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??Objective??To study clinical characteristics and risk factors for mortality of septic shock patients with hematological malignancies in pediatric intensive care unit??PICU??. Methods??A retrospective analysis of records of pediatric patients with hematological malignancy and septic shock was performed. In our study??43 cases admitted to PICU in Sun Yat-sen Memorial Hospital between March 2012 and September 2015 were included. Results??Among the 43 cases??16 died??while the other 27 survived. The percentage of mortality was 37.2%. Pulmonary infection ??74.4%?? and gastrointestinal tract infection??39.5%?? were the common causes of septic shock in these patients. Etiological examination revealed 43 kinds of pathogenic bacteria in 26 cases. The percentage of gram-positive bacteria??gram-negative bacteria and fungus was 11.6%??72.1% and 16.3%??respectively. Escherichia coli was found to be the main pathogen??6/43??14.0%??. Between the survival group and the mortality group??there were significant differences in the parameters of age??chemotherapy courses??time from infection to shock??time from neutropenia to shock??multiple organs dysfunction??pulmonary infection??hypocalcemia??oxygenation index and serum lactic acid concentration after 6 hours of fluid resuscitation??requirement of dopamine and mechanical ventilation ??P??0.05??. By logistic regression analysis??mortality was associated with the variables of serum lactic acid concentration after 6 hours of fluid resuscitation and requirement of mechanical ventilation. Conclusion??The risk factors for mortality of septic shock in pediatric patients with hematological malignancies are hyperlactacidemia after 6 hours of fluid resuscitation??hypocalcemia and requirement of mechanical ventilation.     

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目的探讨影响小儿绿脓杆菌败血症病死率的因素。方法回顾性分析1991～2003年间中山医科大学附属第一医院收治的16例14岁以下绿脓杆菌败血症患儿的临床资料。结果发热和腹泻为最常见的初始症状。病死率与性别、年龄、感染途径、是否化疗和使用激素、白细胞数无关(P>0.05),而与基础疾病的严重程度、有无严重并发症、有无低钠血症、高血糖症、血小板减少及有无敏感抗生素联合用药有关(P<0.05)。结论绿脓杆菌败血症是免疫功能低下患儿的常见危重并发症之一,病死率极高,早期经验性联合应用抗绿脓杆菌抗生素可能在一定程度上降低病死率。     

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目的探讨首次丙种球蛋白静脉注射(IVIG)无反应性川崎病(KD)的发生率及危险因素,及其再治疗方法的选择。方法总结2000—2004年北京45家医院KD患儿的临床资料,IVIG无反应性定义为首次IVIG治疗36h后体温仍超过38·5℃。结果1107例患儿纳入研究对象,1092例有急性期治疗资料,1052例(96·3%)接受IVIG治疗,135例对首次IVIG治疗无反应,发生率12·8%(135/1052)。Logistic回归分析发现血沉、GPT、WBC、发病至用IVIG的时间、血浆白蛋白及IVIG治疗剂量,是IVIG无反应性的独立危险因素(P<0·05)。对IVIG无反应者8例给第2剂2g/kg IVIG,5例热退;114例给1g/kg剂量IVIG治疗,35例(30·7%)热退;11例给400~600mg/kg IVIG,1例(9·1%)热退;2例给糖皮质激素,2例均热退。4种再治疗方法间比较,差异有统计学意义(P=0·015)。第2剂2g/kg IVIG治疗较其它再治疗所需进一步IVIG或激素治疗次数少,体温恢复快。结论约12·8%KD患儿对初次IVIG治疗无反应。血沉、WBC和GPT、血浆白蛋白、IVIG使用方法及起病至用IVIG的时间,是IVIG无反应的独立危险因素。对初次IVIG无反应患儿推荐使用第2剂2g/kgIVIG,对2次2g/kgIVIG治疗仍无效者可以选用糖皮质激素治疗。     

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??Abstract??Objective To explore the risk factors in children with coronary artery lesion ??CAL?? complication in Kawasaki disease??KD??. Methods Retrospective analyses of the clinical data from 143 children with KD from 2009 to 2011 in Shandong Provincial Hospital were performed in terms of 11 aspects including the fever duration??hemoglobin ??Hb????erythrocyte sedimentation rate ??ESR????C-reaction protein ??CRP????white blood cell count ??WBC????blood platelet ??PLT????and the patients??gender and age. 143 children were divided into two groups according to whether these KD patients were with or without CAL.Both single factor and multivariate logistic regression analyses were performed. Results Single factor analysis showed that fever duration over 10 days??ESR??PLT??CRP??ALB??cTnI and NT-porBNP were risk factors to KD complicated with CAL ??P??0.05 or P??0.01??.Multivariate logistic regression analysis showed that a fever duration over 10 days??decrease of ALB and increase of cTnI were significant risk factors to KD complicated with CAL??P??0.05??. Conclusion Fever duration over 10 days??decrease of ALB and increase of cTnI are important risk factors to KD complicated with CAL.     

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??Objective To explore the main risk factors of neonatal pneumothoraxand to provide a basis for early clinical intervention and treatment. Methods A retrospective study was conducted at the neonatal intensive care unit??NICU?? of the Children’s Hospital of Soochow University in 67 infants with neonatal pneumothorax and 60 infants without neonatal pneumothorax from January 2012 to December 2014 .The data was compared by univariate and multivariate Logistic regression analysis. Results Univariate analysis showed that gestational age??pneumonia??neonatal respiratory distress syndrome??NRDS????cesarean section??resuscitation intrapartum??continuous positive airway pressure??CPAP????meconium aspiration syndrome??MAS????wet lung??TTN?? and mechanical ventilation were the risk factors for neonatal pneumothorax??P??0.05??. Logistic regression analysis showed that gestational age??NRDS??CPAP??mechanical ventilation??cesarean section??MAS??TTN and pneumonia were the main risk factors for neonatal pneumothorax??OR values were 6.442??9.933??8.690??27.873??5.051??38.405??11.692 and 9.493??P??0.05??. Conclusion The main risk factors for neonatal pneumothorax are gestational age??NRDS??CPAP??mechanical ventilation??cesarean section?? MAS??TTN and pneumonia.     

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??Objective??To investigate the risk factors of purulent meningitis complicated with subdural effusion in infants and young children. Methods??The clinical data of the infants and young children who were diagnosed with purulent meningitis in PICU of Shengjing Hospital of China Medical University from January 2014 to December 2017 were analyzed retrospectively. All of them were divided into 2 groups according to whether there was complication of subdural effusion. The statistical data were analyzed by SPSS 20.0 software. Results??There were significant differences in hemoglobin??C reactive protein and protein in cerebrospinal fluid between control group and subdural effusion group??P??0.05??. Logistic regression analysis showed that hemoglobin??OR??0.940??95%CI??0.899—0.998????C reactive protein??OR??1.015??95%CI??1.004—1.028?? and protein in cerebrospinal fluid??OR??2.490??95%CI??1.151—6.315?? were independent risk factors for purulent meningitis complicated with subdural effusion??P??0.05??. Conclusion??Infants and young children diagnosed with purulent meningitis are with lower hemoglobin. Higher C reactive protein and higher protein in cerebrospinal fluid are likely to be complicated with subdural effusion.     

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??Objective??To explore the pathogen characteristics and risk factors of nosocomial infection in children with refractory pneumonia caused by Mycoplasma pneumoniae. Methods??A total of 160 children??nosocomial infection??45 cases?? as observation group??no nosocomial infection??115 cases?? as control group?? with refractory MPP who were admitted from January 2015 to January 2018 were selected for pathogen detection and pathogen species analysis. Analyze the differences of two groups to detect the risk factors of hospital infection by means of univariate and multivariate logistic analysis. Results??According to the analysis of the pathogens??32 of them were bacterial infections??71.1%????and Gram-negative bacteria were the main pathogens??including Haemophilus influenza??9 cases????Klebsiella pneumonia??7 cases????Escherichia coli ??5 cases?? and Legionella pneumophila??2 cases????13 of them were viral infections??28.9%????including influenza B virus??6 cases????respiratory syncytial virus??5 cases??and adenovirus??2 cases??. After univariate and multivariate logistic analysis??no differences were found in sex??age??congenital heart disease or anemia between observation group and control group??P??0.05??. However??the length of hospitalization??season of onset??invasive procedures??non-single ward??and the irrational use of antibiotics in the two groups had significant differences??P??0.05??. Conclusion??The main infection pathogen of children with refractory MMP in nosocomial infection is Gram-negative bacteria. The risk factors of nosocomial infection are the length of hospitalization?? season of onset??invasive procedures??non-single ward??and the irrational use of antibiotics.     

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??Objective The aim of this study was to summarize the clinical features in children with neuropsychiatric systemic lupus erythematosus??NPSLE?? and to investigate the related risk factors of in systemic lupus erythematosus??SLE?? patients with NPSLE. Methods The clinical data of 14 children with NPSLE were retrospectively analyzed. The indicators including the age?? sex?? clinical manifestations?? laboratory tests were compared with 31 randomly selected SLE patients with non-NPSLE manifestations by univariate and multivariate analysis. Results The main clinical manifestations in children with NPSLE were headache??50%????lateral type motor disorder??50%????mental disorders??35.7%????epilepsy??35.7%????involuntary movement ??14.29%????blurred vision??14.29%?? etc. 13 patients had abnormalities in brain MRI. Univariate analysis showed that children with oral ulcers??blood lymphopenia??thrombocytopenia??elevated CRP??anticardiolipin antibodies positive??antineutrophil cytoplasmic antibody positive??anti-u1-RNP antibody positive??urine red blood cells increased??elevated serum K+ elevated??blood urea nitrogen??elevated serum creatinine were the risk factors with statistical significance. Further non-selective multivariate regression analysis showed that only the anticardiolipin antibody positive?? elevated CRP?? elevated serum creatinine into the multiple regression equation?? a positive correlation. Conclusion Headache?? movement disorders?? mental disorders and seizures were the most common symptoms in children with NPSLE. The brain MRI is a sensitive indicator of checking NPSLE. Anticardiolipin antibody positive?? elevated CRP and elevated serum creatinine were the most important risk factor for SLE children with NPSLE.     

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目的探讨Tourette综合征(TS)发病的危险因素。方法2000-01—2004-12采用病例对照研究的方法,对温州医学院附属一院100例TS患儿及100名对照病例的相关资料进行非条件Logistic回归和主成分分析。结果非条件Logistic回归多因素分析显示,该病有统计学显著性意义的危险因素是TS家族史、注意缺陷伴多动障碍(ADHD)、过敏性哮喘、母亲孕早期受精神刺激、高热(T>39℃)和极低频磁场暴露,其标准回归系数分别为1.3987、3.6385、1.7657、1.7250、1.8973和1.0677。主成分分析提取了4个主成分,其方差累积贡献率为57.025%。结论TS的发病与遗传因素和环境因素有关。     

婴儿法乐四联症外科治疗的危险因素分析

目的 探讨婴儿法乐氏四联症Ⅰ期矫治手术早期死亡的危险因素。方法 统计56例婴儿法乐氏四联症患儿I期手术治疗的临床资料，对年龄、体重、室缺大小、左室舒张末期容积指数、肺动脉发育、主动脉阻断时间、心肌保护、术后并发症、术后呼吸机辅助时间、手术病死率等15项围手术期指标进行分析。结果 术后早期死亡11例，病死率19．6％(11／56)，单因素分析结果表明术后低氧血症、严重心律失常、低心排血量综合征是手术早期死亡的高危因素，Logistic危险因素多因素回归分析显示，排除病情严重程度的混杂因素后，传统心肌保护方法是婴儿法乐氏四联症Ⅰ期矫治手术早期死亡的主要原因。结论 婴儿法乐氏四联症Ⅰ期根治手术是可行的，改良心肌保护比传统心肌保护疗效好。     

小儿法洛四联症根治术后早期死亡的危险因素分析

目的 探讨小儿法洛四联症(tetralogy of Fallot,TOF)手术后早期死亡的危险因素,以提高手术成功率,降低手术病死率.方法 2003年1 月至2010年12月期间在成都心血管病医院行TOF根治术患儿191例,其中男142例,女49例;年龄4个月～12岁.收集所有患儿术前、术中和术后的临床资料,分析影响TOF手术后早期死亡的危险因素.结果 191例患儿,围术期死亡6例(3.14％),死于手术后感染合并多器官功能衰竭3例,低心排血量综合征2例,脑部并发症1例.其中≤6个月患儿病死率6.67％(2/30),6个月～3岁患儿病死率1.41％ (1/71),3～12岁患儿病死率3.33％ (3/90).多因素Logistic回归分析结果表明,年龄≤6个月(OR=4.606,95％CI 1.811 ～11.719,P＜0.05)、术前血氧饱和度＜70％(OR=0.982,95％ CI 0.501 ～ 1.932,P＜0.01)、Nakata指数＜140mm2/m2(OR=16.960,95％CI 1.414 ～ 150.390,P＜0.01)、体外循环时间＞150 min(OR=4.398,95％CI2.091 ～9.216,P＜0.01)及术后多器官功能衰竭(OR=4.872,95％CI 2.583 ～ 9.192,P＜0.05)是小儿TOF根治术后早期死亡的危险因素.结论 临床上可根据患儿年龄、术前血氧饱和度、Nakata指数、体外循环时间及术后并发症预测TOF根治术后早期死亡的危险性.     

Aortic valve injury 10 years after tetralogy of Fallot repair

Summary Although recognized by pediatric cardiac surgeons, aortic insufficiency as a technical complication after tetralogy of Fallot repair is poorly documented, especially if it occurs late. The case of a boy with aortic insufficiency 10 years after complete tetralogy repair is described. No documentation in the literature other than summary statements in textbooks of this occurence was found.     

Prognosis in fetal tetralogy of Fallot

Summary The outcome in a series of 23 cases of tetralogy of Fallot diagnosed prenatally was compared to published figures for this condition when (a) identified postnatally or (b) treated surgically. There was a marked difference in the survival between these groups, with the highest mortality (75%) occurring when the diagnosis had been made prenatally. The high incidence of chromosomal and extracardiac anomalies in this group (60%) largely accounted for the discrepancy. This, and the potential for progressive changes in the anatomy of the defect during pregnancy, must be taken into account by the pediatric cardiologist offering prognosis in early pregnancy.     

法洛四联症根治术后远期心律失常的随访

目的　了解法洛四联症 (TOF)根治术后远期心律失常的发生率以及影响心律失常发生的相关因素。方法　 54例法洛四联症根治术后患儿 ,男 3 5例 ,女 19例 ;年龄 5～ 14岁 [( 9 4± 2 5)岁 ]。术时年龄 17个月～ 117个月 ,随访年限 2 8～ 9 0年。进行了心电图、2 4小时Holter、二维超声心动图和活动平板运动试验检查。结果　 18例患儿存在不同程度的室性心律失常 ,室性心律失常的发生率为 3 3 % ,其中 1例患儿有短阵室性心动过速发生。 4例右室收缩压增高者均存在明显室性心律失常 ,右室收缩压正常者室性心律失常发生率为 2 8% ( 14 / 50例 ) ,两者差异有显著性 (P =0 0 1)。体外循环时间≥ 90min者室性心律失常发生率为 53 % ( 10 / 19例 ) ,体外循环时间 <90min的患儿室性心律失常发生率为 2 3 % ( 8/ 3 5例 ) ,两者差异有显著性 (P <0 0 5)。轻度肺动脉瓣返流组室性心律失常发生率为 2 1% ( 4 / 19例 ) ,中、重度肺动脉瓣返流组室性心律失常的发生率为 40 % ( 14 / 3 5例 ) ,两组差异无显著性 ( χ2 =1 989,P >0 0 5)。随访年限 5年以内室性心律失常发生率为 3 2 % ( 8/ 2 5例 ) ,5年以上患儿的室性心律失常发生率为 3 4 % ( 10 / 2 9例 ) ,两者差异无显著性 ( χ2 =0 0 3 7,P >0 0 5)。手术年龄     

婴儿期法乐四联症的治疗

目的评价婴儿期法乐四联症(TOF)根治的可行性及优势,围手术期处理的特点.方法选取1991～2000年所有＜1岁的TOF患儿,均行Ⅰ期根治手术.结果全组患儿均治愈出院,术后紫绀消失,B超提示畸形纠正满意,术后心功能良好.结论对于病情较轻的TOF患儿,我们认为在6个月～1岁手术根治较为合适.如果患儿的病情较为严重,缺氧频繁发作,可早期手术治疗.患儿的围手术期处理要适应婴儿的病理生理特点.     

Evaluation of postoperative pulmonary regurgitation after surgical repair of tetralogy of Fallot: comparison between Doppler echocardiography and MR velocity mapping

Background Pulmonary regurgitation is a common finding in patients after correction of tetralogy of Fallot (TOF). Right ventricular impairment and even ventricular arrhythmia have been ascribed to pulmonary valve insufficiency (PI), which is therefore an important issue in follow-up examinations. Objective To compare PI measured by echocardiography (ECHO) with data provided by cardiac MRI (CMR). Materials and methods We studied 54 selected patients (18 female; median age 14.0 years, range 3.8–53.4 years) after surgical correction of TOF. To quantify pulmonary regurgitant fraction (PRF) by CMR, flow velocity mapping was performed. On Doppler ECHO, length, width and localization of regurgitant flow was measured. The severity of PI was categorized as mild, moderate or severe and compared to the data obtained by CMR. Results On CMR the mean PRF was 29.2 ± 13.4%. Patients with a transannular patch had a significantly higher PRF (39.9 ± 11.6%) than patients with an intact annular ring (23.6 ± 11.4%). Differentiation by Doppler ECHO between the categories mild, moderate and severe PI was confirmed by significant differences in PRF measured by CMR (mild vs. moderate P < 0.04; moderate vs. severe P < 0.014; mild vs. severe P < 0.001). Furthermore, PRF correlated with right ventricular end diastolic volume index (r = 0.45, P < 0.01) and right ventricular end systolic volume index (r = 0.39, P < 0.01). Conclusion Doppler ECHO can estimate the severity of PI after repair of TOF with acceptable results compared to CMR flow measurement. In univariate analysis there is only a weak influence of PRF on right ventricular volume.     

法洛四联症患儿类视黄酸受体α基因启动子区序列分析

目的 对法洛四联症（TOF）患儿类视黄酸受体α（RXRA）基因启动子区序列进行分析,探讨RXRA基因变异与TOF的关联性。方法 采取病例对照研究方法,以2007年4月至2012年12月心导管检查及外科手术证实为TOF患儿为TOF组,以同时期年龄和性别与TOF组匹配的健康儿童为对照组。采集静脉血,提取基因组DNA, PCR扩增RXRA基因转录起始位点（TSS）上游1 417 bp的启动子区序列,扩增产物采用ABI Prism Bigdye系统进行测序。结果 TOF组纳入213例（男135例,女78例）,平均年龄1.8岁;对照组纳入500名(男310名,女190名),平均年龄2.5岁。①RXRA基因TSS上游1 191 bp处检测出1个杂合突变,即-1 191A>AG（TSS定为+1）;检测出3个新发SNP,即-1 287C>CT、-800C>CA及-760C>CT。②利用http://www.cbrc.jp/research/db/TFSEARCH.html网站进行分析,发现在该4个位点及其附近有多个转录因子结合位点。-1 191A>AG导致新的CpG位点产生,-800C>CA导致原有CpG位点消失。这些新产生的CpG位点的甲基化可能会影响转录因子的结合,从而影响RXRA基因转录水平,进一步导致RXRA蛋白水平的变化。结论 TOF患儿RXRA基因启动子区序列变化可能通过影响RXRA表达水平而导致TOF的发生。     

小儿法洛四联症根治术106例临床分析

目的探讨小儿法洛四联症根治术的特点,以提高手术疗效。方法对1998-02—2005-07在河南省郑州市第七人民医院接受法洛四联症根治术的106例患儿的临床资料进行回顾性分析。结果治愈103例,死亡3例,病死率为2.9%。术后随访率100%,随访3～93个月,无晚期死亡。全部病例心功能皆恢复至Ⅰ或Ⅱ级(NYHA)。结论绝大部分法洛四联症患儿可施行一期根治术,提高手术成功率的关键在于合理的体外循环灌注、严密修补室间隔缺损和恰当疏通右室流出道。     

Aortopulmonary window coexisting with tetralogy of Fallot: Echocardiographic diagnosis

Summary Aortopulmonary window coexisting with tetralogy of Fallot (TF) was prospectively diagnosed by two-dimensional (2D) echocardiography and Doppler in an 18-month-old boy; the diagnosis was confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed, but the patient died in the operating room from right ventricular dysfunction. The autopsy showed an adequate surgical repair, but the histologic examination of the lungs demonstrated severe pulmonary vascular disease, which was presumed to be the cause of death.