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Drug overview for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate):
Generic name: SODIUM BENZOATE/SODIUM PHENYLACETATE
Drug class: Urea Cycle Disorder Agents
Therapeutic class: Metabolic Modifiers
Sodium phenylacetate and sodium benzoate is a fixed combination of 2 ammonia detoxicants.
No enhanced Uses information available for this drug.
Generic name: SODIUM BENZOATE/SODIUM PHENYLACETATE
Drug class: Urea Cycle Disorder Agents
Therapeutic class: Metabolic Modifiers
Sodium phenylacetate and sodium benzoate is a fixed combination of 2 ammonia detoxicants.
No enhanced Uses information available for this drug.
DRUG IMAGES
SOD PHENYLACET-SOD BENZOATE VL
The following indications for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate) have been approved by the FDA:
Indications:
Arginase deficiency
Argininosuccinate lyase deficiency
Carbamoyl phosphate synthetase deficiency
Citrullinemia
Hyperammonemia
Isovaleric acidemia
Ornithine carbamoyltransferase deficiency
Professional Synonyms:
Abnormally elevated blood ammonia
Ammonemia
Argininosuccinate synthetase deficiency
Argininosuccinic acid synthetase deficiency
Argininosuccinic aciduria
Arginosuccinic aciduria
ASAL deficiency
ASL deficiency
CAPS deficiency
Carbamoyl-phosphate synthetase deficiency
CPS deficiency
Hyperammonemia due to ornithine transcarbamylase deficiency
Hyperammonemic syndrome
Hyperargininemia
Isovaleryl CoA dehydrogenase deficiency
Isovaleryl-coenzyme A dehydrogenase deficiency
Ornithine transcarbamoylase deficiency
Ornithine transcarbamylase deficiency
Indications:
Arginase deficiency
Argininosuccinate lyase deficiency
Carbamoyl phosphate synthetase deficiency
Citrullinemia
Hyperammonemia
Isovaleric acidemia
Ornithine carbamoyltransferase deficiency
Professional Synonyms:
Abnormally elevated blood ammonia
Ammonemia
Argininosuccinate synthetase deficiency
Argininosuccinic acid synthetase deficiency
Argininosuccinic aciduria
Arginosuccinic aciduria
ASAL deficiency
ASL deficiency
CAPS deficiency
Carbamoyl-phosphate synthetase deficiency
CPS deficiency
Hyperammonemia due to ornithine transcarbamylase deficiency
Hyperammonemic syndrome
Hyperargininemia
Isovaleryl CoA dehydrogenase deficiency
Isovaleryl-coenzyme A dehydrogenase deficiency
Ornithine transcarbamoylase deficiency
Ornithine transcarbamylase deficiency
The following dosing information is available for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate):
The manufacturer states that because plasma concentrations of phenylacetate are prolonged, loading doses of sodium phenylacetate and sodium benzoate should not be repeated. However, some experts state that a repeat loading dose within 24 hours should be considered only in neonates with severe disorders and/or those receiving dialysis, and that loading doses should be spaced at least 6 hours apart.
The manufacturer has notified health-care professionals that particulate matter was detected in sodium phenylacetate and sodium benzoate injections. The manufacturer states that because of the possibility that this particulate matter may affect the safe use of the injection and to ensure optimal patient care, health-care professionals are instructed to use a Millex(R) Durapore GV 33 mm sterile syringe filter (0.22 mcm) when diluting sodium phenylacetate and sodium benzoate injection concentrate with dextrose 10% injection. (See Dilution under Dosage and Administration: Administration.) The manufacturer states that since this particulate matter may not be readily observed on visual inspection, a filter (i.e., Durapore GV 33 mm sterile syringe filter) must be used always regardless of whether particulate matter is seen in the vial.
It has been confirmed that this particulate matter is removed when the aforementioned filter is used. As a precautionary measure, the manufacturer will package Millex(R) Durapore GV 33 mm sterile syringe filters with all shipments of sodium phenylacetate and sodium benzoate injection. For further information, see Detection of Particulate Matter in Commercial Preparations under Warnings/Precautions: Warnings, in Cautions.
Commercially available sodium phenylacetate and sodium benzoate injection must be diluted prior to IV infusion. (See Dilution under Dosage and Administration: Admistration.) The drug is infused IV through a central venous line because administration through a peripheral line may cause burns. The drug should be administered only by the IV route.
Because administration of sodium phenylacetate and sodium benzoate has been associated with nausea and vomiting, an antiemetic may be administered during infusion. Sodium phenylacetate and sodium benzoate therapy should be initiated as soon as the diagnosis of hyperammonemia is made. Analogous oral agents (e.g., sodium phenylbutyrate) should be discontinued before initiation of the sodium phenylacetate and sodium benzoate infusion.
Oral therapy (e.g., with sodium phenylbutyrate), dietary management, and protein restriction should be started or resumed when ammonia concentrations have been reduced to the normal range.
It has been confirmed that this particulate matter is removed when the aforementioned filter is used. As a precautionary measure, the manufacturer will package Millex(R) Durapore GV 33 mm sterile syringe filters with all shipments of sodium phenylacetate and sodium benzoate injection. For further information, see Detection of Particulate Matter in Commercial Preparations under Warnings/Precautions: Warnings, in Cautions.
Commercially available sodium phenylacetate and sodium benzoate injection must be diluted prior to IV infusion. (See Dilution under Dosage and Administration: Admistration.) The drug is infused IV through a central venous line because administration through a peripheral line may cause burns. The drug should be administered only by the IV route.
Because administration of sodium phenylacetate and sodium benzoate has been associated with nausea and vomiting, an antiemetic may be administered during infusion. Sodium phenylacetate and sodium benzoate therapy should be initiated as soon as the diagnosis of hyperammonemia is made. Analogous oral agents (e.g., sodium phenylbutyrate) should be discontinued before initiation of the sodium phenylacetate and sodium benzoate infusion.
Oral therapy (e.g., with sodium phenylbutyrate), dietary management, and protein restriction should be started or resumed when ammonia concentrations have been reduced to the normal range.
No dosing information available.
No generic dosing information available.
The following drug interaction information is available for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate):
There are 0 contraindications.
There are 0 severe interactions.
There are 0 moderate interactions.
The following contraindication information is available for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate):
Drug contraindication overview.
Known hypersensitivity to sodium phenylacetate or sodium benzoate.
Known hypersensitivity to sodium phenylacetate or sodium benzoate.
There are 1 contraindications.
Absolute contraindication.
| Contraindication List |
|---|
| Severe renal impairment |
There are 3 severe contraindications.
Adequate patient monitoring is recommended for safer drug use.
| Severe List |
|---|
| Chronic heart failure |
| Edema |
| Metabolic acidosis |
There are 5 moderate contraindications.
Clinically significant contraindication, where the condition can be managed or treated before the drug may be given safely.
| Moderate List |
|---|
| Disease of liver |
| Hyperglycemia |
| Hypokalemia |
| Kidney disease with reduction in glomerular filtration rate (GFr) |
| Seizure disorder |
The following adverse reaction information is available for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate):
Adverse reaction overview.
Adverse effects occurring in 6% or more of patients receiving sodium phenylacetate and sodium benzoate include vomiting, hyperglycemia, hypokalemia, seizures, and mental impairment.
Adverse effects occurring in 6% or more of patients receiving sodium phenylacetate and sodium benzoate include vomiting, hyperglycemia, hypokalemia, seizures, and mental impairment.
There are 18 severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Hypokalemia Seizure disorder |
Anemia Disseminated intravascular coagulation Metabolic acidosis |
| Rare/Very Rare |
|---|
|
Bradycardia Cardiomyopathy Cholestasis Drug-induced hepatitis Drug-induced psychosis Extravasation injury Hemorrhage Hypertension Hypotension Pancytopenia Pericardial effusion Renal failure Thrombocytopenic disorder |
There are 18 less severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Altered mental status Hyperglycemia Infection Vomiting |
Diarrhea Fever Injection site sequelae Nausea |
| Rare/Very Rare |
|---|
|
Alopecia Ataxia Blistering skin Chest pain Flushing Pruritus of skin Skin rash Thrombophlebitis Urinary retention Urticaria |
The following precautions are available for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate):
Efficacy for treatment of hyperammonemia has been established in pediatric patients 0-16 years of age, including neonates 0-30 days of age and infants 31 days-2 years of age. Efficacy was based on analysis of data from a series of hospitalizations in which patients with hyperammonemia or a potential urea cycle disorder were treated with sodium phenylacetate and sodium benzoate, given in conjunction with arginine. (See Uses: Acute Hyperammonemia.)
Contraindicated
Severe Precaution
Management or Monitoring Precaution
Contraindicated
| None |
Severe Precaution
| None |
Management or Monitoring Precaution
| None |
Category C. (See Users Guide)
Not known whether sodium phenylacetate or sodium benzoate or their conjugated metabolites are distributed into milk. Caution is advised if the drug is administered in nursing women.
No enhanced Geriatric Use information available for this drug.
The following prioritized warning is available for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate):
No warning message for this drug.
No warning message for this drug.
The following icd codes are available for SODIUM PHENYLACET-SOD BENZOATE (sodium benzoate/sodium phenylacetate)'s list of indications:
| Arginase deficiency | |
| E72.21 | Argininemia |
| Argininosuccinate lyase deficiency | |
| E72.22 | Arginosuccinic aciduria |
| Carbamoyl phosphate synthetase deficiency | |
| E72.29 | Other disorders of urea cycle metabolism |
| Citrullinemia | |
| E72.23 | Citrullinemia |
| Hyperammonemia | |
| B15.0 | Hepatitis A with hepatic coma |
| B16.0 | Acute hepatitis B with delta-agent with hepatic coma |
| B16.2 | Acute hepatitis B without delta-agent with hepatic coma |
| B17.11 | Acute hepatitis C with hepatic coma |
| B19.0 | Unspecified viral hepatitis with hepatic coma |
| B19.11 | Unspecified viral hepatitis B with hepatic coma |
| B19.21 | Unspecified viral hepatitis C with hepatic coma |
| D81.810 | Biotinidase deficiency |
| E71.110 | Isovaleric acidemia |
| E71.121 | Propionic acidemia |
| E72.20 | Disorder of urea cycle metabolism, unspecified |
| E72.21 | Argininemia |
| E72.22 | Arginosuccinic aciduria |
| E72.23 | Citrullinemia |
| E72.29 | Other disorders of urea cycle metabolism |
| E72.4 | Disorders of ornithine metabolism |
| K72.91 | Hepatic failure, unspecified with coma |
| P74.6 | Transitory hyperammonemia of newborn |
| Isovaleric acidemia | |
| E71.110 | Isovaleric acidemia |
| Ornithine carbamoyltransferase deficiency | |
| E72.4 | Disorders of ornithine metabolism |
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