脂联素基因+45和+276多态性与川崎病的关系

目的 检测川崎病患儿脂联素基因+45T/G、+276G/T多态性分布情况,探讨其与川崎病发病和冠状动脉损伤（CAL）发生的关系。方法 采取病例对照研究方法,选取81例川崎病患儿（其中11例并发CAL）和100例正常体检儿童（对照组）。采用基因测序方法测定脂联素基因+45和+276位点的多态性分布。结果 川崎病组脂联素基因+45位点基因型TT、TG、GG以及T/G的等位基因频率与对照组比较差异无统计学意义（P > 0.05）;川崎病组中并发CAL者与无CAL者该位点的基因型分布频率和等位基因频率比较差异亦无统计学意义（P > 0.05）。川崎病组与对照组脂联素基因+276位点基因型GG、GT、TT及G/T等位基因频率的比较差异均有统计学意义（P < 0.05）;GG基因型为川崎病发病的危险因素（OR=2.313,P=0.006）。川崎病患儿中CAL组脂联素基因+276位点基因型分布与非CAL组比较差异无统计学意义（P > 0.05）。结论 脂联素基因+276位点多态性可能与川崎病的发生相关,但与CAL的发生无关;脂联素基因+45位点多态性可能与川崎病和CAL的发生均无关。     

静脉人免疫球蛋白无反应型川崎病患儿的循证治疗

目的：针对近期收治的1例IVIG无反应型川崎病的患儿,我们进行了证据检索和评价,以期找到更有效的治疗方法。方法：通过计算机检索UpToDate(2012.10)、Cochrane图书馆（Issue 10,2012）、PubMed（1978~2012.10）、CNKI（1978~2012.10）等数据库,查找免疫球蛋白或糖皮质激素治疗IVIG无反应型川崎病及病情缓解有关的系统评价、临床随机对照试验等,并对所获证据进行GRADE评价。结果：高质量的临床证据表明,小剂量激素联合IVIG治疗IVIG无反应型川崎病,其在退热效果及减少冠状动脉扩张方面,均优于单纯IVIG治疗。根据此临床证据,结合医生经验及家属意愿对该患儿实施甲强龙（2mg/kg,ivd）联合第三剂IVIG（1g/kg）治疗,激素逐渐减量维持,并继续采用阿司匹林、双嘧达莫等治疗。经治疗后,患儿体温及各实验室指标逐渐恢复正常,心脏彩超随访半年未见有冠状动脉进一步损伤。结论：小剂量激素联合IVIG治疗IVIG无反应型川崎病可有效退热及减少冠脉扩张,长期效果需待进一步观察。     

不同剂量丙种球蛋白联合糖皮质激素治疗小儿中重度急性吉兰-巴雷综合征的疗效比较

目的 观察并比较静脉注射小剂量丙种球蛋白与大剂量丙种球蛋白联合糖皮质激素冲击治疗小儿中重度急性吉兰-巴雷综合征（GBS）的临床疗效与安全性。方法 将100例中重度急性GBS患儿随机分为小剂量组（48例）和大剂量组（52例）。小剂量组和大剂量组分别采用0.2 g/（kg·d）和0.4 g/（kg·d）丙种球蛋白联合甲基强的松龙治疗。比较治疗后两组症状改善时间、外周血炎性因子水平的变化、有创通气比例、治疗有效性及不良反应。结果 治疗5 d后,小剂量组和大剂量组血清TNF-α、IL-6、CRP水平均较治疗前明显下降,两组TNF-α、IL-6、CRP下降幅度差异均无统计学意义。两组患儿呼吸机麻痹恢复时间、肌力提升1级时间、感觉障碍恢复时间及住院时间比较差异均无统计学意义。小剂量组和大剂量组总体有效率（90% vs 92%）比较差异无统计学意义。两组患儿发热、头痛、恶心、心慌发生率比较差异均无统计学意义。结论 小剂量与大剂量丙种球蛋白联合甲基强的松龙冲击治疗小儿中重度急性GBS临床疗效与安全性相当。     

川崎病抗凝治疗的指征及应用

川崎病血管炎介导的凝血功能异常,以及冠状动脉内血流动力学变化,使冠状动脉内血栓形成的风险增加。因此,川崎病抗凝治疗非常重要。治疗指征和疗程基于冠状动脉严重程度分级,所有川崎病患儿均需接受抗血小板治疗,冠状动脉病变达到Ⅲ级者需接受两种以上抗血小板药物;Ⅳ级以上者需阿司匹林联合华法林抗凝治疗;合并血栓形成及心肌梗死者需及早给予溶栓治疗;抗凝治疗疗程至少需持续3个月,如果冠状动脉病变持续存在需长期抗凝治疗。     

抗中性粒细胞抗体在川崎病诊断中的价值

目的：探讨抗中性粒细胞抗体（ANCA)在川崎病诊断中的价值。方法：检测30例典型川崎病患儿和16 例不完全川崎病患儿急性期、恢复期血清ANCA,并随机选取25例健康儿童作为对照组。同时对川崎病患儿急性期行超声心动图(UCG)检查。结果：川崎病患儿急性期血清ANCA阳性率为65%,其中不完全川崎病组和典型川崎病组血清ANCA阳性率分别为69％、63％,均明显高于对照组(P＜0.01）。川崎病患儿恢复期血清ANCA阳性率显著低于急性期（33％ vs 65％,P＜0.05）。川崎病患儿急性期血清ANCA阳性率显著高于UCG的阳性检出率(P＜0.01)。ANCA阳性患儿冠脉病变发生率明显高于ANCA阴性患儿（43％ vs 13％,P＜0.05)。结论：血清ANCA可以作为一项早期诊断川崎病的参考指标和发生冠脉损害的一项实验室预警指标。     

非典型川崎病25例临床特点回顾性分析

目的总结非典型川崎病的诊疗经验。方法对25例非典型川崎病患儿的临床资料进行回顾性分析。结果 25例非典型川崎病患儿中,10例误诊为咽结合膜热,5例误诊为淋巴结炎,1例误诊为猩红热,1例误诊为金黄色葡萄球菌烫伤样皮肤综合征,8例拟诊为川崎病。25例患儿均有持续发热,误诊患儿中临床症状、体征可有多变性,体征非持续性。血白细胞、血沉、C反应蛋白、血小板均升高。心脏彩超2例出现冠状动脉改变。经使用阿司匹林和丙种球蛋白联合治疗,25例患儿预后良好,随诊1年无冠状动脉异常发现。结论对于持续发热,抗生素治疗无效的患儿,白细胞、血沉、C反应蛋白、血小板等明显升高,结合多变的临床症状、体征,应警惕川崎病的可能性。阿司匹林和丙种球蛋白联合应用是川崎病的标准治疗方法。     

川崎病患儿治疗前后血清脂源性细胞因子的变化及意义

目的 检测急性期川崎病患儿静脉注射丙种球蛋白（IVIG）治疗前后血清中脂源性细胞因子Omentin-1和Chemerin浓度变化及意义。方法 选取2015年1月至2019年4月确诊为川崎病患儿60例为研究对象,同时选取40例健康儿童和40例急性感染性疾病患儿分别作为健康对照组和感染对照组。根据是否对IVIG治疗敏感将川崎病患儿分为IVIG敏感组（n=51）和IVIG不敏感组（n=9）;根据是否合并冠状动脉损害（CAL）将川崎病患儿分为合并CAL组（n=13）和不合并CAL组（n=47）。ELISA法检测川崎病患儿IVIG治疗前后及两对照组儿童血清Omentin-1和Chemerin水平。结果 川崎病患儿血清Omentin-1和Chemerin水平均明显高于健康对照组和感染对照组（P < 0.05）。经过48 h治疗后,IVIG敏感组患儿血清Chemerin水平较治疗前明显降低（P < 0.05）,血清Omentin-1水平在IVIG敏感组患儿治疗前后比较差异无统计学意义（P > 0.05）。治疗前,IVIG不敏感组患儿血清Chemerin水平明显高于IVIG敏感组（P < 0.05）;合并CAL组血清Chemerin水平明显高于不合并CAL组;而血清Omentin-1水平在IVIG敏感与不敏感组间及合并CAL与不合并CAL组间比较差异均无统计学意义（P > 0.05）。结论 川崎病患儿血清中高表达的Chemerin和Omentin-1可能参与川崎病的发生和发展;Chemerin可能参与川崎病所致CAL过程,且血清Chemerin水平可能成为临床预测IVIG敏感性的新监测指标。     

不同剂量更昔洛韦治疗新生儿先天性巨细胞病毒感染的临床观察

目的：更昔洛韦（GCV）是治疗先天性巨细胞病毒(CMV)感染的首选药物,临床疗效是肯定的,但有一定的副作用。该文旨在评价不同剂量GCV治疗先天性CMV感染新生儿的临床疗效和副作用。方法：先天性CMV感染新生儿167例分为大剂量治疗组（n=79）和小剂量治疗组（n=88）,均给予GCV治疗和其他对症支持治疗。大剂量治疗组给予GCV的剂量为：诱导治疗每次7.5 mg/kg,维持治疗每次10 mg/kg;小剂量治疗组给予GCV的剂量：诱导治疗每次5 mg/kg,维持治疗每次5 mg/kg,观察两组的临床疗效和副作用。结果：①两种剂量的GCV治疗先天性CMV感染有相同疗效,临床症状明显好转,大剂量治疗组CMV IgM转阴率93.8%,CMV DNA转阴率80.8%;小剂量治疗组CMV IgM转阴率93.1%,CMV DNA转阴率为86.7%,两组比较差异无显著性（P>0.05）;②小剂量GCV治疗先天性CMV感染新生儿的副作用低于大剂量GCV,大剂量治疗组呕吐发生率11.4%,贫血发生率20.3%,中性粒细胞减少发生率16.5%,血小板增加发生率18.9%;小剂量治疗组呕吐发生率2.3%,贫血发生率8.0%,中性粒细胞减少发生率5.7%,血小板增加发生率8.0%,两组比较差异有显著性（P<0.05）。结论：小剂量GCV治疗新生儿先天性CMV感染与大剂量有同样的临床疗效,且副作用低于大剂量GCV,更安全,值得临床推广使用。［中国当代儿科杂志,2009,11（8）：641-644］     

不完全川崎病的临床诊治策略

不完全川崎病虽不完全符合川崎病的诊断标准,但具有部分川崎病的临床特征。结合临床表现、实验室和超声心动图检查可为不完全川崎病提供诊断线索。治疗主张急性期给予大剂量静脉内丙种球蛋白（IVIG）联合大剂量阿司匹林及恢复期随访;对IVIG不反应者,可应用2剂或3剂IVIG、糖皮质激素及针对细胞因子的单克隆抗体等;已有冠状动脉病变的患儿需长期服用抗凝药物,必要时进行介入或手术治疗。随着对川崎病病因和发病机制以及不完全川崎病的发病率渐增趋势的认识,有必要制定不完全川崎病的诊断和治疗标准。     

病毒性心肌炎和川崎病患儿血小板功能形态与自由基关系的探讨

病毒性心肌炎和川崎病患儿血小板功能形态与自由基关系的探讨西安医科大学第一临床医学院儿科（７１００６１）倪贺虹苏祖佑郗玉娥林淑金１）王鸣英１）病毒性心肌炎、川崎病的发病机理均与氧自由基或其清除酶系统的消长有关。自由基与血小板花生四烯酸代谢关系极为密切。...     

School-aged children with Kawasaki disease: high incidence of cervical lymphadenopathy and coronary artery involvement

OBJECTIVE: We describe 10 school-aged children with Kawasaki disease (KD) with a high incidence of cervical lymphadenopathy and coronary abnormality. METHODS: Based on a database of 1002 children with KD in Chang Gung Children's Hospital from January 1983 to March 2001, 10 (1%) school-aged patients (five boys, five girls) who met the diagnostic criteria of KD were included for analysis. RESULTS: Cervical lymphadenopathy was noted in all (100%) of these patients. Unilateral neck mass mimicking acute suppurative infections not responding to antibiotic therapy was the initial presentation in nine (90%) of the 10 patients. The mean interval between disease onset and diagnosis was 9.9 +/- 3.3 days (range, 6-15 days). Seven (70%) of these patients responded to one course of high-dose intravenous immunoglobulin (IVIG) therapy (2 g/kg) and oral aspirin (80-100 mg/kg per day), two (20%) required a second course of IVIG, and one (10%) responded to high-dose aspirin treatment only. Coronary artery abnormality (dilatation or aneurysm) was documented by echocardiography in seven (70%) patients (four boys, three girls). In six patients, the coronary artery abnormalities resolved in 1 year, while one patient had persistent right coronary artery aneurysm, which necessitated continued anticoagulant and low-dose aspirin therapy. CONCLUSION: The incidence of school-aged children among patients with KD is about 1% in our hospital. These patients are notable for the high incidence of initial manifestations of unilateral neck mass and coronary artery involvement. This disease should be listed as the differential diagnosis in school-aged children presenting with fever and neck mass that do not respond to antibiotic therapy.     

川崎病丙种球蛋白耐药及冠状动脉损伤早期预测指标的研究进展

川崎病是急性全身性血管炎综合征,主要影响冠状动脉.该病的长期预后取决于冠状动脉病变程度.早期应用大剂量静脉丙种球蛋白可以减少冠状动脉病变,丙种球蛋白耐药者冠状动脉病变风险大,早期预测丙种球蛋白耐药及冠脉损伤、及时采取措施对改善预后意义重大.该文对川崎病丙种球蛋白耐药及冠状动脉损伤的预测指标作一介绍.     

Coronary artery involvement in Kawasaki syndrome in Manhattan, New York: risk factors and role of aspirin

Since January 1980, 110 children having 113 attacks of Kawasaki syndrome were studied. Age at onset was 7 weeks to 12 years (mean 3 6/12 years, median 2 9/12 years); 77% were younger than 5 years of age; the male to female ratio was 1.8; racial distribution was 52% white, 19% black, 14% Hispanic, and 16% Asian. Protocol of management consisted of high-dose aspirin (100 mg/kg/d) until afebrile, and then 81 mg every day until free of coronary aneurysm. Two-dimensional echocardiograms were done weekly during the acute stage, at 2 and 6 months after onset, and yearly if a coronary abnormality was detected. At 1 month, 51 coronary arterial abnormalities were present in 25 patients. Risk factors for a coronary abnormality were duration of fever greater than or equal to 2 weeks, level of platelet count, marked elevation of ESR, and age younger than 5 years. No statistically significant difference in incidence of aneurysms was detected between patients on high-dose aspirin and those on medium-or low-dose aspirin.     

川崎病患儿外周血中红细胞和血小板参数的变化

川崎病（KD）是一种以全身血管炎为主要病理改变的急性发热、出诊性疾病：多数患儿还可以出现贫血、血小板数升高等改变。本文通过全自动血细胞计数仪对KD患儿的外周血红细胞和血小板参数的观察，发现其贫血患儿的平均红细胞体积（MCV）降低、红细胞分布宽度（RDW）增高，为小细胞不均一性贫血，符合缺铁性贫血的表现。KD患儿血小板数普遍较高，尤其病程早期的平均血小板体积（MPV）增高，提示有血小板增多的趋势和在血管炎性反应中的重要作用。因此，对KD患儿应尽早使用抗血小板药物，在治疗过程中注意对缺铁性贫血的纠正。     

再发川崎病20 例临床分析

目的：探讨再发川崎病的临床特点及与冠状动脉病变的关系。方法：收集该院1998年1月至2007年5月收治的再发川崎病20 例,分析临床特点及相关实验室数据。结果：收治初发的1 489例川崎病中,再发20 例,再发率为1.34%,再发间期1.2年。再发病例临床病情较初发轻,症状不典型或复杂，热程、血小板、C反应蛋白和红细胞沉降率较初发时明显降低，但冠状动脉损伤发生率显著高于初发组（40% vs 25%, P<0.05）。结论：再发川崎病临床症状不典型，冠状动脉损伤发生率增高。川崎病再发为川崎病并发冠状动脉损伤的高危因素之一,应加强早期诊断和治疗。［中国当代儿科杂志，2008，10（5）：617-619］     

Comparison of high-dose and low-dose aspirin plus intravenous immunoglobulin in the treatment of Kawasaki syndrome

The efficacy of intravenous immunoglobulin (IVIG) in the treatment of Kawasaki syndrome (KS) has been unequivocally established, but questions remain concerning the proper dose of adjunctive aspirin therapy in the treatment of KS. The medical records of 72 children with KS were reviewed. All patients were treated with IVIG; 21 received 400 mg/kg/dose on 4 consecutive days and 51 received 2 g/kg as a single infusion. Seventy patients also received aspirin. Twenty-four of the 70 patients were started on high-dose aspirin (80-100 mg/kg/day) at the time of diagnosis. High-dose aspirin was given for a mean (+/- SE) duration of 6.1+/-0.9 days, then switched to low-dose aspirin (3-5 mg/kg/day). Forty-six of the 70 patients were started on low-dose aspirin at the time of diagnosis and remained on low-dose aspirin for the duration of treatment. Coronary artery abnormalities were present at the time of diagnosis in 12 of 72 patients (17%), including 6 of 6 of patients (100%) with atypical KS and 6 of 66 patients (9%) with typical KS. None of the remaining 60 patients developed coronary artery abnormalities after treatment with IVIG and aspirin. The mean duration of fever after initiation of therapy was 44+/-6 hours in patients treated with IVIG 400 mg/kg/dose on 4 consecutive days and 35+/-5 hours in patients treated with 2 g/kg as a single infusion (p=0.3). The mean duration of fever after the initiation of therapy was 47+/-8 hours in patients treated with high-dose aspirin compared to 34+/-5 hours in patients treated with low-dose aspirin (p=0.13). These preliminary results indicate there is no benefit to high-dose aspirin compared to low-dose aspirin in the treatment of children with KS.     

氯吡格雷联合阿司匹林对川崎病并发中小型冠状动脉瘤患儿抗血栓治疗的疗效及安全性研究

目的 探讨川崎病（KD）合并冠状动脉瘤（CAA）患儿使用氯吡格雷联合阿司匹林抗血栓治疗的疗效及安全性。方法 选取2013年1月至2018年6月经超声心动图诊断为合并多发中小型CAA的KD患儿77例为研究对象,随机分为观察组（n=38）和对照组（n=39）,分别接受氯吡格雷+阿司匹林和低分子肝素+阿司匹林治疗。所有患儿定期随访,以病程前3个月为观察期,总结两组患儿的冠状动脉变化及其他并发症发生情况。结果 随访至3个月时,观察组6例冠状动脉恢复正常,11例冠状动脉较前回缩,19例冠状动脉稳定,2例发展为巨大冠状动脉瘤;对照组7例冠状动脉恢复正常,12例冠状动脉较前回缩,19例冠状动脉稳定,1例发展为巨大冠状动脉瘤;两组冠状动脉变化情况比较差异无统计学意义（P > 0.05）。观察组合并鼻衄2例,皮肤瘀斑6例;对照组合并鼻衄1例,出现注射部位瘀点瘀斑7例;两组均未出现其他严重出血事件。结论 氯吡格雷联合小剂量阿司匹林对KD合并CAA患儿的抗血栓治疗安全有效。     

日本《川崎病诊断指南第6次修订版》要点解读

川崎病（KD）是儿童时期一种急性全身非特异性血管炎,未经治疗的KD患儿约有25％发生冠状动脉瘤或扩张,可导致缺血性心脏病或猝死,已成为发达地区儿童获得性心脏病最常见的病因。由于KD的诊断主要依赖于临床特征,因此,KD的及早诊断,尤其是不完全性KD,仍面临挑战。该文结合国内临床实践情况,对日本循环学会科学委员会修订发表的《川崎病诊断指南第6次修订版》要点问题给予解读,并与2017年美国心脏病学会（AHA）颁布的KD诊断的科学声明进行对比,希望对提高国内KD诊断水平有所帮助。     

日本《川崎病诊断指南第6次修订版》要点解读

川崎病（KD）是儿童时期一种急性全身非特异性血管炎,未经治疗的KD患儿约有25％发生冠状动脉瘤或扩张,可导致缺血性心脏病或猝死,已成为发达地区儿童获得性心脏病最常见的病因。由于KD的诊断主要依赖于临床特征,因此,KD的及早诊断,尤其是不完全性KD,仍面临挑战。该文结合国内临床实践情况,对日本循环学会科学委员会修订发表的《川崎病诊断指南第6次修订版》要点问题给予解读,并与2017年美国心脏病学会（AHA）颁布的KD诊断的科学声明进行对比,希望对提高国内KD诊断水平有所帮助。     

Warfarin Therapy for Giant Aneurysm Prevents Myocardial Infarction in Kawasaki Disease

We retrospectively investigated the effect of warfarin therapy in improving the clinical outcome of Kawasaki disease (KD) patients with giant coronary aneurysms (GAs). We followed 2350 KD patients from 1973 to 2004. The GAs (≥8 mm in diameter) were diagnosed by coronary angiography. Sixty-eight patients (54 males and 14 females) were retrospectively studied. Patients were divided into two groups. One group consisted of 19 patients with 33 branches treated with a combination of low-dose aspirin and warfarin (target international normalized ratio: 1.5 – 2.5 IU). The second group consisted of 49 patients with 102 branches treated with low-dose aspirin without warfarin. The incidence of myocardial infarction was significantly less in the combination therapy group than in patients treated with aspirin without warfarin (1 patient vs. 16 patients, p < 0.05). Sudden death occurred in seven patients taking aspirin without warfarin, but none of the patients receiving warfarin died. No major bleeding events occurred in either group. Combination therapy of warfarin with aspirin is associated with a decreased risk of myocardial infarction in KD patients with GAs.