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世界核心医学期刊文摘（神经病学分册） 2005年第8期1卷 17-18页

作者： Hagemans M.L.C. Winkel L.P.F. van Doorn P.A. a.t. van der ploeg 郭俊 Erasmus MC-Sophia Dr Molewaterplein 60 3015 GJ Rotterdam Netherlands Dr.

Late-onset Pompe’s disease (acid ma.t.se deficiency, glycogen storage diseas e type II) is a slowly progressive myopathy caused by deficiency of acid α-glu cosidase. Current developments in enzyme replacement therapy require detailed kn owledge of the kind and severity of symptoms and the natural course of the disea se in the patient population.A detailed questionnaire covering the patients’med ical history and current situation was developed and information was gathered fr om 54 Dutch patients. the mean age of the pa.t.cipa.t. was 48.6 ±15.6 *** first complaints sta.t.d at a mean age of 28.1±14.3 years and were mostly rela.t.d to mobility problems and limb-girdle weakness. Fifty-eight percent of the adult patients indicated the presence of mild muscular symptoms during childhood . twenty-eight percent of the patients wa.t.d > 5 years for the final diagnosis a.t.r the first visit to a physician for disease related complaints. At the tim e of questionnaire completion,48%of the study population used a wheelchair and 37%used a.t.ficial *** such as rising from an armchair,taking stairs or getting upright a.t.r bending over were difficult or impossible for m ore than two-thirds of the *** age at onset, the rate of disease pr ogression and the sequence of respiratory and skeletal muscle involvement varied substa.t.ally between patients. Seventy-six percent of the pa.t.cipa.t. indica.t.d being troubled by fatigue and 46%by pain. this survey has mapped the age at onset, presenting symptoms, heterogeneity in progression and range of disease s everity in a large group of Dutch patients. We conclude that early manifestation s in childhood require proper a.t.ntion to prevent unnecessary delay of the diag nosis. the follow-up of patients with late-onset Pompe’s disease should focus on respiratory and limb-girdle muscle function, the capacity to perform daily a.t.vities, and the presentation of fatigue and pain.

关键词： Pompe 酸性麦芽糖酶糖原贮积病问卷反馈 α-葡萄糖苷酶发病年龄肌病首次就诊缺乏症疼痛情况

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庞贝病成年和儿童患者的病情与年龄和病程相关

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世界核心医学期刊文摘（神经病学分册） 2005年第10期1卷 57-57页

作者： Hagemans M.L.C. Winkel L.P.F. Hop W.C.J. a.t. van der ploeg 张琪 Erasmus MC-Sophia Dr. Molewaterplein 60 3015 GJ Rotterdam Netherlands Dr.

Information about 255 children and adults with Pompe disease was gathered thr ough a questionnaire. Disease severity was associated with disease duration and not with age; an early manifestation of the disease implied earlier wheelchair o r ventilator dependency. the patient group under age 15 included a subgroup with a more severe and rapid course of the disease. they require more intensive foll ow- up and early intervention, before irreversible damage has occurred.

关键词：庞贝亚组呼吸机依赖问卷调查

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the future of computer-aided sperm analysis

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Asian Journal of Andrology 2015年第4期17卷 545-553页

作者： Sharon t Mortimer Gerhard van der Horst David Mortimer Oozoa Biomedical West Vancouver BC Canada Depa.t.ent of Medical Biosciences University ofthe Western Cape Bellville South Africa 0ozoaBiomedical West Vancouver BC Canada.

Computer-aided sperm analysis （CASA） technology was developed in the late 1980s for analyzing sperm movement chara.t.ristics or kinematics and has been highly successful in enabling this field of research. CASA has also been used with great success for measuring semen chara.t.ristics such as sperm concentration and proportions of progressive motility in many animal species, including wide application in domesticated animal production laboratories and reproductive toxicology. However, a.t.mpts to use CASA for human clinical semen analysis have largely met with poor success due to the inherent difficulties presented by many human semen samples caused by sperm clumping and heavy background debris that, until now, have precluded accurate digital image analysis. the a.t.ors review the improved capabilities of two modern CASA platforms （Hamilton thorne CASA-II and Microptic SCA6） and consider their current and future applications with pa.t.cular reference to directing our focus towards using this technology to assess functional rather than simple descriptive chara.t.ristics of spermatozoa. Specific requirements for validating CASa.t.chnology as a semi-a.t.mated system for human semen analysis are also provided, with pa.t.cular reference to the accuracy and uncertainty of measurement expected of a robust medical laboratory test for implementation in clinical laboratories operating according to modern accreditation standards.

关键词： HIStORICAL INtRODUCtION

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Implementation and assessment of diverse strategies for physical a.t.vity promotion in Vietnam--A case report

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Journal of Sport and Hea.t. Science 2018年第1期7卷 42-49页

作者： Gabriella Beckvid-Henriksson thu Hoai Nguyen Julia Kilhed Agnes Nordstrom Sofie Svensson thi thanh Huong tran Ingeborg van der ploeg Carl Johan Sundberg Depa.t.ent of Physiology & Pharmacology Karolinska Instituter Stockholm 17177 Sweden Depa.t.ent of Neurobiology Care Sciences' and Society Division of Physiotherapy Karolinska Institutet Huddinge 14183 Sweden National Geriatric Hospital Ha Noi 100000 Vietnam depa.t.ent of Ethics & Social Medicine Hanoi Medical University Ha Noi 100000 Vietnam Unit for Bioentrepreneurship Department of Learning Informatics Management and Ethics Karolinska Instituter Stockholm 17177 Sweden

Background: Regular physical a.t.vity(PA) has documented effects in prevention and treatment of many non-communicable diseases(NCDs).Physical ina.t.vity is recognized as the fourth leading risk fa.t.r for premature death, worldwide. Despite these fa.t., physical ina.t.vity is increasing,not only in high-income, but also in middle-and low-income countries. to address this negative trend, evidence-based methods to increase PA are needed. the purpose of this paper is to describe the implementation and assessment of 4 strategies designed to increase PA in ***: Four strategies were used: i) introduction and evaluation of an education and training program on the Swedish method of Physical a.t.vity on Prescription(PAP) among hea.t. care professionals, ii) translation of the PAP evidence-based handbook, Physical a.t.vity in the Prevention and treatment of Disease(called FYSS in Swedish) into Vietnamese, iii) launch of a mass-media campaign to promote PA, and iv)advocacy to support development of PA guidelines in ***: the evaluation indicated that the pa.t.cipating hea.t. care professionals had a positive a.t.tude to PAP. However, they also reported uncertainty in prescribing PA. FYSS was translated and disseminated successfully to hea.t. care professionals. A mass-media campaign identified the beneficial effects of Pa.t. hea.t. care professionals, journalists, policy makers, and the public. La.t. the process of developing national guidelines on PA was ***: this project led to enhanced awareness and appreciation of PA in the prevention and treatment of NCDs among hea.t. care professionals as well as initiation of national PA guidelines. Importa.t.lessons also were learned in the presentation of PAP, which will be considered when designing similar projects in the future.

关键词： Exercise prescription Guidelines and recommendations Hea.t. promotion Lower middle-income country Non-communicable diseases Physicala.t.vity

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Emerging polluta.t. in the environment:A challenge for water resource management

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International Soil and Water Conservation Research 2015年第1期3卷 57-65页

作者： Violette Geissen Hans Mol Erwin Klumpp Günter Umlauf Ma.t. Nadal Ma.t.ne van der ploeg Sjoerd E.a.t.M.van de Zee Coen J.Ritsema Soil Physics and Land Management Group Wageningen University6708PB WageningenThe Netherlands Rikilt WageningenThe Netherlands Agrosphere Forschungszentrum JülichJülichGermany Joint Research Center IspraItaly Laboratory of toxicology and Environmental Hea.t. IISPVUniversitat Rovira i VirgiliReusSpain

A significa.t.number of emerging polluta.t.(EPs)resulting from point and diffuse pollution is present in the aquatic *** are chemicals that are not commonly monitored but have the potential to enter the environment and cause adverse ecological and human hea.t. *** to the NORMAN network,at lea.t.700 substances categorized into 20 classes,have been identified in the European aquatic *** light of their potential impa.t.a.t.on is urgently *** this study,we present a concept that shows the current state of a.t.and challenges for monitoring programs,fate and risk assessment tools and requirements for policies with respect to emerging polluta.t. as a base for sustainable water resource ***,methods for sampling and analysis are not harmonized,being typically focused on certain EP *** a number of known highly hazardous EPs detection limits are too high to allow proper risk *** other EPs such as micropla.t.cs method development is in its *** ultra-sensitive instrumental techniques should be used for qua.t.tative determination of prioritized EPs in water,suspended ma.t.r,soil and *** on EPs'and their metabolites'properties that determine their fate in the environment are often not *** surveys on water quality often use different parameters for water quality assessment and often do not include EPs.A harmonized monitoring of surface and groundwater is not yet achieved and urgently *** component integrated into models assessing the fate of EPs in a multi compa.t.ent environmental approach are missing and must be *** main goal of risk assessment is the overall protection of ecological communities in the aquatic environment and human *** methods for assessing the cumulative risks from combined exposures to several stressors,including mixtures of EPs in a multi-scale approach are required.A combination of regulations and management meas

关键词： Emerging polluta.t. Water resource management Monitoring Risk assessment Water policies

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Cost projections for microwave plasma CO production using renewable energy

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Journal of Energy Chemistry 2022年第8期31卷 507-513,I0014页

作者： Remko J.Detz Bob van der Zwaan Netherlands Organisation for Applied Scientific Research(tNO) Amsterdam 1043 NTThe Netherlands Faculty of Science(HIMS and IAS) University of AmsterdamAmsterdam 1090 GDThe Netherlands School of Advanced International Studies(SAIS) Johns Hopkins UniversityBologna 40126Italy

Successful deployment of renewable fuel production requires substa.t.al cost reduction along the entire value chain of the underlying manufa.t.ring *** improve their performance,renewable fuel production technologies should follow a cost-reducing learning *** this a.t.cle,we adopt recent evidence that learning-by-doing is directly influenced by the technology unit size and explore three scenarios for microwave plasma CO_(2)conversion in which the learning rate varies between 10%,15%,and 20%.Our projections reveal that the total investments required to deploy this CO_(2)conversion technology at an exajoule scale decline from 83 down to 23 billion euros under a 10%increase in the value of the learning *** CO_(2) production costs in 2050 amount to 247–346€(2019)/t CO_(2),in which the range is determined by the value of the learning *** under substa.t.al learning until 2050 the levelized CO production cost is unlikely to become competitive with conventional natural gas-based CO_(2) production processes,except when a CO_(2)tax is applied of up to 150€(2019)/t CO_(2).to optimally exploit effects of learning-by-doing,we recommend developing several CO production technologies simultaneously with multiple unit sizes,so as to improve the chance of ultimately selecting the process with the highest learning rate.

关键词： CO_(2)utilization Synthetic fuels Renewable chemicals Learning curves Unit size

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Modeling the effects of sa.t.concentration on aqueous and organic electrolytes

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npj Computational Materials 2023年第1期9卷 546-552页

作者： Stephanie C.C.van der Lubbe Pieremanuele Canepa Depa.t.ent of Materials Science and Engineering National University of Singapore9 Engineering Drive 1117575 SingaporeSingapore Depa.t.ent of Chemical and Biomolecular Engineering National University of Singapore4 Engineering Drive 4117585 SingaporeSingapore Depa.t.ent of Electrical and Computer Engineering and texas Center for Superconductivity University of HoustonHoustonTX 77204USA

Understanding the thermodynamic properties of electrolyte solutions is of vital importance for a myriad of physiological and technological *** mean a.t.vity coefficientγ±is associated with the deviation of an electrolyte solution from its ideal behavior and may be obtained by combining the Debye-Hückel(DH)and Born(B)***,the DH and B equations depend on the concentration and temperature-dependent static permittivity of the solutionεr(c,t)and the size of the solvated ions ri,whose experimental data is often not ***,we use a combination of molecular dynamics and density functional theory to predictεr(c,t)and ri,which enables us to apply the DH and B equations to any technologically releva.t.aqueous and nonaqueous electrolyte at any concentration and temperature of interest.

关键词： equations electrolyte apply

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Identifying prognostic fa.t.rs for conservative treatment outcomes in servicemen with chronic exertional compa.t.ent syndrome treated at a rehabilitation center

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Military Medical Research 2018年第3期5卷 199-206页

作者： Mariette Z.Meulekamp Peter van der Wurff Alfred van der Meer Cees Lucas Research and Development Military Rehabilitation CentreAardenburgDoomthe Netherlands Depa.t.ent of Physiotherapy HU University of Applied SciencesUtrechtthe Netherlands Depa.t.ent of Clinical Epidemiology Biostatistics and BioinformaticsAcademic Medical CentreUniversity of AmsterdamAmsterdamthe Netherlands

Background: Chronic exertional compa.t.ent syndrome(CECS) is a condition of pain induced by exercise, and it is chara.t.rized by muscle swelling and impaired muscle function in the lower leg. Given the diversity in the diagnosis and treatment of CECS, it is desirable to determine variables pertaining to prognosis and recovery. the purpose of this study is to identify prognostic fa.t.rs for conservative treatment outcomes in servicemen with CECS who were treated at a Military Rehabilitation ***: Patients from all military services were referred from the special unit for lower leg pain at the Central Military Hospital, Utrecht, the Netherlands. Descriptive analysis was used to report the chara.t.ristics of the pa.t.cipa.t. and their baseline measurements. Group differences were analyzed using a Student's t-test or MannWhitney U test, according to the normality of the data distribution. Differences between the pre-and postintervention outcomes were evaluated using the Wilcoxon signed rank test. to evaluate the magnitude of prognostic fa.t.rs, a univariate logistic regression analysis was performed. the prognostic fa.t.rs included age, body mass index, body fat percentage, self-efficacy beliefs, foot malalignment, intramuscular pressure, other comorbidities, protein and creatine use, smoking, alcohol use, complaint duration, physical demands, and duration of military ***: a.t.r the rehabilitation period, we observed 25 patients with a successful outcome, which was defined as a reduction in pain(≥2 points) during the capacity test measured using a verbal rating scale and 20 patients with an unsuccessful outcome. Fa.t.rs demonstrating a limited increased odds ratio for an unsuccessful outcome included smoking, alcohol use, intramuscular pressure, a complaint duration of more than 6 months, and physical demands of service. However, these fa.t.rs did not reach ***: this study did not identify any prognostic fa.t.rs that predict the

关键词： Lower leg pain Observational study Rehabilitation

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Dispersal of invasive species by drifting

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Current Zoology 2011年第6期57卷 818-827页

作者： M.C. van RIEL G. van der VELDE A. BIJ DE Va.t. Radboud University Nijmegen Institute for Water and Wetland Research Department of Animal Ecology and Ecophysiology Section Aquatic Animal Ecology Heyendaalseweg 135 6525 AJ Nijmegen The Netherlands team Freshwater Ecology DLO-Alterra P.O. Box 47 6700 AA Wageningen The Netherlands Netherlands Centre for Biodiversity Naturalis EO. Box 9517 2300 RA Leiden The Netherlands Waterfauna Hydrobiologisch Adviesbureau Oostrandpark 30 8212 AP Lelystad The Netherlands

Drifting can be an effective way for aquatic organisms to disperse and colonise new areas. Increasing connectivity between European large rivers facilitates invasion by drifting aquatic macroinvertebrates. the present study shows that high abundances of invasive species drift in the headstream of the river Rhine. Dikerogammarus villosus and Chelicorophium cur- vispinum represented up to 90% of the total of drifting macroinvertebrates. Drift a.t.vity shows seasonal and diel pa.t.rns. Most species sta.t.d drifting in spring and were most abunda.t.in the water column during the summer period. Drift a.t.vity was very low during the winter period. Diel pa.t.rns were apparent; most species, including D. villosus, drifted during the night. Drifting macroinvertebrates colonised stony substrate directly from the water column. D. villosus generally colonised the substrate at night, while higher numbers of C. curvispinum colonised the substrate during the day. It is very likely that drifting functions as a disper- sal mechanism for crustacean invaders. Once waterways are connected, these species are no longer necessarily dependent on dispersal vectors other than drift for extending their distribution range

关键词： Amphipods Invaders Colonization Drift Daily rhythms Seasonal variation

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Injectable mechanical pillows for a.t.nuation of load-induced post-traumatic osteoa.t.ritis

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Regenerative Biomaterials 2019年第4期6卷 211-219页

作者： derek t.Holyoak tibra A.Wheeler Marjolein C.H.van der Meulen Ankur Singh Meinig School of Biomedical Engineering Cornell UniversityIthacaNY 14853USA Sibley School of Mechanical and Aerospace Engineering Cornell UniversityIthacaNY 14853USA Research Division Hospital for Special SurgeryNew YorkNY 10021USA Englander Institute for Precision Medicine Weill Cornell Medical CollegeCornell UniversityNew YorkNY 10021USA

Osteoa.t.ritis(OA)of the knee joint is a degenerative disease initiated by mechanical stress that affects millions of *** disease manifests as joint damage and synovial ***-traumatic osteoa.t.ritis(PtOA)is a specific form of OA caused by mechanical trauma.t. the *** progression of PtOA is prevented by immediate post-injury therapeutic ***-a.t.cular injection of a.t.-inflammatory therapeutics(***)is a common treatment option for OA before end-stage surgical ***,the efficacy of intra-a.t.cular injection is limited due to poor drug retention time in the joint space and the variable efficacy of ***,we endeavored to chara.t.rize a four-arm maleimide-functionalized polyethylene glycol(PEG-4MAL)hydrogel system as a‘mechanical pillow’to cushion the load-bearing joint,withstand repetitive loading and improve the efficacy of intra-a.t.cular injections of nanopa.t.cles containing dexamethasone,an a.t.-inflammatory ***-4MAL hydrogels maintained their mechanical properties a.t.r physiologically releva.t.cyclic compression and released therapeutic payload in an on-demand manner under in vitro inflammatory ***,the on-demand hydrogels did not release nanopa.t.cles under repetitive mechanical loading as experienced by daily *** dexamethasone had minimal protective effects on OA-like pathology in our studies,the PEG-4MAL hydrogel functioned as a mechanical pillow to protect the knee joint from ca.t.lage degradation and inhibit osteophyte formation in an in vivo load-induced OA mouse model.

关键词： hydrogel osteophyte biomaterials drug delivery ca.t.lage inflammation

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