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Johns Hopkins Symptoms and Remedies

Johns Hopkins Medicine

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Reye's Syndrome

What is it?

Reye's syndrome is a rare, life-threatening disorder that almost exclusively affects children under age 16, typically between ages six and eight. Although rare, cases have been reported in adults. Now widely recognized since it was first identified as a distinct disease in 1963, Reye's syndrome is characterized by swelling and inflammation of the brain and liver. The cause is unknown; however, aspirin may act as a trigger of the disease in children, especially when taken for viral infections such as influenza, chicken pox, or the common cold. Symptoms of Reye's syndrome generally appear during the recovery phase of the viral infection, and the disease may progress rapidly, resulting in a coma. The outcome is dependent upon the severity of the symptoms, especially the length of the coma. Reye's syndrome has been fatal in up to 50 percent of cases, but this percentage has been decreasing in recent years with advances in treatment. Patients who survive generally recover fully, with the exception of those few who suffer permanent brain damage. Early detection and treatment may limit damage from the disease and increase the likelihood of survival.

What Causes It?

  • The cause of Reye's syndrome is unknown.
  • Aspirin may trigger Reye's syndrome in children, especially when given for viral infections such as the flu or chicken pox.
  • The primary damage is to the liver; brain injury results from the metabolic disturbances created by the acute liver abnormalities.

Prevention

  • Do not give aspirin to children under 16 years of age. Acetaminophen or ibuprofen should be used instead to relieve pain and fever.

Diagnosis

  • Patient history and physical examination.
  • Blood tests.
  • Lumbar puncture (spinal tap).
  • Liver biopsy may be done, but it is generally not needed. After local anesthesia is administered to the patient, the doctor inserts a needle under the ribs into the liver to extract a small sample of tissue for microscopic examination.

How to Treat It

  • Hospitalization in an intensive care unit is usually necessary.
  • Intravenous fluids are given to restore blood electrolytes to normal and maintain blood glucose levels.
  • Dexamethasone, a corticosteroid medication, may be given to reduce inflammation of brain tissue.
  • Mannitol, a diuretic drug, may be administered to reduce pressure on the brain.
  • Temporary dialysis may be necessary. Dialysis performs the functions of the kidneys by removing waste products and excess fluid from the blood when the kidneys cannot (see Renal Failure, Acute, for more information).
  • A mechanical respirator may be necessary in the event the patient stops breathing.
  • In some cases surgery may be performed to reduce swelling and pressure on the brain.

When to Call a Doctor

  • EMERGENCY Call an ambulance immediately if a child starts to vomit and becomes excessively drowsy in the aftermath of a viral infection.