Epidemiologic analyses of risk factors affecting long-term prognosis of Kawasaki disease.

为了明确川崎病的长期预后，一个多中心项目组对所有患者进行了随访，甚至停止了就诊。项目小组由52家医院的儿科医生组成。对1982年7月至1992年12月期间到52家医院就诊的所有川崎病患者进行了随访，直到1997年底，如果在1997年12月之前死亡，则随访至死亡日期。一是检查是否通过户籍制度居住。在10年半期间因川崎病就诊的8417例患者中，排除疑似病例652例、复发病例384例、发病15天或15天后就诊的786例、外国人19例；共招募6576例患者进行观察。其中25人在观察结束时死亡。总观测时间为65,562人年，平均观测时间为9.7年。在整个观察期间，观察到的死亡人数高于男性的预期死亡人数，尽管没有统计学意义。标准化死亡率在急性期较高，特别是在男性中。急性期后标准化死亡率接近1.0。然而，对于心脏后遗症患者，特别是男性患者，急性期后的死亡率可能会升高。急性期死亡8例，除伤死1例外，其余均死于川崎病；1例是由于川崎病引起的脑病，6例是由于川崎病引起的心脏病变。急性期后死亡17例；缺血性心脏病3例，先天性心脏病4例，血液/免疫器官恶性肿瘤2例，其他病因8例。

To clarify the long-term prognosis of Kawasaki disease, a multi-center project group followed-up all patients even that stopped visiting medical institutions. The project team consisted of pediatricians of 52 hospitals. All patients who visited the 52 hospitals because of Kawasaki disease from July 1982 through December 1992 were followed-up till the end of 1997, or date of death if it happened before December 1997. First, we checked whether or not to live through resident registration systems. Of 8, 417 patients visiting the hospitals due to Kawasaki disease during the 10 and a half year, 652 suspected cases, 384 recurrent cases, 786 that visited the hospitals on or after 15 days of illness, and 19 foreigners were excluded ; 6, 576 patients were recruited the observation. Of these, 25 had died by the end of observation.The total population-time observed was 65,562 person-years, and the average observation time was 9.7 years. The number of observed deaths during the whole observation period was higher than the expected one for male, in spite of no statistical significance. The standardized mortality ratios were higher during the acute phase, in particular among males. On the other hand, the standardized mortality ratios were closed to 1.0 after the acute phase. However, for patients with cardiac sequelae, in particular male patients, the mortality rate after the acute phase might elevate. Eight patients died during the acute phase, all of whom but one patients dying of injury died of Kawasaki disease ; one was due to encephalopathy caused by Kawasaki disease, and six was due to cardiac lesions caused' by Kawasaki disease. After the acute phase, 17 patients died ; three of ischemic heart disease, 4 of congenital heart disease, 2 of malignant neoplasms in hematologic/immunologic organs, and 8 of other causes.