T H E  M E D I C I N E  C H E T

Reye's Syndrome

Reye’s syndrome is a potentially deadly disorder that can impact many different organs; however, it is the liver and brain that are usually most seriously affected. Reye’s Syndrome is a two-phase illness because it typically follows a previous viral infection, such as influenza, cold, or chicken pox.

If not treated early enough, severe organ damage leading to death can result. Reye’s Syndrome is a potentially deadly disease and the best treatment is prevention.

What is Reye’s Syndrome?

In 1963, Australian doctor Ralph Reye noted encephalopathy and liver failure in children who had been given aspirin when the children had chickenpox. Several months later, Dr. G. M. Johnson described the syndrome in the U.S. In examining case histories, Dr. Johnson discovered that patients with similar symptoms had been described as early as 1929.

Although Reye’s Syndrome can affect children of any age, it usually impacts children between the ages of 4-14 years. Generally, Reye’s Syndrome develops about a week after a viral illness such as chickenpox, influenza, or the common cold.

What Causes Reye’s Syndrome?

No one knows what causes Reye’s Syndrome. Many studies show that giving aspirin to children to treat a viral disease increases the risk of developing the syndrome.

It seems that aspirin affects the mitochondria, which are tiny structures in every cell that provide energy to the cell. Failure of the mitochondria in the liver allows toxins to build up in the patient’s bloodstream.

Once the liver begins to fail, dangerous toxins (such as ammonia) begin to build up in the blood, causing the patient to feel tired and listless and to begin vomiting. As the liver further deteriorates, the brain may begin to swell, causing changes in the patient’s mental state, such as confusion and delirium.

Other possible causes of Reye’s Syndrome include exposure to insecticides, herbicides, paint thinner, and other toxins.

How Common is Reye’s Syndrome?

Most cases of Reye’s Syndrome occur December-March, the time when most viral illnesses occur. Up until the 1980s, there were more than 1,000 cases of Reye’s Syndrome annually in the U.S.

However, since public education campaigns discouraging the use of aspirin for viral illnesses began, the number of cases has dropped to less than 100 cases per year. This reduction is true not only for the U.S., but for the rest of the world as well.

What are the Symptoms?

Reye’s Syndrome is almost always preceded by a viral illness, such as an upper respiratory infection (URI), chickenpox, or a virus causing diarrhea. Reye’s syndrome can occur from one day to two weeks after a viral infection. It is important to remember that though the viral illness is contagious, Reye’s Syndrome is not.

Patients with Reye’s Syndrome usually progress through five stages, with each stage more serious than the previous stage.

Stage I is characterized by persistent heavy vomiting, tiredness, confusion, and nightmares.

Stage II includes unconsciousness (caused by brain inflammation and pressure), hyperventilation (fast and shallow breathing), fatty deposits in the liver, and hyperactive reflexes.

Stage III includes continuation of Stage I and Stage II symptoms but also includes possible coma, perhaps due to swelling of the brain. The breathing also may stop.

Stage IV is distinguished by a deepening coma and enlarged pupils that do not respond to light. The liver still functions, but only minimally.

Stage V usually happens rather quickly after Stage IV. The coma deepens even further, seizures develop, respiratory failure occurs, the blood level of ammonia increases, and death generally ensues.

Remember, without prompt and proper treatment, Reye’s Syndrome can be fatal within days. Between 1981-1987, more than 30 percent of those afflicted with Reye’s syndrome in the U.S. died.

How Is Reye’s Syndrome Diagnosed?

Reye’s Syndrome is initially diagnosed with blood and urine tests that determine how well the liver is functioning and whether the child has some other disease. Other tests that may be included are spinal taps (to collect spinal fluid) and a liver biopsy.

This consists of collecting a small amount of liver tissue with a long needle and examining the tissue under a microscope. Specialized x-rays such as MRIs or CT scans also may be ordered to image the brain.

Although most patients who have Reye’s Syndrome survive, varying degrees of brain damage may occur. This damage can lead to disability that is based on the type of and amount of damage to the brain. To minimize the chance of brain damage, early diagnosis and treatment are paramount!

How Is Reye’s Syndrome Treated?

Because there is no specific treatment for Reye’s Syndrome, treatment is supportive. Intravenous fluids may be given to prevent dehydration, insulin may be given if the blood sugar drops too low, and steroids may be given to minimize swelling of the brain. If the patient is in respiratory failure, he or she may be connected to a ventilator that will allow him/her to breathe.

How Can Reye’s Syndrome Be Prevented?

Although there is no vaccine for Reye’s Syndrome, the most important preventive step is to make sure the child is up to date on all immunizations. By reducing the risk of disease, this automatically reduces the risk of fever and the need to give fever-reducing medication.

Never give aspirin or medication that includes aspirin to children under the age of 18. Always check the labels of medications (both prescription and over-the-counter) for aspirin. Be aware that aspirin may go by other names such as acetylsalicylic acid, acetyl salicylate, salicylic acid, or salicylate.

Aspirin can hide in unexpected places; so read labels carefully and avoid products with salicylate. Remember, some herbal or “natural” medications also may contain aspirin.

Children with certain diseases, such as juvenile rheumatoid arthritis, may need to be on long-term treatment with aspirin or aspirin-containing medications. If a child under your care needs these medications, make sure that he or she is up to date on immunizations, including chickenpox and influenza.

Joe Knight
Medical Writer and Physician Assistant, Chowchilla, California


Resources

National Reye’s Syndrome Foundation, PO Box 829, Bryan, OH 43506; 800-233-7393; www.reyessyndrome.org

Internet Resources

American Liver Foundation, www.liverfoundation.org/education/info/reye/

Kid’s Health, www.kidshealth.org/parent/infections/bacterial_viral/reye.html

Mayo Clinic, www.mayoclinic.com/health/reyes-syndrome/DS00142

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