[Kawasaki disease: new and important problems in cardiology].

Kawasaki disease or mucocutaneous lymph node syndrome (MCLS) is a recently recognized clinical entity in infants and young children with fever and characteristic mucocutaneous involvements accompanied by swelling of the cervical lymph nodes. It has aroused much interest because it may cause sudden death due to coronary arteritis with subsequent aneurysmal formation and thrombotic occlusion. Between January 1973 and September 1982, 611 patients with Kawasaki disease were evaluated with coronary angiography after the acute stage of illness. Of these patients, 136 (22%) were diagnosed as having coronary aneurysms, which were the most common abnormal finding at this stage. Serial two-dimensional echocardiography was useful to evaluate noninvasively the lesions of the coronary artery, and it was discovered that coronary aneurysms appeared in the eighth to 15th day of the illness, and some of them revealed an early restoration. Pericardial effusion appeared in 35% of the patients in the second to third week of the illness. Follow-up coronary angiography was performed in 72 cases who previously had coronary aneurysms five to 18 months after the acute illness. Thirty-nine cases showed completely normal findings at the second study, suggesting the regression of coronary aneurysms in this entity within one or two years after the onset of the illness. The remaining 33 patients showed abnormal findings such as stenotic or obstructed lesions, the irregular arterial wall and persistent aneurysms of coronary arteries at the follow-up study. Among the patients with abnormal angiographic findings myocardial infarction and mitral regurgitation were occasionally present. Three patients died suddenly from myocardial infarction at four months, four and six years after the onset of the illness, respectively. Early initiation of aspirin therapy (10-30 mg/kg) remains the mainstay to prevent thrombus formation and ischemic heart disease. The intracoronary thrombolysis by Urokinase was useful for prevention or treatment of acute myocardial infarction. Patients with Kawasaki disease are mostly in Japan, however, an increasing number of patients have recently been published in the foreign literatures, and this entity has become an important cause of heart disease in children. The long-term follow-up study and establishment of the effective treatment as well as elucidation of the etiology of this disease are essential.