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.
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.
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?
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
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.
Reye syndrome is a medical
emergency and should be suspected in any child showing acute onset of severe
vomiting and altered mental
Diagnosis is based on the clinical picture
and laboratory tests showing abnormal liver function, and confirmed by liver
biopsy showing fatty degeneration.
There is no specific
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.
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
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.
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,
Red Cross Children’s Hospital, Cape Town February 2015.
Previously reviewed by Dr
John D. Burgess, FCPaed (CMSA), Senior Specialist, Red Cross Children's