Mortality Among Japanese With a History of Kawasaki Disease: Results at the End of 2009

Nakamura, Yosikazu; Aso, Eiko; Yashiro, Mayumi; Tsuboi, Satoshi; Kojo, Takao; Aoyama, Yasuko; Kotani, Kazuhiko; Uehara, Ritei; Yanagawa, Hiroshi

doi:10.2188/jea.je20130048

Cited by 52 publications

(33 citation statements)

References 29 publications

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“…51 A more recent study from Japan showed that the SMR beyond the acute illness was elevated for all patients with cardiac sequelae (SMR, 1.86; 95% confidence interval, 1.02-3.13), thus, stressing the importance of long-term surveillance for this subgroup of patients. 52 Patients without cardiac sequelae after the acute phase had a lower mortality relative to the general population (SMR, 0.65; 95% confidence interval, 0.41-0.96). In the continental United States, using administrative data that could include readmissions for coronary disease, the in-hospital mortality rate is ≈0.17%.…”

Section: Applying Classification Of Recommendations and Level Of Evmentioning

confidence: 95%

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

McCrindle¹,

Rowley²,

Newburger³

et al. 2017

Circulation

2,863

4,124

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BACKGROUND:Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. METHODS AND RESULTS:To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and longterm outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. CONCLUSIONS:These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances. K awasaki disease (KD) is an acute, self-limited febrile illness of unknown cause that predominantly affects children <5 years of age. When initially described, the potential for coronary artery complications was not appreciated. KD is now the most common cause of acquired heart disease in children in developed countries. In the absence of pathognomonic tests, the diagnosis continues to rest on the identification of principal clinical findings and the exclusion of other clinically similar entities with known causes. Timely initiation of treatment with intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery aneurysms defined from absolute luminal dimensions from 25% to ≈4%. Ongoing studies with additional therapies have not substantially reduced this residual risk. The long-term prognosis is determined by the initial and current level of coronary artery involvement. Certain subsets of patients are at risk for myocardial ischemia from coronary artery thrombosis and stenoses. Medical management of such patients hinges on judicious use of thromboprophylaxis and vigilance to identify evolving stenoses. Invasive revascul...

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Section: Applying Classification Of Recommendations and Level Of Evmentioning

confidence: 95%

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

McCrindle¹,

Rowley²,

Newburger³

et al. 2017

Circulation

2,863

4,124

View full text Add to dashboard Cite

show abstract

“…The most frequent cause of death was KD-related cardiovascular sequelae (13 subjects, 0.2%) followed by malignancy (7 subjects, 0.1%), trauma (7 subjects, 0.1%), and suicide (7 subjects, 0.1%). There were 1,003 subjects with cardiac sequelae of KD in this cohort and seven (0.6%) died of suspected or confirmed cardiovascular causes over the 27-year period of the study [16]. Only the mortality rate among Japanese with cardiac sequelae due to KD was significantly higher than that of the general population.…”

Section: Discussionmentioning

confidence: 99%

“…CAA may remain silent after KD until adulthood when myocardial ischemia can lead to sudden death [6–15]. In children in whom the disease is diagnosed and treated, the death rate is less than one per hundred and autopsies are rare [16]. Although suspected KD cases have been reported in the forensic literature, there has been no systematic study to determine the incidence of such cases [8].…”

Section: Introductionmentioning

confidence: 99%

Cardiovascular pathology in 2 young adults with sudden, unexpected death due to coronary aneurysms from Kawasaki disease in childhood

Shimizu¹,

Sood²,

Lau³

et al. 2015

Cardiovascular Pathology

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Purpose Coronary artery aneurysms (CAA) may remain silent after Kawasaki disease (KD) until adulthood when myocardial ischemia can lead to sudden death. We postulated that there would be young adults with sudden, unexpected death due to CAA from KD who would have a state-mandated autopsy performed by the San Diego County Medical Examiner’s Office (SDCMEO). Methods We reviewed all autopsy cases <35 years of age from 1997–2012 at the SDCMEO with a cardiovascular cause of death (n=154). Results We found 2 cases meeting inclusion criteria. Case 1 was a 22-year-old Korean male with chronic ischemic changes due to a partially occluded and diffusely calcified 15mm aneurysm at the bifurcation of the left main coronary artery. Interview of the mother revealed that this patient had been diagnosed with KD complicated by giant aneurysms at age two years. Case 2 was a 30-year-old Hispanic male with myocardial infarction due to thrombosis of a calcified left anterior descending artery aneurysm. Histologic findings included diffuse myocardial fibrosis and a recanalized aneurysm in the right coronary artery. Interview of the family revealed a KD-compatible illness in childhood. Immunohistochemical staining showed expression of transforming growth factor β pathway molecules in the aneurysmal arterial wall. Conclusions In a medical examiner’s office serving a population of approximately 3 million people, 2 of 154 (1.3%) cardiovascular deaths in persons <35 years were attributed to cardiovascular complications of KD in childhood. Antecedent KD should be considered in the evaluation of all cases of sudden, unexpected death in young adults.

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“…Currently, data both supporting and not supporting the progression, exist . Follow‐up epidemiologic observation would provide meaningful data on the question . If KD vasculitis promotes the development of atherosclerosis, this has implications for circulatory disease in general, because so many people have the risk factor, as shown in this study.…”

Section: Discussionmentioning

confidence: 76%

Cumulative incidence of Kawasaki disease in Japan

Nakamura

Yashiro

Yamashita

et al. 2018

Pediatrics International

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Background: Although the incidence rates of Kawasaki disease (KD) in Japan have been determined in nationwide surveys, the cumulative incidence, that is, the proportion of those with a history of KD in the general population of 10-year-olds, is currently unknown. The aim of this study was therefore to assess the cumulative incidence of KD in Japan.Methods: Using the age-and sex-specific incidence rate of KD in Japan from the results of the nationwide surveys, incidence probabilities, that is, the age-specific number of KD patients divided by the population used in the vital statistics, and cumulative proportions of those not affected by KD up to the end of 9 years of age, were calculated. The cumulative incidence was then defined as 1 minus the cumulative proportion. The observed age classes were 0, 1, 2, 3, 4, and 5-9 years. All data were calculated by sex.Results: The cumulative incidence was 0.004833 for boys and 0.003474 for girls in 1991, but was 0.015284 and 0.012145 in 2014, respectively. According to these figures, 15.284 per 1,000 boys and 12.145 per 1,000 girls have been affected by KD by the age of 10 years. The birth-cohort cumulative incidences had similar trends.Conclusions: More than 10 persons in 1,000 have a history of KD at age 10 years in Japan.

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Mortality Among Japanese With a History of Kawasaki Disease: Results at the End of 2009

Cited by 52 publications

References 29 publications

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

Cardiovascular pathology in 2 young adults with sudden, unexpected death due to coronary aneurysms from Kawasaki disease in childhood

Cumulative incidence of Kawasaki disease in Japan

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