Abstract
In 1967, Dr. T. Kawasaki reported 50 cases of a previously undescribed infant disease. This was named acute febrile mucocutaneous lymph node syndrome, MCLS or MLNS, and was later called Kawasaki disease. The major symptoms of Kawasaki disease are summarized in Table 1. At the time of the first report by Kawasaki, this syndrome was thought to offer a good prognosis. However, since cases of sudden death from myocardial infarction were later reported, this syndrome is now considered to be sometimes serious. In this review, an outline of Kawasaki disease will first be given and then the prevention of thrombus formation in the disease will be discussed.
Incidence-Epidemiology: According to the investigations of a research group supported by the Ministry of Health of Japan, the patients have continously increased in number. The total numbers of cases with Kawasaki disease in Japan were 2, 798 in 1977 and 3, 489 in 1978 (Fig. 1). As regards the age distribution, there were peaks from 6 to 11 months old in males and from 12 to 17 months old in females respectively. Eighty percent of all cases were under 4 years old. The male: female ratio was 1.4-1.5: 1. The numbers of patients increased gradually from January to reach a peak in May or June, and then rapidly decreased in August or September.
Cases from Korea, Hawaii and Australia have also been described, as well as from Greece, West Germany and the mainland U. S. A. Replies submitted to a questionaire by Shibata, et al. in 1980 revealed that cases with Kawasaki disease had also been encountered in England, France, Denmark, Holland, Switzerland, Hungary, Kuwait and Canada.
Etiology: The precise etiology of Kawasaki disease remains unknown, although the possibility of infections such as by virus, Rickettia or Candida, or erythema exudativum multiforme induced by toxic effects of Streptococcus has been suspected. All investigations on pollutants such as compound cleansers, mercury, etc. have proved negative.
Pathology: The mortality rates of Kawasaki disease ranged from 1% to 1.7%. The morbid anatomy cases studied by Ohkawa, et al. consisted mostly of infants aged less than one year who died within 20 to 29 days after the onset of illness. Among them, cases with cardiac death amounted to 85%. The common findings in the morbid anatomy cases were arteritis, and thrombotic occlusions and aneurysma resulting from coronary arteritis.
Hamashima classified the course into four stages. At stage 1 (0-9 days), perivasculitis and vasculitis of capillaries, arterioles, venules and small arteries, associated with pericarditis, interstitial myocarditis and endocarditis, were seen. At stage II (12-25 days), panvasculitis and perivasculitis of larger arteries were prominent. Severe stenosis or obstruction of larger arteries due to th- or proliferative changes of the intima were also seen. Aneurysma appeared at this stage. At stage III (28-31 days), granulation of larger arteries was observed, but the vasculitis of the small arteries and arterioles disappeared. At stage IV (40 daysor more), severe stenosis or obstruction of larger arteries due to thickning of the intima was prominent. Scarring of larger arteries with calcification and recanalization was seen.
Although Kawasaki disease is clearly differentiated from classic periarteritis nodosa (Kussmaul-Maier type), it can be difficult to differentiate from some cases reported as infantile periarteritis nodosa (IPN). In this respect, it is possible that Kawasaki disease might represent IPN syndrome occurring through a certain, common etiology.
Laboratory findings, especially regarding coagulation: Leukocytosis, granulocytosis, str
