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Kawasaki Disease

Kawasaki Disease was named after a Japanese paediatrician (Tomisaku Kawasaki) who first described it in 1967. It is an illness that mostly affects children under 5 years of age.

The symptoms and signs of Kawasaki disease are caused by inflammation of blood vessels, particularly in the heart (coronary vessels). This inflammation is known as vasculitis. There have been many theories for what causes this, but there isn't one that is generally accepted. It is thought that an infection of some sort causes Kawasaki. This has not been proven. Kawasaki disease is not contagious to other children.

There is no simple test for Kawasaki disease. The diagnosis is made by recognising the symptoms and signs of Kawasaki disease in a child who has a persistent fever with no other likely explanation. Blood tests may be helpful. An echocardiogram (ultrasound of the heart) is performed to look for any changes in the coronary (heart) arteries. This is a safe and painless test; it is similar to the ultrasound examination that is used to look at the baby in a pregnant woman.

Signs and symptoms

The disease usually begins with a high fever (over 39 degrees Celcius) which continues for at least 5 days.

Many, but not all, children also develop other symptoms such as:

  • Large, swollen glands in the neck
  • A rash (that often peels later on in the illness)
  • Red shiny or dry cracked lips
  • Red, lumpy (strawberry looking) tongue
  • Red eyes (conjunctivitis) without discharge
  • Swollen / red hands or feet
  • An unusual nappy rash
  • Joint pains
  • Extreme irritability (especially in young children)

Other diseases can be confused with Kawasaki disease. They may require different treatment.

The most important part of Kawasaki disease is that it may cause inflammation of the arteries that supply blood to the heart. This occurs in about 25% of patients if they do not receive treatment.

Treatment

The treatment for Kawasaki disease is intravenous (into a vein through a drip, also called IV) gammaglobulin. This is made from donated blood transfusions and contains concentrated amounts of antibodies.

Large doses of intravenous gammaglobulin are usually effective in stopping the fever and other symptoms of Kawasaki disease, and seem to help prevent coronary artery problems as well. 

Some parents worry about their child being given blood products. It is important to understand that the risks of this are very low, yet the risks from untreated Kawasaki disease are quite high.

Most children will also be given aspirin for a few weeks after the onset of Kawasaki disease. This helps to prevent problems with the coronary arteries.

Your child may have to stay in hospital for a few days until the illness begins to settle. Once you go home it may take 3 or 4 weeks for your child to be fully back to normal. Don't be alarmed if your child gets some peeling of the skin on the hands, feet or groin area - this is very common in the second week of the illness.

Care at home

A small number of children develop problems with either the heart muscle or the coronary arteries. The heart problems appear in the first phase of the illness and this is why the initial echocardiogram is performed. If the test is negative, then it is very unlikely that problems will develop later but a further check test is usually done at 6-8 weeks.

A small percentage of children have recurrence of the symptoms weeks or months later - if this happens consult your paediatrician.

If your child has Kawasaki disease, they will need to be followed up long-term by their GP (family doctor) and a paediatrician. 

If your child is due a routine MMR (measles, mumps, rubella) immunisation - this should be delayed until 3 months after the gammaglobulin treatment. 

The vast majority of children with Kawasaki disease recover and live normal lives.

Key points to remember

  • Disease starts with a high fever that lasts 5 days or more.
  • It is treated with intravenous gammaglobulin and aspirin.
  • Strict follow-up is required  by your GP and Paediatrician.
  • Most children with Kawasaki disease recover completely. 

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The content for this fact sheet has been contributed to by the following RCH departments: General Medicine. Reviewed October 2008.

 

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