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川崎病(Kawasaki disease,KD)于1967年由Tomosaki Kawasaki首次报道,是一种全身中小血管炎性病变为主的急性发热出疹性疾病,尤其累及冠状动脉,目前已成为儿童最常见的获得性心脏病之一[1]。KD急性期的诊断和治疗方案已经比较成熟。尽管通过正规治疗,冠状动脉病变     

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目的分析4月龄以下小婴儿川崎病的临床发病特点。方法对1999-01—2005-12首都儿科研究所附属儿童医院收治的4月龄以下川崎病患儿30例,进行临床资料回顾性分析。结果4月龄以下小婴儿川崎病以男孩多发,无季节差异;以典型病例多见,但仍有约20%患儿为非典型病例,平均确诊时间长,误诊率较高。结论冠状动脉异常可作为小婴儿非典型川崎病惟一确诊的金指标。同时对于发热>5d,在感染性疾病恢复期再次高热,有明确病毒或细菌感染存在,有预防接种后不明原因发热持续不退的4月龄以下小婴儿应警惕川崎病的发生。     

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目的探讨血管内皮细胞抗体(AECA)检测对川崎病(KD)患儿诊断及预后评价。方法2007年8月至2008年5月在广州市儿童医院住院的5岁以下KD患儿55例,采用以人脐静脉内皮细胞和猴骨骼肌为基质的间接免疫荧光技术检测55例急性期KD患儿和43例对照组(发热对照23例,健康对照20名)血清中AECA,并通过比较AECA阴性和阳性组相应的实验室、临床指标,初步评估AECA对KD早期诊断、预后转归的临床应用价值。结果KD患儿中AECA阳性率为40.0%,明显高于健康对照组(5.0%,P=0.004),但与发热对照组差异无统计学意义(17.4%,P=0.053);AECA对KD诊断的灵敏度为40.0%,特异性为88.4%,阳性预测值与阴性预测值则分别为80.0%、53.5%,准确率为60.4%。抗中性粒细胞浆抗体(ANCA)、蛋白酶3抗体(抗-PR3)阳性KD患儿中AECA阳性率显著高于阴性者(P值分别为0.013、0.034)。AECA阳性组与阴性患儿组临床指标、实验室指标差异无统计学意义。AECA阳性组冠状动脉瘤发生率高于阴性组(P<0.05),发生动脉瘤的相对危险度为6.67(95%CI1.2～36.1)。结论...     

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目的探讨10d内已退热的川崎病(KD)患儿应用丙种球蛋白(IVGG)治疗的必要性以及不同剂量IVGG治疗对KD预后的影响。方法研究对象为1999-10—2005-10山东省菏泽市立医院收治的56例KD患儿,所有患儿均为10d内退热后确诊且无冠脉病变。按IVGG治疗剂量分成3组,A组(11例)用1g/kg,B组(26例)用2g/kg,C组(19例)未使用,余治疗相同。对其冠状动脉损害(CAL)情况进行对比。结果病程14~21d时发生CAL例数:A组2例(18·18%),B组4例(15·38%),C组16例(84·21%),A、B组比较差异无显著性意义(P>0·05);A、B组与C组之间差异有非常显著性意义(P<0·01)。随访0·5年CAL例数:A组1例(9·09%),B组1例(3·85%),C组11例(57·89%),A、B组比较差异无显著性意义(P>0·05),而A、B组与C组之间差异有非常显著性意义(P<0·01)。结论10d内一经确诊的KD无论是否已退热均应给予IVGG治疗,对已退热且无冠脉损害的患儿应用总量1g/kg IVGG治疗可以达到满意的效果。     

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??Abstract?? The incidence of Kawasaki disease??KD?? is increasing in Asian countries??even worldwide. Refractory cases still have a strong challenge. Incomplete KD and non-responders to initial IVIG treatment may increase the rate of the coronary artery lesions??CAL??. Because of the rate of resistance to initial IVIG treatment??timely additional treatment can significantly reduce the risk of echocardiologically defined CAL in the majority of patients. It’s important to diminish inflammation?? endothelial dysfunction and CAL formation??including application of tumor necrosis factor-alpha antagonist?? methylprednisolone pulse therapy??ulinastatin and cytotoxic agents??which helps to decrease the life-long sequelae of the cardiovascular lesions.     

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??ObjectiveTo investigate the clinical characteristics and prognosis of coronary aneurysm in children with Kawasaki disease.MethodsCoronary aneurysm was identified by using echocardiography in 63 children with Kawasaki disease from 2000 to 2007.Their gender,age,clinical presentations,use of intravenous gamma immunoglobulin (IVIG),laboratory study results and echocardiographic study results in the acute and convalescent phase were examined retrospectively.Results(1) Boys and infants were more likely to develop coronary aneurysms,the male to female ratio being 5.3??1,and for patients with giant aneurysms the male to female ratio was 8.3??1.(2)The rate of incomplete KD,IVIG non-responders and recurrence in this group of patients were 36.5%,30.2% and 7.9%,respectively,which were higher than the whole KD group.In the acute phase,57 of 63 patients were treated by IVIG with different doses,3 of them did not receive IVIG treatment and the other 3 patients?? IVIG treatments were unknown.The duration from onset to IVIG treatment was longer than 10 days in 36 of the 63 (57.1%) children.(3) Small aneurysms were detected by 2-D echo in 7 patients,medium aneurysms in 19 patients and giant aneurysm in 37 patients.In all of these patients,76.2% of them had left coronary artery aneurysms,and 87.3% of them had right coronary artery aneurysms;47.3% of them had both left and right aneurysms.In the patients with left coronary aneurysms,58.3% of them have left anterior descending branch aneurysms; and in patients with right coronary aneurysms,47.3% of them were located in the right coronary artery segment II.(4) Coronary aneurysm regressed in 71.4 % of the branches.The lumen diameter of the affected branches returned to normal in 45.2% of them,with a mean regression time of 2.1??1.5 years.ConclusionBeing male,younger than 1 year old,incomplete KD,IVIG non-responder,recurrence and later IVIG treatment are possible risk factors of coronary aneurysms in children with Kawasaki disease.Most children have multi-coronary artery aneurysms and left anterior descending branch and right coronary artery are the most commonly affected branches??and most of the coronary aneurysms have the tendency to regress during the convalescent phase.     

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??Objective To analyze the clinical characteristics of patients with CAL complicated by KD??and to improve the understanding of this disease. Methods Clinical data of 19 cases diagnosed as KD complicated by CAL from September 2008 to August 2015 in the Pediatric Department of First Hospital of China Medical University were retrospectively analyzed. Results KD children complicated with CAL was more in male than in female??male??female was 3.75??1??children with coronary aneurysm were more in male than in female??male??female was 8??1??it was more often in less than 1-year old children??accounting for 36.8%. The incidence rate of coronary artery lesions in this group was 16.4%??CAD was in 10 cases??CAA in 5 cases??GCAA in 4 cases??the incidence rate being 8.6%??4.3% and 3.5%??respectively??in terms of CAL time 73.7% was 2-3 weeks after the disease?? according to the number of branches involved??both coronary artery involved accounted for 68.4%??and according to the location involved??right coronary artery involvement accounted for 41.5%??left main coronary artery involvement accounted for 39.0%??the left anterior descending coronary artery involvement accounted for 17.1%??and left circumflex artery involvement accounted for 2.4%. The clinical grade of severity in CAL children were???? grade in 5 cases??accounting for 26.3%???? grade in 8 cases??accounting for 42.1%???? grade in 6 cases??accounting for 31.6%. During follow-up??71.4% patients had coronary artery aneurysm or dilatation significantly retracted??the average time of retraction being ??1.5±0.7??years??of which 1 case was completely dissipated. Conclusion The incidence rate of the KD patients with CAL is 16.4%??in which CAD has the highest incidence rate while GCAA the lowest. CAL mostly occurs at 2-3 weeks after the disease. Any branch can be damaged??with the right coronary artery to be most likely involved. As to coronary artery disease severity??grade ?? is of the highest proportion. Most coronary artery aneurysm or dilatation has remarkable retraction within 1-2 years.     

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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。     

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目的分析川崎病(KD)并发冠状动脉瘤(CAA)的高危因素及预后。方法回顾性分析重庆医科大学附属儿童医院1993年1月至2009年12月3902例住院KD患儿的临床资料,对与冠状动脉损害发生有关的因素进行计数资料χ2检验、计量资料t检验,将单因素分析筛选出的变量行多因素Logistic回归分析;对随访的46例CAA患儿病后1、3、6个月和1、2、3、4、5年及5年以上CAA的恢复情况及不同剂量静脉注射丙种免疫球蛋白(IVIG)的远期疗效进行χ2检验。结果 (1)单因素分析结果:性别、年龄、热程、IVIG使用时机、血红蛋白(Hb)、红细胞沉降率(ESR)、血清白蛋白与KD并发CAA有关(P<0.05),将单因素分析筛选出的上述因素作为自变量进一步行多因素分析,结果显示,Hb、ESR、IVIG使用时机与KD并发CAA呈独立相关性(P<0.05),而性别、年龄、热程、血清白蛋白与KD并发CAA非独立相关(P>0.05);(2)小型CAA、中型CAA、巨大CAA的回缩时间逐渐延长(P<0.05),年龄及IVIG的剂量与CAA的回缩时间无统计学相关性(P>0.05)。结论 Hb<100 g/L、ESR>50 mm/h、I...     

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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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目的观察尿白三烯(LTs)E4(LTE4)在儿童典型川崎病(KD)中的变化,探讨是否可以将LTs作为KD后的监测指标。方法东南大学附属盐城医院儿科2007年10月至2010年9月收治68例完全性KD患儿,采用竞争性酶联免疫吸附技术检测其在治疗前后尿LTE4水平,55名正常儿童尿LTE4作为对照组,同时对KD急性期患儿行心脏彩超检查,分为有冠状动脉损害组(8例)和无冠状动脉损害组(60例)。结果 KD患儿治疗前尿LTE4均高于正常对照组,差异有统计学意义(P<0.01);恢复期与正常对照组比较,差异无统计学意义(P>0.05);KD合并冠状动脉损害组尿LTE4明显高于无冠状动脉损害组,差异有统计学意义(P<0.01);所有KD患儿治疗前后比较,LTE4均明显降低(P<0.01)。结论 KD尤其是合并冠状动脉损害的患儿LTs水平明显增高,尿LTE4可作为KD后临床监测指标,可以考虑通过早期应用LTs受体拮抗剂干预来达到预防合并冠状动脉病变发生、促进冠状动脉损伤修复的目的。     

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目的探讨川崎病(KD)发生冠状动脉病变(CAL)的危险因素。方法回顾性分析2000年1月至2004年12月期间942例住院KD患儿的临床资料,对发生CAL(170例)和未发生CAL(772例)的病例进行组间对照研究,对13种影响CAL发生的因素进行Logistic回归分析。结果Logistic回归分析结果表明,热程大于10d(X1)的OR值为1.203(95%CI=1.104～1.312,P<0.001)、血红蛋白(Hb)小于100g/L(X2)的OR值为0.658(95%CI=0.472～0.995,P<0.05)。建立KD发生CAL的危险因素主效应模型是Logit(P)=β0(-2.995)+0.185X1+(-0.378)X2(χ2=16.920,P=0.031)。结论热程大于10d、Hb<100g/L是KD发生CAL的高危因素。     

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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.     

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??Kawasaki disease??KD?? is an acute vasculitis of childhood??which is the leading cause of acquired heart disease in children in developed countries. Its annual incidence has been increasing year by year. The 2017 edition of Diagnosis??Treatment??and Long-termManagementofKawasaki Disease??A Scientific Statement for Health Professionals from the American Heart Association has identified new evidence of pathological processes. The diagnosis of KD is based on the non-specific clinical pictures. Evaluation of suspected incomplete KD is simplified. Patients believed to be at high risk for IVIG-resistance and development of coronary artery aneurysms may benefit from an improved primary adjunctive therapy. Z value is used to evaluate and classify the coronary artery complications of KD. The long-term management of KD becomes more standardized.     

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目的观察川崎病(KD)患儿血清降钙素原(PCT)、白介素-6(IL-6)水平的变化,探讨其与KD并发症间的关系。方法研究对象为2003-10—2005-12在首都儿科研究所附属儿童医院住院的88例KD患儿,测定其血清PCT、IL-6水平;正常对照为同期33例健康儿童,进行同样检测。结果(1)71例急性期患儿PCT水平与14例亚急性期患儿、33例正常对照儿童PCT水平相比,其差异均有统计学意义(P<0.05和<0.01)。(2)急性期24例其他系统并发症患儿PCT水平显著高于47例无其他系统并发症患儿,且差异有统计学意义(P<0.01);当PCT≤0.5ng/mL时,其他系统并发症发生率16.3%;当PCT>0.5时,其他系统并发症发生率41.0%,两者差异有统计学意义(P<0.05)。(3)56例急性期患儿IL-6水平与11例亚急性期患儿、14例正常对照儿童IL-6水平相比,其差异均有统计学意义(P值均<0.01)。(4)急性期17例其他系统并发症患儿IL-6水平显著高于39例无其他系统并发症患儿IL-6水平,二者差异有统计学意义(P<0.01)。结论(1)KD患儿急性期血清PCT、IL-6水平增高,亚急性期下降,PCT增高维持时间较IL-6稍长。(2)血清PCT、IL-6水平在发生其他系统并发症患儿中是显著升高的,对其检测有助于KD的病情评价。     

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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...