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Reye's syndrome

Reye's syndrome is a rare disorder that occurs after a viral infection. It results in sudden and severe decline in brain and liver function.

What is going on in the body? 
Reye's syndrome occurs mostly in otherwise healthy children between the ages of 4 and 12. Studies have linked the syndrome to the use of aspirin-containing medication by children who had chickenpox or a flu-like illness. Now children rarely use aspirin, and there are few cases reported each year.

It is not known how aspirin and certain viral infections change the way the body functions. It is known that damage occurs to the mitochondria, the tiny organs in each cell that control cell energy and breathing. Biopsy and autopsy studies of organs of people with Reye's syndrome have shown significant fatty buildup in liver and brain cells.

What are the signs and symptoms of the disease? 
Reye's syndrome can be broken down into a two phases. First, a child has an upper respiratory infection, chicken pox or a flu-like illness followed by a brief recovery. Then, about 5 to 7 days after the onset of the viral infection (or 3 to 6 days after the chickenpox rash appears), vomiting suddenly begins. Either at the same time the vomiting begins or a few hours later, the child becomes stuporous, delirious and combative. In more severe cases, this may progress quickly to seizures (sudden, uncontrollable muscle spasms or convulsions), coma and death.

A staging system for this disorder has been developed. Stages 1 to 3 represent mild to moderate illness, with increasing degrees of metabolic dysfunction and swelling in the brain. Stages 4 and 5 represent severe illness, with increased pressure inside the cranium (the part of the skull that holds the brain) and the brainstem.
  • Stage 1: protracted vomiting and lethargy
  • Stage 2: vomiting, confusion, deep lethargy, combative, increased reflexes
  • Stage 3: light coma, possible seizures, arms rigid & flexed, hyperventilation (quick breathing)
  • Stage 4: deepening coma, seizures, arms rigid and extended, pupils fixed-don't respond
  • Stage 5: deep coma, loss of reflexes, pupils fixed and dilated, no breathing or heartbeat
A child can progress from stage 1 to 5 quickly, sometimes in less than 24 hours. Whether the child lives depends on which stage he or she reaches. Stage 1 has the best outcome; stage 5 has the worst. Most children with Reye's syndrome will have a mild illness without serious brain problems.

What are the causes and risks of the disease? 
Children who take aspirin-containing drugs increase their risk of developing Reye's syndrome after a viral upper respiratory infection, influenza B or chickenpox.

Some children with certain chronic diseases (like rheumatoid arthritis) must be treated long-term with drugs that contain aspirin. These children should receive the varicella and influenza vaccines to reduce their risk of developing Reye's syndrome.

What can be done to prevent the disease? 
Children should not be given aspirin-containing drugs without a doctor's approval.

How is the disease diagnosed? 
Any child with prolonged vomiting that begins after an upper respiratory infection, flu-like illness or chicken pox, especially if he or she is lethargic, confused or has seizures, may have Reye's syndrome. Blood tests and a liver biopsy can confirm Reye's syndrome.

There are many childhood disorders or problems with symptoms like Reye's syndrome. These include:
  • genetic liver diseases
  • infections of the central nervous system, like meningitis (an infection of the membrane covering the brain and spinal cord) or encephalitis (an infection of the brain)
  • ingestion of certain drugs such as aspirin and valproic acid, liver biopsy is an important way to rule out these disorders in children under 2 years old.
What are the long-term effects of the disease? 
Children who do not progress beyond stage 1 disease have a complete, rapid recovery. Those who progress to stages 4 and 5 may die or have severe neurological problems and limited mental abilities, if they live. As a rule, infants have a poorer outcome.

What are the risks to others? 
Reye's syndrome is not contagious. There is no risk to other people.

What are the treatments for the disease? 
All children suspected of having Reye's syndrome should be transferred to a paediatric intensive care facility right away. This disorder often progresses rapidly and unpredictably and doctors need to be on hand.

There is no specific cure for Reye's syndrome. The goal of all treatment is build up the strength of the child. It is important to avoid low blood sugar, control seizures and minimise pressure in the cranium (the part of the skull that holds the brain). Children with disease that does not progress beyond stage 1 may only need intravenous fluids and observation. Those who progress beyond stage 1 may need treatment to minimise brain swelling. Comatose people will probably need machines to help them breathe, as well as intracranial pressure monitoring.

How is the disease monitored? 
survivors of Reye's syndrome should be followed closely for evidence of mental deficits, especially learning disabilities, that may not become apparent until the child is in school.

Author: John Wegmann, MD
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Potential conflict of interest information for reviewers available on request

This website and article is not a substitute for independent professional advice. Nothing contained in this website is intended to be used as medical advice and it is not intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice.  All Health and any associated parties do not accept any liability for any injury, loss or damage incurred by use of or reliance on the information.

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