Çocuklarda Kawasaki Hastalığına Güncel Yaklaşım
Synopsis
Kawasaki disease (KD) is an acute, self-limiting febrile systemic vasculitis of unknown etiology occurring primarily in children under 5 years of age, and is the most common cause of acquired heart disease in developed countries. In the pathogenesis of the disease, which has a higher incidence in Japan and among individuals of Asian descent, genetic susceptibility and an excessive immune response triggered by viral or environmental factors play a key role. Since there is no specific diagnostic test, diagnosis is based on clinical criteria; complete KD is diagnosed when prolonged fever lasting more than 5 days is accompanied by at least 4 of the 5 main criteria—conjunctivitis, maculopapular rash, cervical lymphadenopathy, extremity changes, and oral mucosal involvement—whereas incomplete KD is evaluated when criteria are missing. The most feared complication is coronary artery aneurysm and stenosis, which can occur in up to 25% of untreated patients. The mainstay of treatment is single-dose intravenous immunoglobulin (IVIG) at 2 g/kg administered between days 5 and 10 of fever, combined with acetylsalicylic acid (ASA). In IVIG-resistant cases, repeat IVIG, corticosteroids, or biological agents such as infliximab or anakinra are preferred, and thromboprophylaxis with clopidogrel, warfarin, or LMWH is administered based on coronary lesion size. Long-term prognosis depends on early intervention during the acute phase, management of coronary sequelae, and regular echocardiographic follow-up.
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