Incomplete and Atypical Kawasaki Disease: A Clinicopathologic Paradox at High Risk of Sudden and Unexpected Infant Death
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- Pucci, A., Martino, S., Tibaldi, M. et al. Pediatr Cardiol (2012) 33: 802. doi:10.1007/s00246-012-0186-y
Incomplete Kawasaki disease (IKD) and atypical Kawasaki disease (AKD) represent rare conditions. Two cases of unexpected or sudden infant death are reported. The diagnosis for a 3-month-old girl was determined by echocardiography, and the child unexpectedly died despite appropriate treatment, whereas autopsy determined the diagnosis of AKD for a 4-month-old boy. In both patients, giant coronary artery aneurysms with thrombosis and vasculitis, myocarditis, and coagulative necrosis were shown at autopsy. These rare forms of IKD and AKD carry a poor prognosis and represent a paradox between the severe cardiovascular damage and the clinical presentation that mimics common and usually self-limiting exanthematic infectious disease in infancy.