Reye Syndrome

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It is essential to make the diagnosis as early as possible and start appropriate supportive measures immediately as progression to more severe stage is associated with higher mortality (7).
Must investigate for inborn errors of metabolism in any patient suspected to have RS as well as other etiology due to RS being a diagnosis of exclusion (1).

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TREATMENT

GENERAL MEASURES

Supportive care dictated by the severity of illness (5)[A]
IV glucose and frequent monitoring of serum glucose to treat hypoglycemia (7)[B].
Hyperventilation, mannitol, glycerol, and corticosteroids to control intracranial pressure (7)[B]
Vitamin K, fresh frozen plasma, and platelets as needed for treatment of coagulopathy (7)[B]
Mechanical ventilation as needed
Dialysis to remove toxins and toxic metabolites (7)[B]

MEDICATION

All treatment is supportive and may include the following (7)[B]:Glucose 10 " “15% IVVitamin KFor increased intracranial pressureMannitolDexamethasoneGlycerol
Contraindications: NSAIDs such as diclofenac and mefenamic acid may complicate clinical picture so should not be used (3)[A].
Precautions: Mannitol and poor renal output may result in vascular overload and pulmonary edema.
Resveratrol used in animals studies showed protection against the oxidative stress occurring in RS but has yet been researched in clinical practice (10).

SURGERY/OTHER PROCEDURES

Liver transplant (11)[C] ‚

INPATIENT CONSIDERATIONS

Admission Criteria/Initial Stabilization

Medical emergency requiring immediate hospitalization and frequent monitoring, often in an intensive care setting (3)[A] ‚

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Complete bed rest until recovery is noted and able to return to normal activity as tolerated. ‚

Patient Monitoring

Depends on specific residual effects. may require care of physicians; nurses; psychologists; and/or physical, occupational, and/or speech therapists (7)[B] ‚

DIET

NPO during the acute phase ‚

PATIENT EDUCATION

National Reye Syndrome Foundation, P.O. Box 829, Byron, OH 43506-0829 (800) 233-7393; www.reyessyndrome.org
National Institutes of Health: http://www.ninds.nih.gov/disorders/reyes_syndrome/reyes_syndrome.htm

PROGNOSIS

Overall prognosis is related to degree of cerebral edema and ammonia level on admission, correlated with severity of stage (7)[B].
Outcomes range from a mild illness without progression and complete resolution to a critical illness with significant lasting sequelae or death (7)[B].
Possible neurologic sequelae include brain damage and disability, problems with attention, concentration, speech, language, and fine and gross motor skills.
Sequelae are more common with higher stages.

COMPLICATIONS

Aspiration pneumonia
Respiratory failure
Cardiac dysrhythmia/arrest
Inappropriate vasopressin excretion
Diabetes insipidus
Cerebral edema
Seizures
Death

REFERENCES

11 Glasgow ‚ JF. Reye 's syndrome: the case for a causal link with aspirin. Drug Saf. 2006;29(12):1111 " “1121.22 Autret-Leca ‚ E, Jonville-Bera ‚ AP, Llau ‚ ME, et al. Incidence of Reye 's syndrome in France: a hospital-based survery. J Clin Epidemiol. 2001;54(8):857 " “862.33 Pugliese ‚ A, Beltramo ‚ T, Torre ‚ D. Reye 's and Reye 's-like syndromes. Cell Biochem Funct. 2008;26(7):741 " “746.44 Bennett ‚ CL, Starko ‚ KM, Thomsen ‚ HS, et al. Linking drugs to obscure illnesses: lessons from pure red cell aplasia, nephrogenic systemic fibrosis, and Reye 's syndrome. A report from the Southern Network on Adverse Reactions (SONAR). J Gen Intern Med. 2012;27(12):1697 " “1703.55 Degnan ‚ LA. Reye 's syndrome: a rare but serious pediatric condition. US Pharm. 2012;37(3):HS6 " “HS8.66 Gosalakkal ‚ JA, Kamoji ‚ V. Reye syndrome and Reye-like syndrome. Pediatr Neurol. 2008;39(3):198 " “200.77 Wulur ‚ H, Kho ‚ LK. Treatment of Reye 's syndrome at Sumber Waras Hospital. Acta Paediatr Jpn. 1990;32(4):435 " “442.88 Tein ‚ I. Impact of fatty acid oxidation disorders in child neurology: from Reye syndrome to Pandora 's box. Dev Med Child Neurol. 2015;57(4):304 " “306.99 Singh ‚ P, Goraya ‚ JS, Gupta ‚ K, et al. Magnetic resonance imaging findings in Reye syndrome: case report and review of the literature. J Child Neurol. 2011;26(8):1009 " “1014.1010 Abdin ‚ A, Sarhan ‚ N. Resveratrol protects against experimental induced Reye 's syndrome by prohibition of oxidative stress and restoration of complex I activity. Can J Physiol Pharmacol. 2014;92(9):780 " “788.1111 Ca „ Ÿ ‚ M, Saouli ‚ AC, Audet ‚ M, et al. Reye syndrome and liver transplantation. Turk J Pediatr. 2010;52(6):662 " “664.

ADDITIONAL READING

Belay ‚ ED, Bresee ‚ JS, Holman ‚ RC, et al. Reye 's syndrome in the United States from 1981 through 1997. N Engl J Med. 1999;340(18):1377 " “1382.
Fischer ‚ H. Aspirin and Reye 's syndrome. Pediatrics. 2001;107(1):214.
Glasgow ‚ JF, Middleton ‚ B. Reye syndrome " ”insights on causation and prognosis. Arch Dis Child. 2001;85(5):351 " “353.
Monto ‚ AS. The disappearance of Reye 's syndrome " ”a public health triumph. N Engl J Med. 1999;340(18):1423 " “1424.
Schr ƒ ¶r ‚ K. Aspirin and Reye syndrome: a review of the evidence. Paediatr Drugs. 2007;9(3):195 " “204.
Whitten ‚ R, Milner ‚ DAJr, Yeh ‚ MM, et al. Liver pathology in Malawian children with fatal encephalopathy. Hum Pathol. 2011;42(9):1230 " “1239.

CODES

ICD10

G93.7 Reye 's syndrome ‚

ICD9

331.81 Reye 's syndrome ‚

SNOMED

74351001 Reye 's syndrome (disorder) ‚

CLINICAL PEARLS

RS is a rare, acute noninflammatory encephalopathy largely affecting children and adolescents.
Associations include antecedent viral/bacterial infection and aspirin use.
Most current cases of RS are actually Reye-like syndromes, caused by an inborn error of metabolism or a toxin exposure.
All current treatment is supportive.
Acetaminophen is first choice to treat fever followed by ibuprofen, in children.
Prognosis is related to the degree of cerebral edema and ammonia level on admission and ranges from complete resolution to persistent neurologic sequelae or death.