Deficiency
500.0 mg, , 0.94 mg/mg, 0.483 mg/mg
, Oral, Tablet, Tablet - Oral, Granule, Granule - Oral, Powder, Powder - Oral
Warnings
Sodium Phenylbutyrate has two contraindications and should be avoided when afflicted with any of the conditions listed in the following table.Sodium Phenylbutyrate ContraindicationsCondition
Risk Level
Notes
Severe Hypersensitivity Reactions
Do Not Combine
Phenylbutyric Acid may interact with Pulse Frequency
Common Sodium Phenylbutyrate Drug Interactions
Drug Name
Risk Level
Description
4-Methoxyamphetamine
Moderate
The metabolism of 4-Methoxyamphetamine can be decreased when combined with Phenylbutyric acid.
5-methoxy-N,N-dimethyltryptamine
Moderate
The metabolism of 5-methoxy-N,N-dimethyltryptamine can be decreased when combined with Phenylbutyric acid.
Acebutolol
Moderate
The metabolism of Acebutolol can be decreased when combined with Phenylbutyric acid.
Acetaminophen
Moderate
The metabolism of Acetaminophen can be decreased when combined with Phenylbutyric acid.
Almotriptan
Moderate
The metabolism of Almotriptan can be decreased when combined with Phenylbutyric acid.
Sodium Phenylbutyrate Toxicity & Overdose RiskTaking too much sodium phenylbutyrate can be deadly, with the lethal dose being 1586mg/kg. No dangerous side effects of overdosing have been reported in patients with urea cycle disorders. High levels of phenylacetate, the active metabolite of phenylbutyric acid, can cause nausea, headache, fatigue, weakness, dizziness, slurred speech, memory loss, confusion, and disorientation. If you have overdosed on sodium phenylbutyrate, stop taking it and get medical help. Dialysis may also be used to help reduce the amount of the drug in your body.
Sodium Phenylbutyrate Novel Uses: Which Conditions Have a Clinical Trial Featuring Sodium Phenylbutyrate?
Two active investigations are currently assessing the potential of Sodium Phenylbutyrate to address Citrullinemia, Ornithine Transcarbamylase Deficiency and Carbamyl Phosphate deficiencies.Condition
Clinical Trials
Trial Phases
Protein Restriction
0 Actively Recruiting
neonatal-onset deficiency
0 Actively Recruiting
Citrullinemia
0 Actively Recruiting
Hyperammonemia
0 Actively Recruiting
Disorder of the Urea Cycle
0 Actively Recruiting
Ornithine Transcarbamylase Deficiency
0 Actively Recruiting
Carbamyl Phosphate
0 Actively Recruiting
Amyotrophic Lateral Sclerosis
14 Actively Recruiting
Phase 1, Phase 2, Not Applicable
Enzymes
0 Actively Recruiting
Late Onset Disorders
4 Actively Recruiting
Not Applicable
Sodium Phenylbutyrate Reviews: What are patients saying about Sodium Phenylbutyrate?
4.7Patient Review
2/10/2009 I was born with Ornathine TransCarbamylase Deficiency and I have been taking the drug since I was 2 years of age. Its very important for me to take care of my disease or it could result in coma or death.
Patient Q&A Section about sodium phenylbutyrate
These questions and answers are submitted by anonymous patients, and have not been verified by our internal team.What is sodium phenylbutyrate used for?
"Sodium phenylbutyrate is a drug used to treat urea cycle disorders, which are conditions that prevent the body from removing ammonia (nitrogen) from the blood. Ammonia is produced when protein is broken down in the body."
Answered by AI
Is sodium phenylbutyrate FDA approved?
"After nearly two years of discussions, the US Food and Drug Administration has approved Ucyclyd Pharma's orphan drug, Buphenyl (sodium phenylbutyrate), to treat urea cycle disorders. The drug allows patients to excrete waste nitrogen which would otherwise accumulate as ammonia and may cause brain damage and death."
Answered by AI
Is sodium phenylbutyrate over the counter?
"Ammonia is a substance that is produced when protein breaks down in the body. If the body is unable to get rid of the ammonia, it can cause serious problems. sodium phenylbutyrate helps to reduce the amount of ammonia in the blood. This drug is only available with a prescription from a doctor."
Answered by AI
Is sodium phenylbutyrate the same as sodium butyrate?
"PBA is a derivative of the short-chain fatty acid butyrate and is approved for treatment of urea cycle disorders and progressive familial intrahepatic cholestasis type 2."
Answered by AI