[Kawasaki disease in children--9 years experience]

Abstract

Purpose: The aim of the study was assessment of the course and treatment of Kawasaki disease on the basis of own experience.

Material and methods: Between November 1995 and December 2004 Kawasaki disease (KD) was diagnosed in 30 patients, (20 boys, 10 girls). The median age in acute stage was 20 months (range 1-96 months). Patients were divided into 4 groups (gr) according to changes in coronary arteries (CA). Group 1 (N=13) without CA aneurysms (CAA), group II (N=5) with small (<5mm) CAA. Group III (N=5) with medium (5-8mm) CAA, group IV (N=7) with giant (>8mm) CAA. Echocardiographic examination was performed in all patients. In echocardiographic examination the following were observed: CAA and valvar insufficiency in 15 patients, left ventricle enlargement in 3 patients, pericardial effusion in 6 patients. The treatment was intravenous immunoglobulin and aspirin in all patients, steroid treatment in 2 patients methylprednisolone, in 2 patients prednisone, in l patient hydrocortisone. The anti-inflammatory treatment was started after 10 days of illness in 66.7% of patients. Thrombi in CAA were treated in 6 patients, the treatment was: in all tissue - type plasminogen activator iv and intracoronary in 2 patients; heparin in all; enoxaparine in 3 patients; abciximab in 2 patients; acenocumarol and aspirin in all. Coronary angiographies were performed in 2 patients in acute stage of disease for thrombolytic therapy and in 4 patients during follow-up. Myocardial perfusion was assessed in SPECT (Single Photon Emission Computed Tomography) in 6 patients. Two children with giant CAA died during the first 8 months of acute stage (6.7%), 2 patients we lost from follow-up. 26 patients are in follow-up.

Results: during anti-inflammatory treatment (immunoglobulin, aspirin, steroid treatment) loss of fever, normalization of inflammatory markers, and no side effect, were observed except for 1 patient treated with methylprednosolone with thrombi in CAA during treatment and no side effects during treatment. During the follow-up period CAA regressed in 5 patients (35.7%), CAA became smaller in 7 patients (50%), CAA were the same in 2 patients (14.3%). Changes in echocardiographic examination: valvar insufficiency, left ventricle enlargement, pericardial effusion, disappeared during follow-up. Perfusion defects were observed in 4 patients in SPECT. In coronary angiography changes were observed in coronary arteries in 4 patients.

Conclusions: 1. Kawasaki disease was diagnosed in 66.7% children after 10 days of illness. 2. Coronary artery aneurysms were found in 56.6% treated children. 3. Regression of CAA was observed in 37.5% patients mainly with small CAA. They became smaller in 50% cases mainly with medium and giant CAA. 4. Mortality rate was 6.7% and concerned children with giant CAA; they died during the first 8 months of illness. 5. Thrombolytic treatment of thrombi in CAA (rt-PA, heparin, enoxaparine, abciximab, acenocumarol, aspirin) was safe and successful. 6. In all patients with medium and giant CAA in the acute stage, changes were observed in echocardiographic, angiographic examination and myocardial perfusion during follow-up.