Reye's syndrome

• What is Reye’s Syndrome
• Statistics on Reye’s Syndrome
• Risk Factors for Reye’s Syndrome
• Progression of Reye’s Syndrome
• Symptoms of Reye’s Syndrome
• Clinical Examination of Reye’s Syndrome
• How is Reye’s Syndrome Diagnosed?
• Prognosis of Reye’s Syndrome
• How is Reye’s Syndrome Treated?
• Reye’s Syndrome References

What is Reye’s Syndrome

Reye’s syndrome is a condition in which there is brain and liver damage of an unknown cause. It is associated with the use of aspirin to treat chicken pox or influenza in children.
This condition can potentially affect all organs of the body, though the brain and liver are at the greatest risk of damage. It can result in cerebral oedema (swelling of the brain) and the accumulation of fat in the liver and other organs.

Statistics on Reye’s Syndrome

Reye’s syndrome is rare, occurring almost exclusively in children aged less than 18 years. There are estimated to be 0.03-1 cases per 100,000 people aged less than 18 years.
Most cases occur between 5 and 14 years of age, with males and females being affected with the same frequency. The incidence of Reye’s syndrome has decreased dramatically in recent years, possibly due in part to the decreased use of aspirin in children.

Risk Factors for Reye’s Syndrome

Although the cause of Reye’s syndrome is unknown, it typically follows a viral illness such as an upper respiratory tract infection, chicken pox, influenza or gastroenteritis, and is associated with aspirin use during the illness. Any young child who has recently suffered a previous viral infection, such as a flu or chicken pox, should be monitored closely.

Progression of Reye’s Syndrome

The onset of Reye’s syndrome usually follows an upper respiratory tract infection. Within 1-3 days there is persistent vomiting, often with drowsiness and reduced consciousness. This may progress rapidly to convulsions and coma. The liver is often enlarged.
The mortality rate of Reye’s syndrome has fallen from 50% to less than 20% as a result of earlier diagnoses, recognition of milder cases, and more aggressive therapy. Death usually occurs due to swelling of the brain or increased pressure in the brain, but it may also be due to heart failure, respiratory failure, acute renal failure, gastrointestinal bleeding, seizures, or infection.

How is Reye’s Syndrome Diagnosed?

• Blood chemistry tests shows low glucose (hypoglycaemia)
• Elevated liver enzymes (liver function tests)
• Elevated serum ammonia test
• Liver biopsy is sometimes indicated
• Lumbar puncture for cerebral spinal fluid analysis
• An MRI or CT of the head may show swelling of the brain and can rule out other causes of an altered consciousness

Prognosis of Reye’s Syndrome

The prognosis for those who survive the acute illness is good. Long term complications include permanent neurological problems as a result of the brain damage occurring during acute illness. The risk of permanent brain damage is not known, but it is thought to be related to the severity of the acute illness. Permanent liver disease has not been reported in survivors.

How is Reye’s Syndrome Treated?

There is no specific treatment for Reye’s syndrome. Treatment is supportive and will depend on the severity of the illness. Intravenous fluids to provide electrolytes and glucose are given. Steroids are given to reduce swelling in the brain.
Intensive monitoring of the pressure within the brain, blood gases, and blood pH is required. Support for breathing using a mechanical ventilator may be needed during a deep coma.

Reye’s Syndrome References

1. Belay ED, Bresee JS, Holman RC, et al. Reye’s syndrome in the United States from 1981 through 1997. N Engl J Med 1999; 340(18): 1377-82.
2. De Vivo DC. Reye syndrome. Neurol Clin 1985; 3(1): 95-115.
3. emedicine
4. Harrison’s Principles of Internal Medicine, 15th ed.
5. Medline Plus
6. National Institute of Neurological disorders and Stroke, USA