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Clinical features of Huntington disease in 243 Chinese patients

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Neural Regeneration Research 2010年第2期5卷 102-107页

作者： Xiaoyan Guo Shushan Zhang jean-marc Burgunder Huifang Shang Department of Neurology West China Hospital Sichuan University Chengdu 610041 Sichuan Province China Department of Neurology Inselspital BernUniversity CH-3010 Bern Switzerland

OBJECTIVE： To thoroughly explore the clinical characteristics of Huntington disease in China. METHODS： A computer-based online search of China National Knowledge Infrastructure was performed to review case reports concerning Huntington disease published between 1980 and 2008; the clinical characteristics were analyzed. RESULTS： A total of 80 studies involving 243 patients （142 males and 101 females） were collected, 82.0% of which were from provinces of North China. In addition, 97.1% of the cases had a family history of Huntington disease, and paternal inheritance （64.6%） was greater than maternal inheritance （35.4%）. Moreover, onset age was significantly less than from maternal inheritance. The mean onset age of Huntington disease was （35.2 ± 11.5） years, mean age of death was （45.6 ± 13.5） years, and the mean course of disease from onset to death was （11.6 ± 5.6） years. Onset characterized by involuntary movement accounted for 47.7%, including 66.4% in the entire body, 16.4% in the upper limbs, and 14.7% in the head and face. Psychiatric symptoms accounted for 18.1%, and disturbed intelligence accounted for 2.1%. With disease progression, 99.5% of patients exhibited involuntary movement, 69.8% displayed cognitive impairment, and 39.2% suffered from psychiatric symptoms. In addition, 38.7% of patients were complicated by dysarthria, dysphagia, and cough after drinking. A total of 70.8% of patients exhibited an abnormal electroencephalogram, 18.8% had mild abnormalities in the cerebrospinal fluid, and 70.1% displayed brain atrophy and lateral ventriculomegaly on CT or MRI. A total of 88.9% of patients scored ≤ 23 in the Mini-Mental State Examination （MMSE）. Of the reported patients, only 22 underwent/T15 gene testing with positive results. CONCLUSION： Huntington disease is more frequently detected in males than females, and the majority has a family history. The disease has high incidence in Northern China, in particular with paternal inheritance. In addition, the d

关键词： Huntington disease clinical characteristics literature evaluation neurodegenerative disease

Concomitant autoimmune and genetic pancreatitis leads to severe inflammatory conditions

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World Journal of Gastroenterology 2008年第16期14卷 2596-2598页

作者： jean Louis Frossard jean marc Dumonceau Catherine Pastor Laurent Spahr Antoine Hadengue Division de Gastroentérologie et Hépatologie Geneva University Hospital

Chronic pancreatitis characterized by an early onset should be extensively investigated including the search for a mutation of the PRSS1, SPINK-1 or CFTR genes and potential features of autoimmune pancreatitis. We here describe a case of chronic pancreatitis with an onset at a very young age in which a mutation of the PRSS1 and several features of autoimmune pancreatitis were identified.

关键词： Chronic pancreatitis Genetics Autoimmunepancreatltis SPINK-1 CFTR PRSS1

Bio-inspired Topological Skeleton for the Analysis of Quadruped Kinematic Gait

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Journal of Bionic Engineering 2018年第5期15卷 839-850页

作者： Benjamin Boudon jean-marc Linares Anick Abourachid Arthur Vauquelin Emmanuel Mermoz Aix Marseille University CNRS ISM Marseille 13009 France Museum National d'Histoire Naturelle Pavillon d'Anatomie Comparbe 57 rue Cuvier F-75231 Paris Cedex 05 France Airbus Helicopters Abroport de Marseille Provence Marignane 13700 France

In bio-inspired design activities, nature is a basis of knowledge. Over the last twenty years, many solutions to measure and analyze human or animal gaits have been developed （VICON system, X-ray radiography...）. Although, these methods are becoming more and more accurate, they are quite expensive, long to set up and not easily portable. In this paper, a method called the bio-inspired topological skeleton is proposed in order to complement the classic videography process and to enable animal gait analysis. A new predictive kinematic model with closed-loops of an unguligrade quadruped is suggested. This kinematic model includes three segments per leg and takes into account the scapula movements. The proposed method allows us to improve the accuracy of the kinematic input data measured from a single video including an additional artefact. To show the benefits of this method, joint parameters that are difficult to measure are derived symbolically from the kinematic model and compared with experimental data.

关键词： quadruped locomotion motion analysis topological skeleton closed-form solutions Multibody Systems (MBS)

Insight on Viscoelasticiy of Ti16.7Zr16.7Hf16.7Cu16.7Ni16.7Be16.7 High Entropy Bulk Metallic Glass

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Journal of Iron and Steel Research International 2016年第1期23卷 19-23页

作者： Ji-chao QIAO jean-marc PELLETIER Ning LI Yao YAO School of Mechanics Civil Engineering and ArchitectureNorthwestern Polytechnical UniversityXi'an 710072ShaanxiChina Universite de Lyon MATEISUMR CNRS5510Bat.B.PascalINSA-LyonF-69621 Villeurbanne cedexFrance Department of Materials Science and Engineering State Key Laboratory for Materials Processing and Die&Mould TechnologyHuazhong University of Science and TechnologyWuhan 430074HuheiChina

High entropy bulk metallic glasses show promising mechanical and physical *** mechanical properties of Ti_（16.7）Zr_（16.7）Hf_（16.7）Cu_（16.7）Ni_（16.7）Be_（16.7）high entropy bulk metallic glass were investigated by mechanical spectroscopy（or called dynamic mechanical analysis）.The main（α）relaxation was observed in the framework of the loss modulus G″,which is related to the dynamic glass transition behaviour for the glassy *** physical model point of view,dynamic mechanical properties of the Ti_（16.7）Zr_（16.7）Hf_（16.7）Cu_（16.7）Ni_（16.7）Be_（16.7）high entropy bulk metallic glass show good agreement compared with the quasi-point defects theory.

关键词： high entropy bulk metallic glass viscoelasticity mechanical spectroscopy quasi-point defect theory

Sinistroposition of the gallbladder and common bile duct

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Hepatobiliary & Pancreatic Diseases International 2005年第2期4卷 313-315页

作者： jean-marc Regimbeau Department of Hepatobiliary Surgery Beaujon Hospital University of Paris Paris France

BACKGROUND: Despite its rare incidence, few cases of left-side gallbladder have been already published. METHODS: We reported herein the case of a 29-year-old man with a left liver tumor in whom left lateral bisegmen- tectomy was mandatory. It represents the first description of a sinistroposition of both gallbladder and common bile duct. RESULTS: Surgical exploration revealed a left-side gall- bladder , located under the left lobe of the liver. During he- patic parenchyma dissection at the left side of the round liga- ment and the Rex recessus, the common bile duct was in- jured. Complete separation of hepatic pedicle structures showed that the upper biliary convergence passed on the left side of the Rex recessus before reaching the hepatoduo- denal ligament. CONCLUSION: Only careful dissection of the hepatoduo- denal ligament up to Rex recessus level prior to liver paren- chyma resection could avoid biliary tract injury during left lobectomy.

关键词： intrahepatic lymphoma primary liver lymphoma hepatobiliary anatomy left-side gallbladder common bile duct surgery

Carbon dioxide for gut distension during digestive endoscopy:Technique and practice survey

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World Journal of Gastroenterology 2009年第12期15卷 1475-1479页

作者： Filip Janssens Jacques Deviere Pierre Eisendrath jean-marc Dumonceau Division of Gastroenterology and Hepatology Geneva University HospitalsMicheli-du-Crest street241205 GenevaSwitzerland Medical-Surgical Department of Gastroenterology and Hepato-Pancreatology Erasme HospitalUniversité Libre de BruxellesBrusselsBelgium

AIM:To assess the adoption of Carbon dioxide(CO2)insufflation by endoscopists from various European countries,and its ***:A survey was distributed to 580 endoscopists attending a live course on digestive ***:The response rate was 24.5%.Fewer than half the respondents(66/142,46.5%)were aware of the fact that room air can be replaced by CO2 for gut distension during endoscopy,and 4.2%of respondents were actually using CO2 as the insufflation *** aware of the possibility of CO2 insufflation mentioned technical difficulties in implementing the system and the absence of significant advantages of CO2 in comparison with room air as barriers to adoption in daily practice(84%and 49%of answers,respectively;two answers were permitted for this item).CONCLUSION:Based on this survey,adoption of CO2 insufflation during endoscopy seems to remain relatively exceptional.A majority of endoscopists were not aware of this possibility,while others were not aware of recent technical developments that facilitate CO2 implementation in an endoscopy suite.

关键词： Colonoscopy Practice survey Carbondioxide Digestive endoscopy

Medical treatment for sphincter of oddi dysfunction:Can it replace endoscopic sphincterotomy?

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World Journal of Gastroenterology 2012年第14期18卷 1610-1615页

作者： Véronique Vitton Salah Ezzedine jean-Michel Gonzalez Mohamed Gasmi jean-Charles Grimaud marc Barthet Department of Gastroenterology Hôpital NordChemin des Bourrely13915 Marseille cedex 20France

AIM:To report the results of a medical management of sphincter of oddi dysfunction(SOD) after an intermediate follow-up ***:A total of 59 patients with SOD(2 men and 57 women,mean age 51 years old) were included in this prospective *** medical treatment for one year,the patients were clinically re-evaluated after an average period of 30 ***:The distribution of the patients according to the Milwaukee's classification was the following:11 patients were type 1,34 were type 2 and 14 were type *** patients underwent an endoscopic sphincterotomy(ES) after one year of medical *** median intermediate follow-up period was 29.8 ± 3 mo(3-72 mo).The initial effectiveness of the medical treatment was complete,partial and poor among 50.8%,13.5% and 35%,respectively,of the *** the end of the follow-up period,37 patients(62.7%) showed more than 50% *** rate of improvement in patients who required ES was not significantly different compared with the patients treated conservatively(64.2% vs 62.2%,respectively).CONCLUSION:Our study confirms that conservative medical treatment could be an alternative to endoscopic sphincterotomy because,after an intermediate follow-up period,the two treatments show the same success rates.

关键词： Sphincter of oddi dysfunction Cholecystectomy Endoscopic sphincterotomy Biliary scintigraphy

Effect of physical aging and cyclic loading on power-law creep of high-entropy metallic glass

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Journal of Materials Science & Technology 2022年第20期115卷 1-9页

作者： Langting Zhang Yajuan Duan Eloi Pineda Hidemi Kato jean-marc Pelletier Jichao Qiao School of Mechanics Civil Engineering and ArchitectureNorthwestern Polytechnical UniversityXi’an 710072China Department of Physics Institute of Energy TechnologiesUniversitat Politècnica de Catalunya-BarcelonaTechBarcelona 08019Spain Institute for Materials Research Tohoku UniversitySendai 980-8577Japan Universitéde Lyon MATEISUMR CNRS5510Bat.B.PascalINSA-LyonVilleurbanne Cedex F-69621France Innovation Center NPU-ChongqingChongqing 401135China

The power-law relationship between creep rate decay and time is one of the intrinsic characteristics of metallic *** the current work,a La_(30)Ce_(30)Ni_(10)Al_(20)Co_(10) high-entropy metallic glass was selected as the model alloy to test the influences of physical aging and cyclic loading on the power-law creep mechanism,which was probed by the dynamic mechanical analysis in terms of the stochastic activation,and contiguous interplay and permeation of shear transformation *** is demonstrated that a notable discrepancy appears between thermal treatment and mechanical treatment on the power-law creep mechanism of this high-entropy metallic *** the one hand,physical aging below the glass transition temperature introduces the annihilation of potential shear transformation zones which contribute to *** the other hand,cyclic loading can tailor the“forward”jump operations competing with the“backward”ones of shear transformation zones by controlling the interval time(recovery time).The current research offers a new pathway towards understanding the creep mechanism of high-entropy metallic glasses.

关键词： High-entropy metallic glass Physical aging Cyclic loading Power-law creep Shear transformation zone

Relationship of preoperative androgen levels and metabolic syndrome with quality of life and erectile function in patients who are to undergo radical prostatectomy

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Asian Journal of Andrology 2021年第5期23卷 520-526页

作者： Yann Neuzillet jean-Francois Dreyfus jean-Pierre Raynaud Mathieu Rouanne marc Schneider Morgan Roupret Sarah Drouin marc Galiano Xavier Cathelineau Thierry Lebret Henry Botto Department of Urology Hopital FochUVSQ-Paris-Saclay UniversitySuresnes 92150France Department of Clinical Research and Innovation Hopital FochUVSQ-Paris-Saclay UniversitySuresnes 92150France Sorbonne University Paris 75013France Department of Urology Hfipital Louis PasteurColmar 68000France Department of Urology Hopital Pitie-SalpetriereAssistance Publique-Hopitaux de ParisSorbonne UniversityParis 75013France Department of Urology Institut Mutualiste MontsourisParis-Descartes UniversityParis 75014France

This study aims to investigate whether clinical and biological preoperative characteristics of patients who were to undergo radical prostatectomy were associated with impairment in patient-reported quality of life(QoL)and erectile dysfunction immediately before *** evaluated patient-reported outcomes among 1019 patients(out of 1343)of the AndroCan study,willing to score the Aging Male Symptom(AMS)and the International Index of Erectile Function 5-item(IIEF-5)*** linear regression and robust multiple regression were used to ascertain the relationship between demographic,clinical,and hormonal parameters and global AMS or IIEF-5 *** a result,most patients(85.1%)of the Androcan cohort agreed to complete *** higher IIEF-5 global scores were found in non-Caucasian and obese patients,with larger waist circumference,metabolic syndrome,diabetes mellitus,cardiovascular disease,hypertension,high blood sugar,concomitant medications,and hypogonadism,while the AMS global score was significantly higher in patients with larger waist circumference,metabolic syndrome,high blood pressure,raised glycemia,and concomitant *** IIEF-5 global score was correlated to age,dehydroepiandrosterone(DHEA),fat mass percentage,and androstenediol(D5).The AMS global score was significantly correlated to DHEA,D5,and DHEA ***,the multivariate models showed that QoL and erectile function were significantly affected,before surgery,by symptoms and signs that are usually considered as pertaining to the metabolic syndrome,while sexual hormones are essentially correlated to erectile dysfunction.

关键词： erectile function metabolic syndrome quality of life radical prostatectomy testosterone