Which medications in the drug class Hyperammonemia Treatment Agents are used in the treatment of Reye Syndrome?

Updated: Apr 02, 2018
  • Author: Debra L Weiner, MD, PhD; Chief Editor: Kirsten A Bechtel, MD  more...
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Answer

Hyperammonemia Treatment Agents

Ammonia detoxicants are used for treatment of hyperammonemia; they enhance elimination of nitrogen. Sodium benzoate/sodium phenylacetate (Ammonul) is approved by the US Food and Drug Administration (FDA) for treatment of hyperammonemia caused by urea-cycle defects. Bioequivalent generics are available in the U.S.

Sodium benzoate/sodium phenylacetate (Ammonul)

Sodium benzoate/sodium phenylacetate may be effective for treatment of hyperammonemia, though hemodialysis is preferred for ammonia levels higher than 500-600 µg/dL. It can be used until dialysis is started or along with dialysis.

Benzoate combines with glycine to form hippurate (which is excreted in urine); 1 mole of benzoate removes 1 mole of nitrogen. Phenylacetate conjugates (by acetylation) with glutamine in the liver and kidneys to form phenylacetylglutamine (which is excreted by the kidneys). The nitrogen content of phenylacetylglutamine per mole is identical to that of urea (2 mol).

The preparation contains 100 mg/mL each of sodium phenylacetate and sodium benzoate and is supplied as 50-mL vials. The intravenous (IV) dose must be diluted in at least 25 mL/kg of 10% dextrose, up to 600 mL. Sodium benzoate/sodium phenylacetate should not be directly mixed with other medications but may be piggybacked. It should be given in addition to the daily fluid requirement.


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