The clinical course and findings of 171 children (97 boys and 74 girls) with Kawasaki disease admitted to our hospital during the 10-year period from March 1973 to July 1983 were studied.
1. Age and incidence
The age at onset ranged from three months to nine years. The incidence was the highest among one year-old Children (51/171 cases, 29.8 %), and 95 % (162/171 cases) of the subjects studied were less than five years old. The ratio of boys to girls was 1.3 to 1.0. The seasonal peak incidence was observed in the months from January to June, 1979 and from January to May, 1982.
2. Clinical studies
a. MCLS score : MCLS scores in 99 patients without coronary lesions ranged from 0 to 13 points. There were 83 cases with less than 5 pointr (83.8 %) and 16 with, more than 6 points (16.1 %). The scores in 28 subjects with coronary lesions ranged from 2 to 16 points. Among the 28 patients, there were five with less than 5 points (17.9 %) and 23 with more than 6 points (82.1 %).
b. Complications : The most important and serious complication is cardiovascular involvement, particularly coronary arteritis. Echocardiography performed in 67 patients demonstrated coronary arterial dilatations and aneurysms in 30.1 % at the acute stage. Of 171 cases, 17 (99.9 %) had neurological symptoms such as meningismus, meningitis, convulsion and abnormal EEG records, seven (4.1 %) arthritic symptoms, and four (2.3 %) enlargement of the gallbladder; thrombocytopenia, nephrotic syndrome and uveitis were seen one case each (0.58 %). The mortality rate was 1.1 % (2/171). Disorders of coronary arteries such as aneurysms, dilatations, infarctions, calcifications, axillary arterial aneurysms and iliac arterial aneurysms were found in 30 cases (17.5 %)
3. Studies on laboratory data
a. CRP and ESR : CRP and ESR showed strongly positive at the acute stage or at the relapse stage, but were normalized rapidly with Clinical improvement. In cases with coronary lesions, strongly positive CRP and ESR were sustained longer than in cases without coronary lesions.
b. Platelet counts : Mean values of the highest platelet counts in 85 cases without coronary lesions and in 22 cases with coronary lesions, were 73.3±23.8x10
4/mm
3 and 92±2.9x10
4/mm
3 respectively, and the mean durations from the onset of the disease to reaching the peak count were 16±6.5 days for the former and 20±7.7 days for later.
c. Serum CH50 and complement proteins : In 28 case without coronary lesions, the values of serum CH50 and complement proteins were elevated at the acute stage and at the relapse stage, but were normalized gradually with clinical improvement. klIn seven out of 14 cases with coronary lesions, the values of CH50 and complement proteins except for C9 and C3b INA dropped transiently from the second to the third week after onset. There after, these were normalized or even rose above normal ranges within about a week, at which time the coronary lesions were found by echocardiography.
d. Serum immunoglobulins and immune complexes : The values of serum IgG, IgA, IgM and IgE were elevated at the acute stage and diminished gradually with clinicalimprovement. The volues of serum immunoglobulins in patients with and without coronary lesions showed no significant differences. The elevated circulating immune complexes values of IgG, IgA, IgM and IgE at acute stage, measured by 3%-4% polyethylene glycol (PEG) precipitation and gel filtration, returned to normal ranges during convalescence in subjects without coronary lesions, but, in those with coronary lesions, the values that were not elevated during the early acute stage gradually increased and were sustained in relatively high ranges in the subsequent course. In an autopsy case, those values persisted in higher ranges until the terminal stage.
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