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??Abstract?? Objective To analyze the characteristics and risk factors of coronary artery lesions ??CAL?? in Kawasaki disease ??KD?? based on the clinical grading. Methods The clinical records of 1253 KD patients admitted to Children’s Hospital of Fudan university from January 2000 to June 2011 were analyzed retrospectively. Results ??1??Coronary arterial lesions occurred in 304 cases with the incidence of 24.3% in KD patients. According to the clinical grading of CAL of KD?? among 304 CAL patients?? 32 cases ??10.5%?? were grade II??251 cases ??82.6%?? were grade ?? 13 cases ??4.3%?? were grade ????and 8 cases??2.6%?? were grade V?? with the prevalence in KD patients of 2.6%??20.0%??1.0%??and 0.64% respectively. ??2??In all CAL patients?? single?? double?? three and four coronary branch lesions accounted for 42.4%?? 29.3%??16.1% and 12.2% respectively. The lesions of left main trunk??left anterior descending branch??left circumflex branch and right coronary artery accounted for 43.5 %??18.6%??6.8 % and 31.1% of all the branches involved respectively. ??3??The results of single factor analysis and multivariate logistic regression analysis showed that incomplete KD and elevated C reactive protein ????100 mg/L?? were the risk factors of grade ?? CAL?? while male sex?? age younger than 1 year?? duration of fever over 10 days?? incomplete KD and low serum albumin ????30 g/L?? were the risk factors of Grade ????CAL. Conclusions ??1??Grade III CAL is the most common type in KD patients, accounting for 82.6% of CAL. ??2??About 57.6% CAL occurs in two and abovetwo branches of coronary arteries. ??3??The most frequently involved branch is left main trunk, followed by right coronary artery??left anterior descending branch and circumflex branch. ??4??Being male?? age??1 year old?? incomplete KD?? long-lasting fever ????10 d?? and low serum albumin ????30 g/L?? are the independent risk factors of grade ????CAL.     

川崎病所致严重冠状动脉病变单中心队列研究

背景：川崎病（KD）所致严重冠状动脉病变（CAL）容易导致患儿发生冠状动脉事件或死亡,对远期预后及生存质量具有重要影响。 目的：总结分析伴严重CAL的KD患儿的中远期临床转归,为KD患儿长期随访管理及预后评估提供依据。 设计：队列研究。 方法：检索复旦大学附属儿科医院（我院）HIS系统,纳入2008年1月至2019年12月出院诊断为KD,经我院冠状动脉造影（CAG）确诊为KD所致严重CAL的＜18岁患儿。排除合并其他引起严重CAL和影响严重CAL预后疾病者。所有患儿在门诊及病房由资深心血管专科医生随访,均以药物治疗为基础,伴冠状动脉严重狭窄及心肌缺血的患儿经多学科讨论后予手术治疗。以确诊及随访期间出现心肌缺血、心肌梗死或死亡的患儿为预后不良组,反之则为预后良好组,观察出现不良预后及CAL的演变情况,分析不良预后的影响因素。 主要结局指标：不良预后发生情况和CAL的恢复情况。 结果：144例KD所致严重CAL患儿纳入本文分析。其中男117例（81.2%）,女27例;KD中位发病年龄2.2（1.0,4.5）岁,中位随访时间1.6（0,3.4）年,确诊为严重CAL时病程为5.0（3.0,21.3）月。144例KD所致严重CAL患儿共累及276支冠状动脉,56例（38.9%）累及单支冠状动脉,88例（61.1%）累及≥2支冠状动脉。发生巨大冠状动脉瘤（GCAA)122例,单支冠状动脉多发中小型冠状动脉瘤（CAA)8例,GCAA合并单支冠状动脉多发中小型CAA 6例,受累冠状动脉均狭窄8例;62例发生冠状动脉狭窄,其中冠状动脉闭塞31例。预后不良组54例（37.5%）,均存在心肌缺血,其中14例患儿缺血心肌处发生过有记录的急性心肌梗死,1例患儿因心肌梗死所致严重心力衰竭死亡。多因素分析结果显示,≥2支冠状动脉受累的患儿是单支冠状动脉受累的患儿发生不良预后风险的2.6倍（OR=2.602,95%CI：1.201~5.634）,合并冠状动脉狭窄的患儿是未合并冠状动脉狭窄的患儿发生不良预后风险的3倍（OR=3.055,95%CI：1.450~6.437）。共行179次CAG,114例（208支受累冠状动脉）行1次CAG,30例（68支受累冠状动脉）行≥2次CAG。68支受累冠状动脉首次确诊时,51支为GCAA,末次随访时好转率为4.0%;3支为多发中小型CAA,末次随访时好转率为66.6%;2支为单个中小型CAA,至末次随访均发生狭窄（狭窄程度约50%）;12支为狭窄,至末次随访时均未好转。 结论：GCAA是KD所致严重CAL的主要类型,且难以恢复,单支冠状动脉多发中小型CAA好转率较高。冠状动脉狭窄及多支冠状动脉受累是KD所致严重CAL患儿发生不良预后的影响因素。     

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??Abstract??Objective To explore the risk factors in children with coronary artery lesion ??CAL?? complication in Kawasaki disease??KD??. Methods Retrospective analyses of the clinical data from 143 children with KD from 2009 to 2011 in Shandong Provincial Hospital were performed in terms of 11 aspects including the fever duration??hemoglobin ??Hb????erythrocyte sedimentation rate ??ESR????C-reaction protein ??CRP????white blood cell count ??WBC????blood platelet ??PLT????and the patients??gender and age. 143 children were divided into two groups according to whether these KD patients were with or without CAL.Both single factor and multivariate logistic regression analyses were performed. Results Single factor analysis showed that fever duration over 10 days??ESR??PLT??CRP??ALB??cTnI and NT-porBNP were risk factors to KD complicated with CAL ??P??0.05 or P??0.01??.Multivariate logistic regression analysis showed that a fever duration over 10 days??decrease of ALB and increase of cTnI were significant risk factors to KD complicated with CAL??P??0.05??. Conclusion Fever duration over 10 days??decrease of ALB and increase of cTnI are important risk factors to KD complicated with CAL.     

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目的探讨儿童川崎病(KD)的临床、治疗和预后特点。方法对2000—2005年广东省中山市博爱医院儿科收治的100例KD患儿进行随访,对其临床特征、治疗方案以及预后进行回顾分析。结果 (1)临床特点:发病年龄(2.01±1.35)岁,随访年龄(8.2±1.7)岁;男女之比为1.7∶1。典型KD88例,其中再发病例2例;不完全KD12例。心血管系统并发症25例,包括冠状动脉改变22例,心包积液2例,心肌炎1例,其中一过性冠脉扩张18例,冠脉瘤形成4例;非心血管系统并发症包括肝损害34例,胆囊积液3例,麻痹性肠梗阻1例,无菌性脑膜炎3例,面神经麻痹5例,肺炎53例,尿道炎12例,关节炎6例。(2)治疗和疗效:89例病程10d内给予丙种球蛋白(IVIG)2g/kg,12例无效,需追加第2次IVIG,其中2例加用肾上腺皮质激素。11例亚急性期确诊者给予IVIG1～2g/kg,其中3例并发冠脉瘤(P<0.05)。无冠脉损害者病程6～8周停药,18例有冠脉扩张者跟踪至半年至1年后停药,3例中小型冠脉瘤者在病程1～3年停药,1例巨大冠脉瘤者服用阿司匹林至今已7年。(3)随访和预后:56例追踪随访至病程≥5年,最长10年。随访...     

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。     

川崎病冠状动脉病变严重程度临床分级及高危因素研究

目的 探讨基于临床分级的川崎病（KD）冠状动脉病变临床分级特点及不同危险等级冠状动脉病变的危险因素。方法 采用回顾性调查方法,对复旦大学附属儿科医院2000年1月至2011年6月住院且病史资料完整的1253例KD患儿,依据《川崎病冠状动脉病变的临床处理建议》提出的冠状动脉病变临床分级方法,分析冠状动脉病变临床分级及病变部位特点,采用单因素和多因素Logistic回归分析发生不同临床分级冠状动脉病变的危险因素。结果 （1）KD患儿急性期冠脉病变发生率为24.3%（304/1253）。根据冠状动脉病变临床分级进行分类,冠脉正常的Ⅰ级病例949例,占75.7%;Ⅱ级32例,发生率2.6%;Ⅲ级251例,发生率20.0%;Ⅳ级13例,发生率1.0%;Ⅴ级8例,发生率0.6%。（2）累及单支、双支、三支、四支冠状动脉者分别占冠状动脉病变患儿的42.4%、29.3%、16.1%和12.2%;发生病变的冠状动脉分支中左主干、左前降支、左回旋支、右冠状动脉病变分别占43.5 %、18.6%、6.8 %、31.1 %。（3）多因素Logistic回归分析显示,不完全型KD、CRP＞100 mg/L是Ⅱ级冠状动脉病变的独立危险因素;男性、年龄≤1岁、发热持续时间＞10 d、不完全型KD、ALB＜30 g/L是发生Ⅲ～Ⅴ级病变的独立危险因素。结论 （1）KD冠状动脉病变以Ⅲ级为最多见,占82.6%;V级最少见,占2.6%。（2）57.6%的冠状动脉病变累及二支或二支以上的冠状动脉。（3）KD冠状动脉病变部位以左冠状动脉主干最常见,其后依次为右冠状动脉、左前降支和左回旋支。（4）男性、年龄≤1岁、发热持续时间＞10 d、不完全型KD、ALB＜30 g/L是Ⅲ～Ⅴ级冠状动脉病变的独立危险因素。     

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??Objective To analyze the correlation of heart rate variability??HRV?? parameters with troponin??cTn?? and N-terminal pro-B-type natriuretic peptide??NT-proBNP?? in children with Kawasaki disease??KD??. Methods According to presence or absence of coronary artery lesion??CAL????the enrolled 118 children with KD as KD group were divided into CAL group??n??43?? and non-CAL??NCAL?? group??n??75??. Meanwhile??32 children without cardiovascular disease in convalescent period and 106 healthy children at the same term were respectively selected as non-KD group and control group. Long-term HRV parameters were detected in each group??and levels of cTn?? and NT-proBNP were detected in KD group and non-KD group. Results HRV parameters in KD group were all higher during recovery phase than those during acute phase??except for LF/HF value lower than that during acute phase ??P??0.05 or P??0.01??. HRV parameters in KD group were all lower than those in control group??except for LF/HF value higher than that in control group ??P??0.05 or P??0.01??. Each index in time domain of HRV and high frequency ??HF????low frequency ??LF?? and very low frequency ??VLF?? in CAL group were all lower than those in control group and non-KD group ??P??0.05 or P??0.01????and SDANN??SDNN and PNN50 were also lower than those in NCAL group ??P??0.01??. Besides??the LF/HF value in CAL group was higher than that in control group??non-KD group and NCAL group ??P??0.05 or P??0.01??. The levels of cTn?? and NT-proBNP in CAL group and NCAL group were significantly higher than those in non-KD group??in which the level of cTn?? in CAL group was also higher than that in NCAL group ??P??0.01??. The level of cTn?? was negatively correlated with SDNN and HF in children with KD??while positively correlated with LF/HF value ??P??0.01??. The level of NT-proBNP had a significantly negative correlation with SDANN??SDNN and HF ??P??0.01??. Conclusion The heart autonomic nervous function is damaged in children with KD. HRV parameters have a certain clinical value for assessment of CAL in children with KD.     

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??Abstract??Objective??This study was aimed at evaluating present randomized controlled trials ??RCT?? regarding the drug therapy for intravenous immunoglobulin ??IVIG?? resistant Kawasaki disease ??KD??. Methods??According to inclusion and exclusion criteria?? articles were selected from medical electronic databases. RCT were then assessed based on the Juni assessment?? and meta-analysis was completed by the Review Manager 4.2 software. Indications to evaluate effects were the change of body temperature and change of coronary artery situation detected by ultrasound-cardiogram. The results were stated as relative risk ??RR?? or odd ratio ??OR???? with a 95% confidence interval ??CI?? and a P < 0.05 significant level. Results??In total?? three RCT were selected. Funel plot analysis showed possible publication bias. Meta-analysis of the three RCT?? including all 59 patients in the 2nd intravenous immunoglobulin treatment group and 66 patients in the glucocorticosteroid treatment control group?? indicated that after their first doses of IVIG treatment?? the temperatures of IVIG resistant KD patients who received 2nd IVIG treatment could be more effectively improved than those who received glucocorticosteroid treatment ??RR = 1.29??95% CI??1.10??1.52??P = 0.002???? but there were no differences on the incidence of coronary artery aneurysm between two groups ??OR = 0.91??95% CI??0.33??2.49??P = 0.85??. Conclusion??The Meta-analysis of currently published RCT demonstrates that the 2nd IVIG can more effectively improve the temperature than glucocorticosteroid in IVIG resistant KD patients?? but there were no differences on the incidence of coronary artery aneurysm between two groups.     

川崎病冠状动脉损害超声心动图及心电图分析

目的　探讨超声心动图与心电图对川崎病冠状动脉损害的诊断价值。方法　对川崎病患儿 78例行超声心动图及心电图检查 ,根据超声检查结果分为 3组 ,并对各组进行分析。结果　超声检出冠状动脉病变 45例 ,其中冠状动脉扩张 32例 ,冠状动脉瘤 1 3例 ;心电图异常 1 6例。冠状动脉损害组心电图异常率高于无冠状动脉损害组 (P <0 .0 1 ) ;冠状动脉瘤组心电图异常率明显高于冠状动脉扩张组 (P <0 .0 0 5)。结论　超声心动图与心电图相结合可更全面地反映川崎病的冠状动脉损害。     

川崎病并冠状动脉损害的危险因素

目的探讨川崎病(KD)并冠状动脉损害(CAL)的危险因素。方法收集1999年1月-2001年12月住院的145例KD患儿的临床资料,分析无CAL93例和CAL52例患儿的实验室检查资料及治疗、随访结果。结果无CAL组发热日程(8.7±3.4)d.血沉(79.5±34.6)mm/1h,CAL组发热日程(11.5±4.8)d,血沉(91.9±36.6)mm/1h,两组比较差异有显著性(P<0.01,0.05)。发病10d内接受静脉注射人血丙种球蛋白(IVIG)治疗患儿CAL发生率为18.8%(18/96),未予IVIG治疗患儿CAL发生率为69.4%(34/49),两者比较差异有显著性(P<0.001)。随访并CAL患儿38例,冠状动脉扩张20例均恢复正常;14例冠状动脉瘤中12例恢复正常,2例缩小;4例巨大冠状动脉瘤2例缩小,另2例超过2年仍未恢复。结论KD并CAL与热程过长及血沉显著增快密切相关,病程早期未予IVIG治疗的患儿并CAL的危险性明显增高,应用IVIG治疗对防治冠状动脉损害有重要作用。