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What is Kawasaki disease?

Kawasaki disease was first described by Dr Tomisaku Kawasaki in 1961. It is also called muco-cutaneous lymphnode syndrome and commonly affects children under 5 years of age. Kawasaki disease is less common in UK (approximately 8 in 100,000 affected) but more common in Asia and Japan. The characteristic symptom occurring in various combination are: high fever lasting for 5 days or more with rash, swollen glands in the neck, dry cracked lips, red fingers or toes, red eyes. The fever is generally at least 38.0 degrees C or higher. The swollen lands are lymph nodes. Dry cracked lips are associated with red tongue and inside of the mouth. Fingers and toes are red, shiny and puffy and may start to peel in the later stage of the disease. Red eyes are not associated with any discharge.


What causes Kawasaki disease?

The exact cause of Kawasaki disease is not known. It is believed that an infection triggers in some children a reaction in their body leading to inflammation of various organs, and affecting the walls of blood vessels (arteries, hence arteritis). The most dangerous consequence is involvement of coronary arteries that supply the heart muscle, that could lead to blockage of coronary arteries leading to heart attack, or weakness within the wall of the arteries, causing aneurysms.



What are the symptoms?

The classic symptoms could be evolving in various combinations and it is important to suspect Kawasaki disease in children who do not have a clear cause for their fever associated with the other symptoms. The combination of the classic symptoms described with fever, swellings in neck, cracked or red lips, red shiny hands or toes or red eyes suggest Kawasaki disease. Sometimes, all features may not be present and it is called “Atypical Kawasaki disease”.



How is it diagnosed?

The diagnosis is essentially clinical and is usually made by the Paediatrician or GP. There is no test that can confirm or rule out Kawasaki disease. A set of investigations involving blood tests and X rays are generally necessary.


Why would you need to see a Paediatric Cardiologist?

One of the most serious complications of Kawasaki disease involves the coronary arteries, blood tubes that supply blood and oxygen to the heart muscle itself. Sometimes, it can cause fluid accumulation around the heart, or involvement of the heart valves. To check if this is the case, Dr Khambadkone needs to perform investigations such as electrocardiogram (ECG) and echocardiogram. Echocardiography is usually sufficient to check involvement of the coronary arteries although it allows only some part of the coronary arteries to be seen well. In some instances, more tests such as CT angiography or Magnetic Resonance Imaging (MRI) may be necessary. Cardiac catheterization and coronary angiography is required in severe condition. A small proportion of patients with severe coronary involvement need life-long follow-up.

How is it treated?

Kawasaki disease is treated by a multi-disciplinary team including the Paediatrician, Paediatric Cardiologist and Rheumatologist. Once suspected, prompt use of immunoglobulins, special proteins also called antibodies produced by the body to fight infections, is advisable. This is given through the vein and needs hospital admission. In addition to this, high dose aspirin is given for early phase of the disease followed by low dose aspirin for at least 6 weeks. If there is coronary enlargement or aneurysm formation, aspirin may be needed for a longer duration, and a stronger blood thinning agent, warfarin, may have to be added.


What is the prognosis?

In most cases, Kawasaki disease is mild and responds well to treatment and does not need any long term follow-up. Those with severe coronary involvement or aneurysms need lifelong monitoring. It is important to reduce the cardiovascular risks by avoiding smoking, maintaining weight, regular exercise, good diet and regular monitoring.

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