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| AuthorMasato Takahashi, MD | Section EditorsDavid R Fulton, MDBernard J Gersh, MB, ChB, DPhil, FRCP | Deputy EditorMelanie S Kim, MD |
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Kawasaki disease (KD) is a vasculitis of infancy and early childhood. It was first described by Dr Tomisaku Kawasaki in 1967, and initially designated as the mucocutaneous lymph node syndrome [1]. KD is an immune-mediated vasculitis with self-limited clinical course and unknown etiology.
Fever and manifestations of acute inflammation last for an average of 12 days without therapy. However, the disease may be complicated by the development of aneurysms in coronary arteries, which can lead to myocardial ischemia or infarction (MI) (movie 1 and movie 2 and movie 3). In untreated patients, the incidence of coronary artery aneurysms is about 20 to 25 percent with a mortality rate of approximately 2 percent.
Early detection of the disease and prompt initiation of therapy with aspirin and intravenous immune globulin (IVIG) have reduced the death rate to well below 1 percent and the prevalence of coronary artery aneurysms to approximately 5 percent.
An overview of the clinical features, natural history, treatment and follow-up of cardiovascular sequelae of Kawasaki disease will be presented here. The etiology, epidemiology, clinical manifestations, diagnosis, and treatment of Kawasaki disease are discussed separately. (See "Epidemiology and etiology of Kawasaki disease" and "Clinical manifestations and diagnosis of Kawasaki disease" and "Initial treatment and prognosis of Kawasaki disease".)
CARDIAC MANIFESTATIONS IN THE ACUTE PHASE
After a brief nonspecific prodrome of respiratory or gastrointestinal symptoms, the patient with KD typically experiences a combination of the following signs and symptoms.
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