eMedicine Specialties > Emergency Medicine > Pediatric

Pediatrics, Reye Syndrome: Follow-up

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

Updated: Feb 3, 2009

Follow-up

Further Inpatient Care

  • Admission to the intensive care unit (ICU) is warranted for continued monitoring and treatment.

Further Outpatient Care

  • Monitor and treat long-term neurologic sequelae.

Inpatient & Outpatient Medications

  • Prescribe outpatient anticonvulsants if ongoing seizures occur.

Transfer

  • Arrange for ICU admission to a facility that can monitor and manage increased ICP in children.

Deterrence/Prevention

  • Avoid salicylates in children except for in children with conditions for which salicylates are a mainstay of therapy such as Kawasaki disease. Of approximately 200,000 children in Japan treated with aspirin for Kawasaki disease, only one child was reported to have developed Reye syndrome. For children who require long-term use of salicylates, discontinue use immediately at the first signs or symptoms of Reye syndrome.
  • Recognize early symptoms.
  • An IEM may be the actual etiology of the symptoms. Evaluate and treat this possibility.  
  • In addition to recommending influenza vaccine for all children aged 6-59 months age, the CDC recommends influenza and varicella vaccines for children and adolescents aged 5-18 years who require salicylates long term.

Complications

  • Brain herniation, status epilepticus, syndrome of inappropriate secretion of antidiuretic hormone (SIADH), and diabetes insipidus
  • Acute respiratory failure, aspiration pneumonia
  • Cardiovascular collapse
  • GI bleeding, pancreatitis
  • Acute renal failure
  • Sepsis
  • Death

Prognosis

  • The mortality rate has decreased in recent years from 50% to less than 20%. In 1980-1997, the overall case mortality rate in the United States, as the CDC reports, was 31%.
  • Some patients have a poor prognosis.  
    • Patients younger than 5 years have a relative risk of 1.8.
    • The literature is contradictory regarding the cutoff value of ammonia that best predicts prognosis. While most reports indicate that values of >300 mcg/dL are associated with poor prognosis, Belay et al 1999, reported that an ammonia level of 45 mcg/dL was the most accurate predictor, with a relative risk of 3.4.3
    • Rapid progression from stage 1 to stage 3 or presentation with stage 4 or 5 is associated with a poor prognosis. The death rate based on stage at time of admission is 18% for stage 0 and 90% for stage 5.
    • The prognosis is worse with liver and muscle involvement than with either involvement of liver alone (ie, AST/ALT >1, LDH isoenzymes 1-5, elevated creatine kinase MM [CK-MM], creatine kinase MB [CK-MB]).
    • Hypoproteinemia unresponsive to fresh frozen vitamin K and FFP indicates a poor prognosis.
  • Survivors have an increased risk of long-term neurologic sequelae, particularly if ammonia levels are >45 mcg/dL, if they have stage 2-5 disease, and/or if they are younger than 2 years.  
    • The ammonia level is the best predictor.
    • Approximately 3% of patients have neurologic sequelae if levels are <45 mcg/dL, and 11% have sequelae if levels are >45 mcg/dL.

Patient Education

  • Salicylates are contraindicated in children, particularly in children with influenzalike illness or varicella.

Miscellaneous

Medicolegal Pitfalls

  • Failure to identify, treat hypoglycemia
  • Failure to identify, treat hyperammonemia
  • Overhydrating the patient with exacerbation of cerebral edema
  • Failure to aggressively treat cerebral edema (the major cause of morbidity and mortality)
  • Failure to recognize that progression of disease may be extremely rapid
  • Failure to evaluate for, diagnose other etiology (ie, IEM, toxin)

Special Concerns

  • Reye syndrome is now exceedingly rare.
  • Evaluate patients for an IEM that mimics Reye syndrome, particularly (but not exclusively) patients younger than 3 years.
  • Consider a metabolic disease (eg, amino or organic acidemia, defect in the urea cycle or fatty-acid oxidation [particularly medium-chain acyl-CoA dehydrogenase deficiency]) if the following conditions pertain:
    • No viral prodrome
    • No exposure to aspirin or toxin with association to Reye syndrome
    • Patients younger than 3 years (especially those <1 y)
    • Patient or family history of Reye syndrome–type illness
    • Preexisting failure to thrive
    • Baseline neurologic abnormalities
    • Liver dysfunction and/or elevated ammonia level, particularly if it is >1200 mcg/dL and/or if it is elevated longer than 1 week with or without waxing and waning
 


More on Pediatrics, Reye Syndrome

Overview: Pediatrics, Reye Syndrome
Differential Diagnoses & Workup: Pediatrics, Reye Syndrome
Treatment & Medication: Pediatrics, Reye Syndrome
Follow-up: Pediatrics, Reye Syndrome
References
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References

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  2. CDC. National Reye syndrome surveillance--United States, 1982 and 1983. MMWR Morb Mortal Wkly Rep. Feb 3 1984;33(4):41-2. [Medline].

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Further Reading

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Keywords

Reye's syndrome, Reye syndrome, acute noninflammatory encephalopathy, inborn error of metabolism, IEM, Reye syndrome in children, hepatic failure, upper respiratory tract infection, URTI, influenza, varicella, gastroenteritis, use of aspirin, aspirin use in children

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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.

Medical Editor

Garry Wilkes, MBBS, FACEM, Director of Emergency Medicine, Bunbury Health Service, Western Australia Country Health Service; Adjunct Associate Professor, School of Exercise, Biomedical and Health Sciences, Faculty of Computing, Health and Science, Edith Cowan University; Medical Director, St John Ambulance Service
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

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, Assistant 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: none None None

 
 
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