Sodium phenylacetate/sodium benzoate

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Sodium phenylacetate/sodium benzoate is a combination medication used in the treatment of urea cycle disorders, specifically for acute hyperammonemia and associated encephalopathy. It is a mixture of two ammonia scavenging drugs, sodium phenylacetate and sodium benzoate.

Mechanism of Action[edit | edit source]

Sodium phenylacetate and sodium benzoate work by a process known as alternative pathway therapy. They provide an alternative pathway for the removal of ammonia from the body. Sodium phenylacetate conjugates with glutamine in the kidneys to form phenylacetylglutamine, which is excreted in the urine. Sodium benzoate combines with glycine in the liver to form hippurate, which is also excreted in the urine.

Indications[edit | edit source]

This medication is indicated for the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle.

Side Effects[edit | edit source]

Common side effects include nausea, vomiting, headache, and fatigue. Serious side effects may include allergic reactions, seizures, and liver damage.

Contraindications[edit | edit source]

Sodium phenylacetate/sodium benzoate is contraindicated in patients with known hypersensitivity to either of the active ingredients.

Drug Interactions[edit | edit source]

This medication may interact with other drugs that affect the kidneys or liver, including certain antibiotics and antiviral medications.

Dosage and Administration[edit | edit source]

The dosage of sodium phenylacetate/sodium benzoate is based on the patient's weight and the severity of the condition. It is administered intravenously.

See Also[edit | edit source]

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Contributors: Prab R. Tumpati, MD