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Results These two patients were confirmed to suffer from DMD. They were characterized by typical features of DMD including typical clinical manifestations, increased serum enzymes, EMG presenting myogenic impairment, HE staining presentation belonging to DMD, negative dystrophin in brother, and inconstantly positive on the sarcolemma of sister. Furthermore, no deletion or duplication was found in the 1-79 exons of dystrophin gene. The suffering brother and sister carried the same maternal X chromosome.

结果 兄妹二人符合DMD诊断,具有典型的DMD临床表现,肌酸激酶、肌酸激酶同工酶、乳酸脱氢酶、羟丁酸脱氢酶和谷草转氨酶的水平均显著高于正常值,肌电图呈肌源性损害,肌肉HE染色符合DMD,男患者的抗肌萎缩蛋白表达阴性,女患者的少量肌纤维仍可见不连续膜阳性,两患者抗肌萎缩蛋白基因的1~79号外显子未见缺失和重复突变,女患者与男患者携带相同的母源性X染色体。

The indene maleic anhydride copolymers were synthesized with naphtha oil at 70~85℃ in the presence of 1.0%~2.0% initiator.The yield was more than 90%.The chemical structure of the copolymer was confirmed by IR.

以轻煤焦油为原料,在温度为70~85℃,引发剂为单体总质量的1.0%~2.0%的聚合条件下,合成了茚-马来酸酐共聚物,得率在90%以上。

The Diels-Alder cycloadditions of [60] fullerene for preparation of optical limitingmaterials are discussed in chapter 3 and 6. Soluble cycloaddition derivatives of 〓aresynthesized by the reaction of 〓 with indene,1,1'-biindene andcyclopentadiene derivatives.〓Particular, the Diels-Alder cycloaddition of 〓 with1,1'-biindene under controlled conditions affords the stable monoadduct 〓〓of novel structure, which was confirmed by HPLC, FTIR, FD-MS,〓NMR,〓 NMR, HMQC and HMBC spectra, Its two bridgehead cage carbons resonate at70.91ppm, The 〓 NMR spectra showed 38 lines consistent with the 〓 symmetryassociated with [6,6] addition. The 〓 shows similar optical limitingproperties to that of 〓 in toluene for 8ns laser at wave length 532nm. Moreover, thehigh solubility of this adduct in polar solvents such as THF, acetone enables itsincorporation into sol-gel glass matrix. The thin films prepared via the sol-gel methodshow improved OL behaviour compared to those of 〓. The langmuir-Blodgett filmformation and uv-visible absorption of 〓 are also investigated. LBmonolayer of this compound is prepared on the air-water interface at 〓 mol/l. In chapter 4, three water-soluble derivatives of [60] fullerene: fullerol(1), theaddition product (2)of fullerol (1)with polyvinylpyrrolidone and fumaric acidderivative of 〓(3) are synthesized and characterized.

通过Diels-Alder环加成反应,用茚〓和1,1'-联茚〓及取代茂合成了一系列可溶性的〓环加成衍生物,发现可控制反应条件,使1,1'-联茚与〓反应,并高产率地得到具有新颖结构的单加成物,用HPLC、FTIR、FD-MS 及〓、HMQC、HMBC等多种波谱技术对其结构进行表征,测得它的两个〓杂化的桥头碳的化学位移为〓ppm,证明生成的衍生物为6∶6闭式环加成,〓NMR谱中共给出38个信号,表明〓联茚衍生物分子具有〓对称性;在波长532nm,脉冲宽度8ns的激光下,〓联茚衍生物的甲苯溶液的光限幅性能与〓的甲苯溶液相近,由于〓联茚单加成衍生物熱稳定性好,在四氢呋喃,丙酮等极性溶剂中溶解性好,能分别均匀地掺入溶胶-凝胶中,已发现它的溶胶(so1)光限幅性能优于纯〓的光限幅性能;使用Langmuir-Blodgett技术将〓联茚衍生物在空气和水的界面进行了LB单层膜和多层膜实验以及UV-Vis吸收谱研究,通过等温压缩曲线测试,证明浓度为〓〓时,〓联茚衍生物能够形成单层膜。

It was confirmed by TTC histochemistry, India ink solution perfused through the left ventricle of heart, BBB permeability examination and neurological status grading that this model was very well simulated the pathological process of cerebral ischemia-reperfusion.

经TTC染色、印度墨水灌流、血脑屏障损伤及神经症状的观察证实该模型能较好地模拟脑缺血再灌注的病理发展过程。

The preferred dyeing process was confirmed by analyzing and comparing the K/S value of dyed cotton yarn with indigoid dyes.

通过分析比较靛蓝染料染色后棉纱线的K/S值大小,确定较佳的牛仔布靛蓝染浸染色工艺。

The ineffectiveness of a labor contract shall be confirmed by the labor dispute arbitration committee or the people's court.

劳动合同的无效,由劳动争议仲裁委员会或者人民法院确认。

Have introduced its systematic structure and operation principle in the system of loading of the inertia, and the vibration question that should be paid attention to while designing a group of rotator inertial mass, describe a critical rotational speed of group of rotator inertial mass in detail, influence the factor of a critical rotational speed of group of rotator inertial mass, and rotate a critical rotational speed computing technology and rotation of group and a balance question of group of inertial mass of inertial mass , and confirmed a design of group of rotator inertia quality finally.

在惯量加载系统中介绍了其系统结构和工作原理,以及在设计旋转惯性质量盘组时应注意的振动问题,详细地论述了旋转惯性质量盘组的临界转速,影响旋转惯性质量盘组的临界转速的因素,以及旋转惯性质量盘组的临界转速的计算方法和旋转惯性质量盘组的平衡问题,并最后确定了旋转惯量质量盘组的设计。

A logical product structure model was constructed which can describe the infinite product variants,and support to solve product configuration under the mixed environment of Assemble-to-Order and Make-to-Order. In order to be identified and managed ,Logical product structure model was described with the knowledge representation method based on production rule.Finally the feasibility of the method was confirmed by simulation of a simplification automobile product family.

构建了一种逻辑产品结构模型,它能够描述无限种可能的产品变体,支持解决按订单制造与按订单装配相混合环境下的产品配置问题;并用基于产生式规则的知识表示方法描述了逻辑产品结构模型,以便于计算机识别及处理,最后通过一个简化汽车产品族的案例仿真来验证本方法的可行性。

Based on analyzing the test data ,three variety types of isothermal adsorption and desorption curves are confirmed . The first type is that the hole structure is provided with slit capillary bore the second is usually the type of upgrowthing one end nipped unparallel crevice the third belongs to the pores shaped " inkpot ".

在测试数据分析的基础上,确定了等温吸附和脱附曲线3种类型:第1类属孔结构是具有平行权构成的狭缝毛细孔型;第2类为发育一端尖灭的不平行的裂隙型;第3类属"墨水瓶"型孔。

On apparent layer, the competition performance, strength and potential of four kinds of paper products——newspaper, printing and writing paper, household and sanitary paper, wrapping and packaging paper and paperboard were calculated quantitatively; on connotative layer, the whole competitive capacity and inner issues were confirmed through ZhaoDongcheng"s "nine factors model.

在显性层面上,通过定量计算,明确新闻纸、书写印刷纸、生活用纸、包装纸及纸板等四种纸产品的竞争业绩、竞争实力及竞争潜力情况,并对竞争业绩进行国际比较;在隐性层面上,借助九要素模型,分析造纸产业整体竞争力状况及发展中的深层次问题。

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On the other hand, the more important thing is because the urban housing is a kind of heterogeneity products.

另一方面,更重要的是由于城市住房是一种异质性产品。

Climate histogram is the fall that collects place measure calm value, cent serves as cross axle for a few equal interval, the area that the frequency that the value appears according to place is accumulated and becomes will be determined inside each interval, discharge the graph that rise with post, also be called histogram.

气候直方图是将所收集的降水量测定值,分为几个相等的区间作为横轴,并将各区间内所测定值依所出现的次数累积而成的面积,用柱子排起来的图形,也叫做柱状图。

You rap, you know we are not so good at rapping, huh?

你唱吧,你也知道我们并不那么擅长说唱,对吧?