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Kawasaki Disease (cont.)

Kawasaki Disease Symptoms and Signs

Kawasaki disease is an acute vasculitis that affects otherwise healthy children. The diagnosis of the disease is based on criteria as follows:

Fever of at least five days duration, exclusion of other causes of fever, and at least four of the following five clinical features

    1. Bilateral nonpurulent conjunctival injection (red eyes)

    2. Changes in the lips and oral cavity (red and cracked lips, strawberry tongue)

    3. Rash (nonpetechial, nonbullous, nonvesicular)

    4. Changes in the extremities (edema of the hands or feet, red hands or feet, peeling of the palms or soles)

    5. Cervical lymphadenopathy (large lymph nodes of the neck, often unilateral)

    6. Or fewer of these findings with evidence of coronary aneurysms or coronary enlargement seen on echocardiogram

Typically, a child with Kawasaki disease will have a with sudden onset of fever that has no apparent source. The fever lasts longer than five days, and the child is irritable and generally ill appearing. In addition to the fever, the above symptoms may develop in any order and duration. The diagnosis is made when the above criteria are met and there is no other explanation for the symptoms such as strep throat or an acute drug reaction.

Kawasaki disease can be divided into phases, including the acute, early phase (fever and other symptoms) that lasts from five to 10 days and is followed by the subacute phase (development of coronary artery aneurysms) from 11-30 days, and finally the convalescent phase (resolution of acute symptoms) from four to six weeks. For untreated patients, 25% develop coronary artery aneurysms that often will result in an acute heart attack (myocardial infarction) from months to years after the diagnosis.

The clinical features of Kawasaki disease can be mistaken for other illnesses such as streptococcal or staphylococcal infections (scarlet fever, toxic shock syndrome), viral infections (measles, adenovirus), and drug reactions (Stevens-Johnson syndrome).

In addition, some patients, especially younger or older patients, may develop incomplete Kawasaki disease or atypical Kawasaki disease in which the child may not have all four clinical features. Diagnosis in these situations is much more difficult.

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