What is the treatment and prevention for Reye syndrome?

Treatment and Prevention of Reye Syndrome

The treatment of Reye syndrome focuses on supportive care with correction of metabolic imbalances and reduction of intracranial pressure, while prevention primarily involves avoiding aspirin use in children and teenagers with viral illnesses. 1

Understanding Reye Syndrome

Reye syndrome is a rare but potentially fatal childhood acute encephalopathy associated with liver dysfunction. Key characteristics include:

• Typically follows a viral illness (particularly influenza B)
• Clear association with aspirin therapy during viral infections
• Presents with protracted vomiting and encephalopathy in afebrile patients
• Features minimal or absent jaundice, with hepatomegaly in 50% of cases 1

Clinical Presentation

The classic presentation involves:

• Acute non-inflammatory encephalopathy with altered consciousness
• Elevated ammonia levels 24-48 hours after mental status changes
• Hepatic dysfunction (fatty metamorphosis on liver biopsy or >3x increase in liver enzymes)
• Neurological symptoms typically 24-48 hours after vomiting onset
• Lethargy as the first neurological manifestation
• Diarrhea and hyperventilation may be first signs in children under 2 years 1

Treatment Approach

Since there is no specific treatment for Reye syndrome, management focuses on:

1. Correction of metabolic imbalances:

   • Continuous infusion of hypertonic glucose
   • Management of electrolyte disturbances
   • Monitoring and correction of ammonia levels

2. Reduction of intracranial pressure:

   • Intermittent infusion of hypertonic mannitol
   • Measures to reduce cerebral edema
   • Specialist consultation is essential 1, 2

3. Specialist consultation:

   • Advice should be requested from a specialist in metabolic medicine
   • Many children have underlying inborn errors of metabolism that require specific management 1

4. Supportive care:

   • Careful monitoring of vital signs
   • Management of complications
   • Attention to fluid balance

With improved diagnosis and aggressive therapy, mortality has decreased from 50% to less than 20% 1.

Prevention Strategies

Prevention is critical and focuses on:

1. Avoid aspirin in children and teenagers:

   • Children and teenagers who have or are recovering from chicken pox, flu, or flu-like symptoms should not use aspirin 3
   • The FDA requires warning labels on aspirin products about the risk of Reye syndrome 3

2. Special considerations for children requiring aspirin therapy:

   • Children on long-term aspirin treatment (e.g., for Kawasaki disease) may be at increased risk if they develop influenza 1
   • For children with influenza who require antiplatelet therapy, consider:
     • Using alternative antipyretic drugs (e.g., acetaminophen) for fever
     • Using alternative antiplatelet agents for a minimum of 2 weeks 1

3. Vaccination recommendations:

   • All children ≥6 months on aspirin therapy should receive annual inactivated influenza vaccine
   • Only inactivated vaccine should be administered to children on aspirin therapy
   • Family members should also be vaccinated to reduce transmission risk 1
   • Children with acute conditions during influenza season should receive the inactivated influenza vaccine before hospital discharge 1

4. Varicella vaccine considerations:

   • Some physicians substitute another antiplatelet medication for aspirin during the 6-week period after varicella vaccination
   • Physicians must weigh theoretical risks against known risks of wild-type varicella in children on long-term salicylate therapy 1

Warning Signs and Monitoring

Parents and healthcare providers should be vigilant for early signs of Reye syndrome:

• Changes in behavior
• Nausea and vomiting after viral illness
• Lethargy or confusion
• Rapid progression of symptoms

These symptoms could be early signs of Reye syndrome and require immediate medical attention 3, 4.

Epidemiological Perspective

Since government health warnings about aspirin use in children were implemented, there has been a dramatic decline in Reye syndrome cases 5. This underscores the importance of continued education about aspirin avoidance in children and teenagers, particularly during viral illnesses.