Kawasaki disease

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Abstract
Kawasaki disease is still a dilemma for pediatricians, especially its atypical or incomplete presentation. The current diagnostic criteria appear inadequate, and the early diagnosis frequently remains challenging with high risk of coronary damage. This review focuses on recent data concerning Kawasaki disease etiology and pathogenesis, and emphasizes the proposal of including altered laboratory values in revised diagnostic criteria aimed at identifying patients with incomplete disease. Despite a timely treatment, approximately 15% of children have persistent or recurrent fever, thus requiring additional intravenous immunoglobulin, corticosteroids, immunosuppressant, and eventually biologic drugs. Recent cardiologic tests strongly suggest that endothelial damage may lead to coronary stenosis and myocardial ischemia over time, even in children apparently normal at initial cardiac evaluation. New noninvasive tools are suggested to monitor coronary artery aneurysm outcome. Giant coronary artery aneurysms still represent a severe life-threatening complication. There is as yet no agreement regarding the prevention of thrombosis and stenosis in children with giant aneurysms. Due to earlier recognition, aggressive medical treatment, and surgical procedures, the quality of life in patients with Kawasaki disease is significantly improved. The identification of children at high risk for coronary artery aneurysms is crucial in order to reduce the occurrence of sudden death in adolescence and early adulthood. High parameters of inflammation, anemia, and low sodium and albumin levels, along with persistent unexplained fever, should alert clinicians to suspect the disease even though all clinical symptoms are lacking.