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。     

兰州地区川崎病患儿并发冠状动脉损害的危险因素研究

目的探讨兰州地区川崎病(KD)患儿并发冠状动脉损害(CAL)的危险因素。方法对确诊的174例KD患儿,根据其是否并发CAL分为CAL组和NCAL组,分析比较其年龄、性别、热程、静脉用丙种球蛋白(IVIG)开始使用时间、IVIG使用剂量、C反应蛋白(CRP)、血清白蛋白、红细胞沉降率(ESR)、血小板(PLT)、血红蛋白等资料的差异。结果 174例KD患儿并发CAL者46例(占26.44%),无并发CAL者128例;两组患儿平均热程、自发热到开始IVIG治疗的时间、IVIG使用剂量,以及PLT、CRP、ESR、红细胞计数(RBC)的差异均有统计学意义(P0.05)。结论热程10 d、发热10 d后开始使用IVIG,PLT、CRP、ESR升高,RBC降低的KD患儿应警惕并发CAL的危险性,对于KD的治疗及预后判断有一定的参考价值。     

川崎病患儿治疗前后血浆血栓调节蛋白变化的意义

目的探讨川崎病(KD)患儿静脉注射用丙种球蛋白(IVIG)治疗前后血浆血栓调节蛋白(TM)水平变化及其意义。方法治疗组选择本院确诊KD患儿44例。分为冠状动脉损伤组20例和无冠状动脉损伤组24例;正常对照组选择年龄相仿的门诊体检健康儿童15例。采用酶联免疫吸附法检测KD患儿经IVIG治疗前后及对照组血浆TM水平。结果KD患儿治疗前急性期血浆TM水平明显高于正常对照组(P<0.01);与无冠状动脉损伤组比较,冠状动脉损伤组血浆TM水平升高更明显(P<0.05)。经IVIG治疗后KD患儿急性期血浆TM水平明显降低(P<0.01)。结论血浆TM水平在KD患儿急性期、尤其有冠状动脉损伤时明显升高;血浆TM可能参与KD冠状动脉损伤的病理过程。     

肾上腺糖皮质激素联合乌司他丁治疗儿童川崎病的非随机对照临床研究

目的 探讨肾上腺糖皮质激素联合乌司他丁治疗儿童川崎病的临床疗效。方法 根据患儿病情和家长意愿,将2011年1月至2013年12月入院确诊为典型川崎病(KD)的104例患儿分为乌司他丁组(甲基强的松龙+乌司他丁,n=46)和静脉注射丙种球蛋白(IVIG)组(n=58)。观察两组患儿治疗前、治疗后1周、3个月及6个月时的冠状动脉内径的变化,热退时间,再次治疗情况,治疗前、治疗后1周及3周时白细胞(WBC)、血小板(PLT)、血红蛋白(HB)、C反应蛋白(CRP)、血沉的变化以及住院总费用。结果 两组患儿在治疗前、治疗后1周、3个月及6个月时冠状动脉内径比较差异均无统计学意义(P> 0.05)。治疗48 h乌司他丁组患儿体温均正常(100%),正常率高于IVIG组(83%)(PPP结论 甲基强的松龙联合乌司他丁治疗儿童KD没有增加冠状动脉瘤的发生风险;并能大大降低住院费用;与IVIG治疗相比,在KD急性期能更好的控制患儿的实验室指标,缩短发热时间。     

静脉注射丙种球蛋白无反应型川崎病的危险因素及预测分析

目的探讨川崎病(KD)静脉注射丙种球蛋白(IVIG)治疗无反应的危险因素,为及时诊治IVIG治疗无反应病例提供依据。方法选择1993年3月-2010年12月在本院住院治疗的KD患儿219例。患儿均予IVIG治疗,根据患儿对IVIG治疗的反应分为IVIG敏感组和IVIG无反应组。对KD患儿的年龄、性别、主要临床表现、治疗前及治疗24～48 h血常规结果、血生化辅助检查、心脏超声检查结果、治疗过程及疗效进行回顾性分析,并应用SPSS 13.0软件对结果进行统计学分析。结果 IVIG治疗无反应组治疗前中性粒细胞比例、CRP水平及治疗后白细胞水平、中性粒细胞比例、CRP水平均显著高于IVIG敏感组(Pa<0.05);IVIG治疗无反应组血总胆固醇、血钠、血钾水平均显著低于IVIG敏感组(Pa<0.05);上述指标积分≥6的ROC曲线下面积达0.955,预测患儿IVIG无反应的敏感度为95.1%,特异度为80.0%。结论联合应用治疗前中性粒细胞比例、CRP水平,治疗后白细胞水平、中性粒细胞比例、CRP水平、血胆固醇、血钠及血钾水平积分,对于预测IVIG无反应KD病例的发生有较高的价值。     

儿童川崎病并发冠状动脉损害的危险因素分析

目的 探讨儿童川崎病(KD)并发冠状动脉损害(CAL)的危险因素。方法 回顾性分析895例KD患儿的病历资料,将其分为并发CAL 组(n=284)和未并发CAL 的对照组(n=611),比较两组临床及实验室指标,并对KD 患儿并发CAL的危险因素进行多因素logistic 回归分析。结果 男性、发生CAL 以外并发症、不典型KD、静脉注射丙种球蛋白(IVIG)抵抗、IVIG 治疗前发热时间>5 d、血清降钙素原(PCT)增高为KD患儿并发CAL 的独立危险因素(OR 值分别为1.712、2.028、3.655、2.912、1.350、1.068,均POR=0.931,P结论 男性KD患儿、不典型KD 患儿以及发生CAL 以外并发症、发热时间较长、IVIG 治疗抵抗的KD患儿并发CAL 的风险较高。血清PCT与ALB预测KD患儿并发CAL的价值不大。     

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。     

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。     

CD40L在川崎病发病机制中的作用

目的探讨T淋巴细胞、血小板CD40L在急性期川崎病血管炎及冠状动脉病变发生中的可能作用。方法用流式细胞术双标法,检测23例急性期及IVIG治疗后川崎病(KD)患儿、23例非KD发热患儿T淋巴细胞、血小板CD40L表达的阳性率及平均荧光强度。结果急性期KD组T淋巴细胞表达CD40L阳性细胞率(27.91±5.47)及平均荧光强度(11.74±3.35)明显高于IVIG治疗后组(分别为12.32±1.89%及6.18±1.71%P均<0.01)及发热对照组;同样,急性期KD组血小板CD40L表达的阳性率及平均荧光强度明显高于IVIG治疗后组及发热对照组。结论T淋巴细胞、血小板CD40L在急性期KD患儿中高表达现象,可能在KD急性期血管炎及冠状动脉病变中发挥一定作用。     

2012至2016年单中心川崎病流行病学及临床特征研究

目的了解川崎病(KD)患病情况及临床特征,探讨KD冠状动脉损害(CAL)及IVIG耐药的危险因素。方法回顾性分析华中科技大学同济医学院附属同济医院2012年1月1日至2016年12月31日初诊的KD患儿的临床资料,比较分析KD治疗前后,典型和不完全KD,KD伴或不伴CAL,IVIG敏感或耐药的临床特征,分析CAL发生和IVIG耐药的危险因素。结果725例KD患儿进入本文分析,男∶女为1.61∶1,平均年龄（2.7±2.3）岁;不完全KD 206例（28.4%）,典型KD 519例;CAL 216例（29.8%）,IVIG耐药61例（8.4%）;治疗中仅使用阿司匹林者70例（9.6%）。KD伴CAL的危险因素为IVIG耐药（OR=5.138,95%CI：1.835~14.836）和氨基末端脑钠肽前体（NT-proBNP）≥1 000 pg·mL-1（OR=2.723,95%CI：1.110~6.679）。IVIG耐药的危险因素为出现CAL（OR=2.586,95%CI：1.067~6.271）。结论KD患病人数、CAL和IVIG耐药患儿有增加趋势。IVIG耐药和NT-proBNP≥1 000 pg·mL-1为KD伴CAL的危险因素,而发生CAL为IVIG耐药的危险因素。     

川崎病患儿并发冠状动脉病变的危险因素分析

目的：探讨川崎病(KD)并发冠状动脉病变(CAL)的危险因素。方法：收集2006年1月至2012年1月间诊断为KD的527例患儿的临床资料,对15个可能与CAL发生有关的因素进行单因素和多因素logistic回归分析。结果：单因素分析显示,患儿年龄、性别、KD类型、大剂量丙种球蛋白（IVIG）治疗起始时间、对IVIG治疗的反应、使用糖皮质激素、发热持续时间及C反应蛋白等因素在合并和未合并CAL两组患儿中差异有统计学意义（P8岁、男性、非典型KD、IVIG治疗开始于发热后10 d 以上、对IVIG治疗无反应、发热持续时间>10 d为CAL发生的独立危险因素（OR分别为2.076、1.890、1.972、1.426、3.251、2.301、1.694,均P8岁）、男性、非典型KD、IVIG治疗起始时间较晚、对IVIG治疗无反应、发热持续时间较长是CAL发生的独立危险因素。     

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.     

免疫球蛋白无应答川崎病再次免疫球蛋白、英夫利昔单抗和激素治疗队列研究

背景 IVIG无应答KD患儿的治疗方案包括再次IVIG、英夫利昔单抗(IFX)和激素(IMP),目前不同治疗方案的疗效评价不一.目的 IVIG无应答KD患儿再次IVIG、IFX和IMP治疗效果比较.研究设计队列研究.方法 以IVIG无应答KD患儿为队列人群,再次治疗和补救治疗(再次治疗无反应)在江西省儿童医院(我院)中...     

Kawasaki disease in Thai infants compared with older children

Infants with Kawasaki disease (KD) are at increased risk of having coronary artery abnormalities (CAA). The purpose of this study was to evaluate the clinical features of KD in infants and compare these with findings in older children to determine the risk factors for CAA in infants. All children with KD admitted to a tertiary care hospital between January 1993 and April 2003 were studied retrospectively. Of a total of 51 patients included in the study, 22 (43%) were <1 year of age (mean 8 months, range 2-12 months). All had classical clinical manifestations such as fever, skin rash and mucositis; extremity change occurred in 95%, conjunctivitis in 81% and cervical lymphadenopathy in 27%. Infants had significantly more non-classical symptoms, e.g. diarrhoea (68%), than older children (38%) (p=0.04). The mean number of days before intravenous immunoglobulin (IVIG) treatment was given to infants was 2 days later than in older children. The predictors of CAA in infants were resistance to IVIG treatment (p=0.02) and long duration of fever (p=0.009). Compared with older children, the less typical presentations and delay in diagnosis and treatment in infants might be important factors in CAA in KD.     

Clinical features of recurrent Kawasaki disease and its risk factors

The clinical features and risk factors for recurrence of Kawasaki disease (KD) remain unclear. In order to summarize clinical features of recurrent KD and identify risk factors associated with recurrence, we conducted a retrospective review of the medical records of consecutive cases of KD from January 2002 to December 2010. Demographic, clinical, laboratory, and echocardiographic data were analyzed. The maximum coronary artery Z score normalized against body surface area was assessed using coronary artery diameters. At the first onset of recurrent KD, children had longer durations of fever before intravenous immunoglobulin (IVIG) treatment and higher levels of alanine aminotransferase, serum aspartate aminotransferase (AST), and lower hemoglobin levels than those with a single episode of KD. Multivariate logistic regression analysis showed that long durations of fever before IVIG treatment, high AST levels, and reduced hemoglobin levels were significantly associated with recurrent KD. Ten of the 22 recurrent KD children had coronary artery complications during the first onset episode, and six (60 %) of these also had coronary artery complications during the recurrence. Children with longer durations of fever, lower hemoglobin levels, and higher AST levels may be at increased risk for KD and coronary artery complications are more likely to occur in children with recurrent KD if they were present during the first episode.     

���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.     

静脉注射不同剂量丙种球蛋白治疗川崎病的临床研究

目的 评价静脉注射丙种球蛋白（intravenous immune globulin,IVIG）1g／kg单次静脉注射治疗川崎病（Kawasaki disease,KD）的临床效果。方法 242例KD患儿随机分为IVIG1s／kg组与IVIG 2g／kg组,对两种治疗方法的疗效进行前瞻性对比研究。分别采用IVIG 1g／kg和2s／kg单次静脉注射,观察患儿总热程、退热时间、黏膜充血、手足肿胀和颈淋巴结肿大消退时间,监测外周血白细胞计数（white blood cells count,WBC）、血小板计数（platelet count,PLT）、血清丙种球蛋白（immunoglobulin,Ig）、C反应蛋白（Creacting protein,CRP）、血沉（erythrocyte sedimentation rate,ESR）、心电图（electrocardiogram,ECG）和冠状动脉病变（coronary artery lesion,CAL）恢复情况,并对治疗前后组内结果、治疗后组间结果进行比较。结果 IVIG 1g／kg组平均热程为10．6d,WBC、PLT、CRP、ESR及ECG异常率与治疗前比较显著降低（P〈0．001）,IVIG1g／kg组与IVIG2g／kg组比较差异无统计学意义（P〉0．05）。WIG1g／kg组CAL发生率为29．5％（36／122）,随访1年有87．5％的CAL恢复正常,12．5％未能恢复正常,其中9．4％为IVIG耐药病例;IVIG2g／kg组CAL发生率为24．2％（29／120例）,随访1年有89．3％的CAL恢复正常,10．7％未能恢复正常,均为IVIG耐药病例,两组比较,差异亦无统计学意义（P〉0．05）。结论 IVIG1g／kg单次静脉注射治疗KD,可有效缓解临床症状,减低CAL发生率,减轻心血管系统损害,与IVIG2g／kg比较具有同样的近期和远期治疗效果。