Reye’s syndrome is an illness characterized by a group of symptoms that include brain dysfunction and degeneration of the liver. It occurs almost exclusively in children and adolescents. Reye’s syndrome is seen worldwide; the incidence in the United States was approximately 0.3 cases per 100,000 persons between 1982 and 1985. It has been decreasing in incidence since the 1980s and is very rare in the Twenty-first Century.
Who is likely to develop Reye’s syndrome?
The cause of Reye’s syndrome is unknown. However, the adolescent who develops Reye’s syndrome often has a history of a preceding viral infection, which may include chicken pox, influenza or gastroenteritis. There is a strong epidemiological association between the ingestion of aspirin during the preceding viral infection and the development of Reye’s syndrome.
Studies have not established a genetic basis for adolescents who develop Reye’s syndrome. Two epidemiological patterns have been associated with the illness. One pattern of Reye’s syndrome cases is clustered mainly in the winter and early spring with cases occurring in certain localities. This pattern is associated with outbreaks of Influenza A and Influenza B. The other epidemiological pattern is seen on a sporadic basis and is associated with chicken pox illness. The patients who develop Reye’s syndrome in association with chicken pox are usually between ages five years and nine years. Those patients who develop Reye’s syndrome in association with influenza are usually older—ages ten years to fourteen years. Use of aspirin is associated with Reye’s syndrome and there has been a dramatic reduction in cases since this association was noted.
In 1980, the Centers for Disease Control and Prevention (CDC) warned physicians and parents not to use aspirin or salicylates in children with influenza or chicken pox. Subsequently, in 1982, the Surgeon General issued a similar advisory and since 1986, a warning label is required on all aspirin containing medications. As a result of the epidemiological association between aspirin and Reye’s syndrome, most clinicians caring for children warn caregivers not to use aspirin or salicylates at all for children and adolescents under age eighteen years. This public health effort has paid off. In 1980, 555 cases of Reye’s syndrome were reported to the CDC, and from 1994 through 1997, no more than two cases were reported each year.
What are the symptoms of Reye’s syndrome?
Reye’s Syndrome is characterized by encephalopathy and fatty degeneration of the liver. The symptoms of encephalopathy could include lethargy, sleepiness, delirium, combative behavior, paralysis and coma. Usually the first sign of illness is recurrent vomiting. The adolescent then can become irritable and gradually develop lethargy. As the illness worsens, the teen may develop restlessness, disorientation, agitation, seizures and coma.
Fatty degeneration of the liver is characterized by changes in the blood levels of certain liver enzymes. Sometimes the patient’s blood coagulation is abnormally long, and some develop increases in the blood ammonia levels. Some patients develop bleeding problems during the Reye’s syndrome illness.
Approximately one third of patients with Reye’s syndrome either do not survive or they develop long-term neurological problems. Survivors may have deficits in measured intelligence, achievement, visual-motor integration and concept formation.
How is Reye’s syndrome evaluated?
If an adolescent has symptoms that are consistent with Reye’s syndrome, then laboratory studies of liver function including liver enzymes and ammonia may be ordered. Other studies include an electroencephalogram to establish brain wave function, and a biopsy of the liver to determine if there is fatty degeneration. A spinal tap or imaging of the brain by computerized tomography or MRI may also be done.
How is Reye’s syndrome treated?
There is no specific therapy for Reye’s syndrome. Usually an adolescent will be hospitalized in the intensive care unit of a hospital for close monitoring of vital functions. Attempts are made to correct liver abnormalities, seizures may be treated with anticonvulsant medications and there will be efforts made to control the pressure inside the brain, which can be elevated in Reye’s syndrome. Needless to say, salicylates will not be used; occasionally vitamin K is administered since this is manufactured in the liver and can be diminished in Reye’s syndrome. A low vitamin K level could lead to bleeding problems. If an adolescent develops a coma, then assisted ventilation will likely be needed.
How can Reye’s syndrome be prevented?
First and foremost, one should never administer aspirin to any child or adolescent under age eighteen years unless a physician orders it. This is the most important tool to prevent cases of Reye’s syndrome and it works. Adolescents should be vaccinated against chicken pox since cases of Reye’s syndrome have occurred during epidemics of chicken pox. And if one is required to give aspirin to an adolescent because he or she has been diagnosed with arthritis or another medical problem, then one should be certain that the patient is vaccinated annually against influenza as well as chicken pox.
Related topics:
Arthritis, chicken pox, colds and flu, encephalitis, immunizations, learning disorders, seizures
