Reyes Syndrome

Reye syndrome can occur in individuals of any age following a viral illness. However, it is most common in children between the ages of 4 and 12.

Studies have linked many cases of the syndrome to the use of aspirin by children who had chickenpox or other viral illnesses. Since the connection has been recognized, children under the age of 19 are rarely given aspirin, and as a result, 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. Autopsies of people with Reye syndrome have shown significant fatty buildup in liver and brain cells.

Reye syndrome can be broken down into two phases. First, a person has a viral infection, such as a cold, chickenpox, or flu. The second phase starts when symptoms of Reye syndrome occur during recovery from the viral illness. Symptoms can also start 3 to 5 days after the viral illness begins.

A staging system for this disorder has been developed. Stages 1 to 3 represent mild to moderate illness. There is swelling in the brain and involvement of other body systems. Stages 4 and 5 represent severe illness, with increased intracranial pressure.

• Stage 1: protracted vomiting and lethargy
• Stage 2: vomiting, confusion, deep lethargy, combative behavior, and increased reflexes
• Stage 3: light coma, possible seizures, rigid and flexed arms, and fast breathing
• Stage 4: deepening coma, seizures, rigid and extended arms, and pupils that are fixed and unresponsive
• Stage 5: deep coma, loss of reflexes, fixed and dilated pupils, and lack of breathing or heartbeat

An individual can progress from stages 1 to 5 quickly, sometimes in less than 24 hours. Stage 1 has the best outcome; stage 5 has the worst. Most people with Reye syndrome will have a mild illness without serious brain problems.

Reye syndrome can develop in a person who hasn't taken aspirin. However, most individuals with the disease have recently used medicines containing aspirin or other salicylates. Viral infections associated with Reye syndrome include the following:

• chickenpox
• colds
• flu
• rubella (formeraly known as German measles)
• herpes
• mononucleosis caused by cytomegalovirus or Epstein-Barr virus
• mumps
• poliomyelitis

Children under the age of 19 should not be given medicines containing aspirin or other salicylates, unless prescribed by the healthcare professional. Parents should read medicine labels carefully and avoid those that list salicylates or salicylic acid. Adolescents should also be taught to read labels. Salicylates are commonly found in the following types of medicines:

• antacids
• antinausea medicines
• arthritis medicines
• cold remedies
• pain medicines, both over the counter and by prescription
• sinus medicines

Acetaminophen (i.e., Tylenol) is a safe alternative for relief of fever or pain.

Diagnosis of Reye syndrome begins with a medical history and physical exam. Blood tests and a liver biopsy can confirm Reye syndrome.

Some children with chronic diseases such as rheumatoid arthritis must be treated long term with medicines that contain aspirin. These children should receive the chickenpox and flu vaccines to reduce their risk of developing Reye syndrome.

Children who do not progress beyond stage 1 disease generally have a complete, rapid recovery. Those who progress to stages 4 and 5 may die or develop permanent, severe disabilities including mental retardation.

Individuals diagnosed and treated early have a much higher survival rate, as high as 90% in some reports. Late diagnosis lowers the survival rate considerably. When the syndrome was first reported, the overall death rate was about 40%. Now, it is less than a third, and probably would be even lower if all the mild cases were recognized and reported. As a rule, infants have a poorer outcome than older children with Reye syndrome.

Reye syndrome is not contagious and poses no risk to others.

There is no specific cure for Reye syndrome. The goal of all treatment is to build up the strength of the child.

Treatments include the following:

• avoiding low blood sugar
• controlling seizures
• providing breathing treatments to avoid lung complications
• providing fluids through an intravenous line (IV)
• reducing brain swelling with medicines
• using a ventilator (an artificial breathing machine)

Survivors of Reye syndrome should be followed closely for evidence of mental retardation. Learning disabilities may not become apparent until the child is in school. Any new or worsening symptoms should be reported to the healthcare professional.