Adult-onset type II citrullinemia and idiopathic neonatal hepatitis caused by citrin deficiency: involvement of the aspartate glutamate carrier for urea synthesis and maintenance of the urea cycle

Citrin is a mitochondrial aspartate glutamate carrier primarily expressed in the liver, heart, and kidney. We found that adult-onset type II citrullinemia is caused by mutations in the SLC25A13 gene that encodes for citrin. In this report, we describe the frequency of SLC25A13 mutations, the roles of citrin as a member of the urea cycle and as a member of the malate-aspartate shuttle, the relationship between its functions and symptoms of citrin deficiency, and therapeutic issues.     

Novel micro-crosslinked poly(organophosphazenes) with improved mechanical properties and controllable degradation rate as potential biodegradable matrix

As biodegradable materials, linear polyphosphazenes undergo rapid hydrolysis degradation but exhibit poor mechanical properties. Blending with biodegradable polyesters or inorganic particles strengthen their mechanical properties but give rise to slower degradation rate. To balance the mechanical properties and the degradation rate, micro-crosslinked polyphosphazenes were synthesized in this study. Their glass transition temperatures, mechanical properties, and in vitro degradation behavior were investigated. 2-hydroxyethyl methacrylate (HEMA) was firstly attached to the side chain along with glycine ethyl ester to prepare co-substituted poly(organophosphazene) with pendant ethenyl substituents. The co-substituted poly(organophosphazene) was blended with HEMA or acrylic acid (AA) followed by a free radical polymerization to prepare micro-crosslinked poly(organophosphazenes). The resulting crosslinked polymers showed two separate glass transition temperatures depending on the HEMA or AA feed. Incorporation of crosslinking affected the mechanical properties positively. Crosslinked poly(organophosphazenes) showed an approximately 11-17 fold increase in terms of modulus of elasticity when compared to the linear counterpart. In vitro degradation tests indicated that HEMA-crosslinked polymers hydrolyzed at a retarded rate while AA-crosslinked polymers hydrolyzed at a moderate rate compared to linear polymers.     

构建基于移动互联网的患者赋能型心衰健康管理平台

目的构建基于移动互联网的患者赋能型心衰健康管理平台,促进心衰患者进行自我管理,改善临床结局。方法开展课题研究型品管圈活动,搜集高质量临床证据,构建患者赋能型心衰健康管理平台,通过平台推广与应用,对心衰患者行为进行干预。结果平台应用后,医护人员心衰知识水平显著提升,心衰患者自我护理能力明显提升,患者再入院率、死亡率明显下降,应用效果良好。结论基于移动互联网的患者赋能型心衰健康管理平台是心衰慢性病管理的有效手段。     

改良Sugiura术治疗门静脉脉高压症20例报告

目的：探讨改良Sugiura术治疗门静脉高压症的疗效。方法：对20例门静脉高压症行改良Sugirua术者进行临床分析。结果：20例术后近期止血效果确切,随访观察发现19例食管静脉曲张消失或明显减轻。1例出现吻合口瘘及狭窄,经非手术治疗而愈。1例因肝肾功能衰竭死亡。结论：改良Sugiura术是治疗门静脉高压症的一种合理可取的术式。     

肝硬化患者红细胞内镁测定及其临床意义

目的　探讨肝硬化肝功能失代偿期患者红细胞内镁含量的变化及其临床意义。方法　研究对象为 32例肝硬化患者 ,按Child -Pugh分级法计肝功能B级 14例 ,C级 18例 ,合并肝性脑病 9例。对照组 5 9例为首次义务献血者。采用原子吸收光谱法测定受试者静脉血红细胞内镁浓度。结果　肝硬化组与对照组红细胞内镁浓度比较差异有非常显著意义 (P <0 .0 1)。肝硬化肝功能B级、C级红细胞内镁浓度与正常对照组比较均有非常显著差异 (P <0 .0 5 ,P <0 .0 1)。肝硬化合并肝性脑病 9例 ,其红细胞内镁浓度与C级无肝性脑病者比较 ,差异有非常显著意义 (P <0 .0 1)。结论　肝硬化患者红细胞内镁浓度的变化与肝性脑病的发生有一定关系。     

先天性心脏病儿童白细胞介素2活性及膜表面白细胞介素2受体的研究

目的 研究先天性心脏病（先心病）患儿的免疫功能状态。方法 采用间接免疫荧光检测膜表面白细胞介素2受体（Tac）、植物血凝素刺激后的膜表面白细胞介素2受体（Tac-a),MTT比色法检测白细胞介素2活性（IL－2）。结果 与健康儿童比较,先心病患儿IL－2活性和Tac-a都显著降低（P＜0．01）;与非紫绀型先心病患儿比较,紫绀型先心病患儿IL－2活性显著降低（P＜0．01）。结论 先心病患儿免疫功能缺陷可能是其易患感染性疾病的原因之一,增强免疫治疗对预防先心病患儿感染十分必要。     

药用辅料在制剂中的应用概述

药用辅料是药物制剂的基础材料的重要的组成部分,在制剂型和生产中起着关键作用,它不仅赋予药物一定剂型,并且与提高药物的疗效,降低毒副作用有很大的关系,因此,研究开发,合理应用辅仅可提高药物制剂质量和生产技术水平,而且可取得较大的社会及经济效益。     

经尿道前列腺气化术治疗前列腺增生症（附60例报道）

目的：观察经尿道气化术治疗前列腺增生症（BPH）的近期疗效。方法：应用经尿道前列腺气化术治疗BPH60例。结果：60例患手术均成功。手术时间20－＃120min。术中心电图、血压无改变。结论：认为经尿道前列腺气化术（TUVP）损伤小、安全性高、疗效确切且并发症少,尤其适用于有严重并发症的高危患,TUVP治疗BPH近期效果好,其远期效果有待进一步随访观察。     

脂多糖对大鼠肺微血管内皮细胞Gsα和Giα蛋白亚型的影响*

目的：观察脂多糖（LPS）对大鼠肺微血管内皮细胞（RPMVECs)G蛋白的影响及甲基强的松龙的干预作用。方法：用流式细胞技术（FCM）检测G蛋白亚型的变化。结果：①10μg/ml LPS作用于RPMVECs30和90min后,Gsα蛋白水平较对照组显著下降。甲基强的松龙干预30和90min后,对LPS致RPMVECs膜Gsα蛋白水平的变化有显著抑制作用。②10μg/ml LPS作用于RPMVECs30和90min后,Giα蛋白水平较对照组显著下降。甲基强的松龙干预30和90min后,对LPS致RPMVECs膜Giα蛋白水平的变化有显著抑制作用。结论：①LPS诱导RPMVECS Gsα和Giα蛋白水平的变化可能是LPS诱导RPMVECs单层通透性增加的机制之一。②甲基强的松龙参与抑制LPS诱导RPMVECs单层通透性增加的作用。