���鲡����׳���ļ�����

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     

���鲡����Ѫ����Ƥϸ���������ٴ��������

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...     

���鲡���������ƽ�չ

??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.     

���鲡�ϲ���״������63���ٴ�����

??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.     

���鲡������״������19���ٴ�����

??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.     

���鲡���������ϩE4�ⶨ���� �ٴ��������

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。     

���鲡������״������Ԥ���о�

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...     

A multicenter,randomized, controlled trial of intravenous gamma globulin therapy in children with acute Kawasaki disease

We studied the effect of intravenous, polyethyleneglycol-treated, human immunoglobulin, administered at 200 mg/kg per day (group A: n = 147; male 86, female 61; age < 1 year, 50) or 400 mg/kg per day (group B: n = 152; male 87, female 65; age < l year, 52) for five consecutive days and compared it with freeze-dried, sulfonated human immunoglobulin [group C: n = 152; male 87, female 65; age < 1 year, 51), administered at 200 mg/kg per day for five consecutive days, on the prevention of coronary artery abnormalities in Kawasaki disease. Echocardiograms were interpreted blindly and independently. Proportions of 87.1%, 95.4%, and 82.3% in groups A, B, and C, respectively, had no coronary artery abnormalities. The confidence limits of difference between the proportions of groups A and C, groups B and C, and groups B and A were −4.4% and 10.4%, 7.8% and 15.9%, and 4.0% and 10.8%, respectively. Duration of fever and serum immunoglobulin G (IgG) levels were correlated with the prevalence of coronary artery abnormalities. We concluded that intravenous, polyethyleneglycol-treated, human immunoglobulin and freeze-dried, sulfonated human immunoglobulin had clinically equivalent effects on coronary artery abnormalities, and that five daily doses of 400 mg/kg of intravenous, polyethyleneglycol-treated, human immunoglobulin is more effective than that of 200 mg/kg gamma globulin.     

Efficacy of intravenous immune globulin therapy combined with dexamethasone for the initial treatment of acute Kawasaki disease

We studied the effects of a new regimen consisting of intravenous immune globulin (IVIG) combined with dexamethasone (DEX) on clinical outcome and serum levels of vascular endothelial growth factor (VEGF) in the initial treatment of Kawasaki disease (KD). A total of 46 KD patients received 0.3 mg/kg per day DEX plus heparin i.v. for 3 consecutive days, together with 2 g/kg IVIG over 4 to 5 days (DEX group). Low-dose acetylsalicylic acid was started after completion of DEX therapy. The control group consisted of 46 KD patients retrospectively treated earlier with 2 g/kg IVIG over 4 to 5 days plus higher dose acetylsalicylic acid (CONTROL group). No serious adverse effect was noted in either group. There were no differences in baseline and post-treatment laboratory data except for C-reactive protein between the groups. Post-treatment C-reactive protein in the DEX group (median 0.9 mg/dl, range 0.0 to 24.7 mg/dl) was lower than that (1.2 mg/dl, range 0.2 to 19.5 mg/dl) in the CONTROL group ( P =0.033 by Mann-Whitney U test). In addition, the mean duration of fever after the first IVIG infusion was 2.2 days (median 1 day, range 1 to 12 days) in the DEX group and 2.8 days (2 days, 1 to 16 days) in the CONTROL group ( P =0.015 by Mann-Whitney U test). The new regimen did not reduce VEGF levels. Two patients in each group developed small- or medium-sized coronary artery aneurysms. Conclusion:although this regimen did not affect coronary outcome, intravenous immune globulin therapy combined with dexamethasone for the initial treatment of Kawasaki disease was safe and may accelerate the resolution of systemic inflammation.Abbreviations CAA coronary artery aneurysms - DEX dexamethasone - IVIG intravenous immune globulin - KD Kawasaki disease - VEGF vascular endothelial growth factor     

Effect of corticosteroids in addition to intravenous gamma globulin therapy on serum cytokine levels in the acute phase of Kawasaki disease in children

OBJECTIVE: The aim of this multicenter prospective and randomized study was to determine the effect of adding corticosteroids to intravenous gamma globulin (i.v.GG) therapy on serum cytokine levels, as well as to see its effect on the clinical course in children in the acute phase of Kawasaki disease (KD). STUDY DESIGN: Patients with KD (n=32) were randomized to receive either i.v.GG alone (G group) or i.v.GG plus corticosteroids (G+S group). The clinical course and cytokine responses between groups were compared. RESULTS: The pretreatment serum levels of interleukin (IL)-2, IL-6, IL-8, and IL-10 were significantly higher in patients with KD than in healthy controls. Although i.v.GG alone failed to reduce cytokine concentrations within 24 hours of i.v.GG administration, corticosteroids plus i.v.GG reduced IL-2, IL-6, IL-8, and IL-10 levels. The levels of IL-2, IL-6, IL-8, and IL-10 within 24 hours after initiating i.v.GG therapy were significantly lower in the G+S group than in the G group. The duration of fever was shorter, and the C-reactive protein concentration decreased more quickly in the G+S group than in the G group. CONCLUSIONS: These findings suggest that corticosteroids rapidly ameliorate symptoms by reducing cytokine levels in children with KD.     

Early intravenous gamma-globulin treatment for Kawasaki disease: the nationwide surveys in Japan

OBJECTIVE: To determine the optimal period of intravenous gamma-globulin (IVGG) treatment, using the database from nationwide Kawasaki disease surveys in Japan. STUDY DESIGN: We selected patients who first visited a doctor within 3 days of illness and received IVGG treatment within 9 days of illness. We divided these patients into 2 groups: an early group (treated on days 1-4: 4731 cases) and a conventional group (days 5-9: 4020 cases). We compared the rate of additional IVGG and prevalence of cardiac sequelae between these groups. RESULTS: The rate of additional IVGG in the early group was significantly higher than those of the conventional group (OR, 1.12 [95% CI, 1.10-1.16]). There were no significant differences in cardiac sequelae between the two groups. CONCLUSIONS: There is no evidence that IVGG treatment on day 4 or earlier has greater efficacy in preventing cardiac sequelae than treatment on days 5 to 9. In addition, early treatment is likely to result in a greater requirement for additional IVGG. However, there is also no evidence that early treatment increases the prevalence of cardiac sequelae in a clinical practice setting, where additional IVGG can be given to those whose initial treatment fails.     

联合糖皮质激素初始治疗川崎病的临床分析

目的 探讨糖皮质激素在联合静脉丙种球蛋白和阿司匹林传统治疗方面的优势,探索激素运用在整个病程中的合适时机和合适剂量.方法 参照川崎病诊断标准,将363例川崎病患儿随机分为常规治疗组和联合激素组,将联合激素组根据临床表现分为完全川崎病组和不完全川崎病组,分别分析治疗前和治疗1周内两组患儿发病年龄、性别、治疗应用后发热病程、丙种球蛋白治疗时间、住院天数、总住院费用;以及患儿病程急性期,恢复期中有关化验指标,1月后冠状动脉损害情况.结果 联合激素组比常规治疗组在治疗后热程(P<0.05)、住院天数(P<0.05)和住院费用(P<0.05)中,有明显减少,两者差异有统计学意义,在各项临床化验指标及冠状动脉损害病变中无明显统计学意义(P值大于0.05).在不同类型川崎病中,完全组激素治疗较不完全组同样在治疗后热程(P<0.02),住院天数(P<0.03)和住院费用(P<0.04)方面显著减少,两者对比存在统计学意义,而在各项临床化验指标及冠状动脉损害病变中无明显统计学意义(P>0.05).结论 联合激素治疗对缩短川崎病治疗后热程及患儿经济负担方面有积极作用.     

Incidence and clinical features of incomplete Kawasaki disease

During the nine-year period from 1983 to 1991, a total of 242 patients (142 males and 100 females) presenting with Kawasaki disease were seen at one hospital. Among them, 25 (10%) patients demonstrated incomplete Kawasaki disease and 17 of these 25 (68%) lacked two of the six principal symptoms of Kawasaki disease, with the most frequently missing symptoms being cervical lymphadenopathy and polymorphous exanthema. The typical laboratory features of Kawasaki disease, such as elevated erythrocyte sedimentation rate, leukocytosis, anemia, positive C-reactive protein and thrombocytosis were also seen in the incomplete cases. None of the 25 patients underwent iv gamma-globulin therapy while in 1 (4%), transient dilatation of the coronary artery was recognized. Incomplete Kawasaki disease may therefore be characterized by a less frequent association of rash, cervical lymphadenopathy and coronary involvement.     

Time interval of measles vaccination in patients with Kawasaki disease treated with additional intravenous immune globulin

A standard treatment with a dose of 2 g/kg of intravenous immune globulin (IVIG) is sometimes ineffective in some patients with Kawasaki disease (KD), who are commonly treated with additional IVIG. An interval of 6 to 7 months in Japan and 11 months in the United States is recommended before vaccination against measles after IVIG treatment for KD, but it is not known how long after treatment with additional IVIG. We studied the persistence of measles antibody titers in six episodes of KD in five patients without history of measles infection or vaccination, 4 to 28 months of age, after additional infusion of IVIG. The total dose was 4 g/kg in five episodes and 6 g/kg in one episode. Enzyme immunoassay antibody titers against measles were still positive (400) in all patients tested 3 months after additional infusion of IVIG and positive in one patient and equivocal (200 and <400) in three patients after 6 months, but negative (<200) in all after 9 months following infusion. Neutralization test antibody titers against measles were still positive (1:4) in all patients 3 months after additional infusion of IVIG, but only one after 6 months, and negative (<1:4) in all after 9 months following infusion. Conclusion: we suggest that an appropriate interval between infusion of 4 g/kg of intravenous immune globulin and measles vaccination be 9 months. The 11-month interval recommended in the United States for 2 g/kg may be longer than necessary.Abbreviations EIA enzyme immunoassay - HI hemagglutination inhibition - IVIG intravenous immune globulin - KD Kawasaki disease - NT neutralization test     

再次应用静脉丙种球蛋白无反应川崎病的预测因素分析

目的 探讨再次应用丙种球蛋白（简称丙球）无反应川崎病(KD)的危险因素及预测指标。方法 收集2010年1月至2015年1月在重庆医科大学附属儿童医院收治的初次丙球无反应并接受再次丙球治疗的KD患儿,分为再次丙球有反应组和无反应组,对2组的临床特征及实验室指标进行回顾性分析。结果 81例KD患儿进入分析,再次丙球有反应组占75.3%（61/81）,无反应组占24.7%（20/81）。两组性别、年龄、初次丙球治疗前后临床表现和2次丙球使用间隔时间差异均无统计学意义。在初次丙球使用前,冠状动脉病变发生率、血常规（WBC、PLT、Hb、N）、CRP、肝功能、电解质等指标再次丙球有反应组和无反应组差异均无统计学意义。再次丙球治疗前与初次应用丙球前,血常规和CRP指标差值再次丙球有反应组和无反应组差异均无统计学意义。结论 初次丙球使用前的临床表现及实验室指标不能预测初次丙球无反应KD再次应用丙球是否有效,初次应用丙球后的临床表现及血常规、CRP指标对再次应用丙球是否有效亦无提示作用。