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Kawasaki disease

Alternative Names
mucocutaneous lymph node syndrome

Definition
Kawasaki disease is a poorly understood condition that affects young children. It causes a fever and severe inflammation in different areas of the body.

What is going on in the body?
Kawasaki disease was first reported in Japanese children after World War II. It was initially thought to only affect Japanese children. However, it has now passed rheumatic fever as the leading cause of heart disease in children in countries like the US.

Children 5 years old or younger are usually affected. Children with this disease have a severe flu-like illness that usually goes away within a week or two. The cause is unclear, although it is thought that toxins from bacteria or a virus could play a role. This disease can be serious because it may lead to inflammation or damage of the heart and its arteries.

What are the signs and symptoms of the disease?
Symptoms may include:
  • fever, often greater than 39 degrees Celsius, for at least 5 days
  • irritability
  • conjunctivitis, or inflammation of the front part of the eye
  • dry, red cracked lips
  • so-called strawberry tongue. In this condition, the tongue turns bright red with little dark red dots all over it, resembling a strawberry.
  • redness of the mouth and throat area
  • swollen lymph nodes in the neck. Sometimes, lymph nodes are swollen all over the body.
  • red skin lesions or a rash, usually on the trunk. Occasionally, the rest of the body may also be affected
  • peeling of the skin of the hands and feet, and sometimes the genital area
  • swelling of the hands, feet, fingertips, and toes
  • joint swelling and inflammation, or arthritis, which may affect both large and small joints
  • symptoms of possible heart involvement, such as shortness of breath, chest pain, and a rapid heartbeat, or tachycardia
Other symptoms that may be observed include: What are the causes and risks of the disease?
The cause is currently unknown. Adults are rarely affected by this disease.

Children have a higher risk of getting it if they are Japanese or younger than 5 years old.

What can be done to prevent the disease?
There is no known way to prevent Kawasaki disease.

How is the disease diagnosed?
This disease is hard to diagnose. It is fairly rare and resembles common childhood infections. When a child fails to get better with antibiotics, a doctor often begins to suspect this disease. The history and physical examination are the main way to make the diagnosis.

Because this disease resembles other infections, various blood tests may be done to help rule out other conditions. These tests include:
  • a full blood count, or FBC
  • a sedimentation rate, which detects inflammation or infection in the body
  • the level of C-reactive protein, which is an abnormal protein that can be detected only during the acute stage of certain illnesses
Additional tests may be done, such as: What are the long-term effects of the disease?
In at least half of the cases, children get better within a few weeks without any long-term effects.

Inflammation of the heart and/or heart arteries occurs in some children. This can cause many problems, some of which are life-threatening. Heart involvement can cause death, though this is rare. Most of the heart problems go away after a short time. However, aneurysms, which are permanent areas of abnormal widening in the heart arteries, may occur. This increases the risk of heart attack long after the disease goes away.

What are the risks to others?
This disease does not seem to be contagious, but the exact cause is still not known.

What are the treatments for the disease?
Treatment is usually done in the hospital. The main treatments are high doses of aspirin and a protein mixture called immunoglobulin. Immunoglobulin is given through an intravenous line, or IV. An IV is a small tube inserted through the skin into a vein, usually in the hand or forearm.

Treatment for other complications is given as needed. For example, congestive heart failure, a condition in which the heart cannot pump blood well, is treated with medications called diuretics, or water pills. Treatment usually lasts 5 to 10 days for uncomplicated cases.

An imaging test called an echocardiogram is done routinely during the course of the disease or afterward. This test uses ultrasound waves to look at the heart. It is done to look for aneurysms, which do not always cause symptoms. A child who has an aneurysm needs to take aspirin for long periods of time to help reduce the risk of a heart attack in the future.

What are the side effects of the treatments?
Aspirin can cause: Aspirin should never be given to children unless it is prescribed by a doctor. Aspirin use by children has been linked to a rare, sometimes deadly condition called Reye's syndrome. Immunoglobulin can cause allergic reactions.

What happens after treatment for the disease?
Most children get better within a few weeks and have no further problems. Monitoring of the heart and heart arteries is needed after this disease, however, to detect any potential problems. Those who do develop heart problems will need further monitoring and therapy.

How is the disease monitored?
During or after treatment, an echocardiogram can be done to monitor the child for heart problems. Further heart testing, monitoring, or treatment may be needed if this test is abnormal.

Author: Jorge Allende, MD
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request


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