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Sodium Benzoate

Pronunciation

(SOW dee um BENZ oh ate)

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Powder: 454 g

Pharmacologic Category

  • Ammonium Detoxicant
  • Hyperammonemia Agent
  • Urea Cycle Disorder (UCD) Treatment Agent

Pharmacology

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

Excretion

Clearance is largely attributable to metabolism with urinary excretion of hippurate, the major metabolite

Half-Life Elimination

0.75 to 7.4 hours

Use: Labeled Indications

Urea cycle disorders: Adjunctive therapy for the prevention and treatment of hyperammonemia due to suspected or proven urea cycle disorders

Dosing: Adult

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: Pediatric

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 to 500 mg/kg/day in 3 to 4 divided doses given with meals (Häberle 2012; Kliegman 2016, Maestri 1991; Maestri 1996).

Administration

Oral: Administer with meals and abundant fluid (Häberle 2012). Not commercially available; must be compounded using chemical powder; consult metabolic specialist.

Drug Interactions

Probenecid: May increase the serum concentration of Sodium Benzoate. Specifically, probenecid may inhibit the renal transport of the hippuric acid metabolite of sodium benzoate. Monitor therapy

Adverse Reactions

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)

Warnings/Precautions

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).

Other warnings/precautions:

• Availability: Sodium benzoate is not commercially available as a ready-to-use product; however, some centers are still using compounded formulations when necessary.

Monitoring Parameters

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).

Pregnancy Considerations

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).

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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