Septated pericarditis associated with Kawasaki disease: a brief case report

Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.     

Kawasaki disease with severe cardiac sequelae: lessons from recent New Zealand experience

OBJECTIVES: To review recent cases of Kawasaki disease (KD) with significant cardiac sequelae in New Zealand. It is known that intravenous immunoglobulin (IVIG) reduces the risk of coronary artery aneurysm formation if given within 8-10 days of onset of KD. METHODS: Retrospective review of medical course, criteria for KD, laboratory and cardiac findings for six children identified with KD and significant coronary artery sequelae. RESULTS: There was delay in diagnosis of KD in three of the six children. Three cases were atypical by extremes of age (2 months, 10 years, 14 years). By definition all six children had significant coronary artery involvement. One patient had a thrombus detected in a coronary aneurysm 3 weeks after KD. One patient underwent coronary artery bypass grafting for unstable angina 2 years after KD. One patient developed coronary artery aneurysms after an initial 'toxic shock' type illness evolving to KD. Three patients died, one due to rupture of a coronary artery aneurysm, two from rapid early coronary artery obstruction occurring at three and 4 months after initial KD. CONCLUSIONS: Kawasaki disease remains an important cause of mortality and morbidity for children. Diagnostic delay beyond 8 days reduces the chances of successful IVIG therapy in KD. Current studies supported by the Paediatric Surveillance Unit should establish the epidemiology of KD in New Zealand.     

Clinical responses of patients with Kawasaki disease to different brands of intravenous immunoglobulin

OBJECTIVE: To determine whether different brands of intravenous immunoglobulin (IVIG) administered to children with Kawasaki disease (KD) result in different outcomes. STUDY DESIGN: We analyzed children with KD and divided them into 4 groups according to the brand of IVIG. A coronary artery abnormality (CAA) was defined as having a lumen diameter (inner border to inner border) of > or =3 mm in KD cases <5 years old and > or =4 mm in cases > or =5 years old, and giant aneurysm was defined as a lumen diameter > or =8 mm. Patients were considered nonresponsive to IVIG therapy if fever persisted longer than 2 days after completion of treatment and needed retreatment with IVIG. RESULTS: We collected 437 cases, 29 (6.6%) were nonresponsive, 17 (3.9%) had CAA at convalescence, and 3 (0.7%) had giant aneurysm, 2 of whom had development of myocardial infarcts. Patients receiving Brand C IVIG, prepared with beta-propiolactone, had higher rates (10%, 9/93, P = .01) of CAA at convalescence and nonresponsiveness (13%, 12/93, P = .001); giant aneurysm occurred in 3/93 (3%) receiving Brand C IVIG and in 0/344 who received the other 3 brands (P = .008). CONCLUSIONS: IVIG, prepared with beta-propiolactone, was most significantly associated with nonresponsiveness, CAA at convalescence, and giant aneurysm. Physicians should be cautious when using IVIG prepared with beta-propiolactone or enzyme digestion to treat KD.     

Coronary Involvement in Infants with Kawasaki Disease Treated with Intravenous γ-Globulin

Aim To analyze the clinical spectrum and the incidence of coronary involvement in infants with typical Kawasaki's disease (KD). Patients and Methods A retrospective study was performed on children one year of age or younger diagnosed from February 1992 to January 2006 with typical KD. Children with incomplete forms of the disease were not included. Results Twenty-five infants were diagnosed with KD during the study period. The median age of the patients was 10 months (range, 4–12 months). All children but one received intravenous gammaglobulin (IVIG), 84% before the 10th day of disease. Seven patients (28%) required the administration of more than one dose of IVIG, because persistence of fever. Coronary artery disease (CAD) was recorded in 6 cases (24%), five of them being boys. All patients with CAD were treated with ASA plus IVIG and 84% of them received this therapy within the first 10 days of the KD onset. Conclusions In spite of the exclusion of our study of incomplete presentations and of an early administration of IVIG in our patients, we have observed a high rate of infants who developed CAD, which is similar to the one reported in children who do not receive IVIG.     

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。     

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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.     

川崎病并冠状动脉损害的危险因素

目的探讨川崎病(KD)并冠状动脉损害(CAL)的危险因素。方法收集1999年1月-2001年12月住院的145例KD患儿的临床资料,分析无CAL93例和CAL52例患儿的实验室检查资料及治疗、随访结果。结果无CAL组发热日程(8.7±3.4)d.血沉(79.5±34.6)mm/1h,CAL组发热日程(11.5±4.8)d,血沉(91.9±36.6)mm/1h,两组比较差异有显著性(P<0.01,0.05)。发病10d内接受静脉注射人血丙种球蛋白(IVIG)治疗患儿CAL发生率为18.8%(18/96),未予IVIG治疗患儿CAL发生率为69.4%(34/49),两者比较差异有显著性(P<0.001)。随访并CAL患儿38例,冠状动脉扩张20例均恢复正常;14例冠状动脉瘤中12例恢复正常,2例缩小;4例巨大冠状动脉瘤2例缩小,另2例超过2年仍未恢复。结论KD并CAL与热程过长及血沉显著增快密切相关,病程早期未予IVIG治疗的患儿并CAL的危险性明显增高,应用IVIG治疗对防治冠状动脉损害有重要作用。     

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。     

A meta-analysis on the effect of corticosteroid therapy in Kawasaki disease

The current recommended therapy for Kawasaki disease (KD) is the combination of intravenous immunoglobulin (IVIG) and aspirin. However, the role of corticosteroid therapy in KD remains controversial. Using meta-analysis, this study aimed to investigate the efficacy of corticosteroid therapy in KD by comparing it with standard IVIG and aspirin therapy. We included all related randomized and quasi-randomized controlled trials by searching Medline, the Cochrane Central Register of Controlled Trials, EMBASE, Pub Med, Chinese BioMedical Literature Database, China National Knowledge Infrastructure, and the Japanese database (Japan Science and Technology) as well as hand searches of selected references. Data collection and meta-analysis were performed to evaluate the effect of corticosteroids. Our search yielded 11 studies; 7 of which evaluated the effect of corticosteroid for primary therapy in KD, and 4 investigated the effect of corticosteroid therapy in IVIG-resistant patients. Meta-analysis of these studies revealed a significant reduction in the rates of initial treatment failure among patients who received corticosteroid therapy in combination with IVIG compared to IVIG alone (odds ratio (OR) = 0.50; 95% CI, 0.32~0.79; p = 0.003). Furthermore, the use of corticosteroids reduced the duration of fever and the time required for C-reactive protein to return to normal. Our data did not show any significant increase in the incidence of coronary artery lesions or coronary aneurysms (OR = 0.67; 95% CI, 0.35~1.28; p = 0.23) in the corticosteroid group. Conclusion. Corticosteroid combined with IVIG in primary treatment or as treatment of IVIG-resistant patients improved clinical course without increasing coronary artery lesions in children with acute KD.     

川崎病治疗前发热时间与丙种球蛋白耐药的相关性临床研究北大核心CSCD

目的 探讨川崎病(Kawasaki disease,KD)患儿丙种球蛋白(intravenous immunoglobulin,IVIG)治疗前发热时间与IVIG耐药的关系。方法 回顾性收集2018年1月至2020年12月收治KD患儿317例的病例资料,根据IVIG治疗前发热时间分为短热程组(发热时间≤4 d,n=92)和长热程组(发热时间>4 d,n=225),根据是否发生IVIG耐药将每组再分为耐药组和非耐药组。分析比较不同热程耐药组及非耐药组的基线资料及实验室结果,并采用多因素logistic回归分析IVIG耐药的影响因素。结果 短热程组中IVIG耐药19例(20.7%),并发冠状动脉瘤5例(5.4%);长热程组中IVIG耐药22例(9.8%),并发冠状动脉瘤19例(8.4%);短热程组IVIG耐药率明显高于长热程组(P<0.05),而冠状动脉瘤发生率在两组间差异无统计学意义(P>0.05)。短热程组中,耐药患儿治疗前血钠水平低于非耐药患儿,而降钙素原、C反应蛋白及N末端B型利钠肽原水平则明显高于非耐药患儿(P<0.05)。长热程组中,耐药患儿治疗前血钠及肌酸激酶水平低于非耐药患儿(P<0.05)。多因素logistic回归分析显示,血钠水平降低与长热程组KD患儿IVIG耐药有关(P<0.05)。结论 KD患儿IVIG耐药因IVIG治疗前发热时间不同而异。在治疗前发热时间>4 d的KD患儿中,血钠降低与IVIG耐药具有相关性。     

川崎病并发严重冠状动脉病变患儿旁路移植术术前评估及术后随访

目的回顾性总结川崎病(KD)并发严重冠状动脉病变患儿的影像学诊断、冠状动脉旁路移植术结果及随访情况。方法复旦大学附属儿科医院2006年8月至2008年3月收治5例(男3例,女2例)KD并发严重冠状动脉病变患儿,年龄12个月至10岁4个月。所有患儿均在KD急性期予IVIG治疗,其中3例予2次。病程第15～21天均行超声心动图(ECHO)检查发现冠状动脉病变;之后常规随访ECG和ECHO,并口服阿司匹林和双嘧达莫或氯吡格雷。1例患儿行64排螺旋CT(MSCT)检查,2例行99Tcm-MIBI心肌灌注显像检查。在病程6～65个月行选择性冠状动脉造影检查,并行冠状动脉旁路移植术及冠状动脉成形术。结果 1/5例在病程中有心绞痛发作,3/5例有心功能不全,1/5例无症状。1/5例有心肌梗死发生并在恢复过程有ECG记录;2/5例ECG有ST-T改变;2/5例ECG未见异常。ECHO除均显示多发性冠状动脉瘤(CAA)外,3/5例还显示左心房和左心室增大,左室射血分数(LVEF)和短轴缩短率的降低,2/5例左室壁运动不协调,与99Tcm-MIBI检查结果一致。冠状动脉造影均可见多发性CAA且至少一处为中等或巨大CAA,并伴有血栓形成和远端闭塞。MSCT与冠状动脉造影检查结果一致。年龄最小的1例患儿(手术时22个月)在冠状动脉移植术中死亡,其余4例术后即刻效果满意。在8～24个月的随访中,1例LVEF较低,随访1年未达到正常;1例术后18个月复发KD,治疗顺利,复查ECHO和MSCT未见冠状动脉进一步损害。4例患儿目前仍在随访中。结论 KD并发冠状动脉病变应定期随访ECG、ECHO,必要时进行99Tcm-MIBI和MSCT检查;如考虑手术需行冠状动脉造影仔细评估冠状动脉病变部位和程度。有心肌缺血表现需及时行冠状动脉旁路移植术和冠状动脉成形术,手术的近期效果显著,远期疗效有待长期随访结果。     

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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...     

静脉输注丙种球蛋白防治川崎病冠状动脉病变的疗效

目的评价静脉输注丙种球蛋白(IVIG)治疗和预防川崎病(KD)冠状动脉病变(CAL)的疗效,探讨IVIG疗效的影响因素。方法对314例KD患儿的临床资料进行回顾性对比观察。按治疗将患儿分为阿司匹林(ASA) IVIG组和ASA组,观察两组CAL发生、恢复情况、不同时机不同剂量IVIG治疗KD疗效、临床及实验室指标,急性期出现CAL者分别于病程1,3,6,12个月复查。结果ASA IVIG组CAL发生率34.3%,ASA组56.0%,两组比较P<0.001。应IVIG2.0g/kg或1.0g/kg以及在病程3～10d应用IVIG,CAL发牛率低,P<0.05。22.2?L发生在IVIG治疗后;13.4?L在病程12个月仍不能恢复正常,多数为IVIG治疗开始时间超过10d者。ASA IVIG组住院时间、退热时间、总热程缩短,血小板计数、血沉、C反应蛋白显著降低(P<0.05)。IVIG耐药病例占10.5%。结论IVIG治疗可显著缩短KD病程和降低CAL发生,但对川崎病CAL防治并非人们所预期的那样有效,实际疗效需要再评价。     

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.     

Re-treatment for immune globulin-resistant Kawasaki disease: A comparative study of additional immune globulin and steroid pulse therapy

BACKGROUND: We compared the efficacy and safety of additional intravenous immune globulin (IVIG) therapy with steroid pulse therapy in patients with IVIG-resistant Kawasaki disease. METHODS: Two-hundred and sixty-two consecutive patients had been treated with a single dose of IVIG (2 g/kg) and aspirin (30 mg/kg per day). Thirty-five patients (13.4%) were not clinical responders to the initial IVIG treatment. They received an additional IVIG treatment (1 g/kg) within 48 h after the initial treatment. Seventeen patients (6.5%) did not respond to the additional IVIG treatment. We randomly divided these patients into two groups: group 1 consisted of eight patients who were treated with a single additional dose of IVIG (1 g/kg), while group 2 consisted of nine patients who were treated with steroid pulse therapy. RESULTS: The IVIG-resistant patients had a high incidence of coronary artery lesions (CAL; 48.6%). Five patients (62.5%) in group 1 had CAL, including two patients who each had a giant aneurysm and three patients who each had a small aneurysm. Seven patients (77.8%) in group 2 had CAL, including two patients who each had a giant aneurysm, two patients who each had a small coronary aneurysm and three patients who each showed transient dilatation during steroid pulse therapy. There was no significant difference in the incidence of CAL between the two groups. The duration of high fever in group 2 (1.4~0.7 days) was significantly shorter than in group 1 (4.8~3.4 days; P<0.05). The medical costs for the treatment of patients in group 2 (113, 012 yen +/- 22,084) were significantly lower than those for group 1 (144,194 yen +/- 12,914; P<0.05). CONCLUSIONS: Steroid pulse therapy may be useful in the treatment of patients with IVIG-resistant Kawasaki disease who experience prolonged fever. However, transient dilatation of the coronary artery is observed during steroid pulse therapy, so careful echocardiographic examination should be performed for those patients receiving steroid pulse therapy for the sake of early detection of coronary artery abnormalities.     

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??Abstract??Objective??This study was aimed at evaluating present randomized controlled trials ??RCT?? regarding the drug therapy for intravenous immunoglobulin ??IVIG?? resistant Kawasaki disease ??KD??. Methods??According to inclusion and exclusion criteria?? articles were selected from medical electronic databases. RCT were then assessed based on the Juni assessment?? and meta-analysis was completed by the Review Manager 4.2 software. Indications to evaluate effects were the change of body temperature and change of coronary artery situation detected by ultrasound-cardiogram. The results were stated as relative risk ??RR?? or odd ratio ??OR???? with a 95% confidence interval ??CI?? and a P < 0.05 significant level. Results??In total?? three RCT were selected. Funel plot analysis showed possible publication bias. Meta-analysis of the three RCT?? including all 59 patients in the 2nd intravenous immunoglobulin treatment group and 66 patients in the glucocorticosteroid treatment control group?? indicated that after their first doses of IVIG treatment?? the temperatures of IVIG resistant KD patients who received 2nd IVIG treatment could be more effectively improved than those who received glucocorticosteroid treatment ??RR = 1.29??95% CI??1.10??1.52??P = 0.002???? but there were no differences on the incidence of coronary artery aneurysm between two groups ??OR = 0.91??95% CI??0.33??2.49??P = 0.85??. Conclusion??The Meta-analysis of currently published RCT demonstrates that the 2nd IVIG can more effectively improve the temperature than glucocorticosteroid in IVIG resistant KD patients?? but there were no differences on the incidence of coronary artery aneurysm between two groups.     

A multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome

OBJECTIVE: To investigate the role of corticosteroids in the initial treatment of Kawasaki disease (KD). STUDY DESIGN: Between September 2000 and March 2005, we randomly assigned 178 KD patients from 12 hospitals to either an intravenous immunoglobulin (IVIG) group (n = 88; 1 g/kg for 2 consecutive days) or an IVIG plus corticosteroid (IVIG+PSL) group (n = 90). The primary endpoint was coronary artery abnormality (CAA) before a 1-month echocardiographic assessment. Secondary endpoints included duration of fever, time to normalization of serum C-reactive protein (CRP), and initial treatment failure requiring additional therapy. Analyses were based on intention to treat. RESULTS: Baseline characteristics of groups were similar. Fewer IVIG+PSL patients than IVIG patients had a CAA before 1 month (2.2% vs 11.4%; P = .017). The duration of fever was shorter (P < .001) and CRP decreased more rapidly in the IVIG+PSL group than in the IVIG group (P = .001). Moreover, initial treatment failure was less frequent (5.6% vs 18.2%; P = .010) in the IVIG+PSL group. All patients assigned to the IVIG+PSL group completed treatment without major side effects. CONCLUSIONS: A combination of corticosteroids and IVIG improved clinical course and coronary artery outcome without causing untoward effects in children with acute KD.     

Kawasaki disease: review of risk factors for coronary aneurysms

Between June 1, 1979, and May 31, 1984, at The Hospital for Sick Children in Toronto, Kawasaki disease was diagnosed in 163 patients (112 boys, 51 girls, P less than 0.001). Fifteen percent of the children had coronary artery aneurysms. Prior to diagnosis, 24% had been given low doses of aspirin, and 50% acetaminophen. Children with coronary aneurysms had significantly higher temperature during days 10 to 13 of the disease. The febrile phase of the disease was also significantly longer in these children. Coronary artery involvement occurred with equal frequency in boys and girls. There was no significantly greater incidence of coronary artery involvement in infants younger than 1 year of age than in older children. Duration of fever (greater than or equal to 14 days vs less than 14 days) was equally as predictive of the eventual occurrence of coronary aneurysms as the modified Asai score.     

川崎病急性期肝损害与冠状动脉损伤和免疫球蛋白无反应的关系北大核心CSCD

目的总结川崎病(Kawasaki disease,KD)患儿急性期肝损害的临床特点,分析肝损害在预测KD患儿发生冠状动脉损伤及静脉注射免疫球蛋白(intravenous immunoglobulin,IVIG)治疗无反应中的临床价值。方法收集2016年1月1日至2017年12月31日在北京儿童医院首次确诊为KD的925例住院患儿的临床资料,按照入院时有无丙氨酸氨基转移酶(alanine transaminase,ALT)水平异常分为肝损害组(n=284)和非肝损害组(n=641),应用logistic回归分析肝损害等指标预测KD患儿冠状动脉损伤及IVIG无反应的临床价值。结果肝损害组KD患儿入院时间较非肝损害组更早,血清炎性指标水平更高(P<0.05)。肝损害组KD患儿入院时冠状动脉损伤发生率高于非肝损害组(P=0.034);给予首次IVIG治疗后,肝损害组发生IVIG无反应的比例显著高于非肝损害组(P<0.001)。KD患儿发生冠状动脉损伤与血红蛋白水平下降、血小板计数升高、C反应蛋白水平升高和ALT水平升高有关(P<0.05);KD患儿发生IVIG无反应与四肢改变、血红蛋白水平下降、血小板计数升高、C反应蛋白水平升高、ALT水平升高及冠状动脉损伤有关(P<0.05)。结论KD急性期合并肝损害患儿的临床症状出现早且炎性指标水平更高,更容易发生冠状动脉损伤及IVIG治疗无反应。     

川崎病丙种球蛋白治疗策略与心脏损害及冠状动脉病变的相关性研究(1998-2008上海地区调查报告)(英文)

目的 川崎病(Kawasaki disease,KD)是一种病因未明的全身血管炎性综合征,伴冠状动脉病变(coronary artery lesion,CAL);大剂量静脉注射丙种球蛋白(intravenous immunoglobulin,IVIG)治疗KD的临床疗效肯定,但目前IVIG的用法和用量尚存在争议.该研究主要为评价不同IVIG方法治疗.KD的效果,探讨最佳治疗方案.方法 由上海市儿科心血管学组制定统一的KD调查表,发放到上海提供儿科服务的50家医院.回顾性分析1998-2008年上海地区住院KD患者的临床资料.共收集完全符合要求的KD患者资料表格1682例,其中男性1064例(63.3%),女性618例(36.7%);发病年龄(2.57±2.33)岁(0.1～18.8岁).治疗KD的IVIG方案包括1 g/ks×1次、2 g/kg×1次、0.4～0.5 g/kg×5次、1 g/kg×2次、2 g/kg×2次及其他.采用SAS 6.12统计软件包进行统计分析,计数资料采用X2检验计算;计量资料数据以X-±s表示,采用t检验.结果 在KD病程的5～10 d应用IVIG有助于最大化降低KD患者的CAL发生率;所有IVIG的KD患者中,应用方案1g/kg×2次治疗者心脏损害、冠脉病变的发生率均为最低,差异有统计学意义(P<0.05).结论 在KD病程5～10 d以IVIG 1g/kg×2次的剂量,有助于最大化降低KD患者的CAL发生率.