BACKGROUND Mucopolysaccharidosis type Ⅱ(MPS Ⅱ)is a chronic inherited disease with multiorgan involvement,a progressive course,and restricted life expectancy.AIM To evaluate the predictors of fatal outcomes in MPS Ⅱ...BACKGROUND Mucopolysaccharidosis type Ⅱ(MPS Ⅱ)is a chronic inherited disease with multiorgan involvement,a progressive course,and restricted life expectancy.AIM To evaluate the predictors of fatal outcomes in MPS Ⅱ patients.METHODS In the retrospective cohort study,the clinical,laboratory data and enzyme replacement therapy(ERT)(84.2%)of about 160 patients were extracted and analyzed from the Russian MPS Ⅱ registry,with death as a primary outcome.We compared patients who died(n=20;12.5%)with severe form(n=13;68.4%)and attenuated form(n=6,31.6%)to 140 alive patients.RESULTS Fatal outcomes occurred in 5%,35%,20%,and 40%of patients before 10,10-14,15-19,and≥20 years.The most common causes of death were cardiovascular(29.4%),respiratory failure(17.6%),including pneumonia(17.6%),and their associations(17.6%)and MPS Ⅱ progression(11.8%).Acute or chronic respiratory failure was in 53%.Died patients had higher birth weight,higher age of diagnosis,and start of ERT.Hydrocephalus,hydrocephalus bypass surgery,epilepsy,difficulty swallowing,and impaired movement after 12 years of age were significantly more common in the deceased patients.Cox regression analysis has revealed the following time-dependent covariates of the lethal outcome:1^(st)-year psychomotor development delay,delayed mental and speech development,hydrocephalus,swallow disorders,impossible walking at age>12 years,respiratory disorders,tracheostomy,neuronopathic form.CONCLUSION Increased birth weight,delayed diagnosis and the start of ERT,and development of neuronopathic form with impossible walking after 12 years were the main predictors of the fatal outcome.展开更多
Stimulated Raman scattering(SRS)under a new ignition path that combines the advantages of direct-drive(DD)and indirect-drive(ID)schemes is investigated experimentally at the Shenguang-100 kJ facility.The results show ...Stimulated Raman scattering(SRS)under a new ignition path that combines the advantages of direct-drive(DD)and indirect-drive(ID)schemes is investigated experimentally at the Shenguang-100 kJ facility.The results show that collective SRS in the plasma produced by ablating a polyimide film is detected for the ID beams,but is suppressed by adding a toe before the main pulse of the ID beams.The toe also strongly influences SRS of both the ID and DD beams excited in the plasma generated in the hohlraum.When a toe is used,the SRS spectra of the DD beams show that SRS tends to be excited in lower plasma density,which will result in a lower risk of super-hot electrons.Measurements of hot electrons support this conclusion.This research will help us produce a better pulse design for this new ignition path.展开更多
本文旨在探讨基于全寿命周期理念的实物ID全流程贯通管控模式。通过对全寿命周期管理理念的深入解析,结合物资管理的实际需求,本文提出了一种创新的物资管理模式。该模式通过为实物资产分配唯一的实物ID,并将其与项目编码、WBS编码、物...本文旨在探讨基于全寿命周期理念的实物ID全流程贯通管控模式。通过对全寿命周期管理理念的深入解析,结合物资管理的实际需求,本文提出了一种创新的物资管理模式。该模式通过为实物资产分配唯一的实物ID,并将其与项目编码、WBS编码、物料编码、设备编码、调度编码、资产编码和废旧物资编码等关键编码进行关联,实现了资产全寿命周期内的信息整合和共享。本文首先分析了全寿命周期管理存在的问题和挑战,然后详细阐述了实物ID全流程贯通模式的构建,包括编码规则制定、八码信息关联、信息管理系统构建、典型场景应用等方面。最后,通过典型场景验证了该模式的可行性和有效性,并展望了其未来的发展趋势。The purpose of this paper is to discuss the full-process control mode of physical ID based on the concept of full life cycle. Through the in-depth analysis of the whole life cycle management concept and the actual needs of material management, this paper proposes an innovative material management model. This model realizes the integration and sharing of information throughout the life cycle of assets by assigning unique physical IDs to physical assets and associating them with key codes such as project code, WBS code, material code, equipment code, scheduling code, asset code and waste material code. This paper first analyzes the existing problems and challenges of life cycle management, and then elaborates on the construction of the whole process of physical ID, including the formulation of coding rules, the association of eight-code information, the construction of information management system, and the application of typical scenarios. Finally, the feasibility and effectiveness of the model are verified by typical scenarios, and its future development trend is prospected.展开更多
文摘BACKGROUND Mucopolysaccharidosis type Ⅱ(MPS Ⅱ)is a chronic inherited disease with multiorgan involvement,a progressive course,and restricted life expectancy.AIM To evaluate the predictors of fatal outcomes in MPS Ⅱ patients.METHODS In the retrospective cohort study,the clinical,laboratory data and enzyme replacement therapy(ERT)(84.2%)of about 160 patients were extracted and analyzed from the Russian MPS Ⅱ registry,with death as a primary outcome.We compared patients who died(n=20;12.5%)with severe form(n=13;68.4%)and attenuated form(n=6,31.6%)to 140 alive patients.RESULTS Fatal outcomes occurred in 5%,35%,20%,and 40%of patients before 10,10-14,15-19,and≥20 years.The most common causes of death were cardiovascular(29.4%),respiratory failure(17.6%),including pneumonia(17.6%),and their associations(17.6%)and MPS Ⅱ progression(11.8%).Acute or chronic respiratory failure was in 53%.Died patients had higher birth weight,higher age of diagnosis,and start of ERT.Hydrocephalus,hydrocephalus bypass surgery,epilepsy,difficulty swallowing,and impaired movement after 12 years of age were significantly more common in the deceased patients.Cox regression analysis has revealed the following time-dependent covariates of the lethal outcome:1^(st)-year psychomotor development delay,delayed mental and speech development,hydrocephalus,swallow disorders,impossible walking at age>12 years,respiratory disorders,tracheostomy,neuronopathic form.CONCLUSION Increased birth weight,delayed diagnosis and the start of ERT,and development of neuronopathic form with impossible walking after 12 years were the main predictors of the fatal outcome.
基金supported by the National Natural Science Foundation of China(Grant Nos.12205274,12275251,12105270,12205272,12305262,and 12035002)the National Key Laboratory of Plasma Physics(Grant No.JCKYS2024212803)+2 种基金the Fund of the National Key Laboratory of Plasma Physics(Grant No.6142A04230103)the National Key R&D Program of China(Grant No.2023YFA1608400)the National Security Academic Fund(Grant No.U2430207).
文摘Stimulated Raman scattering(SRS)under a new ignition path that combines the advantages of direct-drive(DD)and indirect-drive(ID)schemes is investigated experimentally at the Shenguang-100 kJ facility.The results show that collective SRS in the plasma produced by ablating a polyimide film is detected for the ID beams,but is suppressed by adding a toe before the main pulse of the ID beams.The toe also strongly influences SRS of both the ID and DD beams excited in the plasma generated in the hohlraum.When a toe is used,the SRS spectra of the DD beams show that SRS tends to be excited in lower plasma density,which will result in a lower risk of super-hot electrons.Measurements of hot electrons support this conclusion.This research will help us produce a better pulse design for this new ignition path.
文摘本文旨在探讨基于全寿命周期理念的实物ID全流程贯通管控模式。通过对全寿命周期管理理念的深入解析,结合物资管理的实际需求,本文提出了一种创新的物资管理模式。该模式通过为实物资产分配唯一的实物ID,并将其与项目编码、WBS编码、物料编码、设备编码、调度编码、资产编码和废旧物资编码等关键编码进行关联,实现了资产全寿命周期内的信息整合和共享。本文首先分析了全寿命周期管理存在的问题和挑战,然后详细阐述了实物ID全流程贯通模式的构建,包括编码规则制定、八码信息关联、信息管理系统构建、典型场景应用等方面。最后,通过典型场景验证了该模式的可行性和有效性,并展望了其未来的发展趋势。The purpose of this paper is to discuss the full-process control mode of physical ID based on the concept of full life cycle. Through the in-depth analysis of the whole life cycle management concept and the actual needs of material management, this paper proposes an innovative material management model. This model realizes the integration and sharing of information throughout the life cycle of assets by assigning unique physical IDs to physical assets and associating them with key codes such as project code, WBS code, material code, equipment code, scheduling code, asset code and waste material code. This paper first analyzes the existing problems and challenges of life cycle management, and then elaborates on the construction of the whole process of physical ID, including the formulation of coding rules, the association of eight-code information, the construction of information management system, and the application of typical scenarios. Finally, the feasibility and effectiveness of the model are verified by typical scenarios, and its future development trend is prospected.
