简介:Lanthanidehasattractedmuchattentioninthefieldofopticalcommunicationsinrecentyears.SomepropertyanalysesonopticalwaveguideofNd-dopedcrystalNdxY1-xAl3(BO3)4andNd:MgO:LiNbO3aremadeinthispaper,followedbyintroductionofthemethodsofexperimentationandtheoreticalcalculationfortheplanaropticalwaveguides.Therefractiveindexprofilesoftheopticalwaveguidesareanalyzed.Theaboveworkoffersusefulinformationforstudyonnewtypematerialsforopticalcommunications.
简介:ThetransportpropertiesintheLa2/3(Ca1-xSrx)1/3MnO3(x=0,1/3,2/3)filmspreparedusingtheRFmagnetronsputteringmethodwereinvestigated.TheeffectoftheCa,Srdouble-dopingattheApositionintheLa2/3A1/3MnO3onthestructureofthetargetsandtransportofthefilmshasbeenstudied.Withtheincreaseofx,thestructuresofthetargetstransformfromtherhombohedralphasetothecubicphase;themetal-insulatorphasetransitiontemperature(Tp)ofthefilmsincreases;andthecorrespondingpeakresistivitydecreases.Allthephenomenacanbequalitativelyexplainedbythelatticeeffect.
简介:目的探讨3-羟基-3-甲基戊二酸尿症患儿的主要临床表现、生化特点及3-羟基-3-甲基戊二酰裂解酶(HMGCL)基因突变检测结果。方法收集2014年12月7日于北京大学第一医院儿科就诊的1例3-羟基-3-甲基戊二酸尿症患儿的临床病历资料为研究对象。采用回顾性研究方法分析该患儿的入院查体及辅助检查结果、诊疗情况;对患儿及父母进行HMGCL基因检测,并于患儿出院后6个月,对其进行随访。本研究遵循的程序符合北京大学第一医院人体试验委员会制定的伦理学标准,得到该委员会批准,对患儿及其父母进行基因检测均获得受试对象及受试对象监护人的知情同意。结果患儿年龄为1岁1个月,男性。因"不明原因腹泻、呕吐,反复呕血,发热2d,昏迷3h"收入本院。入院查体及辅助检查结果示:白细胞计数升高、肝功能损害、电解质紊乱、低血糖、凝血功能异常、低蛋白、血氨水平高、代谢性酸中毒;头颅MRI检查结果示:双侧侧脑室旁及双侧额颞顶皮层下异常信号。确诊为3-羟基-3-甲基戊二酸尿症。经限制蛋白质摄入,静脉滴注葡萄糖和左旋肉碱治疗后症状缓解。该患儿HMGCL基因c.509G〉T(鸟嘌呤〉胸腺嘧啶)发生点突变,c.348+1G〉C(鸟嘌呤〉胞嘧啶)发生剪切位点突变,导致外显子拼接错误,其中c.509G〉T是来自母亲的杂合突变,c.348+1G〉C是患儿新出现纯合突变,c.348+1G〉C剪切位点突变,导致第170位的半胱氨酸剪切突变为苯丙氨酸。出院后6个月后随访结果示:患儿年龄为1岁7个月,智力运动发育稍落后,但无明显倒退,无抽搐发作,全身情况良好,各项生化指标及尿3-羟基-3-甲基戊二酸水平降低。结论3-羟基-3-甲基戊二酸尿症系少见的常染色体隐性遗传疾病,通过HMGCL基因检测及HMGCL基因分析不仅有助于诊断该病,对于指导家系遗传咨询及产前诊断亦至关重要。
简介:海马在国内终于有了一款彻底自主研发的车型,一上来就瞄准了高手如林的紧凑级运动型家轿市场,而它面对着即将到来的拼杀也显得信心十足。
简介:when I told students that they could design and draw a picture about the future world on a piece of paper,I can launch a project to solve the problem. (I can do something to change the present situation.)☆The methods used in problem analysis Are the methods used to analyze the problem acceptable,Week 3Teaching notes Students’ handout Diary-keeping ☆The methods used in project implementationIn what way has the project been implemented