Reye Syndrome Medication

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

Medication Summary

No specific treatment is available for Reye syndrome. Supportive care should be provided to treat hyperammonemia, hypoglycemia, acidosis, electrolyte disturbances, nausea, vomiting, seizures and increased intracranial pressure.

Corticosteroids are of no proven benefit with regard to managing increased intracranial pressure (ICP) and may even be harmful. Accordingly, they are not indicated in this setting.

The mainstay of treatment is supportive care is based on the clinical stage of the syndrome. Provide aggressive treatment to correct or prevent metabolic abnormalities, particularly hypoglycemia and hyperammonemia, and to prevent or control cerebral edema.

Hyperammonemia treatment consists of sodium benzoate/sodium phenylacetate IV. For highly elevated ammonia levels, hemodialysis may be the appropriate initial treatment if it is readily available, and it is also recommended for patients whose condition fails to respond to initial course of sodium benzoate/sodium phenylacetate. Continuing the administration of sodium benzoate/sodium phenylacetate during hemodialysis may be considered.

Hypoglycemia treatment with dextrose 25% (D25) should be administered to treat hypoglycemia, and, as needed dextrose 10%) (D10) may be provided in IV maintenance fluids infused at 1-1.5 mL/min maintenance to provide 8-12 mg/kg/min. Serum glucose should be maintained in the 120-170 mg/dL range to avoid catabolism. Glucose can be modulated with an insulin dose of 0.2-0.3 units.

For life threatening increased intracranial pressure (ICP), mannitol or hypertonic (3%) saline should be administered. Hypertonic saline should not be given to patients with elevated sodium.

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Hyperammonemia Treatment Agents

Class Summary

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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Alkalinizing Agents

Class Summary

Bicarbonate treatment is controversial due to paradoxical CNS acidosis.

Sodium bicarbonate

Administration of sodium bicarbonate 0.25-2mEq/kg can be considered for acidosis with a pH <7.0-7.2

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Antiemetics

Class Summary

Off-label use of ondansetron may be considered to control nausea and vomiting associated with Reye syndrome and with IV administration of sodium benzoate/sodium phenylacetate.

Ondansetron (Zofran, Zuplenz)

Selective 5-HT3 receptor antagonist that blocks serotonin both peripherally and centrally.

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Anticonvulsants

Class Summary

Seizures may occur with increased ICP and ammonia levels.

Lorazepam (Ativan)

Widely used off-label in children to abort and/or prevent seizures and status epilepticus. Contains benzyl alcohol, so it is contraindicated in neonates and its association with gasping syndrome.

Fosphenytoin (Cerebyx)

Indicated for the treatment of generalized tonic-clonic seizures (eg, status epilepticus).

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