Kawasaki Disease

Leading cause of acquired heart disease in children

Kawasaki disease, also known as mucocutaneous lymph node syndrome, causes inflammation of blood vessels throughout the body. What causes Kawasaki disease is not clear. Some researchers believe it is an infection, others an autoimmune disorder stimulated by an infection or exposure to an environmental toxin. Most cases of Kawasaki disease (80 percent) occur in children younger than four years old, but occasionally it may occur in very young infants or adolescents. Children of Asian ethnicity are at higher risk for developing the disease.

The inflammation caused by the disease can lead to coronary aneurysm and heart attack, making Kawasaki disease the most common cause of acquired heart disease in children in developed countries. Up to 25 percent of children with untreated disease will develop heart problems. Fortunately, only 5-10 percent of children with Kawasaki disease who are treated before the tenth day of illness will develop heart disease.

Symptoms

Kawasaki disease has a typical set of symptoms that occur in 90% of children affected by it, including:

• High fever for at least five days
• Eye irritation (conjunctivitis) without pus
• Dry, red lips that crack and bleed and/or bumpy red tongue ("strawberry tongue")
• Palms of hands and soles of feet are red; skin starts to peel off fingers and toes about two weeks after illness starts
• Red rash on body

Other symptoms may include enlarged lymph nodes in the neck (50-75 percent of children), extreme irritability, joint pain or swelling, and poor appetite.

Diagnosis

Diagnosis of Kawasaki disease is based on the child having a high fever for five days plus four of the other signs. Very young infants may not show as many of the typical signs of the disease. Blood tests will show elevated erythrocyte sedimentation rate (ESR), C-reactive protein, and alpha-1 antitrypsin levels. An echocardiogram (ultrasound of the heart) is done to check for any aneurysms or heart disease.

Treatment

The main goals of treatment for Kawasaki disease are to reduce or prevent heart disease and to relieve the symptoms. Intravenous gamma globulin and aspirin are given to reduce the risk of heart disease. An echocardiogram will be done 3-4 weeks after the first one to watch for changes in the arteries of the heart. The disease has a long recovery time, and the child may need to take aspirin for three months or more. Fortunately, if treatment is started early in the disease, the child will, in most cases, avoid heart complications.

_Source:

Newburger, Jane W., Masato Takahashi, Michael A. Gerber, Michael H. Gewitz, Lloyd Y. Tani, Jane C. Burns, Stanford T. Shulman, Ann F. Bolger, Patricia Ferrieri, Robert S. Baltimore, Walter R. Wilson, Larry M. Baddour, Matthew E. Levison, Thomas J. Pallasch, Donald A. Falace, & Kathryn A. Taubert. "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease." Circulation 110(2004): 2747-2771.