The study of sedimentary mélanges holds pivotal importance in understanding orogenic processes and unveiling geodynamic mechanisms.In this study,we present findings on zircon U-Pb isotopes and whole-rock elementa...The study of sedimentary mélanges holds pivotal importance in understanding orogenic processes and unveiling geodynamic mechanisms.In this study,we present findings on zircon U-Pb isotopes and whole-rock elemental data concerning the recently uncovered Zongzhuo Formation sedimentary mélanges within the Dingri area.Field observations reveal the predominant composition of the Zongzhuo Formation,characterized by a matrix of sandstone-mudstone mixed with sand-conglomerates within native blocks exhibiting soft sediment deformation.Moreover,exotic blocks originating from littoral-neritic seas display evidence of landslide deformation.Our study identifies the depositional environment of the Zongzhuo Formation in Dingri as a slope turbidite fan,with its provenance traced back to the passive continental margin.Notably,this contrasts with the Zongzhuo Formation found in the Jiangzi-Langkazi area.Based on existing data,we conclude that the Zongzhuo Formation in the Dingri area was influenced by the Dingri-Gamba fault and emerged within a fault basin of the passive continental margin due to Neo-Tethys oceanic subduction during the Late Cretaceous period.Its provenance can be attributed to the littoral-neritic sea of the northern Tethys Himalaya region.This study holds significant implications for understanding the tectonic evolution of Tethys Himalaya and for reevaluating the activity of the Dingri-Gamba fault,as it controls the active deposition of the Zongzhuo Formation.展开更多
目的探讨Cornelia de Lange综合征(CdLS)的临床表型及基因型特点。方法回顾分析1例确诊CdLS患儿的临床资料,并总结分析国内已报道病例的情况。结果女性患儿,1岁2月龄,有特殊外貌,智力及运动发育落后,合并四肢畸形及听力异常。基因检测...目的探讨Cornelia de Lange综合征(CdLS)的临床表型及基因型特点。方法回顾分析1例确诊CdLS患儿的临床资料,并总结分析国内已报道病例的情况。结果女性患儿,1岁2月龄,有特殊外貌,智力及运动发育落后,合并四肢畸形及听力异常。基因检测发现患儿HDAC8基因c.675C>A(p.Y 225X)存在新发杂合无义变异,根据ACMG指南预测为致病性变异,确诊CdLS。通过对万方、维普、中国知网及PubMed数据库搜索,发现国内报道CdLS病例46例。其中26例行基因检查,20例(76.9%)存在NIPBL基因变异,3例(11.5%)HDAC8基因变异,1例(3.8%)SCM1A基因变异,2例未发现与临床吻合的致病性基因变异,表型各异。结论CdLS患儿存在特殊外貌、生长发育迟缓、多器官受累、听力障碍,多数可通过典型临床表型诊断,基因检测有助于非典型患者的早期诊断。展开更多
As the Interlanguage is a lingual continuum between the native language and the target language, it is strongly influenced by both of them during its formation. On the other hand, language is inseparable from culture....As the Interlanguage is a lingual continuum between the native language and the target language, it is strongly influenced by both of them during its formation. On the other hand, language is inseparable from culture. Without the proper knowledge and understanding of the target language culture, one can never rally master it. So, to reduce the negative language transfer, the L2 learners should strengthen the target language culture study throughout their whole lingual development.展开更多
提议贡山三尖杉的新名称Cephalotaxus talonensis Cheng et Feng ex S.G.Lu et X.D.Lang替代不合法的旧名称Cephalotaxus lanceolata K.M.Feng in Cheng et al.(晚出同名)。对比贡山三尖杉和三尖杉Cephalotaxus fortunei Hooker的模式...提议贡山三尖杉的新名称Cephalotaxus talonensis Cheng et Feng ex S.G.Lu et X.D.Lang替代不合法的旧名称Cephalotaxus lanceolata K.M.Feng in Cheng et al.(晚出同名)。对比贡山三尖杉和三尖杉Cephalotaxus fortunei Hooker的模式标本和自然生长状态下的叶片形态,支持将贡山三尖杉处理为种的等级,不赞同Silba(1990)将贡山三尖杉降为三尖杉的变种[Cephalotaxus fortunei Hooker var.lanceolata(Feng)Silba]。文中提供4幅参照图片。展开更多
提出了一种新型的多层实现的Lange耦合器,它的耦合系数为3 d B。相较于传统加工工艺而言,多层技术克服了传统Lange耦合器由于线窄又紧靠在一起而加工困难的问题。该耦合器采用50Ω阻抗线进行匹配和进行终端测量。在中心频率处实现2.8 d ...提出了一种新型的多层实现的Lange耦合器,它的耦合系数为3 d B。相较于传统加工工艺而言,多层技术克服了传统Lange耦合器由于线窄又紧靠在一起而加工困难的问题。该耦合器采用50Ω阻抗线进行匹配和进行终端测量。在中心频率处实现2.8 d B耦合,相对带宽达到80%,实现超宽带。展开更多
Auld Lang Syne是苏格兰民族诗人罗伯特·彭斯的经典之作。二百多年以来,这首诗被译为多种语言,在全世界广为传唱,经久不衰。本文回顾Auld Lang Syne在中国的译介,并选取该诗的三个汉译本,从时代背景、译者主体性和语言使用三个方...Auld Lang Syne是苏格兰民族诗人罗伯特·彭斯的经典之作。二百多年以来,这首诗被译为多种语言,在全世界广为传唱,经久不衰。本文回顾Auld Lang Syne在中国的译介,并选取该诗的三个汉译本,从时代背景、译者主体性和语言使用三个方面进行比较研究,旨在说明经典作家作品的研究和重译在寻求文化共通性和跨文化交际方面的意义。展开更多
BACKGROUND Cornelia de Lange syndrome(CdLS)is a congenital multisystemic genetic disorder.The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent yea...BACKGROUND Cornelia de Lange syndrome(CdLS)is a congenital multisystemic genetic disorder.The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent years.However,they still more frequently undergo cardiac surgery.There are some challenges for clinicians when faced with CdLS patients.We present the perioperative management of a child with CdLS undergoing open-heart surgery.CASE SUMMARY Severe pulmonic and subpulmonic valvular stenosis,enlargement of the right side of the heart,mild tricuspid regurgitation,atrial septal defect,and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis,developmental delay,and malnutrition.Attempted balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve,therefore it was decided to perform an open surgical repair.Following a successful and uncomplicated intraoperative course,the patient was extubated on postoperative day 5,and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10,respectively.Moderate laryngomalacia and suboptimal vocal cord movements were diagnosed,and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day 32.The patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional problems.CONCLUSION This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.展开更多
Cornelia de Lange综合征(CdLS)是一种以严重神经发育障碍为主要表现的遗传综合征,临床表现为智力障碍、典型的面部特征、宫内和生后生长发育迟缓及多器官系统畸形等,发病率约1/10 000~1/30 000。2017年国际CdLS共识小组成立,并于2018年...Cornelia de Lange综合征(CdLS)是一种以严重神经发育障碍为主要表现的遗传综合征,临床表现为智力障碍、典型的面部特征、宫内和生后生长发育迟缓及多器官系统畸形等,发病率约1/10 000~1/30 000。2017年国际CdLS共识小组成立,并于2018年7月发表了国际上首个关于CdLS的共识"Diagnosis and management of Cornelia de Lange syndrome:first international consensus statement"(《Cornelia de Lange综合征的诊断和管理:第一份国际共识声明》)。该共识通过改良德尔菲法制定,对CdLS患儿的诊断和管理提出了若干指导性意见。该文对共识进行解读,旨在帮助临床医生早期识别、诊断、规范随访及管理CdLS患者。展开更多
基金supported by the Geological Survey Project of the China Geological Survey(Grant No.DD20211547)the Basic Survey Project of the Command Center of Natural Resources Comprehensive Survey(Grant No.ZD20220508)。
文摘The study of sedimentary mélanges holds pivotal importance in understanding orogenic processes and unveiling geodynamic mechanisms.In this study,we present findings on zircon U-Pb isotopes and whole-rock elemental data concerning the recently uncovered Zongzhuo Formation sedimentary mélanges within the Dingri area.Field observations reveal the predominant composition of the Zongzhuo Formation,characterized by a matrix of sandstone-mudstone mixed with sand-conglomerates within native blocks exhibiting soft sediment deformation.Moreover,exotic blocks originating from littoral-neritic seas display evidence of landslide deformation.Our study identifies the depositional environment of the Zongzhuo Formation in Dingri as a slope turbidite fan,with its provenance traced back to the passive continental margin.Notably,this contrasts with the Zongzhuo Formation found in the Jiangzi-Langkazi area.Based on existing data,we conclude that the Zongzhuo Formation in the Dingri area was influenced by the Dingri-Gamba fault and emerged within a fault basin of the passive continental margin due to Neo-Tethys oceanic subduction during the Late Cretaceous period.Its provenance can be attributed to the littoral-neritic sea of the northern Tethys Himalaya region.This study holds significant implications for understanding the tectonic evolution of Tethys Himalaya and for reevaluating the activity of the Dingri-Gamba fault,as it controls the active deposition of the Zongzhuo Formation.
文摘目的探讨Cornelia de Lange综合征(CdLS)的临床表型及基因型特点。方法回顾分析1例确诊CdLS患儿的临床资料,并总结分析国内已报道病例的情况。结果女性患儿,1岁2月龄,有特殊外貌,智力及运动发育落后,合并四肢畸形及听力异常。基因检测发现患儿HDAC8基因c.675C>A(p.Y 225X)存在新发杂合无义变异,根据ACMG指南预测为致病性变异,确诊CdLS。通过对万方、维普、中国知网及PubMed数据库搜索,发现国内报道CdLS病例46例。其中26例行基因检查,20例(76.9%)存在NIPBL基因变异,3例(11.5%)HDAC8基因变异,1例(3.8%)SCM1A基因变异,2例未发现与临床吻合的致病性基因变异,表型各异。结论CdLS患儿存在特殊外貌、生长发育迟缓、多器官受累、听力障碍,多数可通过典型临床表型诊断,基因检测有助于非典型患者的早期诊断。
文摘As the Interlanguage is a lingual continuum between the native language and the target language, it is strongly influenced by both of them during its formation. On the other hand, language is inseparable from culture. Without the proper knowledge and understanding of the target language culture, one can never rally master it. So, to reduce the negative language transfer, the L2 learners should strengthen the target language culture study throughout their whole lingual development.
基金supported by the National Forestry Public Welfare Foundation of China(Grant no.200804024)the National Natural Science Foundation of China(Grant nos.30770164&30970186)
文摘提议贡山三尖杉的新名称Cephalotaxus talonensis Cheng et Feng ex S.G.Lu et X.D.Lang替代不合法的旧名称Cephalotaxus lanceolata K.M.Feng in Cheng et al.(晚出同名)。对比贡山三尖杉和三尖杉Cephalotaxus fortunei Hooker的模式标本和自然生长状态下的叶片形态,支持将贡山三尖杉处理为种的等级,不赞同Silba(1990)将贡山三尖杉降为三尖杉的变种[Cephalotaxus fortunei Hooker var.lanceolata(Feng)Silba]。文中提供4幅参照图片。
文摘Auld Lang Syne是苏格兰民族诗人罗伯特·彭斯的经典之作。二百多年以来,这首诗被译为多种语言,在全世界广为传唱,经久不衰。本文回顾Auld Lang Syne在中国的译介,并选取该诗的三个汉译本,从时代背景、译者主体性和语言使用三个方面进行比较研究,旨在说明经典作家作品的研究和重译在寻求文化共通性和跨文化交际方面的意义。
文摘BACKGROUND Cornelia de Lange syndrome(CdLS)is a congenital multisystemic genetic disorder.The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent years.However,they still more frequently undergo cardiac surgery.There are some challenges for clinicians when faced with CdLS patients.We present the perioperative management of a child with CdLS undergoing open-heart surgery.CASE SUMMARY Severe pulmonic and subpulmonic valvular stenosis,enlargement of the right side of the heart,mild tricuspid regurgitation,atrial septal defect,and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis,developmental delay,and malnutrition.Attempted balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve,therefore it was decided to perform an open surgical repair.Following a successful and uncomplicated intraoperative course,the patient was extubated on postoperative day 5,and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10,respectively.Moderate laryngomalacia and suboptimal vocal cord movements were diagnosed,and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day 32.The patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional problems.CONCLUSION This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.
文摘Cornelia de Lange综合征(CdLS)是一种以严重神经发育障碍为主要表现的遗传综合征,临床表现为智力障碍、典型的面部特征、宫内和生后生长发育迟缓及多器官系统畸形等,发病率约1/10 000~1/30 000。2017年国际CdLS共识小组成立,并于2018年7月发表了国际上首个关于CdLS的共识"Diagnosis and management of Cornelia de Lange syndrome:first international consensus statement"(《Cornelia de Lange综合征的诊断和管理:第一份国际共识声明》)。该共识通过改良德尔菲法制定,对CdLS患儿的诊断和管理提出了若干指导性意见。该文对共识进行解读,旨在帮助临床医生早期识别、诊断、规范随访及管理CdLS患者。
