针对国内船舶管路系统详细设计与生产设计分离导致的设计数据源不统一、人工信息转换效率低、模型与原理校对困难和设计更改难一致等问题,提出基于三维体验平台(3D Experience Platform,3DEXP)逻辑到物理(Logical to Physical,L2P)关联...针对国内船舶管路系统详细设计与生产设计分离导致的设计数据源不统一、人工信息转换效率低、模型与原理校对困难和设计更改难一致等问题,提出基于三维体验平台(3D Experience Platform,3DEXP)逻辑到物理(Logical to Physical,L2P)关联设计技术的解决方案。L2P关联设计技术紧密联系逻辑对象和物理对象,是对船舶管路系统设计模式的创新。分析现有设计模式下各种问题产生的原因,研究L2P关联设计的设计流程、设计准备、原理图设绘和三维建模等内容,打通原理图与三维建模关联设计的技术路线,创建原理图与三维模型之间的关联,实现快速三维建模、智能模型校对、设计更改一致和单一数据源。应用测试结果表明,采用管路系统L2P关联设计技术能有效提高管路系统设计的效率和质量。展开更多
BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients wi...BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients with DCM contributes to variable disease severity and complicates overall prognosis,which can be very poor.AIM To identify pathogenic genes in DCM through pedigree analysis.METHODS Our research team identified a patient with DCM in the clinic.Through invest-igation,we found that the family of this patient has a typical DCM pedigree.High-throughput sequencing technology,next-generation sequencing,was used to sequence the whole exomes of seven samples in the pedigree.RESULTS A novel and potentially pathogenic gene mutation-ANK2p.F3067L-was discovered.The mutation was completely consistent with the clinical information for this DCM pedigree.Sanger sequencing was used to further verify the locus of the mutation in pedigree samples.These results were consistent with those of high-throughput sequencing.CONCLUSIONS ANK2p.F3067L is considered a novel and potentially pathogenic gene mutation in DCM.展开更多
二维驱动三维(Logical to Physical,L2P)是三维体验(3D Experience)平台中由二维原理图设计过渡到三维模型设计的重要方法,采用该方法可提高三维设计中船舶设备布置的准确性和效率。结合工程实际,从资源配置和驱动过程2个方面系统阐述L2...二维驱动三维(Logical to Physical,L2P)是三维体验(3D Experience)平台中由二维原理图设计过渡到三维模型设计的重要方法,采用该方法可提高三维设计中船舶设备布置的准确性和效率。结合工程实际,从资源配置和驱动过程2个方面系统阐述L2P的驱动机制,运用该核心技术实现对设备的位置驱动,并通过EKL编程实现基于船舶坐标系的设备精准布置。通过研究,为三维设计中轮机、电气和舾装等设备的布置提供具体的技术路线。展开更多
文摘针对国内船舶管路系统详细设计与生产设计分离导致的设计数据源不统一、人工信息转换效率低、模型与原理校对困难和设计更改难一致等问题,提出基于三维体验平台(3D Experience Platform,3DEXP)逻辑到物理(Logical to Physical,L2P)关联设计技术的解决方案。L2P关联设计技术紧密联系逻辑对象和物理对象,是对船舶管路系统设计模式的创新。分析现有设计模式下各种问题产生的原因,研究L2P关联设计的设计流程、设计准备、原理图设绘和三维建模等内容,打通原理图与三维建模关联设计的技术路线,创建原理图与三维模型之间的关联,实现快速三维建模、智能模型校对、设计更改一致和单一数据源。应用测试结果表明,采用管路系统L2P关联设计技术能有效提高管路系统设计的效率和质量。
基金Supported by the Jilin Provincial Healthcare Talent Special Program,No.2019SCZT08.
文摘BACKGROUND Dilated cardiomyopathy(DCM)is a genetically heterogeneous cardiac disorder characterized by left ventricular dilation and contractile dysfunction.The substantial genetic heterogeneity evident in patients with DCM contributes to variable disease severity and complicates overall prognosis,which can be very poor.AIM To identify pathogenic genes in DCM through pedigree analysis.METHODS Our research team identified a patient with DCM in the clinic.Through invest-igation,we found that the family of this patient has a typical DCM pedigree.High-throughput sequencing technology,next-generation sequencing,was used to sequence the whole exomes of seven samples in the pedigree.RESULTS A novel and potentially pathogenic gene mutation-ANK2p.F3067L-was discovered.The mutation was completely consistent with the clinical information for this DCM pedigree.Sanger sequencing was used to further verify the locus of the mutation in pedigree samples.These results were consistent with those of high-throughput sequencing.CONCLUSIONS ANK2p.F3067L is considered a novel and potentially pathogenic gene mutation in DCM.
文摘二维驱动三维(Logical to Physical,L2P)是三维体验(3D Experience)平台中由二维原理图设计过渡到三维模型设计的重要方法,采用该方法可提高三维设计中船舶设备布置的准确性和效率。结合工程实际,从资源配置和驱动过程2个方面系统阐述L2P的驱动机制,运用该核心技术实现对设备的位置驱动,并通过EKL编程实现基于船舶坐标系的设备精准布置。通过研究,为三维设计中轮机、电气和舾装等设备的布置提供具体的技术路线。
