BackgroundThe long-term outcomes of Kawasaki disease (KD) are unknown.MethodsFifty-two collaborating hospitals collected data on all patients who had received a new definite diagnosis of KD between July 1982 and December 1992. Patients were followed until December 31, 2009 or death. Standardized mortality ratios (SMRs) were calculated based on Japanese vital statistics data.ResultsOf the 6576 patients enrolled, 46 (35 males and 11 females) died (SMR: 1.00; 95% CI: 0.73–1.34). Among persons without cardiac sequelae, SMRs were not high after the acute phase of KD (SMR: 0.65; 95% CI: 0.41–0.96). Among persons with cardiac sequelae, 13 males and 1 female died during the observation period (SMR: 1.86; 95% CI: 1.02–3.13).ConclusionsIn this cohort, the mortality rate among Japanese with cardiac sequelae due to KD was significantly higher than that of the general population. In contrast, the rates for males and females without sequelae were not elevated.

By May 1996, 43 cases of Creutzfeldt-Jakob disease with cadaveric dura transplantation were reported during a nationwide survey in Japan. With the assumption that the number of patients receiving the transplantation each year was 20,000, the incidence of the disease among patients receiving cadaveric dura grafts was compared with the annual incidence in the general population. Cumulative incidence was between 0.017 and 0.048%. The estimated relative maximum risk was 104.9, and even the minimum relative risk was as high as 29.2.

BackgroundThe long-term prognosis of those having a history of Kawasaki disease (KD) is still unknown. Methods and Results Between July 1982 and December 1992, 52 collaborating hospitals collected data on all patients having a new definite diagnosis of KD. Patients were followed-up until December 31, 2004 or their death. Standardized mortality ratios (SMRs) were calculated based on the Japanese vital statistics data. Of 6,576 patients enrolled, 36 (27 males, 9 females) died and the SMR was 1.14. Despite the high SMRs during the acute phase, the mortality rate was not high after the acute phase for the entire group of patients. Although the SMR after the acute phase was 0.71 for those without cardiac sequelae, 10 males (but none of the females) with cardiac sequelae died during the observation period; and the SMR for the male group with cardiac sequelae was 2.55 (95% confidence interval: 1.23-4.70). Conclusions The mortality rate among males with cardiac sequelae because of KD was significantly higher in this cohort than in the general population. On the other hand, those for females with the sequelae and for both males and females without sequelae were not elevated. (Circ J 2008; 72: 134 -138)

Although it was not statistically significant, the mortality rate among males with cardiac sequelae due to Kawasaki disease appeared to be higher than in the general population. On the other hand, the mortality rates for females with the sequelae and both males and females without sequelae were not elevated.