[Kawasaki syndrome]

Abstract

Kawasaki syndrome was first described in 1967 in Japan. It is due to a generalized vasculitis of probably infectious etiology. Diagnosis is based exclusively on clinical criteria. Both morbidity and mortality are mainly determined by coronary artery involvement. The principal purpose of the present analysis of the 32 patients treated for Kawasaki syndrome at the University Children's Hospital in Berne (22 males = 69%, 11 females = 31%; mean age +/- SD = 2.7 +/- 2 years) was to describe the clinical and laboratory presentation and to evaluate high-risk factors and therapeutic efficacy with regard to course of the disease and cardiac involvement. Treatment has included aspirin and, since 1986, intravenous immunoglobulins (IVIG) as well. Routine electrocardiographic and echocardiographic checks detected 10 patients (31%) with cardiovascular complications, all of whom remained clinically asymptomatic: 9 with carditis (28%) and 3 with coronary artery aneurysms (CAA) (9%). The 3 patients with CAA were boys aged 18-25 months who presented an extremely long duration of fever (12-30 days) and markedly elevated ESR (100-128 mm/h). Since the therapeutic use of high-dose IVIG (9 patients), no CAA has been observed, and carditis in only 2 patients (22%). All patients are clinically cured. The carditis findings and 2 of the 3 CAA completely resolved; one patient has persistent CAA and myopathic cardiomegaly after more than 3 1/2 years. This study, together with the review of the literature, should enable the physician to diagnose and correctly manage the childhood disease Kawasaki syndrome.