Sodium Phenylbutyrate
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Questions & Answers
Side Effects & Adverse Reactions
Sodium Phenylbutyrate 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). Sodium Phenylbutyrate Powder 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 Sodium Phenylbutyrate Powder 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 Sodium Phenylbutyrate Powder as well as its metabolite.
Use of corticosteroids may cause the breakdown of body protein and increase plasma ammonia levels.
Legal Issues
There is currently no legal information available for this drug.
FDA Safety Alerts
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Manufacturer Warnings
There is currently no manufacturer warning information available for this drug.
FDA Labeling Changes
There are currently no FDA labeling changes available for this drug.
Uses
Sodium Phenylbutyrate Powder is indicated as adjunctive therapy in the chronic management of patients with urea cycle disorders involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS). It is indicated in all patients with neonatal-onset deficiency (complete enzymatic deficiency, presenting within the first 28 days of life). It is also indicated in patients with late-onset disease (partial enzymatic deficiency, presenting after the first month of life) who have a history of hyperammonemic encephalopathy. It is important that the diagnosis be made early and treatment initiated immediately to improve survival. Any episode of acute hyperammonemia should be treated as a life-threatening emergency.
Sodium Phenylbutyrate Powder must be combined with dietary protein restriction and, in some cases, essential amino acid supplementation. (See Nutritional Supplementation subsection of the DOSAGE AND ADMINISTRATION section.)
Previously, neonatal-onset disease was almost universally fatal within the first year of life, even when treated with peritoneal dialysis and essential amino acids or their nitrogen-free analogs. However, with hemodialysis, use of alternative waste nitrogen excretion pathways (sodium phenylbutyrate, sodium benzoate, and sodium phenylacetate), dietary protein restriction, and, in some cases, essential amino acid supplementation, the survival rate in newborns diagnosed after birth but within the first month of life is almost 80%. Most deaths have occurred during an episode of acute hyperammonemic encephalopathy. Patients with neonatal-onset disease have a high incidence of mental retardation. Those who had IQ tests administered had an incidence of mental retardation as follows: ornithine transcarbamylase deficiency, 100% (14/14 patients tested); argininosuccinic acid synthetase deficiency, 88% (15/17 patients tested); and carbamylphosphate synthetase deficiency, 57% (4/7 patients tested). Retardation was severe in the majority of the retarded patients.
In patients diagnosed during gestation and treated prior to any episode of hyperammonemic encephalopathy, survival is 100%, but even in these patients, most subsequently demonstrate cognitive impairment or other neurologic deficits.
In late-onset deficiency patients, including females heterozygous for ornithine transcarbamylase deficiency, who recover from hyperammonemic encephalopathy and are then treated chronically with sodium phenylbutyrate and dietary protein restriction, the survival rate is 98%. The two deaths in this group of patients occurred during episodes of hyperammonemic encephalopathy. However, compliance with the therapeutic regimen has not been adequately documented to allow evaluation of the potential for Sodium Phenylbutyrate Powder and dietary protein restriction to prevent mental deterioration and recurrence of hyperammonemic encephalopathy if carefully adhered to. The majority of these patients tested (30/46 or 65%) have IQ's in the average to low average/borderline mentally retarded range. Reversal of pre-existing neurologic impairment is not likely to occur with treatment and neurologic deterioration may continue in some patients.
Even on therapy, acute hyperammonemic encephalopathy recurred in the majority of patients for whom the drug is indicated.
Sodium Phenylbutyrate Powder may be required life-long unless orthotopic liver transplantation is elected.
(See CLINICAL PHARMACOLOGY, Pharmacodynamics subsection for the biochemical effects of Sodium Phenylbutyrate Powder).
History
There is currently no drug history available for this drug.
Other Information
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 C10H11O2Na.
Chemical Structure:
Each gram of Sodium Phenylbutyrate Powder contains 0.94 grams of sodium phenylbutyrate and the inactive ingredients calcium stearate NF, and colloidal silicon dioxide NF.
Sources
Sodium Phenylbutyrate Manufacturers
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Sigmapharm Laboratories, Llc
![Sodium Phenylbutyrate Powder [Sigmapharm Laboratories, Llc]](/wp-content/themes/bootstrap/assets/img/loading2.gif)
Sodium Phenylbutyrate | Sigmapharm Laboratories, Llc
For oral use only.
The use of Sodium Phenylbutyrate Tablets is indicated for children weighing more than 20 kg and for adults.
The usual total daily dose of Sodium Phenylbutyrate Powder for patients with urea cycle disorders is 450 – 600 mg/kg/day in patients weighing less than 20 kg, or 9.9 – 13 g/m2/day in larger patients. The powder is to be taken in equally divided amounts with each meal or feeding (i.e., three to six times per day).
Sodium Phenylbutyrate 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, Sodium Phenylbutyrate 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 Sodium Phenylbutyrate 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 yellow teaspoon (enclosed) dispenses 3.2 grams of powder and 3 grams of sodium phenylbutyrate. Each level blue 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 per day has not been established. -
Mikart, Inc.
Sodium Phenylbutyrate | Mikart, Inc.
For oral use only.
The use of Sodium Phenylbutyrate Tablets is indicated for children weighing more than 20 kg and for adults.
The usual total daily dose of Sodium Phenylbutyrate Tablets for patients with urea cycle disorders is 450 to 600 mg/kg/day in patients weighing less than 20 kg, or 9.9 to 13.0 g/m2/day in larger patients. The tablets are to be taken in equally divided amounts with each meal or feeding (i.e., three to six times per day). The effect of food on sodium phenylbutyrate has not been determined.
The safety or efficacy of doses in excess of 20 grams (40 tablets) per day has not been established.
![Sodium Phenylbutyrate Tablet [Mikart, Inc.]](http://dailymed.nlm.nih.gov/dailymed/image.cfm?setid=cab4b768-da1f-4f27-93a0-4d2e40fd0a1b&name=cab4b768-da1f-4f27-93a0-4d2e40fd0a1b-02.jpg)
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