Comparative study on Kawasaki disease between Japan and North America

本项目的目的是调查日本和美国在川崎病的流行病学、病理生理学和治疗方面的比较评价。在流行病学方面，Yanagawa展示了日本全国调查的结果，特别是患者人口的转变、心脏后遗症、死亡率、复发率和兄弟姐妹病例的发生率。Shulman及其同事评估了IgA浆细胞在急性期血管组织中的病理生理作用。Newberger及其同事认为，超声心动图评估的冠状动脉尺寸可能被错误地归类为川崎病的正常。虽然日本厚生省的标准只是公布的明确界定该病冠状动脉异常的标准，但正常儿童的冠状动脉尺寸已被证明随着体表面积或体长等体型指标的增加而线性增加。他们引入了更多体表面积校正冠状动脉尺寸的数据。在治疗领域，Takahashi等人介绍了小剂量瓦法林加阿司匹林治疗并发巨大冠状动脉瘤的患者，这是导致心肌梗死的原因。如何治疗最初抵抗丙种球蛋白治疗的患者一直存在争议。加藤和他的同事证明了类固醇脉冲治疗在这类患者中的临床疗效。他们目前的结果显示，类固醇脉冲治疗和丙种球蛋白治疗的冠状动脉病变发生率没有差异，但类固醇脉冲治疗的医疗费用往往较低。这方面需要进一步的调查。其他治疗主题与冠状动脉受累及其长期预后有关。日本对导管介入作为一种新的治疗策略的重要性进行了全国性调查。Kato和他的同事报道，直接输注乙酰胆碱后，冠状动脉瘤消退部位出现内皮功能障碍。少

The objective of this project was to investigate of comparative evaluation between Japan and United States in terms of epidemiology, pathophysiology and treatment of Kawasaki disease. In the area of epidemiology, Yanagawa demonstrated the result of Japanese nationwide survey, especially the transition of patient's population, cardiac sequelae, mortality, recurrent rate and incidence of sibling case. Pathophysiological role of IgA plasma cells in vascular tissue during the acute stage was evaluated by Shulman and colleagues. Newberger and colleagues suggested that coronary arterial dimensions evaluated by echocardiography might be misclassified as normal in Kawasaki disease. Although the criteria of Japanese Ministry of Health is only published criteria the specifically define coronary artery abnormality in this disease, coronary artery dimensions in normal children have been shown to increase linearly with indices of body size, such as body surface area or body length. They introduced … More the data of body surfacarea corrected coronary dimensions. In the therapeutic area, Takahashi and colleagues introduced of low-dose wairfarin plus aspirin therapy in patients complicated with giant coronary aneurysm, which cause of myocardial infarction. Controversy has been persisted how to treat the patients who resisted initial gamma globulin treatment. Kato and colleagues demonstrated the clinical trail of steroid pulse therapy in such patients. Their current results shown the incidence of coronary artery lesion was not different between steroid pulse and gamma globulin therapy, however medical cost of steroid pulse therapy tend to be lower. Further investigation in this area should be required. Other topics of treatment were relatedof coronary artery involvements and its long-term outcome. Result of Japanese nationwide survey of catheter intervention was reported their importance as a new therapeutic strategies. Kato and colleagues reported that the presence of endothelial dysfunction at the site of regressed coronary aneurysm using direct infusion of aceylcholine. Less