Buphenyl

Late Onset Disorders, Enzymes, neonatal-onset deficiency + 7 more
Treatment
19 Active Studies for Buphenyl

What is Buphenyl

Phenylbutyric acidThe Generic name of this drug
Treatment SummaryPhenylbutyric acid is a type of acid that acts as an inhibitor of histone deacetylase. It is used to treat cancer and can also reduce cell proliferation, invasion, and migration as well as induce apoptosis in glioma cells. Additionally, it can reduce protein isoprenylation, lower plasma glutamine levels, increase production of fetal hemoglobin, and affect hPPARγ activation.
Buphenylis the brand name
image of different drug pills on a surface
Buphenyl Overview & Background
Brand Name
Generic Name
First FDA Approval
How many FDA approvals?
Buphenyl
Phenylbutyric acid
1996
20

Effectiveness

How Buphenyl Affects PatientsPhenylbutyric acid helps to reduce high levels of ammonia and glutamine in the blood of people with urea cycle disorders. It can also reduce inflammation in the digestive tract, regulate fluid movement between cells, and improve antioxidant levels. Additionally, studies suggest that phenylbutyric acid can stop or slow the growth of cancer cells. The way it works is by acting like a sponge to take up ammonia, helping proteins fold correctly, and by blocking certain enzymes from working.
How Buphenyl works in the bodySodium phenylbutyrate is a drug that gets converted into phenylacetate when it enters the body. Phenylacetate is then combined with other molecules to create phenylacetylglutamine, which is eliminated from the body through the kidneys. This helps remove waste nitrogen from the body, in a similar way to the urea cycle. Each molecule of phenylacetylglutamine contains two moles of nitrogen.

When to interrupt dosage

The advocated dose of Buphenyl is contingent upon the diagnosed disorder, including Ornithine Transcarbamylase Deficiency, Citrullinemia and Enzymes. The amount of dosage is contingent upon the method of administration (e.g. Powder, for suspension - Oral or Oral) featured in the table underneath.
Condition
Dosage
Administration
Protein Restriction
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
Amyotrophic Lateral Sclerosis
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
Carbamyl Phosphate
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
Enzymes
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
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

Buphenyl Contraindications
Condition
Risk Level
Notes
Severe Hypersensitivity Reactions
Do Not Combine
Phenylbutyric Acid may interact with Pulse Frequency
There are 20 known major drug interactions with Buphenyl.
Common Buphenyl 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.
Buphenyl Toxicity & Overdose RiskTaking too much sodium phenylbutyrate may be lethal in animals, but is unlikely for humans. High levels of its active metabolite, phenylacetate, have been known to cause nausea, headaches, fatigue, weakness, confusion, and disorientation. If an overdose occurs, medical attention should be sought and dialysis may be helpful. Animal studies have also shown that phenylacetate has the potential to be toxic to embryos and fetuses.
image of a doctor in a lab doing drug, clinical research

Buphenyl Novel Uses: Which Conditions Have a Clinical Trial Featuring Buphenyl?

Two active trials are assessing the potential of Buphenyl in mitigating Citrullinemia, Ornithine Transcarbamylase Deficiency and Carbamyl Phosphate deficiency.
Condition
Clinical Trials
Trial Phases
Protein Restriction
0 Actively Recruiting
neonatal-onset deficiency
0 Actively Recruiting
Citrullinemia
0 Actively Recruiting
Carbamyl Phosphate
0 Actively Recruiting
Ornithine Transcarbamylase Deficiency
0 Actively Recruiting
Hyperammonemia
0 Actively Recruiting
Disorder of the Urea Cycle
0 Actively Recruiting
Amyotrophic Lateral Sclerosis
14 Actively Recruiting
Phase 1, Phase 2, Not Applicable
Enzymes
0 Actively Recruiting
Late Onset Disorders
5 Actively Recruiting
Not Applicable, Phase 1, Phase 2

Buphenyl Reviews: What are patients saying about Buphenyl?

4.7Patient Review
3/26/2012
Buphenyl for High Amount of Ammonium in the Blood
I found that Buphenyl worked just as well as Lactulose, but without any of the negative side effects.
image of drug pills surrounding a glass of water symbolizing drug consumption

Patient Q&A Section about buphenyl

These questions and answers are submitted by anonymous patients, and have not been verified by our internal team.

Is Buphenyl generic?

"– Par launched an AB-rated generic version of Horizon's Buphenyl (sodium phenylbutyrate) 500 mg tablets."

Answered by AI

How much does Buphenyl cost?

"Buphenyl is a prescription medicine that is used to manage high levels of ammonia in the blood. Sodium phenylbutyrate is the generic version of Buphenyl. The average price of Buphenyl is around $14,236 for a supply of 250 g. The Buphenyl is taken orally in powder form and must be reconstituted before use."

Answered by AI

What is Buphenyl used for?

"This medication is used for long-term treatment of an inherited disorder called urea cycle disorder. The medication helps remove ammonia from the body. Too much ammonia in the body can cause brain damage and sometimes death."

Answered by AI

Who makes Buphenyl?

"Orphan drugs are a type of medication used to treat rare medical conditions.

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."

Answered by AI

Clinical Trials for Buphenyl

Have you considered Buphenyl clinical trials? We made a collection of clinical trials featuring Buphenyl, we think they might fit your search criteria.
Have you considered Buphenyl clinical trials? We made a collection of clinical trials featuring Buphenyl, we think they might fit your search criteria.
Have you considered Buphenyl clinical trials? We made a collection of clinical trials featuring Buphenyl, we think they might fit your search criteria.