buphenyl
Buphenyl (Sodium Phenylbutyrate) is a nitrogen binding agent used to treat Sickle Cell Anemia, Cystic Fibrosis, Myeloproliferative Disorders, Beta-Thalassemia.
Sodium Phenylbutyrate · by Horizon Therap US
Key facts
- Active ingredient
- Sodium Phenylbutyrate
- Drug class
- Nitrogen Binding Agent
- Form
- Tablet, Powder
- Strength
- Sodium Phenylbutyrate 500MG
- Type
- Prescription (Rx)
- Brand or generic
- Brand-name
- Manufacturer
- Horizon Therap US
- FDA application
- NDA020572
- Availability
- Discontinued (per FDA)
What is Buphenyl?
From the FDA label:BUPHENYL (sodium phenylbutyrate) Tablets for oral administration and BUPHENYL (sodium phenylbutyrate) Powder for oral, nasogastric, or gastrostomy tube administration contain sodium phenylbutyrate. Sodium phenylbutyrate is an off-white crystalline substance which is soluble in water and has a strong salty taste. Sodium phenylbutyrate also is freely soluble in methanol and practically insoluble in acetone and diethyl ether. It is known chemically as 4-phenylbutyric acid, sodium salt with a molecular weight of 186 and the molecular formula C 10 H 11 O 2 Na. Chemical Structure: Each tablet of BUPHENYL contains 500 mg of sodium phenylbutyrate and the inactive ingredients microcrystalline cellulose NF, magnesium stearate NF, and colloidal silicon dioxide NF. Each gram of BUPHENYL Powder contains 0.94 grams of sodium phenylbutyrate and the inactive ingredients calcium stearate NF, and colloidal silicon dioxide NF. Chemical Structure
How to use
Buphenyl is sold in more than one form (Tablet and Powder), and each is dosed differently. The instructions below come from the FDA label for application NDA020572 — follow the label that came with the product you were actually prescribed.
For oral use only. The use of BUPHENYL Tablets is indicated for children weighing more than 20 kg and for adults. The usual total daily dose of BUPHENYL Tablets and Powder for patients with urea cycle disorders is 450–600 mg/kg/day in patients weighing less than 20 kg, or 9.9–13.0 g/m2/day in larger patients. The tablets and powder are to be taken in equally divided amounts with each meal or feeding (i.e., three to six times per day). BUPHENYL Powder is indicated for oral use (via mouth, gastrostomy, or nasogastric tube) only. The powder is to be mixed with food (solid or liquid) for immediate use; however, when dissolved in water, BUPHENYL Powder has been shown to be stable for up to one week at room temperature or refrigerated. Sodium phenylbutyrate is very soluble in water (5 grams per 10 mL). When BUPHENYL Powder is added to a liquid, only sodium phenylbutyrate will dissolve, the excipients will not. The effect of food on sodium phenylbutyrate has not been determined. Each level teaspoon (enclosed) dispenses 3.2 grams of powder and 3.0 grams of sodium phenylbutyrate. Each level tablespoon (enclosed) dispenses 9.1 grams of powder and 8.6 grams of sodium phenylbutyrate. Shake lightly before use. The safety or efficacy of doses in excess of 20 grams (40 tablets) per day has not been established. NUTRITIONAL MANAGEMENT To promote growth and development, plasma levels of…
Side effects
The assessment of clinical adverse events came from 206 patients treated with sodium phenylbutyrate. Adverse events (both clinical and laboratory) were not collected systematically in these patients, but were obtained from patient visit reports by the 65 co-investigators. Causality of adverse effects is sometimes difficult to determine in this patient population because they may result from either the underlying disease, the patient's restricted diet, intercurrent illness, or BUPHENYL. Furthermore, the rates may be underestimated because they were reported primarily by parent or guardian and not the patient. Clinical Adverse Events In female patients, the most common clinical adverse event reported was amenorrhea/menstrual dysfunction (irregular menstrual cycles), which occurred in 23% of the menstruating patients. Decreased appetite occurred in 4% of all patients. Body odor (probably caused by the metabolite, phenylacetate) and bad taste or taste aversion were each reported in 3% of patients. Other adverse events reported in 2% or fewer patients were: Gastrointestinal : abdominal pain, gastritis, nausea and vomiting; constipation, rectal bleeding, peptic ulcer disease, and pancreatitis each occurred in one patient. Hematologic : aplastic anemia and ecchymoses each occurred in one patient. Cardiovascular : arrhythmia and edema each occurred in one patient. Renal : renal…
Warnings
Important safety information
Each BUPHENYL Tablet contains 62 mg of sodium (9.2% w/w) (corresponding to 124 mg of sodium per gram of sodium phenylbutyrate [12.4% w/w]) and BUPHENYL Powder contains 11.7 grams of sodium per 100 grams of powder, corresponding to 125 mg of sodium per gram of sodium phenylbutyrate (12.4% w/w). BUPHENYL should be used with great care, if at all, in patients with congestive heart failure or severe renal insufficiency, and in clinical states in which there is sodium retention with edema. Because BUPHENYL is metabolized in the liver and kidney, and phenylacetylglutamine is primarily excreted by the kidney, use caution when administering the drug to patients with hepatic or renal insufficiency or inborn errors of beta oxidation. Probenecid is known to inhibit the renal transport of many organic compounds, including hippuric acid, and may affect renal excretion of the conjugated product of BUPHENYL as well as its metabolite. Use of corticosteroids may cause the breakdown of body protein and increase plasma ammonia levels.
Who should not take Buphenyl
BUPHENYL should not be used to manage acute hyperammonemia, which is a medical emergency.
Overdose — what happens if you take too much
Overdoses of BUPHENYL exceeding ten-fold the maximum recommended dosage may produce emesis, CNS depression, metabolic acidosis with or without respiratory alkalosis, hypernatremia, hypokalemia, and hypophosphatemia. Symptoms of overdose overlap with those of acute hyperammonemia. If overdose occurs, discontinue BUPHENYL, monitor plasma phenylacetate and ammonia levels closely, and institute appropriate emergency management, which may include hemodialysis, continuous veno-venous hemofiltration (CVVH), or extracorporeal membrane oxygenation (ECMO).
U.S. Poison Control: call or text 1-800-222-1222 (free, 24/7). In an emergency call 911. From the FDA label; not medical advice.
Drug class
May treat (source: NIH RxClass)
See how Buphenyl ranks — best-rated nitrogen binding agent for:
Dosage forms
Tablet and Powder
The forms currently marketed in the U.S., per the FDA National Drug Code Directory.
Ways to save
Ask for the generic
Same active ingredient, far cheaper. Is there a generic? →
Request a 90-day supply
Bulk fills usually lower the per-dose price vs monthly refills.
Use copay cards
Manufacturer copay cards & patient-assistance programs — especially for brand drugs.
Compare alternatives
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Frequently asked questions
- What does Buphenyl treat?
- Buphenyl (Sodium Phenylbutyrate) may be used to treat sickle cell anemia, cystic fibrosis, myeloproliferative disorders, beta-thalassemia, based on NIH RxClass drug-classification data (conditions a drug may treat — not a verbatim copy of the FDA-approved label). This is general reference, not medical advice.
- Is there a coupon or discount for Buphenyl?
- pharmaranks is an independent ratings site, not a pharmacy — we don't issue coupons or sell Buphenyl. To pay less, ask your prescriber or pharmacist whether a generic equivalent exists, check the manufacturer's copay/savings card and patient-assistance program, and compare a pharmacy discount card against your insurance copay. Prices vary by pharmacy, insurance, and location, so always confirm at the counter. This is general reference, not medical or financial advice.
- Who makes Buphenyl?
- Buphenyl is marketed by Horizon Therap US. You can see Horizon Therap US's full profile, rating, and other products on pharmaranks.
- Is Buphenyl a brand-name or generic drug?
- Buphenyl is a brand-name product with the active ingredient Sodium Phenylbutyrate.
- Is Buphenyl available over the counter?
- No. Buphenyl is a prescription (Rx) medication in the US — you need a prescription from a licensed clinician (in person or via telehealth); it isn't sold over the counter. For some conditions there are OTC alternatives — ask your pharmacist.
- What forms does Buphenyl come in?
- Buphenyl is currently marketed as tablet and powder, per the FDA's National Drug Code Directory.
- What class of drug is Buphenyl?
- Buphenyl is classified as nitrogen binding agent, per the FDA's Established Pharmacologic Class.
- Is Buphenyl FDA-registered?
- Buphenyl is on record with the U.S. FDA under application number NDA020572. You can verify this on its official FDA label.
- Is Buphenyl safe?
- There's no single safe-or-not verdict, and we don't yet have a composite recall-safety score for Buphenyl. Review its FDA-label side effects and warnings below, and always consult a licensed professional.
- What are the side effects of Buphenyl?
- Buphenyl's side effects are taken directly from its FDA label. From the label: The assessment of clinical adverse events came from 206 patients treated with sodium phenylbutyrate. Adverse events (both clinical and laboratory) were not collected systematically in these patients, but were obtained from patient visit reports by the 65 co-investigators.… Both common and serious reactions are documented in full on this page. This is general reference from the FDA, not medical advice — always consult a professional.
Clinical content sourced from the FDA label via openFDA (U.S. FDA). View the FDA label on DailyMed → Provided for general reference only — not medical advice. Always consult a licensed professional and the current prescribing information.
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