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To clarify whether patients with Kawasaki disease have a higher death rate than the age-matched healthy population after the disease occurrence, 6576 persons with a history of Kawasaki disease have been followed-up by the Research Committee. Between July 1982 and December 1992, 52 collaborating hospitals collected data on all patients having a new definite diagnosis of Kawasaki disease. Patients were followed until December 31, 1999, or death. The expected number of deaths was calculated from Japanese vital statistics data and compared with the observed number. Of 6576 patients enrolled, 27 (19 males and 8 females) died. The standardized mortality ratio (SMR) was 1.25. In spite of the high SMRs during acute phase, the mortality rate was not high after the acute phase for all patients. Although the SMR after the acute phase was 0.76 for those without cardiac sequelae, 6 males (no females) with cardiac sequelae died during this period and the SMR for the male group with cardiac sequelae was 2.35.Conclusion. The mortality rate among males with cardiac sequelae due to Kawasaki disease was 2.4 times as high as in general population, whereas mortality rates for females with the sequelae and both males and females without sequelae were not elevated.

Mortality among Persons with a History of Kawasaki Disease in Japan: The Fifth Look