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Updated 19 February 2016

Reye syndrome

Reye syndrome is a rare cause of brain disease and death in children.

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Reye syndrome is a disease complex consisting of an acute encephalopathy and fatty degeneration of the liver with associated hypoglycaemia. Dr R. Reye, an Australian pathologist, first described the syndrome in 1963.

Causes

The cause is unknown, but there have been reports of a correlation between the incidence of Reye syndrome and children suffering from a viral upper respiratory tract infection, especially influenza (or chicken pox) that were given aspirin to reduce their fever.

The syndrome reached a peak in the 1970’s and declined steadily after the warnings against the use of aspirin in children in the 1980s.  It is now rarely diagnosed.

In recent years, better laboratory techniques and facilities have resulted in the identification and diagnosis of a number of Reye-like syndromes. These are usually caused by rare inherited metabolic defects and occur in young children.

Who gets Reye syndrome and who is at risk?

All children are at risk but the majority occurs in children between the ages of 4 and 12 years who are suffering from a viral illness, especially influenza or chicken pox.

The situation may be compounded by the use of aspirin in these children.

Symptoms and signs

The typical presentation is a previously well child who appears to be recovering from an upper respiratory tract infection and then suddenly develops severe and protracted vomiting, along with an altered level of consciousness.

The child may just be lethargic and irritable, but in some, there may be a rapid deterioration with seizures and finally coma. This is due to inflammation and swelling of the brain (cerebral oedema), which is the main cause of death.

The liver becomes enlarged and there will be variable manifestations of liver dysfunction from (most cases) mild to (a few cases) complete liver failure. Hypoglycaemia (low blood glucose), is a severe complication as is a tendency to bleed easily.

Diagnosing Reye syndrome

Reye syndrome is a medical emergency and should be suspected in any child showing acute onset of severe vomiting and altered mental status.

Diagnosis is based on the clinical picture and laboratory tests showing abnormal liver function, and confirmed by liver biopsy showing fatty degeneration.

Treatment and management

There is no specific treatment. Successful supportive management depends on the severity of the illness; reducing the brain swelling and countering the effects of the liver failure.

All children are admitted and given intravenous glucose and fluids. Careful monitoring of a child’s level of consciousness and liver function is established.

Blood tests are needed to monitor the illness and exclude other causes of Reye- like illness. Brain imaging may be warranted. Treatment to reduce the brain swelling and prevent seizures may be required. It may also be necessary to provide breathing and blood pressure support and use blood products.

Outcome

The outcome depends on how quickly the diagnosis is made and management started, as well as the severity and duration of the encephalopathy.

Mild cases will recover quickly with no neurological deficit, whereas the more severe cases take longer to recover and may have significant neurological deficit.

Death is rare and is due to brain death from the cerebral oedema.

When to see your doctor

A child with a viral upper respiratory tract infection –particularly influenza or chicken pox) – who suddenly develops severe vomiting associated with changes in behaviour or decreasing consciousness, should be seen by a doctor.

Read more:

Watch out for these flu complications

Signs that your child has the flu

Pneumonia - the big child killer of our time

Reviewed by Dr Rowan Dunkley, Paediatrician, Red Cross Children’s Hospital, Cape Town February 2015.

Previously reviewed by Dr John D. Burgess, FCPaed (CMSA), Senior Specialist, Red Cross Children's Hospital.

 
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