Reye's syndrome (patient information)

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What is Reye's syndrome?

Reye syndrome is sudden (acute) brain damage (encephalopathy) and liver function problems of unknown cause.

The syndrome has occurred with the use of aspirin to treat chickenpox or the flu in children. However, it has become very uncommon since aspirin is no longer recommended for routine use in children.

What are the symptoms of Reye's syndrome disease?

Reye's syndrome progresses through five stages, explained below:

• Stage I
  • Persistent, heavy vomiting that is not relieved by eating
  • Generalized lethargy
  • General mental symptoms, e.g. confusion
  • Nightmares
  • High Fever
• Stage II
  • Stupor caused by minor brain inflammation
  • Hyperventilation
  • Fatty liver (found by biopsy)
  • Hyperactive reflexes
• Stage III
  • Continuation of Stage I and II symptoms
  • Possible coma
  • Possible cerebral edema
  • Rarely, respiratory arrest
• Stage IV
  • Deepening coma
  • Large pupils with minimal response to light
  • Minimal but still present hepatic dysfunction
• Stage V
  • Very rapid onset following stage IV
  • Deep coma
  • Seizures
  • Multiple Organ failure
  • Flaccidity
  • Extremely high blood ammonia (above 300 mg/dL of blood)
  • Death

Who is at risk for Reye's Syndrome?

Reye syndrome is most often seen in children ages 4 - 12. Most cases that occur with chickenpox are in children ages 5 - 9. Cases that occur with the flu (influenzae type B) are usually in children ages 10 - 14.

Children with Reye syndrome get sick very suddenly. Reye syndrome usually follows an upper respiratory infection (URI) or chickenpox by about 1 week.

How to know you have Reye's syndrome (Diagnosis)?

There are a range of tests, having varying accuracy. Some require only a blood sample while others require more invasive measures, such as a biopsy. The tests are:

• Blood chemistry tests
• Head CT or head MRI scan
• Liver biopsy
• Liver function tests
• Serum ammonia test
• Spinal tap

When to seek urgent medical care

Go to the emergency room or call the local emergency number (such as 911) immediately if your child has confusion, lethargy, or other mental changes.

Treatment options

There is no specific treatment for this condition. The health care provider will monitor the pressure in the brain, blood gases, and blood acid-base balance (pH).

Treatments may include:

• Breathing support (a breathing machine may be needed during a deep coma)
• Fluids by IV to provide electrolytes and glucose
• Steroids to reduce swelling in the brain

Where to find medical care for yourdisease

Directions to Hospitals Treating Reye's syndrome

Prevention of Reye's syndrome

Never give a child aspirin unless told to do so by your doctor.

When a child must take aspirin, take care to reduce the child's risk of catching a viral illness such as the flu and chickenpox. Avoid aspirin for several weeks after the child has received a varicella (chickenpox) vaccine.

Note: Other over-the-counter medications, such as Pepto-Bismol and substances with oil of wintergreen also contain aspirin compounds called salicylates. Do not give these to a child who has a cold or fever.

What to expect (Outlook/Prognosis)

Documented cases of Reye’s syndrome in adults are rare. The recovery of adults with the syndrome is generally complete, with liver and brain function returning to normal within two weeks of the illness. In children, however, mild to severe permanent brain damage is possible, especially in infants. Over thirty percent of the cases reported in the United States from 1981 through 1997 resulted in fatality.

Sources

• Medline
• Wikipedia

References

Cohen J, Powderly WG. Infectious Diseases. 2nd ed. New York, NY: Elsevier; 2004:310-311.

Goetz, CG. Textbook of Clinical Neurology. 2nd ed. St. Louis, Mo: WB Saunders; 2003: 656-657.

Ferri FF. Ferri’s Clinical Advisor: Instant Diagnosis and Treatment. 2005 ed. St. Louis, Mo: Mosby; 2005:738.

Noble J. Textbook of Primary Care Medicine. 3rd ed. St. Louis, Mo: Mosby; 2001:46-47.

Michaels MG. Rye Syndrome. In: Long SS, Pickering LK, Prober CG, eds. Principles and Practice of Pediatric Infectious Diseases. 2nd ed. Philadelphia, Pa:Churchill Livingstone; 2003:chap 50.