Friday, July 15, 2011

Kawasaki disease is a frightening, but treatable condition

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STATEN ISLAND, N.Y. — Kawasaki disease is a condition that causes inflammation in the arteries throughout the body, including the coronary arteries, which supply blood to the heart. It predominantly affects infants and children, and involves peeling skin and high fevers. 

The cause of Kawasaki disease remains a mystery. All the recent evidence suggests an infectious agent, although no organism has been found. 


This disease occurs in phases. The first phase, which can last for up to two weeks, usually involves a persistent fever higher than 104 degrees that lasts for at least five days. 

The second phase occurs two weeks after the fever begins, and the skin of the hands and feet may begin to peel in large pieces. The child may also experience joint pain, diarrhea, vomiting or abdominal pain. 

In addition to a fever that lasts at least five days, the diagnostic criteria for Kawasaki disease requires four of the five following criteria: (1) blood-shot eyes; (2) changes in the mucous membranes of the upper respiratory tract such as red pharynx, fissured or cracked lips, or strawberry appearing tongue; (3) rash; (4) swelling, redness or skin peeling of the extremities and (5) enlarged lymph nodes in the neck region. 

A sonogram (echocardiogram) is done as soon as the diagnosis is entertained to rule out inflammation of the heart and to check for aneurysm formation of the coronary arteries. The screenings are then done periodically to monitor the condition. 

If the disease is suspected, treatment is started immediately with aspirin and intravenous immunoglobulin. 

These both have anti-inflammatory effects. The sooner these medications are started, the less likely complications such as aenurysms may occur. 

If a child is diagnosed with an aneurysm, aspirin is continued indefinitely to prevent rupture of the aneurysm or clot formation. 

Coronary artery aneurysms occur in 20 to 25 percent of untreated children. Resolution of aneurysms occurs in one to two years in approximately 50 percent of patients. Heart attacks caused by a clot in an abnormal coronary artery is the principal cause of death. The greatest risk occurs in the first year after diagnosis. 

Recent evidence suggests there may be an incomplete form of Kawasaki disease occurring in some individuals. Some patients do not fulfill the clinical criteria for Kawasaki disease and are diagnosed based on echocardiographic findings of coronary artery anomalies. 

Therefore, strict adherence to the diagnostic criteria may miss some cases. So when should incomplete Kawasaki disease be considered? If a child has unexplained fever for five or more days associated with two or three of the diagnostic criteria mentioned above. 


Other diseases that may present in a similar manor include: Measles, strep throat and drug hypersensitivity reactions. 

Kawasaki disease can be quite devastating. It is imperative to seek medical attention as soon as possible, as the earlier it is diagnosed, the better the outcome. 

Kawasaki disease was first described in Japan in 1967 by Tomisaku Kawasaki; the disease is now known to occur in the Americas, Europe and Asia in children of all races. 

It occurs in 150 cases out of every 100,000 children younger than 5 in the United States and Canada and is markedly more common in Japan and in children of Japanese ethnicity. 

Recurrence rates approach 3 percent. About 1 percent have a family history of it. The risk of occurrence in twins is approximately 13 percent. The data suggests a genetic predisposition may play a role. 


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