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Alternative Names Return to topMucocutaneous lymph node syndrome; Infantile polyarteritis
Definition Return to top
Kawasaki disease is a rare condition in children that involves inflammation of the blood vessels.
Causes Return to top
Kawasaki disease occurs most frequently in Japan, where the disease was first discovered. In the United States, after congenital heart defects, Kawasaki disease is the leading cause of heart disease in children. Most of these patients are younger than age 5. The disease occurs more often in boys than in girls.
Kawasaki disease is a poorly understood illness. The cause has not been determined. It may be an autoimmune disorder. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart.
Kawasaki disease can cause inflammation of blood vessels in the arteries, especially the coronary arteries. This inflammation can lead to aneurysms. An aneurysm can lead to a heart attack, even in young children, although this is rare.
Symptoms Return to top
Kawasaki disease often begins with a high and persistent fever greater than 102°F, often as high as 104°F. A persistent fever lasting at least 5 days is considered a classic sign. The fever may last for up to 2 weeks and does not usually go away with normal doses of acetaminophen (Tylenol) or ibuprofen.
Other symptoms often include:
Additional symptoms may include:
Exams and Tests Return to top
No tests specifically diagnose Kawasaki disease. The diagnosis is usually made based on the patient having most of the classic symptoms.
However, some children may have a fever lasting more than 5 days, but not all of the classic symptoms of the disease. These children may be diagnosed with atypical Kawasaki disease. Therefore, all children with fever lasting more than 5 days should be evaluated, with Kawasaki disease considered as a possibility. Early treatment is essential for those who do have the disease.
The following tests may be performed:
Procedures such as ECG and echocardiography may reveal signs of myocarditis, pericarditis, arthritis, aseptic meningitis, and inflammation of the coronary arteries.
Treatment Return to top
Children with Kawasaki disease are admitted to the hospital. Treatment must be started as soon as the diagnosis is made to prevent damage to the coronary arteries and heart.
Intravenous gamma globulin is the standard treatment. It is given in high doses. The child's condition usually greatly improves within 24 hours of treatment with IV gamma globulin.
High-dose aspirin is often given along with IV gamma globulin.
Even when they're treated with aspirin and IVIG, up to 25% of children may still develop problems in their coronary arteries. Some research has suggested that adding steroids to the usual treatment routine may improve a child's outcome, but more research is needed.
Outlook (Prognosis) Return to top
With early recognition and treatment, full recovery can be expected. However, about 1% of patients die from complications of coronary blood vessel inflammation. Patients who have had Kawasaki disease should have an echocardiogram every 1 - 2 years to screen for heart problems.
Possible Complications Return to top
Complications involving the heart, including vessel inflammation and aneurysm, can cause a heart attack at a young age or later in life.
When to Contact a Medical Professional Return to top
Call your health care provider if symptoms of Kawasaki disease develop. A persistent high fever that does not come down with acetaminophen or ibuprofen and lasts more than 24 hours should be evaluated by a health care provider.
Prevention Return to top
There are no known measures that will prevent this disorder.
References Return to top
Sakata K, Hamaoka K, Ozawa S, et al. A randomized prospective study on the use of 2 g-IVIG or 1 g-IVIG as therapy for Kawasaki disease. Eur J Pediatr. 2007 Jun;166(6):565-71.
Newburger JW, Sleeper LA, McCrindle BW, et al. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Engl J Med. 2007 Feb 15;356(7):663-75.
Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.Update Date: 12/10/2007 Updated by: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.; and Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of Philadelphia.