目的探讨3例儿童糖原累积病Ib型(glycogen storage disease type Ib,GSDIb)的临床特征和基因变异特点,分析恩格列净治疗GSDIb患儿的临床效果。方法回顾性分析2020年6月至2023年5月在郑州大学附属儿童医院就诊的3例GSDIb型患儿临床资料...目的探讨3例儿童糖原累积病Ib型(glycogen storage disease type Ib,GSDIb)的临床特征和基因变异特点,分析恩格列净治疗GSDIb患儿的临床效果。方法回顾性分析2020年6月至2023年5月在郑州大学附属儿童医院就诊的3例GSDIb型患儿临床资料及基因检测结果。3例患儿均接受恩格列净治疗及饮食干预,随访1~3年,并回顾相关文献。结果3例患儿均为男性,就诊年龄7个月~1岁,主要临床表现为肝脏增大及智力运动发育落后,代谢异常均表现为肝功能异常、空腹低血糖、血清乳酸及三酰甘油升高,均伴有中性粒细胞减少,3例患儿均检出SLC37A4基因复合杂合变异。应用恩格列净治疗年龄分别为1岁2个月、1岁、1岁10个月,随访时间分别为12、17、36个月,均未发生低血糖、泌尿系统感染、肝肾功能异常等不良反应,临床症状得到改善。本研究发现SLC37A4基因1个新变异位点c.1287_1290del,拓展了SLC37A4基因变异谱。结论GSDIb型患儿的临床表现多样,存在中性粒细胞减少及功能缺陷,确诊依赖于基因检测,恩格列净可增加GSDIb患儿中性粒细胞数目,改善患儿临床症状,尽早应用可避免炎症性肠病的发生。展开更多
根据国家教育部门的统计,目前海外将近有1.5亿人在学习中文,许多国家学习中文的人数以百分之五十甚至更高幅度增产,非母语中文学习者的年龄也在逐渐下移。这些留学生或来自或未来将继续IB课程体系,那么其中文学习部分是孤立进行还是可...根据国家教育部门的统计,目前海外将近有1.5亿人在学习中文,许多国家学习中文的人数以百分之五十甚至更高幅度增产,非母语中文学习者的年龄也在逐渐下移。这些留学生或来自或未来将继续IB课程体系,那么其中文学习部分是孤立进行还是可向IB课程靠拢以及如何靠拢正是本文研究的要义。IB国际文凭项目将课程分为语言和文学(语言A)、语言习得(语言B)、个人与社会等六个组别。语言B又称作“第二语言”,其教学理念及培养目标都对国际青少年中文教育的发展具有借鉴意义。话题是语言交际的依托,所以教材中有关课文、词汇和语法项目的编写都离不开话题,都需要依托话题来展开。笔者将以《YCT考试大纲与应考指南》里的话题分类为参考依据,对《YCT标准教程》系列教材的话题内容、出现次数进行归类,其后将其与《IB国际文凭语言B指导大纲》建议话题进行对比,最后为《YCT标准教程》系列教材话题选择和国际中文教育工作者的课堂教学话题选择提供建议。According to statistics from the national education authorities, nearly 150 million people overseas are currently learning Chinese, with the number of learners in many countries increasing by 50% or even higher. The age of non-native Chinese learners is also gradually decreasing. These students either come from or will continue to engage with the IB curriculum system. Therefore, whether their Chinese learning is conducted in isolation or can align with the IB curriculum—and how to achieve such alignment—is the key focus of this study. The IB Diploma Programme divides its curriculum into six groups: Language and Literature (Language A), Language Acquisition (Language B), Individuals and Societies, among others. Language B, also referred to as the second language has teaching philosophies and learning objectives that provide valuable insights for the development of international Chinese education for young learners. Topics serve as the foundation for language communication. Thus, the compilation of texts, vocabulary, and grammar items in teaching materials must revolve around topics and be structured accordingly. The author will use the topic classification in the YCT Test Syllabus and Exam Guide as a reference to categorize and analyze the topics and their frequency of occurrence in the YCT Standard Course series of textbooks. Subsequently, these will be compared with the suggested topics in the IB Diploma Programme Language B Guide. Finally, recommendations will be provided for topic selection in the YCT Standard Course series and for classroom instruction by international Chinese educators.展开更多
The aim of this paper is to simulate and study the early moments of the reactive ballistics of a large caliber projectile fired from a gun,combining 0D and 2D axisymmetric Computational Fluid Dynamics(CFD)approaches.F...The aim of this paper is to simulate and study the early moments of the reactive ballistics of a large caliber projectile fired from a gun,combining 0D and 2D axisymmetric Computational Fluid Dynamics(CFD)approaches.First,the methodology is introduced with the development of an interior ballistics(IB)lumped parameter code(LPC),integrating an original image processing method for calculating the specific regression of propellant grains that compose the gun propellant.The ONERA CFD code CEDRE,equipped with a Dynamic Mesh Technique(DMT),is then used in conjunction with the developed LPC to build a dedicated methodology to calculate IB.First results obtained on the AGARD gun and 40 mm gun test cases are in a good agreement with the existing literature.CEDRE is also used to calculate inter-mediate ballistics(first milliseconds of free flight of the projectile)with a multispecies and reactive approach either starting from the gun muzzle plane or directly following IB.In the latter case,an inverse problem involving a Latin hypercube sampling method is used to find a gun propellant configuration that allows the projectile to reach a given exit velocity and base pressure when IB ends.The methodology developed in this work makes it possible to study the flame front of the intermediate flash and depressurization that occurs in a base bleed(BB)channel at the gun muzzle.Average pressure variations in the BB channel during depressurization are in good agreement with literature.展开更多
文摘目的探讨3例儿童糖原累积病Ib型(glycogen storage disease type Ib,GSDIb)的临床特征和基因变异特点,分析恩格列净治疗GSDIb患儿的临床效果。方法回顾性分析2020年6月至2023年5月在郑州大学附属儿童医院就诊的3例GSDIb型患儿临床资料及基因检测结果。3例患儿均接受恩格列净治疗及饮食干预,随访1~3年,并回顾相关文献。结果3例患儿均为男性,就诊年龄7个月~1岁,主要临床表现为肝脏增大及智力运动发育落后,代谢异常均表现为肝功能异常、空腹低血糖、血清乳酸及三酰甘油升高,均伴有中性粒细胞减少,3例患儿均检出SLC37A4基因复合杂合变异。应用恩格列净治疗年龄分别为1岁2个月、1岁、1岁10个月,随访时间分别为12、17、36个月,均未发生低血糖、泌尿系统感染、肝肾功能异常等不良反应,临床症状得到改善。本研究发现SLC37A4基因1个新变异位点c.1287_1290del,拓展了SLC37A4基因变异谱。结论GSDIb型患儿的临床表现多样,存在中性粒细胞减少及功能缺陷,确诊依赖于基因检测,恩格列净可增加GSDIb患儿中性粒细胞数目,改善患儿临床症状,尽早应用可避免炎症性肠病的发生。
文摘根据国家教育部门的统计,目前海外将近有1.5亿人在学习中文,许多国家学习中文的人数以百分之五十甚至更高幅度增产,非母语中文学习者的年龄也在逐渐下移。这些留学生或来自或未来将继续IB课程体系,那么其中文学习部分是孤立进行还是可向IB课程靠拢以及如何靠拢正是本文研究的要义。IB国际文凭项目将课程分为语言和文学(语言A)、语言习得(语言B)、个人与社会等六个组别。语言B又称作“第二语言”,其教学理念及培养目标都对国际青少年中文教育的发展具有借鉴意义。话题是语言交际的依托,所以教材中有关课文、词汇和语法项目的编写都离不开话题,都需要依托话题来展开。笔者将以《YCT考试大纲与应考指南》里的话题分类为参考依据,对《YCT标准教程》系列教材的话题内容、出现次数进行归类,其后将其与《IB国际文凭语言B指导大纲》建议话题进行对比,最后为《YCT标准教程》系列教材话题选择和国际中文教育工作者的课堂教学话题选择提供建议。According to statistics from the national education authorities, nearly 150 million people overseas are currently learning Chinese, with the number of learners in many countries increasing by 50% or even higher. The age of non-native Chinese learners is also gradually decreasing. These students either come from or will continue to engage with the IB curriculum system. Therefore, whether their Chinese learning is conducted in isolation or can align with the IB curriculum—and how to achieve such alignment—is the key focus of this study. The IB Diploma Programme divides its curriculum into six groups: Language and Literature (Language A), Language Acquisition (Language B), Individuals and Societies, among others. Language B, also referred to as the second language has teaching philosophies and learning objectives that provide valuable insights for the development of international Chinese education for young learners. Topics serve as the foundation for language communication. Thus, the compilation of texts, vocabulary, and grammar items in teaching materials must revolve around topics and be structured accordingly. The author will use the topic classification in the YCT Test Syllabus and Exam Guide as a reference to categorize and analyze the topics and their frequency of occurrence in the YCT Standard Course series of textbooks. Subsequently, these will be compared with the suggested topics in the IB Diploma Programme Language B Guide. Finally, recommendations will be provided for topic selection in the YCT Standard Course series and for classroom instruction by international Chinese educators.
基金the French Defense Innovation Agency (AID)the French Procurement Agency for Armament (DGA)ONERA's scientific direction for funding and supporting the present work
文摘The aim of this paper is to simulate and study the early moments of the reactive ballistics of a large caliber projectile fired from a gun,combining 0D and 2D axisymmetric Computational Fluid Dynamics(CFD)approaches.First,the methodology is introduced with the development of an interior ballistics(IB)lumped parameter code(LPC),integrating an original image processing method for calculating the specific regression of propellant grains that compose the gun propellant.The ONERA CFD code CEDRE,equipped with a Dynamic Mesh Technique(DMT),is then used in conjunction with the developed LPC to build a dedicated methodology to calculate IB.First results obtained on the AGARD gun and 40 mm gun test cases are in a good agreement with the existing literature.CEDRE is also used to calculate inter-mediate ballistics(first milliseconds of free flight of the projectile)with a multispecies and reactive approach either starting from the gun muzzle plane or directly following IB.In the latter case,an inverse problem involving a Latin hypercube sampling method is used to find a gun propellant configuration that allows the projectile to reach a given exit velocity and base pressure when IB ends.The methodology developed in this work makes it possible to study the flame front of the intermediate flash and depressurization that occurs in a base bleed(BB)channel at the gun muzzle.Average pressure variations in the BB channel during depressurization are in good agreement with literature.
