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ACVR1基因突变致进行性骨化性肌炎1例

中国当代儿科杂志 2024年第9期26卷 961-966页

作者：谢思琴丁小芳张兵史凤霞钟礼立黄寒湖南师范大学第一附属医院/湖南省人民医院儿科湖南长沙410005 湖南师范大学第一附属医院/湖南省人民医院放射科湖南长沙410005

男性患儿,2岁10个月,因发现颈胸部多发包块3月余就诊。患儿既往步态不稳,摔跤后受伤部位可见硬包块,可自行缓解。最近因外伤后发现后颈部有一包块,突出皮面,伴关节活动受限,逐渐发现左侧颈部、背部平肩胛下角处、肩胛窝、左侧腋中线处... 详细信息

男性患儿,2岁10个月,因发现颈胸部多发包块3月余就诊。患儿既往步态不稳,摔跤后受伤部位可见硬包块,可自行缓解。最近因外伤后发现后颈部有一包块,突出皮面,伴关节活动受限,逐渐发现左侧颈部、背部平肩胛下角处、肩胛窝、左侧腋中线处均有新发包块。2个月前磁共振成像检查显示双侧后颈部、背部肌肉弥漫性T1WI低信号、T2WI高信号;CT扫描显示为肌肉肿胀,部分肌肉内见片状低密度及多发结节样骨化高密度。基因检测结果提示ACVR1基因突变,最终该患儿诊断为进行性骨化性肌炎。该文对1例进行性骨化性肌炎患儿的病因、诊断及治疗进行归纳总结,为临床医生诊治该罕见疾病提供参考。

关键词：进行性骨化性肌炎 ACVR1基因儿童

Expert Consensus on the Treat-to-Target Strategy for Psoriasis With Biological Agents in China(Simplified Edition)

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International Journal of Dermatology and Venereology 2024年第2期7卷 61-69页

作者： Ai-Jun Chen Da-Xing Cai xia.g Chen Yong Cui Yan ding Heng Gu Xing-Hua Gao Chao Ji xia.-Jing Kang Ye-Hong Kuang Cheng-Zhi Lyu Min Li Wei Li xia.Li Yu-Zhen Li xia.-Yong Man Jian-Jun Qiao Juan Su Liang-Dan Sun Yu-Ling shi Juan Tao Jian-Xin xia.Rong xia. Bin Yang Wu Zhu Xue-Jun Zhang Committee on Psoriasis Dermatology and Venereology Branch of the Chinese Medical Association Department of Dermatology The First Affiliated Hospital of Chongqing Medical UniversityChongqing 400016China Department of Dermatology Qilu HospitalShandong UniversityJinanShandong 250100China Department of Dermatology Xiangya HospitalCentral South UniversityChangshaHunan 410008China Department of Dermatology China-Japan Friendship HospitalBeijing 100192China Department of Dermatology Hainan Provincial Hospital of Skin DiseaseHaikouHainan 570311China Department of Dermatology Hospital for Skin DiseasesChinese Academy of Medical SciencesNanjingJiangsu 210042China Department of Dermatology The First Hospital of China Medical UniversityShenyangLiaoning 110002China Department of Dermatology The First Affiliated Hospital of Fujian Medical UniversityFuzhouFujian 350004China Department of Dermatology People’s Hospital of Xinjiang Uygur Autonomous RegionUrumqiXinjiang Uyghur Autonomous Region 830001China Department of Dermatology Dalian Dermatosis HospitalDalianLiaoning 116021China Department of Dermatology Dushu Lake Hospital Affiliated to Soochow UniversitySuzhouJiangsu 215127China Department of Dermatology West China Hospital of Sichuan UniversityChengduSichuan 610044China Department of Dermatology Ruijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Department of Dermatology The Second Affiliated Hospital of Harbin Medical UniversityHarbinHeilongjiang 150086China Department of Dermatology The Second Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiang 310003China Department of Dermatology The First Affiliated Hospital of Zhejiang University School of MedicineHangzhouZhejiang 310003China Department of Dermatology The First Affiliated Hospital of Anhui Medical UniversityHefeiAnhui 230022China Department of Dermatology Shanghai Skin Disease HospitalTongji University School of MedicineShanghai 200093China Department of Dermatology The Union Hospital of Tongji Medical CollegeHuazhong University of Science and Te

Psoriasis is a chronic,recurrent,inflammatory systemic disease that is not only characterized by skin manifestations but may also be accompanied by various comorbidities,imposing a heavy burden on patients’physical and mental health and affecting their quality of *** the continuous approval of various biological agents for clinical use has provided more effective treatment options to patients with psoriasis,no consensus on the evaluation of comprehensive treatment goals has yet been *** treat-to-target(T2T)strategy requires consideration of multiple dimensions of treatment outcomes,the development of long-term management goals,and regular assessments of treatment conditions,which are often used in the management of chronic ***,based on the latest consensuses and guidelines,research data,and clinical experience as well as the combination of survey results and expert group discussions,the present consensus focuses on 4 dimensions of short-and long-term integrated management goals for biological agents:alleviating skin lesions,improving quality of life,screening and managing psoriasis comorbidities,and ensuring drug *** implementation methods,evaluation time,treatment monitoring,and program adjustments are also herein described to achieve comprehensive management of psoriasis to the maximum *** consensus provides a reference for clinical practice.

关键词： psoriasis biological agents treat-to-target comorbidity consensus

数字平台就业是雇佣劳动还是合作关系——基于调查数据的考证

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经济社会体制比较 2024年第1期 107-116页

作者：夏璋煦丁守海中国人民大学应用经济学院

数字平台用工属性的界定是推动平台经济规范、健康和持续发展在劳工领域的体现,也是加强灵活就业和新就业形态劳动者权益保障、推进高质量充分就业的必然要求。文章基于新制度经济学的企业理论,从控制权的视角解构平台用工属性,以劳动... 详细信息

数字平台用工属性的界定是推动平台经济规范、健康和持续发展在劳工领域的体现,也是加强灵活就业和新就业形态劳动者权益保障、推进高质量充分就业的必然要求。文章基于新制度经济学的企业理论,从控制权的视角解构平台用工属性,以劳动关系争议较大的网约车司机、外卖员和快递员为例,按照数字平台对从业者的控制过程、控制结果以及平台是否让渡控制权这一逻辑主线,并结合一项针对平台就业的专项调查数据考证发现,数字平台就业更具雇佣关系属性而非合作关系,但这一属性在不同工种间存在一些差别。对此,文章认为应从制度供给上规范平台的雇主责任,通过法律和监管双管齐下,加强对平台就业的规制,但也要根据不同平台职业的属性进行分类管理,不宜“一刀切”。

关键词：数字平台就业雇佣关系合作关系控制权

Liver transplantation for hepatitis B virus-related cirrhosis with acute-on-chronic liver failure and grade 3-4 hepatic encephalopathy: Survival and quality of life

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中华医学杂志(英文版) 2024年第17期137卷 2119-2121页

作者： shi Shaohua Zhang Minghui Chen Xuliang Wang Zhuoyi ding Songming Chen Zhitao Yang Yu Zheng Shusen Department of Hepatobiliary and Pancreatic Surgery Shulan (Hangzhou) Hospital Affiliated to Zhejiang Shuren University Shulan International Medical College Hangzhou Zhejiang China Department of General Surgery The Sixth People’s Hospital of Zhengzhou City Zhengzhou Henan China Department of Intensive Care Unit Shulan (Hangzhou) Hospital Affiliated to Zhejiang Shuren University Shulan International Medical College Hangzhou Zhejiang China

基于胃络理论探讨慢性胃炎“炎癌转化”的病机与防治

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北京中医药大学学报 2024年第8期47卷 1037-1042页

作者：贾元萍姚欣凝丁霞北京中医药大学东直门医院北京100700 北京中医药大学中医学院

慢性胃炎“炎癌转化”是多因素、多阶段、多机制的复杂过程,随着疾病进展,胃黏膜的腺管开口及微血管形态发生变化,病理表现为腺体数量减少、结构紊乱或细胞异型等。中医学认为,慢性胃炎病发在胃,与外邪犯胃或内生诸邪等相关,呈现由气及... 详细信息

慢性胃炎“炎癌转化”是多因素、多阶段、多机制的复杂过程,随着疾病进展,胃黏膜的腺管开口及微血管形态发生变化,病理表现为腺体数量减少、结构紊乱或细胞异型等。中医学认为,慢性胃炎病发在胃,与外邪犯胃或内生诸邪等相关,呈现由气及血、由经到络的渐进过程,符合“久病入络”理论。基于胃络理论探讨慢性胃炎“炎癌转化”的病机与防治,病机演变特点概括为:胃络受邪、气络失和,胃络瘀阻、浊毒内生,胃络亏虚、积生失荣。临床可采用祛邪调气活络、化瘀解毒通络、补虚消积荣络的治法,使胃络得通、胃腑得养。本文从胃络理论出发,结合现代科学研究,探讨慢性胃炎“炎癌转化”的胃络辨治及动态全程管理,以期为临床提供理论参考和实践指导。

关键词：慢性胃炎炎癌转化胃络

Dynamic intrauterine crosstalk promotes porcine embryo implantation during early pregnancy

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Science China(Life Sciences) 2024年第8期67卷 1676-1696页

作者： Xupeng Zang Shengchen Gu Wenjing Wang Junsong shi Jianyu Gan Qun Hu Chen Zhou Yue ding Yanjuan He Lei Jiang Ting Gu Zheng Xu Sixiu Huang Huaqiang Yang Fanming Meng Zicong Li Gengyuan Cai Linjun Hong Zhenfang Wu State Key Laboratory of Swine and Poultry Breeding Industry National Engineering Research Center for Breeding Swine IndustryCollege of Animal ScienceSouth China Agricultural UniversityGuangzhou 510642China Guangdong Provincial Key Laboratory of Agro-Animal Genomics and Molecular Breeding South China Agricultural UniversityGuangzhou 510642China Yunfu Subcenter of Guangdong Laboratory for Lingnan Modern Agriculture Yunfu 527300China Guangdong Key Laboratory of Animal Breeding and Nutrition Institute of Animal ScienceGuangdong Academy of Agricultural SciencesGuangzhou 510640China Key Laboratory of South China Modern Biological Seed Industry Ministry of Agriculture and Rural AffairsGuangzhou 510520China

Dynamic crosstalk between the embryo and mother is crucial during ***,we comprehensively profile the single-cell transcriptome of pig peri-implantation embryos and corresponding maternal endometrium,identifying 4 different lineages in embryos and 13 cell types in the ***-specific gene expression characterizes 4 distinct trophectoderm subpopulations,showing development from undifferentiated trophectoderm to polar and mural *** expression of genes in different types of endometrial cells illustrates their molecular response to embryos during ***,we developed a novel tool,ExtraCellTalk,generating an overall dynamic map of maternal-foetal crosstalk using uterine luminal proteins as *** cross-species comparisons,we identified a conserved RBP4/STRA6 pathway in which embryonic-derived RBP4 could target the STRA6 receptor on stromal cells to regulate the interaction with other endometrial *** results provide insight into the maternal-foetal crosstalk during embryo implantation and represent a valuable resource for further studies to improve embryo implantation.

关键词： endometrial remodeling ExtraCellTalk implantation maternal-foetal crosstalk trophoblast

Two-dimensional Sb net generated nontrivial topological states in SmAgSb_(2) probed by quantum oscillations

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Chinese Physics B 2024年第7期33卷 51-58页

作者：袁健石贤彪杜红李田郗传英王霞夏威王保田钟瑞丹郭艳峰 School of Physical Science and Technology ShanghaiTech UniversityShanghai 201210China Institute of High Energy Physics Chinese Academy of SciencesBeijing 100049China Tsung-Dao Lee Institute Shanghai Jiao Tong UniversityShanghai 200240China Xingzhi College Zhejiang Normal UniversityJinhua 321100China Anhui Province Key Laboratory of Condensed Matter Physics at Extreme Conditions High Magnetic Field Laboratory of the Chinese Academy of SciencesHefei 230031China ShanghaiTech Laboratory for Topological Physics ShanghaiTech UniversityShanghai 201210China

The REAgSb_(2)(RE = rare earth and Y) family has drawn considerable research interest because the two-dimensional Sb net in their crystal structures hosts topological fermions and hence rich topological properties. We report herein the magnetization and magnetotransport measurements of SmAgSb_(2) single crystal, which unveil very large magnetoresistance and high carrier mobility up to 6.2 × 10^(3)% and 5.58 × 10^(3)cm^(2)·V^(-1)·s^(-1), respectively. The analysis of both Shubnikov–de Haas and de Haas–van Alphen quantum oscillations indicates nontrivial Berry phases in the paramagnetic state while trivial Berry curvature in the antiferromagnetic state, indicating a topological phase transition induced by the antiferromagnetic order. It is also supported by the first-principles calculations. The results not only provide a new interesting topological material but also offer valuable insights into the correlation between magnetism and nontrivial topological states.

关键词： large magnetoresistance quantum oscillations topological phase transition

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失效锂离子电池石墨负极回收利用研究进展

工程科学学报 2024年第5期46卷 949-962页

作者：丁云集史志胜张深根北京科技大学新材料技术研究院金属材料循环利用研究中心北京100083 北京科技大学顺德创新学院佛山528399 中国石化催化剂有限公司工程技术研究院北京101111

新能源汽车产业发展是实现我国“双碳”战略的重要举措.石墨因其高导电率、高容量和高稳定性等优点,成为当前主流的负极材料,其需求量和报废量增长迅速.废石墨负极因含多种金属、黏结剂、电解液等,具有污染性和资源性双重特点,其高效清... 详细信息

新能源汽车产业发展是实现我国“双碳”战略的重要举措.石墨因其高导电率、高容量和高稳定性等优点,成为当前主流的负极材料,其需求量和报废量增长迅速.废石墨负极因含多种金属、黏结剂、电解液等,具有污染性和资源性双重特点,其高效清洁回收利用成为人们研究的热点与重点问题.首先介绍了全球石墨矿产资源分布及其消费结构,表明我国石墨资源较为丰富(约占全球15.7%),但产量与消费量全球第一,分别达到65.4%和86.6%,且电池负极消费比重日益增长.为提高石墨负极利用水平,系统综述了石墨负极回收利用研究进展,阐述了石墨负极的再生方法,包括物理法、湿法浸出、火法及其他方法.为进一步提高再生石墨负极的电化学性能,改性技术(如元素掺杂、碳包覆、复合等方法)也受到人们的广泛关注.此外,还概括了石墨负极合成的其他新型功能材料,如石墨烯及氧化石墨烯、电容器、吸附剂和催化剂等,为石墨负极高值利用提供了新的选择.最后,总结了负极石墨材料回收利用的技术瓶颈和面临的挑战,为其绿色高效循环利用提供了研究思路和发展方向.

关键词：石墨负极失效锂离子电池再生回收再利用

Organization of microtubule plus-end dynamics by phase separation in mitosis

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Journal of Molecular Cell Biology 2024年第2期16卷 24-35页

作者： Fengrui Yang Mingrui ding xia.yu Song Fang Chen Tongtong Yang Chunyue Wang Chengcheng Hu Qing Hu Yihan Yao shihao Du Phil YYao Peng xia.Gregory Adams Jr Chuanhai Fu Shengqi xia.g Dan Liu Zhikai Wang Kai Yuan Xing Liu MOE Key Laboratory for Membraneless Organelles&Cellular Dynamics Hefei National Research Center for Cross-disciplinary SciencesCenter for Advanced Interdisciplinary Science and Biomedicine of IHMDivision of Life Sciences and MedicineUniversity of Science&Technology of ChinaHefei 230027China Anhui Key Laboratory for Cellular Dynamics and Chemical Biology University of Science&Technology of ChinaHefei 230027China Keck Center for Organoids Plasticity Morehouse School of MedicineAtlantaGA 30310USA Hunan Key Laboratory of Molecular Precision Medicine Central South UniversityChangsha 410083China

In eukaryotes,microtubule polymers are essential for cellular plasticity and fate ***-binding(EB)proteins serve as scaffolds for orchestrating microtubule polymer dynamics and are essential for cellular dynamics and chromosome segregation in ***,we show that EB1 forms molecular condensates with TIP150 and MCAK through liquid–liquid phase separation to compartmentalize the kinetochore–microtubule plus-end machinery,ensuring accurate kinetochore–microtubule interactions during chromosome segregation in *** of EB1–TIP150 polymer formation by a competing peptide prevents phase separation of the EB1-mediated complex and chromosome alignment at the metaphase equator in both cultured cells and Drosophila ***220 of EB1 is dynamically acetylated by p300/CBP-associated factor in early mitosis,and persistent acetylation at Lys220 attenuates phase separation of the EB1-mediated complex,dissolves droplets in vitro,and harnesses accurate chromosome *** data suggest a novel framework for understanding the organization and regulation of eukaryotic spindle for accurate chromosome segregation in mitosis.

关键词： phase separation mitosis microtubule dynamics EB1 acetylation