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目的分析45例临床确诊的法布雷病（AFD）患者的临床特征，同时应用超声心动图二维斑点追踪技术（2D-STE）评估法布雷病患者应变图像，以期探索法布雷患者心脏早期受累在超声心动图（UCG）上的表现。方法本研究为观察性研究。共纳入确诊为AFD的患者45例，根据UCG测量左室壁有无肥厚，分为左室壁肥厚组（17例，室间隔或左室后壁厚度≥12 mm）和无左室壁肥厚组（28例）。收集患者基线资料，并对所有患者进行常规UCG检查及二维斑点追踪超声心动图分析。应用TomTec工作站分析超声心动图图像，比较两组的临床基线资料、UCG常规参数及心肌应变特征。结果本研究共纳入45例AFD患者，年龄（32.33±16.11）岁，其中男性27例（60.00%）。左室壁肥厚组17例（37.78%），无左室壁肥厚组28例（62.22%）。45例患者的左室射血分数（LVEF）均正常（＞50%）。与无左室壁肥厚组相比，左室壁肥厚组患者的靶器官受累比例明显增加。与无左室壁肥厚组相比，左室壁肥厚组患者的E/A，平均E/E'明显升高（P＜0.05）；左室壁肥厚组的整体、心肌层、心内膜以及各节段的纵向应变（LS），周向应变（CS）及径向应变（RS）的差异均无统计学意义（P 值均＞0.05）。AFD患者心肌层的整体和各节段LS、CS的绝对值均低于心内膜的对应值（P 值均＜0.05）。AFD患者的中间段的LS、RS绝对值较基底段和心尖段高（P 值均＜0.05）。结论早期收缩功能障碍与左室壁厚度无明显关系。2D-STE心肌应变可以早期监测AFD室壁肌层受累较心内膜严重，而中间段受累较心尖段和基底段轻。

为提高钢桁框体系的抗震可恢复性，提出了自复位钢桁框体系的概念，研究了其在脉冲地震下的强度折减系数。以等效单自由度体系表征具有三折线滞回特征的钢桁框体系，选取182条脉冲型地震动对等效单自由度体系进行非线性动力分析，得到包含6 022万个强度折减系数（R）的数据库，揭示了不同滞回参数和脉冲型地震动对强度折减系数的影响规律。此外，借助BP神经网络拟合强度折减系数在地震下的对数正态分布模型参数，并建立了基于概率的钢桁框体系强度折减系数模型。结果表明提出的模型具有较高的准确性。

为实现房屋的折叠与展开并满足房屋的受力需求，在传统剪支铰折叠结构基础上，提出一种新型杆式折叠结构。首先，对新型杆式折叠结构进行设计和优化，采用螺旋理论对其进行自由度分析。同时，建立高度为2.2m的ABAQUS有限元模型，对其施加平面内竖向荷载，获得不同铰接约束次数、钢材强度等级、销轴孔径、槽钢壁厚、杆件宽度条件下折叠结构的初始刚度、极限承载力和破坏模式。最后，对折叠结构进行稳定性分析，提出设计建议。结果表明：当折叠结构展开后高宽比为1/2时，可以达到最大折叠率；折叠结构展开过程中机构自由度为1，当完全展开，施加一定铰接约束后结构自由度为负；次要受力杆件、销轴孔径对结构初始刚度和极限承载力影响不大；提高钢材强度等级、槽钢立柱壁厚、主要受力构件宽度可提高结构初始刚度和极限承载力，但是其值过高时会引起结构失稳破坏；为避免结构失稳，可通过促进塑性铰形成和将特定杆件改为箱形截面两种途径来实现。

综合形状记忆合金（shape memory alloy，SMA）材料和摩擦机制的优点，提出了一种具有耗能时序的变摩擦自复位阻尼器（self-centering variable friction damper with energy-dissipation sequences，SVFDES）。首先说明了SVFDES的构造设计及变形模式，开展了SMA棒的循环拉伸试验研究以确定材料的力学参数。之后利用经试验验证的数值模拟方法建立了SVFDES的精细化有限元模型，通过参数分析识别并研究了影响该阻尼器滞回性能的关键参数。最后通过对阻尼器的隔离体进行受力分析，推导了阻尼器滞回行为的理论计算公式。结果表明：（1） SVFDES在往复荷载作用下兼具自复位能力和耗能能力，并且呈现出“变摩擦”、“变刚度”的特征；（2） 阻尼器的滞回行为对摩擦副的几何构形、摩擦面的摩擦系数较为敏感，而与SMA元件的预紧力弱相关；（3） 理论计算公式和数值模拟的结果较为吻合。

目的 分析首次青年缺血性卒中患者出院、发病90d预后不良的相关危险因素。 方法 回顾性纳入18～45岁、发病72 h内的首次缺血性卒中患者，收集患者人口学信息、血管危险因素、实验室检查结果、入院和出院时NIHSS，以出院时和发病90 d mRS≥2分为预后不良，分别比较预后不良组和预后良好组危险因素的差异和病因分型特点，并采用多因素logistic回归分析出院和90 d预后不良的独立危险因素。 结果 研究共纳入691例首次青年缺血性卒中患者，出院预后不良234例（33.9%），90 d预后不良154例（22.3%）。出院时预后的独立危险因素包括入院时高NIHSS（OR 2.76，95%CI 2.07～3.68，P

为实现装配外露式钢柱脚节点的全预制装配化,提出了预埋波纹管后灌入高强浇筑料来锚固高强锚栓的连接方式。为研究该类高强锚栓的黏结锚固性能,以高强锚栓直径d、锚固长度la、波纹管直径D及其与高强锚栓直径d的比值D/d为试验参数,进行了直接预埋和预埋波纹管浆锚2类锚固方式的27组,共计54个连接件的拉拔试验。试验结果表明:预埋波纹管浆锚高强锚栓相较于直接预埋锚固高强锚栓具有更稳定的黏结锚固性能;高强锚栓的黏结应力主要集中分布在la=10d以内;波纹管孔径比D/d可取为2.0～3.1;当高强锚栓直径为16 mm时,锚固长度取7d;当高强锚栓直径为20 mm和24 mm时,锚固长度取10d即可,规范中规定的锚固长度偏保守。

目的 汉化10条目医院版卒中失语抑郁问卷（stroke aphasic depression questionnaire-hospital version， SADQ-H10），评价该量表在卒中后失语伴抑郁患者中的可接受度、信度和效度，为评价卒中后失语患 者的抑郁提供标准化测量工具。 方法 对SADQ-H10原版进行汉化，选取2016年1月-2018年2月在首都医科大学附属北京天坛医院神 经内科就诊的120例卒中后失语患者作为研究对象，进行SADQ-H10测评，同时完成汉密尔顿抑郁量表 （Hamilton depression scale，HAMD）及Beck抑郁自评量表（Beck depression inventory，BDI）测评为效标，并随 机选取其中42例患者于1～2周后复测SADQ-H10。根据测评结果分析SADQ-H10的可接受度、信度、效度。 结果 SAD Q-H10的完成率为91.7%（110例），测评时间最短3 m in，最长10 m in。重测信度0.821 （P 0.05。 结论 SADQ-H10汉化版简洁易懂，使用方便，量表可接受度较好。量表具有良好的信度和效度，对 中国卒中后失语患者抑郁的诊断具有良好的诊断效能。 Abstract: Objective To adapt stroke aphasic depression questionnaire-hospital version (SADQ-H10) into Chinese version, and test its repeatability, reliability and validity. Methods 120 patients with poststroke aphasia from Beijing Tiantan hospital from January 2016 to February 2018 were included in this study. All the patients were assessed with SADQ-H10, Hamilton depression scale (HAMD), and Beck depression inventory (BDI). A randomly selected sample of 42 patients were re-evaluated with SADQ-H10 within 1-2 week(s) after the first evaluation. Based on the evaluation results, the repeatability, reliability and validity of the Chinese version of SADQ-H10 were tested. Results 110 patients completed SADQ-H10 (91.7%). The time of completing the SADQ-H10 ranged from 3 to 10 minutes. The test-retest reliability was 0.821 (P

目的 研究Sneddon综合征（Sneddon syndrome，SS）的临床表现、诊断及治疗，以提高临床对该综合征 的认识，提高诊疗水平。 方法 收集2014年8月1日-2017年6月1日于首都医科大学附属北京天坛医院神经病学中心住院治疗的 Sneddon 综合征患者2例，回顾性分析患者临床资料，总结其临床表现、实验室检查、神经影像学检 查、认知功能评价、神经病理学特征及治疗。 结果 2例患者均出现皮肤广泛网状青斑合并多次缺血性卒中事件，均未发现特异性的血生化及脑 脊液异常。头颅磁共振成像（magnetic resonance imaging，MRI）均可见颅内多发的异常信号，颅内外血 管影像学评价均未发现明显的大血管狭窄。认知功能评价可见患者认知功能均存在下降。行皮肤病 理组织活检，病理结果均符合SS表现。治疗上均采取了抗血小板聚集治疗，对于认知功能下降，予以 改善认知疗法。 结论 S neddon 综合征的诊断要综合考虑患者的临床表现、实验室检查、影像学检查结果以及皮肤 病理组织活检结果。患者的特征性皮肤表现及多次卒中事件有助于该病的诊断，必要时可行皮肤活 检以明确诊断。 Abstract: Objective To investigate the clinical features, diagnosis and treatment in patients with Sneddon syndrome (SS), aiming to improve the clinical doctors’ understanding of this syndrome and enhance the ability of diagnosis and treatment of SS. Methods A total of 2 cases who were admitted to neurological disease center of Beijing Tiantan hospital, Capital Medical University during Aug.1st, 2014 to Aug.1st, 2017 and diagnosed with SS were reviewed. Their clinical descriptions, laboratory examinations, neuroimaging, cognitive function evaluation, pathological characteristic and treatment were analyzed. Results Clinical manifestations: 2 cases both suffered from generalized livedo racemosa of the skin and multiple recurrent ischemic stroke events. No specific blood biochemical and cerebral spinal fluid (CSF) abnormalities were found in both patients. Intracranial multiple abnormal signals was seen in magnetic resonance imaging (MRI). Intracranial and extracranial large vascular imaging evaluations were not significantly abnormal. Both patients had cognitive dysfunction. Skin biopsy was performed in 2 patients and the pathologic results were consistent with SS feature. Both cases were given antiplatelet and improving cognitive function therapy. Conclusion The diagnosis of SS should be made considering the patient's clinical manifestations, laboratory tests, imaging findings and the skin biopsy. Characteristic manifestations of the skin and multiple recurrent ischemic stroke events are helpful to the diagnosis of the disease. When necessary, skin biopsy can be performed in order for make clear diagnosis.

：【目的】研究Caspase-1特异性抑制剂AC-YVAD-CMK对大鼠颈动脉球囊损伤后血管内膜增生的影响及可能机制。【方法】33只雄性成年SD大鼠，随机均分为假手术组、球囊损伤组、球囊损伤+AC-YVAD-CMK组。采用球囊损伤大鼠颈动脉的方法建立血管内膜增生动物模型，14 天后留取球囊损伤段血管，15 个血管片段制作冰冻切片，苏木精-伊红（HE）染色法测量内膜与中膜（I/M）面积比值；18根血管片段采用Western blot 检测NOD样受体蛋白3（NLRP3）炎症小体、 cleaved-Caspase-1、白介素（IL）-1β和IL-18 的表达。【结果】HE 染色发现，AC-YVAD-CMK 显著抑制了内膜增生的程度（0.78 ± 0.13 vs 1.52 ± 0.14，P = 0.000）。Western blot结果提示球囊损伤+ AC-YVAD-CMK组中NLRP3蛋白表达较球囊损伤组显著降低（P = 0.009）；cleaved-Caspase-1蛋白在三组中的表达趋势与NLRP3蛋白表达一致（P = 0.000）。且球囊损伤+ AC-YVAD-CMK 组大鼠的促炎性细胞因子IL-1β和IL-18 水平较球囊损伤组显著降低（P = 0.000）。【结论】AC-YVADCMK 可以抑制大鼠颈动脉球囊损伤后内膜增生，其机制可能是其阻断Caspase-1蛋白活化，从而抑制促炎因子IL-1β和IL- 18的释放而产生一定的保护作用。

摘要：【目的】探讨超声早孕期筛查帆状脐带胎盘入口的可行性。【方法】收集大连市妇女儿童医疗中心行早孕筛 查、中孕系统筛查且在我院分娩的孕妇2 796 例，胎儿2998 个。早孕期（11~13+6 周）筛查脐带胎盘入口位置，将检查结果 分成三组，Ⅰ组为：明确显示脐带胎盘入口位于与胎膜上，沿着胎膜走行一段距离后，再进入胎盘，该组诊断为帆状胎盘。 Ⅱ组为：脐带胎盘入口位于胎盘边缘，无法区分插入点位于胎盘实质及胎膜上。Ⅲ组为：组明确显示脐带胎盘入口处为胎 盘实质，该组诊断为正常胎盘。将每组结果与中孕期（22~26周）筛查结果及产后临床检查结果比较。【结果】①早、中孕期 超声检查对脐带附着位置的显示率：早孕期一次检查显示率99.23%（2 975/2 998）。其中单胎显示率99.77%（2 590/2 596）， 双胎显示率95.96%（380/396），三胎显示率83.33%（5/6）。中孕期一次检查显示率86.99%（2 608/2 998）。其中单胎显示：90.99% （2 362/2 596）双胎显示率61.61%（244/396）三胎显示率33.33%（2/6）。早孕与中孕检查脐带胎盘入口一次检查显示率的比 较有统计学差异P < 0.05（P = 0.00）。其中单胎、双胎、三胎早孕期一次显示率与中孕期比较均有统计学差异P < 0.05（均 P = 0.00）。②早孕期、中孕、出生后检查结果：早孕期检查结果：Ⅰ组28 例、Ⅱ组42 例、Ⅲ组2 928 例；中孕期：帆状胎盘： 37 例球拍状胎盘：30例正常胎盘：2931例；出生后检查：帆状胎盘：36例球拍状胎盘：30例正常胎盘：2 932例。③早孕期各 组检查结果与中孕及出生后结果的比较：早孕期Ⅰ组中28例中孕及出生后均诊断为帆状胎盘，早孕期Ⅱ组42例中孕期诊 断为帆状胎盘7例，球拍状胎盘22例，正常胎盘13例，出生后诊断为帆状胎盘6例，球拍状胎盘23例，正常胎盘13例；早孕 期Ⅲ组2 928例，中孕期诊断为帆状胎盘2例，球拍状胎盘8例，正常胎盘2 918例，出生后诊断为帆状胎盘2例，球拍状胎盘 7例，正常胎盘2 919例。早孕期诊断的准确率为98.29%。【结论】早孕期应用高分辨率血流成像（HD-FLOW）血流模式可以 对部分帆状胎盘做出诊断。

光子晶体光纤（PCF）以其独特的光学特性和灵活设计的特点,而成为近年来研究的热点。随着各种新型PCF的问世以及研究的深入,使其应用领域不断扩大。文章对PCF在全光网中的各项应用进行了分析,重点对PCF在光纤光栅和光纤激光器、放大器、光开关、波长变换器方面的研究进展进行了介绍。

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[Objective]To understand the structural characteristics of the plankton community and its key environmental impact factors in the rivers of Muli mining area, and to provide data support and reference basis for the management and protection of water resources in the mining area. [Method]Collection and analysis of water and plankton in the rivers (Duosuo and Mori) that surround the opencast mine in the town of Muli. [Result]6 Phylum (21 species) of phytoplankton and 2 Phylum (15 species) of zooplankton were collected in the Dosuo River and 6 Phylum (19 species) of phytoplankton and 2 Phylum (9 species) of zooplankton were collected in the Mori River. In both rivers diatoms, green algae and protozoa were the dominant species, and the density and biomass of plankton were quantitatively higher upstream and lower downstream. The Shannon-Wiener, Margalef and Pielou indices for phytoplankton and zooplankton in Duosuo River are 1. 64 and 0. 58, 0. 86 and 0. 33, 0. 69 and 0. 42, respectively;the Shannon-Wiener, Margalef and Pielou indices for phytoplankton and zooplankton in Mori River are 1. 86 and 0. 21, 0. 87 and 0. 24, 0. 79 and 0. 36. Water temperature, pH, and nitrogen nutrients were identified as the main environmental factors affecting the structure of the planktonic communities in the Dosso and Mojito Rivers by redundancy analysis (RDA) and correlation analysis. [Conclusion]The ecological protection of water in the Muli mining area should focus on nutrient pollution and strengthen the control of non-point source pollution caused by overgrazing. [ABSTRACT FROM AUTHOR]

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The improvement of computing power provides a support for finite element simulation analysis, which can solve more complex structures and nonlinear problems more efficiently. In this study, based on the finite element simulation analysis technology, the complex mechanical properties of Mn-Cu alloy are simulated. Prony series constitutive model and parallel rheological constitutive model are respectively adopted to simulate the stress relaxation test of Mn-Cu alloy under different deformation conditions. The stress relaxation experimental data are extracted and converted into the corresponding model parameters and the finite element simulation calculation is carried out. The simulation model parameters which can accurately describe the nonlinear mechanical properties of Mn-Cu alloy are obtained. By comparing the simulation and experimental results, it is concluded that the parallel rheological framework can more accurately characterize the stress relaxation behavior of Mn-Cu alloy. Under different initial strain conditions, the relative error of the simulation results and the test results under corresponding working conditions is less than 1%. Compared with Prony series constitutive model, parallel rheological framework is more suitable for the simulation of Mn-Cu alloy under complex working conditions. [ABSTRACT FROM AUTHOR]

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