Reye Syndrome Medication

  • Author: Debra L Weiner, MD, PhD; Chief Editor: Richard G Bachur, MD   more...
 
Updated: Feb 1, 2011
 

Medication Summary

No specific treatment is available. Supportive care to reduce hyperammonemia with sodium phenylacetate/sodium benzoate may be required. For highly elevated levels of ammonia, 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 phenylacetate/sodium benzoate. Continuing the administration of sodium phenylacetate/sodium benzoate with hemodialysis may be considered.

In terms of managing increased ICP, steroids are of no proven benefit, they may be harmful, and they are not indicated.

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Ammonia detoxicants

Class Summary

Treatment of hyperammonemia; enhances elimination of nitrogen. Sodium phenylacetate/sodium benzoate is FDA approved for treatment of hyperammonemia due to urea-cycle defects and is available only from a specialty wholesaler, Ucyclyd Pharma, Inc (in the United States and Canada, 24-hour toll-free number: 888-829-2593; 8125 N. Hayden Road, Scottsdale, AZ 85258). For more information, see Ammonul prescribing information.

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Sodium phenylacetate and sodium benzoate (Ammonul)

 

May be effective to treat hyperammonemia. For levels >500-600 mcg/dL, hemodialysis preferred. Can be used until dialysis started or with dialysis. Benzoate combines with glycine to form hippurate (excreted in urine). One mole of benzoate removes 1 mole of nitrogen. Phenylacetate conjugates (by acetylation) with glutamine in liver and kidneys to form phenylacetylglutamine (excreted by kidneys). Nitrogen content of phenylacetylglutamine per mole identical to that of urea (2 mol). Preparation contains 100 mg/mL each of sodium phenylacetate and sodium benzoate; supplied as 50-mL vials. Must dilute IV dose in at least 25 mL/kg of dextrose 10% up to 600 mL. Do not directly mix with other medications; may be piggybacked. Give in addition to daily fluid requirement.

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Antiemetic agents

Class Summary

Administer ondansetron to decrease vomiting and during initiation of sodium phenylacetate and sodium benzoate.

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Ondansetron (Zofran)

 

Controls nausea and vomiting associated with Reye syndrome and with IV administration of sodium benzoate and phenylacetate.

Selective 5-HT3-receptor antagonist that blocks serotonin both peripherally and centrally. Prevents nausea and vomiting associated with IV administration of sodium benzoate and phenylacetate.

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Contributor Information and Disclosures
Author

Debra L Weiner, MD, PhD  Attending Physician, Division of Emergency Medicine, Children's Hospital, Boston; Assistant Professor, Department of Pediatrics, Harvard Medical School

Disclosure: Nothing to disclose.

Specialty Editor Board

Garry Wilkes, MBBS, FACEM  Director of Emergency Medicine, Bunbury Hospital; Medical Consultant, St John Ambulance, WA Ambulance Service; Adjunct Associate Professor, Edith Cowan University; Clinical Associate Professor, Rural Clinical School, University of Western Australia

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Grace M Young, MD  Associate Professor, Department of Pediatrics, University of Maryland Medical Center

Grace M Young, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Emergency Physicians

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Richard G Bachur, MD  Associate Professor of Pediatrics, Harvard Medical School; Associate Chief and Fellowship Director, Attending Physician, Division of Emergency Medicine, Children's Hospital of Boston

Richard G Bachur, MD is a member of the following medical societies: American Academy of Pediatrics, Society for Academic Emergency Medicine, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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