Kawasaki Disease

Overview

Kawasaki disease is a condition in which the inside walls of small and medium sized blood vessels (arteries) throughout the body become inflamed, including the blood vessels (coronary arteries) that supply oxygen to heart muscle. It’s also called mucocutaneous lymph node syndrome because it also affects lymph nodes, skin and the mucous membrane linings of the mouth, nose, and throat. It’s uncommon, but the leading cause of coronary artery disease in children.  No one knows it’s origin. If the disease isn’t recognized and treated promptly the coronary arteries will become inflamed and abnormal. Most cases occur in children 1-8 years old, with the greatest number between 18 and 24 months of age. It occurs primarily in winter and spring.

Symptoms and Diagnosis

Kawasaki disease occurs in stages. The first phase includes a fever higher than 101.3 F (38.5 C), and is usually above 102.2 F (39.0 C), lasting at least 5 days and up to 2 weeks. The eyes are very red and inflamed (conjunctivitis), but there is no discharge. The lips are dry and cracked, the tongue very swollen and red, called “strawberry tongue.” Palms and soles are very swollen and red, and lymph nodes in the neck are swollen. The child is irritable. In the second phase the skin on the hands and feet peels off, often in large sheets. There is joint pain, diarrhea and abdominal pain. In the third phase of the illness the symptoms slowly resolve over 6-8 weeks, unless complications occur.
Kawasaki disease is the leading cause of acquired heart disease in children About 20 percent of children who get Kawasaki disease develop heart problems, but only a few have lasting damage. Heart complications include: inflammation of the heart muscle (myocarditis); heart valve problems (mitral regurgitation); abnormal heart rhythm (dysrhythmia); and inflammation of the coronary arteries (vasculitis) that supply blood to the heart muscle.

There is no specific test for Kawasaki disease. Diagnosis is made by excluding other diseases with similar characteristics. Some of the diseases which could be confused with Kawasaki disease are: Stevens-Johnson syndrome, which is a disease of the mucous membranes, scarlet fever, measles, reactions to medications, toxic shock syndrome, tick-borne diseases such as Rocky Mountain spotted fever, and juvenile rheumatoid arthritis. The doctor may order blood tests that can rule out other infections. The white blood cell count is likely to be elevated, and the presence of anemia and inflammation are indications of Kawasaki disease. Urine tests may be done to rule out other diseases. The doctor may order echocardiography, which is an ultrasound of the heart, to monitor the coronary arteries, and an electrocardiogram (ECG) to measure the heart rhythm. Continue reading for Causes, Prevention, and Treatment Information . . .

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