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22例基底细胞痣综合征患者的临床和遗传学研究(法语)

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世界核心医学期刊文摘（皮肤病学分册） 2006年第10期2卷 23-23页

作者： Pruvost-Balland C. Gorry P. Boutet N. m.-F.Avril 冯义国 Service de Dermatologie Hpital Cochin-Tarnier89rue d'Assas75006 ParisFrance

*** basal cell carcinoma syndrome is an autosomal dominant disorder characterized by developmental abnormalities and cancer *** PTCh 1 gene,the human homolog of the Drosophila segment polarity gene patched,has been shown to be involved in the development of nevoid basal cell carcinoma *** 1 is mapped to chromosome *** aim of the present study was to report on clinical and genetic characteristics in patients followed for nevoid basal cell carcinoma syndrome and to compare them to the data in the *** and *** for PTCh 1 mutations was done in 22 patients followed between 1981 and 2003 for clinical suspicion of nevoid basal cell carcinoma *** and radiological data were reviewed retrospectively from *** analysis was performed using blood samples after patient informed consent was *** possible,DNA was also analyzed from the parents of patients in whom PTCh 1 mutations were *** patients had developed basal cell carcinomas:45%palmar and plantar pitting,62%jaw cysts and 66%calcification of falx *** and meningiomas were the most common associated *** 1 mutations were identified in 13 patients:6 familial cases,3 sporadic cases and for 4 patients,it was not possible to *** different new germ-line mutations were *** analysis allows molecular confirmation of diagnosis in about half of all *** diagnosis is essential for detection of clinical and radiological manifestations in young patients and for provision of advice concerning protection of the skin from the sunlight.

关键词：基底细胞遗传学分析易患性放射检查掌跖遗传学特征发育异常颌骨囊肿基因突变脑膜瘤

结肠切除术联合回肠直肠吻合术可维持女性溃疡性结肠炎患者的生殖能力

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世界核心医学期刊文摘（胃肠病学分册） 2006年第11期2卷 17-17页

作者： mortier P.-E. Gambiez L. karoui m. J.-F. Colombel 王顺涛 Clinique des maladies de l'Appareil Digestif Hpital Huriez CHRU 59037 Lille Cedex France

Objectives -Restorative proctocolectomy with ileoanal anastomosis (IPAA) is the surgical standard for patients with ulcerative colitis (UC). Significant reduction in female fertility and fecundity after IPAA has been shown in recent studies. In selected cases, colectomy with ileorectal anastomosis (IRA) is another surgical option. The aim of this study was to evaluate fertility in women with UC who underwent IRA. Patients and methods -This study included all women with UC who underwent IRA between 1962 and 1999 and who were 40 years old or younger at the time of surgery, and older than 18 years of age at the time of the interview. Data were collected using a structured telephone interview concerning reproductive behavior and waiting times to pregnancy. Results -Among 40 eligible patients, 37 whose mean age at IRA was 28 years (range 11-39) answered the questionnaire. Twenty-two were unmarried, not wishful of pregnancy and / or already had children. Among 15 females wishing children after IRA, 10 (66%) became pregnant: one had therapeutic abortion, two had a miscarriage, four had 1 child, two had 2 children and one had 4 children. Five patients were sterile after IRA. Conclusion -These preliminary results suggest that IRA for UC preserves female fertility. If confirmed in other series this information should be provided to young women with UC before deciding surgical option.

关键词：结肠切除术回肠直肠吻合术生殖能力溃疡性结肠炎结肠直肠切除术治疗性流产不育者电话访谈自

减肥期间低糖负荷饮食对静息能量消耗和心脏病危险因素的作用

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世界核心医学期刊文摘（心脏病学分册） 2005年第4期1卷 21-21页

作者： Pereira m. A. Swain J. Goldfine A. B. D. S. Ludwig 王亭忠 Department of medicine Children's Hospital 300 Longwood Ave Boston MA 02115 United States Dr.

Context: Weight loss elicits physiological adaptations relating to energy inta ke and expenditure that antagonize ongoing weight loss. Objective: To test wheth er dietary composition affects the physiological adaptations to weight loss, as assessed by resting energy expenditure. Design, Study, and Participants: A rando mized paralleldesign study of 39 over-weight or obese young adults aged 18 to 4 0 years who received an energy-restricted diet, either low-glycemic load or lo w-fat. Participants were studied in the General Clinical Research Centers of th e Brigham and Women’s hospital and the Children’s hospital, Boston, mass, befo re and after 10%weightless. The study was conducted from January 4,2001, to may 6, 2003. main Outcome measures: Resting energy expenditure measured in the fast ing state by indirect calorimetry, body composition by dual-energy X-ray absor ptiometry, cardiovascular disease risk factors, and self-reported hunger. Resul ts: Resting energy expenditure decreased less with the low-glycemic load diet t han with the low-fat diet, expressed in absolute terms(mean[SE], 96 [24] vs 176 [27] kcal/d; P=.04) or as a proportion(5.9%[1.5%] vs 10.6%[1.7%]; P=.05). Pa rticipants receiving the low-glycemic load diet reported less hunger than those receiving the low-fat diet(P=.04). Insulin resistance(P=.01), serum triglyceri des(P=.01), C-reac-tive protein(P=.03), and blood pressure(P=.07 for both syst olic and diastolic) improved more with the low-gly-cemic load diet. Changes in body composition (fat and lean mass) in both groups were very similar(P=.85 and P=.45, respectively). Conclusions: Changes in dietary composition within prevai ling norms can affect physiological adaptations that defend body weight. Reducti on in glycemic load may aid in the prevention or treatment of obesity, cardiovas cular disease, and diabetes mellitus.

关键词：糖负荷静息能量消耗低脂饮食生理性代偿反应饥饿感空腹状态胰岛素抵抗临床研究中心儿童医院心血管疾病

幼儿急性阑尾炎超声与CT诊断的成本效益比较——一个markov决策分析模型

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国际医学放射学杂志 2009年第2期32卷 185-185页

作者： m.krahn W.J.Ungar E.aku L.Sung L.S.medina 刘洪超 Department of Diagnostic Imaging Hospital for Sick Children

目的使用决策分析模型来比较不同成像检查诊断小儿阑尾炎的成本效益。方法本回顾性研究以复习文献为基础，无需研究伦理委员会批准。markov决策模型由从文献中得到的成本、效能和概率所构成。

关键词：成本效益比急性阑尾炎决策模型决策分析 CT诊断超声原子弹爆炸幸存者幼儿

早发性炎症性肠道疾病患儿:对协会登记的IBD患儿的分析

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世界核心医学期刊文摘（儿科学分册） 2005年第6期1卷 32-32页

作者： heyman m. B. kirschner B. S. Gold B. D. 王一飞

To determine the characteristics of inflammatory bowel disease (IBD) in young patients. Uniform data were collected from a cohort of patients with IBD who were enrolled from January 2000 to November 2002 at six pediatric centers (Pediatric IBD Consortium). Of 1370 children in the registry, the mean age at IBD diagnosis was 10.3 ±4.4 years; 54%were male, and 86%were white. Diagnosis was confirmed in 87 (6.1%) under 3 years of age, 211 (15.4%) before 6 years, 654 (47.7%) at 6 to 12 years, and 505 (36.9%) at 13 to 17 years. more than 63%of children younger than 8 years of age had isolated colonic disease, whether Crohn disease, ulcerative colitis (UC), or indeterminate colitis. Conversely, only 35%of those 8 years of age or older had isolated colonic disease (Phad one or more family members with IBD. The subgroup of children younger than 3 years of age with UC had the highest prevalence of first-degree relatives with IBD (44%). This demographically diverse pediatric IBD cohort revealed age-related variation in the distribution of IBD phenotype, with a high prevalence of isolated colonic disease in young children. Positive family history was especially common in young patients with UC.

关键词：疾病患儿 IBD 早发性克罗恩病结肠疾病年龄相关性阳性家族史人口统计学显型家庭成员

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Biomarkers of aging

Science China(Life Sciences) 2023年第5期66卷 893-1066页

作者： Aging Biomarker Consortium hainan Bao Jiani Cao mengting Chen min Chen Wei Chen Xiao Chen Yanhao Chen Yu Chen Yutian Chen Zhiyang Chen Jagadish k Chhetri Yingjie Ding Junlin Feng Jun Guo mengmeng Guo Chuting he Yujuan Jia haiping Jiang Ying Jing Dingfeng Li Jiaming Li Jingyi Li Qinhao Liang Rui Liang Feng Liu Xiaoqian Liu Zuojun Liu Oscar Junhong Luo Jianwei Lv Jingyi ma kehang mao Jiawei Nie Xinhua Qiao Xinpei Sun Xiaoqiang Tang Jianfang Wang Qiaoran Wang Siyuan Wang Xuan Wang Yaning Wang Yuhan Wang Rimo Wu kai Xia Fu-hui Xiao Lingyan Xu Yingying Xu haoteng Yan Liang Yang Ruici Yang Yuanxin Yang Yilin Ying Le Zhang Weiwei Zhang Wenwan Zhang Xing Zhang Zhuo Zhang min Zhou Rui Zhou Qingchen Zhu Zhengmao Zhu Feng Cao Zhongwei Cao Piu Chan Chang Chen Guobing Chen hou-Zao Chen Jun Chen Weimin Ci Bi-Sen Ding Qiurong Ding Feng Gao Jing-Dong Jhan kai huang Zhenyu Ju Qing-Peng kong Ji Li Jian Li Xin Li Baohua Liu Feng Liu Lin Liu Qiang Liu Qiang Liu Xingguo Liu Yong Liu Xianghang Luo Shuai ma Xinran ma Zhiyong mao Jing Nie Yaojin Peng Jing Qu Jie Ren Ruibao Ren moshi Song Zhou Songyang Yi Eve Sun Yu Sun mei Tian Shusen Wang Si Wang Xia Wang Xiaoning Wang Yan-Jiang Wang Yunfang Wang Catherine CL Wong Andy Peng Xiang Yichuan Xiao Zhengwei Xie Daichao Xu Jing Ye Rui Yue Cuntai Zhang hongbo Zhang Liang Zhang Weiqi Zhang Yong Zhang Yun-Wu Zhang Zhuohua Zhang Tongbiao Zhao Yuzheng Zhao Dahai Zhu Weiguo Zou Gang Pei Guang-hui Liu Aging Biomarker Consortium(ABC) Beijing 100101China CAS key Laboratory of Genomic and Precision medicine Beijing Institute of GenomicsChinese Academy of Sciences and China National Center for BioinformationBeijing 100101China State key Laboratory of Stem Cell and Reproductive Biology Institute of ZoologyChinese Academy of SciencesBeijing 100101China Department of Dermatology Xiangya HospitalCentral South UniversityChangsha 410008China hunan key Laboratory of Aging Biology Xiangya HospitalCentral South UniversityChangsha 410008China National Clinical Research Center for Geriatric Disorders Xiangya HospitalCentral South UniversityChangsha 410008China Clinic Center of human Gene Research Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430022China hubei Clinical Research Center of metabolic and Cardiovascular Disease Huazhong University of Science and TechnologyWuhan 430022China hubei key Laboratory of metabolic Abnormalities and Vascular Aging Huazhong University of Science and TechnologyWuhan 430022China Stem Cell Translational Research Center Tongji HospitalTongji University School of MedicineShanghai 200065China Department of Nuclear medicine Daping HospitalThird Military Medical UniversityChongqing 400042China CAS key Laboratory of Nutrition Metabolism and Food SafetyShanghai Institute of Nutrition and HealthUniversity of Chinese Academy of SciencesChinese Academy of SciencesShanghai 200031China Shanghai key Laboratory of maternal Fetal medicine Clinical and Translational Research Center of Shanghai First Maternity and Infant HospitalFrontier Science Center for Stem Cell ResearchShanghai Key Laboratory of Signaling and Disease ResearchSchool of Life Sciences and TechnologyTongji UniversityShanghai 200092China The Department of Endovascular Surgery The First Affiliated Hospital of Zhengzhou UniversityZhengzhou 450052China key Laboratory of Regenerative medicine of ministry of Education Institute of Ageing and Regenerative MedicineJ

Aging biomarkers are a combination of biological parameters to(i)assess age-related changes,(ii)track the physiological aging process,and(iii)predict the transition into a pathological *** a broad spectrum of aging biomarkers has been developed,their potential uses and limitations remain poorly *** immediate goal of biomarkers is to help us answer the following three fundamental questions in aging research:how old are we?Why do we get old?And how can we age slower?This review aims to address this ***,we summarize our current knowledge of biomarkers developed for cellular,organ,and organismal levels of aging,comprising six pillars:physiological characteristics,medical imaging,histological features,cellular alterations,molecular changes,and secretory *** fulfill all these requisites,we propose that aging biomarkers should qualify for being specific,systemic,and clinically relevant.

关键词： aging senescence biomarker clock

质子密度脂肪分数是少女和年轻女性脂肪肝的一个准确生物标志物

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国际医学放射学杂志 2015年第6期 606-606页

作者： J.L.Rehm P.m.Wolfgram D.hernando J.C.Eickhoff D.B.Allen S.B.Reeder 谢双双 Department of Pediatrics University of Wisconsin School of Medicine and Public Health

摘要目的比较复合定量mRI和mRS对脂肪肝的定量,并确定具有临床意义的年轻女性脂肪肝的mRI诊断阈值。方法 132名健康女性[年龄11～22岁,平均(13.3±2)岁]被

关键词：脂肪肝 mRI PDFF 年轻女性肝疾病脂肪分数质子密度少女

Epstein-Barr virus-encoded latent membrane protein 1 modulates cyclin D1 by c-Jun/Jun B heterodimers

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Science China(Life Sciences) 2005年第4期48卷 385-393页

作者： SONG Xin1, TAO Yongguang1, ZENG Liang1, YANG Jing1, TANG Faqing1, Leo m. Lee2, GONG Jianping3, WU Qiao4 & CAO Ya1 1. Cancer Research Institute, Xiangya School of medicine, Central South University, Changsha 410078, China 2. Laboratory of molecular Technology SAIC-Frederick, National Cancer Institute P.O. Box B, Frederick, mD 21702, USA 3. molecular medical Center, Tongji hospital, Tongji medical University, Wuhan 430030, China 4. key Laboratory of the ministry of Education for Cell Biology, and Tumor Cell Engineering, School of Life Sciences, Xiamen University, Xiamen 361005, China Cancer Research Institute Xiangya School of Medicine Central South University Changsha 410078 China Laboratory of molecular Technology SAIC-Frederick National Cancer Institute P.O. Box B Frederick MD 21702 USA molecular medical Center Tongji Hospital Tongji Medical University Wuhan 430030 China key Laboratory of the ministry of Education for Cell Biology and Tumor Cell Engineering School of Life Sciences Xiamen University Xiamen 361005 China

In our recent studies, we found that LmP1 encoded by Epstein-Barr virus could accelerate the formation of active c-Jun/Jun B heterodimer. We studied the regulation of cyclinD1 by c-Jun/Jun B heterodimers by laser scanning confocal influorescence microscopy, Western blot, luciferase activity assay, super-EmSA and flow cytometry in the Tet-on-LmP1 hNE2 cell line, in which LmP1 expression was regulated by Tet-on system. c-Jun/Jun B heterodimers induced by LmP1 could up regulate cyclin D1 promoter activity and expression. Overexpression of cy-clinD1 accelerated the progression of cell cycle.

关键词： latent membrane protein1, c-Jun, Jun B, heterodimer, cyclin D1, cell cycle.

CEPC Technical Design Report

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Radiation Detection Technology and methods 2024年第1期8卷 I0003-I0016,1-1091页

作者： Waleed Abdallah Tiago Carlos Adorno de Freitas konstantin Afanaciev Shakeel Ahmad Ijaz Ahmed Xiaocong Ai Abid Aleem Wolfgang Altmannshofer Fabio Alves Weiming An Rui An Daniele Paolo Anderle Stefan Antusch Yasuo Arai Andrej Arbuzov Abdesslam Arhrib mustafa Ashry Sha Bai Yu Bai Yang Bai Vipul Bairathi Csaba Balazs Philip Bambade Yong Ban Tripamo Bandyopadhyay Shou-Shan Bao Desmond P.Barber Ayse Bat Varvara Batozskaya Subash Chandra Behera Alexander Belyaev michele Bertucci Xiao-Jun Bi Yuanjie Bi Tianjian Bian Fabrizio Bianchi Thomas Biekotter michela Biglietti Shalva Bilanishvili Deng Binglin Denis Bodrov Anton Bogomyagkov Serge Bondarenko Stewart Boogert maarten Boonekamp marcello Borri Angelo Bosotti Vincent Boudry mohammed Boukidi Igor Boyko Ivanka Bozovic Giuseppe Bozzi Jean-Claude Brient Anastasiia Budzinskaya masroor Bukhari Vladimir Bytev Giacomo Cacciapaglia hua Cai Wenyong Cai Wujun Cai Yijian Cai Yizhou Cai Yuchen Cai haiying Cai huacheng Cai Lorenzo Calibbi Junsong Cang Guofu Cao Jianshe Cao Antoine Chance Xuejun Chang Yue Chang Zhe Chang Xinyuan Chang Wei Chao Auttakit Chatrabhuti Yimin Che Yuzhi Che Bin Chen Danping Chen Fuqing Chen Fusan Chen Gang Chen Guoming Chen hua-Xing Chen huirun Chen Jinhui Chen Ji-Yuan Chen kai Chen mali Chen mingjun Chen mingshui Chen Ning Chen Shanhong Chen Shanzhen Chen Shao-Long Chen Shaomin Chen Shiqiang Chen Tianlu Chen Wei Chen Xiang Chen Xiaoyu Chen Xin Chen Xun Chen Xurong Chen Ye Chen Ying Chen Yukai Chen Zelin Chen Zilin Chen Gang Chen Boping Chen Chunhui Chen hok Chuen Cheng huajie Cheng Shan Cheng Tongguang Cheng Yunlong Chi Pietro Chimenti Wen han Chiu Guk Cho ming-Chung Chu Xiaotong Chu Ziliang Chu Guglielmo Coloretti Andreas Crivellin hanhua Cui Xiaohao Cui Zhaoyuan Cui Brunella D'Anzi Ling-Yun Dai Xinchen Dai Xuwen Dai Antonio De maria Nicola De Filippis Christophe De La Taille Francesca De mori Chiara De Sio Elisa Del Core Shuangxue Deng Wei-Tian Deng Zhi Deng Ziyan Deng Bhupal Dev Tang Dewen Biagio Di micco Ran Ding Siqin Dingl Yadong Ding haiyi Dong Jianin AGh University of Science and Technology Faculty of Physics and Applied Computer ScienceKrakow Anhui University HefeiAnhui Applied Physics Department Federal Urdu University of ArtsScience and TechnologyIslamabad Banaras hindu University Institute of ScienceVaranasi Bandirma Onyedi Eylul University Bandirma Beihang University Beijing Beijing Computational Science Research Center Beijing Beijing Conveyi LTD Beijing Beijing Glass Research Institute Co. LtdBeijing Beijing Institute of Technology Beijing Beijing Normal University Beijing Brown University ProvidenceRI Budker Institute of Nuclear Physics Novosibirsk Cadi Ayyad University Laboratory of Fundamental and Applied PhysicsPolydisciplinary FacultyMarrakech Cairo University Giza Center for Theory and Computation National Tsing Hua UniversityHsinchu Central China Normal University WuhanHubei Central South University ChangshaHunan Centro de Fisica Teorica de Particulas Universidade Tecnica de LisboaLisboa Chengdu kaitengSifang Digital Radio&TV Equipment Co. LtdChengduSichuan China academy of engineering physics MianyangSichuan China building materials academy Beijing China Center of Advanced Science and Technology Beijing China Institute of Atomic Energy Beijing China Jiliang University HangzhouZhejiang China Nuclear Instrument CO. LTDBeijing China University of Geosciences Beijing China University of Geosciences WuhanHubei China University of mining and Technology XuzhouJiangsu China West Normal University NanchongSichuan Chongqing University Department of PhysicsChongqing Chulalongkorn University Bangkok City University of hong kong Department of PhysicsHong Kong Cockcroft Institute Daresbury Cornell University IthacaNY CPPm(AmU/IPhU&CNRS/INP) Marseille Dalian minzu University DalianLiaoning Dalian University of Technology Department of PhysicsDalian Deutsches Elektronen-Synchrotron DESY Hamburg Dzhelepov Laboratory of Nuclear Problems of Joint Institute for Nuclear Research(DLNP JINR) Dubna East China University Of Science And Technol

The Circular Electron Positron Collider(CEPC)is a large scientific project initiated and hosted by China,fostered through extensive collaboration with international *** complex comprises four accelerators:a 30 GeV Linac,a 1.1 GeV Damping Ring,a Booster capable of achieving energies up to 180 GeV,and a Collider operating at varying energy modes(Z,W,h,and tt).The Linac and Damping Ring are situated on the surface,while the subterranean Booster and Collider are housed in a 100 km circumference underground tunnel,strategically accommodating future expansion with provisions for a potential Super Proton Proton Collider(SPPC).The CEPC primarily serves as a higgs *** its baseline design with synchrotron radiation(SR)power of 30 mW per beam,it can achieve a luminosity of 5×10^(34)cm^(-2)s^(-1)per interaction point(IP),resulting in an integrated luminosity of 13 ab^(-1)for two IPs over a decade,producing 2.6 million higgs *** the SR power to 50 mW per beam expands the CEPC's capability to generate 4.3 million higgs bosons,facilitating precise measurements of higgs coupling at sub-percent levels,exceeding the precision expected from the hL-LhC by an order of *** Technical Design Report(TDR)follows the Preliminary Conceptual Design Report(Pre-CDR,2015)and the Conceptual Design Report(CDR,2018),comprehensively detailing the machine's layout,performance metrics,physical design and analysis,technical systems design,R&D and prototyping efforts,and associated civil engineering ***,it includes a cost estimate and a preliminary construction timeline,establishing a framework for forthcoming engineering design phase and site selection *** is anticipated to begin around 2027-2028,pending government approval,with an estimated duration of 8 *** commencement of experiments and data collection could potentially be initiated in the mid-2030s.

关键词： initiated exceeding precise