中国川崎病患儿并发冠状动脉病变高危因素的Meta分析

Objective Coronary artery lesion (CAL)is a serious complication of Kawasak disease (KD).Whether there is CAL and the severity arc the most critical factors of the prognosis of KD.The incidence of KD is currently increasing year by year.KD has replaced rheumatic fever as the main entity of acquired heart disease of children.This study aimed to identify risk factors of CAL secondary to KD and take early interventions to prevent CAL or reduce its incidence.MethOd Literature search was performed at Chinese Academic Literature Main Database,Chinese Science and Technology Periodical Database,Wanfang Periodicals and Dissertation Database.and the Chinese Biomedical Literature Database comprehensively,besides,retrospective retrieval and manual retrieval were also performed from the domestic public actions and the dissertations dating from January,2000 to December, 2009.RayMan 4.2 provided by Cochrane was used for meta analysis.Fixed or random model was selected according to the results of heterogeneity test.Sensitivity analysis was done according to the different results.The publication bias was evaluated by funnel plots.Odds ratio(OR)and 95% confidence interval(CI)were estimated in the dissertation.Result Twenty studies were confirmed to be eligible.A11 the 20 studies were retrospective.OR and 95%CI of the risk factors were as follows:age≤1 year,OR=1.58,and 95%CI(1.23,2.04),P=0.0004;male gender, OR=1.48,95%CI(1.29,1.71),P＜0.000 01;WBC＞20×109/L,OR=1.73,95% CI(1.32,2.26),P＜0.000l;C-reactive protein(CRP)＞100 mg/L,OR=2.37,95%CI(1.49,3.77),P=0.0003:fever duration＞10 d,OR=3.23,95%CI(2.08,5.02),P＜0.000 01;use of intravenous gamma globulin(IVIG)＞10 d,OR=2.50,95%CI(1.98,3.16),P＜0.000 01.Conclusion The high risk factors for coronary artery lesion secondary to Kawasaki disease are age≤1 year,male,WBC＞20×109/L,CRP＞100 mg/L,fever duration＞10 d,and use of intravenous gamma globulin(IVIG)＞10 d.     

Predictive value of Apelin-13 on coronary artery lesion in Kawasaki disease北大核心CSCD

Objective To investigate the relationship between the level of Apelin-13 and coronary artery lesion (CAL) in patients with Kawasaki disease (KD), and assess the predictive value of Apelin-13 for CAL in acute phase of KD. Methods A total of 240 children with KD treated in Chengdu Women and Children's Central Hospital from September 2017 to October 2019 were recruited, and were divided into KD with CAL (KD CAL) group and KD without CAL (KD-NCAL) group.Thirty children with acute upper respiratory infection and 30 healthy children were recruited into the febrile control group and the healthy control group, respectively.Blood routine and serum levels of albumin, C-reactive protein (CRP), N-terminal pro-brain natriuretic peptide (NT-proBNP) and Apelin-13 were mea-sured in KD children prior to intravenous gamma globulin injection and after the diagnosis of children in the febrile control group and physical examination of children in the healthy control group.The clinical data of children in each group were compared, and the risk factors of KD complicated with CAL and the predictive value of Apelin-13 were determined by using receiver operating characteristic (ROC) curve and multiple Logistic regression analysis. Results Apelin-13 and hemoglobin in children with KD were significantly decreased compared with those in the healthy control group and fever control group (all P<0.001). However, white blood cell(WBC) count, platelet count, CRP and NT-proBNP in KD group were significantly increased compared with those in the healthy control group and fever control group (all P<0.001). Serum albumin in KD children was significantly lower than that in the healthy control group (P=0.004), and there was no difference when compared with the fever control group (P=0.485). Apelin-13 and hemoglobin were significantly decreased in KD-CAL group compared with KD-NCAL group (t=10.102, P<0.001; t=2.034, P=0.043), while NT-proBNP and CRP were significantly increased (t=5.982, 3.728, all P<0.001). Multiple logistic regression analysis showed that Apelin-13 and NT-proBNP were independent predictors of CAL in KD.The ROC curve analysis showed that the cut-off value of Apelin-13 for predicting CAL was 2.99 μg/L, with an area under the curve (AUC) of 0.869 (95%CI: 0.820-0.909), sensitivity of 77.78% and specificity of 88.67%.While NT-proBNP cutoff value of 822 ng/L yielded sensitivity of 57.78% and specificity of 84.62% for predicting CAL with an AUC of 0.718(95%CI: 0.656-0.774). Conclusions Apelin-13 plays a protective role in KD complicated with CAL, and could be used to predict CAL in the acute phase of KD. © 2022 Chin J Pathol, June. All rights reserved.     

川崎病患儿血清脑利钠肽的水平改变及其与肌钙蛋白Ⅰ的对比分析

Objective To determine the serum levels of brain natriuretie peptide(BNP)and cardiac troponin I(cTnⅠ)in children with Kawasaki disease(KD),and to assess their value in the early diagnosis of KD.Methods Serum levels of BNP and cTnI were measured in 21 KD children(KD group)and 20 healthy children as control group by enzyme-linked immunoabsorbent assay.The internal diameter of coronary artery was measured by two-dimensional eehocardiography.Results Mean serum level of BNP[(517.26±213.40)μg/L]was significantly higher than that in the control group[(37.55±7.56)μg/L](P ＜ 0.01),and cTnI level[(0.31±0.17)μg/L]was significantly higher than that of the control group[(0.13±0.04)μg/L](P ＜0.01).Among KD patients,the percentage of patients with increased BNP(100%)was significantly higher than that of increased cTnⅠ(47.7%),there was significant difference(P ＜ 0.01).Serum levels of BNP and cTnⅠ in the coronary artery lesion(CAL)group were significantly higher than those in the group without coronary artery lesion(NCAL)[(719.33±221.17)μg/L vs(416.23±120.45)μg/L,(0.44±0.22)μg/L vs(0.24±0.11)μg/L,t = 3.03,2.52,P ＜ 0.05].There were no significant difference in serum BNP and cTnⅠ concentrations between the typical and incomplete KD group[(536.15±243.80)μg/L vs(515.71±208.16)μg/L,(0.33±0.21)μg/L vs(0.31±0.16)μg/L,t = 1.06,1.21,P＞0.05].Conclusion Serum levels of BNP and cTnⅠ increase significantly in the KD patients,and BNP has higher sensitivity and specificity than cTnⅠ.Serum BNP level is useful for diagnosis of KD,especially for the incomplete KD.     

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目的探讨川崎病(KD)发生冠状动脉病变(CAL)的危险因素。方法回顾性分析2000年1月至2004年12月期间942例住院KD患儿的临床资料,对发生CAL(170例)和未发生CAL(772例)的病例进行组间对照研究,对13种影响CAL发生的因素进行Logistic回归分析。结果Logistic回归分析结果表明,热程大于10d(X1)的OR值为1.203(95%CI=1.104～1.312,P<0.001)、血红蛋白(Hb)小于100g/L(X2)的OR值为0.658(95%CI=0.472～0.995,P<0.05)。建立KD发生CAL的危险因素主效应模型是Logit(P)=β0(-2.995)+0.185X1+(-0.378)X2(χ2=16.920,P=0.031)。结论热程大于10d、Hb<100g/L是KD发生CAL的高危因素。     

What is the appropriate strategy for diagnosing NAFLD using ultrasonography in obese children?

Background:The aim of this study is to identify obese children who are candidates for a potential diagnosis of non-alcoholic fatty liver disease (NAFLD).Methods:We enrolled 242 obese children (122 boys and 120 girls) aged 7-16 years who were examined with abdominal ultrasonography in our pediatric obesity clinic.We compared patients in the normal group with those in the NAFLD group (mild disease,moderate to severe disease) and identified the optimal anthropometric parameters among height,weight,body mass index (BMI),waist circumference,hip circumference,waist to height ratio (WHtR),and waist to hip ratio to predict NAFLD using a receiver operating characteristic curve analysis.We also investigated risk factors associated with NAFLD for the anthropometric parameters and the biochemical model using logistic regression.Results:The high-and low-risk groups for hepatic steatosis relative to a WHtR of 0.56 as the standard point showed significant differences in hepatic steatosis severity grade (P＜0.001),BMI (P=0.004),hip circumference (P=0.090),aspartate aminotransferase (P＜0.001),alanine aminotransferase (P＜0.001),triglycerides (P=0.001),and the triglyceride to high-density lipoprotein (HDL) cholesterol ratio (P=0.006).Risk factors for hepatic steatosis on logistic regression analysis were male sex (odds ratio:3.68,95％ confidence interval:1.76-7.70),WHtR ＞0.56 (2.25,1.05-4.81),and waist circumference ＞90th percentile (20.22,9.21-44.36) in the anthropometric parameter model and elevated alanine aminotransferase levels (boys ＞25.8 U/L,girls ＞22.1 U/L) (6.93,2.52-19.03),hypertriglyceridemia (＞110 mg/dL) (3.80,1.23-11.75),and triglyceride to HDL cholesterol ratio ＞3 (9.23,2.95-8.83) in the biochemical parameter model.Conclusions:A diagnostic approach to hepatic steatosis is recommended as part of the proper screening and stratification of risk factors in obese children.WHtR is a simple and convenient method of effectively identifying obese children who are candidates for hepatic steatosis screening.     

儿童肱骨髁上骨折急诊与延期手术治疗的对比研究

目的 在较大样本量的前提下评价儿童肱骨髁上骨折急诊手术与延期手术的疗效.方法 收集国内外儿童肱骨髁上骨折急诊手术与延期手术对照文献,对结果进行Meta分析.其中,试验组为急诊手术,对照组为延期手术.疗效及差异评价指标OR以95%CI表示,P＜0.05为差异有统计学意义.统计学分析采用Review Manager 4.2软件.结果 纳入分析的是国外5个随机对照研究,Meta分析结果显示,急诊手术与延期手术在闭合复位失败转化为开放手术不存在显著性差异[OR=0.62,95%CI(0.37,1.03),P=0.06].并发症不存在显著性差异[OR=1.10,95%CI(0.65,1.84),P=0.73].结论 完善术前准备的情况下应该尽早对儿童移位肱骨髁上骨折进行手术治疗,但不推荐急诊手术.

Abstract：

Objective To compare early and delayed operation for humeral supracondylar fracture in children. Methods Computer-based online search all database was performed to collect contrast studies on early and delayed operation in the treatment of humeral supracondylar fracture in children,and meta-analysis was performed on the results. Early operation group was compared to the delayed operation group (control). Differences in efficacy and evaluation indicators were odds ratio (OR),weighted mean difference and 95% confidence interval (CD. Review Manager 4. 2 was used for statistical analysis. Results A total of 3 randomized, controlled trials by foreign researches were collected.Meta analysis showed that there is no significant difference between the two groups in open operation OR = 0. 62,95 %CI(0. 37,1.03), P = 0. 06 and complication OR = 1.10,95 %CI(0. 65,1.84), P = 0. 73.Conclusions We should deal with humeral supracondylar fracture in children as soon as feasible. Emergency operation is not recommended.     

川崎病流行病学特征及冠状动脉损害的相关因素分析

目的 总结川崎病(KD)流行特点,探讨并发冠状动脉损害(CAL)的危险因素.方法 采用回顾性分析方法,对1997-2008年禹州市三所医院确诊的186例KD患儿临床资料进行研究,分析KD流行特点,比较KD并发CAL病例和非CAL病例暴露因素,用Logistic回归分析筛选CAL发生的显著危险因素.结果 住院治疗KD患儿逐年上升,1～3岁为KD高发年龄段,男女比例为1.66:1,合并CAL发生率33.10%;单因素分析显示年龄、居住环境、发病至就诊时间、总发热天数、静脉注射免疫球蛋白(IVIG)用量和时间、血清白蛋白(ALB)含量、血小板(PLT)数与KD并发CAL相关联(P均＜0.05);Logistic回归分析显示年龄、ALB、PLT、发热天数进入的回归方程,有统计学意义.结论 KD发病率呈升高趋势,1～3岁男性儿童是发生KD的高危人群;KD主要危害冠状动脉,年龄≤2岁、ALB＜40g/L、PLT＞500×109/L、热程＞10 d是KD并发CAL的显著危险因素.     

过敏性紫癜患儿血管内皮细胞损伤及凝血功能变化的临床意义

Objective To investigate the changes and the role of plasma thrombomodulin(TM),von willebrand factor (vWF) ,and coagulation status indicators in children with Henoch-Schonlein purpura(HSP).Methods The plasma concentrations of TM,vWF were measured by ELISA method and the levels of D-dimer,PT,APTT,palatelet count were measured in 56 acute SHP patients,50 recovery patients and 40 healthy controls. Results The plasma levels of TM、vWF、D-dimer、palatelet count in acute group were significantly higher than those in healthy controls group and those of recovery group ( P = 0. 000). The plasma levels of PT、APTT showed no significant difference among three groups( P ＞ 0. 05 ). The plasma levels of TM、vWF in recovery group were significantly higher than those in healthy controls group ( P ＜ 0. 05, P ＜ 0. 01 ), while the levels of D-dimer、palatelet count reduced to nomal levels(P ＞0. 05). As compared to non-renal damage group, The plasma levels of TM、vWF、 D-dimer were higher in renal damage group ( P ＜ 0. 05, P ＜ 0. 01 ), the levels of PT 、APTT、palatelet count showed no significant difference ( P ＞ 0. 05 ). Conclusion The damage of vascular endothelium and hypercoagulability play an important role in the pathogenesis of HSP. Changes of plasma TM、 vWF、palatelet count can be used as an indicator of the early lesion of renal function.     

小剂量肝素微量泵持续定时静脉注射治疗小儿原发性肾病综合征的临床研究

Objective To explore the efficacy and safety of low-dose heparin in the treament of children with primary nephrotic syndrome (PNS). Methods It was an open and comparative trial. Eightyeight children with PNS in the hypercoagulable state,on the basis of administrating with glucocorticosteroid,were administrated with low-dose heparin that infused by micro pump oriented to time ( group A). Eighty patients only treated with glucocorticosteroid were chosen as control (group B). Results Serum-albumin and activated partial thromboplastin time (APTT) increased,but fibrinogen (Fib) decreased after therapy in the group A,and they all showed significant differences (P ＜ 0. 01 ). Serum-albumin increased after therapy in the group B and there was significant difference (P＜0. 01 ). However,APTT and Fib in the group B showed no significant difference( P ＞ 0. 05 ) between post-treatment and pretherapy. Post-treatment serum-albumin and APTT in the group A were significantly higher than those in group B, and Fib was significantly lower than that in group B ( P ＜ 0. 01 ). The rate of urine protein remission in group A (82/88) was significantly higher than that in group B (63/80). Urine protein remission time and edema disappearance time were significantly shorter in group A than group B ( P ＜ 0. 01 ). APTT of group A at the peak concentration of heparin after therapy was significantly higher than that of pretherapy ( P ＜ 0. 01 ), and the ratio was 2. 38. However, there was no significant difference in APTT at the valley concentration of heparin between post-treatment and pretherapy ( P ＞ 0.05 ). Conclusion Low dose-heparin infused by micro pump oriented to time in the treatment of children with PNS has an obvious anticoagulative effect. It can improve the rate of urine protein remission and shorten edema disappearance time. Meanwhile it is safety ,requires no laboratory monitor and has few drug side effects,thus it deserves further clinical application.     

胆道闭锁Kasai术后早期并发症的分析

Objective The purpose of this study was to evaluate the relation between early complications of Kasai operation and high-dose steroids and antibiotics and to demonstrate the prognosis of biliary atresia(BA). Methods 281 patients diagnosed with biliary atresia(BA) type Ⅲ,admitted from Aug. 1994 to Aug 2008 were retrospectively reviewed and followed up for two years. The patients were divided into two groups. Patients in Group A were controls while patients in Group B were treated with high-dose steroids and antibiotics. We evaluated the relation between early complications and age at operation (days), baseline bilirubin, different treatments and 2 year survival. Results 93 patients had early complications,and cholangitis is the most common, There is no statistics difference of complications in age at operation and baseline bilirubin. The complication rate was 63. 6% in group A, and 25. 7% in group B(P＜0. 01). The cholangitis rate was 56. 4% in group A and 23. 9% in group B (P＜0. 01). Two groups showed no significant difference in digestive tract bleeding. The visit frequency rate was 92. 5%, while 2 year survival rate was 51. 2%. Patients with early complications or cholangitis have a 34. 4% or 32. 9% 2 year survival rate, while those without have a 59. 6% or 59. 2%(P＜ 0. 01). Patients with high-dose treatment in group B have a better 2 year survival rate (54. 0% vs 40. 0% ,P＜0. 05). Conclusions Early cholangitis was the risk factor of outcome after Kasai operation. These data implied that the use of high-dose steroids and antibiotics can lower the occurrence of cholangitis and elevate 2 year survival rate.     

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。     

糖皮质激素联合免疫球蛋白用于川崎病初始治疗有效性的Meta分析

目的 系统评价糖皮质激素(GCs)联合静脉注射免疫球蛋白(IVIG)用于川崎病(KD)初始治疗的有效性和安全性。方法 计算机检索MEDLINE数据库、PubMed数据库、CNKI、万方数据库、维普电子期刊全文数据库等,收集GCs联合IVIG初始治疗儿童KD的前瞻性对照研究或回顾性对照研究,检索时间为各数据库建库至2016年3月。由 2位研究者独立筛选文献、提取资料并对纳入文献进行质量评价后,采用RevMan5.2软件进行Meta分析。结果 共纳入11篇文献,均为英文文献,其中前瞻性研究7项,回顾性研究4项。Meta分析结果显示:与单用IVIG相比,GCs联合IVIG组的冠状动脉损害(CAL)发生率更低 (OR = 0.44,95%CI: 0.23~0.86,P = 0.02),发热持续时间更短 (MD = -1.66,95%CI: -2.32 ~ -1.01,P < 0.00001),且首次治疗未反应率低于单用IVIG组 (OR = 0.37,95%CI: 0.27~ 0.51,P < 0.00001)。两组复发率和不良反应发生率比较差异均无统计学意义。结论 GCs联合IVIG初始治疗KD能降低患儿CAL发生率及首次治疗未反应率,缩短发热时间,且不增加复发率和不良反应的发生。     

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FCGR2A基因单核苷酸多态性的研究

目的：探讨中国汉族儿童FCGR2A基因 rs1801274位点单核苷酸多态性（SNP）与川崎病（KD）易感性及静脉注射免疫球蛋白(IVIG)治疗KD疗效的关系。方法：应用聚合酶链反应（PCR）联合直接基因测序技术对35例KD患儿和25例健康儿童的FCGR2A基因SNP rs1801274位点进行检测和分析,并将KD患儿根据IVIG治疗后有无并发冠脉损害（CAL）分为CAL和无CAL（NCAL）两个亚组。结果：在研究对象中均检测到FCGR2A基因rs1801274位点,该位点存在3种基因型(AA、AG、GG)。该SNP位点的基因型分布及等位基因频率在KD组与对照组及CAL组与NCAL组之间比较差异均有统计学意义（P<0.05）。其中携带A等位基因或AA基因型者发生KD的危险性大(分别OR=3.39,95%CI:1.53~7.50;OR=4.93,95%CI：1.61~15.1);携带基因型AG或G等位基因使KD患儿发生CAL的危险性增高（分别OR=5.43,95%CI:1.06~27.8;OR=4.88,95%CI: 1.44~16.5）。结论：FCGR2A基因rs1801274位点SNP可能是影响中国汉族儿童KD易感性以及IVIG治疗KD疗效的一个重要因素。     

基于北京市儿童川崎病6年流行病学资料的实验室指标分析

摘要 目的 川崎病（KD）缺乏特异性诊断方法，部分患儿为不完全性KD临床表现，本文拟对KD患儿实验室检查指标中的ESR、CRP、PLT、血浆白蛋白（ALB）、ALT、Hb和WBC进行分析，探讨各项实验室指标异常协助临床诊断的意义及与冠状动脉损害和IVIG抵抗的关系。方法 根据KD的诊断标准，对北京小儿川崎病流行病学调查协作组45家医院2000至2005年收治的急性期KD患儿的实验室资料进行汇总，将各指标同冠状动脉损害和IVIG抵抗行Pearson卡方检验和Logistic回归分析。结果 共收集KD患儿1 745例，依据纳入和排除标准有1 335例进入分析，其中男868例，女467例。 本组患儿ESR、CRP、PLT和WBC增高，ALB减低和Hb降低的发生率分别为96.0%、90.0%、92.2%和80.4%，45.7%和48.8%。Hb降低的发生率在不同的年龄组中存在明显差异，ALB和Hb降低与冠状动脉损害有相关性（P﹤0.05），CRP、ALB、ALT、Hb和WBC计数与IVIG抵抗有相关性（P﹤0.05）。Logistic回归显示ALB、Hb降低与冠状动脉损害有相关性，CRP升高、Hb降低、ALT和WBC升高与IVIG抵抗存在相关性（P﹤0.05）。结论 KD急性期ESR增快、CRP升高、外周血PLT、WBC计数增高的发生率均>90%,可协助KD急性期临床诊断。对于ALB和Hb降低的KD患儿应注意是否存在冠状动脉损害，对于CRP、ALT、WBC升高和Hb降低的患儿应注意出现IVIG抵抗的可能。     

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。     

942例川崎病的临床分析

目的总结川崎病（Kawasaki disease,KD）的临床特征,探讨KD预后与治疗的关系。方法回顾性分析2000年1月—2004年12月期间942例住院KD患儿的临床资料：（1）比较典型KD与不完全性KD（incomplete KD）的临床特征;（2）总结KD对静脉注射免疫球蛋白（intravenous immune globulin,IVIG）治疗无反应的影响因素;（3）随访观察其中的510例KD患儿,比较IVIG 1g／kg和2g／kg治疗的远期疗效。结果（1）942例中,典型KD774例,不完全性KD168例。不完全性KD冠状动脉病变（coronary artery lesion,CAL）发生率较高（P〈0．05）,除肛周脱屑外,其他临床症状发生较少,出现较晚（P〈0．05或0．01）;（2）与IVIG治疗反应敏感组比较,IVIG治疗无反应组的发热时间较长,血红蛋白（Hb）、白蛋白（ALB）、血细胞比容（Hct）及血小板（PLT）较低（P〈0．05或0．01）;（3）WIG 1g／kg和2g／kg治疗组在KD发病后2年内,CAL的恢复率及新发生率,两组差异均无统计学意义（P〉0．05）。结论（1）不完全性KD的CAL发生率较高,肛周脱屑可以作为不完全性KD的早期诊断依据之一;（2）急性期发热时间较长,PLT无升高及Hb、Hct、ALB持续降低是IVIG治疗无反应的影响因素;（3）IVIG 1g／kg和2g／kg治疗KD的疗效在KD发病后2年内相似。     

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。     

Assessment of Risk Factors for Korean Children with Kawasaki Disease

Kawasaki disease (KD) is the most common cause of acquired heart disease in children. Intravenous immunoglobulin (IVIG) is the standard therapy for KD, but more than 10% of KD patients do not respond to IVIG and are at high risk for the development of coronary artery lesions (CALs). To identify clinical and genetic risk factors associated with CAL development and IVIG nonresponsiveness, this study analyzed the clinical data for 478 Korean KD patients. Multivariate logistic regression analysis showed that incomplete KD, IVIG nonresponse, fever duration of 7?days or longer, and the CC/AC genotypes of the rs7604693 single nucleotide polymorphism (SNP) in the PELI1 gene were significantly associated with the development of CALs, with odds ratios (ORs) ranging from 2.06 to 3.04. The risk of CAL formation was synergistically increased by the addition of individual risk factors, particularly the genetic variant in the PELI1 gene. Multivariate analysis also showed that a serum albumin level of 3.6?g/dl or lower was significantly associated with nonresponsiveness to IVIG [OR, 2.76; 95% confidence interval (CI), 1.34-5.68; P?=?0.006]. Conclusively, incomplete KD, IVIG nonresponsiveness, long febrile days, and the rs7604693 genetic variant in the PELI1 gene are major risk factors for the development of CALs, whereas low serum albumin concentration is an independent risk factor for IVIG nonresponsiveness.     

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。