Factors related to cardiac sequelae of Kawasaki disease

Among the 35,210 patients with Kawasaki disease who were reported in nationwide surveys from 1991 to 1996, 83% were treated with gamma-globulin (GG). Those treated with a total dose of 1000 mg/kg (9098 patients) and 2000 mg/kg (7012 patients) were selected as the subjects of the study on the relationship between the development of cardiac sequelae, in particular of giant aneurysms, and related factors using logistic regression models. Among the two groups that received 1000 mg/kg and 2000 mg/kg, respectively, the odds ratios for cardiac sequelae were significantly high for males, those <6 months and ≧ 7 years of age, and typical or recurrent cases. Except for those <6 months of age, the odds ratios of all the factors mentioned above were higher for the incidence of giant coronary aneurysms than those for cardiac sequelae. The preventive effects of GG therapy for cardiac sequelae and in particular giant aneurysms, were significantly low when GG administration was initiated on day 8 or later after onset. In the group treated with 1000 mg/kg, the preventive effect was low when GG administration was spread over 3 days or longer. Conclusion To prevent the development of cardiac sequelae, in particular giant coronary aneurysms, gamma-globulin therapy should be started as soon as possible and be completed within 2 days. Received: 8 July 1998 / Accepted: 18 December 1998     

Factors relating to the cardiac sequelae of Kawasaki disease one month after initial onset

This study aimed to determine the risk factors related to the presence of cardiac sequelae 1 mo after initial onset and to examine the preventive effect of the early administration of high-dose gamma-globulin (GG) on cardiac sequelae in patients with Kawasaki disease. Patients treated with high-dose GG of 2000 +/- 100 mg kg(-1) were selected as subjects from the 15th nation-wide survey in Japan. Univariate and logistic multiple variable analyses were used to test the effects of background variables such as age and gender, variables relating to laboratory findings such as the percentage of neutrophil leucocytes, and variables relating to the GG treatment on the presence of cardiac sequelae. The odds ratios were significantly higher for males (1.48), those younger than 1 y of age (1.71), recurrent cases (2.42), and those with a low haematocrit (<32.5%) (1.45) and high percentage of neutrophil leucocytes (>68%) (1.63). The odds ratio was low for those who started GG administration in less than 6 d from onset between the patients with and without cardiac sequelae. The odds ratio for the duration of GG treatment was not significantly different between those with and without cardiac sequelae. Conclusion: Patients who received early administration of GG, less than 6 d from onset of the disease, had a lower risk than those received GG more than 6 d from the onset. The percentage of neutrophil leucocytes and the haematocrit level are useful indicators in predicting the development of cardiac sequelae.     

Pelviureteric junction obstruction as sequelae of Kawasaki disease

We present a 7-year-old boy who was admitted with a history of cough for a week, neck pain with associated swelling for 4 days, fever, and vague periumbilical pain. He was diagnosed with Kawasaki disease, and subsequently developed vasculitis of the ureter and stricture of the ureteric lumen at the level of the pelviureteric junction.     

Coronary arteriovenous fistulae mimicking cardiovascular sequelae of Kawasaki disease

Summary A case of coronary artery dilatation in childhood, initially attributed to Kawasaki disease, was determined by careful serial echocardiography to be the result of congenital coronary arteriovenous fistulae.     

《川崎病心血管后遗症的诊断和管理指南(JCS/JSCS2020)》解读

川崎病是导致儿童后天性心脏病的主要原因.川崎病并发冠状动脉损害及巨大冠状动脉瘤等心血管后遗症给儿童的身心健康造成很大影响.日本循环学会(JCS)联合日本心脏外科学会(JSCS)2020年7月共同发布了《川崎病心血管后遗症的诊断和管理指南(JCS/JSCS 2020)》,系统介绍了川崎病心血管后遗症的诊疗和管理新进展.该...     

Leukocyte counts in patients with Kawasaki disease: from the results of nationwide surveys of Kawasaki disease in Japan

In the latest survey of Kawasaki disease in Japan, questionnaires on maximal leukocyte counts of the patients were included to clarify whether leukocyte counts could be of value for the diagnosis and prediction of outcome. A questionnaire form and diagnostic guidelines for Kawasaki disease were sent to all pediatric departments of hospitals with ≥100 beds throughout Japan, and information including maximal leukocyte counts was obtained on patients with Kawasaki disease diagnosed during the 2-y period from January 1993 to December 1994. Of the 11 458 patients diagnosed during the 2-y period, maximal leukocyte counts were reported in 11 062 patients (96.5%). The mean value and the distribution of maximal leukocyte counts were lower in the age group under 1 y. The mean values and the distribution of leukocyte counts were lowest in suspected cases among three diagnostic categories: typical cases of Kawasaki disease, atypical cases, and suspected cases. The mean values of maximal leukocyte counts of the patients with cardiac sequelae were significantly higher than those without cardiac sequelae in each age group. The proportion of patients with cardiac sequelae increased with leukocyte counts in each age group. The Receiver/Response Operating Characteristic (ROC) curve for maximal leukocyte counts in Kawasaki disease revealed that the accuracy of maximal leukocyte counts for prediction of cardiac sequelae was highest in the age group <6 months, and the most accurate cut-off point was 16 × 10⁹/l. The strongest association between higher leukocyte counts (≥16 × 10⁹/l) and cardiac sequelae was observed in the age group <6M. A large-scale analysis of leukocyte counts in patients with Kawasaki disease revealed age-dependent relationship between maximal leukocyte counts, diagnostic categories and outcome. Maximal leukocyte counts may be helpful for the prediction of outcome with the consideration of age.     

Incidence rate of recurrent Kawasaki disease in Japan

To calculate the incidence of recurrent Kawasaki disease and to discuss some potential risk factors for its recurrence, we observed a cohort consisting of those followed-up since the first episode of the disease. A total of 4560 persons, with 16 500.4 person-years were observed from the second month after the first episode of the disease to the end of 1989. The mean observation period was 3.62 years. The overall incidence rate was 5.21 per 1000 person-years, with a higher incidence within the 2 years from the first episode; although not statistically significant, the incidence was higher among males and those who experienced the first episode at ± 2 years of age. The possibility of i.v. gamma globulin therapy being one of the risk factors was negated by a stratified analysis to control confounding factors but supported by univariate analysis.     

Serum Sodium Levels in Patients with Kawasaki Disease

The purpose of this study was to assess the hypothesis that lower serum sodium levels are associated with cardiovascular sequelae in patients with Kawasaki disease (KD). We used the database of the 16th nationwide survey of KD in Japan. We investigated the distribution of serum sodium levels and the relationship between serum sodium levels and cardiovascular sequelae. Of the reported cases, serum sodium levels were reported in 13,569 patients (89%). The proportion of patients with serum sodium levels 130 mEq/L or less, was greater in complete cases than in incomplete cases. The proportion of patients with serum sodium levels 130 mEq/L or less was increased with age. The largest proportion of patients with serum sodium levels 130 mEq/L or less was found in the category of 3–5 days since onset of illness. A serum sodium of level 135 mEq/L or less was an independent risk factor for cardiovascular sequelae (odds ratio, 1.79, 95% confidence interval, 1.42–2.26). Among patients with KD, there are significant differences in serum sodium levels between diagnostic categories, age, and days since the onset of illness. The sodium level may be a simple predictor of cardiovascular sequelae.     

Cardiac sequelae of Kawasaki disease in Japan: statistical analysis.

The proportions of Kawasaki disease patients with cardiac sequelae in Japan were analyzed using nationwide survey data from the 6 1/2-year period July 1982 through December 1988. Of 46,864 cases of Kawasaki disease reported in the surveys, 7637 or 16.3% had cardiac sequelae such as dilation or stenosis of coronary arteries, myocardial infarction, and valvar lesions 1 month or more after onset. The prevalence of cardiac sequelae was particularly high in males, infants younger than 1 year, and children older than 5 years of age. In sequential observation, there was no correlation between the prevalence of cardiac sequelae and periods of high or low incidence of the disease. The prevalence of cardiac sequelae overall declined steadily over the observation period, perhaps as a consequence of increasing use of intravenous gamma globulin. However, children older than the age of 5 years manifested increasing prevalence of cardiac sequelae over the observation period, probably as a result of lower rates of intravenous gamma globulin administration.     

Kawasaki disease with severe cardiac sequelae: lessons from recent New Zealand experience

OBJECTIVES: To review recent cases of Kawasaki disease (KD) with significant cardiac sequelae in New Zealand. It is known that intravenous immunoglobulin (IVIG) reduces the risk of coronary artery aneurysm formation if given within 8-10 days of onset of KD. METHODS: Retrospective review of medical course, criteria for KD, laboratory and cardiac findings for six children identified with KD and significant coronary artery sequelae. RESULTS: There was delay in diagnosis of KD in three of the six children. Three cases were atypical by extremes of age (2 months, 10 years, 14 years). By definition all six children had significant coronary artery involvement. One patient had a thrombus detected in a coronary aneurysm 3 weeks after KD. One patient underwent coronary artery bypass grafting for unstable angina 2 years after KD. One patient developed coronary artery aneurysms after an initial 'toxic shock' type illness evolving to KD. Three patients died, one due to rupture of a coronary artery aneurysm, two from rapid early coronary artery obstruction occurring at three and 4 months after initial KD. CONCLUSIONS: Kawasaki disease remains an important cause of mortality and morbidity for children. Diagnostic delay beyond 8 days reduces the chances of successful IVIG therapy in KD. Current studies supported by the Paediatric Surveillance Unit should establish the epidemiology of KD in New Zealand.     

Advances in Kawasaki disease

Recent studies have increased our understanding of the etiopathogenesis of Kawasaki disease (KD). The inflammatory infiltrate in KD coronary artery aneurysms has been shown to consist of CD8 T lymphocytes, macrophages, and IgA plasma cells, consistent with an immune response to an intracellular pathogen with a mucosal portal of entry. The identification of an oligoclonal IgA response in the vascular wall and the detection of a KD-associated antigen in inflamed KD tissues using a synthetic antibody derived from KD oligoclonal IgA antibodies have provided new approaches to identification of the etiologic agent. Highly effective therapy has evolved for KD, even in the absence of identification of the etiologic agent. The existence of incomplete KD cases remains a significant diagnostic dilemma for the clinician. Conclusion: the development of a diagnostic test, more specific therapy, and ultimate prevention of this potentially fatal illness of childhood are dependent upon continued advances in determining the etiopathogenesis of this fascinating disorder.Abbreviations KD Kawasaki disease - IVIG intravenous immunoglobulin     

5岁以上年长儿川崎病临床特征分析

目的 总结5岁以上儿童川崎病（KD）的临床及实验室特征。方法 将确诊为KD或不完全KD的250例年长儿分为 > 5~9岁（191例）及 > 9岁亚组（59例）,与266例1~5岁KD患儿进行回顾性分析比较,比较3组间的临床及实验室特点。结果 3组间手足肿胀、指端或肛周脱屑的发生率均以 > 9岁组最低,而颈淋巴结肿大的发生率以 > 5~9岁组最高,总热程以 > 9岁组最长（P < 0.05）。3组间皮疹、球结膜充血、口唇改变的发生率及不完全KD所占比例、静脉丙种球蛋白（IVIG）无反应发生率的差异无统计学意义（P > 0.05）。PLT、白蛋白水平以 > 9岁组最低,中性粒细胞百分比以 > 5~9岁组最高（P < 0.05）。3组间WBC总数及CRP、ESR、ALT的差异无统计学意义（P > 0.05）。轻度冠脉扩张以1~5岁组最多,中型冠状动脉瘤的发生以 > 9岁组最多（P < 0.05）;3组间巨大冠脉瘤发生率的差异无统计学意义（P > 0.05）。结论 5岁以上年长儿KD临床表现不典型,但颈部淋巴结肿大发生率和中性粒细胞百分比相对较高,PLT及白蛋白水平偏低。年龄越大发生中型冠状动脉瘤的风险越大。     

Late-Appearing Brachiocephalic Aneurysm: An Atypical Vascular Sequella of Kawasaki Disease

Kawasaki disease (KD) is an acute vasculitis that can result in cardiovascular sequelae. One of the most serious consequences of KD is the development of arterial aneurysms that can lead to thrombosis and ischemia. Commonly, these lesions, occurring early in the course of KD, are found in the coronary arteries. However, this location is not exclusive. This report presents a case of KD with atypical findings of a late-appearing brachiocephalic artery aneurysm. The authors suggest that for patients with complicated KD, surveillance should include a careful vascular survey to discover aneurysms distant from the heart.     

Hospital facilities available to patients with Kawasaki disease: Results of a national survey of Kawasaki disease in Japan

Since 1970, thirteen nationwide epidemiologic surveys of Kawasaki disease have been conducted, and questions on the current situation of hospitals and their facilities have been included in the survey form to assess the care available to patients with Kawasaki disease. To clarify the characteristics of medical care for Kawasaki disease, this paper summarizes the present condition of hospital facilities available to patients with Kawasaki disease. A questionnaire form including questions about the situation of hospital facilities was sent to all pediatric departments of hospitals with 100 or more beds throughout Japan. The proportion of hospitals in which successive observations with echocardiography (UCG) is available (UCG available hospital) was significantly increased compared with the ninth survey (1985–86), whereas that of the hospitals in which coronary angiography (CAG) is available (CAG available hospital) was significantly decreased. Almost all (96.2%) of the patients were reported from the UCG available hospitals. The proportion of patients reported from hospitals with a set policy for intravenous gamma globulin (IVGG) therapy was 74.5%. The proportion of UCG available and CAG available hospitals and that of hospitals with a set policy for IVGG therapy correlated with the increase in the number of patients. In conclusion, hospital facilities available to patients with Kawasaki disease in Japan have satisfactory capability for diagnosis and management of Kawasaki disease.     

Kawasaki disease in india

Kawasaki disease was first described 30 years ago. The authors report 5 children with Kawasaki disease who presented within 18-month-period and describe their clinical presentation and management.     

Use of Intravenous γ-Globulin for Kawasaki Disease: Effects on Cardiac Sequelae

The administration of intravenous γ-globulin (IVGG) for Kawasaki disease was investigated throughout Japan in 1993 by obtaining information from pediatric departments in 2652 hospitals that had more than 100 beds. Of 11,221 reported patients, 8958 patients (79.8%) received IVGG treatment. Of all the patients to whom IVGG was administered, the most common total dose was 1000 mg/kg (36.3%) followed by 2000 mg/kg (16.9%) and 1200 mg/kg (16.8%). The treatment was started in 53.8% by day 5 of the illness and in 83.7% by day 7. The proportion of those with cardiac sequelae was higher among patients administered >2000 mg/kg or in those started on IVGG on day 9 of their illness or later. The possible reasons are (1) those who were more severely affected were treated with high-dose IVGG earlier; or (2) IVGG does not effectively prevent cardiac sequelae. We concluded that there is a risk of unfavorable effects with IVGG regarding cardiac sequelae when the IVGG dose is >2000 mg/kg or if IVGG is started on day 9 or later. We believe that only a randomized controlled trial, undertaken prospectively, can adequately address the question of the optimal use of IVGG.