Mortality among Japanese with a history of Kawasaki disease: results at the end of 2009.

BACKGROUND: The long-term outcomes of Kawasaki disease (KD) are unknown. METHODS: Fifty-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. RESULTS: Of 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). CONCLUSIONS: In 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.

Mortality among persons with a history of kawasaki disease in Japan: mortality among males with cardiac sequelae is significantly higher than that of the general population.

BACKGROUND: The 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.

Mortality among persons with a history of Kawasaki disease in Japan: can paediatricians safely discontinue follow-up of children with a history of the disease but without cardiac sequelae?

AIM: To clarify the question of whether patients with Kawasaki disease suffer a higher mortality rate after the incidence of the disease in comparison with age-matched healthy individuals. METHODS: 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 up until 31 December 2001 or their death. The expected number of deaths was calculated from Japanese vital statistics data and compared with the observed number. RESULTS: Of 6576 patients enrolled, 29 (20 males and 9 females) died. The standardized mortality ratio (SMR: the observed number of deaths divided by the expected number of deaths based on the vital statistics in Japan) was 1.15 (95% CI: 0.77-1.66). In spite of 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 0.75 for those without cardiac sequelae, six males (but none of the females) with cardiac sequelae died during this period; and the SMR for the male group with cardiac sequelae was 1.95 (95% CI: 0.71-4.25). The mortality from congenital anomalies of the circulatory system was elevated, but no increase in cancer deaths was observed. CONCLUSION: 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.