Case-control study of giant coronary aneurysms due to Kawasaki disease

BACKGROUND: Epidemiologic features of the formation of giant coronary aneurysm due to Kawasaki disease and its risk factors are not still clear. METHODS: One hundred and five patients with giant coronary aneurysms were selected from the 15th and 16th nationwide surveys of Kawasaki disease in Japan. With all other patients reported from the same hospital as a control group, odds ratios (OR) were calculated for some potential risk factors. RESULTS: Some factors affecting the formation of giant coronary aneurysms were revealed. Of these factors, the following were considered to be useful for the treatment of patients with Kawasaki disease: male sex (OR compared with females = 1.99), 0 years old (OR compared with 1-2 years = 1.71), visiting the hospital on 1-3 days of illness (OR compared to 4-6 days = 1.72), leukocyte count (OR with 1000 /mm3 increase = 0.56), neutrophil concentration among leukocyte (OR with 10% increase = 1.11), hemoglobin concentration (OR with 1 mg/dL increase = 0.73), alanine aminotransferase level (ALT; OR with 10 IU/dL increase = 1.02) and serum sodium level (OR with 1 mEq/L increase = 0.80). CONCLUSIONS: The observation of 105 cases with giant coronary aneurysms due to Kawasaki disease reported in the nationwide survey provides information about some risk factors and other considerations about the aneurysms.     

Giant coronary aneurysms due to Kawasaki disease: A case−control study

BACKGROUND: Epidemiologic features of giant coronary aneurysm due to Kawasaki disease and its risk factors are still not clear. METHODS: Sixty-six patients with giant coronary aneurysms were reported to a 15th nationwide survey of Kawasaki disease in Japan. With all other patients treated in the same hospital as a control group, odds ratios were calculated for certain potential risk factors. RESULTS: Infant males aged less than 1 year,neutrophil concentration among leukocyte, late administration of intravenous gammaglobulin (IVGG) therapy and additional administration of IVGGwere considered as risk factors of giant coronary aneurysms due to Kawasaki disease. In univariate analysis, use of IVGG therapy and a large amount of IVGG (2500+ mg/kg)elevated the risk, whereas the relationship disappeared after the adjustment. CONCLUSIONS: The observation of 66 cases with giant coronary aneurysms due to Kawasaki disease reported to the nationwide survey provides some risk factors and consideration about the aneurysms.     

Case–control study of giant coronary aneurysms due to Kawasaki disease: The 19th nationwide survey

Background: The risk factors for recently reported cases of giant coronary aneurysms due to Kawasaki disease have not been elaborated. Methods: Fifty‐three patients with giant coronary aneurysms, diagnosed as Kawasaki disease in 2005 and 2006, were selected from the 19th nationwide survey of the disease in Japan. With all the other patients recorded at the same hospitals as a control group, OR and their 95%CI were calculated to delineate the risk factors. Results: In multivariate analyses, patients aged younger than 1 year (OR compared with 1–2‐year‐olds = 6.57) and those older than 5 years (OR compared with 1–2‐year‐olds = 4.24), those who received additional intravenous immunoglobulin (IVIG) without the use of steroid (OR = 8.38) and those who received steroid administration with or without the additional use of IVIG (OR = 220.51 and 83.83, respectively), showed significantly higher OR for giant coronary aneurysms. As for IVIG therapy, the additional use of IVIG (OR = 14.84), total dosage of IVIG exceeding 2500 mg/kg (OR compared with 1500–2499 mg/kg = 12.26) and the duration of IVIG administration for more than 3 days (OR = 30.12), were found to significantly increase the risk of developing giant aneurysms in univariate analyses that were adjusted for sex and age. Conclusions: The observation of 53 patients with giant coronary aneurysms due to Kawasaki disease among those included in the nationwide survey presented some risk factors, together with considerations about the associated aneurysms.     

Incidence of Kawasaki disease in Japan: the nationwide surveys of 1999–2002

BACKGROUND: The purpose of the present study was to describe the results of nationwide epidemiologic surveys of Kawasaki disease for the 4 year period 1999-2002. METHODS: The design is a retrospective incidence survey. The patients reported in these two surveys are all new patients who were reported during the two study periods (1999-2000 and 2001-2002), although the data were collected retrospectively. A questionnaire was sent to all pediatric departments of hospitals with 100 beds or more throughout Japan, requesting data on patients with Kawasaki disease, such as sex, age, date of first hospital visit, recurrence, and cardiac lesions. RESULTS: The total number of patients reported in the 4 year period 1999-2002 was 32 266 (18 604 male, 13 662 female), with an average annual incidence of 137.7 per 100 000 children younger than 5 years old. The male/female ratio was 1.30. The incidence peaked at 9-11 months of age, and the proportion of patients under 1 year of age was 26%. The monthly distribution had a high peak in January and a gradual increase in summer. Geographically, the high-incidence areas were limited to certain prefectures and moved from year to year. The cardiac lesions at acute stage and cardiac sequelae occurred more in children under 1 year and older than 4 years. Among the principal symptoms, fever persisting >or=5 days occurred most commonly, followed by conjunctival congestion, changes in lips and oral cavity, polymorphous exanthema, and changes of extremities. Cervical lymphadenopathy occurred less. CONCLUSION: More than 32 000 patients with Kawasaki disease during the 4 year period 1999-2002 were reported to the nationwide incidence surveys. The number of patients is steadily increasing despite the decrease of children. The seasonal variation, geographical distribution, and age-specific distribution support the infection theory for the etiology of Kawasaki disease.     

Six principal symptoms and coronary artery sequelae in Kawasaki disease

Background:  The relationship between coronary artery sequelae due to Kawasaki disease and the six principal symptoms associated with the latter are still unknown.
Methods:  Among the 16 952 patients reported in the 17th nationwide survey on Kawasaki disease, 14 068 were found to have definite and typical cases with five or six principal symptoms. The proportions of coronary artery aneurysms including dilatation of the artery were compared between the patient group with six symptoms and that with five.
Results:  Percentages for those with all six symptoms (7223) were 4.7% for coronary dilatations (i.e. small aneurysms), 1.7% for coronary aneurysms (i.e. middle-sized aneurysms), and 0.42% for giant coronary aneurysms (i.e. large aneurysms) 1 month after onset, whereas for those with five symptoms (6845), the corresponding percentages were 4.1%, 1.4%, and 0.22%, respectively. For overall observation, the odds ratios were 1.44, 1.28, and 1.70 for the three types of aneurysm, respectively. For male patients, all the odds ratios were >1.0, and some were significant. In contrast, odds ratios <1.0 were observed among those with six symptoms in some subgroups among female patients despite the lack of significance.
Conclusions:  Because odds ratios were elevated – especially for male patients with Kawasaki disease – with all six principal symptoms, patients with the six symptoms are more problematic for coronary sequelae among the definite cases, although there was not a specific symptom for the sequelae.     

川崎病合并冠状动脉瘤患儿应用华法林治疗的安全性

目的探讨川崎病合并冠状动脉瘤患儿应用华法林治疗的安全性和转归。方法选择2011至2020年湖南省人民医院收治的川崎病合并巨大冠状动脉瘤、1支冠状动脉血管内发生多个冠状动脉瘤或冠状动脉内发生血栓的患儿共21例进行前瞻性研究,应用华法林治疗,将国际标准化比值(INR)目标范围控制在2.0~3.0。在治疗开始时,治疗后1、2、3、4周及2、3、6、12个月,观察治疗前后冠状动脉瘤直径、数量、部位和冠状动脉内血栓的变化,并监测INR、心电图、肌酸激酶同工酶(CK-MB)、肌钙蛋白I。实施规范的华法林出血风险培训与管理,并根据家长实际落实情况,分为落实组和未落实组,比较2组患儿出血的发生情况。组间比较采用秩和检验或Fisher确切概率法。结果21例患儿男15例、女6例,发病年龄为2月龄至6岁。入组时评估冠状动脉病变为Ⅱ级有4例、Ⅲ级有7例、Ⅳ级有7例、Ⅴ级有3例。10例发生血栓的患儿临床发现血栓的时间为病程第4天至4个月。应用华法林治疗后,其剂量分布在0.06~0.10 mg/(kg·d),INR为1.80~2.59,10例有血栓的患儿有8例消失,2例未消失的Ⅴ级患儿的血栓有不同程度机化。治疗后,4例Ⅱ级病变患儿的冠状动脉均恢复正常;7例Ⅲ级病变患儿中有3例冠状动脉瘤消失,4例无改变;7例Ⅳ级病变患儿中5例冠状动脉瘤缩小为Ⅲ级,2例无改变;3例Ⅴ级病变患儿瘤体无改变。治疗后均没有新发血栓及新冠状动脉瘤出现。21例患儿治疗前后心电图无特殊改变,治疗前后肌钙蛋白I和CK-MB差异均无统计学意义[0.07(0~3.01)比0.04(0~0.29)μg/L,20.6(11.2~58.2)比29.0(16.7~47.0)U/L,Z=0.932、1.906,均P>0.05]。华法林出血风险管理落实组患儿出血发生率明显低于未落实组,差异有统计学意义(2/15比4/6,Fisher值=5.689,P=0.031)。结论应用INR 2.0~3.0为目标范围,结合川崎病并冠状动脉瘤的严重程度来调整华法林剂量,并予以规范而严格的管理与培训,可增加川崎病患儿华法林治疗的安全性,改善患儿的冠状动脉病变,治疗血栓及预防新血栓,降低出血风险。     

川崎病合并巨大冠状动脉瘤101例中长期随访

目的总结分析川崎病合并巨大冠状动脉瘤(GCAA)患儿的临床特点及中长期预后。方法回顾性队列研究。自首都医科大学附属北京儿童医院2004年建立的川崎病冠状动脉瘤随诊患儿队列中选择符合标准的101例GCAA患儿进行总结。GCAA诊断标准为冠状动脉瘤内径≥8.0 mm,患儿均完成定期随诊。随访终点为患儿最后一次随诊或死亡时间。组间比较采用t检验或χ²检验。结果101例符合纳入标准GCAA患儿入选研究对象,其中男82例(81.2%)、女19例(18.8%);发病年龄2.5(1.0,4.5)岁,随访时间4.5(2.7,7.5)年,最长19年。患儿常规接受阿司匹林、华法林治疗,严重者另加氯吡格雷。截至随访终点,13例(12.9%)患儿出现心脏增大,11例(10.9%)合并心功能不全,13例(12.9%)发生心肌梗死,2例(2.0%)接受冠状动脉搭桥术,6例(5.9%)死亡。受累冠状动脉共170支,位于左侧主干24支(14.1%),左回旋支10支(5.9%),左前降支57支(33.5%),右侧冠状动脉中段78支(45.9%),右侧冠状动脉远段1支(0.6%)。11例(10.9%)患儿GCAA内径回缩至4.0 mm及以下;受累的170支冠状动脉中,28支(16.5%)冠状动脉内径回缩到4.0 mm及以下。左侧、右侧GCAA回缩情况差异无统计学意义[18.7%(17/91)比13.9%(11/79),χ²=2.473,P=0.116]。单侧GCAA与双侧GCAA患儿回缩情况差异无统计学意义[16.1%(9/56)比4.4%(2/45),χ²=2.381,P=0.123]。结论川崎病GCAA以右冠状动脉中段、左冠状动脉前降支最常见,患儿心血管事件发生率及病死率较高,预后欠佳。     

Kawasaki Disease: A Pathology Survey in Western Europe

There is an increasing awareness that Kawasaki disease does occur outside Japan, but reliable data regarding the incidence of the disease in western Europe are lacking. Such information may be important, not only providing insight into the incidence but also indicating whether or not the pathology is comparable to that reported from Japan. For these reasons a survey has been initiated among pediatric pathologists in western Europe with the use of a questionnaire. The results show that the experience with Kawasaki disease of pediatric pathologists in western Europe is extremely limited. Only 25 cases have been reported. One may infer from these data that the incidence of Kawasaki disease in western Europe is far below that in Japan. The pathology encountered appears to be the same as that reported from Japan and the United States. This finding in particular may be important for further epidemiological studies regarding etiology and pathogenesis of Kawasaki disease.     

Repeated systematic surveillance of Kawasaki disease in Ontario from 1995 to 2006

Background: Rising incidences of Kawasaki disease (KD) have been reported worldwide. Reported herein are the results of 4 triennial KD surveillances conducted in Ontario. Methods: Between 1995 and 2006 all hospitals in Ontario were asked on 4 occasions to identify all patients with discharge diagnoses of KD and report incident cases. Results: The latest surveillance identified 697 new KD patients (100% response rate) for a total of 2378 KD patients through all 4 surveillances. Yearly incidence was 26.2/100 000 for <5 years old, 6.7/100 000 for 5–9 years old and 0.9/100 000 for 10–14 years old. KD incidence significantly increased from 1995 to 2006, although the increase seemed to plateau between the 3rd and 4th surveillance. There was an increase in the proportion of patients diagnosed with incomplete KD and a significant reduction in the rate of coronary artery abnormalities, possibly due to better disease recognition and treatment. Hospitals reporting <20 cases per surveillance were found to be more likely to report cases with incomplete KD. These patients were also less likely to be treated with i.v. immunoglobulin and aspirin but were more likely to be treated with antibiotics, suggesting uncertainties regarding diagnosis and management of KD patients in those centers. Conclusions: The incidence of KD in Ontario is possibly one of the highest outside of Asia and has been rising since 1995. Although the most recent surveillance demonstrated improved cardiac outcomes, treatment delays or absence thereof continue to be a problem. Effective diagnosis and prompt treatment remain critical aspects of KD management.     

Parents with a history of Kawasaki disease whose child also had the same disease

Background: The aim of this study was to describe time trends of prevalence and detailed characteristics of parents with a history of Kawasaki disease (KD) who had a child with the same disease using data from Japanese nationwide surveys on KD. Methods: Parents with a history of KD were identified using nationwide surveys of KD patients who visited hospitals between 1999 and 2008. To confirm KD histories, a parental questionnaire was sent to pediatricians who reported parental cases to the surveys for KD patients who visited hospitals from 2001 through 2004. Parental history was ascertained by merging data from the parental questionnaire with data from previous nationwide surveys, and detailed information about childhood KD was obtained from survey reports. Results: During the 10‐year study period, 407 parents with a child with KD were reported as having a KD history (0.43%). The prevalence of such parents significantly increased during this study period (regression coefficient: 0.13, 95% confidence interval: 0.05–0.20). Thirty‐one confirmed parents with the history were diagnosed between 1966 and 1984. All parents met the case definition of KD, and 33% had been treated with steroids. No parent with a KD history received intravenous immunoglobulin (IVIG). Conclusions: The prevalence of parents with KD history with a child with the same disease increased during the study period. Some of these parents had not been treated with IVIG because they were diagnosed before its use was widespread in Japan. An increase in the number of parents with the history is expected, thus continuous monitoring of familial cases with KD is required.     

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目的探讨川崎病(KD)冠脉瘤远期血管改变及其与冠脉粥样硬化的关系。方法选择首都医科大学附属北京儿童医院2008年6月至2009年6月病程大于1年且合并中型以上冠脉瘤的KD患儿31例为研究对象(KD冠脉瘤组),以CD34/CD133/KDR+为血管内皮祖细胞(EPC)表面分子标记,用流式细胞仪测定血周血EPC数量;采用酶联免疫法测定血清超敏C反应蛋白(hs-CRP);全自动生化分析仪测定血脂及载脂蛋白。选取年龄、性别匹配的21名健康体检儿童作对照(对照组)。结果 KD冠脉瘤组患儿病程1.0～12.5年,中位病程2.5年;中型冠脉瘤9例,巨大瘤22例,12例存在心肌缺血证据。KD冠脉瘤组血清hs-CRP为(2.77±1.06)mg/L,对照组为(1.60±1.53)mg/L,KD冠脉瘤组显著升高(P<0.01)。外周血EPC及血脂两组比较差异无统计学意义(P>0.05)。结论 KD冠脉瘤组恢复期血清hs-CRP显著升高,提示仍有慢性炎症反应,可能与KD成年后早发动脉粥样硬化有关。     

2000～2004年北京川崎病住院患儿流行病学调查

目的 进一步了解北京儿童川崎病（KD）的发病率及流行病学特点，并探讨其临床表现和急性期预后。方法 按照日本全国儿童KD调查及北京第1次KD流行病学调查方法，向北京及其郊区所有拥有儿科住院病床的医院发出调查问卷，回顾性记录自2000年1月～2004年12月的所有KD患儿。结果 北京45家有儿科住院病床的医院参加调查，共计报告1107例患儿。5年KD发病率为40．9～55、1／10万5岁以下儿童，平均49．4（95％CI，46、6～52．4）／10万5岁以下儿童。2000～2004年发病率有逐年增高趋势（Pα〈0．05）。发病年龄1个月～13．8岁，中位年龄2．0岁，其中5岁以下占87．4％；男女发病比例为1、83：1；春、夏有2次发病高峰，以夏季最高，2000和2004年在2次高峰基础上冬季还有1次发病高峰。复发15例，复发率1．4％；有家族史2例，占0．2％。发热仍是最常见的临床表现，而多形性皮疹为最少见的临床特征，患儿急性期无死亡。结论 2000～2004年北京KD发病率较前5年升高，但仍低于日本。发病年龄和性别比例与以往报道相似，但季节分布不同，除每年春、夏2次流行高峰外，2000和2004年冬季出现第3个发病高峰。     

川崎病85例

目的探讨川崎病（KD）患儿临床表现及并冠状动脉损害（CAL）的危险因素。方法对85例KD患儿进行临床分析。分析其年龄、性别、临床表现及心血管并发症的发生率，辅助检查等异常情况。对无CAL31例和并CAL54例患儿病程、实验室检查结果进行比较，分析CAL及冠脉瘤（CAA）发生的危险因素及治疗。结果1．男56例，女29例；年龄26个月～5岁者77例（90％）。KD并CAL发生率与WBC计数、血小板、血沉增高及血红蛋白、血清钠降低密切相关（P〈0．05）；与年龄、性别、发热天数均无相关性（P〉0．05）。除发热外，口唇潮红者占95％，皮疹占82％，球结膜充血占80％，颈部淋巴结大占75％，指趾端脱皮占70．5％，口唇及口腔改变占68％。2．心血管改变情况：并CAL54例（63．5％），3例形成CAA，在冠状动脉病变人群中占5．5％，占KD3．5％。左房左室扩大30例（35％），心包积液6例（7％），有心电图改变23例（27％）。病程5d以上确诊KD的患儿静脉注射人血丙种球蛋白（IVIG）和口服阿司匹林疗效均显著。结论5岁以下KD男童发病率高，早期大剂量应用IVIG对防治CAL有重要作用。