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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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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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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...     

川崎病合并冠状动脉瘤63例临床分析

目的分析川崎病(KD)合并冠状动脉(以下简称冠脉)瘤患儿的临床特点。方法对首都医科大学附属北京儿童医院2000—2007年收治的63例超声心动图诊断为冠脉瘤的KD患儿临床资料、实验室检查、超声及心电图检查结果、治疗情况及随诊资料进行回顾性分析。结果(1)冠脉瘤患儿男性明显多于女性,男∶女为5.3∶1;冠脉巨大瘤男女比例为8.3∶1;<1岁患儿多发,占28.6%。(2)本组患儿中不完全KD、静脉注射丙种球蛋白(IVIG)抵抗以及KD复发的发生率均较高,分别为36.5%、30.2%和7.9%;急性期57例(90.5%)患儿使用IVIG冲击治疗,3例未用,3例使用情况不详;36例(57.1%)患儿发病至丙种球蛋白应用的时间间隔大于10 d。(3)超声检查发现小冠脉瘤患儿7例,中等冠脉瘤19例,巨大瘤37例,左冠脉受累者占76.2%,其中58.3%发生在前降支;右冠脉受累者达87.3%,其中47.3%发生在右冠Ⅱ段;双侧冠脉同时受累者占63.5%。(4)随诊发现71.4%冠脉瘤呈现回缩趋势,45.2%的受累分支冠脉瘤消退,平均消退时间为(2.1±1.5)年。结论对于男性、发病年龄<1岁、不完全KD、发生IVIG抵抗、复发患儿及应用IVIG治疗较晚患儿要警惕冠脉瘤的发生;左冠前降支及右冠脉瘤样病变最多见,多数冠脉瘤在恢复期发生回缩。     

Neonatal kawasaki disease

The authors report a case of Kawasaki disease that manifested in a newborn and presented at the age of 6 weeks of age with coronary artery aneurysms     

547例川崎病临床分析及伴冠脉病变高危因素探讨

目的 总结川崎病(Kawasaki disease,KD)的临床特征,探讨KD临床表现与预后的关系,了解KD并发冠状动脉病变(coronary artery lesion,CAL)的危险因素.方法 同顾性分析1990年1月至2006年10月间547例住院KD患儿的临床资料,比较典型与不典型KD的临床特征,并探讨KD并发冠状动脉病变的高危因素.结果 547例中典型KD 434例,不典型KD 113例,不典型KD CAL发生牢较高(P＜0.05);除肛周脱屑外,其他临床症状发生少,出现晚(P＜0.05).547例KD中并发CAL 103例(18.82%),相关因素分析显示发病年龄、性别、血沉、血小板、C-反应蛋白与CAL发生率有密切关系.结论 不典型KD的CAL发生率较高,肛周脱屑较其他临床症状出现早,可以作为早期诊断线索之一;3岁以下的男孩及血沉、血小板、C-反应蛋白明显升高是CAL的高危因素.     

川崎病患儿血浆内皮素检测与冠状动脉病变

目的　了解川崎病 (KD)患儿血浆内皮素 (ET)在急性期和亚急性期变化 ,及其与KD及并冠状动脉病变的关系。方法　检测 5 0例KD患儿急性期和亚急性期血浆ET含量 ,并以 30例急性感染及 30例健康儿童作对照组。结果　 1.KD组亚急性期血浆ET较急性期显著增高 ;2 .KD组与健康对照组比较 ,急性期和亚急性期血浆ET均显著增高 ;与感染对照组相比 ,仅亚急性期显著增高 ;3.彩超探及KD组 5 4 %患儿冠状动脉扩张(CAD) ,其亚急性期血浆ET较无CAD组显著增高。结论　KD患儿亚急性期ET进一步增高 ,对发生冠状动脉及其他心血管并发症有一定提示 ,同时应作相关检查     

川崎病并发巨大冠状动脉瘤的远期预后研究

目的探讨川崎病(KD)并发GCAA患儿的远期预后,为进一步探索KD引起心血管损害后期的诊断治疗提供依据。方法选取2001年5月至2006年5月在广州市妇女儿童医疗中心儿童医院诊断为KD并发冠状动脉损害的患儿为研究对象。依据冠状动脉损害严重程度分为冠状动脉扩张(CAD)组、中小冠状动脉瘤(CAA)组和巨大冠状动脉瘤(GCAA)组。随访起止时间为急性期出院后至2009年5月。所有纳入随访的患儿均于急性期出院后6个月、1年、2年和3年各随访超声心动图1次,观察冠状动脉形态;对急性期3个月后常规超声心动图提示冠状动脉形态未恢复患儿行ATP负荷超声心动图及冠状动脉造影检查,观察心脏室壁运动、左右冠状动脉血流储备和冠状动脉分支病变情况。死亡患儿行尸解及冠状动脉病理学检查。结果CAD组纳入166例,中小CAA组纳入59例,GCAA组纳入15例。①随访3年时超声心动图显示GCAA组2/15例(13.3%)恢复正常,显著低于CAD组和中小CAA组(P均0.001);GCAA组1例(6.7%)显示冠状动脉扩张;10例(66.7%)表现为CAA,其中3例为GCAA,3例合并冠状动脉狭窄或闭塞。②ATP负荷超声心动图显示,GCAA组室壁运动异常的发生率显著高于CAD组(6/9例vs8/55例,P0.001),与CAA组差异无统计学意义(6/9例vs17/31例,P0.05);非参数检验提示,3组间室壁运动异常发生率有统计学差异,以GCAA组发生率较高(Z=-4.2,P0.001)。③GCAA组6/15例行冠状动脉造影,4/6例仍存在CAA,1/6例GCAA退缩为轻度CAD。5/6例出现冠状动脉狭窄或(和)闭塞,其中1例闭塞后再通,1例合并侧支血管形成。④GCAA组死亡2例,尸解冠状动脉大体可见,左右冠状动脉各分支呈串珠样膨大,瘤样扩张的冠状动脉管腔内可见充满暗红色血栓样物质,管壁显著增厚。冠状动脉分支和前降支病理学检查均显示:内膜增厚,炎症细胞浸润。结论KD并发GCAA患儿的预后较差,冠状动脉损害可持续存在;后期可发生冠状动脉狭窄或闭塞,导致缺血性心脏病;常规超声心动图与ATP负荷超声心动图、冠状动脉造影结合应用,对KD并发GCAA的远期随访有重要意义。     

Hemodynamic factors of thrombus formation in coronary aneurysms associated with Kawasaki disease

BACKGROUND: We studied the mechanism of thrombus formation in coronary aneurysms based on rheological findings. METHODS: We studied 43 coronary aneurysms in 33 patients with Kawasaki disease (mean (+/- SD) age 6.1 +/- 4.3 years). These lesions were divided into three groups on the basis of maximum diameter: (i) small (group S); (ii) medium sized (group M); and (iii) large (group L) aneurysms. Using a Doppler flow guidewire and a pressure-monitoring guidewire, we measured coronary flow velocity and perfusion pressure inside aneurysms and in adjacent normal-looking vessels. We calculated the average peak velocity (APV) index, the mean coronary perfusion pressure (P) index and shear index. RESULTS: The APV index and shear index decreased significantly (p < 0.005) as the aneurysm size increased (APV index in groups S, M and L was 0.893 +/- 0.149, 0.573 +/- 0.242 and 0.128 +/- 0.131, respectively; shear index in groups S, M and L was 0.750 +/- 0.149, 0.328 +/- 0.153 and 0.020 +/- 0.028, respectively). However, coronary perfusion pressure showed no relationship to aneurysm size and was not significantly different from that in adjacent normal-looking vessels. CONCLUSIONS: The results of the present study suggest that the stagnation of flow and the reduction of shear stress in coronary aneurysms could initiate thrombus formation.     

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.     

川崎病并发巨大冠状动脉瘤急性期临床特征与危险因素分析

目的以川崎病(KD)并发巨大冠状动脉瘤(GCAA)的病例对照研究,分析KD急性期的临床特征,并探讨并发GCAA的危险因素。方法选取2001年5月至2009年5月在广州市妇女儿童医疗中心儿童医院住院的KD并发GCAA患儿为GCAA组;选取同期KD并发中小冠状动脉瘤(CAA)患儿为对照组。对两组患儿的临床特征进行比较,对可能影响GCAA发生的因素进行单因素分析,并进行多因素Logistic逐步回归分析。结果GCAA组纳入22例,其中男19例,女3例,发病年龄3个月至10岁,平均(2.9±2.8)岁。中小CAA组纳入65例,其中男51例,女14例。Pearson χ2检验结果提示,年龄≤6个月或≥5岁、发热时间>14 d、延误诊断、确诊前单独使用糖皮质激素、Hb降低、ESR升高和ALB降低与GCAA发生相关(P均<0.05)。多因素Logistic逐步回归分析提示,延误诊断（OR＝2.998,95%CI：1.004～8.950,P=0.047),确诊前单独使用糖皮质激素（OR＝6.556,95%CI：1.561～28.542,P=0.010),ESR≥100 mm·h-1(OR＝3.591,95%CI：1.164～11.079,P=0.026)为发生GCAA的独立危险因素。结论延误诊断、确诊前单独使用糖皮质激素和ESR≥100 mm·h-1为KD并发GCAA的独立危险因素。     

A case of Kawasaki disease with coronary artery aneurysms documenting Yersinia pseudotuberculosis infection

A case of a 5-year-old boy who fulfilled all the criteria for Kawasaki disease (KD) was described. He had associated bilateral coronary artery aneurysms. Our study revealed the isolation of Yersinia pseudotuberculosis in stool cultures, and the elevation and seroconversion of the agglutination antibody titres, and hence he was diagnosed as Y. pseudotuberculosis infection-positive. We also demonstrated the positive mitogenic activity of the culture supernatant of the isolated bacterium from the patient and detected Y. pseudotuberculosis-derived mitogen by PCR. This case therefore suggests that Y. pseudotuberculosis might be closely related to the cause of KD.