J Korean Pediatr Soc.  1994 Jul;37(7):890-899.

Unusual Presentation of Kawasaki Disease Complicated by Coronary Aneurysms


Patients with atypical or incomplete Kawasaki disease are at same risk for development of coronary artery complications as typical Kawasaki disease. In this communication we report six patients with unusual presentation of Kawasaki disease complicated by coronary artery aneurysms, in whom correct diagnosis were not made in time for proper treatment. One of these patients died from massive myocardial ischemia due to giant aneurysms along the entire coronary artery system. These patients had either less than enough number of diagnostic criteria at initial presentation or diagnostic signs which occurred over an extended period of time, resulting in difficulty in diagnosis during the acute phase. As a result, none of these patients received intravenous gamma globulin treatment. Thus strict adherence to currently accepted criteria for diagnosis of Kawasaki disease may lead to failure to recognize atypical form of this illness with potential sequelae of myocardial ischemia or sudden death. We would like to emphasize from this experience that clinicians must be aware of the wide variations in clinical presentation of Kawasaki disease and take an aggressive approach in making correct diagnosis by obtaining early cardiac evaluation in order to initiate prompt treatment with intravenous gamma globulin.

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