Tapering duodenoplasty and gastrojejunostomy in the management of idiopathic megaduodenum in children

PurposeThe purpose of this study is to present the management of idiopathic megaduodenum in children.MethodsA retrospective analysis of 4 cases of megaduodenum admitted from 2005 to 2011 was performed evaluating clinical features, radiologic data, treatment, pathologic findings, and prognosis. The corresponding literature was reviewed.ResultsThe diagnosis of nonobstructive megaduodenum was confirmed by upper gastrointestinal contrast study, ultrasonography, and exploratory laparotomy. Treatment consisted of either tapering duodenoplasty with pylorus division and closure of the proximal stump plus Roux-en-Y gastrojejunostomy or tapering duodenoplasty with closure of the proximal stump and end-to-side gastrojejunostomy. On pathologic evaluation, neural and vascular structures appeared normal in all sections. All symptoms, including diarrhea, bloating, vomiting, and nausea, had resolved on follow-up, and all patients experienced rapid weight gain after their operation.ConclusionsIdiopathic megaduodenum without organic obstruction is a rare clinical condition. Massive dilatation confined to the duodenum was shown by upper gastrointestinal contrast studies and ultrasonography and can also be identified on antenatal ultrasonography. In children with megaduodenum, satisfactory results can be obtained by tapering duodenoplasty with proximal stump closure and gastrojejunostomy with either Roux-en-Y or end-to-side anastomosis.     

Application of MPVR and TL-VR with 64-row MDCT in neonates with congenital EA and distal TEF

AIM:To assess the application of multiple planar volume reconstruction(MPVR) and three-dimensional (3D) transparency lung volume rendering(TL-VR) with 64-row multidetector-row computed tomography (MDCT) in neonates with congenital esophageal atresia (EA) and distal tracheoesophageal fistula(TEF).METHODS:Twenty neonates(17 boys,3 girls) with EA and distal TEF at a mean age of 4.6 d(range 1-16 d) were enrolled in this study.A helical scan of 64-row MDCT was performed at the 64 mm×0.625 mm collimation.EA and T...     

儿童1型糖尿病合并自身免疫性甲状腺疾病12例临床分析

目的 分析儿童1型糖尿病(T1DM)合并自身免疫甲状腺疾病(AITD)对T1DM治疗的影响。方法 1993～2002年在我院诊治的T1DM患儿合并甲状腺疾病者12例。对患儿的病史、家族史、体格检查及内分泌相关检查的结果进行分析。抗体检查：GAD-Ab、IAA、ICA、TG-Ab、TPO-Ab、TRAb和肾上腺皮质细胞浆抗体(ACC)。采用t检验将T1DM合并Graves病(GD)与合并桥本甲状腺炎(HT)者进行糖化血红蛋白(HbA1c)和胰岛素用量的分组比较；采用秩和检验对病程进行比较。结果 该组儿童患GD和HT者，分别为4例和8例。T1DM和AITD发病间隔为0～10年；GD的临床表现可不突出。有内分泌家族史者占25％。GD控制前患儿的HbA1c分别为10％、12％和14％，1例结束GD疗程者的HbA1c为7．8％。合并HT者的糖尿病代谢控制相对较好，HbA1c为7．2％～10％，1例新诊断T1DM合并HT且尚未治疗HT的患儿，HbA1c为6．3％。经t检验显示两组患儿的HbA1c差异有显著意义(P=0．011)，而病程和胰岛素用量差异无显著意义。结论该组患儿女性多于男性；HT患儿较GD患儿为多；有较强的内分泌疾病家族史。AITD影响糖尿病控制。T1DM患儿宜定期进行甲状腺自身抗体和甲状腺功能检查。     

Ceramides are associated with inflammatory processes in human mediastinal adipose tissue

Background and AimCeramides are poorly characterized in human adipose tissue. The aim of this study was to investigate concentrations of different ceramide species in human subcutaneous and visceral adipose tissue depots and to determine associations between ceramides and global gene expression profiles.Methods and ResultsConcentrations of six ceramide species were determined in plasma and in subcutaneous and mediastinal adipose tissue from 10 overweight subjects (BMI 29.4 ± 4.9 kg/m²). In the adipose tissue biopsies gene expression arrays were performed and relationships between ceramides and gene expression analyzed. Immunostaining of the two adipose tissue depots was performed in an independent group of 10 patients. Mediastinal adipose tissue contained significantly higher concentrations (p < 0.05) of all six ceramide species than the subcutaneous depot. Of the six ceramides in plasma, concentrations of only two (Cer d18:1/18:0 and Cer d18:1/22:0) correlated significantly (p < 0.05) with the corresponding species in mediastinal adipose tissue, but there were no significant correlations between ceramides in plasma and subcutaneous adipose tissue. Multivariate analysis identified significant correlations between the total ceramide concentration and global gene expression within mediastinal, but not subcutaneous adipose tissue, according to cross-validation. Gene ontology analysis of genes related to ceramides in the mediastinal depot revealed that genes positively correlated with ceramides were associated mainly with immune and inflammatory categories, while genes negatively correlated with ceramides were associated mainly with lipid and carbohydrate metabolism.ConclusionsCeramides in human mediastinal adipose tissue may be involved in inflammation and lipid and carbohydrate metabolism.     

颅内外血管架桥术治疗缺血性脑血管病疗效的再评价

目的 对颅内外血管架桥术治疗缺血性脑血管病的疗效进行再评价。 方法 对30例经正规内科治疗后仍有颈内动脉系统缺血症状的患者,进行了颞浅动脉-大脑中动脉吻合术,并对术后患者的脑血流改善情况及临床情况进行了分析。 结果 术前患者均为一过性缺血发作神经性缺损(TIA),可逆性缺血性神经障碍(RIND)和不典型脑缺血症状,术前CT,MRI检查无大面积脑梗死,SPECT·rCBF均有脑血流减低区。术后血管造影显示吻合口通畅率为86.7％,术后局部脑血流改善率为70％,随访仅发现2例患者有2次TIA,无完全性卒中发生,术前有轻度神经功能障碍的8例患者,术后均有不同程度的恢复。 结论颞浅动脉-大脑中动脉吻合术可预防颈内动脉系统血流动力学障碍性脑缺血发展成完全性卒中。     

腔内心电图定位技术在儿童PICC置管中的应用进展

本文报告了腔内心电图(EKG)定位技术在儿童PICC置管中应用的重要意义,从国内外儿童患者PICC置管过程中EKG的应用情况、EKG定位工具的选择、引导方式、如何通过P波变化判断导管尖端位置,并分析了影响儿童EKG稳定性的相关因素,展望了EKG定位技术在儿童PICC置管中的应用前景,并提出了建议,便于对临床提供指导。     

沉默囊性纤维化跨膜传导调节因子对大鼠海马神经元细胞线粒体功能及氧化应激的影响

目的探讨沉默囊性纤维化跨膜传导调节因子(cystic fibrosis transmembrane conductance regulator,CFTR)在大鼠海马神经元细胞线粒体功能及氧化应激中的作用。方法18只SD大鼠随机分为CFTR小干扰RNA(small interfering RNA,siRNA)组、阴性对照组、空白对照组各6只。分离3组海马神经元细胞,取对数生长期细胞进行实验。CFTR siRNA组神经元细胞转染CFTR siRNA,阴性对照组转染阴性对照siRNA,空白对照组不转染。转染后培养48 h,采用Western blot法检测3组神经元细胞线粒体CFTR蛋白相对表达量,JC-1荧光探针检测线粒体膜电位,荧光分光光度法检测线粒体活性氧(reactive oxygen species,ROS)和过氧化氢(hydrogen peroxide,H2O2)表达,氧电极法测定线粒体呼吸控制率(respiratory control rate,RCR),荧光素酶发光法检测线粒体三磷酸腺苷(adenosine triphosphate,ATP)浓度,高效液相色谱法检测线粒体谷胱甘肽(glutathione,GSH)和氧化型谷胱甘肽(oxidized glutathione,GSSG)表达。结果转染后培养48 h,CFTR siRNA组神经元细胞线粒体CFTR蛋白相对表达量(0.09±0.01)、线粒体膜电位[(247.16±34.28)mV]、ATP[(11.71±2.54)mol/g]、RCR(4.69±0.82)、GSH[(0.71±0.12)nmol/μg]、GSH/GSSG值(0.31±0.08)低于空白对照组[0.42±0.07、(432.18±56.04)mV、(24.18±3.64)mol/g、7.94±1.21、(1.62±0.23)nmol/μg、1.65±0.27]和阴性对照组[0.38±0.06、(434.07±52.13)mV、(22.97±3.43)mol/g、7.83±1.18、(1.57±0.21)nmol/μg、1.63±0.24](P<0.05),线粒体H2O2水平(1.73±0.11)高于空白对照组(1.00±0.02)和阴性对照组(1.08±0.06)(P<0.05);空白对照组神经元细胞线粒体CFTR蛋白相对表达量及线粒体膜电位、ATP、RCR、H2O2、GSH、GSH/GSSG值与阴性对照组比较差异均无统计学意义(P>0.05)。结论沉默大鼠神经元细胞线粒体CFTR可引起海马神经元细胞线粒体功能障碍,增强氧化应激反应。     

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目的探讨应用维持性血液透析(maintenance hemodialysis,MHD)治疗的终末期肾病(end-stage renaldisease,ESRD)患儿的临床特点,为今后儿童MHD的治疗提供参考。方法首都医科大学附属北京儿童医院血液净化中心2000年1月至2011年3月因ESRD行MHD的患儿共23例,其中男12例,女11例;年龄2.5～15.6岁。对其原发病、退出原因、透析并发症及透析充分性等进行统计和回顾性分析。结果在MHD儿童中ESRD的原发病中慢性肾小球肾炎8例,居首位,占34.8%,原因不明者5例,占21.7%。HD开始时均以临时中心静脉双腔管为血管通路,87.0%成功建立动静脉内瘘。动静脉瘘组尿素清除指数(spKt/V)(1.52±0.39),尿素下降率为(70.70±10.52)%,明显优于临时静脉双腔管组的(1.08±0.50)和(56.44±17.33)%。透析并发症以高血压、低血压和透析失衡综合征为常见,高血压发生率达87.0%,而低血压和失衡综合征发生率仅为5.6%和4.7%。退出原因43.5%为回当地医院透析,17.4%肾移植,死亡1例(4.3%),放弃治疗2例(8.7%)...     

儿童缺血性脑卒中远期预后追踪研究

目的 探讨儿童缺血性脑卒中（IS）远期预后并初步探讨影响其预后的因素。方法 收集1992年1月至1997年5月首次入住首都医科大学附属北京儿童医院（我院）的IS连续病例。对住院病历进行回顾性分析,了解其发病时的病因、临床表现及影像学特征。愿意接受随访的患儿到我院随访,项目包括问卷调查、物理检查、日常生活活动（ADL）评定、智力测量、患肢运动功能评定（FMA评估）、头颅MRI和磁共振血管造影术（MRA）检查,分析IS的远期预后。结果 44例IS连续病例符合纳入标准,其中1例病初死亡,3例无法取得联系,15例拒绝随访。25例进入分析,其中男14例,女11例。起病至本次随访的间隔时间平均为12.2年,随访时平均年龄16.3岁（11.3～24.2岁）。①起病时23例表现为偏瘫症状,2例四肢瘫。10例患儿病因不明,呼吸道感染5例,轻微头外伤5例,Moyamoya病2例,同时呼吸道感染和头外伤1例,心脏病和脑动脉炎各1例。②5/25例起病入院时出现惊厥的患儿出院后未再出现惊厥;起病时未发生惊厥的2例患儿出现癫发作;2例Moyamoya病患儿出院后出现IS复发。③25例患儿肌力均较起病时提高;25例患儿ADL得分在118～126分,均符合ADL功能独立;FMA评分结果显示11例（44％）患儿患肢运动功能达到正常。④7例（28%）患儿全量表智商<70分;15例（60%）患儿存在心理问题,包括记忆力差11例,注意力不集中10例,自信心不强9例,动作过多7例,社交恐怖7例。⑤MRA检查显示3例起病时血管狭窄的患儿其狭窄程度明显减轻,余22例患儿无明显改变。⑥基底节、内囊和丘脑部位梗死者以及特发性脑卒中患儿FMA评分较高。结论 儿童IS远期预后较好,均可达到生活自理。约50%患儿仍存在不同程度的运动功能障碍和心理问题,整体认知能力偏低。基底节、内囊和丘脑部位梗死和特发性IS者运动功能预后良好。     

儿童EBV感染的非肿瘤性相关疾病

EBV是一种亲人类B细胞的疱疹病毒.EBV可引起全身各系统和器官的疾病,包括肿瘤性和非肿瘤性相关疾病[1].在儿童非肿瘤性EBV感染相关性疾病中,传染性单核细胞增多症(IM) 、慢性活动性EBV感染(CAEBV)和EBV相关噬血淋巴组织细胞增生症(EBV-HLH) 最具临床意义.IM是良性自限性疾病,而CAEBV和EBV-HLH具有潜在恶性倾向,预后不良.本文就首都医科大学附属北京儿童医院(我院)诊断的IM、CAEBV和EBV-HLH各1例典型病例进行分析,以期提高对该类疾病的认识.