Kawasaki Disease

Kawasaki Disease

Alternate Names

  • mucocutaneous lymph node syndrome
  • Kawasaki's syndrome

Definition

Kawasaki's syndrome is a poorly understood condition that affects young children. It causes severe inflammation in different areas of the body, including the heart and coronary arteries.

What is going on in the body?

Scientists are not sure what causes inflammation of body organs seen in Kawasaki's syndrome. Many experts believe that a virus attacks the body, involving various organs and other tissues.
Children with this syndrome have a severe flulike illness that usually goes away within a week or two. Twenty percent of the children with Kawasaki's syndrome have inflammation of the heart and coronary arteries.

Risks

What are the causes and risks of the disease?

While the exact cause of Kawasaki's syndrome is unknown, many experts believe it is related to a viral infection. Eighty percent of the children with Kawasaki's syndrome are under 5 years old. It is rarely seen in adults or children after age 8. It is more common in boys than in girls. While it can occur in every race, it is more common in children of Asian descent.

Prevention

What can be done to prevent the disease?

There is no known way to prevent Kawasaki's syndrome.

Diagnosed

How is the disease diagnosed?

Diagnosis of Kawasaki's syndrome begins with a medical history and physical exam. This syndrome is hard to diagnose. It is fairly rare and resembles common childhood infections.
When a child fails to get better with antibiotics, a healthcare provider often begins to suspect this syndrome. Because it resembles other infections, various blood tests may be done to help rule out other conditions. These tests include:
  • a complete blood count, or CBC
  • the level of C-reactive protein, which is an abnormal protein seen only during the acute stage of certain illnesses
  • a sedimentation rate, which detects inflammation or infection in the body
  • a spinal tap to collect cerebrospinal fluid
  • blood tests to rule out other infections, like measles
Additional tests may be done, such as:
  • chest X-ray
  • echocardiogram, or ultrasound of the heart
  • EKG, to look for damage to the heart
  • liver function tests
  • urinalysis

Long Term Effects

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. Kawasaki's syndrome recurs in only 2% of the children who recover from the initial episode.
The 20% of children who have heart involvement face many problems, some of which are life-threatening. Heart involvement can cause death, though this is rare. The child may develop congestive heart failure, a condition in which the weakened heart fails to pump enough blood to body organs.
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 condition goes away.

Other Risks

What are the risks to others?

Although many experts believe Kawasaki's syndrome may be linked to a viral infection, it is not considered contagious from person to person.

Treatments

What are the treatments for the disease?

Treatment usually takes place in the hospital. High doses of aspirin (80-100 mg/kg/day) are usually given to relieve symptoms and prevent blood clots. Intravenous gamma globulin may be given early in the condition to limit damage to the coronary arteries.
Treatment for other complications is given as needed. For example, congestive heart failure is treated with diuretics, or water pills. Treatment usually lasts 5 to 10 days for uncomplicated cases.

Side Effects

What are the side effects of the treatments?

Aspirin can cause:
  • allergic reactions
  • kidney damage
  • stomach upset
Aspirin should never be given to children unless it is prescribed by a healthcare provider. Aspirin use by children has been linked to a rare, sometimes deadly condition called Reye's syndrome. Gamma globulin can also cause allergic reactions.

After Treatment

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 condition, however, to detect any potential problems. Those who do develop heart problems will need further monitoring and therapy.

Monitor

How is the disease monitored?

An echocardiogram is done routinely during the course of the syndrome and afterward. It is done to look for aneurysms, which do not always cause symptoms. A child who has an aneurysm may be given aspirin for long periods of time to help reduce the risk of a heart attack in the future. Any new or worsening symptoms should be reported to the healthcare provider.

Sources

The American Heart Association Committee on Rheumatic Fever and Kawasaki Disease: Diagnostic Guidelines for Kawasaki Disease.

Nelson's Textbook of Pediatrics, 15th edition.

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