Friday, July 22, 2011

Kawasaki's Syndrome - Kawasaki Disease

Overview, Causes, & Risk Factors

Kawasaki's disease 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 disease. Many experts believe that a virus attacks the body, involving various organs and other tissues. Children with this disease have a severe flulike illness that usually goes away within a week or two. Twenty percent of the children with Kawasaki's disease have inflammation of the heart and coronary arteries.
What are the causes and risks of the disease?
While the exact cause of Kawasaki's disease is unknown, many experts believe it is related to a viral infection. Eighty percent of the children with Kawasaki's disease 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.

Symptoms & Signs

What are the signs and symptoms of the disease?
Kawasaki's disease may cause the following symptoms:
  • arthritis, or joint pain and swelling

  • conjunctivitis, or inflammation of the front part of the eye

  • fever, often greater than 104 degrees F, for at least 5 days

  • irritability

  • peeling of the skin of the hands and feet and sometimes the genital area

  • rash, usually on the trunk

  • redness of the mouth, tongue, lips, and throat

  • swelling of the hands, feet, fingertips, and toes

  • swollen lymph nodes, primarily in the neck
    If the heart and coronary arteries are involved, the child may have the following symptoms:

  • chest pain

  • a rapid heartbeat

  • shortness of breath


  • Diagnosis & Tests

    How is the disease diagnosed?
    Diagnosis of Kawasaki's disease begins with a medical history and physical exam. 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 healthcare provider often begins to suspect this disease.
    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
    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


  • Prevention & Expectations

    What can be done to prevent the disease?
    There is no known way to prevent Kawasaki's disease.
    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 disease 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 disease goes away.
    What are the risks to others?
    Although many experts believe Kawasaki's disease may be linked to a viral infection, it is not considered contagious from person to person.

    Treatment & Monitoring

    What are the treatments for the disease?
    Treatment usually takes place in the hospital. High doses of aspirin are usually given to relieve symptoms and prevent blood clots. Intravenous gamma globulin may be given early in the disease 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.
    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.
    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?
    An echocardiogram is done routinely during the course of the disease 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.


  • Attribution

    Author:Jorge Allende, MD
    Date Written:
    Editor:Crist, Gayle P., MS, BA
    Edit Date:08/31/01
    Reviewer:Eileen McLaughlin, RN, BSN
    Date Reviewed:08/01/01
    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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