Version May 2024
Note: Sodium benzoate is not commercially available as a ready-to-use product; however, some centers are still using compounded formulations when necessary. IV dosing is usually required for acute episodes; if IV administration is necessary, see the Sodium Phenylacetate and Sodium Benzoate monograph for information about a commercially available parenteral product.
Dosing information not available; use with caution.
Dosing information not available; use with caution.
(For additional information see "Sodium benzoate: Pediatric drug information")
Note: Sodium benzoate is not commercially available as a ready-to-use product; however, some centers are still using compounded formulations when necessary. IV dosing is usually required for acute episodes; if IV administration is necessary, see the Sodium Phenylacetate and Sodium Benzoate monograph for information about a commercially available parenteral product.
Urea cycle disorders, long-term therapy: Very limited data available: Dosage should be individualized based on patient response; consult metabolic specialist:
Infants, Children, and Adolescents: Oral: ≤250 mg/kg/day in 3 to 4 divided doses administered with meals as part of an appropriate combination therapy; maximum daily dose: 12 g/day (Häberle 2019; Maestri 1991; Maestri 1996). Note: Higher doses may be required in some patients (Häberle 2019) in consultation with a metabolic specialist.
Dosing information not available; use with caution.
Dosing information not available; use with caution.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Frequency not defined. Adverse reactions are from multiple indications and dosing.
Cardiovascular: ECG abnormality (Sushma 1992)
Gastrointestinal: Anorexia (Wolff 1986), dyspepsia (Sushma 1992), epigastric distress (Sushma 1992), gastritis (Häberle 2012), mucositis (Häberle 2012), nausea (Sushma 1992), vomiting (Sushma 1992; Wolff 1986)
Endocrine & metabolic: Hypokalemia (Häberle 2012), increased serum sodium (Sushma 1992), metabolic acidosis (Häberle 2012)
Renal: Renal tubular disease (Wolff 1986)
Concerns related to adverse effects:
• Fluid overload: Use with caution, if at all, in patients at risk for fluid overload (eg, heart failure, severe renal impairment) or sodium retention; contains a significant amount of sodium (Häberle 2019; Misel 2013).
Disease-related concerns:
• Acidemia: Use with caution in patients with propionic or methylmalonic acidemia (Kliegman 2016).
• Reye syndrome: Use with caution in patients with Reye syndrome (Kliegman 2016).
Special populations:
• Neonates: Use with caution in neonates with hyperbilirubinemia due to potential displacement of bilirubin from albumin binding sites (Kliegman 2016).
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Powder: 454 g
Oral: Administer with meals and abundant fluid (Häberle 2012). Not commercially available; must be compounded using chemical powder; consult metabolic specialist.
Oral: Administer with meals/feeds and fluid to minimize gastritis/mucositis (Häberle 2019; Maines 2020). Not commercially available; must be compounded using chemical powder; consult metabolic specialist.
Urea cycle disorders: Adjunctive therapy for the prevention and treatment of hyperammonemia due to suspected or proven urea cycle disorders
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the Lexicomp drug interactions program by clicking on the “Launch drug interactions program” link above.
Corticosteroids (Systemic): May diminish the therapeutic effect of Sodium Benzoate. Risk C: Monitor therapy
Penicillins: May diminish the therapeutic effect of Sodium Benzoate. Risk C: Monitor therapy
Probenecid: May diminish the therapeutic effect of Sodium Benzoate. Risk C: Monitor therapy
Valproate Products: May diminish the therapeutic effect of Sodium Benzoate. Risk C: Monitor therapy
Benzoate can be detected in the umbilical cord and newborn serum following maternal administration of sodium benzoate prior to delivery (Das 2009).
Information related to the use of sodium benzoate for urea cycle disorders (UCD), including ornithine transcarbamylase (OTC) deficiency, during pregnancy is limited (Häberle 2012; Lamb 2013; Mendez-Figueroa 2010). Pregnancy is a high metabolic state that can trigger hyperamonemic episodes in females with known OTC deficiency as well as asymptomatic female carriers; close monitoring is recommended during pregnancy and for 5 days postpartum (Arn 1990; Häberle 2012; Mendez-Figueroa 2010). Maternal treatment with oral sodium benzoate is recommended if serum ammonia is 1.5 to 2 times normal (Mendez-Figueroa 2010). Administration of sodium benzoate to the mother to prevent adverse events in newborns prenatally diagnosed with a UCD has also been described (Das 2009). In general, females with inherited metabolic disease should achieve adequate metabolic control prior to conception (Häberle 2012; Langendonk 2012).
It is not known if sodium benzoate is present in breast milk.
In general, females with inherited metabolic disease should ensure adequate energy intake if breastfeeding (Häberle 2012; Langendonk 2012). Breastfed infants with urea cycle disorders require close protein monitoring (Häberle 2012).
Sodium benzoate contains ~81 mg (~3.5 mmol) of sodium per 500 mg of sodium benzoate (Häberle 2019; Misel 2013). Dietary protein restriction should be part of treatment of urea cycle disorders. Caloric supplementation (goal ≥80 kcal/kg/day) is recommended for patients in a catabolic state (NORD 2013).
Plasma ammonia, plasma amino acid (quantitative) and glutamine concentrations, blood glucose, serum electrolytes, hepatic and renal function tests, blood gases, neurologic status, physical signs/symptoms of hyperammonemia (ie, lethargy, ataxia, confusion, vomiting, seizures, and memory impairment) (Häberle 2012; Lichter-Konecki 2016).
Assists in lowering serum ammonia levels by activation of a nonurea cycle pathway (the benzoate-hippurate pathway); ammonia in the presence of benzoate will conjugate with glycine to form hippurate which is excreted by the kidney
Half-life elimination: 0.75 to 7.4 hours
Excretion: Clearance is largely attributable to metabolism with urinary excretion of hippurate, the major metabolite
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