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Progress in treatment and newborn screening for Duchenne muscular dystrophy and spinal muscular atrophy

World Journal of Pediatrics 2019年第3期15卷 219-225页

作者： Qing Ke Zheng-Yan Zhao Jerry R.Mendell mei baker Veronica Wiley Jennifer M.Kwon Lindsay N.Alfano Anne M.Connolly Catherine Jay Hanna Polari Emma Ciafaloni Ming Qi Robert C.Griggs Michele A.Gatheridge Department of Neurology The First Affiliated Hospital College of MedicineZhejiang UniversityHangzhouZhejiangChina Children’S Hospital Zhejiang University School of Medicine HangzhouChina Department of Pediatrics and Neurology Nationwide Children’S HospitalColumbusOHUSA Department of Pediatrics University of Wisconsin School of Medicine and Public HealthMadisonWIUSA Disciplines of Genetic Medicine and Pediatric and Child Health University of SydneySydneyAustralia Department of Neurology University of Wisconsin School of Medicine and Public HealthMadisonWIUSA Department of Pediatrics Nationwide Children’S HospitalColumbusOHUSA Department of Neurology Washington University School of MedicineSt.LouisMOUSA Department of Neurology University of Rochester School of Medicine and DentistryRochesterNY’USA PerkinElmer Inc TurkuFinland Department of Clinical Laboratory Zhejiang University School of MedicineHangzhouChina Department of Neurology University of Rochester School of Medicine and Dentistry60 1 Elmwood AveBox 673RochesterNY 14642USA

Background Advances in treatment for Duchenne muscular dystrophy (DMD) and spinal muscular atrophy (SMA) hold promise for children with these *** genetic diagnosis,early in the disease process,will allow these treatments to be most *** screening (NBS) for SMA has been recommended in the United States,and a pilot DMD NBS program is underway in Hangzhou,*** sources A PubMed search,limited to the past 5 years,was conducted to identify:(1) therapeutic advancements for DMD/SMA approved by the United States Food and Drug Administration or the European Medicine Agency and (2) The status of NBS for DMD/*** We review the current state of approved treatments for DMD/*** present recommendations regarding the future of NBS for these diseases,with a focus on the outcomes and challenges of SMA NBS in New York,USA,and the DMD NBS pilot program in Hangzhou,*** Approved treatments for DMD and SMA may change the natural history of these ***-term studies of these treatments are *** avoid the known diagnostic delay associated with these disorders and provide optimal effectiveness of these treatments,early identification of patients through NBS will be *** comprehensive follow-up plans for positively identified patients will need to be in place for NBS programs to be successful.

关键词： Duchene muscular dystrophy Spinal muscular atrophy Newborn screening Neuromuscular disorders Neurology

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Evaluation and optimization of secondary water supply system renovation

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Journal of Zhejiang University-Science A(Applied Physics & Engineering) 2007年第9期8卷 1488-1494页

作者： TIAN Yi-mei SI Yan-jie LI Hong WU Mi-fang School of Environmental Science and Engineering Tianjin University Tianjin 300072 China Michael baker Corporation Alexandria VA 22304 USA

Due to pollution in second water supply system (SWSS),nine renovation alternative plans were proposed and com-prehensive evaluations of different plan based on Analytical Hierarchy Process (AHP) were presented in this paper. Comparisons of advantages and disadvantages among the plans of SWSS renovations provided solid foundation for selecting the most appro-priate plan for engineering projects. In addition,a mathematical model of the optimal combination of renovation plans has been set up and software Lingo was used to solve the model. As a case study,the paper analyzed 15 buildings in Tianjin City. After simulation of the SWSS renovation system,an optimal scheme was obtained,the result of which indicates that 10 out of those 15 buildings need be renovated in priority. The renovation plans selected for each building are the ones ranked higher in the com-prehensive analysis. The analysis revealed that the optimal scheme,compared with two other randomly calculated ones,increased the percentage of service population by 19.6% and 13.6% respectively,which significantly improved social and economical benefits.

关键词： Secondary water supply system (SWSS) Renovation plan Analytical Hierarchy Process (AHP) Hierarchical model Optimization

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Temporal changes in circulating P-selectin,plasminogen activator inhibitor-1,magnesium,and creatine kinase after percutaneous coronary intervention

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Journal of Zhejiang University-Science B(Biomedicine & Biotechnology) 2010年第8期11卷 575-582页

作者： Shu-qin YING mei-xiang XIANG Lu FANG Jian-an WANG Department of Cardiology Hangzhou 310009 China) Cardiovascular Key Lab of Zhejiang Province the Second Affiliated Hospital School of Medicine Zhejiang University Hangzhou 310009 China baker IDI Heart and Diabetes Institute Melbourne Victoria 3004 Australia

Objective： This study aims to determine the mechanisms underlying restenosis and ischemia-reperfusion injury of the myocardium after percutaneous coronary intervention （PCI）. Methods： The present study examined serial changes （5 min, 30 min, 2 h, 6 h, and 24 h after PCI） in circulating P-selectin, plasminogen activator inhibitor-1 （PAl-l）, magnesium （Mg）, and creatine kinase-myocardial band fraction （CK-MB） levels, which may be associated with restenosis and myocardial injury in patients undergoing PCI. The occurrence rates of major adverse cardiovascular events were collected over a six-month follow-up. Results： PCI induced an early elevation of P-selectin, which cor- related positively with the inflation pressure used in the PCI procedure. PCI also caused a significant and sustained decrease in serum Mg in PCI patients, without an effect on PAI-I. An increase in CK-MB was observed in PCI patients, although values were within normal reference range. In addition, elevated P-selectin and decreased Mg measured shortly after the coronary angioplasty procedure were associated with recurrent treatment and heart failure, respec- tively. Conclusions： Our study demonstrates that PCI induces temporal changes of P-selectin, Mg, and CK-MB, which may be involved in restenosis and ischemia-reperfusion injury. These findings highlight the need for using antiplatelet therapy and Mg to reduce the risks associated with PCI.

关键词： Percutaneous coronary intervention Restenosis Ischemia-reperfusion injury P-selectin Magnesium

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STUDY ON THE POLLUTION OF URBAN SCENIC WATER BODY BY MUNICIPAL DRAINAGE IN FLOOD SEASON AND ITS CONTROL PLANNING

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Journal of Hydrodynamics 2008年第6期20卷 797-803页

作者： TIAN Yi-mei ZHOU Ying Li Hong LIU Zhi-qiang PENG Xiu-hua School of Environmental Science and Technology Tianjin University Tianjin 300072 China Veolia Water Systems （Beijing） Co. Ltd. Beijing 100027 China Michael baker Corporation Alexandria Virginia 22304 USA

In this article, based on river quality simulation and system optimization, a water quality model was established for scenic river after rainfall discharge in flood season, with the target of making water pollutants meet the standard in priority and saving expenditure on pollution control. With the principle of reducing sewage from combined sewage pumping station and heavily polluted initial rainwater, a mathematical multiobjective planning model was constructed for rain sewage pollution control in flood season, and one scenic river in a northern city was taken for simulation example. The results show that： the optimization result meets the requirements of planning, among which, sewage reduction from the combined pumping station accounts for 17.38% in the total reduction of rain sewage, and the reduction in the heavily polluted rain water accounts for 77.24% in the total reduction of rainwater pumping station. The planning scheme can provide theoretical basis for pollution control of scenic river in flood season, and for rational reconstruction and layout of outfalls along two banks of the river.

关键词： scenic river flood season water quality simulation water pollutant controlling multiobjective planning

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经济政策不确定性会影响货币政策有效性吗——基于信贷渠道的视角

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当代财经 2017年第6期 18-27页

作者：段梅江西财经大学金融学院江西南昌330013

基于baker等编制的中国经济政策不确定性指数,实证检验了经济政策不确定性、货币政策与企业信贷配置之间的关系,进而从微观层面考察了经济政策不确定性对我国货币政策有效性的影响。研究发现:(1)经济政策不确定性上升会抑制企业的新增借... 详细信息

基于baker等编制的中国经济政策不确定性指数,实证检验了经济政策不确定性、货币政策与企业信贷配置之间的关系,进而从微观层面考察了经济政策不确定性对我国货币政策有效性的影响。研究发现:(1)经济政策不确定性上升会抑制企业的新增借款,尤其是导致新增短期借款减少;(2)较高的经济政策不确定性导致货币政策对企业信贷融资的调节作用下降,降低了我国货币政策的有效性。此外,经济政策不确定性对国企和非国企的影响也存在显著差异。

关键词：经济政策不确定性货币政策信贷配置所有权性质

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球形装药动态爆炸冲击波超压场计算模型

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爆炸与冲击 2017年第5期37卷 951-956页

作者：聂源蒋建伟李梅北京理工大学爆炸科学与技术国家重点实验室北京100081

为获得球形装药动态爆炸冲击波超压场计算模型,对静态爆炸冲击波超压baker计算公式加入修正因子进行修正,并建立了构造包含装药运动速度、对比距离和方位角的修正因子函数的方法。为获得修正因子的函数表达式,采用高精度显式欧拉流体动... 详细信息

为获得球形装药动态爆炸冲击波超压场计算模型,对静态爆炸冲击波超压baker计算公式加入修正因子进行修正,并建立了构造包含装药运动速度、对比距离和方位角的修正因子函数的方法。为获得修正因子的函数表达式,采用高精度显式欧拉流体动力学软件SPEED针对具有典型运动速度的球形装药空中爆炸过程进行了数值模拟,得到了沿装药不同对比距离和方位角处的动态爆炸冲击波超压峰值。在对数值模拟结果处理的基础上,经过数据拟合获得了动态爆炸冲击波超压场计算模型。校验结果表明,该模型能较准确描述动态爆炸冲击波超压分布,具有普适性。

关键词：冲击波超压动态爆炸装药运动速度超压计算模型

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Whole Grain Gluten-Free High Protein Vegetable Snacks of Buckwheat Peanut Meal and Kale

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Food and Nutrition Sciences 2018年第4期9卷 335-345页

作者： Talwinder S. Kahlon Roberto J. Avena-Bustillos mei-Chen M. Chiu Western Regional Research Center USDA-ARS Albany CA USA

Whole grain gluten-free high protein vegetable snacks were evaluated. The snacks were Buckwheat-Peanut Meal-Kale (BPK), BPK-Garlic, BPK-Onion and BPK-Ginger. Peanut meal was utilized to increase the protein content of these snacks as well as to add value to this agricultural byproduct. Snack dough was prepared using water nearly 1:1 as other as is ingredients. About 20 g of snack dough was placed on preheated KrumKake Express 839 baker and cooked for 2 minutes. Sixty-nine in-house volunteers judged Color/Appearance and Texture/Mouth feel to be similar for the snacks tested. Taste/Flavor for BPK, BPK-Garlic and BPK-Onion snacks was similar and significantly higher (p ≤ 0.05) than for BPK-Ginger snacks. Odor/Aroma for BPK-Garlic snacks was significantly higher than for BPK-Ginger snacks. Acceptance for BPK and BPK-Garlic snacks was significantly higher than for BPK-Ginger snacks. Ex-pansion 3.6 - 4.2, porosity 0.72 - 0.75 and water activity 0.35 - 0.38 suggests that the snacks tested were light, crispy and have good anti-microbial stability. Acceptance was BPK and BPK-Garlic 94%, BPK-Onion 86% and BPK-Ginger 78%. These snacks contained only 3 - 4 ingredients and could be made in any house hold kitchen and/or in commercial production. Acceptance of 78% - 94% is very desirable. These whole grain gluten-free high protein vegetable healthy nutritious tasty snacks offer choice for all consumers, including individual’s sensitive to gluten.

关键词： Sensory Evaluation Buckwheat Kale Peanut Meal Gluten-Free Water Activity

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黄酮醇类化合物的合成研究进展

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有机化学 2015年第1期35卷 70-84页

作者：梅青刚袁伟成王淳中国科学院成都有机化学研究所成都610041 中国科学院成都生物研究所成都610041

黄酮醇即3-羟基黄酮,是一类独特的黄酮化合物,广泛存在于植物界中,具有许多重要的生物活性和药理作用,其化学合成研究一直备受关注.综述了黄酮醇骨架的4种主要合成方法:Auwers法,查尔酮氧化关环即Algar-Flynn-Oyamada(AFO)反应,baker-Ve... 详细信息

黄酮醇即3-羟基黄酮,是一类独特的黄酮化合物,广泛存在于植物界中,具有许多重要的生物活性和药理作用,其化学合成研究一直备受关注.综述了黄酮醇骨架的4种主要合成方法:Auwers法,查尔酮氧化关环即Algar-Flynn-Oyamada(AFO)反应,baker-Venkataraman法,黄酮DMDO(3,3-二甲基双环氧乙烷)氧化法;并对多羟基黄酮醇的区域选择性甲基化、异戊烯基化和糖苷化等衍生化方法进行了概述.

关键词：黄酮醇合成 AFO反应衍生化区域选择性

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河南省鳞毛蕨科耳蕨属一新记录种——亮叶耳蕨

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西北植物学报 2012年第2期32卷 416-418页

作者：李家美夏至河南农业大学郑州450002

报道了河南省鳞毛蕨科耳蕨属一新记录种——亮叶耳蕨(Polystichum lanceolatum baker)。对该种进行了特征描述,并编制了河南耳蕨属植物检索表。该种近似正宇耳蕨(*** Ching),但羽片上侧耳状凸起不明显,边缘有7～8个具短刺头的牙状齿,孢... 详细信息

报道了河南省鳞毛蕨科耳蕨属一新记录种——亮叶耳蕨(Polystichum lanceolatum baker)。对该种进行了特征描述,并编制了河南耳蕨属植物检索表。该种近似正宇耳蕨(*** Ching),但羽片上侧耳状凸起不明显,边缘有7～8个具短刺头的牙状齿,孢子囊群在主脉上侧最多3个,下侧不育或偶有1个,囊群盖圆盾形,全缘等特征而不同。凭证标本存放于河南农业大学植物标本馆(HEAC)。

关键词：亮叶耳蕨鳞毛蕨科新记录河南

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基于量维和形维的风水联合出力波动量化评估

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华中科技大学学报（自然科学版） 2016年第5期44卷 103-107,132页

作者：王现勋梅亚东肖伟华丛翔宇武汉大学水资源与水电工程科学国家重点实验室湖北武汉430072 武汉大学水资源安全保障湖北省协同创新中心湖北武汉430072 中国水利水电科学研究院北京100038 中国电建集团昆明勘测设计研究院有限公司云南昆明650051

为了使出力的波动程度能直接反映水电平抑风电的效果,从量变和形变的角度提出了基于量维和形维的波动量化评估方法.以随机和实测过程为测验对象,并加以滑动平均、叠加平均、缩放等处理,与现行常用的波动量化评估方法——RBF方法进行了... 详细信息

为了使出力的波动程度能直接反映水电平抑风电的效果,从量变和形变的角度提出了基于量维和形维的波动量化评估方法.以随机和实测过程为测验对象,并加以滑动平均、叠加平均、缩放等处理,与现行常用的波动量化评估方法——RBF方法进行了对比分析,多方位验证了所提方法的有效性和优越性.将其用于风电水电联合运行实例后,在多种的资源和装机条件下的计算结果均符合客观实际,结果表明了该方法在风电水电联合运行中量化评估出力波动程度的可行性.

关键词：风电水电联合调度波动指标量维形维 RBF方法

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