What is Reye syndrome?

Reye syndrome is a rare cause of encephalopathy (brain disease) and liver dysfunction in children.

Summary

• Reye syndrome is a rare cause of encephalopathy (brain disease) and death in children
• There is a correlation between the incidence of Reye syndrome and children suffering from a viral illness, especially influenza or chicken pox, which are given aspirin to reduce their fever.
• For this reason, aspirin is not recommended for routine use in children.
• Most cases occur between the ages of 4 and 12 years, affecting boys and girls equally.
• Usually soon after symptoms of the viral infection have resolved the child suddenly develops intractable vomiting rapidly associated with an altered state of consciousness, ranging from lethargy to coma and death.
• There is concomitant liver involvement and dysfunction that ranges from mild to severe.
• The cause is unknown.
• There is no specific treatment, but careful monitoring and supportive treatment is needed to reduce brain swelling and assist liver function.
• The outcome depends on how soon supportive management is started as well as the severity of the disease.
• The majority of children recover completely but there can be residual affects to learning and behaviour. 

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.

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.