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目的 比较不同机械通气方式对胎粪吸入综合征(MAS)患儿的治疗作用及并发症的发生.方法 河北省儿童医院新生儿科重症监护室2006年7月至2008年7月收治的84例氧合指数(OI)≥15的重症胎粪吸入综合征患儿随机分为3组:A组25例患儿采用高频振荡(HFOV)模式治疗;B组29例患儿采用同步间歇正压联合容量保证(SIPPV+VG)模式治疗:C组30例采用间歇指令通气(IMV)模式治疗.比较3组治疗前后各时间点OI、动脉,肺泡氧分压比值(a/APO2)的变化,并对3组患儿的氧暴露时间、机械通气时间,以及肺气漏、呼吸机相关性肺炎、Ⅲ度以上颅内出血等并发症的发生率进行比较.结果 A组与B组在机械通气24h后OI和a/APO2值均与0h差异有统计学意义(P<0.05),而C组直到72 h方与0 h时差异有统计学意义(P<0.05).A组与B组各时间点相比差异均无统计学意义.A组与B组相比,氧暴露时间和上呼吸机时间均无统计学差异(P>0.05),但是A组与C组、B组与C组相比在统计学上差异均有统计学意义(P<0.05).不仅如此,A组与C组、B组与C组相比,肺气漏与呼吸机相关性肺炎的发生率差异均有统计学意义(P<0.05).Ⅲ度以上颅内出血的发生率3组相比差异无统计学意义(P>0.05).结论 HFOV、SIPPV+VG通气模式与IMV比较,可以较快改善MAS的氧合,缩短氧暴露时间和呼吸机使用时间,降低肺气漏和呼吸机相关性肺炎的发生,可作为更有效、更安全的抢救性治疗手段.     

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目的通过分子流行病学调查研究兰州地区婴幼儿病毒性腹泻的病原学特点。方法采用酶联免疫吸附试验(ELISA)及逆转录聚合酶链反应(RTPCR),对2003年7月至2004年6月兰州地区收集的624例婴幼儿腹泻粪便标本随机抽取271例进行轮状病毒(RV)、杯状病毒(HuCV)及星状病毒(AstV)检测。结果在271例标本中共检出RV感染153例(56.46%),其中G394例(61.44%),G24例(2.61%),G93例(1.96%),未发现G1、G4型和混合感染;在随机抽取的69例G分型阳性标本中,检出P[8]型28例(40.58%);RV的感染对象主要为6～23月龄的婴幼儿,发病高峰在10、11月份(86.27%、73.81%)。在118例RVELISA阴性标本中检出HuCV感染13例(11.02%),其中诺如病毒(NLV)GⅡ型11例,札如病毒(SLV)2例,未检出NLVGⅡ型,发病年龄1～18个月(11.31±4.53个月);同时检出AstV感染7例(5.93%),发病年龄4～12个月(8.27±2.69)个月,其中有1例合并有SLV感染,另有1例为迁延性腹泻。HuCV和AstV感染均未表现出明显的季节性。结论RV是兰州婴幼儿病毒性腹泻的主要病原,其流行的主要血清型为G3型,HuCV和AstV亦是重要病原之一。     

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??Abstract?? Objective To identify the clinical features and the prognosis of anti-NMDAR encephalitis in children??so as to improve the pediatrician’s awareness about this disease. Methods The clinical features??therapeutic regimen and prognosis of 17 children with anti-NMDAR encephalitis were summarized by a retrospective and follow-up study. Results Among the 17 patients with anti-NMDAR encephalitis??12 were males and 5 were females.Their age varied from 2 to 12 years??and the average age was 5 years 8 months.The most common initial symptoms were convulsions ??7 cases?? and psychiatric symptoms ??7 cases??.The common symptoms in the course of the disease were psychiatric symptoms??17 cases????sleep disorders ??16 cases????extrapyramidal symptoms ??14 cases????and convulsion ??11 cases??.Only 4 patients had autonomic symptoms.The anti-NMDAR antibody were found in all the patients’CSF.The EEG test of 16 patients showed diffused slow background.Tumors were not found in any patient. Fourteen cases were treated with IVIg and methylprednisolone??2 cases only received IVIg therapy and 1 case gave up.Rituximab ??2 cases?? and cyclophosphamide ??1 cases?? were administrated in 3 cases with no improvement after the above treatment.The patients had been followed up for 3 to 15 months.Twelve of them recovered completely??2 cases died??2 cases had epilepsy??and 1 case had speech disorder. Conclusion Psychiatric symptoms??sleep disorders??extrapyramidal symptoms and convulsion are the common symptoms of anti-NMDAR encephalitis in children.Tumors and autonomic nervous system dysfunction rarely happen. Immunotherapy is effective in the majority of the patients.The prognosis of anti-NMDAR encephalitis in children is optimistic.     

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目的 通过比较小于胎龄儿(SGA)和足月正常新生儿脐血Ghrelin水平的变化,分析脐血Ghrelin与新生儿出生体重、身长、头围、BMI和胎盘重量、胎盘体积的关系,以及脐血Ghrelin水平与IGF-1、胰岛素、胰岛素敏感性和血糖的关系.探讨脐血Ghrelin水平与胎儿发育和代谢轴之间,尤其是Ghrelin与IGF-1的相互关系.方法 2006年12月至2007年3月选择中山大学附属一院新生儿78例,SGA组22例为观察组,足月适于胎龄儿(AGA)42例为正常对照组.采用酶联免疫反应法(ELISA)对新生儿脐血Ghrelin、胰岛素、IGF-1进行测定分析,测量新生儿净体重、身长、头围、胎盘体积、胎盘重量,计算体重指数(BMI)和胰岛素敏感指数(ISI).结果 (1)SGA组脐血Ghrelin水平(38.74±4.90 ng/mL)明显高于正常对照组(23.37±8.78 ng/mL),其差异有统计学意义(P＜0.05).(2)脐血Ghrelin水平与新生儿出生体重、身长、头围、BMI、胎盘重量、胎盘体积呈负相关,均有统计学意义(P＜0.05).(3)脐血Ghrelin水平与IGF-1、胰岛素、ISI呈负相关均有统计学意义(P＜0.05).与血糖无相关性(r=0.081,P=0.454).(4)通过偏相关分析,控制出生体重、身长、胎盘重量、BMI、胰岛素后,Ghrelin与IGF-1有明显负相关性,有统计学意义.(5)剖宫产新生儿脐血Ghrelin水平与顺产儿无明显差异(P=0.956);男性与女性新生儿脐血Ghrelin水平无明显差异(P=0.385).结论 新生儿脐血Ghrelin水平是反映胎儿的宫内发育及营养状态的指标之一.并以负反馈形式参与胎儿宫内发育和代谢轴的调控过程.小于胎龄儿出生时可能存在胰岛素抵抗.小于胎龄儿高Ghrelin并没有刺激胰岛素释放,提示小于胎龄儿可能存在Ghrelin受体或受体后信号传导通路的抵抗.     

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??Abstract?? Objective To evaluate the clinical value of dual-source computed tomography angiography ??DSCTA?? in diagnosis of the heterotaxy syndrome. Methods Nine patiens with heterotaxy syndrome underwent DSCTA and echocardiography before operation?? then?? retrospective analysis was performed. Prospective ECG-triggering DSCTA scanning with low dosage was applied?? scanning range was from the superior aperture of thorax to the epigastrium?? and then anomalies of the heart?? great vessels?? lung?? spleen?? and liver were observed by means of multiplanar reconstruction. Result Left atrium isomerism was seen in 5 cases?? 1 was atrial septal defect ??ASD???? 1 was ASD and ventricular septal defect ??VSD???? 1 was single ventricle ??SV???? and 2 were double outlet of right ventricle ??DORV??. Bilateral morphologic left bronchi?? bilaterally hyparterial bronchus and polysplenia were identified?? accompanied with abnormal abdominal visceral position in 4 cases?? left ventricular loops and mirror dextrocardia in 1 case?? interrupted inferior vena cava and double superior vena cava in 3 cases?? right aortic arch in 2 cases?? total anomalous pulmonary venous connection in 1 case?? and a right-sided stomach in 4 cases. Right atrial isomerism was seen in 4 cases??3 of which were associated with SV. Bilateral morphologic right bronchi?? bilaterally eparterial bronchus, asplenia, and symmetrical liver were identified in the patients?? accompanied with a right-sided stomach in 2 cases. As well as right-sided atrioventricular connectionless in 1 case and transposition of the great artery in 1 case. The main vascular malformations outside the heart were 21 detected with DSCTA ?? contrasting to 14 detected with echocardiography. In the 9 patients?? 5 cases were performed anatomic surgical correction?? 3 cases were performed palliative treatment?? 1 case died of persistent hypoxia and function failure in multiple organ after Fanton operation. Following-up time was from half year to seven years. Conclusion DSCTA can accurately diagnose heterotaxy syndrome and clear show the status of atrium?? spleen?? liver and bronchus; furthermore?? it can provide an accurate diagnosis of complex congential malformations of cardiovascular?? which is very helpful in making decision before operation.     

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目的探讨肺泡表面活性物质蛋白A1(SPA1)基因多态性与新生儿支气管肺发育不良(BPD)相关性。方法应用聚合酶链反应-限制酶切技术(PCR-RFLP)分析2008年7月至2010年10月同济医院接诊的38例武汉汉族BPD患儿与55例对照组的SPA1基因分布。结果 BPD组和对照组之间AA50基因型分布(P=0.038)和等位基因频率(P=0.012)差异有统计学意义。AA219、AA62、AA19三个位点基因型分布和等位基因频率差异无统计学意义(P>0.05)。AA50病例组CC基因型明显低于对照组(7vs23),差异有统计学意义(P<0.05)。结论 SPA1上AA50位点基因多态性可能与新生儿支气管肺发育不良有关,CC基因型可能对BPD患病有保护作用。     

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??Objective??To investigate the value of serum intestinal fatty acid binding protein??I-FABP?? and serum amyloid A??SAA?? in the diagnosis of necrotizing enterocolitis??NEC??in the newborn. Methods??Fifty-six preterm infants with a confirmed diagnosis of NEC from October 2014 to October 2015 were recruited as case group??stage??26 cases??stage??/??30 cases??. Thirty children diagnosed with non-digestive diseases in the same period were recruited as the control group. Serum levels of I-FABP and SAA were determined by enzyme-linked immunosorbent assay.The diagnostic value of I-FABP and SAA for severe NEC was assessed using the receiver operating characteristic??ROC??curve. Results??Stage??/?? cases in the case group had significantly higher serum I-FABP levels and SAA levels than the control group and Stage??cases??P??0.05??. The area under the ROC curve for serum I-FABP was 0.80??95%CI??0.69-0.92????with the optimal cut-off point of 21.8 μg/L. Under this cut-off point??the sensitivity and specificity were 70.0%and 81.0%??respectively. The area under the ROC curve for SAA was 0.76??95%CI??0.63-0.89????with the optimal cut-off point of 1657.8 μg/L. Under this cut-off point??the sensitivity and specificity were 67.0% and 80.0%??respectively. Conclusion??In newborn infants with NEC??serum I-FABP and SAA l can be used as biomarkers for the diagnosis of severe NEC.     

2003-2008�걱����ͯҽԺŧ�崯����A��β��Ѫ���������������ҩ�����⼰emm�����о�

目的研究北京儿童医院脓疱疮患儿临床分离A组β溶血性链球菌(GAS)及其对抗菌药物的敏感性情况。方法收集北京儿童医院2003—2008年收治的1735例脓疱疮儿童皮损部位分离出的52株GAS菌株,采用琼脂稀释法测定9种抗菌药物的最低抑菌质量浓度(MIC)值;用聚合酶链反应方法对上述菌株进行大环内酯类抗生素耐药基因ermB、ermA和mefA检测以及emm分型情况。结果分离株对四环素及大环内酯类抗生素耐药率均为100%,MIC>256mg/L;对青霉素、头孢拉啶及氧氟沙星的敏感率高达100%。大环内酯类抗生素耐药基因ermB、ermA和mefA和阳性率分别为92.30%、7.70%和0;分离株的emm分型最常见的为emm12.0(53.85%),emm1.0(36.54%)。结论北京儿童医院收治的脓疱疮儿童GAS感染流行菌株对大环内酯类抗生素耐药率很高,主要耐药机制为ermB编码的23SrRNA甲基化酶导致靶位改变,在北京地区青霉素类和头孢菌素类抗生素仍是治疗GAS感染首选药物。     

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??Abstract??Objective??To observe the change of NT-proBNP in incomplete Kawasaki disease and study the value in the diagnosis of incomplete Kawasaki disease. Methods??From Mar. 2007 to Feb. 2009 in department of paediatrics??Provincial Clinical Medical College of Fujian Medical University??we detected the acute- and restoration-stage plasma NT-proBNP level in 36 children with typical Kawasaki disease??TKD?? and 20 with incomplete Kawasaki disease ??IKD??respectively and 30 children with respiratory tract infection and 30 healthy children?? and made a mutual comparison. Meanwhile ultrasound cardiogram examination was made in Kawasaki disease. Results??There was no statistical significance in the difference of plasma NT-proBNP between TKD group and IKD group. Acute-stage plasma NT-preBNP in TKD group and in IKD group was clearly higher than that in restoration stage and control group??respiratory tract infection group and healthy children group??. Coronary lesion in IKD group was significantly higher than that in TKD. Conclusion??The plasma NT-proBNP can be used as a reference index in early diagnosis of IKD.     

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目的探讨儿童体位性心动过速综合征(POTS)的临床特点。方法2008年5月至2009年10月于北京大学第一医院儿科门诊就诊,经直立试验或直立倾斜试验确诊POTS的患儿109例(POTS组),平均年龄(11.79±2.55)岁;20名健康儿童为对照组,平均年龄(11.55±3.65)岁。对每例POTS患儿详细询问病史并进行体格检查,对比分析两组儿童在生活习惯、家族史及体质特征方面特点,总结POTS组患儿发病的临床特征。结果与对照组相比,POTS组患儿在性别比例、年龄、身高、体重、平卧心率、平均动脉压方面差异无统计学意义。POTS患儿主要症状以晕厥多见(52.3%),42.2%症状发作频繁(就诊时>10次),主要症状发生季节以夏秋季多见(42.1%),发作持续时间多在1min以内(29%)。83.5%患儿发作前有诱因,发作诱因以持久站立为多见(50.5%),发作前多伴有先兆症状(78.0%),其中以头晕、黑矇、大汗、面色苍白最为常见。18例(16.5%)患儿有伴随症状,32例(29.4%)发作后仍有不适,以乏力最常见(24例)。90例(82.6%)患儿无既往疾病史,30例(27.6%)有直立不耐受家族史,46例(...     

儿童体位性心动过速的发病机制研究进展

体位性心动过速综合征(postural orthostatic tachycardia syndrome,POTS)是以窦性心率增加为主要临床表现的一类疾病,与患儿的体位变化有密切关系.患儿由卧位到站立位体位变换后,10 min内心率突然增快,超过一定的界值可以诊断为POTS.POTS的发病机制目前尚未完全明确.目前研究发现,POTS主要与中心血容量变化、自主神经及肌肉泵功能障碍、血管内皮功能异常等有关.     

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??Abstract??Objective The time-effect relationship in treating postural orthostatic tachycardia syndrome ??POTS?? of children with alpha receptor agonist midodrine hydrochloride was analyzed to explore the optimum course of treatment for POTS children. Methods A total of 104 cases of the POTS children were studied from 2005 to 2011 in Peking University First Hospital. A chi-square test was used to evaluate the relationship between effect and time of medication?? and the time-effect curve was constructed. Results According to the 104 cases?? the time accumulative total efficacy for 1 month?? 2 months?? 3 months?? 4 months?? 5 months and 6 months were 19.23%?? 48.08%?? 69.23%?? 73.08%?? 74.04% and 75.96%?? respectively. The time accumulative total efficacy for 3 months was higher than that for 1 month or 2 months ??P??0.05???? but there was no difference in the efficacy among 4 months?? 5 months and 6 months treatment and with 3 months treatment. Conclusion It has the best treatment efficacy when the course of treatment for POTS children with midodrine hydrochloride is 3 months?? and prolonging the duration of treatment does not significantly improve the therapeutic effect.     

儿童体位性心动过速综合征诊治策略

体位性心动过速综合征(POTS)是一组以慢性直立不耐受及直立后心率过度增快为主要特征的临床综合征,在儿童及青少年时期较为常见,可显著影响儿童的生活质量及学习。POTS症状涉及多个系统,并可伴有多种共患疾病,临床表现复杂,在诊断中需详细询问病史,正确解读直立试验结果,谨慎鉴别诊断并全面评估共患病。POTS主要有相对低血容量、高肾上腺素能及血管功能障碍三种核心机制。在治疗POTS患儿时,应以非药物治疗为基础,针对患儿的临床特点及生物标志物水平评估主要发病机制,注意共患疾病的管理,从而进行个体化的综合治疗。     

儿童体位性心动过速综合征的诊断与治疗

<正>体位性心动过速综合征(postural tachycardia syndrome,POTS)是指存在直立不耐受(OI)的症状,并且患者在直立或直立倾斜试验(HUTT)10min内的心率比卧位时增加≥     

儿童体位性心动过速综合征合并血管迷走性晕厥的诊断探讨

目的：探讨儿童体位性心动过速综合征（POTS）合并血管迷走性晕厥（VVS）的诊断。方法：回顾性地复习2007年1月至2010年12月经直立倾斜试验（HUT）诊断为POTS的57例儿童的临床资料,其中男29例,女28例,年龄5～16(12.2±1.9)岁。结果：57例POTS儿童中,在可以耐受倾斜体位的前提下,通过延长HUT时间,24例(42%)经HUT诊断合并VVS,其中血管抑制型20例,混合型3例,心脏抑制型1例。合并VVS儿童平均年龄（13.0±1.4岁）大于未合并VVS的POTS儿童（11.5±2.1岁）,差异有统计学意义（P0.05）。结论：部分POTS儿童合并VVS,在可以耐受倾斜体位的前提下,延长HUT时间有利于防止VVS漏诊。与未合并VVS的POTS儿童比较,合并VVS儿童年龄较大,但性别、临床症状无明显差异。     

血管舒张反应关系研究

目的 探讨体位性心动过速综合征（POTS）儿童血浆硫化氢（H2S）含量和血流介导血管舒张反应（FMD）的变化及二者之间的关系。方法 收集自2013年1月至7月在北京大学第一医院儿科门诊或住院部经直立试验或直立倾斜试验确诊为POTS的儿童36例,（11±3）岁;以18例正常儿童为对照组,（11±4）岁。POTS的诊断依据临床表现及直立试验或直立倾斜试验,血浆H2S含量测定使用敏感硫电极方法,FMD的测定使用彩色多普勒超声检测方法。结果 两组儿童在年龄、性别分布、身高、体重、体重指数、平均动脉压方面差异无统计学意义（P＞0.05）,POTS组儿童平卧位心率明显高于正常对照,分别为（88±15）次/min和（77±11）次/min（P = 0.006）。血浆H2S含量在POTS组明显高于正常对照［（30.25±11.64） μmol/L和（17.58±1.74） μmol/L, P＜0.01］, FMD水平在POTS组明显高于正常对照［（9.87±1.63）%和（5.92±2.14）%, P＜0.001］,血浆H2S含量与FMD水平呈明显正相关,相关系数为0.565（P＜0.001）。结论 POTS儿童血浆H2S含量和FMD水平明显升高,提示POTS儿童中异常的血管舒张反应。     

选择性α1受体激动剂对儿童体位性心动过速综合征的治疗研究

目的 探讨选择性α1受体激动剂盐酸米多君对体位性心动过速综合征(POTS)的治疗效果.方法 本组55例,为2000年5月-2007年9月在北京大学第一医院就诊或住院的POTS患儿,其中男23例,女32例;年龄5～19岁,平均(12.3±3.1)岁,随机分为治疗组(应用盐酸米多君及口服补液盐治疗)22例和对照组(应用口服补液盐治疗)33例,对两组儿童进行临床研究及直立试验、基础直立倾斜试验(BHUT)或硝酸甘油激发直立倾斜试验(SNHUT)研究,比较治疗前后患者的好转率、HUT转阴率以及随访终点时的临床痊愈率和有效率.采用SPSS10.0软件进行统计.结果 治疗3周和6周后治疗组的好转率均明显高于对照组(100.0% vs 42.4%,P＜0.001;100.0% vs 42.4%,X2=19.352,P＜0.001),随访终点时治疗组的临床痊愈率明显高于对照组(77.3% vs 27.3%,X2=13.239,P＜0.001),有效率也明显高于对照组(100.0% vs 36.4%,X2=22.647,P＜0.001).治疗3周后治疗组和对照组HUT转阴率相比差异无统计学意义(31.8% vs 12.1%,P＞0.05);治疗6周后治疗组的HUT转阴率明显高于对照组(81.0% vs 48.5%,P＜0.05).结论 选择性α1受体激动剂盐酸米多君对POTS患儿的治疗有效.