Kawasaki disease (KD), an acute inflammation of blood vessels, affecting at least 20 to 25 per 100,000 children under age 5 in this country (as well as infants and teens), can be fatal. If not detected and treated early with IV gamma globulin, it can lead to life-threatening coronary artery aneurysms, ischemic heart disease and myocardial infarction. To complicate matters, the cause of this particular vasculitis is unknown, there is no one diagnostic test, and its signs and symptoms, which mirror those of a number of infectious diseases – including measles and scarlet fever – can be misleading.
“The pediatricians, nurse practitioners and family practitioners on the front lines are the people who make the initial diagnosis,” says Hopkins Children’s cardiologist Candace Gibbin. “If they miss it, we’re in trouble because delayed or no treatment significantly increases the risk of coronary artery aneurysms.”
The classic criteria for Kawasaki disease include fever for at least five days plus four of the following: conjunctivitis without discharge, rash, redness of the mouth and lips, the appearance of so-called “strawberry tongue,” a swollen cervical lymph node, and swelling of the hands and feet with a reddish-purple discoloration of the palms and soles. But some children with Kawasaki disease have an atypical presentation and never fulfill these criteria, yet are still at risk of developing aneurysms. Four-year-old Ian Cardona of Westminster, Md., for example, had fever for five days, sore throat and conjunctivitis but not the characteristic redness on his tongue, hands and feet. Although Ian was still caught early, many atypical Kawasaki patients like him are being missed.
To help pediatricians improve detection, the American Heart Association has developed additional criteria. Among the guidelines, Kawasaki disease should be considered in all children with unexplained fever for more than five days and if they have two to three other features of KD. More importantly, infants age 6 months or younger who present with an unexplained fever for seven or more days must get an ESR erythrocyte sedimentation rate) and/or CRP (C-reactive protein) test to detect inflammation. If either test result is abnormal, the physician must order an echocardiogram to detect any damage to the heart or coronary arteries.
“This is critically important,” says Gibbin, “because children with the worst prognosis for aneurysms are the infants and adolescents.”
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Literature Check
“The Riddle of Kawasaki Disease,” New England Journal of Medicine, Feb. 15, 2007