Reye Syndrome
What is Reye Syndrome?
Reye syndrome (or Reye’s syndrome; pronounced “rye”) is a rare but serious and potentially life-threatening illness. While it can impact all organs, Reye syndrome primarily affects the brain and liver. Swelling of the brain may increase the pressure inside of the head (called intracranial pressure, or ICP) which may result in acute neurological changes. Studies have shown a link between Reye syndrome and the use of aspirin or aspirin-containing products in children and teenagers during a viral illness, most commonly influenza A and B (the flu) and varicella (chickenpox). Therefore, the American Academy of Pediatrics (AAP) recommends to NOT give aspirin or aspirin-containing medications to children and teenagers unless instructed by your child’s healthcare provider.
What Causes Reye Syndrome?
The exact cause of Reye Syndrome is unknown. While Reye syndrome can affect someone of any age, it is most common in children ages 4-14 years old. Studies performed by the Centers for Disease Control and Prevention (CDC) have found an association between the use of salicylates (aspirin) and the development of Reye syndrome. While rare (less than 0.1% of children who take aspirin develop Reye syndrome), studies have shown that 80% of children diagnosed with Reye syndrome took aspirin within 3 weeks prior to diagnosis. These studies have also shown that cases of Reye syndrome usually occur after viral infections, primarily after influenza A and B (the flu), varicella (chickenpox), and gastroenteritis (often called the “stomach flu”).
What Are the Signs and Symptoms of Reye Syndrome?
Since Reye syndrome usually develops after a viral infection, the AAP recommends remaining on the lookout for the following pattern of signs and symptoms that is typical in Reye syndrome. This pattern usually starts with a viral infection such as the flu, upper respiratory infection (URI), or chickenpox. During recovery from the illness the child may seem to be improving but then suddenly starts vomiting, frequently and repeatedly. Lethargy (sleepiness), personality or behavioral changes (irritability, anger/aggressive behavior, confusion/delirium) may follow. If left untreated, the child may have seizures, become unresponsive, and have loss of consciousness. Reye syndrome is considered a medical emergency—take your child to the nearest emergency department immediately once this pattern of illness is suspected.
Signs and Symptoms:
Persistent or recurrent vomiting
Lethargy (sleepiness)
Confusion/delirium
Personality/behavioral changes (irritability, anger, aggressive/combative behavior)
Rapid breathing (hyperventilation)
High heart rate (tachycardia)
Seizures
Loss of consciousness (coma)
Signs and Symptoms in Infants:
Full or bulging fontanelle (soft spot located on top of infant’s head)
Rapid breathing (hyperventilation)
Diarrhea
High-pitched cry
How is Reye Syndrome Diagnosed?
Reye syndrome is diagnosed with a physical exam where your child will be assessed, and their past medical history will be discussed including the occurrence of a recent cold or viral illness or the recent use of any aspirin or other aspirin-containing medications. Diagnostic tests may be used to confirm the diagnosis of Reye syndrome. These possible tests include:
Blood- and liver-function tests—To look at liver function and other signs such as elevated serum ammonia levels
Urine and stool tests—To detect any signs of illness
Liver biopsy—A small amount of tissue from the liver is removed and studied
Electroencephalogram (EEG)—A test that measures the brain’s electrical activity using electrodes placed on the scalp
Lumbar puncture (spinal tap)—The pressure of the spinal canal and brain can be measured using a special needle placed into the lower back. A small sample of cerebrospinal fluid (or CSF, the fluid that surrounds the brain and spinal cord) is collected and sent for testing (to detect any infection)
Intracranial pressure (ICP) monitoring—To measure the pressure inside of the head
How is Reye Syndrome Treated?
There is no cure for Reye syndrome. Since Reye syndrome is a serious illness that can rapidly progress, early detection is key. Treatment is mainly supportive (treatment of symptoms) and requires close monitoring in a hospital setting, usually in the intensive care unit (ICU). The main goal of treatment is to monitor and reduce the swelling of the brain and resulting increased pressure in the head. Medications may be used to reduce inflammation of the brain and to control seizures, fever, or other issues. In severe cases, a child may need to be intubated and placed on a ventilator (breathing machine).
The prognosis, or outlook, for children with Reye syndrome has improved significantly thanks to early recognition and aggressive treatment. While brain damage and disability or death can happen in late stages, two-thirds of patients diagnosed with Reye syndrome have a full recovery.
How Can I Help Prevent Reye Syndrome in My Child?
After studies linked the use of aspirin in children to Reye syndrome, widespread warnings were issues in the United States advising against the use of aspirin in children in 1980. Following these warnings, a sharp decline in the occurrence of Reye syndrome was noted. Therefore, the AAP recommends avoiding the use of aspirin or other salicylate-containing medications in children and teenagers, particularly during any viral illness such as the flu or chickenpox unless instructed to by their healthcare provider for certain other health conditions (such as Kawasaki disease). It is important to read all labels as many over-the-counter drugs contain salicylates, such as bismuth subsalicylate (or Pepto-Bismol). Consider the use of acetaminophen (Tylenol) or ibuprofen (Motrin) for fever or pain instead (talk to your child’s healthcare provider first for specific medication recommendations). If you are unsure if a medication is safe, talk to your child’s primary care provider or pharmacist.
Education of parents and caregivers about the signs and symptoms of Reye syndrome and when to seek immediate medical attention can help to improve treatment and prognosis. Furthermore, the CDC recommends that all children over the age of 6 months old should receive a flu vaccine every season (with rare exceptions).
Key Points to Remember
Reye syndrome is an acute and potentially fatal illness. Since it is a rapidly progressing disease, early detection is key. If not treated quickly, Reye syndrome can result in loss of consciousness (coma), brain damage, or death. While rare, it most commonly occurs in children and teens following viral illness. Since studies have shown a link between the use of aspirin and the development of Reye syndrome, the AAP recommends avoiding the use of aspirin or other salicylate-containing medications in children and teenagers. It is important to read all medication labels carefully as many over-the-counter medications may contain salicylates. If in doubt about a medication or ingredient, always talk to your child’s healthcare provider.
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