Reye Syndrome

In 1963, North Carolina doctor George Johnson reported an epidemic of 16 fatal cases of an encephalitis-like illness during an influenza B outbreak. It became known as Reye syndrome syndrome, named after Australian pathologist R. Douglas Reye, who first reported it as a distinct syndrome in 1963. The syndrome had been reported as early as 1929 but now was identified and characterized as a distinct entity. In the late 1960s, the Centers for Disease Control and Prevention (CDC) developed case criteria that included mental status changes (delirium or coma) and a liver biopsy that showed fat accumulation in the liver (or high levels of liver enzymes and ammonia in the blood).

Reye syndrome is still not well understood. It predominantly affects children between 4 and 16 years of age, and occurs more frequently when viral diseases are epidemic, such as during the winter months or following an outbreak of influenza B.

The use of salicylates like aspirin during viral disease appears to be statistically linked to the incidence of Reye syndrome, even though there is no conclusive proof.

No single diagnostic test to detect Reye syndrome currently is available.

Signs and Symptoms

The signs and symptoms of Reye syndrome are almost always preceded by a viral illness. This could be an upper respiratory tract infection,

Seriously ill children will require intensive monitoring during this syndrome. A variety of catheters (nasogastric tubes, urinary catheters, etc.) may be needed to monitor and control fluids, electrolytes, blood gas status, and nutrition. Patients are not given any food by mouth, only intravenous (IV) nutritional fluids. The fluids are also selected to ensure a proper balance of electrolytes, which enhance cell nutrition and help control water balance outside and inside cell walls.

Mechanical ventilation (a breathing machine or respirator) may be necessary if breathing becomes too sluggish. Intracranial pressure (pressure of the fluid within the central nervous system within the skull) and blood pressure may be monitored. The body may be cooled and drugs like barbiturates given in order to slow metabolism and decrease intracranial pressure. Small quantities of insulin may be given to increase glucose metabolism, corticosteroids to reduce brain swelling, and diuretics to increase fluid loss.

The prognosis for children with Reye syndrome has improved. Earlier diagnosis and better treatment have reduced the mortality rate to about 20% in recent years. The earlier the syndrome is detected, the better the chances for survival. Children who progress to the late stages of the syndrome may have continuing neurological defects.

When to Call Your Child's Doctor

Incidence of Reye syndrome has fallen dramatically since its discovery. If, however, your child shows symptoms of nausea, vomiting, or behavioral changes following a viral illness such as the flu or a cold, contact your child's doctor immediately. Of course, many children with viruses will have some of these symptoms. Most will not have Reye syndrome. Nevertheless, early detection is the key to successful treatment of Reye syndrome.

Reviewed by: Joel Klein, MD