Christine Ziebold, MD, PhD, MPH, joins pediatric infectious disease physician and epidemiologist at University of Iowa Children's Hospital, located in UI Hospitals and Clinics, talks about Reye's syndrome:
What is Reye's syndrome?
An uncommon but severe, acute childhood illness affecting the brain and the liver. The cause is unknown. The disease process is thought to be put in motion by a virus with additional damage by using aspirin. Electron microscopy of liver biopsy specimens has shown damage to the so called mitochondria, which are mini power plants in each of our cells. When these don't function right, like in babies born with mitochondrial diseases and what we call "inborn errors of metabolism," we see a similar picture but not acute, more in slow motion. It's unclear if the liver gets damaged first and the brain from liver byproducts later or both at the same time. We basically diagnose it by making sure there are no other explanations for the brain and liver abnormalities like meningitis and hepatitis, intoxications etc.
Who is affected by Reye's syndrome?
Almost exclusively children between 5 and 14 years who have been given aspirin to treat illnesses with fever, most commonly influenza and also chickenpox but also other viruses. Rarely cases have been recognized in adults and neonates.
Is there a connection between aspirin and Reye's syndrome?
Yes. While nobody knows what causes Reye's, we do know from studies looking back in time at cases that occurred, that using aspirin clearly increases the risk. Also we know from lab studies that aspirin interferes with mitochondrial function. Since about 1980, the frequency of Reye's syndrome in the world has decreased dramatically, probably because of education and awareness such as this day, resulting in less aspirin consumption in childhood.
Is Reye's syndrome contagious?
No
Is there a particular time of the year Reye's syndrome is more likely to occur?
Yes, during the influenza season, which in Iowa starts end of September until March.
What are the symptoms of Reye's syndrome?
It starts several days after a typical respiratory illness. There's nothing unique or characteristic about that. No unusual severity. As the child is recovering, severe vomiting begins abruptly. It can last for hours to one to two days. Fever is variable. The brain affliction which we call encephalopathy is a slow and surreptitious change in consciousness. This can be subtle and difficult to pinpoint at first but will progress to lethargy to delirium, seizures, and respiratory arrest. We divide into five stages by Level of consciousness, posture, pupillary reaction, pain response. Some people may progress from one stage to the next over hours to days or they may stop spontaneously at any stage, and their symptoms then regress. About one third of patients have generalized seizures. Few patients whose disease reaches stage V survive. Even with medical intervention three of 10 die.
How important is it to catch this syndrome in the early stages?
It is very important. First, as other serious diseases may cause vomiting and change in consciousness its important to sort out what it is. Secondly, the chance of beating the odds is higher when Reye is caught early. Still three of 10 patients with Reyes syndrome die. The chance to preserve brain and nerve functions before irreversible problems have occurred is higher when attended to early.
How is Reye's syndrome treated?
Basically by supporting major life functions. This means in an intensive care unit. The brain swells, which means the so called "intracranial pressure" needs to be monitored The brain tries to shift the only place it can, which is across tent like structures within the skulland out through the hole in the base of the skullwhere the spinal cord comes in. This squeezes the midbrain and the brainstem. It can cause general under perfusion or heart beat abnormalities and respiratory arrest, which are the major causes of death in Reye, not so much the liver. To decrease the brain pressure we use all kinds of things, drugs, fluids and artificial breathing/ventilation, head positioning. Glucose is given to treat or prevent hypoglycemia, especially in children younger than two years that have less starch in their muscles they can burn in an emergency.
What are the potential problems of the Reye's syndrome survivor? Children who survive can have subtle deficits in coordinating movement with visual cues. They can have behavioral and intelligence problems, or severe neurologic problems, like paralysis of one side of the body or of all four limbs, speech disorders, seizure disorder even blindness due to damage to the visual area in the brain. It depends on age, how advanced the clinical stage was and things like the ammonia level.
What is your best advice to avoid contracting Reye's syndrome? Don't give any aspirin to children. Use acetaminophen and ibuprofen instead. If your child has been prescribed aspirin therapy for example for heart problems, he or she ought to get influenza vaccination to reduce their risk of Reye's syndrome. |
