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。     

川崎病患儿血管内皮细胞抗体检测临床意义分析

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% CI 1.2～36.1）。结论　AECA检测对KD的早期诊断价值有限,但对冠状动脉瘤的发生具有预警意义。AECA可能与抗-PR3相互协同在KD冠状动脉损伤、进展至动脉瘤过程中起一定的作用。     

广州市儿童医院2001至2004年川崎病患儿冠状动脉损害的特点及随访

目的 探讨广州市儿童医院2001至2004年川崎病（KD）患儿冠状动脉损害的发生及恢复期变化特点。方法 采用彩色多普勒超声心动图检查广州市儿童医院收治的KD患儿，对伴冠状动脉损害者进行随访，KD 患儿恢复期接受三磷腺苷负荷超声心动图检查。按急性期冠状动脉扩张的程度，将KD患儿分为冠状动脉扩张组、中小型冠状动脉瘤组和巨大型冠状动脉瘤组，分别比较其发病及变化特点。结果 共纳入KD患儿356例，冠状动脉损害发生率为41.3％（147/356）；巨大型和中小型冠状动脉瘤组确诊前发热时间明显长于冠状动脉扩张组［(18.5±12.0) d、(12.9±8.2) d vs (7.4±3.6) d， P均<0.01］］。有119例（81.0％，119/147）冠状动脉损害患儿定期门诊随访。随访2年，超声心动图显示冠状动脉扩张组72例患儿冠状动脉全部恢复正常；中小型冠状动脉瘤组77.8%（28/36）患儿冠状动脉恢复正常；巨大型冠状动脉瘤组11例患儿无一例恢复正常。40例冠状动脉有损害的KD患儿恢复期三磷腺苷负荷超声心动图检查结果显示中小型和巨大型冠状动脉瘤组室壁运动异常、冠状动脉血流储备下降的发生率显著高于冠状动脉扩张组（P< 0.05）。结论 KD患儿急性期冠状动脉扩张损害为一过性；大部分中小型冠状动脉瘤改变可恢复；巨大型冠状动脉瘤造成的损害可长期存在。超声心动图和三磷腺苷负荷超声心动图对追踪观察KD冠状动脉损害的变化及心肌供血有重要帮助。     

感染因素对川崎病及其冠状动脉病变的影响

目的 探讨感染因素对川崎病(KD)及其冠状动脉病变的影响。 方法 回顾性收集首都医科大学附属北京儿童医院2005年1月至2014年12月出院诊断中包含KD的病历,截取人口学信息、临床发现、急性期和后遗症期超声心动图发现、转归,对每份病历重新行典型KD、不完全KD、IVIG抵抗、冠状动脉病变及其程度和合并感染的甄别。分为KD感染组、KD非感染组分析组间临床发现的差异,分为冠状动脉病变组、非冠状动脉病变组分析感染因素的差异。 结果 2 331例KD患儿进入分析。KD合并感染的发生率为57.1% (1 331/2 331),KD并发冠状动脉病变的发生率为36.0%(840/2 331)。KD感染组与KD非感染组年龄、发热天数及不完全KD比例差异有统计学意义(P均<0.05);冠状动脉病变组与非冠状动脉病变组肺炎支原体感染及消化系统感染伴发腹泻比例差异有统计学意义(P均<0.05)。 结论 合并感染的KD患儿年龄多<1岁,易表现为不完全KD,合并肺炎支原体感染及消化系统感染伴发腹泻的KD患儿易发生冠状动脉病变。     

儿童再发川崎病77例临床特征回顾性分析

目的 探讨再发川崎病(KD)的临床特点及其预后。方法 收集1994年1月至2012年10月重庆医科大学附属儿童医院收治的KD再发病例,比较初发时和复发时的临床特征、实验室指标和随访资料。并选取5～10年未再发KD病例作为对照组,探讨KD再发可能的危险因素。结果 19年间收治KD 4 875例,其中再发KD 77例,再发1次74例,再发2次3例,男∶女为1.4∶1。再发平均间隔时间1.6年,1年以内再发45.4%（35/77）。发热病程再发时较初发时缩短 (7.6±3.1) vs ( 8.9 ± 3.8) d,P＜0.05;WBC和CRP水平再发时较初发时显著降低,(14.3±5.7) vs (16.2 ±5.4)×109·L-1和(61±58) vs ( 95±76) mg·L-1,P均<0.05。急性期（病程≤30 d）冠状动脉病变（CAL ）发生率初发时为17.8%（13/73) ,再发时为13.3%( 10/75);应用IVIG后亚急性期（病程>30 d）CAL发生率初发时为11.0%（8/73),再发时为9.3%（7/75),组间比较差异均无统计学意义。7例初发时与再发时均有CAL,其中1例初发时与再发时均合并冠状动脉瘤（CAA）。52例KD再发患儿有出院后随访资料,随访时间平均2.1年,其中1 例再发时合并左侧冠状动脉主干小型CAA的患儿随访中出现新发部位左前降支瘤样扩张,冠状动脉内径回缩至正常后4年7个月再次出现左侧冠状动脉扩张。多因素Logistic分析显示,年龄＜3岁、性别、热程＞10 d、并发CAL及WBC>20×109·L-1均与KD再发无统计学关联。结论 KD再发多发生在1年内。再发KD的CAL总发生率并未升高,初发时合并CAL患儿,再发时更易发生CAL。     

不完全川崎病136例

目的探讨不完全川崎病(KD)的临床特点。方法对不完全KD 136例及典型KD 443例的临床资料进行回顾性分析。结果两组年龄、性别、临床表现及各项实验室检查方面均无明显差异,但在冠状动脉病变发生率方面,不完全KD(18.4%)明显高于典型KD(11%)。结论不完全KD冠状动脉病变发生率高。临床应重视早期诊断和早期治疗。     

儿童不完全川崎病的临床特点分析

目的 探讨不完全川崎病 （IKD）患儿的临床特点,为早期诊断该病提供可借鉴的经验。方法 回顾性分析22例住院IKD患儿的临床资料,并与同期住院的63例川崎病 （KD）患儿和20例发热患儿进行对比研究。结果 所有患儿均有发热,IKD组患儿的肢端变化、结膜充血及颈部淋巴结肿大的发生比例均显著低于KD组 （均P < 0.05）;且IKD组患儿的血清谷丙转氨酶显著高于KD组 （P < 0.05）,而血浆白蛋白、血清钠、白介素6水平均显著低于KD组 （均P < 0.05）。IKD组丙球使用率与KD组无差异,而IKD组冠脉损害的发生率却显著高于KD组 （P < 0.05）。结论 IKD患儿的症状体征均不典型。肝功能检测及血钠、白介素6水平检测可能有助于IKD的诊断。     

76例小儿川崎病临床分析

目的 总结川崎病(KD)的临床特征,探讨不完全性KD的诊断及治疗.方法 回顾性分析2001年1月～2006年5月76例住院KD患儿的临床资料:比较典型KD与不完全性KD的临床特征,总结不完全性KD的主要临床特征及治疗.结果 76例中,典型KD 51例(67.1%),不完全性KD25例(32.9%).典型KD引起冠状动脉病变有34例,发生率为66.7%,不完全性KD冠状动脉发生病变有17例,发生率为68%,两组比较无显著性差异.典型KD急性期用大剂量IVIG及阿司匹林治疗可取得较好的效果.不完全性KD明确诊断较晚,病情已发展到中晚期,超过用IVIG治疗的时间,一般单用阿司匹林治疗.结论 不完全性KD的发生率较高,典型KD与不完全性.KD所致的冠状动脉损害无显著性差异.肛周脱屑和颈部淋巴结肿大可以作为不完全性KD的早期诊断依据之一.实验室指标的改变非常有助于不完全性KD的诊断.不完全性KD急性期可用IVIG+阿司匹林治疗,中晚期可单用阿司匹林治疗.     

川崎病患儿冠状动脉血流储备测定与影像形态学诊断的对照研究

目的评价持续冠状动脉病变对川崎病（KD）患儿冠状动脉血液动力学的影响, 探讨冠状动脉血流储备（CFR）检测在KD患儿冠状动脉病变远期随访中的应用价值。方法选择因胸痛、心悸以及各类偶发早搏的患儿为对照组，KD并发持续左冠状动脉病变患儿为KD组，均予以ATP 160 μg· kg-1·min-1持续静脉注射，共6 min。运用经胸多普勒超声心动图（TTDE）检测冠状动脉左前降支(LAD) 在ATP注射前后的舒张期峰值流速(PDV)及平均流速(MDV)，并通过计算比值得出相应CFR（PDV）和CFR（MDV）值。结果 对照组纳入25例，CFR（PDV）和CFR（MDV）值均与年龄呈显著正相关(r=0.596, 0.591, P=0.002, 0.002)；<2岁、～8岁和>8岁组的CFR (PDV) 95%参考值范围分别为≥1.22、≥1.94和≥2.22。KD组纳入8例，静息状态下PDV和MDV值分别为（50.0±15.07） cm· s-1和(35.16±11.52) cm· s-1，显著高于对照组(P=0.004, 0.015)；而CFR（PDV）和CFR（MDV）值明显降低，分别为（1.67±0.38） cm· s-1和（1.69±0.47） cm· s-1(P=0.002,0.018)。与对照组比较，KD组7/8例(87.5%)患儿CFR（PDV）值显著降低。结论持续冠状动脉病变可导致KD患儿CFR值显著降低，TTDE检测患儿CFR具无创、安全和便捷的优点，对于KD冠状动脉病变的长期随访与临床诊断治疗策略的选择具有指导意义。     

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。     

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］     

Complete and incomplete Kawasaki disease: two sides of the same coin

We sought to determine differences in clinical presentation, treatment response and coronary artery outcomes between complete and incomplete KD. All patients treated for KD between January 1990 and April 2007 were reviewed. Patients were classified as having an incomplete presentation if presenting with fever ≥ 5 days and <4 "classic" KD clinical signs. A total of 955 patients were included. Incomplete clinical presentation was seen in 217 cases (23%). Patient's demographic, clinical and laboratory characteristics were similar between groups with few exceptions. Patients presenting with incomplete KD had a longer median interval from symptom onset to diagnosis (7 [4-17] versus 6 [6-13] days, p < 0.001) and were less likely to be treated with intravenous immunoglobulin (86% versus 96%, p < 0.001). No significant difference between groups were seen in regard to coronary artery abnormalities (incomplete 13% versus complete 11%, p = 0.58). CONCLUSION: Complete and incomplete KD appear to be different sides of the same coin, differing only in the number of signs and symptoms at presentation. Similar laboratory findings and coronary artery outcomes between the two groups support this conclusion.     

Afebrile Kawasaki disease with coronary artery dilatation

Herein we describe the cases of two afebrile patients who were thought to have Kawasaki disease (KD). Patient 1 was a 7‐month‐old‐Japanese girl. She presented with bulbar conjunctival injection, diarrhea, skin erythema, and redness around the bacillus Calmette–Guerin (BCG) inoculation site. Thirteen days after the first symptoms, ultrasonic cardiogram (UCG) showed dilatations of the bilateral coronary arteries (CA). The dilatations had completely resolved 5 months later. Patient 2 was a 13‐month‐old Japanese boy. He first presented with bulbar conjunctival injection and redness around the BCG inoculation site. Twenty‐two days after the first symptoms, UCG indicated bilateral and peripheral CA dilatations. The mild dilatations of the proximal CA remained. Although fever is the principal symptom of KD, some incomplete KD patients may be afebrile. Although it is difficult to diagnose these patients as having KD, redness at the BCG inoculation site may be a clue to the diagnosis.     

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。     

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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.     

Coronary artery lesions of incomplete Kawasaki disease: a nationwide survey in Japan

Incomplete Kawasaki disease (KD) is associated with delayed diagnosis and treatment, which in turn can lead to the development of coronary artery lesions (CALs). The aim of this study was to determine the epidemiological features of incomplete KD compared with complete KD and to identify risk factors for CALs from incomplete KD patients using data from a nationwide survey of 2007–2008 in Japan. A total of 23,263 patients were classified according to the number of principal clinical signs: 80% (n = 18,620) had complete forms of KD, 14.2% had four principal signs, 4.6% had three signs, and 1.2% had only one or two signs. In comparison with complete KD cases, the prevalence of CAL development tended to be larger and the proportion receiving initial intravenous immunoglobulin (IVIG) treatment were significantly smaller in patients with incomplete forms. In addition, hospital attendance after 7 days of illness or later was significantly associated with CAL development in all incomplete groups (OR: 2.52 in total patients with incomplete KD, 3.26 in those with one or two principal signs, 2.94 in those with three signs, 2.35 in those with four signs). Conclusion The higher prevalence of CALs in incomplete KD reflects difficulties in diagnosis and delays in treatment. More timely diagnosis and treatment of incomplete KD patients could further prevent the development of cardiac lesions.     

儿童再发川崎病临床特征的Meta分析

目的 探讨儿童再发川崎病（KD）的临床特点,以提高对再发KD的认识。方法 检索PubMed、Web of Science、Embase、中国知网、万方医学网和中国科技期刊数据库中关于儿童再发KD与初发时临床特点的对照研究,根据纳入、排除标准筛选文献,采用RevMan 5.3软件进行Meta分析。根据异质性检验结果选择相应效应模型进行数据合并,计算各观察指标合并比值比（OR）或加权均数差（WMD）及其95%CI。结果 最终纳入9个病例对照研究,KD患儿样本总量为12 059例,其中再发KD患儿206例（男127例,占61.7%;女79例,占38.3%）。Meta分析结果显示,与初发时相比,再发时发热时程缩短（WMD=-1.81,95%CI：-2.99~-0.64）,手足硬肿率降低（OR=0.46,95%CI：0.26~0.80）,差异均具有统计学意义（P < 0.05）;KD患儿再发时与初发时冠状动脉病变发生率的比较差异无统计学意义（OR=1.34,95%CI：0.84~2.14,P=0.22）。结论 目前证据显示再发KD患儿热程更短,手足硬肿率较低;KD再发以男童多见;再发患儿冠状动脉病变发生风险未见明显增高。     

Kawasaki disease in New Zealand

AIM: To determine the epidemiology, management and outcome of Kawasaki disease (KD) in New Zealand. DESIGN: Prospective audit using New Zealand Paediatric Surveillance Unit (NZPSU) Reports. SETTING: Single country 2-year epidemiological study. Patients: All patients diagnosed with KD in New Zealand reported to the NZPSU from January 2001 to December 2002. MAIN OUTCOME MEASURES: Incidence of KD; time to diagnosis; use of intravenous immunoglobulin; cardiac features and outcome. RESULTS: Forty-nine new cases were identified. The annual incidence was 8.0 cases/100,000 children aged less than 5 years. Age at onset was less than 5 years in 86% of cases. Incidence was 4.6/100,000 for children of European origin, 9.6 for Maori, 12.2 for Pacific Islanders and 32.2 for children of East Asian origin. KD was diagnosed at a median of 6 days from onset of illness. 89% had fever and four or more diagnostic features. All patients had at least one echocardiogram: There was one small (2%) coronary artery aneurysm only; 13 (26%) had mild coronary artery dilatation. Thirty-five per cent did not have an echocardiogram performed four or more weeks from illness onset. 45 (92%) cases received intravenous immunoglobulin at median day six. There was one death due to occlusive coronary artery disease in a 3-month-old boy with atypical symptoms in whom KD was diagnosed at post-mortem. CONCLUSIONS: The incidence of KD in New Zealand is defined with significantly variable risk according to ethnicity. Most patients received appropriate rapid diagnosis and treatment but there was considerable variation in practice in regard to number and timing of echocardiograms. There was a low coronary artery aneurysm rate (2%). Accelerated vaso-occlusive disease was responsible for the single fatality in an atypical case.