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Mazabraud’s syndrome in female patients:Two case reports

World Journal of Orthopedics 2024年第6期15卷 593-601页

作者： Xiu-mao Li Ze-Hao Chen Ke-Yi Wang Jun-Nan Chen Zhao-Nong Yao Yu-Hong Yao Xiao-Wei zhou Nong Lin Department of Orthopedics The Second Affiliated HospitalSchool of MedicineZhejiang UniversityHangzhou 310009Zhejiang ProvinceChina Orthopedics Research Institute Zhejiang UniversityHangzhou 310009Zhejiang ProvinceChina State Key Laboratory of Transvascular Implantation Devices Hangzhou 310009Zhejiang ProvinceChina Songyang Branch of the Second Affiliated Hospital School of MedicineZhejiang UniversityLishui 331124Zhejiang ProvinceChina

BACKGROUND Mazabraud’s syndrome(MS)is a rare and slowly progressive benign disease characterized by the concurrent presence of fibrous dysplasia of bone and intramuscular myxoma,and is thought to be associated with mutations of the GNAS *** date,only about 100 cases of MS have been reported in the literature,but its standard treatment strategy remains *** SUMMARY We report two cases of MS in young women who underwent different treatments based on their symptoms and disease *** first patient,aged 37,received internal fixation and intravenous bisphosphonate for a pathological fracture of the right femoral neck,excision of a right vastus medialis myxoma was subsequently performed for pain control,and asymptomatic psoas myxomas were monitored without *** testing confirmed a GNAS gene mutation in this *** second patient,aged 24,underwent right vastus intermedius muscle myxoma resection,and conservative treatment for fibrous dysplasia of the *** patients were followed-up for 17 months and 3 years,respectively,and are now in a stable *** Various treatments have been selected for MS patients who suffer different *** main treatment for myxomas is surgical resection,while fibrous dysplasia is selectively treated if the patient experiences pathological fracture or severe ***,given the documented instances of malignant transformation of fibrous dysplasia in individuals with MS,close follow-up is necessary.

关键词： Mazabraud's syndrome Fibrous dysplasia Intramuscular myxoma Bisphosphonate Case report

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Uridine diphosphate glucuronosyltransferase 1A1 prevents the progression of liver injury

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World Journal of Gastroenterology 2024年第9期30卷 1189-1212页

作者： Jin-Lian Jiang Yi-Yang zhou Wei-Wei Zhong Lin-Yan Luo Si-Ying Liu Xiao-Yu Xie mao-Yuan Mu Zhi-Gang Jiang Yuan Xue Jian Zhang Yi-Huai He Department of Infectious Diseases Affiliated Hospital of Zunyi Medical UniversityZunyi 563000Guizhou ProvinceChina Department of Infectious Diseases Jingmen Central HospitalJingmen 448000Hubei ProvinceChina Department of Respiratory Medicine Anshun People’s HospitalAnshun 561099Guizhou ProvinceChina Department of General Practice Affiliated Hospital of Zunyi Medical UniversityZunyi 563000Guizhou ProvinceChina Department of Intervention Radiology Affiliated Hospital of Zunyi Medical UniversityZunyi 563000Guizhou ProvinceChina School of Public Health Zunyi Medical UniversityZunyi 563099Guizhou ProvinceChina Department of Liver Diseases Third People’s Hospital of ChangzhouChangzhou 213000Jiangsu ProvinceChina Department of Digestion Dafang County People’s HospitalBijie 551600Guizhou ProvinceChina

BACKGROUND Uridine diphosphate glucuronosyltransferase 1A1(UGT1A1)plays a crucial role in metabolizing and detoxifying endogenous and exogenous ***,its contribution to the progression of liver damage remains *** To determine the role and mechanism of UGT1A1 in liver damage *** We investigated the relationship between UGT1A1 expression and liver injury through clinical ***,the impact and mechanism of UGT1A1 on the progression of liver injury was analyzed through a mouse model *** Patients with UGT1A1 gene mutations showed varying degrees of liver damage,while patients with acute-onchronic liver failure(ACLF)exhibited relatively reduced levels of UGT1A1 protein in the liver as compared to patients with chronic *** suggests that low UGT1A1 levels may be associated with the progression of liver *** mouse models of liver injury induced by carbon tetrachloride(CCl_(4))and concanavalin A(ConA),the hepatic levels of UGT1A1 protein were found to be *** mice with lipopolysaccharide or liver steatosis-mediated liver-injury progression,the hepatic protein levels of UGT1A1 were decreased,which is consistent with the observations in patients with ***1A1 knockout exacerbated CCl_(4)-and ConA-induced liver injury,hepatocyte apoptosis and necroptosis in mice,intensified hepatocyte endoplasmic reticulum(ER)stress and oxidative stress,and disrupted lipid *** UGT1A1 is upregulated as a compensatory response during liver injury,and interference with this upregulation process may worsen liver ***1A1 reduces ER stress,oxidative stress,and lipid metabolism disorder,thereby mitigating hepatocyte apoptosis and necroptosis.

关键词： Uridine diphosphate glucuronosyltransferase 1A1 Liver injury progression Endoplasmic reticulum stress Oxidative stress Lipid metabolism disorders

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基于优先权与双可变时间窗的大型热轧生产线机会维护建模

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上海交通大学学报 2024年第8期58卷 1221-1230页

作者：毛雯欣周晓军上海交通大学机械与动力工程学院上海200240

为解决大型热轧生产线日常动态维护和支撑辊定期更换带来的维护需求差异问题,综合考虑长距离条件下的维护资源及停机时间限制,引入维护优先权规则识别部件的组合维护判断次序,构建双可变时间窗规则辨别部件是否维护并区分维护需求,进而... 详细信息

为解决大型热轧生产线日常动态维护和支撑辊定期更换带来的维护需求差异问题,综合考虑长距离条件下的维护资源及停机时间限制,引入维护优先权规则识别部件的组合维护判断次序,构建双可变时间窗规则辨别部件是否维护并区分维护需求,进而建立系统整体动态机会维护模型.实例分析表明,该模型可有效解决维护资源和停机时间限制下大型热轧生产线的差异化维护调度问题,且比传统基于时间窗的模型更具成本优势.

关键词：大型热轧生产线机会维护预防维护维护优先权可变时间窗

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Iodine Nutrition, Thyroid-stimulating Hormone, and Related Factors of Postpartum Women from three Different Areas in China: A Cross-sectional Survey

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Biomedical and Environmental Sciences 2024年第3期37卷 254-265页

作者： SHAN Xiao Yun ZOU Yan HUANG Li Chun JIANG Shan zhou Wei Wen QIN Qiu Lan LIU Chang Qing LUO Xiao Yan LU Jia Xi mao De Qian LI Min YANG Zhen Yu YANG Li Chen Key Laboratory of Trace Element Nutrition of National Health Committee National Institute for Nutrition and HealthChinese Center for Disease Control and PreventionBeijing 100050China Hunan Key Laboratory of Typical Environmental Pollution and Health Hazards School of Public HealthHengyang Medical SchoolUniversity of South ChinaHengyang 241001HunanChina Zhejiang Provincial Center for Disease Prevention and Control Hangzhou 310051ZhejiangChina Guangxi Zhuang Autonomous Region Center for Disease Control and Prevention Nanning 530028GuangxiChina Hebei Provincial Center for Disease Control and Prevention Shijiazhuang 050021HebeiChina

Objective Studies on the relationship between iodine,vitamin A(VA),and vitamin D(VD)and thyroid function are *** study aimed to analyze iodine and thyroid-stimulating hormone(TSH)status and their possible relationships with VA,VD,and other factors in postpartum *** A total of 1,311 mothers(896 lactating and 415 non-lactating)from Hebei,Zhejiang,and Guangxi provinces were included in this *** urinary iodine concentration(UIC),TSH,VA,and VD were *** The median UIC of total and lactating participants were 142.00µg/L and 139.95µg/L,*** median TSH,VA,and VD levels in all the participants were 1.89 mIU/L,0.44μg/mL,and 24.04 ng/mL,*** differences in the UIC were found between lactating and non-lactating *** and TSH levels were significantly different among the three *** rural UIC was higher than the urban *** mothers had a higher UIC and a higher prevalence of excessive *** UICs and TSHs levels were observed in both the VD deficiency and insufficiency groups than in the VD-sufficient *** adjustment,no linear correlation was observed between UIC and VA/*** interaction was found between vitamins A/D and UIC on TSH *** The mothers in the present study had no iodine ***,area type,BMI,and VD may be related to the iodine status or TSH levels.

关键词： Median urinary iodine concentration Thyroid-stimulating hormone Vitamin A Vitamin D Postpartum women

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Consensus of Chinese experts on glioma multidisciplinary team management(2nd edition)

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Chinese Medical Journal 2024年第11期137卷 1267-1270页

作者： Jinsong Wu Zhirui zhou Zanyi Wu Zhiwei Tang Ming Li Siyuan Yin Xufei Luo Ling Wang Yaolong Chen Guoguang Zhao Tao Jiang Ying mao The National Glioma MDT Alliance of National Center for Neurological Diseases Department of Neurosurgery Huashan HospitalShanghai Medical CollegeFudan UniversityShanghai 200040China Department of Radiation Oncology Huashan HospitalShanghai Medical CollegeFudan UniversityShanghai 200040China Department of Neurosurgery The First Affiliated Hospital of Fujian Medical UniversityFuzhouFujian 350005China Neurosurgery Department The First Affiliated Hospital of Kunming Medical UniversityKunmingYunnan 650032China Department of Neurosurgery Henan Provincial People’s HospitalPeople’s Hospital of Zhengzhou UniversityZhengzhouHenan 463599China State Key Laboratory of Pharmaceutical Biotechnology Institute for Brain SciencesAdvanced Institute for Life SciencesSchool of Life SciencesNanjing UniversityNanjingJiangsu 210023China Evidence-Based Medicine Center School of Basic Medical SciencesLanzhou UniversityLanzhouGansu 730000China School of Public Health Lanzhou UniversityLanzhouGansu 730000China Department of Neurosurgery Xuanwu HospitalCapital Medical UniversityBeijing 100053China Department of Neurosurgery Beijing Tiantan HospitalCapital Medical UniversityBeijing 100050China 不详

To increase the consistency of glioma multidisciplinary team(MDT)management across different regions and hospitals at varying levels,we have updated the Expert Consensus on MDT of Glioma in China based on the currently available *** version has revised and updated the process-management rules and quality-control standards for a glioma MDT,providing reference and guidance for relevant clinical disciplines and *** members of the Consensus Expert Group,abstract,background,and prospects can be seen in supplementaryle,http://***/CM9/B999.

关键词： glioma MDT Expert

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Salinity fronts shape spatial patterns in zooplankton distribution in Hangzhou Bay

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Acta Oceanologica Sinica 2024年第6期43卷 96-106页

作者： Yepeng Xu Yiqi Wang Lin Zhan Yijun Ou Kangning Jia Ming mao Xuyu Zhu Zhibing Jiang Yuanli Zhu Wei Huang Ping Du Jiangning Zeng Lu Shou Feng zhou Key Laboratory of Marine Ecosystem Dynamics Second Institute of OceanographyMinistry of Natural ResourcesHangzhou 310012China Key Laboratory of Nearshore Engineering Environment and Ecological Security of Zhejiang Province Hangzhou 310012China State Key Laboratory of Satellite Ocean Environment Dynamics Ministry of Natural ResourcesHangzhou 310012China Nantong Marine Environmental Monitoring Center Ministry of Natural ResourcesNantong 226002China Key Laboratory of Ocean Space Resource Management Technology Ministry of Natural ResourcesHangzhou 310012China Observation and Research Station of Marine Ecosystem in the Yangtze River Delta Ministry of Natural ResourcesZhoushan 316021China

Ocean fronts play important roles in nutrient transport and in the shaping ecological *** zones in small bays are typically small in scale,have a complex structure,and they are spatially and temporally variable,but there are limited data on how biological communities respond to this *** Bay,a mediumsized estuary in China,is an ideal place in which to study the response of plankton to small-scale ocean fronts,because three water masses(Qiantang River Diluted Water,Changjiang River Diluted Water,and the East China Sea current) converge here and form dynamic salinity fronts throughout the *** investigate zooplankton communities,and temperature,salinity and chlorophyll a(Chl a) in Hangzhou Bay in June(wet perio d) and December(dry period) of 2022 and examine the dominant environmental factors that affect zooplankton community spatial *** then match the spatial distributions of zooplankton communities with those of salinity fronts.S alinity is the most important explanatory variable to affect zooplankton community spatial variability during both wet and dry periods,in that it contributes>60% of the variability in community ***,the spatial distributions of zooplankton match well with salinity *** December,with weaker Qiantang River Diluted Water and a stronger secondary Changjiang River Plume,zooplankton communities occur in moderate salinity(MS,salinity range 15.6±2.2) and high salinity(HS,22.4±1.7) regions,and their ecological boundaries closely match the Qiantang River Diluted Water *** June,different zooplankton communities occur in low salinity(LS,3.9±1.0),MS(11.7±3.6) and HS(21.3±1.9) *** the LS region occurs abnormally in the central bay rather than its apex because of the anomalous influence of rising and falling tides during the sampling perio d,the ecological boundaries still match salinity ***-salinity or brackish-water zooplankter taxa are relatively more abunda

关键词： zooplankton spatial distribution salinity fronts Hangzhou Bay

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Low-dose immune tolerance induction for severe hemophilia A inhibitor patients:Immunosuppressants are generally not necessary for inhibitor-titer below 200 BU/mL

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Pediatric Investigation 2024年第2期8卷 91-100页

作者： Zhengping Li Jie Sun Zekun Li Zhenping Chen Guoqing Liu Wanru Yao Xiaoling Cheng Gang Li Yingzi Zhen Di Ai Yaohan zhou Qianqian mao Man-Chiu Poon Runhui Wu Hemophilia Comprehensive Care Center Hematology DepartmentHematology CenterBeijing Key Laboratory of Pediatric Hematology-OncologyKey Laboratory of Major Diseases in ChildrenNational Key Discipline of Pediatrics(Capital Medical University)Ministry of EducationBeijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Hematologic Disease Laboratory Hematology DepartmentHematology CenterBeijing Key Laboratory of Pediatric Hematology-OncologyKey Laboratory of Major Diseases in ChildrenNational Key Discipline of Pediatrics(Capital Medical University)Ministry of EducationBeijing Pediatric Research InstituteBeijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Department of Pharmacy Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Departments of Medicine Pediatrics and OncologyUniversity of Calgary Cumming School of MedicineCalgaryCanada

Importance:It remained unclear that the efficacy comparison between low-dose immune tolerance induction(LD-ITI)incorporating immunosuppressants(IS)when severe hemophilia A(SHA)patients had inhibitor-titer≥200 Bethesda Units(BU)/mL(LD-ITI-IS^(200) regimen)and LD-ITI combining with IS when SHA patients had inhibitor-titer≥40 BU/mL(LD-ITI-IS^(40) regimen).Objective:To compare the efficacy of the LD-ITI-IS^(200) regimen with that of the LD-ITI-IS^(40) regimen for SHA patients with high-titer ***:A prospective cohort study on patients receiving LD-ITI-IS^(200) compared to those receiving LD-ITI-IS^(40) from January 2021 to December *** received LD-ITI[FVIII 50 IU/kg every other day].IS(rituximab+prednisone)was added when peak inhibitor tier≥200 BU/mL in the LD-ITI-IS^(200) regimen and≥40 BU/mL in the LD-ITI-IS^(40) *** is defined as a negative inhibitor plus FVIII recovery≥66%of the ***:We enrolled 30 patients on LD-ITI-IS^(200) and 64 patients on LD-ITI-IS^(40),with similar baseline clinical characteristics.A lower IS-use rate was discovered in the LD-ITI-IS^(200) regimen compared to the LD-ITI-IS^(40) regimen(30.0%vs.62.5%).The two regimens(LD-ITI-IS^(200) ***-ITI-IS^(40))had similar success rate(70.0%vs.79.7%),median time to success(9.4 vs.10.6 months),and annualized bleeding rate during ITI(3.7 vs.2.8).The cost to success was lower for LD-ITI-IS^(200) than for LD-ITI-IS^(40)(2107 vs.3256 US Dollar/kg).Among patients with peak inhibitor-titer 40-199 BU/mL,10 non-IS-using(on LD-ITI-IS^(200) regimen)and 28 IS-using(on LD-ITI-IS^(40) regimen)had similar success rates(70.0%vs.78.6%)and time to success(9.0 vs.8.8 months).Interpretation:In LD-ITI,IS are not necessary for inhibitor titer<200 BU/mL.

关键词： Hemophilia A High-titer inhibitor Immune tolerance induction Immunosuppressant Low-dose

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QCA方法在国内外管理学领域的应用:演进、比较与展望

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经济管理 2024年第1期46卷 184-208页

作者：池毛毛潘美钰周敏杜运周中国地质大学(武汉)经济管理学院湖北武汉430078 华南理工大学电子商务系广东广州510006 东南大学经济管理学院江苏南京211189

定性比较分析(QCA)已成为当前国内外管理学领域重要分析方法与研究范式。本文采用文献计量方法,以1987一2022年WebofScience核心数据库的502篇期刊论文和CNKI数据库中的633篇CSSCI期刊论文作为研究样本,系统梳理并分析QCA在国内外管理... 详细信息

定性比较分析(QCA)已成为当前国内外管理学领域重要分析方法与研究范式。本文采用文献计量方法,以1987一2022年WebofScience核心数据库的502篇期刊论文和CNKI数据库中的633篇CSSCI期刊论文作为研究样本,系统梳理并分析QCA在国内外管理学领域的应用现状、研究热点和演进脉络。主要发现包括:(1)在研究热点方面,国外文献主要聚焦在“组织创新及其复杂性”“创业管理”“复杂性理论下的组织行为与消费者行为”和“组织绩效的多重复杂前因”四个大主题;国内研究主要聚焦在“组织创新”“创业管理”“公共管理与政策服务”和“数字经济与数字化转型”四个大主题。(2)在演进脉络方面,QCA在国内外管理学的研究进展均可划分为三个阶段,但在研究主题与范围演化方面,国内外文献呈现出差异化特征。QCA在国外文献的应用主题日趋集中,而国内文献关于QCA应用范围还在发散且更注重其在本土化管理情境的解释与应用。最后,通过对比与总结国内外文献的研究热点与演进脉络,本文从研究主题、理论视角与研究方法三个方面提出QCA在管理学领域有前景的拓展方向。本研究为全面认识和理解QCA在国内外管理学的应用现状与发展动态,进而为深入运用QCA解决中国管理实践问题提供相关理论借鉴或参考。

关键词：定性比较分析(QCA) 研究热点演进脉络文献计量学对比研究

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基于随机森林的北京城区臭氧敏感性分析

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环境科学 2024年第5期45卷 2497-2506页

作者：周红王鸣柴文轩赵昕南京信息工程大学环境科学与工程学院江苏省大气环境与装备技术协同创新中心江苏省大气环境监测与污染控制高技术研究重点实验室南京210044 中国环境监测总站北京100012 南京科略环境科技有限责任公司南京211800

明确臭氧(O_(3))与前体物的非线性关系是O_(3)防控措施制定的基础和关键.基于北京城区站点2020年4~9月O_(3)、挥发性有机物(VOCs)、氮氧化物(NO_(x))和气象要素在线观测,分析了O_(3)及其前体物污染特征,利用随机森林(RF)模型结合SHAP值... 详细信息

明确臭氧(O_(3))与前体物的非线性关系是O_(3)防控措施制定的基础和关键.基于北京城区站点2020年4~9月O_(3)、挥发性有机物(VOCs)、氮氧化物(NO_(x))和气象要素在线观测,分析了O_(3)及其前体物污染特征,利用随机森林(RF)模型结合SHAP值探究了影响O_(3)的关键因素,并通过多情景分析探讨了O_(3)-VOCs-NO_(x)敏感性.相关性分析结果显示O_(3)小时浓度与温度(T)呈显著正相关,与TVOCs和NO_(x)呈显著负相关;但从每日结果来看,O_(3)与T、TVOCs和NO_(x)均呈显著正相关.RF模型模拟的O_(3)浓度与实测值吻合较好,进一步计算了各个特征变量的SHAP值,结果显示T和NO_(x)对O_(3)影响最高,但前者是正向影响,而后者是负向影响.以观测期间O_(3)污染天的NO_(x)和VOCs平均值为基础情景,设置了对应不同NO_(x)和VOCs的多种情景,并利用RF模型计算不同情景下的O_(3),得到O_(3)等值线(EKMA曲线),结果显示北京城区O_(3)-VOCs-NO_(x)敏感性处于VOCs控制区,与基于观测的盒子模型(OBM)得到的结果一致,这说明RF模型可以用作O_(3)-VOCs-NO_(x)敏感性分析的一种补充方法.

关键词：北京臭氧(O_(3)) O_(3)-VOCs-NO_(x)敏感性随机森林(RF) SHAP值

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中国经济学自主知识体系和中国特色社会主义政治经济学理论体系构建研究

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政治经济学评论 2024年第2期15卷 62-76页

作者：周文柴斯捷中国人民大学经济学院中国人民大学全国中国特色社会主义研究中心

一、引言2022年4月25日习近平总书记在中国人民大学考察调研时发表重要讲话指出,加快构建中国特色哲学社会科学,归根结底是建构中国自主的知识体系。^((1))经济学是哲学社会科学重要组成部分,建构中国自主的知识体系必然包含建构中国经... 详细信息

一、引言2022年4月25日习近平总书记在中国人民大学考察调研时发表重要讲话指出,加快构建中国特色哲学社会科学,归根结底是建构中国自主的知识体系。^((1))经济学是哲学社会科学重要组成部分,建构中国自主的知识体系必然包含建构中国经济学自主知识体系。

关键词：中国人民大学中国特色哲学社会科学中国特色社会主义政治经济学理论体系构建考察调研自主习近平总书记建构

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