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Sample records for international collaborative gaucher

  1. Transformation in pretreatment manifestations of Gaucher disease type 1 during two decades of alglucerase/imiglucerase enzyme replacement therapy in the International Collaborative Gaucher Group (ICGG) Gaucher Registry.

    Science.gov (United States)

    Mistry, Pramod K; Batista, Julie L; Andersson, Hans C; Balwani, Manisha; Burrow, Thomas Andrew; Charrow, Joel; Kaplan, Paige; Khan, Aneal; Kishnani, Priya S; Kolodny, Edwin H; Rosenbloom, Barry; Scott, C Ronald; Weinreb, Neal

    2017-09-01

    This study tests the hypothesis that the prevalence of severe clinical manifestations in Gaucher disease type 1 (GD1) patients at the time of treatment initiation has changed since alglucerase/imiglucerase enzyme replacement therapy (ERT) was approved in the United States (US) in 1991. US alglucerase/imiglucerase-treated GD1 patients from the International Collaborative Gaucher Group Gaucher Registry clinicaltrials.gov NCT00358943 were stratified by age at ERT initiation (<18, 18 to <50, ≥50 years), era of ERT initiation (1991-1995, 1996-2000, 2001-2005, 2006-2009), and splenectomy status pre-ERT. Prevalence of splenectomy decreased dramatically across the eras among all age groups. Bone manifestations were more prevalent in splenectomized patients than non-splenectomized patients in all age groups. Prevalence of bone manifestations differed across eras in certain age groups: non-splenectomized patients had a lower prevalence of ischemic bone events (pediatric patients) and bone crisis (pediatric patients and adults 18 to <50 years) in later eras; splenectomized adult (18 to <50 years) patients had a lower prevalence of ischemic bone events and bone crisis in later eras. Over two decades after the introduction of ERT, the prevalence of splenectomy and associated skeletal complications has declined dramatically. Concomitantly, the interval between diagnosis and initiation of ERT has decreased, most strikingly in pediatric patients who have the most severe disease. Together, these findings suggest that since the introduction of alglucerase/imiglucerase ERT, optimal standard of care has become established in the US to prevent destructive complications of GD1. © 2017 The Authors American Journal of Hematology Published by Wiley Periodicals, Inc.

  2. Clinical response to eliglustat in treatment-naïve patients with Gaucher disease type 1: Post-hoc comparison to imiglucerase-treated patients enrolled in the International Collaborative Gaucher Group Gaucher Registry

    Directory of Open Access Journals (Sweden)

    Jennifer Ibrahim

    2016-09-01

    Full Text Available Eliglustat is a recently approved oral therapy in the United States and Europe for adults with Gaucher disease type 1 who are CYP2D6 extensive, intermediate, or poor metabolizers (>90% of patients that has been shown to decrease spleen and liver volume and increase hemoglobin concentrations and platelet counts in untreated adults with Gaucher disease type 1 and maintain these parameters in patients previously stabilized on enzyme replacement therapy. In a post-hoc analysis, we compared the results of eliglustat treatment in treatment-naïve patients in two clinical studies with the results of imiglucerase treatment among a cohort of treatment-naïve patients with comparable baseline hematologic and visceral parameters in the International Collaborative Gaucher Group Gaucher Registry. Organ volumes and hematologic parameters improved from baseline in both treatment groups, with a time course and degree of improvement in eliglustat-treated patients similar to imiglucerase-treated patients.

  3. Clinical response to eliglustat in treatment-naïve patients with Gaucher disease type 1: Post-hoc comparison to imiglucerase-treated patients enrolled in the International Collaborative Gaucher Group Gaucher Registry.

    Science.gov (United States)

    Ibrahim, Jennifer; Underhill, Lisa H; Taylor, John S; Angell, Jennifer; Peterschmitt, M Judith

    2016-09-01

    Eliglustat is a recently approved oral therapy in the United States and Europe for adults with Gaucher disease type 1 who are CYP2D6 extensive, intermediate, or poor metabolizers (> 90% of patients) that has been shown to decrease spleen and liver volume and increase hemoglobin concentrations and platelet counts in untreated adults with Gaucher disease type 1 and maintain these parameters in patients previously stabilized on enzyme replacement therapy. In a post-hoc analysis, we compared the results of eliglustat treatment in treatment-naïve patients in two clinical studies with the results of imiglucerase treatment among a cohort of treatment-naïve patients with comparable baseline hematologic and visceral parameters in the International Collaborative Gaucher Group Gaucher Registry. Organ volumes and hematologic parameters improved from baseline in both treatment groups, with a time course and degree of improvement in eliglustat-treated patients similar to imiglucerase-treated patients.

  4. Long-term hematological, visceral, and growth outcomes in children with Gaucher disease type 3 treated with imiglucerase in the International Collaborative Gaucher Group Gaucher Registry.

    Science.gov (United States)

    El-Beshlawy, Amal; Tylki-Szymanska, Anna; Vellodi, Ashok; Belmatoug, Nadia; Grabowski, Gregory A; Kolodny, Edwin H; Batista, Julie L; Cox, Gerald F; Mistry, Pramod K

    In Gaucher disease (GD), deficiency of lysosomal acid β-glucosidase results in a broad phenotypic spectrum that is classified into three types based on the absence (type 1 [GD1]) or presence and severity of primary central nervous system involvement (type 2 [GD2], the fulminant neuronopathic form, and type 3 [GD3], the milder chronic neuronopathic form). Enzyme replacement therapy (ERT) with imiglucerase ameliorates and prevents hematological and visceral manifestations in GD1, but data in GD3 are limited to small, single-center series. The effects of imiglucerase ERT on hematological, visceral and growth outcomes (note: ERT is not expected to directly impact neurologic outcomes) were evaluated during the first 5years of treatment in 253 children and adolescents (Gaucher Group (ICGG) Gaucher Registry. The vast majority of GBA mutations in this diverse global population consisted of only 2 mutations: L444P (77%) and D409H (7%). At baseline, GD3 patients exhibited early onset of severe hematological and visceral disease and growth failure. During the first year of imiglucerase treatment, hemoglobin levels and platelet counts increased and liver and spleen volumes decreased, leading to marked decreases in the number of patients with moderate or severe anemia, thrombocytopenia, and hepatosplenomegaly. These improvements were maintained through Year 5. There was also acceleration in linear growth as evidenced by increasing height Z-scores. Despite devastating disease at baseline, the probability of surviving for at least 5years after starting imiglucerase was 92%. In this large, multinational cohort of pediatric GD3 patients, imiglucerase ERT provided a life-saving and life-prolonging benefit for patients with GD3, suggesting that, with proper treatment, many such severely affected patients can lead productive lives and contribute to society. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

  5. Long-term clinical outcomes in type 1 Gaucher disease following 10 years of imiglucerase treatment

    NARCIS (Netherlands)

    Weinreb, Neal J.; Goldblatt, Jack; Villalobos, Jacobo; Charrow, Joel; Cole, J. Alexander; Kerstenetzky, Marcelo; vom Dahl, Stephan; Hollak, Carla

    2013-01-01

    We studied the effect of long-term alglucerase/imiglucerase (Ceredase®/Cerezyme®, Genzyme, a Sanofi company, Cambridge, MA, USA) treatment on hematological, visceral, and bone manifestations of Gaucher disease type 1 (GD1). The International Collaborative Gaucher Group (ICGG) Gaucher Registry

  6. Gaucher disease types 1 and 3: Phenotypic characterization of large populations from the ICGG Gaucher Registry.

    Science.gov (United States)

    Grabowski, Gregory A; Zimran, Ari; Ida, Hiroyuki

    2015-07-01

    Study of the natural history of Gaucher disease has revealed marked phenotypic variation. Correlations to genotypes could provide insight into individual susceptibility to varying disease severity, which may impact whole-life medical care, reproductive decisions, and therapeutic choices for affected families. Importantly, pre-symptomatic or prospective interventions or the use of therapies with significant risk require accurate risk-benefit analyses based on the prognosis for individual patients. The body of international data held within the International Collaborative Gaucher Group (ICGG) Gaucher Registry provides an unprecedented opportunity to characterize the phenotypes of Gaucher disease types 1 and 3 and to appreciate demographic and ethnic factors that may influence phenotypes. The diversity of GBA gene mutations from patients with Gaucher disease represented in the ICGG Gaucher Registry database and in the literature provides the basis for initial genotype/phenotype correlations, the outcomes of which are summarized here. © 2015 Wiley Periodicals, Inc.

  7. Understanding the natural history of Gaucher disease.

    Science.gov (United States)

    Mistry, Pramod K; Belmatoug, Nadia; vom Dahl, Stephan; Giugliani, Roberto

    2015-07-01

    Gaucher disease is a rare and extraordinarily heterogeneous inborn error of metabolism that exhibits diverse manifestations, a broad range of age of onset of symptoms, and a wide clinical spectrum of disease severity, from lethal disease during infancy to first age of onset of symptoms in octogenarians. Before the advent of the International Collaborative Gaucher Group (ICGG) Gaucher Registry, the understanding of the natural history and phenotypic range of Gaucher disease was based on isolated case reports and small case series. Limited data hindered understanding of the full spectrum of the disease leading to some early misconceptions about Gaucher disease, notably, that nonneuronopathic (type 1) disease was a disease of adults only. The global scope of the ICGG Gaucher Registry, with its vast body of longitudinal data, has enabled a real appreciation of both the phenotypic spectrum of Gaucher disease and its natural history. This body of evidence represents the foundation for accurate assessment of the response to specific therapies for Gaucher disease and to the development of standard-of-care to monitor disease activity. Here, we outline the key developments in delineating the natural history of this highly complex disease and role of the ICGG Gaucher Registry in this effort. © 2015 Wiley Periodicals, Inc.

  8. Gaucher disease and comorbidities: B-cell malignancy and parkinsonism.

    Science.gov (United States)

    Cox, Timothy M; Rosenbloom, Barry E; Barker, Roger A

    2015-07-01

    Data emerging from the International Collaborative Gaucher Group (ICGG) Gaucher Registry together with other contemporary clinical surveys have revealed a close association between Gaucher disease and non-Hodgkin's B-cell lymphoma and myeloma and Gaucher disease and Parkinson's disease. Several possible explanations for increased B-cell proliferation and neoplasia in Gaucher disease have been proposed, including the possible influence of sphingosine (derived from the extra lysosomal metabolism of glucosylceramide), gene modifiers, splenectomy and immune system deregulation induced by cytokines, chemokines, and hydrolases released from Gaucher cells. Parkinson's disease is frequently seen in the otherwise-healthy relatives of Gaucher disease patients leading to the finding that GBA mutations represent a genetic risk factor for Parkinson's disease. The mechanism of the association between GBA mutations and Parkinson's disease has yet to be elucidated but the pathogenesis appears distinct from that of Gaucher disease. Several pathogenic pathways have been proposed including lysosomal and/or mitochondrial dysfunction. The effect of Gaucher disease specific therapies on the incidence of cancer or Parkinson's disease are not clear and will likely be evaluated in future ICGG Gaucher Registry studies. © 2015 Wiley Periodicals, Inc.

  9. International collaboration

    International Nuclear Information System (INIS)

    Anon.

    1994-01-01

    In the wake of the demise of the US Superconducting Supercollider (SSC) project last year which empoverished both US and world science, some rapid scene shifting is going on. The SSC may be dead, but the underlying physics quest lives on. In the US, the 'future vision' subpanel of the High Energy Physics Advisory Board (HEPAP) is at work formulating its recommendations. On the international front, the International Committee for Future Accelerators (ICFA) at a special meeting in Vancouver in January drafted a statement

  10. Enfermedad de Gaucher en Latinoamérica: Un informe del Registro Internacional y del Grupo Latinoamericano para la Enfermedad de Gaucher Gaucher disease in Latin America: A report from the Gaucher Disease International Registry and the Latin Americam Group for Gaucher Disease

    Directory of Open Access Journals (Sweden)

    Guillermo Drelichman

    2012-08-01

    Full Text Available La enfermedad de Gaucher, por su escasa frecuencia, está incluida dentro de las llamadas enfermedades huérfanas. En 1991 se creó el Registro Internacional de Gaucher y en 1992 se incorporaron los primeros pacientes de Latinoamérica. En el año 2008 se creó el Grupo Latinoamericano para la Enfermedad de Gaucher (GLAEG cuyos principales objetivos son fomentar la realización de consensos regionales, difundir el ingreso de pacientes al registro internacional y aumentar el conocimiento sobre la enfermedad para lograr mejorar la atención y la calidad de vida de los pacientes. Hasta abril del 2010 ingresaron 5828 pacientes de todo el mundo, 911 (15.6% son de Latinoamérica. Este es el primer informe global de la enfermedad en la Región: hay un predominio del sexo femenino, la forma clínica m��s frecuente es el tipo I (95%; al diagnóstico la mayoría son Gaucher disease -due to its low frequency- is considered an orphan disease. In 1991 the International Gaucher Registry was created and in 1992 the first patients from Latin America were enrolled. In 2008 the Latin American Group for Gaucher Disease was initiated. Its main objectives are to promote regional consensus, to stimulate the enrolment of patients into the International Gaucher Registry and the enhancement of knowledge on this disease, and to achieve better care and quality of life of patients in our Region. Until April 2010, 5828 patients have been enrolled all around the world, 911 (15.6% from Latin America. This is the first comprehensive report of the disease in the Region. In our population there is a predominance of females, the most common clinical form is the type I (95% and the age at diagnosis is before 20 years in 68% of patients. The most frequent clinical manifestations at diagnosis are splenomegaly (96% and anemia (49%. Eighty percent of patients had radiographic findings of bone involvement. In our Region, the vast majority of patients (89% had received enzyme

  11. Characteristics of type I Gaucher disease associated with persistent thrombocytopenia after treatment with imiglucerase for 4-5 years

    NARCIS (Netherlands)

    Hollak, Carla E. M.; Belmatoug, Nadia; Cole, J. Alexander; vom Dahl, Stephan; Deegan, Patrick B.; Goldblatt, Jack; Rosenbloom, Barry; van Dussen, Laura; Tylki-Szymańska, Anna; Weinreb, Neal J.; Zimran, Ari; Cappellini, Maria Domenica

    2012-01-01

    The characteristics of Gaucher disease (GD) associated with persistent thrombocytopenia despite imiglucerase enzyme therapy in type 1 GD (GD1) were investigated by retrospective analysis of International Collaborative Gaucher Group (ICGG) Registry data. The study involved 1016 GD1 patients with an

  12. Gaucher Disease

    Science.gov (United States)

    Gaucher disease is a rare, inherited disorder. It is a type of lipid metabolism disorder. If you ... affected. It usually starts in childhood or adolescence. Gaucher disease has no cure. Treatment options for types ...

  13. Gaucher Disease

    Science.gov (United States)

    ... Affecetd children usually die before 2 years of age. In the third category, called type 3 (or chronic neuropathic Gaucher ... Affecetd children usually die before 2 years of age. In the third category, called type 3 (or chronic neuropathic Gaucher ...

  14. INTERNATIONAL COLLABORATION: Panelling

    International Nuclear Information System (INIS)

    Anon.

    1991-01-01

    At the meeting of the International Committee for Future Accelerators (ICFA), in Geneva in July, Chairman A.N. Skrinsky of Novosibirsk reviewed ICFA progress, particularly the activities of the specialist Panels which pursue specific Committee objectives in guiding worldwide collaboration in high energy physics

  15. INTERNATIONAL COLLABORATION: Panelling

    Energy Technology Data Exchange (ETDEWEB)

    Anon.

    1991-10-15

    At the meeting of the International Committee for Future Accelerators (ICFA), in Geneva in July, Chairman A.N. Skrinsky of Novosibirsk reviewed ICFA progress, particularly the activities of the specialist Panels which pursue specific Committee objectives in guiding worldwide collaboration in high energy physics.

  16. Gaucher disease

    Science.gov (United States)

    ... please enable JavaScript. Gaucher disease is a rare genetic disorder in which a person lacks an enzyme called glucocerebrosidase (GBA). Causes Gaucher disease is rare in the general population. People of Eastern and Central European (Ashkenazi) Jewish heritage are more likely to have this disease. It ...

  17. ICFA on international collaboration

    International Nuclear Information System (INIS)

    Anon.

    1993-01-01

    International collaboration in high energy physics is what ICFA - the International Committee for Future Accelerators - is all about. Progress is highlighted every three years when ICFA convenes its 'Future Perspectives in High Energy Physics' seminar to focus attention on major issues and to identify evolving trends. The latest such seminar, held at the DESY Laboratory in Hamburg from 3-7 May, looked at international cooperation in the construction of major facilities. Status reports across the whole range of existing experimental programmes and ongoing plans gave valuable pointers to future needs. For electron-positron linear colliders (EPLC), research and development work towards the next generation of machines is underway in Laboratories throughout the world. At previous such seminars, at Tsukuba, Japan (1984), Brookhaven, USA (1987) and Protvino (1990), ICFA, which has no direct power, could sometimes only stand on the sidelines and comment on the emergence of major new national plans. The lessons learnt, ICFA is keen to make sure that the EPLC debut on the world stage will be better choreographed. In addition to plans for new major experimental facilities, the Hamburg seminar also provided a valuable snapshot of the current scene and the directions in which ongoing research is poised to take. This covered existing facilities and projects, 'factories' to mass-produce specific particles, fixed target studies and non-accelerator experiments as well as the key EPLC development theme. B-physics, the study of particles containing the fifth, or 'beauty' (b) quark, emerged as an important thread running across several machine scenarios

  18. International collaborations through the internet

    DEFF Research Database (Denmark)

    Olson, Gary M.; David, Paul A.; Eksteen, Johan

    2007-01-01

    The past decade has seen remarkable advances in the availability of tools to support scientific collaboration at a distance. This is especially good news for international collaborations, where in the past constraints on collocation and travel have made such collaborations a major challenge. The ...

  19. Gauchers sygdom

    DEFF Research Database (Denmark)

    Leth, Peter Mygind; Knudsen, Ida Mølgård

    1987-01-01

    Gaucher's disease is an autosomal recessive disease due to deficiency of the enzyme glucocerebrosidase with subsequent accumulation of glucocerebroside in the reticuloendothelial system. The disease is subdivided into Types 1, 2 and 3. Type 1 is associated with hepatosplenomegaly and lesions...

  20. International scientific collaboration in nonproliferation

    International Nuclear Information System (INIS)

    Travelli, A.

    1998-01-01

    International collaboration is a vital component of every serious nonproliferation effort. Several examples of the experiences that the Argonne Arms Control and Nonproliferation Program has had in this area are given and, in the process, important components of the program come to light. Some of the main principles that the program has learned to follow while pursuing international collaboration projects are shared, as are the pitfalls that the program has learned to avoid. (author)

  1. Gaucher's disease

    International Nuclear Information System (INIS)

    Hainaux, B.; Christophe, C.; Hanquinet, S.; Perlmutter, N.

    1992-01-01

    We report our observations made by conventional radiography, ultrasound, computerized tomography (CT), and magnetic resonance imaging (MRI) on a 3 1/2-year-old girl with Gaucher's disease. The interest of the case consists in the exceptional lungs involvement, the demonstration by MRI of the bone marrow involvement and the necrosis and fibrosis of the liver, as shown by CT. This liver complication has been previously reported only once. (orig.)

  2. ACC International Academic Collaborative receives special award

    OpenAIRE

    Felker, Susan B.

    2006-01-01

    The Atlantic Coast Conference's new International Academic Collaborative (ACC/IAC) has been singled out by the New York-based Institute of International Education (IIE) for a special award for innovation in international education.

  3. International tuberculosis research collaborations within Asia.

    Science.gov (United States)

    Molton, James S; Singh, Shweta; Chen, Ling Jun; Paton, Nicholas I

    2017-09-07

    Asia bears more than half the global tuberculosis (TB) burden. Economic development in the region has increased available funding for biomedical research and opportunity for collaboration. We explored the extent of international tuberculosis research collaborations between institutions within Asia. We conducted a Pubmed search for all articles with tuberculosis in the title published during a 12 month period with at least one author affiliation listed in Asia, then identified international collaborations from institution websites and internet searches. We identified 99 international collaborations involving an institution within Asia, of which only 8 (8.1%) were collaborations between Asian institutions. The remainder were with institutions outside of Asia. The paucity of intra-Asian international research collaboration represents a lost opportunity to optimise regional research funding, capacity building and the development of an Asia-relevant TB research agenda.

  4. The use of technology in international collaboration

    DEFF Research Database (Denmark)

    Livonen, Mirja; Sonnenwald, Diane H.

    2000-01-01

    International collaboration is emerging as an essential function for organizations, playing an important role in organizational strategy, performance and knowledge management. Two case studies of international collaboration are discussed in this paper. Participants' perceptions and use...... of technology to collaborate are examined from the perspective of sense of presence, participation, task type, productivity and ease of use. The data suggest that technology compatibility with cultural and work style preferences and technology infrastructure is more important than media richness, in contrast...

  5. International collaboration in medical radiation science.

    Science.gov (United States)

    Denham, Gary; Allen, Carla; Platt, Jane

    2016-06-01

    International collaboration is recognised for enhancing the ability to approach complex problems from a variety of perspectives, increasing development of a wider range of research skills and techniques and improving publication and acceptance rates. The aim of this paper is to describe the current status of international collaboration in medical radiation science and compare this to other allied health occupations. This study utilised a content analysis approach where co-authorship of a journal article was used as a proxy for research collaboration and the papers were assigned to countries based on the corporate address given in the by-line of the publication. A convenience sample method was employed and articles published in the professional medical radiation science journals in the countries represented within our research team - Australia, the United Kingdom (UK) and the United States of America (USA) were sampled. Physiotherapy, speech pathology, occupational therapy and nursing were chosen for comparison. Rates of international collaboration in medical radiation science journals from Australia, the UK and the USA have steadily increased over the 3-year period sampled. Medical radiation science demonstrated lower average rates of international collaboration than the other allied health occupations sampled. The average rate of international collaboration in nursing was far below that of the allied health occupations sampled. Overall, the UK had the highest average rate of international collaboration, followed by Australia and the USA, the lowest. Overall, medical radiation science is lagging in international collaboration in comparison to other allied health fields.

  6. The incidence of Parkinsonism in patients with type 1 Gaucher disease: Data from the ICGG Gaucher Registry

    Science.gov (United States)

    Rosenbloom, Barry; Balwani, Manisha; Bronstein, Jeff M.; Kolodny, Edwin; Sathe, Swati; Gwosdow, Andrea R.; Taylor, John S.; Cole, J. Alexander; Zimran, Ari; Weinreb, Neal J.

    2015-01-01

    Purpose Investigate the incidence of Parkinsonism among patients with Gaucher disease type 1 (GD1) and describe demographics, genotypes, and Gaucher disease (GD)-related characteristics for affected and non-affected patients. Methods Study type: Cohort study with age- and gender-matched nested case–control analysis. Calculation of event incidence, standardized morbidity ratio, and event-free survival (Kaplan–Meier). Data source: The International Collaborative Gaucher Group (ICGG) Gaucher Registry data as of June 2010. Study cohort: GD1 patients with any report of Parkinsonism. Pre-matching control group: All GD1 patients with no report of Parkinsonism. Results The matched study cohort comprised of 68 patients with reports of Parkinsonism and 649 patients without Parkinsonism. Demographic and clinical characteristics suggest a milder GD phenotype in patients with Parkinsonism compared to the control group. The most prevalent GD1 genotype was N370S/N370S (39% for controls; 46% for patients with Parkinsonism). Patients with Parkinsonism were diagnosed with GD1 at a mean age of 37 years compared to 31 years in control patients. The standardized morbidity ratio for the development of Parkinsonism among all GD1 patients indicated an approximately 6 to 17 fold increase over that of 2 reference populations. The mean age of reported Parkinsonism onset was 57 years compared to 60 years in the general population (Lees, Hardy, and Revesz, 2009 [1]). The probability that a patient with GD1 will develop Parkinsonism before age 70 years is 5 to 7% and 9 to 12% before age 80 years. Conclusions The incidence of Parkinsonism among GD1 patients is significantly increased compared to two reference populations. GD1 patients with Parkinsonism have a later median age at GD diagnosis, later age at the start of treatment, and later age at death than patients with GD1 alone. The Gaucher-related clinical profile of GD1 patients with Parkinsonism is similar to or milder than the GD1

  7. Legal Considerations for International Collaborative Research Contract

    International Nuclear Information System (INIS)

    Lee, D. S.; Oh, K. B.; Kim, H. J.; Lee, J. H.

    2007-01-01

    Though collaborative research is pure academic activity the research plan and resource allocation for the research are shaped under foam of contract. Thus, legal binding effect and compulsive instrument is adopted at the research contract. This paper aimed at guiding equal collaborative research contract in legal aspect. To reach the goal (1) enforceability and elements of international collaborative contract, (2) damage calculation and related issues with those topics shall be discussed in each section

  8. Processes of international collaboration in management research

    DEFF Research Database (Denmark)

    Jonsen, Karsten; Butler, Christina; Mäkelä, Kristiina

    2013-01-01

    Scientists and academics increasingly work on collaborative projects and write papers in international research teams. This trend is driven by greater publishing demands in terms of the quality and breadth of data and analysis methods, which tend to be difficult to achieve without collaborating...... across institutional and national boundaries. Yet, our understanding of the collaborative processes in an academic setting and the potential tensions associated with them remains limited. We use a reflexive, autoethnographic approach to explicitly investigate our own experiences of international...... collaborative research. We offer systematic insights into the social and intellectual processes of academic collaborative writing, identifying six lessons and two key tensions that influence the success of international research teams. Our findings may benefit the formation of future coauthor teams...

  9. Integrating Diverse Data Systems for International Collaboration

    Science.gov (United States)

    Fox, Peter

    2014-05-01

    International collaborations, especially ones that arise with little or no financial resources, still face challenges in opening up data collections via a wide variety of differing and often non-interoperable means. In turn, this hampers the collaborative process, slows or even prevents scientific exchange. Early efforts that proposed a centralized, and project specific data archive encountered many difficulties, ranging from little or no adoption, to the inability to provide required documentation and metadata to make the datasets findable or usable. In time, virtualized approaches appeared to gain traction, for e.g. virtual observatories. In this contribution, we report on several international collaboration case studies with distributed data systems; their needs, successes, challenges and failures and synthesize a set of suggested practices to inform future international collaboration efforts.

  10. Radionuclide assessment of Gaucher's disease

    International Nuclear Information System (INIS)

    Cheng, T.H.; Holman, B.L.

    1978-01-01

    Gaucher's disease involves multiple organs and may present with variable severity. The scintigraphic appearance of the reticuloendothelial system and bone are described in three patients with Gaucher's disease. Scintigraphic abnormalities reflected the severity of organ involvement and correlated well with the patients' clinical status. Scintigraphy appears useful for the evaluation and followup of patients with Gaucher's disease

  11. Learning Together Through International Collaborative Writing Groups

    Directory of Open Access Journals (Sweden)

    Mick Healey

    2017-03-01

    Full Text Available The International Collaborative Writing Groups (ICWG initiative creates a space for ongoing collaboration amongst scholars of teaching and learning who co-author a manuscript on a topic of shared interest. The second ICWG, linked to the 2015 International Society for the Scholarship of Teaching and Learning Conference in Melbourne, Australia, involved 59 scholars from 11 countries. In this piece, we describe the aims, process, and outcomes for the ICWG, comparing it with the first ICWG in 2012. While international collaboration around a topic of shared interest is generally viewed positively, the realities of collaborating online with limited face-to-face interactions to complete a manuscript can be challenging. We argue, despite such challenges, that ongoing collaboration amongst scholars is vital to the scholarship of teaching and learning (SoTL movement. Drawing on our experience of leading the overall ICWG initiative and our research into participants’ experiences, we suggest there are individual dispositions toward collaboration that enrich and enable successful participation in ICWG experiences. We end by highlighting the final products arising from almost two year of collaborative thinking and writing from six groups.

  12. Timing of initiation of enzyme replacement therapy after diagnosis of type 1 Gaucher disease: effect on incidence of avascular necrosis

    Science.gov (United States)

    Mistry, Pramod K; Deegan, Patrick; Vellodi, Ashok; Cole, J Alexander; Yeh, Michael; Weinreb, Neal J

    2009-01-01

    Data from the International Collaborative Gaucher Group Gaucher Registry were analysed to assess the relationship between enzyme replacement therapy with imiglucerase (ERT) and incidence of avascular necrosis (AVN) in type 1 Gaucher disease (GD1), and to determine whether the time interval between diagnosis and initiation of ERT influences the incidence rate of AVN. All patients with GD1 enrolled in the Gaucher Registry who received ERT and did not report AVN prior to starting therapy (n = 2700) were included. The incidence rate of AVN following initiation of ERT was determined. An incidence rate of AVN of 13·8 per 1000 person-years was observed in patients receiving ERT. Patients who initiated ERT within 2 years of diagnosis had an incidence rate of 8·1 per 1000 person-years; patients who started ERT ≥2 years after diagnosis had an incidence rate of 16·6 per 1000 person-years. The adjusted incidence rate ratio was 0·59 [95% confidence interval (CI) 0·36–0·96, P = 0·0343]. Splenectomy was an independent risk factor for AVN (adjusted incidence rate ratio 2·23, 95% CI 1·61–3·08, P < 0·0001). In conclusion, the risk of AVN was reduced among patients who initiated ERT within 2 years of diagnosis, compared to initiating treatment ≥2 years after diagnosis. A higher risk of AVN was observed among patients who had previously undergone splenectomy. PMID:19732054

  13. Role of Scientific Societies in International Collaboration

    Science.gov (United States)

    Fucugauchi, J. U.

    2007-12-01

    Geophysical research increasingly requires global multidisciplinary approaches. Understanding how deeply interrelated are Earth components and processes, population growth, increased needs of mineral and energy resources, global impact of human activities, and view of our planet as an interconnected system emphasizes the need of international cooperation. International research collaboration has an immense potential and is needed for further development of Earth science research and education. The Union Session is planned to provide a forum for analysis and discussion of the status of research and education of geosciences in developing countries, international collaboration programs and new initiatives for promoting and strengthening scientific cooperation. A theme of particular relevance in the analyses and discussions is the role of scientific societies in international collaboration. Societies organize meetings, publish journals and books and promote cooperation through academic exchange activities. They may further assist communities in developing countries in providing and facilitating access to scientific literature, attendance to international meetings, short and long-term stays and student and young researcher mobility. What else can be done? This is a complex subject and scientific societies may not be seen independently from the many factors involved in research and education. Developing countries present additional challenges resulting from limited economic resources and social and political problems, while urgently requiring improved educational and research programs. Needed are in-depth analyses of infrastructure and human resources, and identification of major problems and needs. What are the major limitations and needs in research and postgraduate education in developing countries? What and how should international collaboration do? What are the roles of individuals, academic institutions, funding agencies, scientific societies? Here we attempt to

  14. International collaboration on CESR-TA program

    International Nuclear Information System (INIS)

    Flanagan, John; Suetsugu, Yusuke

    2009-01-01

    An international collaboration on the CESR-TA (Cornell Electron Storage Ring-Test Accelerator) program, which is a program to investigate electron cloud instability (ECI) issues in the positron damping ring of the ILC (International Linear-Collider), is currently underway a Cornell University, KEK is supporting the program through the development of an X-ray beam profile monitor system to measure the extremely small beam size, and through the development of clearing electrodes to mitigate the ECI. (author)

  15. Sustaining an International Partnership: An Evolving Collaboration

    Science.gov (United States)

    Pierson, Melinda R.; Myck-Wayne, Janice; Stang, Kristin K.; Basinska, Anna

    2015-01-01

    Universities across the United States have an increasing interest in international education. Increasing global awareness through educational collaborations will promote greater cross-cultural understanding and build effective relationships with diverse communities. This paper documents one university's effort to build an effective international…

  16. Pathology of Gaucher's Disease

    African Journals Online (AJOL)

    1974-06-01

    Jun 1, 1974 ... disability in 2 of them. One patient also developed restric- tive lung disease, presumably on the ... Brother of case 9; diagnosed as Gaucher's. 8500 g disease at age 6 yrs on basis of hepato- ... other siblings, one of whom is said to have an enlarged spleen. Estimations of ,a-glucosidase on lymphocytes from.

  17. Pathology of Gaucher's Disease

    African Journals Online (AJOL)

    1974-06-01

    Jun 1, 1974 ... cytoplasm of the Gaucher cells, while ultrastructural examination has confirmed ... For light microscopy, sections from the spleen, bone marrow, liver and ... The dried grid was examined in a vacuum-coating unit at an angle of ...

  18. International energy technology collaboration: benefits and achievements

    International Nuclear Information System (INIS)

    1996-01-01

    The IEA Energy Technology Collaboration Programme facilitates international collaboration on energy technology research, development and deployment. More than 30 countries are involved in Europe, America, Asia, Australasia and Africa. The aim is to accelerate the development and deployment of new energy technologies to meet energy security, environmental and economic development goals. Costs and resources are shared among participating governments, utilities, corporations and universities. By co-operating, they avoid unproductive duplication and maximize the benefits from research budgets. The IEA Programme results every year in hundreds of publications which disseminate information about the latest energy technology developments and their commercial utilisation. The IEA Energy Technology Collaboration Programme operates through a series of agreements among governments. This report details the activities and achievements of all 41 agreements, covering energy technology information centres and Research and Development projects in fossil fuels, renewable energy efficient end-use, and nuclear fusion technologies. (authors). 58 refs., 9 tabs

  19. Ocean Drilling: Forty Years of International Collaboration

    Science.gov (United States)

    Smith, Deborah K.; Exon, Neville; Barriga, Fernando J. A. S.; Tatsumi, Yoshiyuki

    2010-10-01

    International cooperation is an essential component of modern scientific research and societal advancement [see Ismail-Zadeh and Beer, 2009], and scientific ocean drilling represents one of Earth science's longest-running and most successful international collaborations. The strength of this collaboration and its continued success result from the realization that scientific ocean drilling provides a unique and powerful tool to study the critical processes of both short-term change and the long-term evolution of Earth systems. A record of Earth's changing tectonics, climate, ocean circulation, and biota is preserved in marine sedimentary deposits and the underlying basement rocks. And because the ocean floor is the natural site for accumulation and preservation of geological materials, it may preserve a continuous record of these processes.

  20. International Medical Collaboration: Lessons from Cuba

    Science.gov (United States)

    Castelló González, Mauro; Pons Vásquez, Reinaldo; Rodriguez Bencomo, David; Choonara, Imti

    2016-01-01

    Over 50,000 Cuban health professionals are currently working overseas in 67 different countries. They work in conjunction with local health professionals. The majority work in primary care in deprived areas. The aim is to reduce morbidity and mortality but also improve health in the long term by training local health professionals, and building both institutions and a structure to deliver health care alongside educating the local population. Cuba is a small, middle-income country. It has, however, made a significant international contribution in relation to medical collaboration. Cuba’s international collaboration is based on the principles of social justice and equity for all. It has set an example for other countries to emulate. PMID:27763571

  1. International Medical Collaboration: Lessons from Cuba

    Directory of Open Access Journals (Sweden)

    Mauro Castelló González

    2016-10-01

    Full Text Available Over 50,000 Cuban health professionals are currently working overseas in 67 different countries. They work in conjunction with local health professionals. The majority work in primary care in deprived areas. The aim is to reduce morbidity and mortality but also improve health in the long term by training local health professionals, and building both institutions and a structure to deliver health care alongside educating the local population. Cuba is a small, middle-income country. It has, however, made a significant international contribution in relation to medical collaboration. Cuba’s international collaboration is based on the principles of social justice and equity for all. It has set an example for other countries to emulate.

  2. The International Nucleotide Sequence Database Collaboration.

    Science.gov (United States)

    Cochrane, Guy; Karsch-Mizrachi, Ilene; Nakamura, Yasukazu

    2011-01-01

    Under the International Nucleotide Sequence Database Collaboration (INSDC; http://www.insdc.org), globally comprehensive public domain nucleotide sequence is captured, preserved and presented. The partners of this long-standing collaboration work closely together to provide data formats and conventions that enable consistent data submission to their databases and support regular data exchange around the globe. Clearly defined policy and governance in relation to free access to data and relationships with journal publishers have positioned INSDC databases as a key provider of the scientific record and a core foundation for the global bioinformatics data infrastructure. While growth in sequence data volumes comes no longer as a surprise to INSDC partners, the uptake of next-generation sequencing technology by mainstream science that we have witnessed in recent years brings a step-change to growth, necessarily making a clear mark on INSDC strategy. In this article, we introduce the INSDC, outline data growth patterns and comment on the challenges of increased growth.

  3. (International Collaboration on Advanced Neutron Sources)

    Energy Technology Data Exchange (ETDEWEB)

    Hayter, J.B.

    1990-11-08

    The International Collaboration on Advanced Neutron Sources was started about a decade ago with the purpose of sharing information throughout the global neutron community. The collaboration has been extremely successful in optimizing the use of resources, and the discussions are open and detailed, with reasons for failure shared as well as reasons for success. Although the meetings have become increasingly oriented toward pulsed neutron sources, many of the neutron instrumentation techniques, such as the development of better monochromators, fast response detectors and various data analysis methods, are highly relevant to the Advanced Neutron Source (ANS). I presented one paper on the ANS, and another on the neutron optical polarizer design work which won a 1989 R D-100 Award. I also gained some valuable design ideas, in particular for the ANS hot source, in discussions with individual researchers from Canada, Western Europe, and Japan.

  4. Renal involvement in Gaucher's disease.

    Science.gov (United States)

    Siegal, A.; Gutman, A.; Shapiro, M. S.; Griffel, B.

    1981-01-01

    A patient with chronic Gaucher's disease is described who developed glomerulopathy 24 years after splenectomy terminating in renal failure. The pathological changes of this very rare complication of Gaucher's disease are described. The few similar cases reported in the literature are reviewed and the possible pathogenetic pathways discussed. Images Fig. 1 Fig. 2 Fig. 3 PMID:7301691

  5. Gaucher's disease diagnosed by splenectomy.

    Science.gov (United States)

    Adas, Mine; Adas, Gokhan; Karatepe, Oguzhan; Altiok, Merih; Ozcan, Deniz

    2009-08-01

    Splenectomy continues to find common therapeutic indications for hematologic disorders. In addition, recently it is also performed in surgical clinics to assist diagnose of some illnesses. Gaucher's disease, especially Type I, is the most frequently encountered lysosomal storage disorder in man. Manifestations of it are highly variable. The most frequently found symptoms include splenomegaly with anaemia and thrombocytopenia, mostly due to hypersplenism, hepatomegaly and bone disease. Four patients were reported in the present study. Three of them were easily diagnosed with Gaucher's disease via bone marrow cytology, and one with Gaucher's disease was detected by pathological examination following the splenectomy. For the pouse of diagnosis of the Gaucher's disease, performing surgery is generally not necessary. However, for the cases of difficult to diagnose by classical methods, the corect diagnosis of Gaucher's disease can only be made by a special operation.

  6. International Collaboration on CO2 Sequestration

    Energy Technology Data Exchange (ETDEWEB)

    Peter H. Israelsson; E. Eric Adams

    2007-06-30

    On December 4, 1997, the US Department of Energy (USDOE), the New Energy and Industrial Technology Development Organization of Japan (NEDO), and the Norwegian Research Council (NRC) entered into a Project Agreement for International Collaboration on CO{sub 2} Ocean Sequestration. Government organizations from Japan, Canada, and Australia, and a Swiss/Swedish engineering firm later joined the agreement, which outlined a research strategy for ocean carbon sequestration via direct injection. The members agreed to an initial field experiment, with the hope that if the initial experiment was successful, there would be subsequent field evaluations of increasingly larger scale to evaluate environmental impacts of sequestration and the potential for commercialization. The evolution of the collaborative effort, the supporting research, and results for the International Collaboration on CO{sub 2} Ocean Sequestration were documented in almost 100 papers and reports, including 18 peer-reviewed journal articles, 46 papers, 28 reports, and 4 graduate theses. These efforts were summarized in our project report issued January 2005 and covering the period August 23, 1998-October 23, 2004. An accompanying CD contained electronic copies of all the papers and reports. This report focuses on results of a two-year sub-task to update an environmental assessment of acute marine impacts resulting from direct ocean sequestration. The approach is based on the work of Auerbach et al. [6] and Caulfield et al. [20] to assess mortality to zooplankton, but uses updated information concerning bioassays, an updated modeling approach and three modified injection scenarios: a point release of negatively buoyant solid CO{sub 2} hydrate particles from a moving ship; a long, bottom-mounted diffuser discharging buoyant liquid CO{sub 2} droplets; and a stationary point release of hydrate particles forming a sinking plume. Results suggest that in particular the first two discharge modes could be

  7. International collaboration for nuclear competence building

    International Nuclear Information System (INIS)

    Haapalehto, T.; Storey, P.

    2004-01-01

    The life cycle of the nuclear industry is no different to that of any other industry, indeed to most forms of human activity: birth, growth, maturity, decline, rebirth and renewal or death. As a result of the twin facets of long time scales and essential technical competence the industry now faces two problems: how to retain existing skills and competences for the 50 plus years that a plant is operating and how to develop and retain new skills and competences in the areas of decommissioning and radioactive waste management. Different countries are at different stages of the nuclear technology life cycle, a competence that may have declined or be lost in one country may be strong in another. And therein lies one solution to the problems the sector faces - international collaboration. The initiatives such as the NEA Halden project and the Generation IV International Forum lay a ground for quiet optimism that collaboration, information exchange and exchange of personnel continue to be an integral part of the development of nuclear power. Also there is evidence that myriad initiatives are underway in the area of nuclear education and training. Though, national surveys show that still more engineers and scientists having nuclear knowledge are required than are graduating. (author)

  8. Benefits of International Collaboration on the International Space Station

    Science.gov (United States)

    Hasbrook, Pete; Robinson, Julie A.; Brown Tate, Judy; Thumm, Tracy; Cohen, Luchino; Marcil, Isabelle; De Parolis, Lina; Hatton, Jason; Umezawa, Kazuo; Shirakawa, Masaki; hide

    2017-01-01

    The International Space Station is a valuable platform for research in space, but the benefits are limited if research is only conducted by individual countries. Through the efforts of the ISS Program Science Forum, international science working groups, and interagency cooperation, international collaboration on the ISS has expanded as ISS utilization has matured. Members of science teams benefit from working with counterparts in other countries. Scientists and institutions bring years of experience and specialized expertise to collaborative investigations, leading to new perspectives and approaches to scientific challenges. Combining new ideas and historical results brings synergy and improved peer-reviewed scientific methods and results. World-class research facilities can be expensive and logistically complicated, jeopardizing their full utilization. Experiments that would be prohibitively expensive for a single country can be achieved through contributions of resources from two or more countries, such as crew time, up- and downmass, and experiment hardware. Cooperation also avoids duplication of experiments and hardware among agencies. Biomedical experiments can be completed earlier if astronauts or cosmonauts from multiple agencies participate. Countries responding to natural disasters benefit from ISS imagery assets, even if the country has no space agency of its own. Students around the world participate in ISS educational opportunities, and work with students in other countries, through open curriculum packages and through international competitions. Even experiments conducted by a single country can benefit scientists around the world, through specimen sharing programs and publicly accessible "open data" repositories. For ISS data, these repositories include GeneLab and the Physical Science Informatics System. Scientists can conduct new research using ISS data without having to launch and execute their own experiments. Multilateral collections of research

  9. ACE phenotyping in Gaucher disease.

    Science.gov (United States)

    Danilov, Sergei M; Tikhomirova, Victoria E; Metzger, Roman; Naperova, Irina A; Bukina, Tatiana M; Goker-Alpan, Ozlem; Tayebi, Nahid; Gayfullin, Nurshat M; Schwartz, David E; Samokhodskaya, Larisa M; Kost, Olga A; Sidransky, Ellen

    2018-04-01

    Gaucher disease is characterized by the activation of splenic and hepatic macrophages, accompanied by dramatically increased levels of angiotensin-converting enzyme (ACE). To evaluate the source of the elevated blood ACE, we performed complete ACE phenotyping using blood, spleen and liver samples from patients with Gaucher disease and controls. ACE phenotyping included 1) immunohistochemical staining for ACE; 2) measuring ACE activity with two substrates (HHL and ZPHL); 3) calculating the ratio of the rates of substrate hydrolysis (ZPHL/HHL ratio); 4) assessing the conformational fingerprint of ACE by evaluating the pattern of binding of monoclonal antibodies to 16 different ACE epitopes. We show that in patients with Gaucher disease, the dramatically increased levels of ACE originate from activated splenic and/or hepatic macrophages (Gaucher cells), and that both its conformational fingerprint and kinetic characteristics (ZPHL/HHL ratio) differ from controls and from patients with sarcoid granulomas. Furthermore, normal spleen was found to produce high levels of endogenous ACE inhibitors and a novel, tightly-bound 10-30 kDa ACE effector which is deficient in Gaucher spleen. The conformation of ACE is tissue-specific. In Gaucher disease, ACE produced by activated splenic macrophages differs from that in hepatic macrophages, as well as from macrophages and dendritic cells in sarcoid granulomas. The observed differences are likely due to altered ACE glycosylation or sialylation in these diseased organs. The conformational differences in ACE may serve as a specific biomarker for Gaucher disease. Copyright © 2018 Elsevier Inc. All rights reserved.

  10. Scintigraphic appearances of Gaucher's disease

    International Nuclear Information System (INIS)

    Okada, Junichi; Uchiyama, Guio; Hayakawa, Kazushige; Araki, Tsutomu; Hayashi, Sanshin; Yamada, Kayoko; Seto, Kazuhiko

    1985-01-01

    Gaucher's disease is an inborn error of metabolism in which glucocerebroside accumlates within reticuloendotherial cells of spleen, liver and bone marrow. The scintigraphic appearances are described in a 19-year-old man with Gaucher's disease. The colloid scan showed increased uptake in the lung and bone marrow of legs where the reticuloendotherial system was activated. The bone scan demonstrated increased activity in the metaphyseal and diaphyseal region infiltrated by Gaucher's cell. Ga-67 was thought to be accumlated in the progressive focus of the disease. (author)

  11. ELENA’s International Collaboration is born

    CERN Multimedia

    Antonella Del Rosso

    2012-01-01

    On 13 June, ten institutes signed a Memorandum of Understanding (MoU) for the construction of the Extra Low ENergy Antiproton ring (ELENA). Allowing the further deceleration of antiprotons from the Antimatter Decelerator, ELENA will significantly increase the number of particles trapped downstream in the experimental set-ups. This will give an important boost to antimatter research in the years to come.   Electrostatic triplet lenses - a device that will transport antiprotons from ELENA to the experiments. The electrostatic device was successfully tested with the ASACUSA experiment two weeks ago. ELENA - an upgrade of the existing Antiproton Decelerator (AD) - was approved by the CERN Council last year under the condition that external user institutions would contribute to its construction. On 13 June, the foundation stone of the new international collaboration was laid with the signature of the MoU. ELENA is a small magnetic decelerator ring 30 m in circumference that will fit inside the ...

  12. Conversion in the framework of international collaboration. Proceedings

    International Nuclear Information System (INIS)

    Akhmetov, T.; Vagin, S.; Urezchenko, V.

    1996-01-01

    22-26 October 1996 the Republic of Kazakhstan Ministry of Science - Academy of Science, International Science and Technology Center with collaboration of National Nuclear Center of the Republic of Kazakhstan conducted an international workshop C onversion in the framework of international collaboration . In the workshop scientists and specialists from different countries participated. 84 reports were presented in this workshop

  13. Conversion in the framework of international collaboration. Proceedings

    Energy Technology Data Exchange (ETDEWEB)

    Akhmetov, T; Vagin, S; Urezchenko, V [eds.

    1997-12-31

    22-26 October 1996 the Republic of Kazakhstan Ministry of Science - Academy of Science, International Science and Technology Center with collaboration of National Nuclear Center of the Republic of Kazakhstan conducted an international workshop {sup C}onversion in the framework of international collaboration{sup .} In the workshop scientists and specialists from different countries participated. 84 reports were presented in this workshop

  14. A schedule for fusion research development and international collaboration

    International Nuclear Information System (INIS)

    Kakihana, H.

    1983-01-01

    In order to reach their goal of commercial fusion power reactors, development must proceed in a series of basic stages. Each step is expected to incur an increased level of cost. The cost-sharing benefits of international collaboration will become increasingly important and attractive with each successive step preceding commercialization. Outstanding examples of implementation of international collaboration in fusion include the JET project and the INTOR workshop which lend encouragement for the prospects for international collaboration in fusion in the future. (author)

  15. Gaucher disease in sheep.

    Science.gov (United States)

    Karageorgos, Litsa; Lancaster, Malcolm J; Nimmo, Judith S; Hopwood, John J

    2011-02-01

    Gaucher disease, an autosomal recessive lysosomal storage disorder caused by mutations in the β-glucocerebrosidase gene, was recently discovered in sheep on a "Southdown" sheep stud in Victoria, Australia. Clinical signs include neuropathy, thickened leathery skin, and ichthyosis, with lambs unable to stand from birth. Affected lambs were found to be deficient in glucocerebrosidase activity, and mutational analysis found them to be homozygous for the missense mutations c.1142G>A (p.C381Y) and c.1400C>T (p.P467L). In addition, four silent mutations were detected (c.777C>A [p.Y259Y], c1203A>G [p.Q401Q], c.1335T>C [p.I445I], c.1464C>G [p.L488L]). The human equivalent [C342Y] to the C381Y mutation leads to an acute neuronopathic phenotype in patients. Identification of an acute neuronopathic form of Gaucher disease in sheep provides a large animal model that will enable studies of pathology and evaluation of therapies to treat this common lysosomal storage disorder.

  16. Radiological aspects of Gaucher disease

    International Nuclear Information System (INIS)

    Katz, Robert; Booth, Tom; Hargunani, Rikin; Wylie, Peter; Holloway, Brian

    2011-01-01

    Advances in imaging and the development of commercially available enzyme therapy have significantly altered the traditional radiology of Gaucher disease. The cost of treatment and need for monitoring response to therapy have magnified the importance of imaging. There are no recent comprehensive reviews of the radiology of this relatively common lysosomal storage disease. This article describes the modern imaging, techniques and radiological manifestations of Gaucher disease. (orig.)

  17. Radiological aspects of Gaucher disease

    Energy Technology Data Exchange (ETDEWEB)

    Katz, Robert; Booth, Tom; Hargunani, Rikin; Wylie, Peter; Holloway, Brian [Royal Free Hospital, Radiology Department, London (United Kingdom)

    2011-12-15

    Advances in imaging and the development of commercially available enzyme therapy have significantly altered the traditional radiology of Gaucher disease. The cost of treatment and need for monitoring response to therapy have magnified the importance of imaging. There are no recent comprehensive reviews of the radiology of this relatively common lysosomal storage disease. This article describes the modern imaging, techniques and radiological manifestations of Gaucher disease. (orig.)

  18. 12th International Symposium on Open Collaboration Companion

    CERN Document Server

    2016-01-01

    Welcome to the proceedings of OpenSym 2016, the 12th international symposium on open collaboration! Open collaboration is collaboration that is egalitarian (everyone can join, no principled or artificial barriers to participation exist), meritocratic (decisions and status are merit-based rather than imposed) and self-organizing (processes adapt to people rather than people adapt to predefined processes).

  19. International Collaboration Activities in Different Geologic Disposal Environments

    International Nuclear Information System (INIS)

    Birkholzer, Jens

    2015-01-01

    This report describes the current status of international collaboration regarding geologic disposal research in the Used Fuel Disposition (UFD) Campaign. Since 2012, in an effort coordinated by Lawrence Berkeley National Laboratory, UFD has advanced active collaboration with several international geologic disposal programs in Europe and Asia. Such collaboration allows the UFD Campaign to benefit from a deep knowledge base with regards to alternative repository environments developed over decades, and to utilize international investments in research facilities (such as underground research laboratories), saving millions of R&D dollars that have been and are being provided by other countries. To date, UFD's International Disposal R&D Program has established formal collaboration agreements with five international initiatives and several international partners, and national lab scientists associated with UFD have conducted specific collaborative R&D activities that align well with its R&D priorities.

  20. International Collaboration Activities in Different Geologic Disposal Environments

    Energy Technology Data Exchange (ETDEWEB)

    Birkholzer, Jens [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)

    2015-09-01

    This report describes the current status of international collaboration regarding geologic disposal research in the Used Fuel Disposition (UFD) Campaign. Since 2012, in an effort coordinated by Lawrence Berkeley National Laboratory, UFD has advanced active collaboration with several international geologic disposal programs in Europe and Asia. Such collaboration allows the UFD Campaign to benefit from a deep knowledge base with regards to alternative repository environments developed over decades, and to utilize international investments in research facilities (such as underground research laboratories), saving millions of R&D dollars that have been and are being provided by other countries. To date, UFD’s International Disposal R&D Program has established formal collaboration agreements with five international initiatives and several international partners, and national lab scientists associated with UFD have conducted specific collaborative R&D activities that align well with its R&D priorities.

  1. International Collaboration Activities on Engineered Barrier Systems

    Energy Technology Data Exchange (ETDEWEB)

    Jove-Colon, Carlos F. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2016-08-31

    The Used Fuel Disposition Campaign (UFDC) within the DOE Fuel Cycle Technologies (FCT) program has been engaging in international collaborations between repository R&D programs for high-level waste (HLW) disposal to leverage on gathered knowledge and laboratory/field data of near- and far-field processes from experiments at underground research laboratories (URL). Heater test experiments at URLs provide a unique opportunity to mimetically study the thermal effects of heat-generating nuclear waste in subsurface repository environments. Various configurations of these experiments have been carried out at various URLs according to the disposal design concepts of the hosting country repository program. The FEBEX (Full-scale Engineered Barrier Experiment in Crystalline Host Rock) project is a large-scale heater test experiment originated by the Spanish radioactive waste management agency (Empresa Nacional de Residuos Radiactivos S.A. – ENRESA) at the Grimsel Test Site (GTS) URL in Switzerland. The project was subsequently managed by CIEMAT. FEBEX-DP is a concerted effort of various international partners working on the evaluation of sensor data and characterization of samples obtained during the course of this field test and subsequent dismantling. The main purpose of these field-scale experiments is to evaluate feasibility for creation of an engineered barrier system (EBS) with a horizontal configuration according to the Spanish concept of deep geological disposal of high-level radioactive waste in crystalline rock. Another key aspect of this project is to improve the knowledge of coupled processes such as thermal-hydro-mechanical (THM) and thermal-hydro-chemical (THC) operating in the near-field environment. The focus of these is on model development and validation of predictions through model implementation in computational tools to simulate coupled THM and THC processes.

  2. International and interlaboratory collaboration on Neutron Science Project

    Energy Technology Data Exchange (ETDEWEB)

    Oyama, Yukio [Japan Atomic Energy Research Inst., Tokai, Ibaraki (Japan). Tokai Research Establishment

    1997-11-01

    For effectiveness of facility development for Neutron Science Projects at JAERI, international and interlaboratory collaborations have been extensively planned and promoted, especially in the areas of accelerator and target technology. Here status of two collaborations relevant to a spallation neutron target development is highlighted from those collaborations. The two collaborations are experiments on BNL-AGS spallation target simulation and PSI materials irradiation. Both are planned to start in spring of 1997. (author)

  3. EFFECTIVENESS AND SAFETY OF VELAGLUCERASE ALFA IN TREATMENT OF GAUCHER DISEASE TYPE 1 (ACCORDING TO INTERNATIONAL STUDIES

    Directory of Open Access Journals (Sweden)

    O. S. Gundobina

    2014-01-01

    Full Text Available The article is dedicated to modern approaches to treatment of Gaucher’s disease. The authors list the primary aspects of the disease and present data on the origin and introduction of pathogenetic enzyme replacement therapy to clinical practice. Review of international clinical studies demonstrates high effectiveness and safety of long-term enzyme replacement therapy with velaglucerase alfa in patients with confirmed type I Gaucher’s disease. 

  4. Long-term clinical outcomes in type 1 Gaucher disease following 10 years of imiglucerase treatment.

    Science.gov (United States)

    Weinreb, Neal J; Goldblatt, Jack; Villalobos, Jacobo; Charrow, Joel; Cole, J Alexander; Kerstenetzky, Marcelo; vom Dahl, Stephan; Hollak, Carla

    2013-05-01

    We studied the effect of long-term alglucerase/imiglucerase (Ceredase®/Cerezyme®, Genzyme, a Sanofi company, Cambridge, MA, USA) treatment on hematological, visceral, and bone manifestations of Gaucher disease type 1 (GD1). The International Collaborative Gaucher Group (ICGG) Gaucher Registry identified GD1 patients treated with alglucerase/imiglucerase who had dose and clinical data at first infusion and after 10 years of follow-up. Data for hemoglobin, platelet count, organ volumes, bone pain, and bone crisis were analyzed. Tests of the null hypothesis (no change from first infusion to 10 years) were performed using t tests for within-patient absolute change in continuous measurements and McNemar/chi-square tests for change in distributions using categorical values. An alpha level of 0.05 designated statistical significance. As of October 2011, 557 nonsplenectomized and 200 splenectomized patients met the inclusion criteria. The majority of GD1 patients had at least one N370S allele. Compared with nonsplenectomized patients at first infusion, splenectomized patients had lower percentages of anemia (26.0 % vs. 42.8 %) and thrombocytopenia (14.2 % vs. 76.3 %), similar percentages of moderate or severe hepatomegaly (81.2 % vs. 80.0 %), and higher percentages of bone pain (88.9 % vs. 52.4 %) and bone crises (38.3 % vs. 16.0 %). After 10 years, both groups showed significant (p < 0.05) improvements in mean hemoglobin levels, platelet count, liver, and spleen (nonsplenectomized) volumes, and bone crises. Initial dosing in both groups ranged from <15 U/kg to ≤90 U/kg every 2 weeks. After 10 years, the majority was receiving 15 to ≤45 U/kg every 2 weeks. Ten years of imiglucerase treatment results in sustainable improvements in all GD1 parameters.

  5. Hemorrhagic Aspects of Gaucher Disease

    Directory of Open Access Journals (Sweden)

    Hanna Rsenbaum

    2014-10-01

    Full Text Available Gaucher disease (GD is an inherited lysosomal disorder, originating from deficient activity of the lysosomal enzyme glucocerebrosidase (GCase. Normally, GCase hydrolyzes glucocerebroside (GC to glucose and ceramide; however, impaired activity of this enzyme leads to the accumulation of GC in macrophages, termed “Gaucher cells.” Gaucher disease is associated with hepatosplenomegaly, cytopenias, skeletal complications and in some forms involves the central nervous system. Coagulation abnormalities are common among GD patients due to impaired production and chronic consumption of coagulation factors. Bleeding phenomena are variable (as are other symptoms of GD and include mucosal and surgical hemorrhages. Four main etiological factors account for the hemostatic defect in GD: thrombocytopenia, abnormal platelet function, reduced production of coagulation factors, and activation of fibrinolysis. Thrombocytopenia relates not only to hypersplenism and decreased megakaryopoiesis by the infiltrated bone marrow but also to immune thrombocytopenia. Autoimmunity, especially the induction of platelet antibody production, might cause persistent thrombocytopenia. Enzyme replacement therapy reverses only part of the impaired coagulation system in Gaucher disease. Other therapeutic and supportive measures should be considered to prevent and/or treat bleeding in GD. Gaucher patients should be evaluated routinely for coagulation abnormalities especially prior to surgery and dental and obstetric procedures.

  6. How international is internationally collaborated research? A bibliometric study of Russian surname holder collaboration networks

    Energy Technology Data Exchange (ETDEWEB)

    Karaulova, M.; Goek, A.; Shapira, P.

    2016-07-01

    International research performance indicators attain increased attention in science policy. They are seen to reflect relative competitiveness of a country in producing leading research (in terms of cited papers) and its commercialisation (in terms of assigned patents). However, more studies point to ongoing global bias in production, composition and assessment of research performance metrics (Rafols et al., 2012; van Leeuwen et al., 2001). As research performance indicators are used increasingly in national science policy and in influential international rankings, it is important to understand their inherent bias. For instance, explosive growth of international collaboration in science is widely reported (Glänzel, 2001), and is generally perceived as having beneficial ‘knowledge exchange’ effect for involved parties. It is recognised as a capacity-building factor of domestic research indicating the increase in research quality (Bornmann et al., 2015). However, existing research has reported reproduction of uneven global relations between countries in terms of science and technology. For example, patterns of international cooperation in nanotechnology are still centred on the developed countries, which are key nodes in international networks (Shapira and Wang, 2010). (Author)

  7. International collaboration on inherently safe nuclear reactors

    International Nuclear Information System (INIS)

    Barkenbus, J.N.

    1989-01-01

    Science and technology transcend economic and political ideologies, providing a means of communications and approach common to both the United States and the Soviet Union. This paper suggests that the field of nuclear fission is a logical and productive area for superpower and broader collaboration, but that the kind of collaboration characteristic of past and present activity is less than it optimally could be. The case for cost sharing is compelling with budget constraints and mounting concerns over global warming. The case for collaboration is based on economic, psychological, and political grounds. A collaborative effort in nuclear fission is presented as a near term effort by building and testing of a prototype reactor in the 1990s

  8. Boosting China's research collaboration | IDRC - International ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    “China has dispatched Chinese medical teams since 1963,” says 2015 IDRC Research Award recipient Rong Li, “but there has been little public health assistance.” ... “China's cooperation to date has also largely ignored the role of research collaboration,” he says. Li focused ... I accumulated skills on qualitative research.

  9. ICTP: A Successful Model of International Scientific Collaboration

    CERN Multimedia

    CERN. Geneva

    2012-01-01

    The importance of international scientific collaboration in the changing world where the centre of gravity of fundamental research may be moving towards the east and the south is addressed. The unique role of ICTP in supporting global science is highlighted.

  10. ICFA: Protvino meeting looks at trends in international collaboration

    Energy Technology Data Exchange (ETDEWEB)

    Anon.

    1991-01-15

    International collaboration is the lifeblood of Big Science, and in high energy physics the triennial 'Future Perspectives' meeting organized by the International Committee for Future Accelerators (ICFA) provides a valuable opportunity to reappraise trends in this collaboration. The latest meeting was held in October at Protvino, near Moscow, where the Institute for High Energy Physics is the scene of construction work for the 21-kilometre UNK proton rings and the projected home of a big new linear collider for electrons and positrons.

  11. The European Gaucher Alliance: a survey of member patient organisations' activities, healthcare environments and concerns.

    Science.gov (United States)

    Žnidar, Irena; Collin-Histed, Tanya; Niemeyer, Pascal; Parkkinen, Johanna; Lauridsen, Anne-Grethe; Zariņa, Sandra; Cohen, Yossi; Manuel, Jeremy

    2014-09-02

    The European Gaucher Alliance (EGA) was established in 1994 and constituted in 2008 as an umbrella group supporting patient organisations for Gaucher disease. Every two years, the EGA conducts a questionnaire survey of member associations to help develop its priorities and annual work programme. Results of the latest survey are presented. Between June 2012 and April 2013, the 36 members and associate members of the EGA were asked to complete a questionnaire detailing membership numbers, disease specific treatments used by patients, means of access to treatment, availability of treatment centres and home infusions, sources of support for patients with Gaucher disease, patient organisations' activities, collaborations, funding sources and any issues of concern. Questionnaires completed in 2012 were revised in January 2013 and responses analysed between July and September 2013. Thirty three members returned data on one or more questions. Findings identified inequalities in access to treatment both within and between members' countries. Three of 27 countries, for which data were available, relied totally on humanitarian aid for treatment and 6% of untreated patients in 20 countries were untreated because of funding issues, a situation many feared would worsen with deteriorating economic climates. Access to treatment and reimbursement represented 45% of members' concerns, while 35% related to access to specialist treatment centres, home infusions and doctors with expertise in Gaucher disease. Member associations' main activities centred on patient support (59% of responses) and raising awareness of Gaucher disease and patients' needs amongst the medical community, government and healthcare decision makers and the general public (34% of responses). Twenty one (78% of respondents) indicated they were the only source of help for Gaucher disease patients in their country. For many, activities were constrained by funds; two members had no external funding source. Activities

  12. Collaborating internationally on physician leadership development: why now?

    Science.gov (United States)

    Chan, Ming-Ka; de Camps Meschino, Diane; Dath, Deepak; Busari, Jamiu; Bohnen, Jordan David; Samson, Lindy Michelle; Matlow, Anne; Sánchez-Mendiola, Melchor

    2016-07-04

    Purpose This paper aims to highlight the importance of leadership development for all physicians within a competency-based medical education (CBME) framework. It describes the importance of timely international collaboration as a key strategy in promoting physician leadership development. Design/methodology/approach The paper explores published and Grey literature around physician leadership development and proposes that international collaboration will meet the expanding call for development of leadership competencies in postgraduate medical learners. Two grounding frameworks were used: complexity science supports adding physician leadership training to the current momentum of CBME adoption, and relational cultural theory supports the engagement of diverse stakeholders in multiple jurisdictions around the world to ensure inclusivity in leadership education development. Findings An international collaborative identified key insights regarding the need to frame physician leadership education within a competency-based model. Practical implications International collaboration can be a vehicle for developing a globally relevant, generalizable physician leadership curriculum. This model can be expanded to encourage innovation, scholarship and program evaluation. Originality/value A competency-based leadership development curriculum is being designed by an international collaborative. The curriculum is based on established leadership and education frameworks. The international collaboration model provides opportunities for ongoing sharing, networking and diversification.

  13. Collaborative entrepreneurship: On the Influence of Internal and External Collaboration on Corporate Entrepreneurial Innovation

    DEFF Research Database (Denmark)

    Lassen, Astrid Heidemann; Timenes Laugen, Bjørge; Middel, Rick

    2008-01-01

    The present paper empirically tests the effect which internal/external collaboration has on innovation height and identifies characteristics of collaboration patterns leading to entrepreneurial innovation in particular. Doing so adds to the understanding of how corporate entrepreneurship best...... unfolds as interfirm activity, which here is termed collaborative entrepreneurship, and provides details on the particular patterns of Open Innovation. The empirical analysis is based on a data set with responses from 512 Danish engineers. The analysis finds that external collaboration has significantly...... different effects on innovation height depending on the type of partners involved, and furthermore suggests that the development of entrepreneurial innovation is not only dependent on high external involvement, but also on involvement and collaboration among internal functional departments and people....

  14. Scintigraphic picture in Gaucher's disease

    International Nuclear Information System (INIS)

    Bykov, S.A.; Gafton, G.I.; Petrov, V.P.

    1988-01-01

    Analysis of the results of combined radionuclide investigation of the liver, spleen, kidney, lymph nodes and bones in a patient with Gaucher's disease is given. Scintigraphy was shown to be an important method for making correct diagnosis in patients with this rare disease

  15. Genetics Home Reference: Gaucher disease

    Science.gov (United States)

    ... 500 to 1,000 people of Ashkenazi Jewish heritage. The other forms of Gaucher disease are uncommon and do not occur more frequently in people of Ashkenazi Jewish descent. Related Information What information about a genetic condition can statistics provide? Why are some genetic ...

  16. International Collaboration Patterns and Effecting Factors of Emerging Technologies.

    Directory of Open Access Journals (Sweden)

    Xu Bai

    Full Text Available With the globalization of the world economy, international innovation collaboration has taken place all over the world. This study selects three emerging technologies (3D printing, big data and carbon nanotubes and graphene technology among 20 countries as the research objects, using three patent-based indicators and network relationship analysis to reflect international collaboration patterns. Then we integrate empirical analyses to show effecting factors of international collaboration degrees by using panel data. The results indicate that while 3D printing technology is associated with a "balanced collaboration" mode, big data technology is more accurately described by a radial pattern, centered on the United States, and carbon nanotubes and graphene technology exhibits "small-world" characteristics in this respect. It also shows that the factors GDP per capita (GPC, R&D expenditure (RDE and the export of global trade value (ETV negatively affect the level of international collaboration. It could be useful for China and other developing countries to make international scientific and technological collaboration strategies and policies in the future.

  17. International Collaboration Enhances Cancer Screening Efforts

    Science.gov (United States)

    CGH is working with the International Agency for Research on CancerExit Disclaimer (IARC) and the Pan American Health Organization (PAHO) on the ESTAMPA Study, a multi-centric study of cervical cancer screening and triage with HPV testing.

  18. International research collaboration in maritime health

    DEFF Research Database (Denmark)

    Jensen, Olaf Chresten

    2011-01-01

    . The area is regulated by international standards based on international research-based knowledge on health and safety. Moreover, many of the world's seafarers come from developing countries with specific disease problems like HIV and no possibility of independent maritime health research. The international......The new ILO-2006-convention and the EU Commission's strategic objectives for the EU maritime transport policy 2008-2018, mentions the necessity of a modern health and safety system for maritime transportation. However, there is no specific strategy for the development of maritime health and safety...... maritime health research is sparse, and an increase in such research is necessary to help benefit needed shipping as a highly globalized industry. This paper presents an example of such research, accompanied by a discussion of methods and opportunities to increase international maritime health research....

  19. International Education Hubs: Collaboration for Competitiveness and Sustainability

    Science.gov (United States)

    Knight, Jane

    2014-01-01

    This chapter focuses on the development of education hubs, a recent phenomenon in international higher education. Three models of hubs are examined in relation to the forces, risks, and opportunities of globalization and how local and international collaborations are essential for both global competitiveness and sustainability.

  20. The North American Career Development Partnership: Experiment in International Collaboration.

    Science.gov (United States)

    Carslon, Burton L.; Goguen, Robert A.; Jarvis, Phillip S.; Lester, Juliette N.

    2000-01-01

    Describes how career development programs became the focus of an international partnership between the United States and Canada. Traces the history of each country's efforts, beginning in the 1970s, which led to this significant international collaboration. Concludes with a discussion of the lessons learned from these experiences. (Author/JDM)

  1. Women in global science advancing academic careers through international collaboration

    CERN Document Server

    Zippel, Kathrin

    2017-01-01

    Scientific and engineering research is increasingly global, and international collaboration can be essential to academic success. Yet even as administrators and policymakers extol the benefits of global science, few recognize the diversity of international research collaborations and their participants, or take gendered inequalities into account. Women in Global Science is the first book to consider systematically the challenges and opportunities that the globalization of scientific work brings to U.S. academics, especially for women faculty. Kathrin Zippel looks to the STEM fields as a case study, where gendered cultures and structures in academia have contributed to an underrepresentation of women. While some have approached underrepresentation as a national concern with a national solution, Zippel highlights how gender relations are reconfigured in global academia. For U.S. women in particular, international collaboration offers opportunities to step outside of exclusionary networks at home. International ...

  2. International Collaboration Patterns and Effecting Factors of Emerging Technologies

    Science.gov (United States)

    Bai, Xu; Liu, Yun

    2016-01-01

    With the globalization of the world economy, international innovation collaboration has taken place all over the world. This study selects three emerging technologies (3D printing, big data and carbon nanotubes and graphene technology) among 20 countries as the research objects, using three patent-based indicators and network relationship analysis to reflect international collaboration patterns. Then we integrate empirical analyses to show effecting factors of international collaboration degrees by using panel data. The results indicate that while 3D printing technology is associated with a “balanced collaboration” mode, big data technology is more accurately described by a radial pattern, centered on the United States, and carbon nanotubes and graphene technology exhibits “small-world” characteristics in this respect. It also shows that the factors GDP per capita (GPC), R&D expenditure (RDE) and the export of global trade value (ETV) negatively affect the level of international collaboration. It could be useful for China and other developing countries to make international scientific and technological collaboration strategies and policies in the future. PMID:27911926

  3. INTERNATIONAL COLLABORATION: US panel members named

    International Nuclear Information System (INIS)

    Anon.

    1994-01-01

    After the demise of the US Superconducting Supercollider (SSC) project, the US Secretary of Energy has been requested to produce a plan to 'maximize the value of the investment in the project and minimizing the loss to the US, including recommendations as to the feasibility of utilizing SSC assets in whole or in part in pursuit of an international high energy physics endeavour.'

  4. Immersion research education: students as catalysts in international collaboration research.

    Science.gov (United States)

    Anderson, K H; Friedemann, M L; Bűscher, A; Sansoni, J; Hodnicki, D

    2012-12-01

    This paper describes an international nursing and health research immersion program. Minority students from the USA work with an international faculty mentor in teams conducting collaborative research. The Minority Health International Research Training (MHIRT) program students become catalysts in the conduct of cross-cultural research. To narrow the healthcare gap for disadvantaged families in the USA and partner countries. Faculty from the USA, Germany, Italy, Colombia, England, Austria and Thailand formed an international research and education team to explore and compare family health issues, disparities in chronic illness care, social inequities and healthcare solutions. USA students in the MHIRT program complete two introductory courses followed by a 3-month research practicum in a partner country guided by faculty mentors abroad. The overall program development, student study abroad preparation, research project activities, cultural learning, and student and faculty team outcomes are explored. Cross-fertilization of research, cultural awareness and ideas about improving family health occur through education, international exchange and research immersion. Faculty research and international team collaboration provide opportunities for learning about research, health disparities, cultural influences and healthcare systems. The students are catalysts in the research effort, the dissemination of research findings and other educational endeavours. Five steps of the collaborative activities lead to programmatic success. MHIRT scholars bring creativity, enthusiasm, and gain a genuine desire to conduct health research about families with chronic illness. Their cultural learning stimulates career plans that include international research and attention to vulnerable populations. © 2012 The Authors. International Nursing Review © 2012 International Council of Nurses.

  5. National and International Library Collaboration: Necessity, Advantages

    Directory of Open Access Journals (Sweden)

    Timothy Mark

    2007-11-01

    Full Text Available The advantages of cooperation between research library associations can be demonstrated quite clearly. Where cooperation becomes essential is where there is a common cause to champion or an initiative to pursue for the common good. Thanks in part to the power of information communications technology, research is becoming increasingly international in scope. The members of interdisciplinary and multidisciplinary research teams are frequently drawn from across the world. Correspondingly, scholarly publishing (especially in medicine and the sciences is dominated by a few multinational publishers. In this context, issues that play a vital role in global scholarly communication - such as copyright, journal pricing, the managing of datasets, digital preservation and open access - are issues for the research library community everywhere in the world. Certainly there are many challenges at the local or regional level. However there are definite roles that research library associations can play most effectively at the national or international level. It is clear that by sharing expertise, building partnerships and alliances, it is possible to address common issues much more effectively than when working alone. What is special is that potentially the most powerful form of cooperation is advocacy at the national and international levels. It is essential for research library associations to work together at the political level to move their agenda forward. Examples are lobbying about copyright legislation (nationally, or within the EC or at WIPO; or promoting the cause of Open Access. The unique value of LIBER is to provide a forum and a platform for European research library associations to explore and benefit from cooperation and their shared strength.

  6. Astronomical Virtual Observatories Through International Collaboration

    Directory of Open Access Journals (Sweden)

    Masatoshi Ohishi

    2010-03-01

    Full Text Available Astronomical Virtual Observatories (VOs are emerging research environment for astronomy, and 16 countries and a region have funded to develop their VOs based on international standard protocols for interoperability. The 16 funded VO projects have established the International Virtual Observatory Alliance (http://www.ivoa.net/ to develop the standard interoperable interfaces such as registry (meta data, data access, query languages, output format (VOTable, data model, application interface, and so on. The IVOA members have constructed each VO environment through the IVOA interfaces. National Astronomical Observatory of Japan (NAOJ started its VO project (Japanese Virtual Observatory - JVO in 2002, and developed its VO system. We have succeeded to interoperate the latest JVO system with other VOs in the USA and Europe since December 2004. Observed data by the Subaru telescope, satellite data taken by the JAXA/ISAS, etc. are connected to the JVO system. Successful interoperation of the JVO system with other VOs means that astronomers in the world will be able to utilize top-level data obtained by these telescopes from anywhere in the world at anytime. System design of the JVO system, experiences during our development including problems of current standard protocols defined in the IVOA, and proposals to resolve these problems in the near future are described.

  7. ICFA: Protvino meeting looks at trends in international collaboration

    International Nuclear Information System (INIS)

    Anon.

    1991-01-01

    International collaboration is the lifeblood of Big Science, and in high energy physics the triennial 'Future Perspectives' meeting organized by the International Committee for Future Accelerators (ICFA) provides a valuable opportunity to reappraise trends in this collaboration. The latest meeting was held in October at Protvino, near Moscow, where the Institute for High Energy Physics is the scene of construction work for the 21-kilometre UNK proton rings and the projected home of a big new linear collider for electrons and positrons

  8. Gaucher's disease: Magnetic resonance findings

    International Nuclear Information System (INIS)

    Roca, M.; Gomez-Pereda, R.; Blasco, A.; Ros, L.

    1996-01-01

    The objective is to assess the role of magnetic resonance (MR) in determining the initial extension of Gaucher's disease and its complications. A retrospective study of eight patients diagnosed as having Gaucher's disease was carried out using MR. The study focused on pelvis, hip, femur, spine, liver parenchyma and splenic parenchyma. Infiltration of the cancellous portion of the vertebral bodies was observed in all but one of the patients. Three patients presented small hemangiomas in dorsal and lumbar vertebral bodies. Pelvic bone involvement was homogeneous in four cases and spotty in two, while the pelvic marrow was normal in the two patients with no vertebral infiltration. A vascular necrosis of the femoral head was detected in two cases. MR is very useful in determining the initial extension, in the early diagnosis of complications and in managing the posttreatment marrow response to assess the therapeutic efficacy. 16 refs

  9. Bibliometric Analysis of International Collaboration in Wind and Solar Energy

    Directory of Open Access Journals (Sweden)

    Ichiro Sakata

    2013-09-01

    Full Text Available Modern technology is increasingly complex and demands an ever-widening range of knowledge and skills. No single country will possess all the knowledge and skills required for addressing global issues such as climate change. Technology collaboration between leading countries is important to promptly and efficiently address the problem. Previous studies have shown that a high level of collaboration is correlated with high paper productivity. This paper first aims to use objective data and create maps that enable us to see both the distribution of worldwide research competency and the relationship of international collaboration in clean energy research. In the international research network of wind power and solar cell, 4,189 institutions located in 121 countries and 6,600 institutions located in 125 countries are included respectively. This paper discusses various factors that would have an impact on research capability and support strong international relationships. With respect to research capability, governmental policies, stability of governmental commitment, natural conditions and historical and institutional differences have a significant impact on it. For research collaborations, factors such as geographical proximity, international science and technology policy, and developmental stage of technology have been brought to attention. This study demonstrates that bibliometrics is a methodology that is capable of providing a knowledge base that is useful in the development of the international science and technology policy and technological management strategy.

  10. International Collaborative Research Partnerships: Blending Science with Management and Diplomacy.

    Science.gov (United States)

    Lau, Chuen-Yen; Wang, Crystal; Orsega, Susan; Tramont, Edmund C; Koita, Ousmane; Polis, Michael A; Siddiqui, Sophia

    2014-12-01

    As globalization progressively connects and impacts the health of people across the world, collaborative research partnerships provide mutual advantages by sharing knowledge and resources to address locally and globally relevant scientific and public health questions. Partnerships undertaken for scientific research are similar to business collaborations in that they require attention to partner systems, whether local, international, political, academic, or non-academic. Scientists, like diplomats or entrepreneurs, are representatives of their field, culture, and country and become obligatory agents in health diplomacy. This role significantly influences current and future collaborations with not only the immediate partner but with other in country partners as well. Research partnerships need continuous evaluation of the collaboration's productivity, perspectives of all partners, and desired outcomes for success to avoid engaging in "research tourism", particularly in developing regions. International engagement is a cornerstone in addressing the impact of infectious diseases globally. Global partnerships are strategically aligned with national, partner and global health priorities and may be based on specific requests for assistance from the partnering country governments. Here we share experiences from select research collaborations to highlight principles that we have found key in building long-term relationships with collaborators and in meeting the aim to address scientific questions relevant to the host country and strategic global health initiatives.

  11. Juvenile Gaucher disease simulating osteomyelitis

    International Nuclear Information System (INIS)

    Miller, J.H.; Ortega, J.A.; Heisel, M.A.

    1981-01-01

    A case in which several imaging procedures suggested juvenile Gaucher disease in a child who presented with symptomatology of osteomyelitis is discussed. The 20-month girl was given a Technetium-99m radionuclide skeletal examination which revealed intense uptake of tracer agents along the shaft of the right femur. It was also found that the liver and spleen were dramatically Ga-67 avid. The bone pain symptomatology suggested an osteomyelitis of the femur, but skeletal scintigraphy with Tc-99m-labeled bone tracer demonstrated photopenic areas involving the femur, suggesting that the bone pain may have been due to marrow packed with Gaucher cells. This overexpansion of the marrow may lead to microfractures with remodeling seen radiographically as periosteal new bone and scintigraphically as increased periosteal deposition of tracer agent. The radiogallium study was useful to exclude an underlying osteomyelitis in the involved femurs. Although juvenile Gaucher disease is unusual, it should be considered in any child who presents with the constellation of hepatosplenomegaly and bone pain simulating osteomyelitis

  12. Scintigraphic findings in Gaucher's disease

    International Nuclear Information System (INIS)

    Israel, O.; Jerushalmi, J.; Front, D.

    1986-01-01

    Gaucher's disease involves the liver, the spleen, and the bone. Liver-spleen and bone scintigraphy were used during an 8-yr period to evaluate changes caused by this disease. Patients were investigated with a liver-spleen scan for abdominal pain, mechanical discomfort, enlarged liver or spleen on physical examination, abdominal mass, abnormal liver function tests, and symptoms of hypersplenism. Fourteen liver-spleen scans were performed in nine patients. Liver scintigraphy showed various degrees of enlargement and inhomogeneous uptake. In two patients focal defects were detected. In one, focal defects were due to liver involvement with Gaucher's disease, but in the other they were caused by metastatic pancreatic carcinoma. The study was also useful in detecting splenic infarction and in following enlargement of the spleen after partial splenectomy. The main indication for bone scintigraphy in six patients was bone pain. This was found to be caused by either aseptic necrosis of the head of the femur, bone infarction, pathological fractures, or osteomyelitis. Loosening after total hip replacement was ruled out in three patients and missed in one patient. Scintigraphy appears to be a simple, sensitive test for evaluation of the liver, spleen, and bony skeleton in patients with symptomatic Gaucher's disease

  13. Juvenile Gaucher disease simulating osteomyelitis

    Energy Technology Data Exchange (ETDEWEB)

    Miller, J.H.; Ortega, J.A.; Heisel, M.A.

    1981-10-01

    A case in which several imaging procedures suggested juvenile Gaucher disease in a child who presented with symptomatology of osteomyelitis is discussed. The 20-month girl was given a Technetium-99m radionuclide skeletal examination which revealed intense uptake of tracer agents along the shaft of the right femur. It was also found that the liver and spleen were dramatically Ga-67 avid. The bone pain symptomatology suggested an osteomyelitis of the femur, but skeletal scintigraphy with Tc-99m-labeled bone tracer demonstrated photopenic areas involving the femur, suggesting that the bone pain may have been due to marrow packed with Gaucher cells. This overexpansion of the marrow may lead to microfractures with remodeling seen radiographically as periosteal new bone and scintigraphically as increased periosteal deposition of tracer agent. The radiogallium study was useful to exclude an underlying osteomyelitis in the involved femurs. Although juvenile Gaucher disease is unusual, it should be considered in any child who presents with the constellation of hepatosplenomegaly and bone pain simulating osteomyelitis.

  14. International Charter `Space and Major Disasters' Collaborations

    Science.gov (United States)

    Jones, B. K.

    2017-12-01

    The International Charter aims at providing a unified system of space data acquisition and delivery to national disaster authorities of countries affected by natural or man-made disasters. Each of the sixteen Member Agencies has committed resources to support the objectives of the Charter and thus helping to mitigate the effects of disasters on human life and property, getting critical information into the hands of the disaster responders so that they can make informed decisions in the wake of a disaster. The Charter Member Agencies work together to provide remotely sensed imagery to any requesting country that is experiencing a natural or man-made disaster. The Space Agencies contribute priority satellite taskings, archive retrievals, and map production, as well as imagery of the affected areas. The imagery is provided at no cost to the affected country and is made available for the immediate response phase of the disaster. The Charter also has agreements with Sentinel Asia to submit activation requests on behalf of its 30+ member countries and the United Nations Office of Outer Space Affairs (UN OOSA) and United Nations Institute for Training and Research (UNITAR)/ United Nations Operational Satellite Applications Programme (UNOSAT) to submit activations on behalf of United Nations relief agencies such as UNICEF and UNOCHA. To further expand accessibility to the Charter Member Agency resources, the Charter has implemented the Universal Access initiative, which allows any country's disaster management authority to submit an application, attend a brief training session, and after successful completion, become an Authorized User able to submit activation requests without assistance from Member Agencies. The data provided by the Charter is used for many purposes including damage assessments, reference maps, evacuation route planning, search and rescue operations, decision maker briefings, scientific evaluations, and other response activities.

  15. International R&D collaboration networks and free trade agreements

    OpenAIRE

    Song, Hua Sheng

    2006-01-01

    This thesis contributes to the analysis of optimal industrial and strategic trade policy in the presence of oligopoly and other forms of imperfect competition, so as to make contact with important empirical regularities and policy concerns, such as international R&D collaboration, unionization and free trade. First, in the context of international competition in which R&D plays an important role, we study the consequences of allowing governments to subsidize R&D and coalition devi...

  16. Neurolymphomatosis: An International Primary CNS Lymphoma Collaborative Group report

    NARCIS (Netherlands)

    S. Grisariu (Sigal); B. Avni (Batia); T.T. Batchelor (Tracy); M.J. van den Bent (Martin); F. Bokstein (Felix); D. Schiff (David); O. Kuittinen (Outi); M.C. Chamberlain (Marc C.); P. Roth (Patrick); A. Nemets (Anatoly); E. Shalom (Edna); D. Ben-Yehuda (Dina); T. Siegal (Tali)

    2010-01-01

    textabstractNeurolymphomatosis (NL) is a rare clinical entity. The International Primary CNS Lymphoma Collaborative Group retrospectively analyzed 50 patients assembled from 12 centers in 5 countries over a 16-year period. NL was related to non-Hodgkin lymphoma in 90% and to acute leukemia in 10%.

  17. Gendered Patterns in International Research Collaborations in Academia

    Science.gov (United States)

    Uhly, K. M.; Visser, L. M.; Zippel, K. S.

    2017-01-01

    Although women's representation in higher education nears parity with men at the undergraduate level, this representation diminishes as one ascends the academic ranks. Because gender gaps in the "elite" activity of international research collaborations might contribute to the underrepresentation of women in the upper ranks, we ask if…

  18. Gendered patterns in international research collaborations in academia

    NARCIS (Netherlands)

    Uhly, K.M.; Visser, L.M.; Zippel, K.S.

    2017-01-01

    Although women's representation in higher education nears parity with men at the undergraduate level, this representation diminishes as one ascends the academic ranks. Because gender gaps in the ‘elite’ activity of international research collaborations might contribute to the underrepresentation of

  19. International funding opportunities for ideas of collaboration projects in OOHP

    Directory of Open Access Journals (Sweden)

    Ilin, Corina

    2012-04-01

    Full Text Available The present paper is presenting the exploratory workshop organized by the OOHP Master team in December 2011, which opened paths for theoretical knowledge and for collaboration with researchers having international prestige. Also, the paper presents funding opportunities available in national grants, and the strategy chosen by the team to prepare a two-step access to funds.

  20. BRICS and International Collaborations in Higher Education in India

    Science.gov (United States)

    Varghese, N. V.

    2015-01-01

    International cooperation and collaborations played an important role in the economic and educational development of several countries. In the 1950s and 1960s external aid was an important modality to establish cooperation between countries, especially between developing and developed countries. Cross-border activities in higher education used to…

  1. Treatment patterns from 647 patients with Gaucher disease: An analysis from the Gaucher Outcome Survey.

    Science.gov (United States)

    Deegan, P; Fernandez-Sasso, D; Giraldo, P; Lau, H; Panahloo, Z; Zimran, A

    2018-02-01

    The Gaucher Outcome Survey (GOS) is an international disease-specific registry established in 2010 for patients with a confirmed diagnosis of Gaucher disease (GD), regardless of GD type or treatment status. For insight into how GD management varies among countries, we analyzed treatment patterns in GOS. As of October 30, 2015, data on GD-specific treatment (enzyme replacement therapy, substrate reduction therapy, or chemical chaperone therapy) received at any time were available for 647 patients. At analysis, velaglucerase alfa (316/573, 55.1%) and imiglucerase (184/573, 32.1%) were the treatments most widely used. Of the 647 treated patients, 446 (68.9%) had been treated for >5years and 368 (56.9%) had received only one GD-specific drug therapy. There were 377 patients who received velaglucerase alfa. Velaglucerase alfa was most widely used at 60U/kg every other week (134/492 dose entries, 27.2%), but there were differences in dosing between the three highest-enrolling countries (defined as >100 GOS patients enrolled in each), with most patients in Israel receiving <20U/kg, most patients in the United Kingdom receiving 20 to <40U/kg, and most in the United States receiving 60U/kg. This analysis provides a foundation upon which to examine real-life outcomes data from different treatment regimens globally. Copyright © 2016 Shire Human Genetic Therapies, Inc. Published by Elsevier Inc. All rights reserved.

  2. Strengthening International Collaboration: Geosciences Research and Education in Developing Countries

    Science.gov (United States)

    Fucugauchi, J. U.

    2009-05-01

    Geophysical research increasingly requires global multidisciplinary approaches and global integration. Global warming, increasing CO2 levels and increased needs of mineral and energy resources emphasize impact of human activities. The planetary view of our Earth as a deeply complex interconnected system also emphasizes the need of international scientific cooperation. International collaboration presents an immense potential and is urgently needed for further development of geosciences research and education. In analyzing international collaboration a relevant aspect is the role of scientific societies. Societies organize meetings, publish journals and books and promote cooperation through academic exchange activities and can further assist communities in developing countries providing and facilitating access to scientific literature, attendance to international meetings, short and long-term stays and student and young researcher mobility. Developing countries present additional challenges resulting from limited economic resources and social and political problems. Most countries urgently require improved educational and research programs. Needed are in-depth analyses of infrastructure and human resources and identification of major problems and needs. Questions may include what are the major limitations and needs in research and postgraduate education in developing countries? what and how should international collaboration do? and what are the roles of individuals, academic institutions, funding agencies, scientific societies? Here we attempt to examine some of these questions with reference to case examples and AGU role. We focus on current situation, size and characteristics of research community, education programs, facilities, economic support, and then move to perspectives for potential development in an international context.

  3. Health literacy: setting an international collaborative research agenda

    Directory of Open Access Journals (Sweden)

    Rowlands Gillian

    2009-07-01

    Full Text Available Abstract Background Health literacy is an increasingly important topic in both the policy and research agendas of many countries. During the recent 36th Annual Meeting of the North American Primary Care Research Group, the authors led an audio-taped 3-hour forum, "Studying Health Literacy: Developing an International Collaboration," where the current state of health literacy (HL in the United States (US and United Kingdom (UK was presented and attendees were encouraged to debate a future research agenda. Discussion of Forum Themes The debate centred around three distinct themes, including: (1 refining HL definitions and conceptual models, (2 HL measurement and assessment tools, and (3 developing a collaborative international research agenda. The attendees agreed that future research should be theoretically grounded and conceptual models employed in studies should be explicit to allow for international comparisons to be drawn. Summary and Authors Reflections The importance of HL research and its possible contribution to health disparities is becoming increasingly recognised internationally. International collaborations and comparative studies could illuminate some of the possible determinants of disparities, and also possibly provide a vehicle to examine other research questions of interest.

  4. [Type 3 Gaucher disease, also an adult disease?

    Science.gov (United States)

    Leurs, A; Chepy, A; Detonellaere, C; Pascal, L; Gallois, P; Tran, T-A-C; Caillaud, C; Hatron, P-Y; Rose, C

    2018-03-30

    Gaucher disease is a genetic lysosomal storage disorder due to a glucocerebrosidase deficiency. Type 3, including neurological impairment, may have a specific phenotype in the context of the D409H mutation. We report the case of a 22-year-old woman who presented with Gaucher disease. Enzyme replacement therapy by imiglucerase was followed by rapid clinical and biological improvement. However, communication difficulties, which were initially attributed to the language barrier, revealed neurological impairment. After complementary assessment, the diagnosis of type 3 Gaucher disease was suspected. Gene analysis of the glucocerebrosidase showed a homozygous D409H mutation. This mutation results in calcified heart valves, corneal opacities, alteration of oculomotricity and hydrocephalus. The mild manifestation at onset and the late neurological involvement in the medical history make the diagnosis more difficult. This particular clinical phenotype deserves to be known in adult medicine departments. Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  5. Magnetic resonance spectroscopy in patients with Fabry and Gaucher disease

    International Nuclear Information System (INIS)

    Gruber, S.; Bogner, W.; Stadlbauer, A.; Krssak, M.; Bodamer, O.

    2011-01-01

    Objective: Fabry and Gaucher diseases are rare progressive inherited disorders of glycosphingolipid metabolism that affect multiple organ systems. The aim of this study was to investigate evidence for metabolic changes in the central nervous system involvement using proton magnetic resonance spectroscopic imaging. Methods: Seven Fabry and eight Gaucher patients were included into this study. A two-dimensional, spectroscopic imaging method with an ultra-short echo-time of 11 ms was used at a 3 T whole body magnet. Absolute metabolic values were retrieved using internal water scaling. Results were compared, with sex- and age-matched controls. Results: In contrast to previous findings, absolute and relative metabolite values of N-acetyl-aspartate (NAA) or NAA/Creatine (Cr), Cr, Choline (Cho) or Cho/Cr and myo-Inositol (mI) or mI/Cr revealed no, differences between Fabry and Gaucher Type 1 (GD1) patients and controls. Average values were, 10.22, 6.32, 2.15 and 5.39 mMol/kg wet weight for NAA, Cr, Cho and mI, respectively. In this study, we found significantly decreasing NAA/Cho with increasing age in all three groups (Fabry, GD1, patients and healthy controls) (between 5 and 8% per decade). Conclusions: There were no changes of the quantified metabolites detected by MRS in normal appearing white matter. This study shows the importance of sex- and age-matched controls.

  6. Magnetic resonance spectroscopy in patients with Fabry and Gaucher disease

    Energy Technology Data Exchange (ETDEWEB)

    Gruber, S., E-mail: stephan@nmr.at [Department of Radiology, MR-Centre of Excellence, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna (Austria); Bogner, W. [Department of Radiology, MR-Centre of Excellence, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna (Austria); Stadlbauer, A. [MR Physics Group, Department of Radiology, Landesklinikum St. Poelten (Austria); Krssak, M. [Department of Radiology, MR-Centre of Excellence, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna (Austria); Bodamer, O. [Department of Pediatrics, Medical University of Vienna (Austria)

    2011-08-15

    Objective: Fabry and Gaucher diseases are rare progressive inherited disorders of glycosphingolipid metabolism that affect multiple organ systems. The aim of this study was to investigate evidence for metabolic changes in the central nervous system involvement using proton magnetic resonance spectroscopic imaging. Methods: Seven Fabry and eight Gaucher patients were included into this study. A two-dimensional, spectroscopic imaging method with an ultra-short echo-time of 11 ms was used at a 3 T whole body magnet. Absolute metabolic values were retrieved using internal water scaling. Results were compared, with sex- and age-matched controls. Results: In contrast to previous findings, absolute and relative metabolite values of N-acetyl-aspartate (NAA) or NAA/Creatine (Cr), Cr, Choline (Cho) or Cho/Cr and myo-Inositol (mI) or mI/Cr revealed no, differences between Fabry and Gaucher Type 1 (GD1) patients and controls. Average values were, 10.22, 6.32, 2.15 and 5.39 mMol/kg wet weight for NAA, Cr, Cho and mI, respectively. In this study, we found significantly decreasing NAA/Cho with increasing age in all three groups (Fabry, GD1, patients and healthy controls) (between 5 and 8% per decade). Conclusions: There were no changes of the quantified metabolites detected by MRS in normal appearing white matter. This study shows the importance of sex- and age-matched controls.

  7. Enhancing international collaboration among early-career researchers

    Science.gov (United States)

    Carroll, Jennifer K; Albada, Akke; Farahani, Mansoureh; Lithner, Maria; Neumann, Melanie; Sandhu, Harbinder; Shepherd, Heather L

    2010-01-01

    Objective The European Association of Communication in Healthcare (EACH) Early Career Researchers Network (ECRN) aims are to (1) promote international collaboration among young investigators and (2) provide a support network for future innovative communication research projects. In October 2009, Miami, USA at a workshop facilitated by the ECRN at the International Conference on Communication in Healthcare (ICCH) hosted by the American Academy of Communication in Healthcare we explored common facilitators and challenges faced by early career researchers in health communication research. Methods Attendees introduced themselves, their research area(s) of interest, and listed one facilitator and one barrier for their career development. EACH ECRN members then led a discussion of facilitators and challenges encountered in communication research projects and career development. We discussed potential collaboration opportunities, future goals, and activities. Results Having supportive collegial relationships, institutional support, job security, and funding are critical facilitators for early career investigators. Key challenges include difficulty with time management and prioritizing, limited resources, and contacts. Conclusion International collaboration among early career researchers is a feasible and effective means to address important challenges, by increasing opportunities for professional support and networking, problem-solving, discussion of data, and ultimately publishing. Practice Implications Future AACH-EACH Early Career Researcher Networks should continue to build collaborations by developing shared research projects, papers, and other scholarly products. PMID:20663630

  8. Power Institutions and International Collaboration on the Kola Peninsula

    Directory of Open Access Journals (Sweden)

    Geir Hønneland

    2006-05-01

    Full Text Available The article discusses how international cooperative projects have contributed to increased interaction between civilian authorities and the military or other power agencies in Murmansk Oblast. The cases of fisheries enforcement, nuclear safety and the fight against communicable diseases, especially tuberculosis in prisons, are reviewed. The main lesson is that international collaboration ventures can sometimes provide arenas for initiating new coordination patterns that would otherwise not have evolved. Occasionally, the international project is simply the pretext necessary for changing a situation that both civilian and power agencies view as irrational. Whether these changes are fundamental and structural, however, remains to be seen.

  9. Mongolize or Westernize - international collaboration in educational change

    DEFF Research Database (Denmark)

    Baltzersen, Johnny

    The paper presented at the 10th International Congress of Mongolists, Ulaanbaatar, August 2011 discuss conflicting approaches to international collaboration in development aid with Mongolia as a case. The paper introduces the dilemmas facing education reform in Mongolia after the collapse of soci...... of socialism in 1990 and Mongolia's struggle to find a balance between (re)defining a Mongolian-based philosophical and practical foundation guiding education development and the flood of Western-based ideas following the international donor funded aid programs....

  10. International Collaboration in Satellite Observations for Disaster Management

    Science.gov (United States)

    Duda, Kenneth A.; Abrams, Michael

    2012-01-01

    When lives are threatened or lost due to catastrophic disasters, and when massive financial impacts are experienced, international emergency response teams rapidly mobilize to provide urgently required support. Satellite observations of affected areas often provide essential insight into the magnitude and details of the impacts. The large cost and high complexity of developing and operating satellite flight and ground systems encourages international collaboration in acquiring imagery for such significant global events in order to speed delivery of critical information to help those affected, and optimize spectral, spatial, and temporal coverage of the areas of interest. The International Charter-Space and Major Disasters was established to enable such collaboration in sensor tasking during times of crisis and is often activated in response to calls for assistance from authorized users. Insight is provided from a U.S. perspective into sensor support for Charter activations and other disaster events through a description of the Advanced Spaceborne Thermal Emission and Reflection Radiometer (ASTER), which has been used to support emergency situations for over a decade through its expedited tasking and near real-time data delivery capabilities. Examples of successes achieved and challenges encountered in international collaboration to develop related systems and fulfill tasking requests suggest operational considerations for new missions as well as areas for future enhancements.

  11. International Collaboration in the Development of NPP Software

    International Nuclear Information System (INIS)

    Jiang, S.; Liu, L.; Yu, H.

    2015-01-01

    In this paper, we first review the progress and current status of international collaboration and technical exchange in the development of nuclear power plant (NPP) software by The State Nuclear Power Software Development Center (SNPSDC) in China. Then we discuss the importance of the international collaboration and exchange in the trend of globalisation of NPP technology. We also identify the role and contribution of professional women in this process. SNPSDC, the first professional software development centre for NPP in China, has been developing COSINE — a self-reliance NPP design and analysis software product with China brand—since 2010. Through participating in OECD/NEA’s joint projects, such as ROSA-2 Project, PKL–3 Project, HYMERES Project and ATLAS Project, SNPSDC shared data with other countries involved with respect to particular areas, such as high quality reactor thermal hydraulics test data. SNPSDC’s engineers have also been actively participating in international technical and research exchange for presenting their innovative work to the community while learning from peers. Our record shows that over 30 papers have been presented in international conferences with respect to nuclear reactor thermal hydraulics, safety analysis, reactor physics and software engineering within the past 4 years. The above international collaboration and technical exchange helped SNPSDC’s engineers to keep up with the state-of-art technology in this field. The large amount of valuable experimental data transferred to SNPSDC ensured the functionality, usability and reliability of software while greatly reduced the cost and shortened the cycle of development. Female engineers and other employees of SNPSDC either drove or got actively involved in a lot of aspects of the above collaboration and exchange, such as technical communication, business negotiation and overseas affairs management. These professional women played an irreplaceable role in this project by

  12. Reasoning about the value of cultural awareness in international collaboration

    Directory of Open Access Journals (Sweden)

    Helena Bernáld

    Full Text Available As international collaborations become a part of everyday life, cultural awareness becomes crucial for our ability to work with people from other countries. People see, evaluate, and interpret things differently depending on their cultural background and cultural awareness. This includes aspects such as appreciation of different communication patterns, the awareness of different value systems and, not least, to become aware of our own cultural values, beliefs and perceptions. This paper addresses the value of cultural awareness in general through describing how it was introduced in two computer science courses with a joint collaboration between students from the US and Sweden. The cultural seminars provided to the students are presented, as well as a discussion of the students\\' reflections and the teachers\\' experiences. The cultural awareness seminars provided students with a new understanding of cultural differences which greatly improved the international collaboration. Cultural awareness may be especially important for small countries like New Zealand and Sweden, since it could provide an essential edge in collaborations with representatives from more \\'powerful\\' countries.

  13. Status Report on Laboratory Testing and International Collaborations in Salt.

    Energy Technology Data Exchange (ETDEWEB)

    Kuhlman, Kristopher L. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Matteo, Edward N. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Hadgu, Teklu [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Reedlunn, Benjamin [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Sobolik, Steven R. [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Mills, Melissa Marie [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Kirkes, Leslie Dawn [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Xiong, Yongliang [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Icenhower, Jonathan [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2017-09-01

    This report is a summary of the international collaboration and laboratory work funded by the US Department of Energy Office of Nuclear Energy Spent Fuel and Waste Science & Technology (SFWST) as part of the Sandia National Laboratories Salt R&D work package. This report satisfies milestone levelfour milestone M4SF-17SN010303014. Several stand-alone sections make up this summary report, each completed by the participants. The first two sections discuss international collaborations on geomechanical benchmarking exercises (WEIMOS) and bedded salt investigations (KOSINA), while the last three sections discuss laboratory work conducted on brucite solubility in brine, dissolution of borosilicate glass into brine, and partitioning of fission products into salt phases.

  14. Norway's role in international collaboration towards rehabilitation of Andreeva Bay.

    Science.gov (United States)

    Dowdall, M; Sneve, M; Standring, W J F; Amundsen, I

    2009-12-01

    Andreeva Bay is one of the largest and most hazardous nuclear legacy sites in northwest Russia. The site is the location of large amounts of Spent Nuclear Fuel (SNF) and radioactive wastes and the risks associated with the site have precipitated an extensive international collaborative effort towards securing and rehabilitating the site. Given the location and proximity of the site, Norway has and continues to contribute in a number of ways towards this effort. Norway's activities in relation to rehabilitative efforts at Andreeva Bay are focused on both infrastructural and remediative initiatives as well as regulatory collaboration with Russia towards ensuring effective and safe operations during handling and removal of SNF and radioactive materials. This article describes Norway's role within international efforts in the context of the rehabilitation of Andreeva Bay and outlines previous activities and Norway's future direction with respect to the site.

  15. Efferocytosis is impaired in Gaucher macrophages.

    Science.gov (United States)

    Aflaki, Elma; Borger, Daniel K; Grey, Richard J; Kirby, Martha; Anderson, Stacie; Lopez, Grisel; Sidransky, Ellen

    2017-04-01

    Gaucher disease, the inherited deficiency of lysosomal glucocerebrosidase, is characterized by the presence of glucosylceramide-laden macrophages resulting from impaired digestion of aged erythrocytes or apoptotic leukocytes. Studies of macrophages from patients with type 1 Gaucher disease with genotypes N370S/N370S, N370S/L444P or N370S/c.84dupG revealed that Gaucher macrophages have impaired efferocytosis resulting from reduced levels of p67 phox and Rab7. The decreased Rab7 expression leads to impaired fusion of phagosomes with lysosomes. Moreover, there is defective translocation of p67 phox to phagosomes, resulting in reduced intracellular production of reactive oxygen species. These factors contribute to defective deposition and clearance of apoptotic cells in phagolysosomes, which may have an impact on the inflammatory response and contribute to the organomegaly and inflammation seen in patients with Gaucher disease. Copyright© Ferrata Storti Foundation.

  16. Gaucher disease of the liver: CT appearance

    International Nuclear Information System (INIS)

    Glass, R.B.J.; Poznanski, A.K.; Young, S.; Urban, M.A.

    1987-01-01

    We present a child with Gaucher disease with hepatic involvement that caused portal hypertension. Computerized tomography (CT) showed distortion of liver parenchyma and central necrosis of the liver. (orig.)

  17. Key Success Factors and Guidance for International Collaborative Design Projects

    Directory of Open Access Journals (Sweden)

    Robby Soetanto

    2015-11-01

    Full Text Available In the built environment (BE sector, the co-creation process of design demands understanding of requirements (as viewed by parties involved, mobilisation of tacit knowledge, negotiation, and complex exchange of information. The need to collaborate over distance has further exacerbated the complexity of the process, and, in itself, represents a significant challenge for BE professionals who are increasingly expected to undertake this process within globally distributed virtual teams. The research aims to identify key success factors and develop guidance for international collaborative design projects, via the implementation of collaborative design courses in UK and Canadian universities over three academic years. Questionnaire surveys, focus groups, observation of online meetings, personal reflections provided data for the analysis. The findings reveal the significance of the perceived risk of collaboration and a difference in preferred communication mode between architects and civil/structural engineers. These findings suggest the impact of training in the subject discipline, and that the opportunity for co-located working has helped the development of trust. The guidance is aimed at BE educators who wish to implement this activity in their courses.

  18. Therapeutic goals in the treatment of Gaucher disease

    NARCIS (Netherlands)

    Pastores, Gregory M.; Weinreb, Neal J.; Aerts, Hans; Andria, Generoso; Cox, Timothy M.; Giralt, Manuel; Grabowski, Gregory A.; Mistry, Pramod K.; Tylki-Szymańska, Anna

    2004-01-01

    Gaucher disease, the most common lysosomal storage disorder, is a heterogeneous multisystem condition. Patients with non-neuronopathic (type 1) Gaucher disease may suffer from hepatomegaly, splenomegaly, thrombocytopenia, bleeding tendencies, anemia, hypermetabolism, skeletal pathology, growth

  19. Extraosseous Gaucher cell deposition without adjacent bone involvement.

    Science.gov (United States)

    Meyer, Brendan J; Mills, Anne M; Gaskin, Cree M

    2014-10-01

    Extraosseous Gaucher cell deposits are a rare complication of Gaucher disease that can mimic malignancy. We describe a case of Gaucher cell deposition in the subcutaneous soft tissues overlying the lower thoracic spine in an 18-year-old woman with known type III Gaucher disease. This case is unique in the literature because this subcutaneous Gaucher mass was not associated with extension from underlying bone involvement or clear lymph node origin. It demonstrated no discernible continuity with the adjacent thoracic spinous processes, the cortices of which appeared intact. Although patients with Gaucher disease are at increased risk of malignancy, Gaucher cell deposition should remain a differential consideration for soft tissue masses with or without adjacent bone involvement in patients with known Gaucher disease.

  20. CCL18: a urinary marker of Gaucher cell burden in Gaucher patients

    NARCIS (Netherlands)

    Boot, Rolf G.; Verhoek, Marri; Langeveld, Mirjam; Renkema, G. Herma; Hollak, Carla E. M.; Weening, Jan J.; Donker-Koopman, Wilma E.; Groener, Johanna E.; Aerts, Johannes M. F. G.

    2006-01-01

    Glucosylceramide-laden tissue macrophages in Gaucher patients secrete large quantities of chitotriosidase and CC chemokine ligand 18 (CCL18), resulting in markedly increased plasma levels. We have comparatively investigated the occurrence of both parameters in plasma and urine samples of Gaucher

  1. Collaborating internationally on physician leadership education: first steps.

    Science.gov (United States)

    Matlow, Anne; Chan, Ming-Ka; Bohnen, Jordan David; Blumenthal, Daniel Mark; Sánchez-Mendiola, Melchor; de Camps Meschino, Diane; Samson, Lindy Michelle; Busari, Jamiu

    2016-07-04

    Purpose Physicians are often ill-equipped for the leadership activities their work demands. In part, this is due to a gap in traditional medical education. An emergent international network is developing a globally relevant leadership curriculum for postgraduate medical education. The purpose of this article is to share key learnings from this process to date. Design/methodology/approach The Toronto International Summit on Leadership Education for Physicians (TISLEP) was hosted by the Royal College of Physicians and Surgeons of Canada, and the University of Toronto's Faculty of Medicine and Institute of Health Policy, Management and Evaluation. Of 64 attendees from eight countries, 34 joined working groups to develop leadership competencies. The CanMEDS Competency Framework, stage of learner development and venue of learning formed the scaffold for the work. Emotional intelligence was selected as the topic to test the feasibility of fruitful international collaboration; results were presented at TISLEP 2015. Findings Dedicated international stakeholders engaged actively and constructively through defined working groups to develop a globally relevant, competency-based curriculum for physician leadership education. Eleven principles are recommended for consideration in physician leadership curriculum development. Defining common language and taxonomy is essential for a harmonized product. The importance of establishing an international network to support implementation, evaluation, sustainability and dissemination of the work was underscored. Originality/value International stakeholders are collaborating successfully on a graduated, competency-based leadership curriculum for postgraduate medical learners. The final product will be available for adaptation to local needs. An international physician leadership education network is being developed to support and expand the work underway.

  2. Morbus gaucher: A report of two cases

    Directory of Open Access Journals (Sweden)

    Đokić Milomir

    2006-01-01

    Full Text Available Backround. Clinical features of inherited glucocerebrosidase deficiency were first described by Phillippe Charles Ernest Gaucher, French physician (1854-1918. Deficiency of glucocerebrosidase leads to the accumulation of the lipid glucocerebroside within the lysosomes of the monocyte macrophage system. Lipid-laden cells, known as Gaucher cells, lead to hepatosplenomegaly, multiorgan dysfunction and sceletal deterioration. Case report. We reported a 36- year-old male and a 42-year-old female admitted for the clinical examination due to hepatosplenomegaly. The Clinical diagnosis was provided by a bone marrow examination and demonstration of the characteristic Gaucher cells. Both of the patients had type I Gaucher's disease (a mild form of the disease, which is distinguished by the lack of central nervous system involvement and striking phenotypic variation. We had not a possibility of testing β-glucocerebrosidase activity in peripheral leukocytes (a definitive diagnosis of Gaucher's disease. Also, enzyme replacement therapy had not been available in our country. Conclusion. Althoungh rare, Gaucher's disease is also present in our country. Both molecular genetic, and the enzyme β-glucocerebrosidase activity testing in peripheral leukocytes are needed for the definitive diagnosis of this disease.

  3. Perinatal-lethal Gaucher disease presenting as hydrops fetalis.

    Science.gov (United States)

    BenHamida, Emira; Ayadi, Imene; Ouertani, Ines; Chammem, Maroua; Bezzine, Ahlem; BenTmime, Riadh; Attia, Leila; Mrad, Ridha; Marrakchi, Zahra

    2015-01-01

    Perinatal-lethal Gaucher disease is very rare and is considered a variant of type 2 Gaucher disease that occurs in the neonatal period. The most distinct features of perinatal-lethal Gaucher disease are non-immune hydrops fetalis. Less common signs of the disease are hepatosplenomegaly, ichthyosis and arthrogryposis. We report a case of Gaucher's disease (type 2) diagnosed in a newborn who presented with Hydrops Fetalis.

  4. Gaucher disease: a model disorder for biomarker discovery

    DEFF Research Database (Denmark)

    Boot, Rolf G; van Breemen, Mariëlle J; Wegdam, Wouter

    2009-01-01

    Gaucher disease is an inherited lysosomal storage disorder, characterized by massive accumulation of glucosylceramide-laden macrophages in the spleen, liver and bone marrow as a consequence of deficient activity of glucocerebrosidase. Gaucher disease has been the playground to develop new therape...... in clinical management of Gaucher patients are discussed. Moreover, the use of several modern proteomic technologies for the identification of Gaucher biomarkers is reviewed....

  5. 19th International Conference on Interactive Collaborative Learning

    CERN Document Server

    Guralnick, David; Uhomoibhi, James

    2017-01-01

    This book presents the proceedings of the 19th International Conference on Interactive Collaborative Learning, held 21-23 September 2016 at Clayton Hotel in Belfast, UK. We are currently witnessing a significant transformation in the development of education. The impact of globalisation on all areas of human life, the exponential acceleration of developments in both technology and the global markets, and the growing need for flexibility and agility are essential and challenging elements of this process that have to be addressed in general, but especially in the context of engineering education. To face these topical and very real challenges, higher education is called upon to find innovative responses. Since being founded in 1998, this conference has consistently been devoted to finding new approaches to learning, with a focus on collaborative learning. Today the ICL conferences have established themselves as a vital forum for the exchange of information on key trends and findings, and of practical lessons le...

  6. Internal and External Collaboration in New Product Development

    DEFF Research Database (Denmark)

    Timenes Laugen, Bjørge; Lassen, Astrid Heidemann; Middel, Rick

    2009-01-01

    Industry and academia alike are increasingly becoming aware of the fact that innovation does not take place in isolated cells or functions within the firm. During the last the years the term open innovation has emphasised the importance of internal and external collaboration in order to increase...... strategic priorities influence the degree of external and internal involvement in the NPD process, moderated by contextual factors. Results based on analyses of 584 companies from the International Manufacturing Strategy Survey (IMSS) 2005 indicate that suppliers are heavily involved in the NPD process...... in firms in B2C markets aiming at increasing the innovation volume. For B2B companies the reverse picture emerges. However, when the aim is to increase the radicality of new products, suppliers and customers are heavily involved for firms in B2B markets. Further, market uncertainty, and to some extent...

  7. Collaborative innovation: Internal and external involvement in new product development

    DEFF Research Database (Denmark)

    Timenes Laugen, Bjørge; Lassen, Astrid Heidemann

    2011-01-01

    Industry and academia alike are increasingly becoming aware of the fact that innovation does not take place in isolated cells or functions within the firm. During the last the years the term open innovation has emphasized the importance of internal and external collaboration in order to increase...... strategic priorities influence the degree of external and internal involvement in the NPD process, moderated by contextual factors. Results based on analyses of 584 companies from the International Manufacturing Strategy Survey (IMSS) 2005 indicate that suppliers are heavily involved in the NPD process...... in firms in B2C markets aiming at increasing the innovation volume. For B2B companies the reverse picture emerges. However, when the aim is to increase the radicality of new products, suppliers and customers are heavily involved for firms in B2B markets. Further, market uncertainty, and to some extent...

  8. Twenty years of treatment for Gaucher disease: emerging challenges

    NARCIS (Netherlands)

    van Dussen, L.

    2014-01-01

    Gaucher disease is an autosomal recessively inherited lysosomal storage disorder (LSD). Type I Gaucher disease, the so-called non-neuronopathic variant, is mainly characterised by cytopenia, hepatosplenomegaly and bone complications. Gaucher disease was the first LSD for which enzyme replacement

  9. Evaluation of an international and interprofessional collaboration forum.

    Science.gov (United States)

    Stone, Teresa; Hua, Susan; Turale, Sue

    2016-11-01

    International and interprofessional collaborations are increasingly becoming a core requirement for health professionals in our globalized world. The aim of this study was to evaluate the effectiveness of the Asia Pacific Alliance of Health Leaders (APAHL) Forum to enhance the development of international perspectives and leadership among students and faculty in the discipline of health. This pilot study used a student-designed questionnaire to evaluate the views of students and faculty members about the effectiveness of APAHL in meeting its goals. Quantitative data from the scaled items on the questionnaire were analyzed by aggregating the data. Qualitative data were analyzed using a qualitative descriptive approach. Study participants comprised of 22 health science (nursing and laboratory science) students and 15 faculty members. Both faculty and students agreed that APAHL was effective in leadership development of students, as well as in advancing internationalization, interprofessional collaboration, and cultural awareness among students. A clear theme among the students was acknowledgement of the importance of communication, in particular being proficient in English. Difficulties in communication were an issue for both students and faculty members. This pilot study has shown the benefits of a student-focused international forum in developing cross-cultural awareness, and will provide the groundwork for evaluating the effectiveness of cross-cultural and interprofessional leadership forums aimed particularly at students of health. Copyright © 2016 Elsevier Ltd. All rights reserved.

  10. International collaborative fire modeling project (ICFMP). Summary of benchmark

    International Nuclear Information System (INIS)

    Roewekamp, Marina; Klein-Hessling, Walter; Dreisbach, Jason; McGrattan, Kevin; Miles, Stewart; Plys, Martin; Riese, Olaf

    2008-09-01

    This document was developed in the frame of the 'International Collaborative Project to Evaluate Fire Models for Nuclear Power Plant Applications' (ICFMP). The objective of this collaborative project is to share the knowledge and resources of various organizations to evaluate and improve the state of the art of fire models for use in nuclear power plant fire safety, fire hazard analysis and fire risk assessment. The project is divided into two phases. The objective of the first phase is to evaluate the capabilities of current fire models for fire safety analysis in nuclear power plants. The second phase will extend the validation database of those models and implement beneficial improvements to the models that are identified in the first phase of ICFMP. In the first phase, more than 20 expert institutions from six countries were represented in the collaborative project. This Summary Report gives an overview on the results of the first phase of the international collaborative project. The main objective of the project was to evaluate the capability of fire models to analyze a variety of fire scenarios typical for nuclear power plants (NPP). The evaluation of the capability of fire models to analyze these scenarios was conducted through a series of in total five international Benchmark Exercises. Different types of models were used by the participating expert institutions from five countries. The technical information that will be useful for fire model users, developers and further experts is summarized in this document. More detailed information is provided in the corresponding technical reference documents for the ICFMP Benchmark Exercises No. 1 to 5. The objective of these exercises was not to compare the capabilities and strengths of specific models, address issues specific to a model, nor to recommend specific models over others. This document is not intended to provide guidance to users of fire models. Guidance on the use of fire models is currently being

  11. Productive international collaboration in the large coil task

    International Nuclear Information System (INIS)

    Haubenreich, P.N.; Komarek, P.; Shimamoto, S.; Vecsey, G.

    1987-01-01

    The Large Coil Task (LCT), initiated in 1977, has been very productive of useful technical information about superconducting toroidal field (TF) coil design and manufacture. Moreover, it has demonstrated close international collaboration in fusion technology development, including integration of large components built in four different countries. Each of six 40-t test coils was designed and produced by a major industrial team, with government laboratory guidance, to a common set of specifications. The six were assembled into a toroidal array for testing in the International Fusion Superconducting Magnet Test Facility (IFSMTF) at Oak Ridge. Testing was done by a team of representatives of EURATOM, Japan, Switzerland, and the United States, with each participant having full access to all data. Coils were thoroughly instrumented, enabling penetrating analysis of behavior

  12. Brazilian Participations in the International Astronomical Search Collaboration

    Science.gov (United States)

    Rojas, G. A.; Dalla-Costa, L. J.; Kalmus, A. T.; Kroth, E. C.; Matos, M. F.; Silva, A. L.; Silva, G. G.

    2014-10-01

    International Astronomical Search Collaboration (IASC) is an international educational project between universities, schools, observatories and research institutions. Its main objective is to enroll high school and college students in the monitoring and discovery of asteroids and Near Earth Objects (NEOs), especially Potentially Hazardous Asteroids. The methodology consists in the analysis of astronomical images obtained in several observatories in North America and Hawaii. The images are distributed throughout the school network and the results must be delivered in a 72-hour timeframe. Since 2010 Brazilian universities and schools have joined IASC, resulting in over a dozen new asteroids found (3 of them NEOs), and hundreds of measurements for already known asteroids. A major event in this collaboration was the All-Brazil Asteroid Search Campaign, which was conducted in September 2012. 2013 marks the fourth year of Brazilian participations in IASC, with one important milestone: the third straight appearance of a Brazilian institution in the Pan-STARRS campaign, which uses the PS1 telescope in Haleakala, Hawaii. We will present a summary of the overall results, as well as the latest news from 2013 campaigns. We will discuss the impact promoted by the past events, such as how the interest in astronomy changed before and after the campaigns, and it has helped the students to choose their future careers.

  13. International collaboration in the history of science of Central Europe

    Directory of Open Access Journals (Sweden)

    Soňa ŠTRBÁŇOVÁ

    2015-12-01

    Full Text Available In the last ten years, approximately, we could witness an evolution in informal international collaboration focusing on shared and interconnected history of science in the Habsburg Monarchy and in Central Europe in general. This effort, which includes mainly historians of science from Austria, Czech Republic, Hungary and Poland, has already produced a number of important results and contributed to the thematization of some timeless topics of history of sciences such as, for instance, nationalization and internationalization of science. In the context of this cooperation, the seminar of Jan Surman, a historian of science of Polish descent, held at the Institute of Contemporary History of the Czech Academy of Sciences in Prague in May 2015, concentrated on the formation of national scientific terminologies. It also underlined the necessity and usefulness of international collaboration in achieving a deeper understanding of the “national” histories of science, which cannot be separated from the “international” history.

  14. Establishing and maintaining international collaborative research teams: an autobiographical insight

    Directory of Open Access Journals (Sweden)

    T J Carr

    2013-07-01

    Full Text Available Despite the growing impetus for international collaborative research teams (ICRT, there are relatively few resources available to guide and support researchers through the processes of establishing and maintaining ICRTs. In particular, no articles were found that provided researchers’ firsthand accounts of being a member of such a team. Having access to such personal accounts can help both experienced and novice researchers learn more directly about what to expect, as well as the benefits, challenges, pitfalls, and success strategies for establishing and maintaining ICRTs. The authors used phenomenological autobiographical reflective journaling to capture their experiences as members of ICRTs. In this article we provide an overview of key themes that emerged from the analysis of our reflections as members of ICRTs. These themes include: benefits, challenges, and strategies for success. Our aim is to share our first-hand experiences of what it is like to establish and participate in ICRT. It is not our intention to provide readers with prescriptive guidelines on how to set up and maintain ICRTs. Every ICRT is unique and some of these ideas may or may not apply in every case. Instead, we are describing what worked for us, hoping that others may benefit from our experience. Consequently, we suggest that the focus of ICRT should be on the benefits thereof which promote and encourage interaction between disciplines, transfer of knowledge and techniques and personal and professional development. Keywords: international, collaborative, research, teams, interdisciplinary

  15. Mandibular and Dental Manifestations of Gaucher Disease

    Science.gov (United States)

    Saranjam, Hamid R.; Sidransky, Ellen; Levine, William Z.; Zimran, Ari; Elstein, Deborah

    2012-01-01

    Gaucher disease is a systemic lysosomal storage disorder with a high prevalence among Ashkenazi Jews. It is caused by an inherited deficiency of the lysosomal enzyme glucocerebrosidase. Common signs and symptoms include hepatosplenomegaly, anemia, thrombocytopenia, and skeletal involvement. Oral and dental manifestations are less commonly seen. These manifestations are often asymptomatic, although they may be detected by routine dental x-rays. There are several case reports and a few larger series published describing patients with Gaucher disease who have mandibulo-maxillofacial involvement. This review aims to examine the oral manifestations observed in Gaucher disease and to suggest practical guidelines for dealing with these often worrisome signs. Among the critical issues are the benign nature of Gaucher cell infiltration of the mandible and the critical importance of being prepared for post-procedure bleeding and/or infections. Therefore, it is essential that dental practitioners be aware of the possible oral and dental complications of Gaucher disease, as well as the available treatment modalities. PMID:22251146

  16. Dental profile of patients with Gaucher disease

    Science.gov (United States)

    Fischman, Stuart L; Elstein, Deborah; Sgan-Cohen, Harold; Mann, Jonathan; Zimran, Ari

    2003-01-01

    Background This study was conducted to determine whether patients with Gaucher disease had significant dental pathology because of abnormal bone structure, pancytopenia, and coagulation abnormalities. Methods Each patient received a complete oral and periodontal examination in addition to a routine hematological evaluation. Results Gaucher patients had significantly fewer carious lesions than otherwise healthy carriers. Despite prevalence of anemia, there was no increase in gingival disease; despite the high incidence of thrombocytopenia, gingival bleeding was not noted; and despite radiological evidence of bone involvement, there was no greater incidence loss of teeth or clinical tooth mobility. Conclusions These data represent the first survey of the oral health of a large cohort of patients with Gaucher disease. It is a pilot study of a unique population and the results of the investigation are indications for further research. Based on our findings, we recommend regular oral examinations with appropriate dental treatment for patients with Gaucher disease as for other individuals. Consultation between the dentist and physician, preferably one with experience with Gaucher disease, should be considered when surgical procedures are planned. PMID:12875661

  17. International Combined Orthopaedic Research Societies: A model for international collaboration to promote orthopaedic and musculoskeletal research

    Directory of Open Access Journals (Sweden)

    Theodore Miclau

    2014-10-01

    Full Text Available In October 2013, the International Combined Orthopaedic Research Societies (ICORS; http://i-cors.org was founded with inaugural member organisations from the previous Combined Orthopaedic Research Society, which had sponsored combined meetings for more than 2 decades. The ICORS is dedicated to the stimulation of orthopaedic and musculoskeletal research in fields such as biomedical engineering, biology, chemistry, and veterinary and human clinical research. The ICORS seeks to facilitate communication with member organisations to enhance international research collaborations and to promote the development of new international orthopaedic and musculoskeletal research organisations. Through new categories of membership, the ICORS represents the broadest coalition of orthopaedic research organisations globally.

  18. Mobility and International Collaboration: Case of the Mexican Scientific Diaspora.

    Directory of Open Access Journals (Sweden)

    Rafael Marmolejo-Leyva

    Full Text Available We use a data set of Mexican researchers working abroad that are included in the Mexican National System of Researchers (SNI. Our diaspora sample includes 479 researchers, most of them holding postdoctoral positions in mainly seven countries: USA, Great Britain, Germany, France, Spain, Canada and Brazil. Their research output and impact is explored in order to determine their patterns of production, mobility and scientific collaboration as compared with previous studies of the SNI researchers in the periods 1991-2001 and 2003-2009. Our findings confirm that mobility has a strong impact on their international scientific collaboration. We found no substantial influence among the researchers that got their PhD degrees abroad from those trained in Mexican universities. There are significant differences among the areas of knowledge studied: biological sciences, physics and engineering have better production and impact rates than mathematics, geosciences, medicine, agrosciences, chemistry, social sciences and humanities. We found a slight gender difference in research production but Mexican female scientists are underrepresented in our diaspora sample. These findings would have policy implications for the recently established program that will open new academic positions for young Mexican scientists.

  19. International energy technology collaboration: wind power integration into electricity systems

    International Nuclear Information System (INIS)

    Justus, D.

    2006-01-01

    A rapid growth of wind power since the 1990s has led to notable market shares in some electricity markets. This growth is concentrated in a few countries with effective Research, Development and Demonstration (RD and D) programmes and with policies that support its diffusion into the market place. The speed and depth of its penetration in these electricity markets have amplified the need to address grid integration concerns, so as not to impede the further penetration of wind power. Research on technologies, tools and practices for integrating large amounts of wind power into electricity supply systems is attempting to respond to this need. In recent years, existing international collaborative research efforts have expanded their focus to include grid integration of wind power and new consortia have been formed to pool knowledge and resources. Effective results benefit a few countries that already have a significant amount of wind in their electricity supply fuel mix, as well as to the potential large markets worldwide. This paper focuses on the challenge of bringing significant amounts of intermittent generating sources into grids dominated by large central generating units. It provides a brief overview of the growth of wind power, mainly since 1990, the technical and operational issues related to integration and selected collaborative programmes underway to address grid integration concerns. (author)

  20. Mobility and International Collaboration: Case of the Mexican Scientific Diaspora.

    Science.gov (United States)

    Marmolejo-Leyva, Rafael; Perez-Angon, Miguel Angel; Russell, Jane M

    2015-01-01

    We use a data set of Mexican researchers working abroad that are included in the Mexican National System of Researchers (SNI). Our diaspora sample includes 479 researchers, most of them holding postdoctoral positions in mainly seven countries: USA, Great Britain, Germany, France, Spain, Canada and Brazil. Their research output and impact is explored in order to determine their patterns of production, mobility and scientific collaboration as compared with previous studies of the SNI researchers in the periods 1991-2001 and 2003-2009. Our findings confirm that mobility has a strong impact on their international scientific collaboration. We found no substantial influence among the researchers that got their PhD degrees abroad from those trained in Mexican universities. There are significant differences among the areas of knowledge studied: biological sciences, physics and engineering have better production and impact rates than mathematics, geosciences, medicine, agrosciences, chemistry, social sciences and humanities. We found a slight gender difference in research production but Mexican female scientists are underrepresented in our diaspora sample. These findings would have policy implications for the recently established program that will open new academic positions for young Mexican scientists.

  1. International Collaboration on Air Pollution and Pregnancy Outcomes (ICAPPO)

    Science.gov (United States)

    Woodruff, Tracey J.; Parker, Jennifer D.; Adams, Kate; Bell, Michelle L.; Gehring, Ulrike; Glinianaia, Svetlana; Ha, Eun-Hee; Jalaludin, Bin; Slama, Rémy

    2010-01-01

    Reviews find a likely adverse effect of air pollution on perinatal outcomes, but variation of findings hinders the ability to incorporate the research into policy. The International Collaboration on Air Pollution and Pregnancy Outcomes (ICAPPO) was formed to better understand relationships between air pollution and adverse birth outcomes through standardized parallel analyses in datasets from different countries. A planning group with 10 members from 6 countries was formed to coordinate the project. Collaboration participants have datasets with air pollution values and birth outcomes. Eighteen research groups with data for approximately 20 locations in Asia, Australia, Europe, North America, and South America are participating, with most participating in an initial pilot study. Datasets generally cover the 1990s. Number of births is generally in the hundreds of thousands, but ranges from around 1,000 to about one million. Almost all participants have some measure of particulate matter, and most have ozone, nitrogen dioxide, sulfur dioxide and carbon monoxide. Strong enthusiasm for participating and a geographically-diverse range of participants should lead to understanding uncertainties about the role of air pollution in perinatal outcomes and provide decision-makers with better tools to account for pregnancy outcomes in air pollution policies. PMID:20644693

  2. Technological learning through international collaboration: Lessons from the field

    Science.gov (United States)

    Wood, Danielle; Weigel, Annalisa

    2013-02-01

    Countries on every continent are making new or renewed commitments to domestic satellite programs. These programs have the potential to address national needs by enhancing access to information, improving infrastructure and providing inspiration to the public. How do countries without local expertise in space technology begin a new satellite program? What is the role of international collaboration in supporting the efforts of a new space fairing country? This paper explores such questions by highlighting outputs from intensive field work in Africa and Asia. Specifically, the study explores case studies of early space activity in these countries to search for lessons about the management of a young space program. The observations from field work are compared to ideas from scholarly literature on technological learning. The findings are organized using principles from systems architecture. The paper presents a model that captures many of the influences and strategic decision areas for a collaborative satellite development project. The paper also highlights the growth of capability among African countries in the area of satellite technology.

  3. International collaboration on used fuel disposition crystalline rocks

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Yifeng [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Gardner, Payton [Univ. of Montana, Missoula, MT (United States); Kim, Geon-Young [Korean Atomic Energy Research Inst. Daejeon (Korea); Ji, Sung-Hoon [Korean Atomic Energy Research Inst., Daejeon (Korea)

    2016-08-01

    Active participation in international R&D is crucial for achieving the UFD long-term goals of conducting “experiments to fill data needs and confirm advanced modeling approaches” (by 2015) and of having a “robust modeling and experimental basis for evaluation of multiple disposal system options” (by 2020). DOE’s Office of Nuclear Energy (NE) and its Office of Used Fuel Disposition Research and Development (UFD) have developed a strategic plan to advance cooperation with international partners. The international collaboration on the evaluation of crystalline disposal media at Sandia National Laboratories (SNL) in FY16 focused on the following four activities: (1) thermal-hydrologic-mechanical-chemical modeling single fracture evolution; (2) simulations of flow and transport in Bedrichov Tunnel, Czech Republic, (3) completion of streaming potential testing at Korean Atomic Energy Research Institute (KAERI), and (4) technical data exchange with KAERI on thermal-hydrologic-mechanical (THM) properties and specifications of bentonite buffer materials. The first two activities are part of the Development of Coupled Models and their Validation against Experiments (DECOVALEX-2015) project.

  4. Thromboelastography as a Surrogate Marker of Perisurgical Hemostasis in Gaucher Disease.

    Science.gov (United States)

    Ioscovich, Alexander; Fadeev, Dmitri; Kenet, Gili; Naamad, Mira; Schtrechman, Gal; Zimran, Ari; Elstein, Deborah

    2016-10-01

    Thromboelastography (TEG) has long been available for routine monitoring of perisurgical and postpartum hemostasis, especially at point of care. The purpose of this study is to retrospectively compare TEG parameters to concomitant standard clotting test results in an unselected cohort of patients with Gaucher disease to ascertain whether TEG values are specific and sensitive enough to substitute for classic coagulation tests for decision making. This remains a cogent concern because of high incidence of thrombocytopenia in patients with Gaucher disease. Thromboelastography values were compared to concomitant platelet counts, partial thromboplastin time, international normalization ratio, and plasma fibrinogen. Demographic characteristics were collected from patients' files. There were 22 patients with Gaucher disease (2 children; 12.5%) for whom there were 24 TEG results at the same time as classic coagulation test results and 30% performed platelet function tests. The current study shows linear and/or monotonic relationships between platelet counts and several TEG values that were significant over a range of platelet counts including severe thrombocytopenia. The fibrinogen component, correlating only with the rate of clot lysis, played a lesser role. Based on these preliminary results albeit in a small cohort with only 1 case of hemorrhage, there is putative support for the intention to treat patients with Gaucher disease based on TEG results using the same TEG protocol as for other patients undergoing comparable procedures in our institution. © The Author(s) 2015.

  5. Optimal therapy in Gaucher disease

    Directory of Open Access Journals (Sweden)

    Ozlem Goker-Alpan

    2010-07-01

    Full Text Available Ozlem Goker-AlpanLysosomal Diseases Research and Treatment Unit, Center for Clinical Trials, O&O Alpan LLC, Springfield, VA, USAAbstract: Gaucher disease (GD, the inherited deficiency of the lysosomal enzyme glucocerebrosidase, presents with a wide range of symptoms of varying severity, and primarily affects the skeletal, hematologic and nervous systems. To date, the standard of care has included enzyme replacement therapy with imiglucerase. Although imiglucerase is highly effective in reversing the visceral and hematologic manifestations, skeletal disease is slow to respond, pulmonary involvement is relatively resistant, and the CNS involvement is not impacted. Because of the recent manufacturing and processing problems, the research and development of alternative therapeutics has become more pressing. The divergent phenotypes and the heterogeneity involving different organ systems implicates the involvement of several pathological processes that include enzyme deficiency, substrate accumulation, protein misfolding, and macrophage activation, that differ in each patient with GD. Thus, the therapy should be tailored individually in order to target multiple pathways that interplay in GD.Keywords: glucocerebrosidase, enzyme replacement therapy, substrate reduction therapy, protein misfolding and chaperone therapy, macrophage

  6. New Directions in Gaucher Disease.

    Science.gov (United States)

    Horowitz, Mia; Elstein, Deborah; Zimran, Ari; Goker-Alpan, Ozlem

    2016-11-01

    In Gaucher disease (GD), mutant lysosomal acid β-glucocerebrosidase fails to properly hydrolyze its substrate, glucosylceramide, which accumulates in the lysosomes. Due to its phenotypic heterogeneity, GD has been classified into type 1, non-neuronopathic, and types 2 and 3, the neuronopathic forms, based on the primary involvement of the central nervous system. Neuroinflammation and necroptotic death may appear in the neuronopathic forms of GD, whereas type 1 GD patients may develop Parkinson disease (PD), a prototype of protein misfolding disorders of the nervous system. PD is significantly more prevalent among GD carriers and patients than among the non-GD populations. It is apparent that the amount of mutant enzyme present in lysosomes depends on the amount of mutant enzyme recognized as correctly folded in the endoplasmic reticulum (ER) for physiologically correct transport through the Golgi apparatus to the lysosome. Mutant enzyme recognized as misfolded is retained in the ER, inducing the Unfolded Protein Response. In the current review, we present three discrete areas of interest: molecular and cellular mechanisms underlying the association between GD and PD; the clinical and genetic associations between GD and PD; and treatment options for GD. We also discuss the relevance of induced pleuripotent stem cells to the above associations. © 2016 WILEY PERIODICALS, INC.

  7. Standard methods for sampling freshwater fishes: Opportunities for international collaboration

    Science.gov (United States)

    Bonar, Scott A.; Mercado-Silva, Norman; Hubert, Wayne A.; Beard, Douglas; Dave, Göran; Kubečka, Jan; Graeb, Brian D. S.; Lester, Nigel P.; Porath, Mark T.; Winfield, Ian J.

    2017-01-01

    With publication of Standard Methods for Sampling North American Freshwater Fishes in 2009, the American Fisheries Society (AFS) recommended standard procedures for North America. To explore interest in standardizing at intercontinental scales, a symposium attended by international specialists in freshwater fish sampling was convened at the 145th Annual AFS Meeting in Portland, Oregon, in August 2015. Participants represented all continents except Australia and Antarctica and were employed by state and federal agencies, universities, nongovernmental organizations, and consulting businesses. Currently, standardization is practiced mostly in North America and Europe. Participants described how standardization has been important for management of long-term data sets, promoting fundamental scientific understanding, and assessing efficacy of large spatial scale management strategies. Academics indicated that standardization has been useful in fisheries education because time previously used to teach how sampling methods are developed is now more devoted to diagnosis and treatment of problem fish communities. Researchers reported that standardization allowed increased sample size for method validation and calibration. Group consensus was to retain continental standards where they currently exist but to further explore international and intercontinental standardization, specifically identifying where synergies and bridges exist, and identify means to collaborate with scientists where standardization is limited but interest and need occur.

  8. Phebus FP: organisation of the project and international collaboration

    International Nuclear Information System (INIS)

    Tattegrain, A.; Hardt, P. von der

    1992-01-01

    PHEBUS Fission Product (FP) Research Programme developed from the initial French design study into a European project, and further into an international programme by agreements with overseas partners during the past two years. The programme is supervised by a Steering Committee which reviews the technical-scientific options and the results. The executive body under the Committee, the Project Group, includes a Commissariat a l'Energie Atomique (CEA) and Commission of the European Communities (CEC) manager as well as three (CEA) project leaders for design and manufacture, experiment operation, and interpretation of test results. The Steering Committee can request expertise from the two working groups the Analytical Group (SAWG) (elaborating test objectives, carrying out reactor calculations and test precalculations) and the Technical Group (TG) (assessing the designs proposed and the results obtained by the Project Group). A third group looks into financial aspects of the CEA-CEC contract only. The two working groups, SAWG and TG, play an important role in the exchange of information and of expertise between all partners. The paper reviews the internal Project organisation and the collaboration network, inside the European Community and through CEA overseas. (author)

  9. International research collaboration as social relation: an Ethiopian-Canadian example.

    Science.gov (United States)

    Bender, Amy; Guruge, Sepali; Aga, Fekadu; Hailemariam, Damen; Hyman, Ilene; Tamiru, Melesse

    2011-06-01

    International collaboration in nursing and other health disciplines is vital for addressing global health issues. While the results and processes of such collaborations have been reported, few publications have addressed their philosophical or theoretical underpinnings, particularly with respect to collaboration between those in low- and high-income countries. Piaget's notion of social relations of cooperation and constraint and Habermas's notion of "lifeworld" provide a theoretical lens through which to examine international collaboration as a construction of knowledge. This article is an exploration of these ideas as seen in the collective experience of Canadians and Ethiopians organizing an interdisciplinary forum on intimate partner violence in Ethiopia. The project is presented as a case study for reflecting on international collaboration as a manifestation of social relations. Such re-visioning of international collaboration may be useful for improving collaborative processes and their outcomes.

  10. Understanding Public-Private Collaboration Configurations for International Information Infrastructures

    NARCIS (Netherlands)

    Klievink, A.J.

    Collaboration between the public and the private sector is seen as an instrument to make governance smarter, more effective, and more efficient. However, whereas there is literature on public-private collaboration, very little of it addresses how these collaborations can be shaped to make use of the

  11. Understanding Public-Private Collaboration Configurations for International Information Infrastructures

    NARCIS (Netherlands)

    Klievink, A.J.

    2015-01-01

    Collaboration between the public and the private sector is seen as an instrument to make governance smarter, more effective, and more efficient. However, whereas there is literature on public-private collaboration, very little of it addresses how these collaborations can be shaped to make use of the

  12. Seafloor 2030 - Building a Global Ocean Map through International Collaboration

    Science.gov (United States)

    Ferrini, V. L.; Wigley, R. A.; Falconer, R. K. H.; Jakobsson, M.; Allen, G.; Mayer, L. A.; Schmitt, T.; Rovere, M.; Weatherall, P.; Marks, K. M.

    2016-12-01

    With more than 85% of the ocean floor unmapped, a huge proportion of our planet remains unexplored. Creating a comprehensive map of seafloor bathymetry remains a true global challenge that can only be accomplished through collaboration and partnership between governments, industry, academia, research organizations and non-government organizations. The objective of Seafloor 2030 is to comprehensively map the global ocean floor to resolutions that enable exploration and improved understanding of ocean processes, while informing maritime policy and supporting the management of natural marine resources for a sustainable Blue Economy. Seafloor 2030 is the outcome of the Forum for Future of Ocean Floor Mapping held in Monaco in June 2016, which was held under the auspices of GEBCO and the Nippon Foundation of Japan. GEBCO is the only international organization mandated to map the global ocean floor and is guided by the International Hydrographic Organization (IHO) and the Intergovernmental Oceanographic Commission of UNESCO. The task of completely mapping the ocean floor will require new global coordination to ensure that both existing data are identified and that new mapping efforts are coordinated to help efficiently "map the gaps." Fundamental to achieving Seafloor 2030 will be greater access to data, tools and technology, particularly for developing and coastal nations. This includes bathymetric post-processing and analysis software, database technology, computing infrastructure and gridding techniques as well as the latest developments in seafloor mapping methods and emerging crowd-sourced bathymetry initiatives. The key to achieving this global bathymetric map is capacity building and education - including greater coordination between scientific research and industry and the effective engagement of international organizations such as the United Nations.

  13. Gaucher disease: Progress and ongoing challenges.

    Science.gov (United States)

    Mistry, Pramod K; Lopez, Grisel; Schiffmann, Raphael; Barton, Norman W; Weinreb, Neal J; Sidransky, Ellen

    Over the past decades, tremendous progress has been made in the field of Gaucher disease, the inherited deficiency of the lysosomal enzyme glucocerebrosidase. Many of the colossal achievements took place during the course of the sixty-year tenure of Dr. Roscoe Brady at the National Institutes of Health. These include the recognition of the enzymatic defect involved, the isolation and characterization of the protein, the localization and characterization of the gene and its nearby pseudogene, as well as the identification of the first mutant alleles in patients. The first treatment for Gaucher disease, enzyme replacement therapy, was conceived of, developed and tested at the Clinical Center of the National Institutes of Health. Advances including recombinant production of the enzyme, the development of mouse models, pioneering gene therapy experiments, high throughput screens of small molecules and the generation of induced pluripotent stem cell models have all helped to catapult research in Gaucher disease into the twenty-first century. The appreciation that mutations in the glucocerebrosidase gene are an important risk factor for parkinsonism further expands the impact of this work. However, major challenges still remain, some of which are described here, that will provide opportunities, excitement and discovery for the next generations of Gaucher investigators. Published by Elsevier Inc.

  14. Partial splenectomy in children with Gaucher's disease

    International Nuclear Information System (INIS)

    Bar-Maor, J.A.; Govrin-Yehudain, J.

    1985-01-01

    Because of hypersplenism and mechanical problems, partial splenectomy was performed in four children with Gaucher's disease. Subsequently, one of the patients underwent a total splenectomy due to bleeding from the remnant of the spleen. At the follow-up of the other three patients, an isotope scan showed that the remaining spleen was functioning well

  15. Haematological Problems Associated with Gaucher's Disease ...

    African Journals Online (AJOL)

    Five recent cases of Gaucher's disease seen at Groote Schuur Hospital illustrate the haematological complications. The main problem is hypersplenism with secondary thrombocytopenia. Two patients underwent splenectomy for this reason, and one gained lasting improvement. None of the more rare haematological ...

  16. Reported outcomes of 453 pregnancies in patients with Gaucher disease: An analysis from the Gaucher outcome survey.

    Science.gov (United States)

    Lau, Heather; Belmatoug, Nadia; Deegan, Patrick; Goker-Alpan, Ozlem; Schwartz, Ida Vanessa D; Shankar, Suma P; Panahloo, Zoya; Zimran, Ari

    2018-02-01

    Gaucher disease (GD) may worsen during pregnancy, leading to the discussion of continuing treatment during pregnancy. We examined fetal outcomes of pregnancies reported in the Gaucher Outcome Survey, an international GD-specific registry established in 2010. A total of 453 pregnancies were reported. Most pregnancies (336/453, 74.2%) were in women who did not receive GD-specific treatment during pregnancy, while enzyme replacement therapy (ERT) was received during 117/453 (25.8%) pregnancies. No pregnancies exposed to substrate reduction therapy were reported. The percentage of normal outcomes (live birth delivered at term with no congenital abnormalities) was similar in untreated and treated pregnancies (92.9% vs. 91.4%). The percentage of spontaneous abortions in untreated pregnancies was 3.6% (95% CI, 1.9%- 6.2%) compared with 6.9% (95% CI, 3.0%-13.1%) in treated pregnancies (p=0.1866). In women who received velaglucerase alfa <1month prior to conception and/or during pregnancy, 34/36 (94.4%) pregnancies had normal outcomes and 2 (5.6%) ended in spontaneous abortion. Normal outcomes were observed in the 20 pregnancies with velaglucerase alfa exposure starting <1month prior to conception and continuing through all trimesters. These observations, in addition to information in the literature, suggest that continuation of ERT during pregnancy may be appropriate for GD patients. Copyright © 2016 Shire Human Genetic Therapies, Inc. Published by Elsevier Inc. All rights reserved.

  17. A familial concurrence of schizophrenia and Gaucher's disease

    Directory of Open Access Journals (Sweden)

    Siomos Konstantinos E

    2007-12-01

    Full Text Available Abstract Background Gaucher's disease (GD is the most frequently encountered lysosomal storage disease. Here, we describe and discuss the observed concurrence of schizophrenia and Gaucher's disease in two siblings. Methods Presentation of a family with two siblings with Gaucher's disease. Results In a six-member family, the first son suffers from schizophrenia, while the third and fourth sons suffer from the Gaucher's disease (type 1 non-neuronopathic. The parents and the second son do not suffer from either illness. Conclusion The concurrence of schizophrenia and Gaucher's disease in the same family is an unusual phenomenon. The literature regarding this coincidence is limited, despite the fact that patients with Gaucher's disease have one or two mutated alleles, considered to be a risk factor leading to conditions such as Dementia, Parkinson's disease and schizophrenia.

  18. Developing a prelicensure exam for Canada: an international collaboration.

    Science.gov (United States)

    Hobbins, Bonnie; Bradley, Pat

    2013-01-01

    Nine previously conducted studies indicate that Elsevier's HESI Exit Exam (E(2)) is 96.36%-99.16% accurate in predicting success on the National Council Licensure Examination for Registered Nurses. No similar standardized exam is available in Canada to predict Canadian Registered Nurse Examination (CRNE) success. Like the E(2), such an exam could be used to evaluate Canadian nursing students' preparedness for the CRNE, and scores on the numerous subject matter categories could be used to guide students' remediation efforts so that, ultimately, they are successful on their first attempt at taking the CRNE. The international collaboration between a HESI test construction expert and a nursing faculty member from Canada, who served as the content expert, resulted in the development of a 180-item, multiple-choice/single-answer prelicensure exam (PLE) that was pilot tested with Canadian nursing students (N = 175). Item analysis data obtained from this pilot testing were used to develop a 160-item PLE, which includes an additional 20 pilot test items. The estimated reliability of this exam is 0.91, and it exhibits congruent validity with the CRNE because the PLE test blueprint mimics the CRNE test blueprint. Copyright © 2013 Elsevier Inc. All rights reserved.

  19. International collaboration in the development of materials for fusion

    International Nuclear Information System (INIS)

    Amelinckx, S.

    1988-01-01

    International collaboration in the field of fusion physics research has become a tradition since many years. There are good reasons for this. Fusion physics experiments require progressively larger and more expensive machines. The construction of a major fusion device is beyond the possibility of single nations, except for the largest ones. Moreover it is desirable to test several fundamentally different design options. It would therefore be unreasonable to duplicate major fusion physics experiments. The necessity to pool and coordinate efforts in this area has therefore been recognized since many years and not only within the European community, but even on a global scale. The situation is somewhat different in the area of fusion materials research. In a number of areas of materials research 'big machines' are not required and meaningful research is within the reach of even small countries, moreover it can be done in decentralized fashion. It should nevertheless be noted that the number of properties to be studied and the number of materials options to be evaluated is so extensive that even here excessive duplication would be harmful. (orig.)

  20. Evolutionary convergence of the patterns of international research collaborations across scientific fields

    NARCIS (Netherlands)

    Wang, L.; Coccia, M.

    2015-01-01

    Frame and Carpenter (1979) analysed the pattern of international research collaboration among scientific fields in 1970s. Starting from this pioneering work, this paper investigates international collaborations over 1997-2012 and compares the critical results with earlier studies to detect the

  1. Building an International Collaboration for GeoInformatics

    Science.gov (United States)

    Snyder, W. S.; Lehnert, K.; Klump, J.

    2005-12-01

    Geoinformatics (cyberinfrastructure for the geosciences) is being developed as a linked system of sites that provide to the Earth science community a library of research data research-grade tools to manipulate, mine, analyze and model interdisciplinary data, and mechanisms to provide the necessary computational resources for these activities. Our science is global in scope and hence, geoinformatics (GI) must be an international effort. How do we build this international GI? What are the main challenges presented by the political, cultural, organizational, and technical diversity of the global science community that we need to address to achieve a truly global cyberinfrastructure for the Geosciences? GI needs to be developed in an internet-like fashion establishing connections among independent globally distributed sites (`nodes') that will share, link, and integrate their data holdings and services. Independence of the GI pieces with respect to goals, scope, and approaches is critical to sustain commitment from people to build a GI node for which they feel ownership and get credit. This should not be fought by funding agencies - and certainly not by state and federal agencies. Communication, coordination, and collaboration are the core efforts to build the connections, but incentives and resources are required to advance and support them. Part of the coordination effort is development and maintenance of standards. Who should set these standards and govern their modification? Do we need an official international body to do so, and should this be a "governing body" or an "advisory body"? What role should international commissions and bodies such as CODATA/ICSU or IUGS-CGI, international societies and unions, the national geological surveys and other federal agencies play? Guidance from the science community is key to construct a system that geo-researchers will want to use, and that meets their needs. Only when the community endorses GI as a fundamental platform to

  2. The challenges of international collaboration: Perspectives from Princess Nourah Bint Abdulrahman University

    Directory of Open Access Journals (Sweden)

    Sana Almansour

    2015-12-01

    Full Text Available This case study addresses the international collaboration challenges faced by Princess Nourah Bint Abdulrahman University for women in Saudi Arabia. The objectives of this investigation are to define the challenging sources of international program collaboration between Princess Nourah Bint Abdulrahman University and foreign institutions from the perspective of the university staff who are involved in initiating these collaborations. A total of 27 university staff members who were involved in initiating institutional collaborations participated in semi-structured interviews. A thematic analysis of the interviews suggested that the major sources of challenges to the university’s international collaboration efforts are difficulties in making contacts with international institutions, language barriers, faculty resistance to international partnerships, cross-cultural issues, and establishing partnership agreements.

  3. Celebrating international collaboration: reflections on the first Virtual International Practice Development Conference

    Directory of Open Access Journals (Sweden)

    Moira Stephens

    2016-11-01

    Full Text Available This article reports on the first Virtual International Practice Development Conference, held in May 2015 to celebrate International Nurses Day. The article describes key aspects of its planning, offers a flavour of the event itself and sets out an evaluation, including learning points and recommendations to assist with planning similar events in the future. Central to our learning are: The need for practice developers to grasp skills in technology associated with virtual space The need to embrace virtual space itself as another means by which creative and communicative spaces can be established for active learning and practice development activities The potential advantages that international virtual engagement has over face-to-face national or international engagement The delivery of this virtual event made a significant international contribution to global practice development activity within the International Practice Development Collaborative and to enabling practice developers to connect and celebrate on a more global basis. Implications for practice: Virtual space technology skills can assist with sharing and translating practice development research, innovations and critical commentary Virtual space can provide an adjunct to creative and communicative learning spaces Global networking opportunities can be developed and enhanced through the use of virtual space technology Practice developers need to role model the use of virtual technologies

  4. International collaboration on capture, storage and utilization of greenhouse gases

    International Nuclear Information System (INIS)

    Freund, P.

    1998-01-01

    Climate change will have world-wide implications. So it is highly appropriate that there should be international collaboration to investigate technologies for reducing emissions of greenhouse gases, the root cause of the problem. Sixteen countries, as well as three industrial sponsors, support the IEA Greenhouse Gas R and D Program and, in many cases, industry is also involved indirectly, through the national memberships. This provides a broad range of interest and expertise to guide the management of the Program, as well as ensuring that the results reach a wide audience. The IEA Greenhouse Gas R and D Program has three main activities: (1) evaluation of technologies for mitigation of greenhouse gas emissions from use of fossil fuels; (2) dissemination of the results of these studies; (3) identification of targets for research, development and demonstration and promotion of these findings. In its first five years of operation, the Program has studied the major greenhouse gases, carbon dioxide and methane, and various means of reducing their emissions. The main emphasis has been placed on capture, storage and utilization of CO 2 from power generation. This option is now much better understood and can be compared with more established measures, such as fuel switching, energy efficiency improvements and use of renewable energy. As well as studying abatement of CO 2 emissions, the Program has conducted a series of studies of technologies for reducing CH 4 emissions from man-made sources. The Program's activities are carried out by the Operating Agent, who develops and manages a series of technical studies to meet members' requirements

  5. Building international collaborative capacity: contributions of community psychologists to a European network.

    Science.gov (United States)

    García-Ramírez, Manuel; Paloma, Virginia; Suarez-Balcazar, Yolanda; Balcazar, Fabricio

    2009-09-01

    Europe is in the process of building a more participative, just, and inclusive European Union. The European Social Fund, which is an initiative developed to actively promote multinational partnerships that address pressing social issues, is a good example of the European transformation. This article describes the steps taken to develop and evaluate the activities of an international network promoting collaborative capacity among regional partners involved in the prevention of labor discrimination toward immigrants in three European countries-Spain, Belgium, and Italy. An international team of community psychologists proposed an empowering approach to assess the collaborative capacity of the network. This approach consisted of three steps: (1) establishing a collaborative relationship among partners, (2) building collaborative capacity, and (3) evaluating the collaborative capacity of the network. We conclude with lessons learned from the process and provide recommendations for addressing the challenges inherent in international collaboration processes.

  6. The world network of scientific collaborations between cities: domestic or international dynamics?

    Energy Technology Data Exchange (ETDEWEB)

    Maisonobe, M.; Eckert, D.; Grossetti, M.; Jégou, L.; Milard, B.

    2016-07-01

    Earlier publication (Grossetti et al., 2014) has established that we are attending a decreasing concentration of scientific activities within “world-cities”. Given that more and more cities and countries are contributing to the world production of knowledge, this article analyzes the evolution of the world network of collaborations both at the domestic and international levels during the 2000s. Using data from the Science Citation Index Expanded, scientific authors’ addresses are geo-localized and grouped by urban areas. Our data suggests that interurban collaborations within countries have increased together with international linkages. In most countries, domestic collaborations have increased faster than international collaborations. Even among the top collaborating cities, sometimes referred to as “world cities”, the share of domestic collaborations is gaining momentum. Our results suggest that, contrary to common beliefs about the globalization process, national systems of research have been strengthening during the 2000s. (Author)

  7. Collaboration in Education: International Field Class on Permafrost

    Science.gov (United States)

    Streletskiy, D. A.; Shiklomanov, N. I.; Grebenets, V. I.

    2011-12-01

    Field work is a dominant research component in the earth sciences. Understanding and proper use of field methods can enhance the quality of research, while lack of understanding in acquiring data can lead to misleading interpretation of results. Early involvement in field work helps students to bridge the gap between theoretical knowledge and practical applications and to be better prepared for future jobs. However, many University curriculums lack adequate, required field methods courses. Presented are results of collaboration between the George Washington and Moscow State Universities in organization of field courses on Arctic physical and social environments. The latest field course took place in summer 2011 in the Central Siberian region and is a part of the International Permafrost Association education and outreach effort initiated during International Polar Year. The 25 day course involved fifteen Russian and US students who traveled from Moscow to Krasnoyarsk, and then along Yenisey river to Norilsk. This route was chosen as having diversity of natural conditions and variety of economic, engineering, and demographic problems associated with development. The main goal of the class was to investigate permafrost conditions of Central Siberia; dynamics of upper permafrost due to changing climate and under anthropogenic influence; and to understand factors responsible for the diversity of permafrost conditions in the region. The students and instructors were required to make presentations on a variety of topics focusing on the region or research methods, such as climate, vegetation, hydrology, history of development, economics, remote sensing, etc. The emphasis in the field was made on understanding permafrost in relation to other components of the natural system. For example, landscape conditions (including microclimatic, biogeographic and pedologic conditions) were described at every site located in natural settings. Sites located in settlements were evaluated

  8. Teaching Social Research Methods on an International, Collaborative Environment & Sustainability Degree Programme: Exploring plagiarism, group work, and formative feedback

    OpenAIRE

    Laycock, R

    2017-01-01

    International collaboration is central to the Sustainable Development agenda given environmental challenges that span national boundaries. Education for Sustainability therefore needs to account for international/intercultural understandings, such as though international collaborative degree programmes in Higher Education. This paper evaluates a module taught on an international collaborative Bachelor’s degree programme in Environment & Sustainability taught between Nanjing Xiaozhuang Univers...

  9. Trend and impact of international collaboration in clinical medicine papers published in Malaysia.

    Science.gov (United States)

    Low, Wah Yun; Ng, Kwan Hoong; Kabir, M A; Koh, Ai Peng; Sinnasamy, Janaki

    2014-01-01

    Research collaboration is the way forward in order to improve quality and impact of its research findings. International research collaboration has resulted in international co-authorship in scientific communications and publications. This study highlights the collaborating research and authorship trend in clinical medicine in Malaysia from 2001 to 2010. Malaysian-based author affiliation in the Web of Science (Science Citation Index Expanded) and clinical medicine journals ( n  = 999) and articles ( n  = 3951) as of 30th Oct 2011 were downloaded. Types of document analyzed were articles and reviews, and impact factors (IF) in the 2010 Journal Citation Report Science Edition were taken to access the quality of the articles. The number of publications in clinical medicine increased from 4.5 % ( n  = 178) in 2001 to 23.9 % ( n  = 944) in 2010. The top three contributors in the subject categories are Pharmacology and Pharmacy (13.9 %), General and Internal Medicine (13.6 %) and Tropical Medicine (7.3 %). By journal tier system: Tier 1 (18.7 %, n  = 738), Tier 2 (22.5 %, n  = 888), Tier 3 (29.6 %, n  = 1170), Tier 4 (27.2 %, n  = 1074), and journals without IF (2.1 %, n  = 81). University of Malaya was the most productive. Local collaborators accounted for 60.3 % and international collaborations 39.7 %. Articles with international collaborations appeared in journals with higher journal IFs than those without international collaboration. They were also cited more significantly than articles without international collaborations. Citations, impact factor and journal tiers were significantly associated with international collaboration in Malaysia's clinical medicine publications. Malaysia has achieved a significant number of ISI publications in clinical medicine participation in international collaboration.

  10. Next-generation models for Canadian collaboration in international ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    ... enhanced and sustained collaboration between Canadian civil society and academia. ... with particular emphasis on the civil society and academic communities; ... in the fields of science, technology, engineering, and mathematics (STEM).

  11. Thalassaemia Trait with Gaucher Disease: A Diagnostic Dilemma.

    Science.gov (United States)

    Kini, Jyoti Ramnath; Sreeram, Saraswathy; Hegde, Anupama; Kamath, Sowmini; Pai, Radha Ramachandra

    2017-09-01

    Gaucher Disease is an autosomal recessive disease caused by the accumulation of glucocerebrosidase due to deficiency in lysosomal glucocerebrosidase. Thalassaemia trait is asymptomatic and is usually an incidental diagnosis. Both thalassaemia and Gaucher disease can have similar haematologic manifestations and hence, their coexistence causes diagnostic dilemma. Our patient presented at one-and-a-half years with weakness, pallor, failure to thrive and massive hepatosplenomegaly. Clinical examination and history pointed to a lipid storage disease. Peripheral smear revealed microcytic hypochromic cells and nucleated red cells with haemolytic blood picture. Thalassaemia trait was indicated on haemoglobin variant analysis using High Performance Liquid Chromatography. Liver biopsy, bone marrow aspirate and therapeutic splenectomy revealed Gaucher-like cells. Type 1 Gaucher disease can be clinically asymptomatic as well as present with massive liver and spleen enlargement and involvement of bone marrow. Anaemia, splenomegaly and thrombocytopenia are the usual presentations at diagnosis, similar to the haemoglobinopathies. Gaucher-like cells with normal beta-glucocerebrosidase (pseudo-Gaucher cells) are seen in thalassaemia, leukaemia, mycobacterial infections and myeloma. Gaucher disease coexisting with thalassaemia trait is uncommon. We report the occurrence of thalassaemia trait and Gaucher disease in a child, which resulted in confusion regarding the haematological diagnosis. This report highlights the necessity of independent establishment of the diagnosis in every patient so that appropriate management decisions are taken.

  12. Liver involvement in Gaucher disease - Review and clinical approach.

    Science.gov (United States)

    Adar, Tomer; Ilan, Yaron; Elstein, Deborah; Zimran, Ari

    2018-02-01

    Gaucher disease (GD), one of the most prevalent lysosomal storage diseases, is associated with glucocerebroside accumulation in cells of the monocyte-macrophage system in various organs, including the liver. Evaluating and managing liver disease in patients with Gaucher disease may be challenging. While hepatic involvement is common in Gaucher disease, its severity, and clinical significance span a wide spectrum, ranging from sub-clinical involvement to liver cirrhosis with its associated complications including portal hypertension. Apart from liver involvement in Gaucher disease, patients with may also suffer from other comorbidities involving the liver. That Gaucher disease itself can mimic hepatic lesions, affect laboratory tests used to characterize liver disease, and may be associated with non-cirrhotic portal hypertension, complicates the diagnostic approach even more. Better understanding of liver involvement in Gaucher disease can spare patients unnecessary invasive testing, and assist physicians in decision making when evaluating patients with Gaucher disease suspected for significant liver disease. This review describes the various clinical manifestations, laboratory and imaging abnormalities that may be encountered when following patients with Gaucher disease for liver involvement. The mechanism for liver disease are discussed, as well as the possible hepato-protective effect of glucocerebroside, and the a diagnostic and treatment approaches. Copyright © 2016 Elsevier Inc. All rights reserved.

  13. Pulmonary Gaucher's disease: high-resolution computed tomographic features

    International Nuclear Information System (INIS)

    Tunaci, A.; Berkmen, Y.M.; Goekmen, E.

    1995-01-01

    CT findings in pulmonary Gaucher's disease have not been previously reported. Chest radiograph of a patient with pulmonary involvement in type I Gaucher's disease proven by biopsy showed linear and reticulo-nodular opacities. High-resolution CT demonstrated thickening of the interlobular septa and between four and six small nodules within secondary lobules, probably each corresponding to an acinus. (orig.)

  14. Gaucher disease with jawbone involvement: a case report.

    Science.gov (United States)

    Ahmadieh, Azadeh; Farnad, Fariborz; Sedghizadeh, Parish P

    2014-11-05

    Gaucher disease is an autosomal recessive systemic condition, and the most common of the lysosomal storage disorders. It is characterized by lipid accumulation in certain cells and organs, particularly macrophages, which appear on light microscopy as 'Gaucher cells' or vacuolated lipid-laden reticuloendothelial cells. Long bone involvement is common in Gaucher disease, whereas craniofacial bone involvement is extremely rare. Reports confirming the diagnoses of Gaucher disease involving craniofacial bones by histopathologic evidence are even rarer. A 46-year-old Caucasian Ashkenazi Jewish woman with Gaucher disease presented with jawbone pain and lytic radiographic lesions of her mandible. Surgical biopsy of a mandibular lesion revealed Gaucher cells infiltrating the mandible, which correlated with radiographic and clinical findings, supporting a diagnosis of Gaucher disease with jawbone involvement. Lysosomal storage diseases can have head and neck manifestations, and bone involvement in Gaucher disease is common. Therefore, careful consideration of signs and symptoms and medical history, with a thorough review of systems, is important when evaluating patients with lysosomal storage disorders to rule out head and neck involvement of disease. Biopsy may be warranted in some cases for more definitive diagnosis of painful jawbone lesions and to rule out other odontogenic and non-odontogenic conditions in the differential diagnosis.

  15. Increased basal glucose production in type 1 Gaucher's disease

    NARCIS (Netherlands)

    Corssmit, E. P.; Hollak, C. E.; Endert, E.; van Oers, M. H.; Sauerwein, H. P.; Romijn, J. A.

    1995-01-01

    To evaluate the metabolic effects of Gaucher's disease, glucose metabolism and parameters of fat metabolism were studied by indirect calorimetry and primed continuous infusion of [3-3H]glucose in seven clinically stable untreated patients with type 1 Gaucher's disease and in seven healthy matched

  16. Radiologic findings of Korean gaucher disease

    International Nuclear Information System (INIS)

    Cho, Jae Hyun; Kim, Byoung Suck; Kim, Moon Kyu; Chung, Yoon Sok; Suh, Jung Ho; Kim, Hyon J; Ha, Doo Hoe

    1999-01-01

    To document the radiologic characteristics of Korean Gaucher disease. Fifteen bone marrow biopsy and laboratory data confirmed Gaucher disease patients (age 1-21, mean 10.9 yr) were undertaken plain X ray and MRI. Number of type I were 10, type II, 2, type III, 3. Seven were splencetomized on initial evaluation or during follow up. Five enzyme treated patient were undertaken follow-up MR examination during 6-40 month with 6 month interval. Conventional T1 and T2WI of spine and femur was performed and FMPSPGR in and out of phase image was also done. Volume of liver and spleen were measured, and bone marrow infiltration and presence of infarction were scored according to 6 scale scoring system. Clinical data were also reviewed and correlated with the MR findings. Marrow infiltration was noted in 71.4% of all patients in MRI, while it was in 45.7% with plain radiography. Type I group showed marrow infiltration in all but one cases, which was parallel with ages, SGPT, and presence of osteopenia, reversely correlated with spleen size. Severe bone complications (infarction of fracture) were noted in 7 of 10 type I group, and 6 patients showed severe growth retardation (below 3rd percentile). Follow up MR examination of 5 patient showed decrease in liver and spleen size first without bone change until 6 months. There showed bone regeneration in 2 patient 1 year after, and increased fat signal in one patient 3.5 years after. In and out of phase images couldn't help in quantifying fat composition in bone marrow. Korean Gaucher patients revealed as more severe skeletal complications than others reported from Western groups. MR examination is a effective modality to evaluate and monitor of Gaucher patients

  17. Type gaucher disease: radiographic and MRI manifestations

    International Nuclear Information System (INIS)

    Dong Yanqing; Li Kuncheng; Wang Yunzhao; Tian Ding

    1999-01-01

    Objective: To enhance the understanding of Gaucher disease (GD) type I bone involvement on imaging findings. Methods: The X-ray plain film and MRI findings of GD type I were reported, and literature reviewed. Results: The X-ray plain film of GD had characteristic change. The extent of bone involvement demonstrated could be depicted in longitudinal direction and the changes of marrow involvement on MRI. Conclusions: MRI is the best way to diagnose the bone involvement of GD

  18. Association Between Progranulin and Gaucher Disease

    OpenAIRE

    Jian, Jinlong; Zhao, Shuai; Tian, Qing-Yun; Liu, Helen; Zhao, Yunpeng; Chen, Wen-Chi; Grunig, Gabriele; Torres, Paola A.; Wang, Betty C.; Zeng, Bai; Pastores, Gregory; Tang, Wei; Sun, Ying; Grabowski, Gregory A.; Kong, Max Xiangtian

    2016-01-01

    Background: Gaucher disease (GD) is a genetic disease caused by mutations in the GBA1 gene which result in reduced enzymatic activity of β-glucocerebrosidase (GCase). This study identified the progranulin (PGRN) gene (GRN) as another gene associated with GD. Methods: Serum levels of PGRN were measured from 115 GD patients and 99 healthy controls, whole GRN gene from 40 GD patients was sequenced, and the genotyping of 4 SNPs identified in GD patients was performed in 161 GD and 142 healthy ...

  19. Animal models for Gaucher disease research

    OpenAIRE

    Farfel-Becker, Tamar; Vitner, Einat B.; Futerman, Anthony H.

    2011-01-01

    Gaucher disease (GD), the most common lysosomal storage disorder (LSD), is caused by the defective activity of the lysosomal hydrolase glucocerebrosidase, which is encoded by the GBA gene. Generation of animal models that faithfully recapitulate the three clinical subtypes of GD has proved to be more of a challenge than first anticipated. The first mouse to be produced died within hours after birth owing to skin permeability problems, and mice with point mutations in Gba did not display sympt...

  20. Advancing Diversity and Inclusion within the IceCube Collaboration: Lessons from an International Particle Astrophysics Research Collaboration

    Science.gov (United States)

    Knackert, J.

    2017-12-01

    The IceCube Collaboration is comprised of 300 scientists, engineers, students, and support staff at 48 institutions in 12 countries. IceCube recognizes the value of increased diversity within STEM fields and is committed to improving this situation both within the collaboration and more broadly. The process of establishing and maintaining a focus on diversity and inclusion within an international research collaboration has yielded many lessons and best practices relevant for broader STEM diversity efforts. Examples of events, training activities, and workshops to promote diversity both internally and within the broader STEM community will be provided. We will outline strategies to promote an environment of inclusivity and increase diversity in hiring within IceCube. We will describe collaborations with local networks and advocacy groups that have helped to guide our efforts and maximize their impact. We will also discuss methods for getting community members interested, informed, and invested, while helping them better understand the benefits associated with increased STEM diversity. This work has been informed by the American Association for the Advancement of Science's inaugural cohort of the Community Engagement Fellows Program. The author has made this submission on behalf of the IceCube Collaboration Diversity Task Force.

  1. Clinical manifestations and management of Gaucher disease.

    Science.gov (United States)

    Linari, Silvia; Castaman, Giancarlo

    2015-01-01

    Gaucher disease is a rare multi-systemic metabolic disorder caused by the inherited deficiency of the lysosomal enzyme β-glucocerebrosidase, which leads to the accumulation of its normal substrate, glucocerebroside, in tissue macrophages with damage to haematological, visceral and bone systems. Anaemia, thrombocytopenia, enlargement of liver and/or spleen, skeletal abnormalities (osteopenia, lytic lesions, pathological fractures, chronic bone pain, bone crisis, bone infarcts, osteonecrosis and skeletal deformities) are typical manifestations of the most prevalent form of the disease, the so-called non-neuronopathic type 1. However, severity and coexistence of different symptoms are highly variable. The determination of deficient β-glucocerebrosidase activity in leukocytes or fibroblasts by enzymatic assay is the gold standard for the diagnosis of Gaucher disease. Comprehensive and reproducible evaluation and monitoring of all clinically relevant aspects are fundamental for the effective management of Gaucher disease patients. Enzyme replacement therapy has been shown to be effective in reducing glucocerebroside storage burden and diminishing the deleterious effects caused by its accumulation. Tailored treatment plan for each patient should be directed to symptom relief, general improvement of quality of life, and prevention of irreversible damage.

  2. Gaucher disease and other storage disorders.

    Science.gov (United States)

    Grabowski, Gregory A

    2012-01-01

    In 1882, Philippe Gaucher described a 32-year-old woman with massive splenomegaly and unusually large cells in the spleen, which he called a "primary epithelioma of the spleen." The systemic nature and inheritance of the disease and its variants involving the viscera and CNS were described over the next century. The delineation of the causal enzymatic defects, genetics, molecular pathology, and genomics have provided pathogenic insights into the phenotypic spectrum and the bases for development of specific therapies for what is now known as Gaucher disease. As a prototype, the clinically and economically successful intracellular enzyme therapy provided the impetus for the expansion of similar research and therapeutic developments for other lysosomal storage diseases (LSDs) and orphan diseases, including Fabry, Pompe, and Niemann-Pick diseases, as well as several mucopolysaccharidoses. Continuing studies of such LSDs, which occur as a group in more than 7000 live births, have revealed the complex molecular interdigitation with the autophagy and apoptotic pathways and proteostasis and the impact of disruptions of the lysosomal/autophagy and proteostasis systems on more common diseases has been recognized. Examples include age-related neurodegenerative diseases (eg, Parkinson disease and Gaucher disease), idiopathic hypertrophic myocardiopathies, stroke and renal failure (eg, Fabry disease), and Nonalcoholic Fatty Liver Disease/Nonalcoholic SteatoHepatitis (NAFLD/NASH) and atherosclerosis (eg, lysosomal acid lipase deficiencies). Although perceived as rare, the availability of treatment and the impact of the LSDs on more common diseases require their integration into routine clinical practice.

  3. An avascular necrosis in Gaucher's disease

    International Nuclear Information System (INIS)

    Mansberg, R.; Uren, R.; Howman-Giles, R.

    1999-01-01

    Full text: Avascular necrosis is frequently associated with sickle cell disease and other haemoglobinopathies. It is less commonly associated with Gaucher's disease. A case with multi-modality imaging is presented. A 33-year-old male patient presented with a 4-day history of severe right knee pain. He was a febrile with mild swelling of the right knee. A diagnosis of Gaucher's disease had been made by bone marrow biopsy on a clinical picture of hepatosplenomegaly and thrombocytopenia some years earlier. A radiograph of the knee demonstrated an Erlenmeyer flask deformity of the distal femur. A bone scan demonstrated reduced perfusion to the distal right femoral shaft and femoral condyles. Delayed images demonstrated decreased tracer uptake in the distal right femur extending to the right medial femoral condyle consistent with a vascular necrosis. An MRI of the thighs demonstrated lipid accumulation in the marrow space of both femora consistent with Gaucher's disease associated with changes of bone oedema in the metadiaphysis and epiphysis of the right femur. The patient was treated with supportive measures and made an uneventful recovery and is being commenced on enzyme replacement therapy (Algucerase)

  4. Collaboration

    Science.gov (United States)

    King, Michelle L.

    2010-01-01

    This article explores collaboration between library media educators and regular classroom teachers. The article focuses on the context of the issue, positions on the issue, the impact of collaboration, and how to implement effective collaboration into the school system. Various books and professional journals are used to support conclusions…

  5. Summary of the findings of the International Collaboration on Mild Traumatic Brain Injury Prognosis

    DEFF Research Database (Denmark)

    Donovan, J.; Cancelliere, C.; Cassidy, J. D.

    2014-01-01

    In 2004, the WHO Collaborating Centre for Neurotrauma, Prevention, Management and Rehabilitation Task Force published the first large systematic review and best evidence synthesis on the clinical course and prognosis for recovery after MTBI. Ten years later, the International Collaboration on Mil...

  6. Internal and External Scripts in Computer-Supported Collaborative Inquiry Learning

    Science.gov (United States)

    Kollar, Ingo; Fischer, Frank; Slotta, James D.

    2007-01-01

    We investigated how differently structured external scripts interact with learners' internal scripts with respect to individual knowledge acquisition in a Web-based collaborative inquiry learning environment. Ninety students from two secondary schools participated. Two versions of an external collaboration script (high vs. low structured)…

  7. Social Network Analysis of 50 Years of International Collaboration in the Research of Educational Technology

    Science.gov (United States)

    Guo, Shesen; Zhang, Ganzhou; Guo, Yufei

    2016-01-01

    The definition of the field of educational technology has evolved over 50 years. New inventions and economic globalization increasingly facilitate people's communication for exchange of ideas and collaboration. This work attempts to describe international research collaboration in educational technology for the past 50 years. This article intends…

  8. "The good work is collaborative" | IDRC - International Development ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    2011-07-08

    Jul 8, 2011 ... ... own peer group and establish ongoing relations with them. “In my experience, economics is less and less a field where people are out there on their own, working independently in an isolated fashion. Nobody knows everything they need to know on a subject, so the good work these days is collaborative.

  9. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus

    DEFF Research Database (Denmark)

    Petri, Michelle; Orbai, Ana-Maria; Alarcón, Graciela S

    2012-01-01

    The Systemic Lupus International Collaborating Clinics (SLICC) group revised and validated the American College of Rheumatology (ACR) systemic lupus erythematosus (SLE) classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new...

  10. Demographics and patient characteristics of 1209 patients with Gaucher disease: Descriptive analysis from the Gaucher Outcome Survey (GOS)

    Science.gov (United States)

    Belmatoug, Nadia; Bembi, Bruno; Deegan, Patrick; Elstein, Deborah; Fernandez‐Sasso, Diego; Giraldo, Pilar; Goker‐Alpan, Ozlem; Lau, Heather; Lukina, Elena; Panahloo, Zoya; Schwartz, Ida Vanessa D.

    2017-01-01

    Abstract The Gaucher Outcome Survey (GOS) is an international Gaucher disease (GD) registry established in 2010 for patients with a confirmed GD diagnosis, regardless of GD type or treatment status, designed to evaluate the safety and long‐term effectiveness of velaglucerase alfa and other GD‐related treatments. As of February 25, 2017, 1209 patients had enrolled, the majority from Israel (44.3%) and the US (31.4%). Median age at GOS entry was 40.4 years, 44.1% were male, and 13.3% had undergone a total splenectomy. Most patients had type 1 GD (91.5%) and were of Ashkenazi Jewish ethnicity (55.8%). N370S/N370S was the most prevalent genotype, accounting for 44.2% of genotype‐confirmed individuals (n = 847); however, there was considerable variation between countries. A total of 887 (73.4%) patients had received ≥1 GD‐specific treatment at any time, most commonly imiglucerase (n = 587), velaglucerase alfa (n = 507), and alglucerase (n = 102). Hematological and visceral findings at the time of GOS entry were close to normal for most patients, probably a result of previous treatment; however, spleen volume of patients in Israel was almost double that of patients elsewhere (7.2 multiples of normal [MN] vs. 2.7, 2.9 and 4.9 MN in the US, UK and rest of world), which may be explained by a greater disease severity in this cohort. This analysis aimed to provide an overview of GOS and present baseline demographic and disease characteristics of participating patients to help improve the understanding of the natural history of GD and inform the overall management of patients with the disease. PMID:29090476

  11. Characteristics of 26 patients with type 3 Gaucher disease: A descriptive analysis from the Gaucher Outcome Survey.

    Science.gov (United States)

    Schwartz, Ida Vanessa D; Göker-Alpan, Özlem; Kishnani, Priya S; Zimran, Ari; Renault, Lydie; Panahloo, Zoya; Deegan, Patrick

    2018-03-01

    The Gaucher Outcome Survey (GOS) is an international disease-specific registry established in 2010 for patients with a confirmed diagnosis of Gaucher disease (GD), regardless of GD type or treatment status. Historically, there has been a limited understanding of type 3 GD (GD3) and its natural history in patients irrespective of their treatment status. Here, we describe the disease characteristics of patients with GD3 enrolled in GOS. As of October 2015, 1002 patients had been enrolled, 26 of whom were reported as GD3. The majority of patients with GD3 were from the US (13; 50.0%), seven (26.9%) were from the UK, three (11.5%) from Israel, and three (11.5%) from Brazil. No patients were of Ashkenazi Jewish origin. Median age of symptom onset was 1.4 (interquartile range: 0.5-2.0) years. The most common GBA1 mutation genotype was L444P/L444P, occurring in 16 (69.6%) of 23 patients who had genotyping information available. Nine patients reported a family history of GD (any type). Of 21 patients with treatment status information, 20 (95.2%) had received GD-specific treatment at any time, primarily imiglucerase (14 patients) and/or velaglucerase alfa (13 patients). Hemoglobin concentrations and platelet counts at GOS entry were within normal ranges for most patients, and there were no reports of severe hepatomegaly or of splenomegaly in non-splenectomized patients, most likely indicative of the effects of treatment received prior to GOS entry. This analysis provides information on the characteristics of patients with GD3 that could be used as the baseline for longitudinal follow-up of these patients.

  12. Characteristics of 26 patients with type 3 Gaucher disease: A descriptive analysis from the Gaucher Outcome Survey

    Directory of Open Access Journals (Sweden)

    Ida Vanessa D. Schwartz

    2018-03-01

    Full Text Available The Gaucher Outcome Survey (GOS is an international disease-specific registry established in 2010 for patients with a confirmed diagnosis of Gaucher disease (GD, regardless of GD type or treatment status. Historically, there has been a limited understanding of type 3 GD (GD3 and its natural history in patients irrespective of their treatment status. Here, we describe the disease characteristics of patients with GD3 enrolled in GOS. As of October 2015, 1002 patients had been enrolled, 26 of whom were reported as GD3. The majority of patients with GD3 were from the US (13; 50.0%, seven (26.9% were from the UK, three (11.5% from Israel, and three (11.5% from Brazil. No patients were of Ashkenazi Jewish origin. Median age of symptom onset was 1.4 (interquartile range: 0.5–2.0 years. The most common GBA1 mutation genotype was L444P/L444P, occurring in 16 (69.6% of 23 patients who had genotyping information available. Nine patients reported a family history of GD (any type. Of 21 patients with treatment status information, 20 (95.2% had received GD-specific treatment at any time, primarily imiglucerase (14 patients and/or velaglucerase alfa (13 patients. Hemoglobin concentrations and platelet counts at GOS entry were within normal ranges for most patients, and there were no reports of severe hepatomegaly or of splenomegaly in non-splenectomized patients, most likely indicative of the effects of treatment received prior to GOS entry. This analysis provides information on the characteristics of patients with GD3 that could be used as the baseline for longitudinal follow-up of these patients.

  13. India's participation in the ITER (International Thermonuclear Experimental Reactor) collaboration

    International Nuclear Information System (INIS)

    Deshpande, Shishir

    2012-01-01

    Keeping its vision of developing fusion energy as a viable source, India joined the ITER collaboration in December 2005. ITER is a seven party collaboration with China, EU, India, Japan, S. Korea, Russia and the USA. ITER has a challenging mission of achieving Q=10 figure of merit at 500 MW fusion power output. The construction of ITER is structured as a set of 'in-kind' procurement packages to be executed by the partners. This involves all activities like design, prototyping, testing, shipping and assembly with commissioning at the ITER site at Cadarache, France. Currently, ITER presents the only opportunity to carry out novel experiments with burning plasmas and the new realms of fusion physics. It is important to participate in such experiments with a view for their exploitation in future. This talk summarizes the ITER device, its key challenges, role played by India and how these enmesh with the future of domestic program in fusion research. (author)

  14. INL - NNL an International Technology Collaboration Case Study - Advanced Fogging Technologies for Decommissioning - 13463

    International Nuclear Information System (INIS)

    Banford, Anthony; Edwards, Jeremy; Demmer, Rick; Rankin, Richard; Hastings, Jeremy

    2013-01-01

    International collaboration and partnerships have become a reality as markets continue to globalize. This is the case in nuclear sector where over recent years partnerships commonly form to bid for capital projects internationally in the increasingly contractorized world and international consortia regularly bid and lead Management and Operations (M and O) / Parent Body Organization (PBO) site management contracts. International collaboration can also benefit research and technology development. The Idaho National Laboratory (INL) and the UK National Nuclear Laboratory (NNL) are internationally recognized organizations delivering leading science and technology development programmes both nationally and internationally. The Laboratories are actively collaborating in several areas with benefits to both the laboratories and their customers. Recent collaborations have focused on fuel cycle separations, systems engineering supporting waste management and decommissioning, the use of misting for decontamination and in-situ waste characterisation. This paper focuses on a case study illustrating how integration of two technologies developed on different sides of the Atlantic are being integrated through international collaboration to address real decommissioning challenges using fogging technology. (authors)

  15. INL - NNL an International Technology Collaboration Case Study - Advanced Fogging Technologies for Decommissioning - 13463

    Energy Technology Data Exchange (ETDEWEB)

    Banford, Anthony; Edwards, Jeremy [National Nuclear Laboratory, 5th Floor Chadwick House, Birchwood Park, Warrington WA3 6AE(United Kingdom); Demmer, Rick; Rankin, Richard [Idaho National Laboratory, Idaho Falls, ID 83401(United States); Hastings, Jeremy [National Nuclear Laboratory, Central Laboratory Sellafield, Seascale, Cumbria CA20 1PG (United Kingdom)

    2013-07-01

    International collaboration and partnerships have become a reality as markets continue to globalize. This is the case in nuclear sector where over recent years partnerships commonly form to bid for capital projects internationally in the increasingly contractorized world and international consortia regularly bid and lead Management and Operations (M and O) / Parent Body Organization (PBO) site management contracts. International collaboration can also benefit research and technology development. The Idaho National Laboratory (INL) and the UK National Nuclear Laboratory (NNL) are internationally recognized organizations delivering leading science and technology development programmes both nationally and internationally. The Laboratories are actively collaborating in several areas with benefits to both the laboratories and their customers. Recent collaborations have focused on fuel cycle separations, systems engineering supporting waste management and decommissioning, the use of misting for decontamination and in-situ waste characterisation. This paper focuses on a case study illustrating how integration of two technologies developed on different sides of the Atlantic are being integrated through international collaboration to address real decommissioning challenges using fogging technology. (authors)

  16. Minority International Research Training Program: Global Collaboration in Nursing Research.

    Science.gov (United States)

    McElmurry, Beverly J.; Misner, Susan J.; Buseh, Aaron G.

    2003-01-01

    The Minority International Research Training Program pairs minority nursing students with faculty mentors at international sites for short-term research. A total of 26 undergraduate, 22 graduate, and 6 postdoctoral students have participated. Challenges include recruitment, orientation, and preparation of students; identification and preparation…

  17. International collaboration in science: The global map and the network

    NARCIS (Netherlands)

    Leydesdorff, L.; Wagner, C.S.; Park, H.W.; Adams, J.

    2013-01-01

    The network of international co-authorship relations has been dominated by certain European nations and the USA, but this network is rapidly expanding at the global level. Between 40 and 50 countries appear in the center of the international network in 2011, and almost all (201) nations are nowadays

  18. Role of international collaboration in PNC's R ampersand D programme for HLW disposal

    International Nuclear Information System (INIS)

    Masuda, Sumio; Umeki, Hiroyuki; Yamakawa, Minoru

    1996-01-01

    PNC has been active in promoting international cooperation in connection with the Japanese HLW disposal programme, based on both a bilateral and multilateral approach. Both types of cooperation are extremely useful; in particular, bilateral cooperation has the advantage of providing opportunities for in-depth discussions in mutual areas of interest. By way of contrast, multilateral cooperation also provides an international arena for broader discussion and corroboration of output from individual R ampersand D programmes. International collaboration also provides young researchers with an opportunity to learn from experience. Depending on the issues to be tackled, appropriate forms of collaboration have been integrated into PNC's strategy for maximizing output. The lessons learned from collaboration are very valuable and can be used directly in their programme to enhance its credibility. The format of collaboration has also been extensively developed: it has been found that resources can be utilized more effectively by sharing them appropriately

  19. International collaborative study for the calibration of proposed International Standards for thromboplastin, rabbit, plain and for thromboplastin, recombinant, human, plain

    DEFF Research Database (Denmark)

    van den Besselaar, A M H P; Chantarangkul, V; Angeloni, F

    2018-01-01

    BACKGROUND: The availability of International Standards for thromboplastin is essential for the calibration of routine reagents and hence the calculation of the International Normalized Ratio (INR). Stocks of the current 4(th) International Standards are running low. Candidate replacement materia......) international standard (rTF/09). The candidate materials have been accepted by WHO as the 5(th) International Standards for thromboplastin, rabbit plain, and thromboplastin, recombinant, human, plain. This article is protected by copyright. All rights reserved.......BACKGROUND: The availability of International Standards for thromboplastin is essential for the calibration of routine reagents and hence the calculation of the International Normalized Ratio (INR). Stocks of the current 4(th) International Standards are running low. Candidate replacement materials...... have been prepared. This report describes the calibration of the proposed 5(th) International Standards for thromboplastin, rabbit, plain (coded RBT/16) and for thromboplastin, recombinant, human, plain (coded rTF/16). METHODS: An international collaborative study was carried out for the assignment...

  20. Trust Management - Building Trust for International Cross Disciplinary Collaboration on Climate Change

    Science.gov (United States)

    Oakley, K. V.; Gurney, R. J.

    2014-12-01

    Successful communication and collaboration entails mutual understanding, and transfer, of information. The risk of misunderstanding and/or miscommunication between collaborating groups is tackled in different ways around the globe; some are well documented whereas others may be unknown outside particular groups, whether defined geographically or by specialism. For example; in some countries legally binding contracts define the terms of collaboration. Some regions place greater emphasis on developing trust relationships, and sometimes an official agreement is implied, such as many electronic data transfers on the web. International collaboration on climate change increasingly involves electronic data exchange (e.g. open access publications, shared documents, data repositories etc.) and with this increased reliance on electronic data a need has arisen for scientists to collaborate both internationally and cross-disciplinarily particularly with information technology and data management specialists. Trust of data and metadata on the internet (e.g. privacy, legitimacy etc.) varies, possibly due to a lack of internationally agreed standards for data governance and management, leaving many national, regional and institutional practices tailored to the needs of that group only. It is proposed that building trust relationships between cross-disciplinary and international groups could help facilitate further communication, understanding and benefits from the relationship, while still maintaining independence as separate groups. Complex international cross-disciplinary group relationship dynamics are not easily mapped and producing a set of trust building rules that can be applied to any current and future collaboration with equal validity may be unfeasible. An alternative to such a set of rules may be found in a Trust Manager, whose role is to improve mutually beneficial knowledge exchange between groups, build trust and increase future collaborative potential. This

  1. Extraosseous manifestation of Gaucher's disease type I: MR and histological appearance

    International Nuclear Information System (INIS)

    Poll, L.W.; Koch, J.A.; Moedder, U.

    2000-01-01

    Gaucher's disease type I is the most prevalent lysosomal storage disorder caused by an autosomal-recessive inherited deficiency of glucocerebrosidase activity with secondary accumulation of glucocerebrosides within the lysosomes of macrophages. The storage disorder produces a multisystem disease characterized by progressive visceral enlargement and gradual replacement of bone marrow with lipid-laden macrophages. Skeletal disease is a major source of disability in Gaucher's disease. Extraosseous extension of Gaucher cells is an extremely rare manifestation of skeletal Gaucher's disease. This is a report on the MRI and histopathological findings of an extraosseous Gaucher-cell extension into the midface in a patient with Gaucher's disease. (orig.)

  2. Extraosseous extension of Gaucher cell deposits mimicking malignancy

    International Nuclear Information System (INIS)

    Hermann, G.; Shapiro, R.; Abdelwahab, I.F.; Klein, M.J.; Pastores, G.; Grabowski, G.

    1994-01-01

    Two cases are described in which patients with type I Gaucher disease developed extraosseous soft tissue masses consisting of Gaucher cell deposits. In one instance the mass destroyed the posterior cortex of the left distal femur and protruded into the soft tissues. In the second case the lesion involved the proximal tibia and gradually extended into the soft tissues. While the incidence of neoplastic disorder such as lymphoproliferative disease appears to be more common in Gaucher disease patients than in the general population, lesions of benign etiology that mimic these aggressive processes should be considered in the differential diagnosis when cortical destruction with coexisting soft tissue most is found in these patients. (orig.)

  3. Extraosseous extension of Gaucher cell deposits mimicking malignancy

    Energy Technology Data Exchange (ETDEWEB)

    Hermann, G. (Dept. of Radiology, Mount Sinai Medical Center of the City Univ. of New York, NY (United States)); Shapiro, R. (Dept. of Radiology, Mount Sinai Medical Center of the City Univ. of New York, NY (United States)); Abdelwahab, I.F. (Dept. of Radiology, Mount Sinai Medical Center of the City Univ. of New York, NY (United States)); Klein, M.J. (Dept. of Pathology, Mount Sinai Center of the City Univ. of New York, NY (United States)); Pastores, G. (Dept. of Human Genetics, Mount Sinai Medical Center of the City Univ. of New York, NY (United States)); Grabowski, G. (Cincinnati Children' s Hospital, Cincinnati Univ., Coll. of Medicine, OH (United States))

    1994-05-01

    Two cases are described in which patients with type I Gaucher disease developed extraosseous soft tissue masses consisting of Gaucher cell deposits. In one instance the mass destroyed the posterior cortex of the left distal femur and protruded into the soft tissues. In the second case the lesion involved the proximal tibia and gradually extended into the soft tissues. While the incidence of neoplastic disorder such as lymphoproliferative disease appears to be more common in Gaucher disease patients than in the general population, lesions of benign etiology that mimic these aggressive processes should be considered in the differential diagnosis when cortical destruction with coexisting soft tissue most is found in these patients. (orig.)

  4. The Human Genome Project: An Imperative for International Collaboration.

    Science.gov (United States)

    Allende, J. E.

    1989-01-01

    Discussed is the Human Genome Project which aims to decipher the totality of the human genetic information. The historical background, the objectives, international cooperation, ethical discussion, and the role of UNESCO are included. (KR)

  5. Deep Learning: Enriching Teacher Training through Mobile Technology and International Collaboration

    Science.gov (United States)

    Naylor, Amanda; Gibbs, Janet

    2018-01-01

    This article presents results from an international collaboration between college students and pre-service teachers in Norway and the UK. This research is part of a large, international project exploring and developing the interrelationship between mobile technology and teachers' perceptions of teaching and learning. Data was collected for this…

  6. Overview of the PPPL International Experimental Stellarator Collaboration Activity

    Energy Technology Data Exchange (ETDEWEB)

    Gates, David [Princeton University

    2012-03-28

    PPPL has initiated and strengthened collaborative experimental programs aimed at developing the required toolsets and scientific knowledge for advancing stellarators as a viable fusion energy source. In particular, activities at LHD and W7-X, the two large superconducting helical confinement systems in the world, have been expanded. The focus at LHD has been on diagnostic development and data analysis, since the device is a mature research facility with more than 20MW of heating power available. High beta stability experiments, ion and electron temperature measurements using a recently installed imaging x-ray crystal spectrometer, and 3D equilibrium reconstructions will be described. The focus on W7-X has been to develop hardware capabilities for divertor heat flux control, including plasma-facing components, error field correction coils, and power supplies. Progress on these and other activities will be presented.

  7. European labs brace for German cuts: international collaboration

    CERN Multimedia

    Clery, D

    1996-01-01

    Germany, the largest contributor to international European research labs, announced plans to reduce its contributions an average of 8% in the nation's latest budget. CERN and other labs are worried that the cuts will endanger ongoing projects and that other countries may follow Germany's lead.

  8. Telework 96: An International Collaborative Learning Package for Information Entrepreneurs.

    Science.gov (United States)

    Davenport, Elisabeth; Wormell, Irene

    1997-01-01

    Describes the implementation of an international joint course developed by Queen Margaret College in Scotland and the Royal School of Librarianship in Denmark introducing undergraduate students to tools and issues relevant to teleworking, or working at home. Discusses course objectives, class assignments, student assessment, and program…

  9. The North American Development Partnership: Experiment in International Collaboration.

    Science.gov (United States)

    Carlson, Burton L.; Goguen, Robert A.; Jarvis, Phillip S.; Lester, Juliette N.

    2000-01-01

    Describes how career development programs became the focus of an international partnership between the United States and Canada. Discusses the partnership's efforts at developing training and materials that promote the use of occupational and labor markets information and the creation of a computer-based career information delivery system.…

  10. A Study on planning of the international collaboration foundation for the Advanced Nuclear Technology Development Project

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Moon Hee; Kim, H. R.; Kim, H. J. and others

    2005-03-15

    Korea has participated in the international collaboration programs for the development of future nuclear energy systems driven by the countries holding advanced nuclear technology and Korea and U.S. have cooperated in the INERI. This study aimed mainly at developing the plan for participation in the collaborative development of the Gen IV, searching the participation strategy for INERI and the INPRO, and the international cooperation in these programs. Contents and scope of the study for successful achievement are as follows; Investigation and analysis of international and domestic trends related to advanced nuclear technologies, Development of the plan for collaborative development of the Gen IV and conducting the international cooperation activities, Support for the activities related to I-NERI between Korea and U.S. and conducting the international cooperation, International cooperation activities for the INPRO. This study can be useful for planning the research plan and setting up of the strategy of integrating the results of the international collaboration and the domestic R and D results by combining the Gen IV and the domestic R and D in the field of future nuclear technology. Futhermore, this study can contribute to establishing the effective foundation and broadening the cooperation activities not only with the advanced countries for acquisition of the advanced technologies but also with the developing countries for the export of the domestic nuclear energy systems.

  11. A Study on planning of the international collaboration foundation for the Advanced Nuclear Technology Development Project

    International Nuclear Information System (INIS)

    Chang, Moon Hee; Kim, H. R.; Kim, H. J. and others

    2005-03-01

    Korea has participated in the international collaboration programs for the development of future nuclear energy systems driven by the countries holding advanced nuclear technology and Korea and U.S. have cooperated in the INERI. This study aimed mainly at developing the plan for participation in the collaborative development of the Gen IV, searching the participation strategy for INERI and the INPRO, and the international cooperation in these programs. Contents and scope of the study for successful achievement are as follows; Investigation and analysis of international and domestic trends related to advanced nuclear technologies, Development of the plan for collaborative development of the Gen IV and conducting the international cooperation activities, Support for the activities related to I-NERI between Korea and U.S. and conducting the international cooperation, International cooperation activities for the INPRO. This study can be useful for planning the research plan and setting up of the strategy of integrating the results of the international collaboration and the domestic R and D results by combining the Gen IV and the domestic R and D in the field of future nuclear technology. Futhermore, this study can contribute to establishing the effective foundation and broadening the cooperation activities not only with the advanced countries for acquisition of the advanced technologies but also with the developing countries for the export of the domestic nuclear energy systems

  12. A Study on planning of promotion for international collaborative development of Generation IV Nuclear Energy Systems

    International Nuclear Information System (INIS)

    Hee, Chang Moon; Yang, M. S.; Ha, J. J.

    2006-06-01

    Korea has participated in the international collaboration programs for the development of future nuclear energy systems driven by the countries holding advanced nuclear technology and Korea and U. S. have cooperated in the INERI. This study is mainly at developing the plan for participation in the collaborative development of the Gen IV, searching the participation strategy for INERI and the INPRO, and the international cooperation in these programs. Contents and scope of the study for successful achievement are as follows; - Investigation and analysis of international and domestic trends related to advanced nuclear technologies - Development of the plan for collaborative development of the Gen IV and conducting the international cooperation activities - Support for the activities related to I-NERI between Korea and U. S. and conducting the international cooperation - International cooperation activities for the INPRO This study can be useful for planning the research plan and setting up of the strategy of integrating the results of the international collaboration and the domestic R and D results by combining the Gen IV and the domestic R and D in the field of future nuclear technology. Furthermore, this study can contribute to establishing the effective foundation and broadening the cooperation activities not only with the advanced countries for acquisition of the advanced technologies but also with the developing countries for the export of the domestic nuclear energy systems

  13. Appendiceal involvement in a patient with Gaucher disease.

    Science.gov (United States)

    Kocic, Marija; Djuricic, Slavisa M; Djordjevic, Maja; Savic, Djordje; Kecman, Bozica; Sarajlija, Adrijan

    2018-02-01

    Almost any anatomical compartment may be involved in Gaucher disease (GD). Abdominal lymphadenopathy occurred during enzyme replacement therapy in more than a dozen children with GD so far. A fourteen-year-old boy from Serbia developed clinical signs of acute appendicitis six years after the onset of GD type 3 related abdominal lymphadenopathy. Ultrasound examination showed diffuse thickening of the intestinal wall in the ileocoecal region with periappendicular infiltration. An appendectomy was performed four months after conservative treatment with antibiotics. Histopathology revealed macrophages with cytological characteristics of Gaucher cells densely crammed in mesoappendiceal adipose tissue. Also the multifocal replacement of subserosal tissue by Gaucher cells and their infiltration to a variable depth of muscularis propria of the appendix were verified. Frank infiltration of the vermiform appendix with Gaucher cells represents a novel observation in a wide spectrum of manifestations reported in GD. A possible causative relationship of this infiltration with appendicitis is considered. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Gaucher disease. Unusual presentation and mini-review.

    Science.gov (United States)

    Rizk, Tamer M; Ariganjoye, Rafiu O; Alsaeed, Gihad I

    2015-07-01

    We aim to describe an 8-year-old boy with an unusual clinical presentation of Gaucher disease (GD). Gaucher disease is a progressive lysosomal storage disorder due to deficiency of the specific enzyme glucocerebrosidase with varying clinical features, but often involving the monocytes-macrophages systems. This child ran a progressive course with a devastating outcome. Three distinct GD subtypes have been described with varying clinical features based on the presence or absence of neurologic involvement. Gaucher disease diagnosis is obtained via: enzyme activity assay, gene mutation study, bone marrow aspiration in addition to multiple other tests that have been successfully used in diagnosis of cases of GD. Treatment modalities include enzyme replacement treatment, substrate reduction therapy, bone marrow transplantation, blood transfusion, and surgery are available management modalities for GD. Gaucher disease is a chronic disease requiring a multidisciplinary team approach with regular follow up with multiple subspecialties.

  15. GBAmutations in Gaucher type I Venezuelan patients: ethnic origins ...

    Indian Academy of Sciences (India)

    Gilberto GÓmez

    2017-09-08

    Sep 8, 2017 ... In Venezuela, 20 unrelated index cases with GD type I were ..... S, splenomegaly; T, thrombocytopenia; Bc, bone crisis; Hy, hypotonia; NA, data not available; ..... Management of neuronopathic Gaucher disease: a European.

  16. Cytokines in Gaucher disease: Role in the pathogenesis of bone ...

    African Journals Online (AJOL)

    Azza A.G. Tantawy

    2015-03-03

    Mar 3, 2015 ... The impact of therapy on bone manifestations of Gaucher disease . ... types: classical or alternative, depending on the predominant cytokine in the .... avascular necrosis, bone infarcts and localised cortical thin- ning may be ...

  17. Mild thrombocytopenia as presenting symptom of type 1 Gauchers's disease.

    Science.gov (United States)

    Müzes, G; Pitlik, E; Somogyi, A; Tulassay, Z

    2001-06-01

    A young woman was examined for a mild thrombocytopenia which was present for some months. No signs of bleeding had so far occurred. Physical examination was normal except for a moderately enlarged spleen. Laboratory investigations showed a low platelet count. There was no evidence of an autoimmune or hematologic disease. Bone narrow aspirate indicated Gaucher's-like cells raising the suspicion of Gaucher's disease. This was further supported by electron microscopic demonstration of Gaucher's bodies in crista biopsy specimens. However, the definitive diagnosis was obtained by verifying deficient lysosomal glucosylceramide-beta-D-glucosidase activity in peripheral blood leukocytes. Upon the absence of neurologic involvement the patient was typical for the adult-onset or type 1 form of Gaucher's disease.

  18. [An adult form of type-I. Gaucher's disease].

    Science.gov (United States)

    Múzes, G; Pitlik, E; Gohér, A; Somogyi, A; Tulassay, Z

    2000-03-26

    A young woman with no previous history of any diseases was admitted for further evaluation of a mild thrombocytopenia she has had for some months. No signs of bleeding have so far occurred. Physical examination was normal except for a moderately enlarged spleen. Routine investigations showed lower platelet count. There was no laboratory evidence of disease conditions with autoimmune/inflammatory or hematologic origin. Bone marrow aspirate indicated Gaucher's-like cells raising the suspicion of Gaucher's disease. This was further supported by electron microscopic demonstration of Gaucher's bodies (with the characteristic tubular structures) in crista biopsy specimens. However, definitive diagnosis was obtained by verifying deficient lysosomal glucosylceramide-beta-D-glucosidase activity in peripheral blood leukocytes. Upon the absence of neurologic involvement the patient was typical for the adult form or type-1 Gaucher's disease.

  19. International stem cell collaboration: how disparate policies between the United States and the United Kingdom impact research.

    Science.gov (United States)

    Luo, Jingyuan; Flynn, Jesse M; Solnick, Rachel E; Ecklund, Elaine Howard; Matthews, Kirstin R W

    2011-03-08

    As the scientific community globalizes, it is increasingly important to understand the effects of international collaboration on the quality and quantity of research produced. While it is generally assumed that international collaboration enhances the quality of research, this phenomenon is not well examined. Stem cell research is unique in that it is both politically charged and a research area that often generates international collaborations, making it an ideal case through which to examine international collaborations. Furthermore, with promising medical applications, the research area is dynamic and responsive to a globalizing science environment. Thus, studying international collaborations in stem cell research elucidates the role of existing international networks in promoting quality research, as well as the effects that disparate national policies might have on research. This study examined the impact of collaboration on publication significance in the United States and the United Kingdom, world leaders in stem cell research with disparate policies. We reviewed publications by US and UK authors from 2008, along with their citation rates and the political factors that may have contributed to the number of international collaborations. The data demonstrated that international collaborations significantly increased an article's impact for UK and US investigators. While this applied to UK authors whether they were corresponding or secondary, this effect was most significant for US authors who were corresponding authors. While the UK exhibited a higher proportion of international publications than the US, this difference was consistent with overall trends in international scientific collaboration. The findings suggested that national stem cell policy differences and regulatory mechanisms driving international stem cell research in the US and UK did not affect the frequency of international collaborations, or even the countries with which the US and UK most

  20. Supply chain process collaboration and Internet utilization: an international perspective of business to business relationships

    Directory of Open Access Journals (Sweden)

    Marcos Paulo Valadares de Oliveira

    2015-01-01

    Full Text Available This paper compiles the findings of an international study which primary objective was to investigate the relationships between Internet utilization in business-to-business relationships, collaborative efforts and their impact over supplier and customer-oriented processes performance. It highlights the Internet as an important enhancer of collaboration in supply chains and addresses the effects of such efforts on companies’ overall performance. As a conclusive-descriptive and quantitative study, data from a survey of 788 companies from the USA, China, Canada, United Kingdom, and Brazil were analyzed with the use of descriptive statistics, reliability evaluation of the research model’s internal scales, path analysis and structural equation modeling to evaluate supply chain processes collaboration, both up- and down-stream. Internet utilization in supplier and customer-oriented processes was found positively related to collaborative practices in business-to-business relationships. Collaborative practices in supplier and customer-oriented processes, in turn, showed potential effects on performance. Also, supplier-oriented processes performance was found positively associated with customer-oriented process performance. Both internet use and collaborative practices are even more important in a high-context country like Brazil. The paper helps clarify the impact of internet use on business-to-business collaborative relationships. In this sense, practitioners can take this impact to redraw the organizational landscape and business processes amongst supply chain participants.

  1. International collaboration, the route to fuel cycle research and development

    International Nuclear Information System (INIS)

    Tinsley, T.; Mathers, D.; Rayment, F.

    2013-01-01

    In hindsight, involvement with European Framework projects such as GoFastR (Gas-cooled Fast Reactors) and ACSEPT (Actinide Recycling by Separation and Transmutation) was a crucial and, at the time, an innovative step in maintaining the UK skills base during a period of major changes in the UK nuclear industry. It has undoubtedly delivered the objectives intended in terms of maintenance of the key skills, developing and training new staff, regenerating facilities and building strong links with the European nuclear research community. Over the last 2-3 years NNL's participation in European projects has moved forward such that NNL (National Nuclear Laboratory) is an integral partner of several major projects, fully engaged with delivering the core objectives of the projects and intent on forging deep collaborations with key organisations across Europe. With the renewed interest in nuclear energy and future fuel cycle options in the UK, NNL is now well positioned to contribute at an even deeper level in European level programmes

  2. Delivering accessible fieldwork: preliminary findings from a collaborative international study

    Science.gov (United States)

    Stokes, Alison; Atchison, Christopher; Feig, Anthony; Gilley, Brett

    2017-04-01

    Students with disabilities are commonly excluded from full participation in geoscience programs, and encounter significant barriers when accessing field-learning experiences. In order to increase talent and diversity in the geoscience workforce, more inclusive learning experiences must be developed that will enable all students to complete the requirements of undergraduate degree programs, including fieldwork. We discuss the outcomes of a completely accessible field course developed through the collaborative effort of geoscience education practitioners from the US, Canada and the UK. This unique field workshop has brought together current geoscience academics and students with disabilities to share perspectives on commonly-encountered barriers to learning in the field, and explore methods and techniques for overcoming them. While the student participants had the opportunity to learn about Earth processes while situated in the natural environment, participating geoscience instructors began to identify how to improve the design of field courses, making them fully inclusive of learners with disabilities. The outcomes from this experience will be used to develop guidelines to facilitate future development and delivery of accessible geoscience fieldwork.

  3. FEBEX: An example of a major international collaborative project

    International Nuclear Information System (INIS)

    Ulibarri, A.M.; Olmo, C. del; Huertas, F.

    1996-01-01

    There are many similarities in the high-level waste (HLW) disposal programmes in Switzerland and Spain. In both cases, alternative crystalline and sedimentary host rocks are currently under investigation, options for disposal of both vitrified reprocessing waste and spent fuel are considered and repository designs utilize massive engineered barriers. For the case of HLW disposal in a granite rock, the reference engineered barrier system (EBS) concepts are almost identical. The waste, in its steel fabrication container, is sealed in a massive steel canister which is emplaced horizontally in drilled tunnels. The canister is surrounded by a highly compacted bentonite backfill. Individual components of this waste package have been tested in isolation or on a small scale, but the aim of the full-scale engineered barrier experiment is to examine some properties of a real size system in a realistic natural environment. FEBEX was proposed by ENRESA and the experimental studies at Grimsel are run as an ENRESA/NAGRA collaboration. The field experiments are, however, only one component of a project which includes a large-scale laboratory 'mockup' and supporting materials tests and modelling. FEBEX, as a whole, is sponsored by the European Union as part of the 'Nuclear Fission Safety' research programme (the Swiss component being supported by the Bundesamt fur Bildung und Wissenschaft)

  4. International collaboration, the route to fuel cycle research and development

    Energy Technology Data Exchange (ETDEWEB)

    Tinsley, T.; Mathers, D.; Rayment, F. [National Nuclear Laboratory, Central Laboratory, Sellafield, Seascale, Cumbria, CA20 1PG (United Kingdom)

    2013-07-01

    In hindsight, involvement with European Framework projects such as GoFastR (Gas-cooled Fast Reactors) and ACSEPT (Actinide Recycling by Separation and Transmutation) was a crucial and, at the time, an innovative step in maintaining the UK skills base during a period of major changes in the UK nuclear industry. It has undoubtedly delivered the objectives intended in terms of maintenance of the key skills, developing and training new staff, regenerating facilities and building strong links with the European nuclear research community. Over the last 2-3 years NNL's participation in European projects has moved forward such that NNL (National Nuclear Laboratory) is an integral partner of several major projects, fully engaged with delivering the core objectives of the projects and intent on forging deep collaborations with key organisations across Europe. With the renewed interest in nuclear energy and future fuel cycle options in the UK, NNL is now well positioned to contribute at an even deeper level in European level programmes.

  5. The International Energy Agency collaboration in wind energy

    International Nuclear Information System (INIS)

    Beurskens, H.J.M.; Pershagen, B.

    1991-07-01

    The International Energy Agency (IEA) wind energy agreements have provided a useful framework for international cooperative efforts during more than thirteen years. Nine comprehensive research Tasks have been successfully completed and three Tasks are currently in progress. The sharing of research and information has clearly contributed to the development of wind technology, has eliminated unnecessary redundancy in national programmes, has encouraged utilization of the most efficient approaches to solve common problems, and has created a cooperative spirit among the professional groups that seems to be unique. After a brief introduction on the activities of the IEA on wind energy an overview is given of the ongoing tasks and other current activities with regard to the subject. 1 fig., 5 tabs., 9 refs

  6. Standard methods for sampling freshwater fishes: opportunities for international collaboration

    OpenAIRE

    Bonar, Scott A.; Mercado-Silva, Norman; Hubert, Wayne A.; Beard, T. Douglas; Dave, Göran; Kubečka, Jan; Graeb, Brian D.S.; Lester, Nigel P.; Porath, Mark; Winfield, Ian J.

    2017-01-01

    With publication of Standard Methods for Sampling North American Freshwater Fishes in 2009, the American Fisheries Society (AFS) recommended standard procedures for North America. To explore interest in standardizing at intercontinental scales, a symposium attended by international specialists in freshwater fish sampling was convened at the 145th Annual AFS Meeting in Portland, Oregon, in August 2015. Participants represented all continents except Australia and Antarctica and were employed by...

  7. Project-based learning with international collaboration for training biomedical engineers.

    Science.gov (United States)

    Krishnan, Shankar

    2011-01-01

    Training biomedical engineers while effectively keeping up with the fast paced scientific breakthroughs and the growth in technical innovations poses arduous challenges for educators. Traditional pedagogical methods are employed for coping with the increasing demands in biomedical engineering (BME) training and continuous improvements have been attempted with some success. Project-based learning (PBL) is an academic effort that challenges students by making them carry out interdisciplinary projects aimed at accomplishing a wide range of student learning outcomes. PBL has been shown to be effective in the medical field and has been adopted by other fields including engineering. The impact of globalization in healthcare appears to be steadily increasing which necessitates the inclusion of awareness of relevant international activities in the curriculum. Numerous difficulties are encountered when the formation of a collaborative team is tried, and additional difficulties occur as the collaboration team is extended to international partners. Understanding and agreement of responsibilities becomes somewhat complex and hence the collaborative project has to be planned and executed with clear understanding by all partners and participants. A model for training BME students by adopting PBL with international collaboration is proposed. The results of previous BME project work with international collaboration fit partially into the model. There were many logistic issues and constraints; however, the collaborative projects themselves greatly enhanced the student learning outcomes. This PBL type of learning experience tends to promote long term retention of multidisciplinary material and foster high-order cognitive activities such as analysis, synthesis and evaluation. In addition to introducing the students to experiences encountered in the real-life workforce, the proposed approach enhances developing professional contracts and global networking. In conclusion, despite

  8. Elevated plasma glucosylsphingosine in Gaucher disease: relation to phenotype, storage cell markers, and therapeutic response

    NARCIS (Netherlands)

    Dekker, Nick; van Dussen, Laura; Hollak, Carla E. M.; Overkleeft, Herman; Scheij, Saskia; Ghauharali, Karen; van Breemen, Mariëlle J.; Ferraz, Maria J.; Groener, Johanna E. M.; Maas, Mario; Wijburg, Frits A.; Speijer, Dave; Tylki-Szymanska, Anna; Mistry, Pramod K.; Boot, Rolf G.; Aerts, Johannes M.

    2011-01-01

    Gaucher disease, caused by a deficiency of the lysosomal enzyme glucocerebrosidase, leads to prominent glucosylceramide accumulation in lysosomes of tissue macrophages (Gaucher cells). Here we show glucosylsphingosine, the deacylated form of glucosylceramide, to be markedly increased in plasma of

  9. Type I Gaucher disease: extraosseous extension of skeletal disease

    International Nuclear Information System (INIS)

    Poll, L.W.; Koch, J.A.; Moedder, U.; Dahl, S. vom; Haeussinger, D.; Sarbia, M.; Niederau, C.

    2000-01-01

    Objective. To investigate the frequency and morphology of extraosseous extension in patients with Gaucher disease type I.Design and patients. MRI examinations of the lower extremities were analyzed in 70 patients with Gaucher disease type I. Additionally, the thoracic spine and the midface were investigated on MRI in two patients.Results. Four cases are presented in which patients with Gaucher disease type I and severe skeletal involvement developed destruction or protrusion of the cortex with extraosseous extension into soft tissues. In one patient, Gaucher cell deposits destroyed the cortex of the mandible and extended into the masseter muscle. In the second patient, multiple paravertebral masses with localized destruction of the cortex were apparent in the thoracic spine. In the third and fourth patient, cortical destruction with extraosseous tissue extending into soft tissues was seen in the lower limbs.Conclusions. Extraosseous extension is a rare manifestation of Gaucher bone disease. While an increased risk of cancer, especially hematopoietic in origin, is known in patients with Gaucher disease, these extraosseous benign manifestations that may mimic malignant processes should be considered in the differential diagnosis of extraosseous extension into soft tissues. A narrow neck of tissue was apparent in all cases connecting bone and extraosseous extensions. (orig.)

  10. Gaucher disease and the synucleinopathies: refining the relationship

    Directory of Open Access Journals (Sweden)

    Campbell Tessa N

    2012-01-01

    Full Text Available Abstract Gaucher disease (OMIM 230800, 230900, 231000, the most common lysosomal storage disorder, is due to a deficiency in the enzyme glucocerebrosidase. Gaucher patients display a wide spectrum of clinical presentation, with hepatosplenomegaly, haematological changes, and orthopaedic complications being the predominant symptoms. Gaucher disease is classified into three broad phenotypes based upon the presence or absence of neurological involvement: Type 1 (non-neuronopathic, Type 2 (acute neuronopathic, and Type 3 (subacute neuronopathic. Nearly 300 mutations have been identified in Gaucher patients, with the majority being missense mutations. Though studies of genotype-to-phenotype correlations have revealed significant heterogeneity, some consistent patterns have emerged to inform prognostic and therapeutic decisions. Recent research has highlighted a potential role for Gaucher disease in other comorbidities such as cancer and Parkinson's Disease. In this review, we will examine the potential relationship between Gaucher disease and the synucleinopathies, a group of neurodegenerative disorders characterized by the development of intracellular aggregates of α-synuclein. Possible mechanisms of interaction will be discussed.

  11. Crossing boundaries to improve mental health in the Americas: international collaborative authorship.

    Science.gov (United States)

    Killion, Chery M

    2007-01-01

    A nurse anthropologist with a background in international collaborations attended Project LEAD for two years, which enabled her to continue to serve as an advocate for the mentally ill in Belize. The anthropologist collaborated with a psychiatrist from Belize to develop a cross-cultural, cross-discipline publication, "Mental Health in Belize: A National Priority, " which highlights the work of psychiatric nurse practitioners in the country. The researcher learned to collaborate with her peer in Belize through face to face discussions and e-mail and overcame technological difficulties and cultural barriers to produce an international publication. Project LEAD gave the author a sense of self-discovery and self-knowledge, reinforced core values, and developed a frame of reference for leadership. The author also benefited from discussions by local, national, and international leaders on leadership in terms of its key components, contexts, challenges, triumphs, and styles.

  12. International energy technology collaboration and climate change mitigation. Case study 1. Concentrating Solar Power Technologies

    Energy Technology Data Exchange (ETDEWEB)

    Philibert, C. [Energy and Environment Division, International Energy Agency IEA, Paris (France)

    2004-07-01

    Mitigating climate change and achieving stabilisation of greenhouse gas atmospheric concentrations will require deep reductions in global emissions of energy-related carbon dioxide emissions. Developing and disseminating new, low-carbon energy technology will thus be needed. Two previous AIXG papers have focused on possible drivers for such a profound technological change: Technology Innovation, Development and Diffusion, released in June 2003, and International Energy Technology Collaboration and Climate Change Mitigation, released in June 2004. The first of these papers assesses a broad range of technical options for reducing energy-related CO2 emissions. It examines how technologies evolve and the role of research and development efforts, alternative policies, and short-term investment decisions in making long-term options available. It considers various policy tools that may induce technological change, some very specific, and others with broader expected effects. Its overall conclusion is that policies specifically designed to promote technical change, or 'technology push', could play a critical role in making available and affordable new energy technologies. However, such policies would not be sufficient to achieve the Convention's objective in the absence of broader policies. First, because there is a large potential for cuts that could be achieved in the short run with existing technologies; and second, the development of new technologies requires a market pull as much as a technology push. The second paper considers the potential advantages and disadvantages of international energy technology collaboration and transfer for promoting technological change. Advantages of collaboration may consist of lowering R and D costs and stimulating other countries to invest in R and D; disadvantage may include free-riding and the inefficiency of reaching agreement between many actors. This paper sets the context for further discussion on the role of

  13. Towards a measurement of internalization of collaboration scripts in the medical context - results of a pilot study.

    Science.gov (United States)

    Kiesewetter, Jan; Gluza, Martin; Holzer, Matthias; Saravo, Barbara; Hammitzsch, Laura; Fischer, Martin R

    2015-01-01

    Collaboration as a key qualification in medical education and everyday routine in clinical care can substantially contribute to improving patient safety. Internal collaboration scripts are conceptualized as organized - yet adaptive - knowledge that can be used in specific situations in professional everyday life. This study examines the level of internalization of collaboration scripts in medicine. Internalization is understood as fast retrieval of script information. The goals of the current study were the assessment of collaborative information, which is part of collaboration scripts, and the development of a methodology for measuring the level of internalization of collaboration scripts in medicine. For the contrastive comparison of internal collaboration scripts, 20 collaborative novices (medical students in their final year) and 20 collaborative experts (physicians with specialist degrees in internal medicine or anesthesiology) were included in the study. Eight typical medical collaborative situations as shown on a photo or video were presented to the participants for five seconds each. Afterwards, the participants were asked to describe what they saw on the photo or video. Based on the answers, the amount of information belonging to a collaboration script (script-information) was determined and the time each participant needed for answering was measured. In order to measure the level of internalization, script-information per recall time was calculated. As expected, collaborative experts stated significantly more script-information than collaborative novices. As well, collaborative experts showed a significantly higher level of internalization. Based on the findings of this research, we conclude that our instrument can discriminate between collaboration novices and experts. It therefore can be used to analyze measures to foster subject-specific competency in medical education.

  14. Status of international collaborative efforts on selected ITER materials

    International Nuclear Information System (INIS)

    Belyakov, V.A.; Fabritsiev, S.A.; Mazul, I.V.; Rowcliffe, A.F.

    2000-01-01

    The paper presents an overview of the performance of refractory metals, beryllium, and copper alloys, for the international thermonuclear experimental reactor (ITER) high heat flux structures. High temperature brazing, hot isostatic pressing (HIP), friction welding, explosive bonding, and other methods were explored to join copper alloys to 316 stainless steel for first wall and limiter applications. It is concluded that the main material problems for the ITER high heat flux components are: (a) degradation of properties after the manufacturing cycle (especially for Be/Cu and Cu/stainless steel (SS) joints); (b) helium embrittlement of Be, and Cu, and; (c) radiation-induced loss of fracture toughness for Be, W, and Cu alloys

  15. A Burning Plasma Experiment: the role of international collaboration

    Science.gov (United States)

    Prager, Stewart

    2003-04-01

    The world effort to develop fusion energy is at the threshold of a new stage in its research: the investigation of burning plasmas. A burning plasma is self-heated. The 100 million degree temperature of the plasma is maintained by the heat generated by the fusion reactions themselves, as occurs in burning stars. The fusion-generated alpha particles produce new physical phenomena that are strongly coupled together as a nonlinear complex system, posing a major plasma physics challenge. Two attractive options are being considered by the US fusion community as burning plasma facilities: the international ITER experiment and the US-based FIRE experiment. ITER (the International Thermonuclear Experimental Reactor) is a large, power-plant scale facility. It was conceived and designed by a partnership of the European Union, Japan, the Soviet Union, and the United States. At the completion of the first engineering design in 1998, the US discontinued its participation. FIRE (the Fusion Ignition Research Experiment) is a smaller, domestic facility that is at an advanced pre-conceptual design stage. Each facility has different scientific, programmatic and political implications. Selecting the optimal path for burning plasma science is itself a challenge. Recently, the Fusion Energy Sciences Advisory Committee recommended a dual path strategy in which the US seek to rejoin ITER, but be prepared to move forward with FIRE if the ITER negotiations do not reach fruition by July, 2004. Either the ITER or FIRE experiment would reveal the behavior of burning plasmas, generate large amounts of fusion power, and be a huge step in establishing the potential of fusion energy to contribute to the world's energy security.

  16. Gaucher disease: haematological presentations and complications.

    Science.gov (United States)

    Thomas, Alison S; Mehta, Atul; Hughes, Derralynn A

    2014-05-01

    Gaucher disease (GD) is an autosomal recessive lysosomal storage disease, caused by deficiency of the enzyme glucocerebrosidase, required for the degradation of glycosphingolipids. Clinical manifestations include hepatosplenomegaly, thrombocytopenia, bone disease and a bleeding diathesis, frequently resulting in presentation to haematologists. Historically managed by splenectomy, transfusions and orthopaedic surgery, the development of specific therapy in the form of intravenous enzyme replacement therapy in the 1990s has resulted in dramatic improvements in haematological and visceral disease. Recognition of complications, including multiple myeloma and Parkinson disease, has challenged the traditional macrophage-centric view of the pathophysiology of this disorder. The pathways by which enzyme deficiency results in the clinical manifestations of this disorder are poorly understood; altered inflammatory cytokine profiles, bioactive sphingolipid derivatives and alterations in the bone marrow microenvironment have been implicated. Further elucidating these pathways will serve to advance our understanding not only of GD, but of associated disorders. © 2014 John Wiley & Sons Ltd.

  17. Animal models for Gaucher disease research.

    Science.gov (United States)

    Farfel-Becker, Tamar; Vitner, Einat B; Futerman, Anthony H

    2011-11-01

    Gaucher disease (GD), the most common lysosomal storage disorder (LSD), is caused by the defective activity of the lysosomal hydrolase glucocerebrosidase, which is encoded by the GBA gene. Generation of animal models that faithfully recapitulate the three clinical subtypes of GD has proved to be more of a challenge than first anticipated. The first mouse to be produced died within hours after birth owing to skin permeability problems, and mice with point mutations in Gba did not display symptoms correlating with human disease and also died soon after birth. Recently, conditional knockout mice that mimic some features of the human disease have become available. Here, we review the contribution of all currently available animal models to examining pathological pathways underlying GD and to testing the efficacy of new treatment modalities, and propose a number of criteria for the generation of more appropriate animal models of GD.

  18. Animal models for Gaucher disease research

    Directory of Open Access Journals (Sweden)

    Tamar Farfel-Becker

    2011-11-01

    Full Text Available Gaucher disease (GD, the most common lysosomal storage disorder (LSD, is caused by the defective activity of the lysosomal hydrolase glucocerebrosidase, which is encoded by the GBA gene. Generation of animal models that faithfully recapitulate the three clinical subtypes of GD has proved to be more of a challenge than first anticipated. The first mouse to be produced died within hours after birth owing to skin permeability problems, and mice with point mutations in Gba did not display symptoms correlating with human disease and also died soon after birth. Recently, conditional knockout mice that mimic some features of the human disease have become available. Here, we review the contribution of all currently available animal models to examining pathological pathways underlying GD and to testing the efficacy of new treatment modalities, and propose a number of criteria for the generation of more appropriate animal models of GD.

  19. Unexpected macrophage-independent dyserythropoiesis in Gaucher disease.

    Science.gov (United States)

    Reihani, Nelly; Arlet, Jean-Benoit; Dussiot, Michael; de Villemeur, Thierry Billette; Belmatoug, Nadia; Rose, Christian; Colin-Aronovicz, Yves; Hermine, Olivier; Le Van Kim, Caroline; Franco, Melanie

    2016-12-01

    Gaucher disease is a rare inherited disease caused by a deficiency in glucocerebrosidase leading to lipid accumulation in cells of mononuclear-macrophage lineage known as Gaucher cells. Visceral enlargement, bone involvement, mild anemia and thrombocytopenia are the major manifestations of Gaucher disease. We have previously demonstrated that the red blood cells from patients exhibit abnormal properties, which indicates a new role in Gaucher disease pathophysiology. To investigate whether erythroid progenitors are affected, we examined the in vitro erythropoiesis from the peripheral CD34 + cells of patients and controls. CD34- cells were differentiated into macrophages and co-cultivated with erythroblasts. We showed an accelerated differentiation of erythroid progenitors without maturation arrest from patients compared to controls. This abnormal differentiation persisted in the patients when the same experiments were performed without macrophages, which strongly suggested that dyserythropoiesis in Gaucher disease is secondary to an inherent defect in the erythroid progenitors. The accelerated differentiation was associated with reduced cell proliferation. As a result, less mature erythroid cells were generated in vitro in the Gaucher disease cultures compared to the control. We then compared the biological characteristics of untreated patients according to their anemic status. Compared to the non-anemic group, the anemic patients exhibit higher plasma levels of growth differentiation factor-15, a marker of ineffective erythropoiesis, but they had no indicators of hemolysis and similar reticulocyte counts. Taken together, these results demonstrated an unsuspected dyserythropoiesis that was independent of the macrophages and could participate, at least in part, to the basis of anemia in Gaucher disease. Copyright© Ferrata Storti Foundation.

  20. Educational Technology Research Journals: "International Journal of Computer-Supported Collaborative Learning," 2006-2014

    Science.gov (United States)

    Howland, Shiloh M. J.; Martin, M. Troy; Bodily, Robert; Faulconer, Christian; West, Richard E.

    2015-01-01

    The authors analyzed all research articles from the first issue of the "International Journal of Computer-Supported Collaborative Learning" in 2006 until the second issue of 2014. They determined the research methodologies, most frequently used author-supplied keywords as well as two- and three-word phrases, and most frequently published…

  1. A Complexity Approach to Evaluating National Scientific Systems through International Scientific Collaborations

    Science.gov (United States)

    Zelnio, Ryan J.

    2013-01-01

    This dissertation seeks to contribute to a fuller understanding of how international scientific collaboration has affected national scientific systems. It does this by developing three methodological approaches grounded in social complexity theory and applying them to the evaluation of national scientific systems. The first methodology identifies…

  2. Assessment of precision and concordance of quantitative mitochondrial DNA assays: a collaborative international quality assurance study

    NARCIS (Netherlands)

    Hammond, Emma L.; Sayer, David; Nolan, David; Walker, Ulrich A.; Ronde, Anthony de; Montaner, Julio S. G.; Cote, Helene C. F.; Gahan, Michelle E.; Cherry, Catherine L.; Wesselingh, Steven L.; Reiss, Peter; Mallal, Simon

    2003-01-01

    Background: A number of international research groups have developed DNA quantitation assays in order to investigate the role of mitochondrial DNA depletion in anti-retroviral therapy-induced toxicities. Objectives: A collaborative study was undertaken to evaluate intra-assay precision and between

  3. Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration.

    Science.gov (United States)

    Dome, Jeffrey S; Graf, Norbert; Geller, James I; Fernandez, Conrad V; Mullen, Elizabeth A; Spreafico, Filippo; Van den Heuvel-Eibrink, Marry; Pritchard-Jones, Kathy

    2015-09-20

    Clinical trials in Wilms tumor (WT) have resulted in overall survival rates of greater than 90%. This achievement is especially remarkable because improvements in disease-specific survival have occurred concurrently with a reduction of therapy for large patient subgroups. However, the outcomes for certain patient subgroups, including those with unfavorable histologic and molecular features, bilateral disease, and recurrent disease, remain well below the benchmark survival rate of 90%. Therapy for WT has been advanced in part by an increasingly complex risk-stratification system based on patient age; tumor stage, histology, and volume; response to chemotherapy; and loss of heterozygosity at chromosomes 1p and 16q. A consequence of this system has been the apportionment of patients into such small subgroups that only collaboration between large international WT study groups will support clinical trials that are sufficiently powered to answer challenging questions that move the field forward. This article gives an overview of the Children's Oncology Group and International Society of Pediatric Oncology approaches to WT and focuses on four subgroups (stage IV, initially inoperable, bilateral, and relapsed WT) for which international collaboration is pressing. In addition, biologic insights resulting from collaborative laboratory research are discussed. A coordinated expansion of international collaboration in both clinical trials and laboratory science will provide real opportunity to improve the treatment and outcomes for children with renal tumors on a global level. © 2015 by American Society of Clinical Oncology.

  4. Changing the face of cyber warfare with international cyber defense collaboration

    CSIR Research Space (South Africa)

    Grobler, M

    2011-03-01

    Full Text Available . The result is that many countries are not properly prepared, nor adequately protected by legislation, in the event of a cyber attack on a national level. This article will address the international cyber defense collaboration problem by looking at the impact...

  5. Improving Collaborative Planning and Reflection Practices at International Baccalaureate Diploma Schools in Amman

    Science.gov (United States)

    Saa'd AlDin, Kawther

    2014-01-01

    In 2010, the International Baccalaureate (IB) Organization mandated that all its schools, including Diploma (DP) schools, adhere to the collaborative planning and reflection requirements, which emphasized the importance of integrating its theory of knowledge (TOK) core component into all disciplines. Many schools officials and educations in Amman…

  6. Complex Collaborations: India and International Agendas on Girls' and Women's Education, 1947-1990

    Science.gov (United States)

    Vaughan, Rosie Peppin

    2013-01-01

    This article explores the dynamics of global campaigns for education through a study of the movement for girls' and women's education in India since independence in 1947. In particular, it uses the trajectory of ideas within India to theorise about international collaboration on educational goals, with UNESCO and the World Bank being two of the…

  7. Association Between Progranulin and Gaucher Disease.

    Science.gov (United States)

    Jian, Jinlong; Zhao, Shuai; Tian, Qing-Yun; Liu, Helen; Zhao, Yunpeng; Chen, Wen-Chi; Grunig, Gabriele; Torres, Paola A; Wang, Betty C; Zeng, Bai; Pastores, Gregory; Tang, Wei; Sun, Ying; Grabowski, Gregory A; Kong, Max Xiangtian; Wang, Guilin; Chen, Ying; Liang, Fengxia; Overkleeft, Herman S; Saunders-Pullman, Rachel; Chan, Gerald L; Liu, Chuan-Ju

    2016-09-01

    Gaucher disease (GD) is a genetic disease caused by mutations in the GBA1 gene which result in reduced enzymatic activity of β-glucocerebrosidase (GCase). This study identified the progranulin (PGRN) gene (GRN) as another gene associated with GD. Serum levels of PGRN were measured from 115 GD patients and 99 healthy controls, whole GRN gene from 40 GD patients was sequenced, and the genotyping of 4 SNPs identified in GD patients was performed in 161 GD and 142 healthy control samples. Development of GD in PGRN-deficient mice was characterized, and the therapeutic effect of rPGRN on GD analyzed. Serum PGRN levels were significantly lower in GD patients (96.65±53.45ng/ml) than those in healthy controls of the general population (164.99±43.16ng/ml, pGaucher-like cells in lung, spleen, and bone marrow. Moreover, lysosomes in PGRN KO mice exhibit a tubular-like appearance. PGRN is required for the lysosomal appearance of GCase and its deficiency leads to GCase accumulation in the cytoplasm. More importantly, recombinant PGRN is therapeutic in various animal models of GD and human fibroblasts from GD patients. Our data demonstrates an unknown association between PGRN and GD and identifies PGRN as an essential factor for GCase's lysosomal localization. These findings not only provide new insight into the pathogenesis of GD, but may also have implications for diagnosis and alternative targeted therapies for GD. Copyright © 2016 Forschungsgesellschaft für Arbeitsphysiologie und Arbeitschutz e.V. Published by Elsevier B.V. All rights reserved.

  8. Organizational approaches to collaboration in vocational rehabilitation − An international literature review

    Directory of Open Access Journals (Sweden)

    Johanna Andersson

    2011-11-01

    Full Text Available Introduction: Collaboration between welfare organizations is an important strategy for integrating different health and welfare services. This article reports a review of the international literature on vocational rehabilitation, focusing on different organizational models of collaboration as well as different barriers and facilitating factors. Methods: The review was based on an extensive search in scientific journals from 1995 to 2010, which generated more than 13 000 articles. The number of articles was reduced in different steps through a group procedure based on the abstracts. Finally, 205 articles were read in full text and 62 were included for content analysis. Results: Seven basic models of collaboration were identified in the literature. They had different degrees of complexity, intensity and formalization. They could also be combined in different ways.  Several barriers and facilitators of collaboration were also identified. Most of these were related to factors as communication, trust and commitment.Conclusion: There is no optimal model of collaboration to be applied everywhere, but one model could be more appropriate than others in a certain context. More research is needed to compare different models and to see whether they are applicable also in other fields of collaboration inside or outside the welfare system.

  9. Organizational approaches to collaboration in vocational rehabilitation − An international literature review

    Directory of Open Access Journals (Sweden)

    Johanna Andersson

    2011-11-01

    Full Text Available Introduction: Collaboration between welfare organizations is an important strategy for integrating different health and welfare services. This article reports a review of the international literature on vocational rehabilitation, focusing on different organizational models of collaboration as well as different barriers and facilitating factors.  Methods: The review was based on an extensive search in scientific journals from 1995 to 2010, which generated more than 13 000 articles. The number of articles was reduced in different steps through a group procedure based on the abstracts. Finally, 205 articles were read in full text and 62 were included for content analysis. Results: Seven basic models of collaboration were identified in the literature. They had different degrees of complexity, intensity and formalization. They could also be combined in different ways.  Several barriers and facilitators of collaboration were also identified. Most of these were related to factors as communication, trust and commitment. Conclusion: There is no optimal model of collaboration to be applied everywhere, but one model could be more appropriate than others in a certain context. More research is needed to compare different models and to see whether they are applicable also in other fields of collaboration inside or outside the welfare system.

  10. Quantification of glucosylceramide in plasma of Gaucher disease patients

    Directory of Open Access Journals (Sweden)

    Maria Viviane Gomes Muller

    2010-12-01

    Full Text Available Gaucher disease is a sphingolipidosis that leads to an accumulation of glucosylceramide. The objective of this study was to develop a methodology, based on the extraction, purification and quantification of glucosylceramide from blood plasma, for use in clinical research laboratories. Comparison of the glucosylceramide content in plasma from Gaucher disease patients, submitted to enzyme replacement therapy or otherwise, against that from normal individuals was also carried out. The glucosylceramide, separated from other glycosphingolipids by high performance thin layer chromatography (HPTLC was chemically developed (CuSO4 / H3PO4 and the respective band confirmed by immunostaining (human anti-glucosylceramide antibody / peroxidase-conjugated secondary antibody. Chromatogram quantification by densitometry demonstrated that the glucosylceramide content in Gaucher disease patients was seventeen times higher than that in normal individuals, and seven times higher than that in patients on enzyme replacement therapy. The results obtained indicate that the methodology established can be used in complementary diagnosis and for treatment monitoring of Gaucher disease patients.A doença de Gaucher é uma esfingolipidose caracterizada pelo acúmulo de glicosilceramida. O objetivo deste estudo foi desenvolver metodologia baseada na extração, purificação e quantificação da glicosilceramida plasmática a qual possa ser usada em laboratórios de pesquisa clínica. Após o desenvolvimento desta metodologia, foi proposto, também, comparar o conteúdo de glicosilceramida presente no plasma de pacientes com doença de Gaucher, submetidos ou não a tratamento, com aquele de indivíduos normais. A glicosilceramida, separada de outros glicoesfingolipídios por cromatografia de camada delgada de alto desempenho (HPTLC, foi revelada quimicamente (CuSO4/H3PO4 e a respectiva banda foi confirmada por imunorrevelação (anticorpo anti-glicosilceramida humana

  11. Complexity in graduate medical education: a collaborative education agenda for internal medicine and geriatric medicine.

    Science.gov (United States)

    Chang, Anna; Fernandez, Helen; Cayea, Danelle; Chheda, Shobhina; Paniagua, Miguel; Eckstrom, Elizabeth; Day, Hollis

    2014-06-01

    Internal medicine residents today face significant challenges in caring for an increasingly complex patient population within ever-changing education and health care environments. As a result, medical educators, health care system leaders, payers, and patients are demanding change and accountability in graduate medical education (GME). A 2012 Society of General Internal Medicine (SGIM) retreat identified medical education as an area for collaboration between internal medicine and geriatric medicine. The authors first determined a short-term research agenda for resident education by mapping selected internal medicine reporting milestones to geriatrics competencies, and listing available sample learner assessment tools. Next, the authors proposed a strategy for long-term collaboration in three priority areas in clinical medicine that are challenging for residents today: (1) team-based care, (2) transitions and readmissions, and (3) multi-morbidity. The short-term agenda focuses on learner assessment, while the long-term agenda allows for program evaluation and improvement. This model of collaboration in medical education combines the resources and expertise of internal medicine and geriatric medicine educators with the goal of increasing innovation and improving outcomes in GME targeting the needs of our residents and their patients.

  12. The ANTOSTRAT legacy: Science collaboration and international transparency in potential marine mineral resource exploitation of Antarctica

    Science.gov (United States)

    Cooper, Alan; Barker, Peter; Barrett, Peter; Behrendt, John; Brancolini, Giuliano; Childs, Jonathan R.; Escutia, Carlota; Jokat, Wilfried; Kristoffersen, Yngve; Leitchenkov, German; Stagg, Howard; Tanahashi, Manabu; Wardell, Nigel; Webb, Peter

    2009-01-01

    The Antarctic Offshore Stratigraphy project (ANTOSTRAT; 1989–2002) was an extremely successful collaboration in international marine geological science that also lifted the perceived “veil of secrecy” from studies of potential exploitation of Antarctic marine mineral resources. The project laid the groundwork for circum-Antarctic seismic, drilling, and rock coring programs designed to decipher Antarctica’s tectonic, stratigraphic, and climate histories. In 2002, ANTOSTRAT evolved into the equally successful and currently active Antarctic Climate Evolution research program. The need for, and evolution of, ANTOSTRAT was based on two simple tenets within SCAR and the Antarctic Treaty: international science collaboration and open access to data. The ANTOSTRAT project may be a helpful analog for other regions of strong international science and geopolitical interests, such as the Arctic. This is the ANTOSTRAT story.

  13. In the Wake of Japan’s Triple Disaster: Rebuilding Capacity through International Collaboration

    Directory of Open Access Journals (Sweden)

    Eric Anthony Des Marais

    2012-08-01

    Full Text Available Natural disasters occur when the destructive forces of natural events, such as earthquakes, flood, and volcanoes, overwhelm the capacities of communities. In the winter of 2011, Japan, a model for disaster-preparedness, was shaken by one of the largest earthquakes on record, a ten-story tsunami, and a nuclear emergency on par with Chernobyl. In the acute stages of the disaster, the Japanese government officially asked for help from a number of countries. During this time period, international collaboration played a key role in providing help to survivors in the form of medical assistance, food aid, and psychosocial support. As provision of aid evolved into capacity building, national and local Japanese government agencies, in partnership with local grassroots non-profits, assumed most responsibilities, and international organizations transitioned into new roles. This paper will present a study of the collaboration facilitated by a global non-profit humanitarian organization between international faculty and local partners in Japan.

  14. Initiatives in national and international collaborations at Variable Energy Cyclotron Centre

    International Nuclear Information System (INIS)

    Viyogi, Yogendra Pathak; Chakrabarti, Alok

    2008-01-01

    Over the last two decades VECC scientists, under the leadership of their director Bikash Sinha, have pursued experimental physics studies under international collaboration programmes, which would not have been possible with the existing facilities at home. The collaboration extended from RIKEN (Japan) in the east to CERN (Switzerland) in the west. It spanned the energy scales from a few tens of MeV per nucleon to several hundred GeV per nucleon and the physics topics on one extreme being the structure of exotic nuclei and their decay modes and on other extreme being the phase transition of hadronic matter and the formation of quark gluon plasma. The dynamic leadership of Dr. Sinha not only helped to shed the initial inhibitions towards such activities, going beyond the national frontiers, but also gave a new dimension to the experimental physics research in the country. It helped to organize an Indian team of scientists from various national institutes and universities. It paved way for full scale funding of the projects and set the trend that enabled many other Indian groups to join several international collaborations in various fields. Here we reflect on the evolution of these national and international collaboration programmes and the physics, technological and sociological benefits resulting from these activities. (author)

  15. Interruption of enzyme replacement therapy in Gaucher disease.

    Science.gov (United States)

    Goldblatt, J; Fletcher, J M; McGill, J; Szer, J; Wilson, M

    2016-05-25

    In Australia, 58 patients with Gaucher disease were managed by a Gaucher Disease Advisory Committee (GDAC) through a centrally adminis-tered national programme, the Life Savings Drug Program (LSDP). In June 2009, Genzyme Corporation, which manufactures imiglucerase (Cerezyme), the only enzyme replacement therapy (ERT) registered for the treatment of Gaucher disease in Australia at that time, announced that due to a viral contamination problem there would be no further shipments of Cerezyme to Australia prior to the end of 2009. The GDAC allocated available drug supplies in order to maintain treatment to those most in need on a hierarchal clinical severity basis. A cohort of 24 patients with Type 1 Gaucher disease was withdrawn from therapy, 22 of whom had no discernible clinically significant adverse effects when reviewed off therapy for up to 6 months. In this paper, we review the course of 20 of the patients who have been on imiglucerase for periods of at least 24 months after the end of their 'drug holiday'. No patient experienced a bone crisis nor clinical nor magnetic resonance imaging evidence of new avascular necrosis events during this period. Two years after recommencing ERT after a 6-month drug holiday, no patient had developed an overt irreversible complication of their Gaucher disease, with the majority returning to their previous clinical status.

  16. Consensus Conference: A reappraisal of Gaucher disease - diagnosis and disease management algorithms

    Science.gov (United States)

    Mistry, Pramod K.; Cappellini, Maria Domenica; Lukina, Elena; Özsan, Hayri; Pascual, Sara Mach; Rosenbaum, Hanna; Solano, Maria Helena; Spigelman, Zachary; Villarrubia, Jesús; Watman, Nora Patricia; Massenkeil, Gero

    2010-01-01

    Type 1 (non neuronopathic) Gaucher disease was the first lysosomal storage disorder for which an effective enzyme replacement therapy was developed and it has become a prototype for treatments for related orphan diseases. There are currently four treatment options available to patients with Gaucher disease, nevertheless, almost 25% of type 1 Gaucher patients do not gain timely access to therapy because of delays in diagnosis after the onset of symptoms. Diagnosis of Gaucher disease by enzyme testing is unequivocal, but the rarity of the disease and non-specific and heterogeneous nature of Gaucher disease symptoms may impede consideration of this disease in the differential diagnosis. To help promote timely diagnosis and optimal management of the protean presentations of Gaucher disease, a consensus meeting was convened to develop algorithms for diagnosis and disease management for Gaucher disease. PMID:21080341

  17. Validation of the Impact of Health Information Technology (I-HIT) Scale: an international collaborative.

    Science.gov (United States)

    Dykes, Patricia C; Hurley, Ann C; Brown, Suzanne; Carr, Robyn; Cashen, Margaret; Collins, Rita; Cook, Robyn; Currie, Leanne; Docherty, Charles; Ensio, Anneli; Foster, Joanne; Hardiker, Nicholas R; Honey, Michelle L L; Killalea, Rosaleen; Murphy, Judy; Saranto, Kaija; Sensmeier, Joyce; Weaver, Charlotte

    2009-01-01

    In 2005, the Healthcare Information Management Systems Society (HIMSS) Nursing Informatics Community developed a survey to measure the impact of health information technology (HIT), the I-HIT Scale, on the role of nurses and interdisciplinary communication in hospital settings. In 2007, nursing informatics colleagues from Australia, England, Finland, Ireland, New Zealand, Scotland and the United States formed a research collaborative to validate the I-HIT across countries. All teams have completed construct and face validation in their countries. Five out of six teams have initiated reliability testing by practicing nurses. This paper reports the international collaborative's validation of the I-HIT Scale completed to date.

  18. Theoretical and practical considerations for the development of online international collaborative learning for dental hygiene students.

    Science.gov (United States)

    Gussy, M G; Knevel, R J M; Sigurdson, V; Karlberg, G

    2006-08-01

    Globalization and concurrent development in computer and communication technology has increased interest in collaborative online teaching and learning for students in higher education institutions. Many institutions and teachers have introduced computer-supported programmes in areas including dental hygiene. The potential for the use of this technology is exciting; however, its introduction should be careful and considered. We suggest that educators wanting to introduce computer-supported programmes make explicit their pedagogical principles and then select technologies that support and exploit these principles. This paper describes this process as it was applied to the development of an international web-based collaborative learning programme for dental hygiene students.

  19. Crop improvement in the CGIAR as a global success story of open access and international collaboration

    Directory of Open Access Journals (Sweden)

    Derek Byerlee

    2009-12-01

    Full Text Available International agricultural research has historically been an example par excellence of open source approach to biological research. Beginning in the 1950s and especially in the 1960s, a looming global food crisis led to the development of a group of international agricultural research centers with a specific mandate to foster international exchange and crop improvement relevant to many countries. This formalization of a global biological commons in genetic resources was implemented through an elaborate system of international nurseries with a breeding hub, free sharing of germplasm, collaboration in information collection, the development of human resources, and an international collaborative network. This paper traces the history of the international wheat program with particular attention to how this truly open source system operated in practice and the impacts that it had on world poverty and hunger. The paper also highlights the challenges of maintaining and evolving such a system over the long term, both in terms of financing, as well the changing ‘rules of the game’ resulting from international agreements on intellectual property rights and biodiversity. Yet the open source approach is just as relevant today, as witnessed by current crises in food prices and looming crop diseases problem of global significance.

  20. A Study on intensifying efficiency for international collaborative development of Advanced Nuclear Energy Technology

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Moon Hee; Kim, H. R.; Kim, H. J.; Chang, J. H.; Hahn, D. H.; Bae, Y. Y.; Kim, W. W.; Jeong, I.; Lee, D. S.; Lee, J. H. [Korea Atomic Energy Research Institute, Daejeon (Korea, Republic of)

    2008-06-15

    Generation IV International Forum(GIF), where 13 countries including Korea collaborate to develop future nuclear energy systems, put into force 'Generation IV International Forum Project Arrangement' in 2007 for the international research and development of Gen IV Systems, following the entry into force of Framework Agreement in 2005. The International Nuclear Research Initiative(I-NERI) between Korea and United States and the International Project on Innovative Nuclear Energy Systems and Fuel Cycles(INPRO) of IAEA are continued in this year, produced lots of visible outcomes. These international activities have a common goal of the collaborative development of advanced nuclear system technologies but differ in the main focusing areas and aspects, so Korea needs to establish the integrated strategy based on the distinguished and complementary approach for the participation of each international programs, as examples the GIF for the advanced system technology development, INPRO for the set-up of institution and infra-structure, and I-NERI for the access of the core technologies and acquisition of the transparency of nuclear R and D.

  1. A Study on intensifying efficiency for international collaborative development of Advanced Nuclear Energy Technology

    International Nuclear Information System (INIS)

    Chang, Moon Hee; Kim, H. R.; Kim, H. J.; Chang, J. H.; Hahn, D. H.; Bae, Y. Y.; Kim, W. W.; Jeong, I.; Lee, D. S.; Lee, J. H.

    2008-06-01

    Generation IV International Forum(GIF), where 13 countries including Korea collaborate to develop future nuclear energy systems, put into force 'Generation IV International Forum Project Arrangement' in 2007 for the international research and development of Gen IV Systems, following the entry into force of Framework Agreement in 2005. The International Nuclear Research Initiative(I-NERI) between Korea and United States and the International Project on Innovative Nuclear Energy Systems and Fuel Cycles(INPRO) of IAEA are continued in this year, produced lots of visible outcomes. These international activities have a common goal of the collaborative development of advanced nuclear system technologies but differ in the main focusing areas and aspects, so Korea needs to establish the integrated strategy based on the distinguished and complementary approach for the participation of each international programs, as examples the GIF for the advanced system technology development, INPRO for the set-up of institution and infra-structure, and I-NERI for the access of the core technologies and acquisition of the transparency of nuclear R and D

  2. Growth and final height of children with Gaucher disease: A 15-year follow-up at an Israeli Gaucher center.

    Science.gov (United States)

    Mendelsohn, Espen; Meir, Amos; Abrahamov, Aya; Elstein, Deborah; Zimran, Ari; Levy-Khademi, Floris

    2018-02-01

    It is held that enzyme replacement therapy (ERT) accelerates the growth rate in children with Gaucher disease, but its effect on final height has not been established with certainty. This study presents final heights of Gaucher patients followed up for 15years. The study included 41 adults with non-neuronopathic Gaucher disease. The final height of the patients and age at puberty was compared to their mid-parental target height and to their siblings' heights. Mean final height standard deviation score (HSDS) in the patients was -0.22, but none of the patients was abnormally short (HSDS of less than -2.2). Mean age at menarche of the female patients (14.7years) was significantly delayed compared to that of their mothers (P=0.0005), and mean age at first shaving in the boys was 16years. Our study showed that the mean final height of Gaucher patients fell below the mean of the 2000 CDC growth charts, but the patients were not of short stature (height less than the 3rd percentile). ERT treatment did not significantly impact the mean final HSDS. The onset of puberty, as indicated by the age at menarche, was delayed in girls with Gaucher disease. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. International Collaboration on Spent Fuel Disposition in Crystalline Media: FY17 Progress Report

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Yifeng [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Hadgu, Teklu [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Kainina, Elena [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Jove-Colon, Carlos [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States)

    2017-09-01

    Active participation in international R&D is crucial for achieving the Spent Fuel Waste Science & Technology (SFWST) long-term goals of conducting “experiments to fill data needs and confirm advanced modeling approaches” and of having a “robust modeling and experimental basis for evaluation of multiple disposal system options” (by 2020). DOE’s Office of Nuclear Energy (NE) has developed a strategic plan to advance cooperation with international partners. The international collaboration on the evaluation of crystalline disposal media at Sandia National Laboratories (SNL) in FY17 focused on the collaboration through the Development of Coupled Models and their Validation against Experiments (DECOVALEX-2019) project. The DECOVALEX project is an international research and model comparison collaboration, initiated in 1992, for advancing the understanding and modeling of coupled thermo-hydro-mechanical-chemical (THMC) processes in geological systems. SNL has been participating in three tasks of the DECOVALEX project: Task A. Modeling gas injection experiments (ENGINEER), Task C. Modeling groundwater recovery experiment in tunnel (GREET), and Task F. Fluid inclusion and movement in the tight rock (FINITO).

  4. Evolution and results of LCT, international collaboration of superconducting coil development for fusion

    International Nuclear Information System (INIS)

    Shimamoto, Susumu

    1987-01-01

    This international collaboration has been promoted centering around the International Energy Agency since ten years ago. This work is that of advancing joint experiments on the equal footing by several countries gathering with large hardwares. As the result, unlike the international collaboration carried out so far, much experiences have been brought in. Now this work is going to be successfully completed. At this time, the realities of the international collaboration experienced through this work are reported while referring to a part of the technical results. Superconductors were found at the end of 1950s, and the technical development of superconducting coils has been advanced mainly for the equipment of high energy physics in foreign countries, while in Japan, for MHD electricity generation and magnetic levitation train. The TFTR (USA), JET (Euratom) and JT-60 (Japan) aiming at the attainment of critical plasma use normal conduction coils, but the agreement on the LCT project was signed in the autumn of 1977, which aims at the development of the superconducting coils for fusion experimental reactors. The development of coil manufacture in respective countries and the experiments in Japan and Euratom, some episode in the negotiation, the experiment on six coils and the results are reported. (Kako, I.)

  5. The Spectrum of Neurological Manifestations Associated with Gaucher Disease

    Directory of Open Access Journals (Sweden)

    Tamanna Roshan Lal

    2017-03-01

    Full Text Available Gaucher disease, the most common lysosomal storage disorder, is due to a deficiency in the enzyme glucocerebrosidase. This leads to the accumulation of its normal substrate, glucocerebroside, in tissue macrophages, affecting the hematological, visceral, bone and neurologic systems. Gaucher disease is classified into three broad phenotypes based upon the presence or absence of neurological involvement: type 1 (non-neuronopathic, type 2 (acute neuronopathic, and type 3 (subacute neuronopathic. Phenotypically, there is a wide spectrum of visceral and neurological manifestations. Enzyme replacement is effective in managing the visceral disease; however, treating the neurological manifestations has proved to be more challenging. This review discusses the various neurological manifestations encountered in Gaucher disease, and provides a brief overview regarding the treatment and ongoing research challenges.

  6. Gaucher Disease: The Metabolic Defect, Pathophysiology, Phenotypes And Natural History

    Science.gov (United States)

    Baris, Hagit N.; Cohen, Ian J.; Mistry, Pramod K.

    2015-01-01

    Gaucher disease (GD), a prototype lysosomal storage disorder, results from inherited deficiency of lysosomal glucocerebrosidase due to biallelic mutations in GBA. The result is widespread accumulation of macrophages engorged with predominantly lysosomal glucocerebroside. A complex multisystem phenotype arises involving the liver, spleen, bone marrow and occasionally the lungs in type 1 Gaucher disease; in neuronopathic fulminant type 2 and chronic type 3 disease there is in addition progressive neurodegenerative disease. Manifestations of Gaucher disease type 1 (GD1) include hepatosplenomegaly, cytopenia, a complex pattern of bone involvement with avascular osteonecrosis (AVN), osteoporosis, fractures and lytic lesions. Enzyme replacement therapy became the standard of care in 1991, and this has transformed the natural history of GD1. This article reviews the clinical phenotypes of GD, diagnosis, pathophysiology and its natural history. A subsequent chapter discusses the treatment options. PMID:25345088

  7. Expanding NASA and Roscosmos Scientific Collaboration on the International Space Station

    Science.gov (United States)

    Hasbrook, Pete

    2016-01-01

    The International Space Station (ISS) is a world-class laboratory orbiting in space. NASA and Roscosmos have developed a strong relationship through the ISS Program Partnership, working together and with the other ISS Partners for more than twenty years. Since 2013, based on a framework agreement between the Program Managers, NASA and Roscosmos are building a joint program of collaborative research on ISS. This international collaboration is developed and implemented in phases. Initially, members of the ISS Program Science Forum from NASA and TsNIIMash (representing Roscosmos) identified the first set of NASA experiments that could be implemented in the "near term". The experiments represented the research categories of Technology Demonstration, Microbiology, and Education. Through these experiments, the teams from the "program" and "operations" communities learned to work together to identify collaboration opportunities, establish agreements, and jointly plan and execute the experiments. The first joint scientific activity on ISS occurred in January 2014, and implementation of these joint experiments continues through present ISS operations. NASA and TsNIIMash have proceeded to develop "medium term" collaborations, where scientists join together to improve already-proposed experiments. A major success is the joint One-Year Mission on ISS, with astronaut Scott Kelly and cosmonaut Mikhail Kornienko, who returned from ISS in March, 2016. The teams from the NASA Human Research Program and the RAS Institute for Biomedical Problems built on their considerable experience to design joint experiments, learn to work with each other's protocols and processes, and share medical and research data. New collaborations are being developed between American and Russian scientists in complex fluids, robotics, rodent research and space biology, and additional human research. Collaborations are also being developed in Earth Remote Sensing, where scientists will share data from imaging

  8. Facilitating learning through an international virtual collaborative practice: A case study.

    Science.gov (United States)

    Wihlborg, Monne; Friberg, Elizabeth E; Rose, Karen M; Eastham, Linda

    2018-02-01

    Internationalisation of higher education involving information and communication technology such as e-learning opens opportunities for innovative learning approaches across nations and cultures. Describe a case in practice of collaborative and transformative learning in relation to 'internationalisation on home grounds' with the broader learning objective of 'becoming aware and knowledgeable'. A mutually developed project established a virtual international collaborative exchange for faculty and students using a course management software (MOODLE) and open access technology (Adobe CONNECT). Two research universities in Sweden and the United States. Approximately 90 nursing students from each university per semester over several semesters. A collaborative process to develop a joint learning community to construct a virtual module and learning activity involving academics and nursing students in two countries using principles of meaning construction and negotiated learning. Developed possibilities for dealing with the challenges and finding strategies for a future higher education system that opens dialogues worldwide. Virtual international exchanges open innovative communication and learning contexts across nations and cultures. Internationalisation is so much more than students and teachers' mobility. 'Internationalisation on home grounds' (internationalisation for all) should receive more attention to support faculty and student collaboration, learning, and professional development. Copyright © 2017 Elsevier Ltd. All rights reserved.

  9. Skeletal Manifestations in Gaucher Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Altınay Göksel Karatepe

    2005-09-01

    Full Text Available Gauchers disease is the most frequent hereditary lysosomal deposit storage disorder. It is characterized by a deficiency of the enzyme glucocerebrosidase that leads to an accumulation of glucocerebroside in the macrophage lysosomes. It is classified in three types, according to the presence of central nervous system involvement (type 2 and 3 or not (type 1. In the majority of patients there are hepatosplenomegaly, anemia and thrombocytopenia. Skeletal involvement is also important and it is the most disabling manifestation. In this case report, there is presented a case of Gauchers disease with multiple skeletal involvement and the literature is reviewed.

  10. Chronic pain in Gaucher disease: skeletal or neuropathic origin?

    Science.gov (United States)

    Devigili, Grazia; De Filippo, Michele; Ciana, Giovanni; Dardis, Andrea; Lettieri, Christian; Rinaldo, Sara; Macor, Daniela; Moro, Alessandro; Eleopra, Roberto; Bembi, Bruno

    2017-08-31

    Pain is one of the most disabling symptoms of Gaucher disease. It is referred by the majority of Gaucher patients and often persists despite long-term enzyme replacement treatment. It has been mainly considered as nociceptive pain secondary to skeletal involvement but it is described even in the absence of bone disease without a clear explanation. In the last years an increasing number of reports have described the presence of neurological manifestation in Gaucher type 1 patients, including subclinical large fibre neuropathy. In our Gaucher clinic we have observed the recurrence of painful symptoms in a group of type 1 Gaucher patients even after a long-term enzyme replacement therapy. A cross-sectional study was designed to investigate the pathophysiology of pain in a cohort of 25 Gaucher patients (13 females, 12 males). Twenty-two patients received enzyme replacement therapy for a period of time ranging from 10 to >20 years, while three were new diagnosis. Pain was classified as bone or neurologic related on the basis of anamnestic data, clinical and electrophysilogical examinations. Intensity and quality of pain were recorded by Douleur Neuropathique en 4 questionnaire and Neuropathic Pain Symptom Inventory. Neuroalgological evaluation, quantitative sensory testing, nerve conduction studies and evaluation of epidermal nerve fibres density were performed. Comorbidities for peripheral neuropathy were excluded. Thirteen patients complained of pain suggestive of neuropathic origin with proximal patchy distribution, six manifested severe pain paroxysmal, nine pinprick hypoesthesia and 17 thermal hypoesthesia. At quantitative sensory testing, all of them showed high cold thresholds with errata sensation (burning instead of cold), paradoxical heat sensation and mechanic hypoesthesia; three patients showed pressure pain hyperalgesia. Epidermal denervation was present in 19 patients, 12 of them with non-length dependent pattern. These results confirm the role of

  11. Investigating the interplay between fundamentals of national research systems: performance, investments and international collaborations

    OpenAIRE

    Cimini, Giulio; Zaccaria, Andrea; Gabrielli, Andrea

    2015-01-01

    We discuss, at the macro-level of nations, the contribution of research funding and rate of international collaboration to research performance, with important implications for the science of science policy. In particular, we cross-correlate suitable measures of these quantities with a scientometric-based assessment of scientific success, studying both the average performance of nations and their temporal dynamics in the space defined by these variables during the last decade. We find signifi...

  12. BIOPROTA: international collaboration on key technical issues in biosphere aspects of long-term radiological assessment

    International Nuclear Information System (INIS)

    Smith, G.M.; Kerrigan, E.L.; Degnan, P.

    2006-01-01

    BIOPROTA is an international collaborative project which was set up to address key uncertainties in biosphere aspects of assessment of the long-term impact of contaminant releases associated with radioactive waste management. The project began in 2002 and has benefited from the knowledge and experience of organisations from Canada, Finland, France, Japan, Russia, Spain, Sweden, UK and the USA. This paper describes the BIOPROTA objectives and scope, the on-going work programme and methods of work. (author)

  13. Cross-border data exchange - a case study on international collaboration gone wrong

    Science.gov (United States)

    Yanko-Hombach, Valentina

    2016-04-01

    The subject of ethics in science has become a hot topic recently (Gleick, 2011). As publication pressure on researchers increases and use of the internet allows faster turn-around, the quality of the peer review process has suffered. This presentation describes one case of scientific ethics violation in which the editors of a high-ranking scientific journal improperly permitted publication of a paper that was based upon unethical acquisition of data and failed to acknowledge scientific collaboration and exchange of intellectual property. We will present "Case description" and "Ethical issues" with a hope that our experience draws attention to important ethical issues in international collaborative research, and prevents such misconduct in the future. Since international research involves cooperation and coordination among many people in different disciplines and institutions across national borders, ethical standards should promote values that are essential to integrity and collaborative work, including trust, accountability, mutual respect, and fairness. One lesson to be learned is not to engage in collaboration without a written agreement stating clearly who is responsible for what and how the results of collaborative research are to be shared. This is especially important in cases of international collaborations, particularly those involving smaller or developing nations who often do not have the high-tech facilities of developed nations. There is also need to establish clear regulations regarding co-authorship on papers in which intellectual property and significant financial investment was made to allow the research to proceed. As such, a system of ethics to guide the practice of science from data collection to publication and beyond is timely and much needed to protect the integrity of scientific collaboration. It will keep science moving forward by validating research findings and confirming or raising questions about results. References Benos, D. J., Fabres

  14. International collaboration including patients is essential to develop new therapies for patients with myositis.

    Science.gov (United States)

    Lundberg, Ingrid E; Vencovsky, Jiri

    2017-05-01

    To discuss the needs for international collaborations between investigators in different disciplines working with myositis and with patients with myositis. Recent advances in detection of several myositis-specific autoantibodies that are associated with distinct clinical phenotypes, will enable studies in new well defined clinically homogenous subgroups of myositis This is likely to lead to development of new information on molecular pathogenesis that might be different in different myositis subgroups. Subgrouping patients according to autoantibody profile may also be important to assess outcome, to identify prognostic biomarkers and in clinical trials. As these are rare disorders international collaboration is essential to enrol large enough cohorts of the subgroups. To facilitate such collaboration we have developed a web-based international myositis register, www.euromyositis.eu, which includes validated outcome measures and patient reported outcome measures. This register is to support research but also to support decision-making in the clinic. We welcome investigators to join the Euromyositis register. Myositis is a heterogeneous disorder with varying treatment response and outcome. There is a high unmet need for new therapies which can only be achieved by increased knowledge on molecular disease mechanisms. Subgrouping patients according to autoantibody profile may be a new way forward to get a better understanding on disease mechanisms and to develop novel therapies.

  15. Research and Collaboration Overview of Institut Pasteur International Network: A Bibliometric Approach toward Research Funding Decisions

    Directory of Open Access Journals (Sweden)

    Ehsan Mostafavi

    2014-01-01

    Full Text Available Background Institut Pasteur International Network (IPIN, which includes 32 research institutes around the world, is a network of research and expertise to fight against infectious diseases. A scientometric approach was applied to describe research and collaboration activities of IPIN. Methods Publications were identified using a manual search of IPIN member addresses in Science Citation Index Expanded (SCIE between 2006 and 2011. Total publications were then subcategorized by geographic regions. Several scientometric indicators and the H-index were employed to estimate the scientific production of each IPIN member. Subject and geographical overlay maps were also applied to visualize the network activities of the IPIN members. Results A total number of 12667 publications originated from IPIN members. Each author produced an average number of 2.18 papers and each publication received an average of 13.40 citations. European Pasteur Institutes had the largest amount of publications, authored papers, and H-index values. Biochemistry and molecular biology, microbiology, immunology and infectious diseases were the most important research topics, respectively. Geographic mapping of IPIN publications showed wide international collaboration among IPIN members around the world. Conclusion IPIN has strong ties with national and international authorities and organizations to investigate the current and future health issues. It is recommended to use scientometric and collaboration indicators as measures of research performance in IPIN future policies and investment decisions.

  16. The importance of international collaboration for rare diseases research: a European perspective.

    Science.gov (United States)

    Julkowska, D; Austin, C P; Cutillo, C M; Gancberg, D; Hager, C; Halftermeyer, J; Jonker, A H; Lau, L P L; Norstedt, I; Rath, A; Schuster, R; Simelyte, E; van Weely, S

    2017-09-01

    Over the last two decades, important contributions were made at national, European and international levels to foster collaboration into rare diseases research. The European Union (EU) has put much effort into funding rare diseases research, encouraging national funding organizations to collaborate together in the E-Rare program, setting up European Reference Networks for rare diseases and complex conditions, and initiating the International Rare Diseases Research Consortium (IRDiRC) together with the National Institutes of Health in the USA. Co-ordination of the activities of funding agencies, academic researchers, companies, regulatory bodies, and patient advocacy organizations and partnerships with, for example, the European Research Infrastructures maximizes the collective impact of global investments in rare diseases research. This contributes to accelerating progress, for example, in faster diagnosis through enhanced discovery of causative genes, better understanding of natural history of rare diseases through creation of common registries and databases and boosting of innovative therapeutic approaches. Several examples of funded pre-clinical and clinical gene therapy projects show that integration of multinational and multidisciplinary expertize generates new knowledge and can result in multicentre gene therapy trials. International collaboration in rare diseases research is key to improve the life of people living with a rare disease.

  17. Research and collaboration overview of Institut Pasteur International Network: a bibliometric approach toward research funding decisions.

    Science.gov (United States)

    Mostafavi, Ehsan; Bazrafshan, Azam

    2014-01-01

    Institut Pasteur International Network (IPIN), which includes 32 research institutes around the world, is a network of research and expertise to fight against infectious diseases. A scientometric approach was applied to describe research and collaboration activities of IPIN. Publications were identified using a manual search of IPIN member addresses in Science Citation Index Expanded (SCIE) between 2006 and 2011. Total publications were then subcategorized by geographic regions. Several scientometric indicators and the H-index were employed to estimate the scientific production of each IPIN member. Subject and geographical overlay maps were also applied to visualize the network activities of the IPIN members. A total number of 12667 publications originated from IPIN members. Each author produced an average number of 2.18 papers and each publication received an average of 13.40 citations. European Pasteur Institutes had the largest amount of publications, authored papers, and H-index values. Biochemistry and molecular biology, microbiology, immunology and infectious diseases were the most important research topics, respectively. Geographic mapping of IPIN publications showed wide international collaboration among IPIN members around the world. IPIN has strong ties with national and international authorities and organizations to investigate the current and future health issues. It is recommended to use scientometric and collaboration indicators as measures of research performance in IPIN future policies and investment decisions.

  18. NASA's Solar System Exploration Research Virtual Institute: Building Collaboration Through International Partnerships

    Science.gov (United States)

    Gibbs, K. E.; Schmidt, G. K.

    2017-01-01

    The NASA Solar System Exploration Research Virtual Institute (SSERVI) is a virtual institute focused on re-search at the intersection of science and exploration, training the next generation of lunar scientists, and community development. As part of the SSERVI mission, we act as a hub for opportunities that engage the larger scientific and exploration communities in order to form new interdisciplinary, research-focused collaborations. This talk will describe the international partner re-search efforts and how we are engaging the international science and exploration communities through workshops, conferences, online seminars and classes, student exchange programs and internships.

  19. Varied autopsy findings in five treated patients with Gaucher disease and parkinsonism include the absence of Gaucher cells.

    Science.gov (United States)

    Monestime, Gianina; Borger, Daniel K; Kim, Jenny; Lopez, Grisel; Allgaeuer, Michael; Jain, Dhanpat; Vortmeyer, Alexander; Wang, Hao-Wei; Sidransky, Ellen

    2016-05-01

    Enzyme replacement therapy is standard of care for patients with Gaucher disease, as it significantly improves skeletal, visceral, and hematological symptoms. Few pathological studies have documented the extent of pathological findings in treated patients. Autopsy findings in five treated patients, who ultimately developed parkinsonism, ranged from the complete absence of Gaucher pathology to extensive involvement of multiple tissues, without correlation to age, genotype, spleen status, or dose/duration of therapy. Additional autopsies may elucidate modifiers and biomarkers contributing to disease burden and response to therapy. Published by Elsevier Inc.

  20. Good collaborative practice: reforming capacity building governance of international health research partnerships.

    Science.gov (United States)

    Ward, Claire Leonie; Shaw, David; Sprumont, Dominique; Sankoh, Osman; Tanner, Marcel; Elger, Bernice

    2018-01-08

    In line with the policy objectives of the United Nations Sustainable Development Goals, this commentary seeks to examine the extent to which provisions of international health research guidance promote capacity building and equitable partnerships in global health research. Our evaluation finds that governance of collaborative research partnerships, and in particular capacity building, in resource-constrained settings is limited but has improved with the implementation guidance of the International Ethical Guidelines for Health-related Research Involving Humans by The Council for International Organizations of Medical Sciences (CIOMS) (2016). However, more clarity is needed in national legislation, industry and ethics guidelines, and regulatory provisions to address the structural inequities and power imbalances inherent in international health research partnerships. Most notably, ethical partnership governance is not supported by the principal industry ethics guidelines - the International Conference on Harmonization Technical Requirements for Registration of Pharmaceutical for Human Use (ICH) Good Clinical Practice (ICH-GCP). Given the strategic value of ICH-GCP guidelines in defining the role and responsibility of global health research partners, we conclude that such governance should stipulate the minimal requirements for creating an equitable environment of inclusion, mutual learning, transparency and accountability. Procedurally, this can be supported by i) shared research agenda setting with local leadership, ii) capacity assessments, and iii) construction of a memorandum of understanding (MoU). Moreover, the requirement of capacity building needs to be coordinated amongst partners to support good collaborative practice and deliver on the public health goals of the research enterprise; improving local conditions of health and reducing global health inequality. In this respect, and in order to develop consistency between sources of research governance, ICH

  1. The Era of International Space Station Utilization Begins: Research Strategy, International Collaboration, and Realized Potential

    Science.gov (United States)

    Thumm, Tracy; Robinson, Julie A.; Ruttley, Tara; Johnson-Green, Perry; Karabadzhak, George; Nakamura, Tai; Sorokin, Igor V.; Zell, Martin; Jean, Sabbagh

    2010-01-01

    With the assembly of the International Space Station (ISS) nearing completion and the support of a full-time crew of six, a new era of utilization for research is beginning. For more than 15 years, the ISS international partnership has weathered financial, technical and political challenges proving that nations can work together to complete assembly of the largest space vehicle in history. And while the ISS partners can be proud of having completed one of the most ambitious engineering projects ever conceived, the challenge of successfully using the platform remains. During the ISS assembly phase, the potential benefits of space-based research and development were demonstrated; including the advancement of scientific knowledge based on experiments conducted in space, development and testing of new technologies, and derivation of Earth applications from new understanding. The configurability and human-tended capabilities of the ISS provide a unique platform. The international utilization strategy is based on research ranging from physical sciences, biology, medicine, psychology, to Earth observation, human exploration preparation and technology demonstration. The ability to complete follow-on investigations in a period of months allows researchers to make rapid advances based on new knowledge gained from ISS activities. During the utilization phase, the ISS partners are working together to track the objectives, accomplishments, and the applications of the new knowledge gained. This presentation will summarize the consolidated international results of these tracking activities and approaches. Areas of current research on ISS with strong international cooperation will be highlighted including cardiovascular studies, cell and plant biology studies, radiation, physics of matter, and advanced alloys. Scientific knowledge and new technologies derived from research on the ISS will be realized through improving quality of life on Earth and future spaceflight endeavours

  2. 'Non-neuronopathic' Gaucher disease reconsidered. Prevalence of neurological manifestations in a Dutch cohort of type I Gaucher disease patients and a systematic review of the literature

    NARCIS (Netherlands)

    Biegstraaten, M.; van Schaik, I. N.; Aerts, J. M. F. G.; Hollak, C. E. M.

    2008-01-01

    Gaucher disease is a lysosomal storage disorder, which is classically divided into three types. Type I Gaucher disease is differentiated from types II and III disease by the absence of nervous system involvement. However, an increasing number of reports has emerged on neurological manifestations in

  3. International workshop: islet transplantation without borders enabling islet transplantation in Greece with international collaboration and innovative technology.

    Science.gov (United States)

    Papas, Klearchos K; Karatzas, Theodore; Berney, Thierry; Minor, Thomas; Pappas, Paris; Pattou, François; Shaw, James; Toso, Christian; Schuurman, Henk-Jan

    2013-01-01

    Recently, initiatives have been undertaken to establish an islet transplantation program in Athens, Greece. A major hurdle is the high cost associated with the establishment and maintenance of a clinical-grade islet manufacturing center. A collaboration was established with the University Hospitals of Geneva, Switzerland, to enable remote islet cell manufacturing with an established and validated fully operational team. However, remote islet manufacturing requires shipment of the pancreas from the procurement to the islet manufacturing site (in this case from anywhere in Greece to Geneva) and then shipment of the islets from the manufacturing site to the transplant site (from Geneva to Athens). To address challenges related to cold ischemia time of the pancreas and shipment time of islets, a collaboration was initiated with the University of Arizona, Tucson, USA. An international workshop was held in Athens, December 2011, to mark the start of this collaborative project. Experts in the field presented in three main sessions: (i) islet transplantation: state-of-the-art and the "network approach"; (ii) technical aspects of clinical islet transplantation and outcomes; and (iii) islet manufacturing - from the donated pancreas to the islet product. This manuscript presents a summary of the workshop. © 2013 John Wiley & Sons A/S.

  4. The saccadic and neurological deficits in type 3 Gaucher disease.

    Directory of Open Access Journals (Sweden)

    William Benko

    Full Text Available Our objective was to characterize the saccadic eye movements in patients with type 3 Gaucher disease (chronic neuronopathic in relationship to neurological and neurophysiological abnormalities. For approximately 4 years, we prospectively followed a cohort of 15 patients with Gaucher type 3, ages 8-28 years, by measuring saccadic eye movements using the scleral search coil method. We found that patients with type 3 Gaucher disease had a significantly higher regression slope of duration vs amplitude and peak duration vs amplitude compared to healthy controls for both horizontal and vertical saccades. Saccadic latency was significantly increased for horizontal saccades only. Downward saccades were more affected than upward saccades. Saccade abnormalities increased over time in some patients reflecting the slowly progressive nature of the disease. Phase plane plots showed individually characteristic patterns of abnormal saccade trajectories. Oculo-manual dexterity scores on the Purdue Pegboard test were low in virtually all patients, even in those with normal cognitive function. Vertical saccade peak duration vs amplitude slope significantly correlated with IQ and with the performance on the Purdue Pegboard but not with the brainstem and somatosensory evoked potentials. We conclude that, in patients with Gaucher disease type 3, saccadic eye movements and oculo-manual dexterity are representative neurological functions for longitudinal studies and can probably be used as endpoints for therapeutic clinical trials.ClinicalTrials.gov NCT00001289.

  5. Gaucher disease in a family from Maranhão

    Directory of Open Access Journals (Sweden)

    Samira Shizuko Parreão Oi

    2014-10-01

    Full Text Available Background: Gaucher disease is an inborn, autosomal recessive error of the metabolism which belongs to the group of lysosomal storage disorders. Objective: This work reports on the treatment of Gaucher disease in several members of the same family from the countryside of Maranhão. Methods: This was an observational, retrospective and prospective, descriptive case study about the efficacy of enzyme replacement therapy. Results: The results showed that women were more affected (80% of patients by the disease, age at diagnosis ranged from 24 to 33 years, the predominant ethnicity was mulatto (80% and all cases were classified as type 1. The diagnosis of these patients was performed by measuring the levels of glucocerebrosidase and chitotriosidase enzymes and confirmed by genotyping. All patients suffering from Gaucher disease had low glucocerebrosidase levels. Before replacement therapy, hepatosplenomegaly was the most common clinical manifestation (100% and osteopenia was seen in 80% of the cases. Regarding hematological manifestations, anemia and leukopenia were found in 40% of patients at diagnosis; however the hemoglobin and leukocyte levels were normalized after four years of therapy. Thrombocytopenia, observed in 20% of cases, was normalized after the second year of treatment. Conclusion: In these cases, despite gaps in the treatment as the family resides in the rural region of the state, the patients with Gaucher disease showed satisfactory therapeutic response over time.

  6. De ziekte van Gaucher op de kinderleeftijd: presentatie en behandeling

    NARCIS (Netherlands)

    Maaswinkel-Mooij, P. D.; Kerstjens-Frederikse, W. S.; de Koning, J.; Kok, A. J.; Poorthuis, B. J.

    1992-01-01

    M. Gaucher is a lysosomal storage disorder. Patients present with hepatosplenomegaly or with complaints of the bones. Clinically 3 subtypes can be distinguished; the 'adult' type I is most frequent found. On the basis of 10 case histories the presentation in childhood is reported. Only recently

  7. GBA mutations in Gaucher type I Venezuelan patients: ethnic origins ...

    Indian Academy of Sciences (India)

    Gaucher disease (GD), the most frequent lysosomal storage disease, is caused by heterogeneous mutations in the locus coding for glucocerebrosidase (GBA). It is an autosomal recessive disorder with different phenotypes of which the most frequent is the nonneuronopathic or type 1, prevalent worldwide. To date, more ...

  8. The clinical management of Type 2 Gaucher disease.

    Science.gov (United States)

    Weiss, Karin; Gonzalez, Ashley; Lopez, Grisel; Pedoeim, Leah; Groden, Catherine; Sidransky, Ellen

    2015-02-01

    Gaucher disease, the inherited deficiency of the enzyme glucocerebrosidase, is the most common of the lysosomal storage disorders. Type 2 Gaucher disease, the most severe and progressive form, manifests either prenatally or in the first months of life, followed by death within the first years of life. The rarity of the many lysosomal storage disorders makes their diagnosis a challenge, especially in the newborn period when the focus is often on more prevalent illnesses. Thus, a heightened awareness of the presentation of these rare diseases is necessary to ensure their timely consideration. This review, designed to serve as a guide to physicians treating newborns and infants with Gaucher disease, discusses the presenting manifestations of Type 2 Gaucher disease, the diagnostic work-up, associated genotypes and suggestions for management. We also address the ethical concerns that may arise with this progressive and lethal disorder, since currently available treatments may prolong life, but do not impact the neurological manifestations of the disease. Published by Elsevier Inc.

  9. Review: Cytokines in Gaucher disease: Role in the pathogenesis of ...

    African Journals Online (AJOL)

    Gaucher disease (GD) is the most frequently encountered lysosomal storage disease caused by inborn defects of themembrane-bound lysosomal enzyme, acid b-glucosidase or glucocerebrosidase. This defective activity causes an accumulation of glucocerebroside (glucosylceramide) in the lysosomes of cells derived from ...

  10. Gaucher's disease: Report of 11 cases with review of literature ...

    African Journals Online (AJOL)

    Gaucher's disease (GD) is a lysosomal storage disorder due to glucocerebrosidase deficiency; it's one of the rare genetic diseases for which therapy is now available. The purpose of this work is to study the epidemiological features of the disease and to highlight the diagnostic difficulties. We performed an 11-year ...

  11. Clinical and genetic assessment of pediatric patients with Gaucher's ...

    African Journals Online (AJOL)

    Background: Gaucher's disease (GD) is an autosomal recessive genetic disorder that results from pathogenic mutations of GBA gene encoding the enzyme glucocerebrosidase (acid b-glucosidase). Of the approximately 300 mutations associated with GD, 4 accounts for the majority of mutations seen in GD patients: N370S, ...

  12. Treatment options for patients with Gaucher disease | Shawky ...

    African Journals Online (AJOL)

    Gaucher disease is the most common lysosomal storage disorder due to deficiency of ß-glucocerebrosidase. Since the introduction of Ceredase in 1991, enzyme replacement therapy has been the mainstay of treatment with its major disadvantage of long life dependency on biweekly IV therapy. It was more than a decade ...

  13. B cell lymphoma and myeloma in murine Gaucher's disease

    NARCIS (Netherlands)

    Pavlova, E. V.; Wang, S. Z.; Archer, J.; Dekker, N. [=Nick; Aerts, J. M. F. G.; Karlsson, S.; Cox, T. M.

    2013-01-01

    Multiple myeloma and B cell lymphoma are leading causes of death in Gaucher's disease but the nature of the stimulus driving the often noted clonal expansion of immunoglobulin-secreting B cells and cognate lymphoid malignancy is unknown. We investigated the long-term development of B cell

  14. How Do I Talk to My Family about Gaucher?

    Science.gov (United States)

    ... to my parents and the rest of our family. My father and mother did not want to accept the ... holding fundraisers and speaking to Jewish organizations. My family also got tested: My father had asymptomatic Gaucher, my mother is a carrier, ...

  15. Gaucher disease: from fundamental research to effective therapeutic interventions

    NARCIS (Netherlands)

    de Fost, M.; Aerts, J. M. F. G.; Hollak, C. E. M.

    2003-01-01

    Gaucher disease type I is the most common lysosomal storage disorder, with a prevalence Of 1:50,000 in most countries. It is caused by an autosomally recessive inherited deficiency of the lysosomal enzyme glucocerebrosidase, leading to the accumulation of glucocerebroside in the macrophages. The

  16. Clinical evaluation of chemokine and enzymatic biomarkers of Gaucher disease

    NARCIS (Netherlands)

    Deegan, Patrick B.; Moran, Mary Teresa; McFarlane, Ian; Schofield, J. Paul; Boot, Rolf G.; Aerts, Johannes M. F. G.; Cox, Timothy M.

    2005-01-01

    Purpose: Gaucher disease is an exemplary orphan disorder. Enzyme replacement therapy with imiglucerase is effective, but very expensive. To improve the assessment of severity of disease and responses to this costly treatment, we have evaluated several enzymatic biomarkers and a newly-described

  17. Simultaneous and Comparable Numerical Indicators of International, National and Local Collaboration Practices in English-Medium Astrophysics Research Papers

    Science.gov (United States)

    Méndez, David I.; Alcaraz, M. Ángeles

    2016-01-01

    Introduction: We report an investigation on collaboration practices in research papers published in the most prestigious English-medium astrophysics journals. Method: We propose an evaluation method based on three numerical indicators to study and compare, in absolute terms, three different types of collaboration (international, national and…

  18. Preparation of IHY-2007 in Indonesia: Local Observational Facilities, International Collaborations, and the Use of International Data

    Science.gov (United States)

    Djamaluddin, T.

    2006-11-01

    t_djamal@hotmail.com Since 1980, the National Institute of Aeronautics and Space (LAPAN) has been carrying out integrated observations of solar activities, geomagnetic disturbance, and ionospheric parameters, as well as other solar-terrestrial relationship research. International collaboration, especially with Japan in the field of solar physics, geomagnetism and equatorial atmosphere and with Australia in the field of ionosphere and upper atmosphere, help us in increasing national capacity building. The international data available on the Internet also helps us in comparing our local data with the global one or in fulfilling our needs of data due to lack of facilities, ground based or space based data. Some results will be reviewed. Preparation for IHY-2007 will also be discussed.

  19. Science diplomacy: Investigating the perspective of scholars on politics-science collaboration in international affairs.

    Science.gov (United States)

    Fähnrich, Birte

    2017-08-01

    Science diplomacy is a widely practiced area of international affairs, but academic research is rather sparse. The role of academia within this field of politics-science interaction has hardly been considered. This article analyzes this scholarly perspective: Based on a literature review, a case study of a German science diplomacy program is used to explore objectives, benefits, and constraints of science diplomacy for participating scholars. While political approaches suggest an ideal world where both sides profit from the collaboration, the findings of the case study point to another conclusion which shows that the interaction of scholars and officials in science diplomacy is far more complex. Thus, the contribution is regarded as both a useful starting point for further research and for a critical reflection of academics and politicians in science diplomacy practice to gauge what can be expected from the collaboration and what cannot.

  20. Project Adopsys as an example of international collaboration in the field of photonics

    Science.gov (United States)

    Zoric, Nenad; Livshits, Irina; Urbach, Paul

    2015-01-01

    Tendencies of international cooperation in engineering education became very visible during recent years. We demonstrate this statement on one currently running EU project ADOPSYS in the field of optical design, which is an important part of engineering education in photonics. This example shows the importance of the input from different countries and organizations - both from industry and academia. Seven universities and eight optical companies are involved in the project ADOPSYS. Sharing experience of Academia education activity we provide new international type of education "free-of borders". We are going to discuss the key enable technology - PHOTONICS, which is widely used in modern society. Engineering science became very international. For communicating between people from different countries the English language is now used almost exclusively. For a fruitful collaboration between people from different nations, in multi-national projects, tolerance and respect are required between people of different political, cultural, educational backgrounds.

  1. International collaborative study for the calibration of proposed International Standards for thromboplastin, rabbit, plain, and for thromboplastin, recombinant, human, plain.

    Science.gov (United States)

    van den Besselaar, A M H P; Chantarangkul, V; Angeloni, F; Binder, N B; Byrne, M; Dauer, R; Gudmundsdottir, B R; Jespersen, J; Kitchen, S; Legnani, C; Lindahl, T L; Manning, R A; Martinuzzo, M; Panes, O; Pengo, V; Riddell, A; Subramanian, S; Szederjesi, A; Tantanate, C; Herbel, P; Tripodi, A

    2018-01-01

    Essentials Two candidate International Standards for thromboplastin (coded RBT/16 and rTF/16) are proposed. International Sensitivity Index (ISI) of proposed standards was assessed in a 20-centre study. The mean ISI for RBT/16 was 1.21 with a between-centre coefficient of variation of 4.6%. The mean ISI for rTF/16 was 1.11 with a between-centre coefficient of variation of 5.7%. Background The availability of International Standards for thromboplastin is essential for the calibration of routine reagents and hence the calculation of the International Normalized Ratio (INR). Stocks of the current Fourth International Standards are running low. Candidate replacement materials have been prepared. This article describes the calibration of the proposed Fifth International Standards for thromboplastin, rabbit, plain (coded RBT/16) and for thromboplastin, recombinant, human, plain (coded rTF/16). Methods An international collaborative study was carried out for the assignment of International Sensitivity Indexes (ISIs) to the candidate materials, according to the World Health Organization (WHO) guidelines for thromboplastins and plasma used to control oral anticoagulant therapy with vitamin K antagonists. Results Results were obtained from 20 laboratories. In several cases, deviations from the ISI calibration model were observed, but the average INR deviation attributabled to the model was not greater than 10%. Only valid ISI assessments were used to calculate the mean ISI for each candidate. The mean ISI for RBT/16 was 1.21 (between-laboratory coefficient of variation [CV]: 4.6%), and the mean ISI for rTF/16 was 1.11 (between-laboratory CV: 5.7%). Conclusions The between-laboratory variation of the ISI for candidate material RBT/16 was similar to that of the Fourth International Standard (RBT/05), and the between-laboratory variation of the ISI for candidate material rTF/16 was slightly higher than that of the Fourth International Standard (rTF/09). The candidate materials

  2. Propionibacterium endocarditis: a case series from the International Collaboration on Endocarditis Merged Database and Prospective Cohort Study

    NARCIS (Netherlands)

    Lalani, Tahaniyat; Person, Anna K.; Hedayati, Susan S.; Moore, Laura; Murdoch, David R.; Hoen, Bruno; Peterson, Gail; Shahbaz, Hasan; Raoult, Didier; Miro, Jose M.; Olaison, Lars; Snygg-Martino, Ulrika; Suter, Fredy; Spelman, Dennis; Eykyn, Susannah; Strahilevitz, Jacob; van der Meer, Jan T.; Verhagen, Dominique; Baloch, Khaula; Abrutyn, Elias; Cabell, Christopher H.

    2007-01-01

    Propionibacterium species are occasionally associated with serious systemic infections such as infective endocarditis. In this study, we examined the clinical features, complications and outcome of 15 patients with Propionibacterium endocarditis using the International Collaboration on Endocarditis

  3. The International Reference Ionosphere 2012 – a model of international collaboration

    Czech Academy of Sciences Publication Activity Database

    Bilitza, D.; Altadill, D.; Zhang, Y.; Mertens, Ch.; Truhlík, Vladimír; Richards, P.; McKinnell, L.- A.; Reinisch, B.

    2014-01-01

    Roč. 4, 20 February (2014), A07/1-A07/12 ISSN 2115-7251 R&D Projects: GA MŠk(CZ) LH11123 Institutional support: RVO:68378289 Keywords : International Reference Ionosphere * empirical models * plasma parameters * real - time IRI Subject RIV: DG - Athmosphere Sciences, Meteorology Impact factor: 2.558, year: 2014 http://www.swsc-journal.org/articles/swsc/abs/2014/01/swsc130043/swsc130043.html

  4. Gaucher disease diagnosed after bone marrow trephine biopsy — a report of two cases

    Directory of Open Access Journals (Sweden)

    Anna Dmoszyńska

    2011-07-01

    Full Text Available The hematologist is at the forefront of specialists to whom patients with Gaucher disease present because of cytopenia and hepatosplenomegaly. Usually, patients with such symptoms have undergone trephine biopsy. We present the cases of two patients in whom Gaucher disease was suspected because of the discovery of Gaucher cells in trephine biopsy, and subsequently confirmed via enzymatic and molecular investigations. (Folia Histochemica et Cytobiologica 2011; Vol. 49, No. 2, pp. 352–356

  5. A study on intensifying efficiency for international collaborative development of advanced nuclear energy technology

    Energy Technology Data Exchange (ETDEWEB)

    Lee, J. H.; Hahn, D. H.; Song, K. C.; Chang, J. H.; Kim, H. J.; Kim, H. J.; Lim, C. Y.; Lee, D. S.; Lee, Y. J. [KAERI, Daejeon (Korea, Republic of)

    2011-03-15

    The objective of the study was to participate the GIF for the efficient propulsion of future nuclear system development. For achieving the objective of this study, the followings were carried out. {Omicron} Analyze the international/domestic trends in the future nuclear energy system {Omicron} Analyze the domestic long-term R and D program for the future nuclear system and assist its implementation - Review the agenda of the executive committee, the technical committee, and sub-technical committee - Assist the committee meetings and workshops related to the future nuclear energy system {Omicron} Develop the participation strategy for the collaborative development of Gen-IV technology and conducting the international cooperation activities - Support the delegation by reviewing the agenda of GIF meetings in the technical and legal perspective - Research the system R and D arrangement and report its progress - Participate in the SFR SIA PA negotiation meeting and report its progress {Omicron} Support the activities related to I-NERI between Korea and U.S. - Support a delegation by reviewing the agenda in the technical/legal point of view - Participate in the BINERIC meetings and Support the related activities The result of this study may be used for 1) contribution to establishing the effective foundation and broadening the cooperation activities between the advanced countries and Korea and 2) contribution effective management of Gen IV international collaboration by technical/legal supporting

  6. A Study on intensifying efficiency for international collaborative development of Advanced Nuclear Energy Technology

    International Nuclear Information System (INIS)

    Chang, Moon Hee; Kim, H. R.; Kim, H. J.

    2009-08-01

    The objective of the study was to participate the GIF for the efficient propulsion of future nuclear system development. For achieving the objective of this study, the followings were carried out. · Investigation and analysis of the international and domestic trends related to future nuclear system · To maximize the national interests by the strategic participation of GIF meeting - To participate of GIF meeting and to support of relative work - To investigate the System R and D Arrangement and to inform its progress situation · To maximize the propulsion results of Korea/U.S nuclear energy joint research(I-NERI) - To support a delegation by the review of agenda in aspect of the technical/legal point - To participate of BINERIC meeting and to support of relative work · Streamline the nuclear energy R and D due to the effective connection between domestic R and D and international collaboration The result of this study may be used for 1) contribution to establishing the effective foundation and broadening the cooperation activities between the advanced countries and Korea and 2) contribution effective management of Gen IV international collaboration by technical/legal supporting

  7. A study on intensifying efficiency for international collaborative development of advanced nuclear energy technology

    International Nuclear Information System (INIS)

    Lee, J. H.; Hahn, D. H.; Song, K. C.; Chang, J. H.; Kim, H. J.; Kim, H. J.; Lim, C. Y.; Lee, D. S.; Lee, Y. J.

    2011-03-01

    The objective of the study was to participate the GIF for the efficient propulsion of future nuclear system development. For achieving the objective of this study, the followings were carried out. Ο Analyze the international/domestic trends in the future nuclear energy system Ο Analyze the domestic long-term R and D program for the future nuclear system and assist its implementation - Review the agenda of the executive committee, the technical committee, and sub-technical committee - Assist the committee meetings and workshops related to the future nuclear energy system Ο Develop the participation strategy for the collaborative development of Gen-IV technology and conducting the international cooperation activities - Support the delegation by reviewing the agenda of GIF meetings in the technical and legal perspective - Research the system R and D arrangement and report its progress - Participate in the SFR SIA PA negotiation meeting and report its progress Ο Support the activities related to I-NERI between Korea and U.S. - Support a delegation by reviewing the agenda in the technical/legal point of view - Participate in the BINERIC meetings and Support the related activities The result of this study may be used for 1) contribution to establishing the effective foundation and broadening the cooperation activities between the advanced countries and Korea and 2) contribution effective management of Gen IV international collaboration by technical/legal supporting

  8. Elevated plasma glucosylsphingosine in Gaucher disease: relation to phenotype, storage cell markers, and therapeutic response

    Science.gov (United States)

    Dekker, Nick; van Dussen, Laura; Hollak, Carla E. M.; Overkleeft, Herman; Scheij, Saskia; Ghauharali, Karen; van Breemen, Mariëlle J.; Ferraz, Maria J.; Groener, Johanna E. M.; Maas, Mario; Wijburg, Frits A.; Speijer, Dave; Tylki-Szymanska, Anna; Mistry, Pramod K.; Boot, Rolf G.

    2011-01-01

    Gaucher disease, caused by a deficiency of the lysosomal enzyme glucocerebrosidase, leads to prominent glucosylceramide accumulation in lysosomes of tissue macrophages (Gaucher cells). Here we show glucosylsphingosine, the deacylated form of glucosylceramide, to be markedly increased in plasma of symptomatic nonneuronopathic (type 1) Gaucher patients (n = 64, median = 230.7nM, range 15.6-1035.2nM; normal (n = 28): median 1.3nM, range 0.8-2.7nM). The method developed for mass spectrometric quantification of plasma glucosylsphingosine is sensitive and robust. Plasma glucosylsphingosine levels correlate with established plasma markers of Gaucher cells, chitotriosidase (ρ = 0.66) and CCL18 (ρ = 0.40). Treatment of Gaucher disease patients by supplementing macrophages with mannose-receptor targeted recombinant glucocerebrosidase results in glucosylsphingosine reduction, similar to protein markers of Gaucher cells. Since macrophages prominently accumulate the lysoglycosphingolipid on glucocerebrosidase inactivation, Gaucher cells seem a major source of the elevated plasma glucosylsphingosine. Our findings show that plasma glucosylsphingosine can qualify as a biomarker for type 1 Gaucher disease, but that further investigations are warranted regarding its relationship with clinical manifestations of Gaucher disease. PMID:21868580

  9. 6 June 2012 - Chinese Nanjing University President J.Chen in the ATLAS visitor centre with Member of the ATLAS Collaboration I. Wingerter and International Relations Office Adviser E. Tsesmelis. M. Qi, Nanjing University and ATLAS Collaboration, accompanies the delegation.

    CERN Multimedia

    Maximilien Brice

    2012-01-01

    6 June 2012 - Chinese Nanjing University President J.Chen in the ATLAS visitor centre with Member of the ATLAS Collaboration I. Wingerter and International Relations Office Adviser E. Tsesmelis. M. Qi, Nanjing University and ATLAS Collaboration, accompanies the delegation.

  10. Fourth Collaborative Materials Exercise of the Nuclear Forensics International Technical Working Group

    International Nuclear Information System (INIS)

    Schwantes, J.M.; Reilly, D.; Marsden, O.

    2018-01-01

    The Nuclear Forensics International Technical Working Group is a community of nuclear forensic practitioners who respond to incidents involving nuclear and other radioactive material out of regulatory control. The Group is dedicated to advancing nuclear forensic science in part through periodic participation in materials exercises. The Group completed its fourth Collaborative Materials Exercise in 2015 in which laboratories from 15 countries and one multinational organization analyzed three samples of special nuclear material in support of a mock nuclear forensic investigation. This special section of the Journal for Radioanalytical and Nuclear Chemistry is devoted to summarizing highlights from this exercise. (author)

  11. Islet Transplantation without Borders Enabling islet transplantation in Greece with international collaboration and innovative technology

    Science.gov (United States)

    Papas, Klearchos K; Karatzas, Theodore; Berney, Thierry; Minor, Thomas; Pappas, Paris; Pattou, François; Shaw, James; Toso, Christian; Schuurman, Henk-Jan

    2012-01-01

    Recently, initiatives have been undertaken to establish an islet transplantation program in Athens, Greece. A major hurtle is the high cost associated with the establishment and maintenance of a clinical-grade islet manufacturing center. A collaboration was established with the University Hospitals of Geneva, Switzerland, to enable remote islet cell manufacturing with an established and validated fully operational team. However, remote islet manufacturing requires shipment of the pancreas from the procurement to the islet manufacturing site (in this case from anywhere in Greece to Geneva) and then shipment of the islets from the manufacturing site to the transplant site (from Geneva to Athens). To address challenges related to cold ischemia time of the pancreas and shipment time of islets, a collaboration was initiated with the University of Arizona, Tucson, USA. An international workshop was held in Athens, December 2011, to mark the start of this collaborative project. Experts in the field presented in three main sessions: [1] Islet transplantation: state-of-the-art, and the “network approach”; [2] Technical aspects of clinical islet transplantation and outcomes; and [3] Islet manufacturing – from the donated pancreas to the islet product. This manuscript presents a summary of the workshop. PMID:23330863

  12. Feasibility Study of Implementing a Mobile Collaborative Information Platform for International Safeguards Inspections

    Energy Technology Data Exchange (ETDEWEB)

    Gastelum, Zoe N. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Gitau, Ernest T. N. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Doehle, Joel R. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Toomey, Christopher M. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)

    2014-09-01

    In response to the growing pervasiveness of mobile technologies such as tablets and smartphones, the International Atomic Energy Agency and the U.S. Department of Energy National Laboratories have been exploring the potential use of these platforms for international safeguards activities. Specifically of interest are information systems (software, and accompanying servers and architecture) deployed on mobile devices to increase the situational awareness and productivity of an IAEA safeguards inspector in the field, while simultaneously reducing paperwork and pack weight of safeguards equipment. Exploratory development in this area has been met with skepticism regarding the ability to overcome technology deployment challenges for IAEA safeguards equipment. This report documents research conducted to identify potential challenges for the deployment of a mobile collaborative information system to the IAEA, and proposes strategies to mitigate those challenges.

  13. Social Media and Population Health Virtual Exchange for Senior Nursing Students: An International Collaboration.

    Science.gov (United States)

    Procter, Paula M; Brixey, Juliana J; Honey, Michelle L L; Todhunter, Fern

    2016-01-01

    The authors have all engaged in using social media with students as a means for collaboration across national and international boundaries for various educational purposes. Following the explosion of big data in health the authors are now moving this concept forward within undergraduate and postgraduate nursing curricula for the development of population health virtual exchanges. Nursing has a global presence and yet it appears as though students have little knowledge of the health and social care needs and provision outside their local environment. This development will allow for explorative exchange amongst students in three countries, enhancing their understanding of their own and the selected international population health needs and solutions through asking and responding to questions amongst the learning community involved. The connection of the students will be recorded for their use in reflection; of particular interest will be the use of information included by the students to answer questions about their locality.

  14. International collaboration between nuclear research centres and the role of research reactors

    International Nuclear Information System (INIS)

    Dodd, B.

    2001-01-01

    A research reactor is a core facility in many nuclear research centres (NRCs) of Member States and it is logical that it should be the focus of any international collaboration between such centres. There are several large and sophisticated research reactors in operation in both developed and developing Member States, such as Belgium, China, Egypt, France, Hungary, Indonesia, India, Japan, ROK, Netherlands, South Africa and the USA. There are also several new, large reactors under construction or being planned such as those in Australia, Canada, China, France, Germany, and Thailand. It is felt that the utilization of these reactors can be enhanced by international co-operation to achieve common goals in research and applications. (author)

  15. The United Kingdom Hydrogen Association Forms with International Collaboration in Mind

    International Nuclear Information System (INIS)

    Karen Hall; John Carolin; Ian Williamson

    2006-01-01

    In April 2006, the United Kingdom Hydrogen Association was launched. This paper will describe the context under which the need was established, and address the challenges and opportunities faced in creating the association. A UK Hydrogen Association can encourage information sharing among regional hydrogen efforts, and provide a mechanism for a larger, single voice on the national level. In addition, a UK Hydrogen Association can serve as a focal point for UK participation in EU activities such as the European Hydrogen and Fuel Cell Technology Platform (HFP), and other international activities such as IPHE and IEA. The results of the stake holder briefing and progress of a UK Hydrogen Association will be presented, with a focus on international collaboration. (authors)

  16. The United Kingdom Hydrogen Association Forms with International Collaboration in Mind

    International Nuclear Information System (INIS)

    Karen Hall; John Carolin; Ian Williamson

    2006-01-01

    In April 2006, the United Kingdom Hydrogen Association was launched. This paper will describe the context under which the need was established, and address the challenges and opportunities faced in creating the association. A UK Hydrogen Association can encourage information sharing among regional hydrogen efforts, and provide a mechanism for a larger, single voice on the national level. In addition, a UK Hydrogen Association can serve as a focal point for UK participation in EU activities such as the European Hydrogen and Fuel Cell Technology Platform (HFP), and other international activities such as IPHE and IEA. The results of the stakeholder briefing and progress of a UK Hydrogen Association will be presented, with a focus on international collaboration. (authors)

  17. Nuclear safety research collaborations between the US and Russian Federation international nuclear safety centers

    International Nuclear Information System (INIS)

    Hill, D.J; Braun, J.C; Klickman, A.E.; Bugaenko, S.E; Kabanov, L.P; Kraev, A.G.

    2000-01-01

    The Russian Federation Ministry for Atomic Energy (MINATOM) and the U.S. Department of Energy (USDOE) have formed International Nuclear Safety Centers to collaborate on nuclear safety research. USDOE established the U. S. Center at Argonne National Laboratory in October 1995. MINATOM established the Russian Center at the Research and Development Institute of Power Engineering in Moscow in July 1996. In April 1998 the Russian center became an independent, autonomous organization under MINATOM. The goals of the centers are to: cooperate in the development of technologies associated with nuclear safety in nuclear power engineering. be international centers for the collection of information important for safety and technical improvements in nuclear power engineering. maintain a base for fundamental knowledge needed to design nuclear reactors.The strategic approach that is being used to accomplish these goals is for the two centers to work together to use the resources and the talents of the scientists associated with the US Center and the Russian Center to do collaborative research to improve the safety of Russian-designed nuclear reactors

  18. International collaboration and comparative research on ocean top predators under CLIOTOP

    Science.gov (United States)

    Hobday, Alistair J.; Arrizabalaga, Haritz; Evans, Karen; Scales, Kylie L.; Senina, Inna; Weng, Kevin C.

    2017-06-01

    Oceanic top predators have ecological, social and economic value of global significance. These wide-ranging marine species, which include sharks, tunas and billfishes, marine mammals, turtles and seabirds, are the focus of international research attention under the Climate Impacts on Oceanic Top Predators (CLIOTOP) science programme, one of the Integrated Marine Biosphere Research (IMBeR) projects. Over more than a decade, research conducted under CLIOTOP has involved scientists from more than 30 countries, with international collaboration increasing markedly over time, and comparative analyses resulting in new knowledge and understanding of oceanic top predators. This special issue presents 27 papers arising from the 3rd CLIOTOP symposium, held in San Sebastián, Spain in September 2015, spanning topics such as conservation biology, trophic ecology, fisheries science, climate change, and adaptive management. The maturation and synthesis of CLIOTOP's collaborative research is now resulting in real-world management applications and improving understanding of potential ecological and socio-economic impacts of climate change in oceanic systems. The ultimate CLIOTOP goal of preparing both climate-sensitive predator populations and the human societies dependent on them for the impending impacts of climate change is now within reach.

  19. Tc-99m-sestamibi scintigraphy in gaucher disease, type 1

    International Nuclear Information System (INIS)

    Park, Chan H.; Pai, Moon S.; Ha, Man J.; Yoon, S. N.; Kim, S.; Whang, K. H.; Kim, Hyun J.

    1999-01-01

    Gaucher disease is an autosomal recessive disorder characterized by lysosomal glycolipid storage in reticuloendothelial cells due to the deficiency of lysosomal enzyme, acid-glucosidase. Type 1 is one of the three subtypes of Gaucher disease and is manifested by a chronic and progressive involvement of the spleen, liver, bone marrow and other visceral organs. This study was done to see imaging feasibility of bone marrow involvement of Gaucher cells using sestamibi. Five patients with Gaucher disease, type I (M:F=4:1, age range: 9-25) underwent a simultaneous anterior and posterior whole body scan as well as spot views of the lower extremities as needed in 10-20 min following the IV administration of 0.2 mCi/kg of Tc-99m-sestamibi. Control group consisted of 10 patients with osteosarcoma, simple bone cyst, nonossifying fibroma, osteoid osteoma, exostosis and neuroblastoma ( M: F=9:1, age range: 2-20, mean : 12.1) and sestamibi images of the group were obtained as in Gaucher cases. For in vitro evaluation, Gaucher cells were isolated from the splenectomy specimen. The cells were incubated in media containing sestamibi for 10, 29, 30 min. After washing the cells twice with saline, cell labeling was checked by external counting. Control group depicted no appreciable sestamibi uptake in the lower extremities while 5 patients with Gaucher disease, type I revealed variable degrees of sestamibi uptake. It was difficult to assess vertebral activities due to hepatosplenomegaly. Ioslated Gaucher cells took up sestamibi supported by an increasing external counting in proportion to incubation time. There was sestamibi uptake in the lower extremities involved by Gaucher disease, type I, which was distinctly different from the control group. Also in vitro study revealed sestamibi uptake in Gaucher cells. On the basis of these results, we believe, it may be possible to evaluate enzyme replacement therapy in Gaucher disease, type I, utilizing sestamibi scintiscan

  20. Tc-99m-sestamibi scintigraphy in gaucher disease, type 1

    Energy Technology Data Exchange (ETDEWEB)

    Park, Chan H.; Pai, Moon S.; Ha, Man J.; Yoon, S. N.; Kim, S.; Whang, K. H.; Kim, Hyun J. [College of Medicine, Ajou Univ., Suwon (Korea, Republic of)

    1999-07-01

    Gaucher disease is an autosomal recessive disorder characterized by lysosomal glycolipid storage in reticuloendothelial cells due to the deficiency of lysosomal enzyme, acid-glucosidase. Type 1 is one of the three subtypes of Gaucher disease and is manifested by a chronic and progressive involvement of the spleen, liver, bone marrow and other visceral organs. This study was done to see imaging feasibility of bone marrow involvement of Gaucher cells using sestamibi. Five patients with Gaucher disease, type I (M:F=4:1, age range: 9-25) underwent a simultaneous anterior and posterior whole body scan as well as spot views of the lower extremities as needed in 10-20 min following the IV administration of 0.2 mCi/kg of Tc-99m-sestamibi. Control group consisted of 10 patients with osteosarcoma, simple bone cyst, nonossifying fibroma, osteoid osteoma, exostosis and neuroblastoma ( M: F=9:1, age range: 2-20, mean : 12.1) and sestamibi images of the group were obtained as in Gaucher cases. For in vitro evaluation, Gaucher cells were isolated from the splenectomy specimen. The cells were incubated in media containing sestamibi for 10, 29, 30 min. After washing the cells twice with saline, cell labeling was checked by external counting. Control group depicted no appreciable sestamibi uptake in the lower extremities while 5 patients with Gaucher disease, type I revealed variable degrees of sestamibi uptake. It was difficult to assess vertebral activities due to hepatosplenomegaly. Ioslated Gaucher cells took up sestamibi supported by an increasing external counting in proportion to incubation time. There was sestamibi uptake in the lower extremities involved by Gaucher disease, type I, which was distinctly different from the control group. Also in vitro study revealed sestamibi uptake in Gaucher cells. On the basis of these results, we believe, it may be possible to evaluate enzyme replacement therapy in Gaucher disease, type I, utilizing sestamibi scintiscan.

  1. Is There a Relationship between the Usage of Active and Collaborative Learning Techniques and International Students' Study Anxiety?

    Science.gov (United States)

    Khoshlessan, Rezvan

    2013-01-01

    This study was designed to explore the relationships between the international students' perception of professors' instructional practices (the usage of active and collaborative learning techniques in class) and the international students' study anxiety. The dominant goal of this research was to investigate whether the professors' usage of active…

  2. Mass spectrometric quantification of glucosylsphingosine in plasma and urine of type 1 Gaucher patients using an isotope standard.

    Science.gov (United States)

    Mirzaian, Mina; Wisse, Patrick; Ferraz, Maria J; Gold, Henrik; Donker-Koopman, Wilma E; Verhoek, Marri; Overkleeft, Herman S; Boot, Rolf G; Kramer, Gertjan; Dekker, Nick; Aerts, Johannes M F G

    2015-04-01

    Deficiency of glucocerebrosidase (GBA) leads to Gaucher disease (GD), an inherited disorder characterised by storage of glucosylceramide (GlcCer) in lysosomes of tissue macrophages. Recently, we reported marked increases of deacylated GlcCer, named glucosylsphingosine (GlcSph), in plasma of GD patients. To improve quantification, [5-9] (13)C5-GlcSph was synthesised for use as internal standard with quantitative LC-ESI-MS/MS. The method was validated using plasma of 55 GD patients and 20 controls. Intra-assay variation was 1.8% and inter-assay variation was 4.9% for GlcSph (m/z 462.3). Plasma GlcSph levels with the old and new methods closely correlate (r=0.968, slope=1.038). Next, we analysed GlcSph in 24h urine samples of 30 GD patients prior to therapy. GlcSph was detected in the patient samples (median 1.20nM, range 0.11-8.92nM), but was below the limit of quantification in normal urine. Enzyme replacement therapy led to a decrease of urinary GlcSph of GD patients, coinciding with reductions in plasma GlcSph and markers of Gaucher cells (chitotriosidase and CCL18). In analogy to globotriaosylsphingsone in urine of Fabry disease patients, additional isoforms of GlcSph differing in structure of the sphingosine moiety were identified in GD urine samples. In conclusion, GlcSph can be sensitively detected by LC-ESI-MS/MS with an internal isotope standard. Abnormalities in urinary GlcSph are a hallmark of Gaucher disease allowing biochemical confirmation of diagnosis. Copyright © 2015. Published by Elsevier Inc.

  3. International collaboration for development of accident-resistant LWR fuel. International Collaboration for Development of Accident Resistant Light Water Reactor Fuel

    International Nuclear Information System (INIS)

    Sowder, Andrew

    2013-01-01

    Following the March 2011 multi-unit accident at the Fukushima Daiichi plant, there has been increased interest in the development of breakthrough nuclear fuel designs that can reduce or eliminate many of the outcomes of a severe accident at a light water reactor (LWR) due to loss of core cooling following an extended station blackout or other initiating event. With this interest and attention comes a unique opportunity for the nuclear industry to fundamentally change the nature and impact of severe accidents. Clearly, this is no small feat. The challenges are many and the technical barriers are high. Early estimates for moving maturing R and D concepts to the threshold of commercialisation exceed one billion USD. Given the anticipated effort and resources required, no single entity or group can succeed alone. Accordingly, the Electric Power Research Institute (EPRI) sees the need for and promise of cooperation among many stakeholders on an international scale to bring about what could be transformation in LWR fuel performance and robustness. An important initial task in any R and D programme is to define the goals and metrics for measuring success. As starting points for accident-tolerant fuel development, the extension of core coolability under loss of coolant conditions and the elimination or reduction of hydrogen generation are widely recognised R and D endpoints for deployment. Furthermore, any new LWR fuel technology will, at a minimum, need to (1) be compatible with the safe, economic operation of existing plants and (2) maintain acceptable or improve nuclear fuel performance under normal operating conditions. While the primary focus of R and D to date has been on cladding and fuel improvements, there are a number of other potential paths to improve outcomes following a severe accident at an LWR that include modifications to other fuel hardware and core internals to fully address core coolability, criticality, and hydrogen generation concerns. The US

  4. Femoral neck fractures complicating gaucher disease in children

    Energy Technology Data Exchange (ETDEWEB)

    Goldman, A.B.; Jacobs, B.

    1984-09-01

    In normal children, fractures of the femoral neck are uncommon and accompany severe trauma and multiple injuries elsewhere in the skeleton. In children with Gaucher disease, a rare hereditary disorder of lipid metabolism, midcervical or basicervical fractures can occur with minor or no trauma and without other injury to the skeleton. Three children with Gaucher disease who developed pathologic fractures of the femoral neck are described. In all three, the fractures occurred between five and nine years of age, and the fracture lines passed through areas of abnormal bone characterized by poorly defined patches of increased and decreased density and cortical thinning along the medial femoral necks. In the affected hips, there was no evidence of avascular necrosis of the femoral heads at the time of injury. One child's fracture was preceeded by multiple bone 'crisis' localized to the proximal femora.

  5. Radiographic findings in type 3 b Gaucher disease

    International Nuclear Information System (INIS)

    Hill, S.C.; Damaska, B.M.; Tsokos, M.; Kreps, C.; Brady, R.O.; Barton, N.W.

    1996-01-01

    The purpose of this paper is to describe the radiographic findings in type 3 b Gaucher disease, a chronic neuronopathic form of the illness with severe systemic manifestations. Between 1980 and 1985 17 consecutive patients were evaluated with radiography of the chest, long bones and spine, CT of the head and chest, abdominal sonography, and MRI of the head, abdomen and spine. Clinical manifestations were severe, and led to death from hepatic, pulmonary or cardiac failure in nine patients. Type 3 b Gaucher disease shares the same spectrum of radiographic findings observed in type 1 disease, but the systemic manifestations are more severe. Pulmonary infiltrates, thoracic lymph node enlargement, vertebral compression fractures and osteonecrosis of the long bones occur much more frequently in patients with type 3 b disease. (orig.). With 7 figs., 2 tabs

  6. Femoral neck fractures complicating gaucher disease in children

    International Nuclear Information System (INIS)

    Goldman, A.B.; Jacobs, B.

    1984-01-01

    In normal children, fractures of the femoral neck are uncommon and accompany severe trauma and multiple injuries elsewhere in the skeleton. In children with Gaucher disease, a rare hereditary disorder of lipid metabolism, midcervical or basicervical fractures can occur with minor or no trauma and without other injury to the skeleton. Three children with Gaucher disease who developed pathologic fractures of the femoral neck are described. In all three, the fractures occurred between five and nine years of age, and the fracture lines passed through areas of abnormal bone characterized by poorly defined patches of increased and decreased density and cortical thinning along the medial femoral necks. In the affected hips, there was no evidence of avascular necrosis of the femoral heads at the time of injury. One child's fracture was preceeded by multiple bone 'crisis' localized to the proximal femora. (orig.)

  7. Femoral neck fractures complicating gaucher disease in children

    Energy Technology Data Exchange (ETDEWEB)

    Goldman, A B; Jacobs, B

    1984-09-01

    In normal children, fractures of the femoral neck are uncommon and accompany severe trauma and multiple injuries elsewhere in the skeleton. In children with Gaucher disease, a rare hereditary disorder of lipid metabolism, midcervical or basicervical fractures can occur with minor or no trauma and without other injury to the skeleton. Three children with Gaucher disease who developed pathologic fractures of the femoral neck are described. In all three, the fractures occurred between five and nine years of age, and the fracture lines passed through areas of abnormal bone characterized by poorly defined patches of increased and decreased density and cortical thinning along the medial femoral necks. In the affected hips, there was no evidence of avascular necrosis of the femoral heads at the time of injury. One child's fracture was preceeded by multiple bone 'crisis' localized to the proximal femora.

  8. Transgenic mice expressing human glucocerebrosidase variants: utility for the study of Gaucher disease.

    Science.gov (United States)

    Sanders, Angela; Hemmelgarn, Harmony; Melrose, Heather L; Hein, Leanne; Fuller, Maria; Clarke, Lorne A

    2013-08-01

    Gaucher disease is an autosomal recessively inherited storage disorder caused by deficiency of the lysosomal hydrolase, acid β-glucosidase. The disease manifestations seen in Gaucher patients are highly heterogeneous as is the responsiveness to therapy. The elucidation of the precise factors responsible for this heterogeneity has been challenging as the development of clinically relevant animal models of Gaucher disease has been problematic. Although numerous murine models for Gaucher disease have been described each has limitations in their specific utility. We describe here, transgenic murine models of Gaucher disease that will be particularly useful for the study of pharmacological chaperones. We have produced stable transgenic mouse strains that individually express wild type, N370S and L444P containing human acid β-glucosidase and show that each of these transgenic lines rescues the lethal phenotype characteristic of acid β-glucosidase null mice. Both the N370S and L444P transgenic models show early and progressive elevations of tissue sphingolipids with L444P mice developing progressive splenic Gaucher cell infiltration. We demonstrate the potential utility of these new transgenic models for the study of Gaucher disease pathogenesis. In addition, since these mice produce only human enzyme, they are particularly relevant for the study of pharmacological chaperones that are specifically targeted to human acid β-glucosidase and the common mutations underlying Gaucher disease. Copyright © 2013 Elsevier Inc. All rights reserved.

  9. Gaucher's disease in a black child in South Africa. A case report.

    Science.gov (United States)

    Patel, R; MacDougall, L G

    1984-09-01

    A 7-year-old Black boy presented with massive splenomegaly and a tendency to haemorrhage due to type 1 Gaucher's disease. After splenectomy he became asymptomatic and the haematological parameters returned to normal. Although type 1 Gaucher's disease has been described in adult Blacks, it has not been reported previously in a Black child in southern Africa.

  10. Low-dose N-butyldeoxynojirimycin (OGT 918) for type I Gaucher disease

    NARCIS (Netherlands)

    Heitner, Rene; Elstein, Deborah; Aerts, Johannes; Weely, Sonja van; Zimran, Ari

    2002-01-01

    The objective of this study was to evaluate the efficacy and safety of low-dose substrate balance therapy with OGT 918 for the treatment of adults with Gaucher disease. Eighteen patients with Gaucher disease from two centers were enrolled in an open-label 6-month study of OGT 918, 50 mg taken three

  11. Ocular manifestations of Gaucher disease: case report and literature review

    Directory of Open Access Journals (Sweden)

    Mejía-Turizo, Juan Carlos

    2017-07-01

    Full Text Available We report the case of a patient with Gaucher disease (GD type 3b, with a homozygous GBA gene mutation (c.1448T > C p.L483P (L444P. Ocular findings characteristic of this mutation are described, including vitreous condensation and macular edema. To our knowledge this is the first case reported in Colombia with these characteristics. A review of the ocular manifestations of this disease is also presented.

  12. Complement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease.

    Science.gov (United States)

    Pandey, Manoj K; Burrow, Thomas A; Rani, Reena; Martin, Lisa J; Witte, David; Setchell, Kenneth D; Mckay, Mary A; Magnusen, Albert F; Zhang, Wujuan; Liou, Benjamin; Köhl, Jörg; Grabowski, Gregory A

    2017-03-02

    Gaucher disease is caused by mutations in GBA1, which encodes the lysosomal enzyme glucocerebrosidase (GCase). GBA1 mutations drive extensive accumulation of glucosylceramide (GC) in multiple innate and adaptive immune cells in the spleen, liver, lung and bone marrow, often leading to chronic inflammation. The mechanisms that connect excess GC to tissue inflammation remain unknown. Here we show that activation of complement C5a and C5a receptor 1 (C5aR1) controls GC accumulation and the inflammatory response in experimental and clinical Gaucher disease. Marked local and systemic complement activation occurred in GCase-deficient mice or after pharmacological inhibition of GCase and was associated with GC storage, tissue inflammation and proinflammatory cytokine production. Whereas all GCase-inhibited mice died within 4-5 weeks, mice deficient in both GCase and C5aR1, and wild-type mice in which GCase and C5aR were pharmacologically inhibited, were protected from these adverse effects and consequently survived. In mice and humans, GCase deficiency was associated with strong formation of complement-activating GC-specific IgG autoantibodies, leading to complement activation and C5a generation. Subsequent C5aR1 activation controlled UDP-glucose ceramide glucosyltransferase production, thereby tipping the balance between GC formation and degradation. Thus, extensive GC storage induces complement-activating IgG autoantibodies that drive a pathway of C5a generation and C5aR1 activation that fuels a cycle of cellular GC accumulation, innate and adaptive immune cell recruitment and activation in Gaucher disease. As enzyme replacement and substrate reduction therapies are expensive and still associated with inflammation, increased risk of cancer and Parkinson disease, targeting C5aR1 may serve as a treatment option for patients with Gaucher disease and, possibly, other lysosomal storage diseases.

  13. Pseudo-osteomyelitic crisis upon presentation of Gaucher disease

    International Nuclear Information System (INIS)

    Weisstein, J.S.; Steinbach, L.S.; Diamond, C.A.; Huang, S.J.; O'Donnell, R.J.

    2001-01-01

    We report on a 4-year-old boy adopted from Paraguay who presented with an acute onset of thigh pain. Initial clinical, imaging, and histopathologic findings suggested florid osteomyelitis. However, the development of pancytopenia on intravenous antibiotics prompted further investigation and the ultimate diagnosis of Gaucher disease. In retrospect, characteristic changes on conventional radiographic and MR images, as well as growth of a contaminant organism, pointed to the diagnosis of pseudo-osteomyelitis rather than osteomyelitis. (orig.)

  14. Multiple pathogenic proteins implicated in neuronopathic Gaucher disease mice.

    Science.gov (United States)

    Xu, You-hai; Xu, Kui; Sun, Ying; Liou, Benjamin; Quinn, Brian; Li, Rong-hua; Xue, Ling; Zhang, Wujuan; Setchell, Kenneth D R; Witte, David; Grabowski, Gregory A

    2014-08-01

    Gaucher disease, a prevalent lysosomal storage disease (LSD), is caused by insufficient activity of acid β-glucosidase (GCase) and the resultant glucosylceramide (GC)/glucosylsphingosine (GS) accumulation in visceral organs (Type 1) and the central nervous system (Types 2 and 3). Recent clinical and genetic studies implicate a pathogenic link between Gaucher and neurodegenerative diseases. The aggregation and inclusion bodies of α-synuclein with ubiquitin are present in the brains of Gaucher disease patients and mouse models. Indirect evidence of β-amyloid pathology promoting α-synuclein fibrillation supports these pathogenic proteins as a common feature in neurodegenerative diseases. Here, multiple proteins are implicated in the pathogenesis of chronic neuronopathic Gaucher disease (nGD). Immunohistochemical and biochemical analyses showed significant amounts of β-amyloid and amyloid precursor protein (APP) aggregates in the cortex, hippocampus, stratum and substantia nigra of the nGD mice. APP aggregates were in neuronal cells and colocalized with α-synuclein signals. A majority of APP co-localized with the mitochondrial markers TOM40 and Cox IV; a small portion co-localized with the autophagy proteins, P62/LC3, and the lysosomal marker, LAMP1. In cultured wild-type brain cortical neural cells, the GCase-irreversible inhibitor, conduritol B epoxide (CBE), reproduced the APP/α-synuclein aggregation and the accumulation of GC/GS. Ultrastructural studies showed numerous larger-sized and electron-dense mitochondria in nGD cerebral cortical neural cells. Significant reductions of mitochondrial adenosine triphosphate production and oxygen consumption (28-40%) were detected in nGD brains and in CBE-treated neural cells. These studies implicate defective GCase function and GC/GS accumulation as risk factors for mitochondrial dysfunction and the multi-proteinopathies (α-synuclein-, APP- and Aβ-aggregates) in nGD. © The Author 2014. Published by Oxford University

  15. Review of the Strategic Plan for International Collaboration on Fusion Science and Technology Research. Fusion Energy Sciences Advisory Committee (FESAC)

    International Nuclear Information System (INIS)

    1998-01-01

    The United States Government has employed international collaborations in magnetic fusion energy research since the program was declassified in 1958. These collaborations have been successful not only in producing high quality scientific results that have contributed to the advancement of fusion science and technology, they have also allowed us to highly leverage our funding. Thus, in the 1980s, when the funding situation made it necessary to reduce the technical breadth of the U.S. domestic program, these highly leveraged collaborations became key strategic elements of the U.S. program, allowing us to maintain some degree of technical breadth. With the recent, nearly complete declassification of inertial confinement fusion, the use of some international collaboration is expected to be introduced in the related inertial fusion energy research activities as well. The United States has been a leader in establishing and fostering collaborations that have involved scientific and technological exchanges, joint planning, and joint work at fusion facilities in the U.S. and worldwide. These collaborative efforts have proven mutually beneficial to the United States and our partners. International collaborations are a tool that allows us to meet fusion program goals in the most effective way possible. Working with highly qualified people from other countries and other cultures provides the collaborators with an opportunity to see problems from new and different perspectives, allows solutions to arise from the diversity of the participants, and promotes both collaboration and friendly competition. In short, it provides an exciting and stimulating environment resulting in a synergistic effect that is good for science and good for the people of the world.

  16. WDS/DSA Certification - International collaboration for a trustworthy research data infrastructure

    Science.gov (United States)

    Mokrane, Mustapha; Hugo, Wim; Harrison, Sandy

    2016-04-01

    Today's research is international, transdisciplinary, and data-enabled, which requires scrupulous data stewardship, full and open access to data, and efficient collaboration and coordination. New expectations on researchers based on policies from governments and funders to share data fully, openly, and in a timely manner present significant challenges but are also opportunities to improve the quality and efficiency of research and its accountability to society. Researchers should be able to archive and disseminate data as required by many institutions or funders, and civil society to scrutinize datasets underlying public policies. Thus, the trustworthiness of data services must be verifiable. In addition, the need to integrate large and complex datasets across disciplines and domains with variable levels of maturity calls for greater coordination to achieve sufficient interoperability and sustainability. The World Data System (WDS) of the International Council for Science (ICSU) promotes long-term stewardship of, and universal and equitable access to, quality-assured scientific data and services across a range of disciplines in the natural and social sciences. WDS aims at coordinating and supporting trusted scientific data services for the provision, use, and preservation of relevant datasets to facilitate scientific research, in particular under the ICSU umbrella, while strengthening their links with the research community. WDS certifies its Members, holders and providers of data or data products, using internationally recognized standards. Certification of scientific data services is essential to ensure trustworthiness of the global research data infrastructure. It contributes to building a searchable, distributed, interoperable and sustainable research data infrastructure. Several certification standards have been developed over the last decade, such as the Network of Expertise in long-term Storage and Accessibility of Digital Resources in Germany (NESTOR) seal

  17. Investigation of novel pharmacological chaperones for Gaucher Disease.

    Science.gov (United States)

    Yilmazer, Buge; Yagci, Z Begum; Bakar, Emre; Ozden, Burcu; Ulgen, Kutlu; Ozkirimli, Elif

    2017-09-01

    Beta-Glucocerebrosidase (GBA) is a lysosomal protein that is responsible for the hydrolysis of glycosylceramide into glucose and ceramide. Mutations in GBA lead to the accumulation of glycosylceramide in the lysosome causing an enlargement of the spleen and the liver and skeletal deformations. This disease is called Gaucher Disease. Enzyme replacement therapies and substrate reduction methods that are used to treat Gaucher Disease fail when the disease is neuropathic because they fail to pass the blood brain barrier. In this work, QSAR, virtual screening, docking and molecular dynamics simulations were performed to obtain a set of compounds that might be pharmacological chaperones for GBA. ZINC Database was screened using ligand-based and structure-based pharmacophore hypotheses. After docking of these molecules and filtration based on druglikeness, top ranking ligands were identified and their binding stabilities were examined using MD simulations. As a result, seven new compounds that can potentially cross the blood brain barrier were proposed as GBA inhibitors. Three of the seven compounds have a tricyclic pyrido-thieno-pyrimidine scaffold and one has the dioxino quinolone scaffold. Derivatives of these scaffolds have been reported as antiallergic agents, antibiotic and anticancer compounds. These results offer a new approach for the development of new drugs against neuropathic Gaucher Disease Type 2 and Type 3. Copyright © 2017 Elsevier Inc. All rights reserved.

  18. A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments.

    Science.gov (United States)

    Stirnemann, Jérôme; Belmatoug, Nadia; Camou, Fabrice; Serratrice, Christine; Froissart, Roseline; Caillaud, Catherine; Levade, Thierry; Astudillo, Leonardo; Serratrice, Jacques; Brassier, Anaïs; Rose, Christian; Billette de Villemeur, Thierry; Berger, Marc G

    2017-02-17

    Gaucher disease (GD, ORPHA355) is a rare, autosomal recessive genetic disorder. It is caused by a deficiency of the lysosomal enzyme, glucocerebrosidase, which leads to an accumulation of its substrate, glucosylceramide, in macrophages. In the general population, its incidence is approximately 1/40,000 to 1/60,000 births, rising to 1/800 in Ashkenazi Jews. The main cause of the cytopenia, splenomegaly, hepatomegaly, and bone lesions associated with the disease is considered to be the infiltration of the bone marrow, spleen, and liver by Gaucher cells. Type-1 Gaucher disease, which affects the majority of patients (90% in Europe and USA, but less in other regions), is characterized by effects on the viscera, whereas types 2 and 3 are also associated with neurological impairment, either severe in type 2 or variable in type 3. A diagnosis of GD can be confirmed by demonstrating the deficiency of acid glucocerebrosidase activity in leukocytes. Mutations in the GBA1 gene should be identified as they may be of prognostic value in some cases. Patients with type-1 GD-but also carriers of GBA1 mutation-have been found to be predisposed to developing Parkinson's disease, and the risk of neoplasia associated with the disease is still subject to discussion. Disease-specific treatment consists of intravenous enzyme replacement therapy (ERT) using one of the currently available molecules (imiglucerase, velaglucerase, or taliglucerase). Orally administered inhibitors of glucosylceramide biosynthesis can also be used (miglustat or eliglustat).

  19. A Review of Gaucher Disease Pathophysiology, Clinical Presentation and Treatments

    Directory of Open Access Journals (Sweden)

    Jérôme Stirnemann

    2017-02-01

    Full Text Available Gaucher disease (GD, ORPHA355 is a rare, autosomal recessive genetic disorder. It is caused by a deficiency of the lysosomal enzyme, glucocerebrosidase, which leads to an accumulation of its substrate, glucosylceramide, in macrophages. In the general population, its incidence is approximately 1/40,000 to 1/60,000 births, rising to 1/800 in Ashkenazi Jews. The main cause of the cytopenia, splenomegaly, hepatomegaly, and bone lesions associated with the disease is considered to be the infiltration of the bone marrow, spleen, and liver by Gaucher cells. Type-1 Gaucher disease, which affects the majority of patients (90% in Europe and USA, but less in other regions, is characterized by effects on the viscera, whereas types 2 and 3 are also associated with neurological impairment, either severe in type 2 or variable in type 3. A diagnosis of GD can be confirmed by demonstrating the deficiency of acid glucocerebrosidase activity in leukocytes. Mutations in the GBA1 gene should be identified as they may be of prognostic value in some cases. Patients with type-1 GD—but also carriers of GBA1 mutation—have been found to be predisposed to developing Parkinson’s disease, and the risk of neoplasia associated with the disease is still subject to discussion. Disease-specific treatment consists of intravenous enzyme replacement therapy (ERT using one of the currently available molecules (imiglucerase, velaglucerase, or taliglucerase. Orally administered inhibitors of glucosylceramide biosynthesis can also be used (miglustat or eliglustat.

  20. Involvement of Gaucher Disease Mutations in Parkinson Disease.

    Science.gov (United States)

    Vilageliu, Lluisa; Grinberg, Daniel

    2017-01-01

    Gaucher disease is an autosomal recessive lysosomal storage disorder, caused by mutations in the GBA gene. The frequency of Gaucher disease patients and heterozygote carriers that developed Parkinson disease has been found to be above that of the control population. This fact suggests that mutations in the GBA gene can be involved in Parkison's etiology. Analysis of large cohorts of patients with Parkinson disease has shown that there are significantly more cases bearing GBA mutations than those found among healthy individuals. Functional studies have proven an interaction between α-synuclein and GBA, the levels of which presented an inverse correlation. Mutant GBA proteins cause increases in α-synuclein levels, while an inhibition of GBA by α-synuclein has been also demonstrated. Saposin C, a coactivator of GBA, has been shown to protect GBA from this inhibition. Among the GBA variants associated with Parkinson disease, E326K seems to be one of the most prevalent. Interestingly, it is involved in Gaucher disease only when it forms part of a double-mutant allele, usually with the L444P mutation. Structural analyses have revealed that both residues (E326 and L444) interact with Saposin C and, probably, also with α-synuclein. This could explain the antagonistic role of these two proteins in relation to GBA. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

  1. Insights into the structural biology of Gaucher disease.

    Science.gov (United States)

    Smith, Laura; Mullin, Stephen; Schapira, Anthony H V

    2017-12-01

    Gaucher disease, the most common lysosomal storage disorder, is caused by mutations in the gene encoding the acid-β-glucosidase lysosomal hydrolase enzyme that cleaves glucocerebroside into glucose and ceramide. Reduced enzyme activity and impaired structural stability arise due to >300 known disease-causing mutations. Several of these mutations have also been associated with an increased risk of Parkinson disease (PD). Since the discovery of the acid-β-glucosidase X-ray structure, there have been major advances in our understanding of the structural properties of the protein. Analysis of specific residues has provided insight into their functional and structural importance and provided insight into the pathogenesis of Gaucher disease and the contribution to PD. Disease-causing mutations are positioned throughout the acid-β-glucosidase structure, with many located far from the active site and thus retaining some enzymatic activity however, thus far no clear relationship between mutation location and disease severity has been established. Here, we review the crystal structure of acid-β-glucosidase, while highlighting important structural aspects of the protein in detail. This review discusses the structural stability of acid-β-glucosidase, which can be altered by pH and glycosylation, and explores the relationship between known Gaucher disease and PD mutations, structural stability and disease severity. Copyright © 2017. Published by Elsevier Inc.

  2. Global gene expression profile progression in Gaucher disease mouse models

    Directory of Open Access Journals (Sweden)

    Zhang Wujuan

    2011-01-01

    Full Text Available Abstract Background Gaucher disease is caused by defective glucocerebrosidase activity and the consequent accumulation of glucosylceramide. The pathogenic pathways resulting from lipid laden macrophages (Gaucher cells in visceral organs and their abnormal functions are obscure. Results To elucidate this pathogenic pathway, developmental global gene expression analyses were conducted in distinct Gba1 point-mutated mice (V394L/V394L and D409 V/null. About 0.9 to 3% of genes had altered expression patterns (≥ ± 1.8 fold change, representing several categories, but particularly macrophage activation and immune response genes. Time course analyses (12 to 28 wk of INFγ-regulated pro-inflammatory (13 and IL-4-regulated anti-inflammatory (11 cytokine/mediator networks showed tissue differential profiles in the lung and liver of the Gba1 mutant mice, implying that the lipid-storage macrophages were not functionally inert. The time course alterations of the INFγ and IL-4 pathways were similar, but varied in degree in these tissues and with the Gba1 mutation. Conclusions Biochemical and pathological analyses demonstrated direct relationships between the degree of tissue glucosylceramides and the gene expression profile alterations. These analyses implicate IFNγ-regulated pro-inflammatory and IL-4-regulated anti-inflammatory networks in differential disease progression with implications for understanding the Gaucher disease course and pathophysiology.

  3. Bone- and bone marrow scintigraphy in Gaucher disease type 1

    International Nuclear Information System (INIS)

    Mikosch, P.; Zitter, F.; Gallowitsch, H.J.; Lind, P.; Wuertz, F.; Mehta, A.B.; Hughes, D.A.

    2008-01-01

    Scintigraphy is a method for imaging metabolism and should be viewed as complimentary to morphological imaging. Bone and bone marrow scintigraphy can particularly contribute to the detection of focal disease in Gaucher disease. In bone crises it can discriminate within three days after pain onset between local infection and aseptic necrosis. A further advantage of bone- and bone marrow scintigraphy is the visualization of the whole skeleton within one setting. Whole body imaging for focal lesions might thus be an objective in GD, in particular in patients complaining of several painful sites. Direct imaging of bone marrow deposits in GD by MIBI scintigraphy might be of special interest in children in whom bone marrow undergoes a developmental conversion from red to yellow marrow in the ap-pendicular skeleton. MRI interpretation in young GD patients is thus difficult in order to estimate the exact amount and extent of bone marrow infiltration by Gaucher cells. 99mTc-MIBI scintigraphy with its direct visualization of lipid storage could thus add interesting additional information not shown with other methods including MRI. Although MRI is the most accepted imaging modality in assessing the skeletal status in GD, a selective use of scintigraphy for imaging bone and bone marrow may add information in the evaluation of patients with Gaucher disease

  4. Setting a research agenda for progressive multiple sclerosis: the International Collaborative on Progressive MS.

    Science.gov (United States)

    Fox, Robert J; Thompson, Alan; Baker, David; Baneke, Peer; Brown, Doug; Browne, Paul; Chandraratna, Dhia; Ciccarelli, Olga; Coetzee, Timothy; Comi, Giancarlo; Feinstein, Anthony; Kapoor, Raj; Lee, Karen; Salvetti, Marco; Sharrock, Kersten; Toosy, Ahmed; Zaratin, Paola; Zuidwijk, Kim

    2012-11-01

    Despite significant progress in the development of therapies for relapsing MS, progressive MS remains comparatively disappointing. Our objective, in this paper, is to review the current challenges in developing therapies for progressive MS and identify key priority areas for research. A collaborative was convened by volunteer and staff leaders from several MS societies with the mission to expedite the development of effective disease-modifying and symptom management therapies for progressive forms of multiple sclerosis. Through a series of scientific and strategic planning meetings, the collaborative identified and developed new perspectives on five key priority areas for research: experimental models, identification and validation of targets and repurposing opportunities, proof-of-concept clinical trial strategies, clinical outcome measures, and symptom management and rehabilitation. Our conclusions, tackling the impediments in developing therapies for progressive MS will require an integrated, multi-disciplinary approach to enable effective translation of research into therapies for progressive MS. Engagement of the MS research community through an international effort is needed to address and fund these research priorities with the ultimate goal of expediting the development of disease-modifying and symptom-relief treatments for progressive MS.

  5. Impact of International Collaborative Project on Cultural Competence among Occupational Therapy Students

    Directory of Open Access Journals (Sweden)

    Divya Sood OTD, OTR/L

    2014-07-01

    Full Text Available Occupational therapy (OT educators recognize a need to ensure that OT students are culturally competent. The researchers developed the International Collaborative Project on Cultural Competence (ICPCC to help students understand the impact of cultural context on client care. Entry-level MOT students from a university in the US (N = 18 collaborated with BOT students (N = 4 and advanced MOT students (N = 9 from two universities in India using an online course management system WebCT. The study explored the impact of the ICPCC on OT students’ cultural competence and discusses students’ perceptions of culture on the OT process. The Inventory for Assessing the Process of Cultural Competence Among Health Care Professionals Revised© measured students’ cultural competence at baseline and immediately after participation in the ICPCC. Qualitative data was collected using a Self-Reflection Form. There was an increase in the cultural competence scores among all three groups of students after participating in the ICPCC at p value < .05. Three themes emerged from the qualitative data analysis: meaning of the term culture, impact of cultural on client- centered practice, and impact of cultural on OT outcomes. OT students recognized the role that cultural differences play in OT evaluation and intervention.

  6. Energy technologies at the cutting edge: international energy technology collaboration IEA Implementing Agreements

    Energy Technology Data Exchange (ETDEWEB)

    Pottinger, C. (ed.)

    2007-05-15

    Ensuring energy security and addressing climate change issues in a cost-effective way are the main challenges of energy policies and in the longer term will be solved only through technology cooperation. To encourage collaborative efforts to meet these energy challenges, the IEA created a legal contract - Implementing Agreement - and a system of standard rules and regulations. This allows interested member and non-member governments or other organisations to pool resources and to foster the research, development and deployment of particular technologies. For more than 30 years, this international technology collaboration has been a fundamental building block in facilitating progress of new or improved energy technologies. There are now 41 Implementing Agreements. This is the third in the series of publications highlighting the recent results and achievements of the IEA Implementing Agreements. This document is arranged in the following sections: Cross-cutting activities (sub-sectioned: Climate technology initiative; Energy Technology Data Eexchange; and Energy technology systems analysis programme); End-use technologies (sub-sectioned: Buildings; Electricity; Industry; and Transport; Fossil fuels (sub-sectioned: Clean Coal Centre; Enhanced oil recovery Fluidized bed conversion; Greenhouse Gas R & D; Multiphase flow sciences); Fusion power; Renewable energies and hydrogen; and For more information (including detail on the IEA energy technology network; IEA Secretariat Implementing Agreement support; and IEA framework. Addresses are given for the Implementing Agreements. The publication is based on core input from the Implementing Agreement Executive Committee.

  7. Setting a research agenda for progressive multiple sclerosis: The International Collaborative on Progressive MS

    Science.gov (United States)

    Thompson, Alan; Baker, David; Baneke, Peer; Brown, Doug; Browne, Paul; Chandraratna, Dhia; Ciccarelli, Olga; Coetzee, Timothy; Comi, Giancarlo; Feinstein, Anthony; Kapoor, Raj; Lee, Karen; Salvetti, Marco; Sharrock, Kersten; Toosy, Ahmed; Zaratin, Paola; Zuidwijk, Kim

    2012-01-01

    Despite significant progress in the development of therapies for relapsing MS, progressive MS remains comparatively disappointing. Our objective, in this paper, is to review the current challenges in developing therapies for progressive MS and identify key priority areas for research. A collaborative was convened by volunteer and staff leaders from several MS societies with the mission to expedite the development of effective disease-modifying and symptom management therapies for progressive forms of multiple sclerosis. Through a series of scientific and strategic planning meetings, the collaborative identified and developed new perspectives on five key priority areas for research: experimental models, identification and validation of targets and repurposing opportunities, proof-of-concept clinical trial strategies, clinical outcome measures, and symptom management and rehabilitation. Our conclusions, tackling the impediments in developing therapies for progressive MS will require an integrated, multi-disciplinary approach to enable effective translation of research into therapies for progressive MS. Engagement of the MS research community through an international effort is needed to address and fund these research priorities with the ultimate goal of expediting the development of disease-modifying and symptom-relief treatments for progressive MS. PMID:22917690

  8. Ethics of international clinical research collaboration - the experience of AlloStem.

    Science.gov (United States)

    Chaplin, C

    2006-02-01

    This paper examines the ethics of international clinical collaboration in stem cell research by focusing on the AlloStem project. AlloStem is an international research programme, financed by the European Union under the Sixth Framework Programme, with the aim of advancing the use of stem cells in treating leukaemia and other haematological diseases. Several areas of ethical importance are explored. Research justification and the need to consider both deontological and teleological aspects are examined. Ethical sensitivity in research and the requirement to respond to areas of ethical concern identified by the European Commission, such as the involvement of human beings, the use of human tissue, and the use of animals are also explored. Ethical issues around project structure and management, such as ethical standardization in international research, and achieving set targets are discussed. The ethical importance of dissemination of findings and teaching in clinical research is also considered. Finally, the distribution of benefits is addressed and the importance of distributive justice is emphasized.

  9. Large Matched-Index-of-Refraction (MIR) Flow Systems for International Collaboration In Fluid Mechanics

    International Nuclear Information System (INIS)

    McEligot, Donald M.; Becker, Stefan; McIlroy, Hugh M. Jr.

    2010-01-01

    In recent international collaboration, INL and Uni. Erlangen have developed large MIR flow systems which can be ideal for joint graduate student education and research. The benefit of the MIR technique is that it permits optical measurements to determine flow characteristics in complex passages and around objects to be obtained without locating a disturbing transducer in the flow field and without distortion of the optical paths. The MIR technique is not new itself; others employed it earlier. The innovation of these MIR systems is their large size relative to previous experiments, yielding improved spatial and temporal resolution. This report will discuss the benefits of the technique, characteristics of the systems and some examples of their applications to complex situations. Typically their experiments have provided new fundamental understanding plus benchmark data for assessment and possible validation of computational thermal fluid dynamic codes.

  10. Large Matched-Index-of-Refraction (MIR) Flow Systems for International Collaboration In Fluid Mechanics

    Energy Technology Data Exchange (ETDEWEB)

    Donald M. McEligot; Stefan Becker; Hugh M. McIlroy, Jr.

    2010-07-01

    In recent international collaboration, INL and Uni. Erlangen have developed large MIR flow systems which can be ideal for joint graduate student education and research. The benefit of the MIR technique is that it permits optical measurements to determine flow characteristics in complex passages and around objects to be obtained without locating a disturbing transducer in the flow field and without distortion of the optical paths. The MIR technique is not new itself; others employed it earlier. The innovation of these MIR systems is their large size relative to previous experiments, yielding improved spatial and temporal resolution. This report will discuss the benefits of the technique, characteristics of the systems and some examples of their applications to complex situations. Typically their experiments have provided new fundamental understanding plus benchmark data for assessment and possible validation of computational thermal fluid dynamic codes.

  11. Building a Collaborative Network for Education and Training in International Trade Facilitation Clusters

    Science.gov (United States)

    Clendenin, John A.; Petrova, Nadya N.; Gill, Joshua K.

    The authors present the benefits of collaboration rather than competition in developing educational and training resources for international trade within a geographic region and explore the challenges for business partners, governments and educational institutions. The paper indicates that flexibility in the 21st Century is critical, particularly when striving for virtual implementations of the solution services. It is essential, say the authors, for educators, governments and business executives to focus on performance and the careful orchestration and integration of business, policy and information technology for “Networking” that successfully stimulates inter-governmental cooperation and innovative policies that foster Regional trade facilitation. An innovative way to enhance 21st Century Trade Facilitation is offered with Supply Chain Centers of Regional Excellence (SCcORE).

  12. International collaborations in learning and teaching: perspectives from a visiting professorship.

    Science.gov (United States)

    Spring, Hannah; Kunkel, Marita; Gilman, Isaac; Henderson, Nancy; White, John

    2016-09-01

    This article provides a reflection on the outcomes of an international collaboration between health librarians and academics at York St John University and Pacific University Oregon. In particular, it describes how a month-long visiting professorship from an academic with a clinical librarian background at the Faculty of Health and Life Sciences helped to develop and inform teaching practice in the areas of information literacy and evidence-based health practice on health programmes at Pacific University. Perspectives are offered from both institutions on the rich exchange of knowledge and practice that took place during the visit and the ongoing impact it has had on teaching practices. H. S. © 2016 Health Libraries Group.

  13. Nuclear safety research collaborations between the U.S. and Russian Federation International Nuclear Safety Centers

    International Nuclear Information System (INIS)

    Hill, D. J.; Braun, J. C.; Klickman, A. E.; Bougaenko, S. E.; Kabonov, L. P.; Kraev, A. G.

    2000-01-01

    The Russian Federation Ministry for Atomic Energy (MINATOM) and the US Department of Energy (USDOE) have formed International Nuclear Safety Centers to collaborate on nuclear safety research. USDOE established the US Center (ISINSC) at Argonne National Laboratory (ANL) in October 1995. MINATOM established the Russian Center (RINSC) at the Research and Development Institute of Power Engineering (RDIPE) in Moscow in July 1996. In April 1998 the Russian center became a semi-independent, autonomous organization under MINATOM. The goals of the center are to: Cooperate in the development of technologies associated with nuclear safety in nuclear power engineering; Be international centers for the collection of information important for safety and technical improvements in nuclear power engineering; and Maintain a base for fundamental knowledge needed to design nuclear reactors. The strategic approach is being used to accomplish these goals is for the two centers to work together to use the resources and the talents of the scientists associated with the US Center and the Russian Center to do collaborative research to improve the safety of Russian-designed nuclear reactors. The two centers started conducting joint research and development projects in January 1997. Since that time the following ten joint projects have been initiated: INSC databases--web server and computing center; Coupled codes--Neutronic and thermal-hydraulic; Severe accident management for Soviet-designed reactors; Transient management and advanced control; Survey of relevant nuclear safety research facilities in the Russian Federation; Computer code validation for transient analysis of VVER and RBMK reactors; Advanced structural analysis; Development of a nuclear safety research and development plan for MINATOM; Properties and applications of heavy liquid metal coolants; and Material properties measurement and assessment. Currently, there is activity in eight of these projects. Details on each of these

  14. Intemational collaborative study on the preparation of 1st international standard for rhTSH for bioassay

    International Nuclear Information System (INIS)

    Huang Ying; Shen Hongzheng; Yu Ting; Xu Ligen

    2007-01-01

    The history of the international collaborative studies on the preparation of standards of TSH for bioassay and immunoassay was reviewed. The result of collaborative study on the 1st international standard for thyroid-stimulating hormone, recombinant, human, for bioassay was reported in detail in this article. Based on the results of this collaborative study, it is proposed that the candidate standard be established as the international standard for rhTSH for bioassay, and be assigned an activity of 9.5 IU per ampoule. The national standard preparation of TSH for immunoassay was also reassayed, revealing the potency to be 0.557 mIU/ampoule, i.e. 92. 8% of the labelled value of 0.600mIU/ampoule, a reasonable consistency. (authors)

  15. International Collaboration in the field of GNSS-Meteorology and Climate Monitoring

    Science.gov (United States)

    Jones, J.; Guerova, G.; Dousa, J.; Bock, O.; Elgered, G.; Vedel, H.; Pottiaux, E.; de Haan, S.; Pacione, R.; Dick, G.; Wang, J.; Gutman, S. I.; Wickert, J.; Rannat, K.; Liu, G.; Braun, J. J.; Shoji, Y.

    2012-12-01

    International collaboration in the field of GNSS-meteorology and climate monitoring is essential, as severe weather and climate change have no respect for national boundaries. The use of Global Navigation Satellite Systems (GNSS) for meteorological purposes is an established atmospheric observing technique, which can accurately sense water vapour, the most abundant greenhouse gas, accounting for 60-70% of atmospheric warming. Severe weather forecasting is challenging, in part due to the high temporal and spatial variation of atmospheric water vapour. Water vapour is currently under-sampled and obtaining and exploiting more high-quality humidity observations is essential to severe weather forecasting and climate monitoring. A proposed EU COST Action (http://www.cost.eu) will address new and improved capabilities from concurrent developments in both GNSS and atmospheric communities to improve (short-range) weather forecasts and climate projections. For the first time, the synergy of the three GNSS systems, GPS, GLONASS and Galileo, will be used to develop new, advanced tropospheric products, stimulating the full potential exploitation of multi-GNSS water vapour estimates on a wide range of temporal and spatial scales, from real-time severe weather monitoring and forecasting to climate research. The Action will work in close collaboration with the Global Climate Observing System (GCOS) Reference Upper Air Network (GRUAN), GNSS Precipitable Water Task Team (TT). GRUAN is a global reference observing network, designed to meet climate requirements and to fill a major void in the current global observing system. GRUAN observations will provide long-term, high-quality data to determine climatic trends and to constrain and validate data from space-based remote sensors. Ground-based GNSS PW was identified as a Priority 1 measurement for GRUAN, and the GNSS-PW TT's goal is to develop explicit guidance on hardware, software and data management practices to obtain GNSS PW

  16. Collaboration between infection control and occupational health in three continents: a success story with international impact

    Directory of Open Access Journals (Sweden)

    Ndelu Lindiwe

    2011-11-01

    Full Text Available Abstract Globalization has been accompanied by the rapid spread of infectious diseases, and further strain on working conditions for health workers globally. Post-SARS, Canadian occupational health and infection control researchers got together to study how to better protect health workers, and found that training was indeed perceived as key to a positive safety culture. This led to developing information and communication technology (ICT tools. The research conducted also showed the need for better workplace inspections, so a workplace audit tool was also developed to supplement worker questionnaires and the ICT. When invited to join Ecuadorean colleagues to promote occupational health and infection control, these tools were collectively adapted and improved, including face-to-face as well as on-line problem-based learning scenarios. The South African government then invited the team to work with local colleagues to improve occupational health and infection control, resulting in an improved web-based health information system to track incidents, exposures, and occupational injury and diseases. As the H1N1 pandemic struck, the online infection control course was adapted and translated into Spanish, as was a novel skill-building learning tool that permits health workers to practice selecting personal protective equipment. This tool was originally developed in collaboration with the countries from the Caribbean region and the Pan American Health Organization (PAHO. Research from these experiences led to strengthened focus on building capacity of health and safety committees, and new modules are thus being created, informed by that work. The products developed have been widely heralded as innovative and interactive, leading to their inclusion into “toolkits” used internationally. The tools used in Canada were substantially improved from the collaborative adaptation process for South and Central America and South Africa. This international

  17. Collaboration between infection control and occupational health in three continents: a success story with international impact.

    Science.gov (United States)

    Yassi, Annalee; Bryce, Elizabeth A; Breilh, Jaime; Lavoie, Marie-Claude; Ndelu, Lindiwe; Lockhart, Karen; Spiegel, Jerry

    2011-11-08

    Globalization has been accompanied by the rapid spread of infectious diseases, and further strain on working conditions for health workers globally. Post-SARS, Canadian occupational health and infection control researchers got together to study how to better protect health workers, and found that training was indeed perceived as key to a positive safety culture. This led to developing information and communication technology (ICT) tools. The research conducted also showed the need for better workplace inspections, so a workplace audit tool was also developed to supplement worker questionnaires and the ICT. When invited to join Ecuadorean colleagues to promote occupational health and infection control, these tools were collectively adapted and improved, including face-to-face as well as on-line problem-based learning scenarios. The South African government then invited the team to work with local colleagues to improve occupational health and infection control, resulting in an improved web-based health information system to track incidents, exposures, and occupational injury and diseases. As the H1N1 pandemic struck, the online infection control course was adapted and translated into Spanish, as was a novel skill-building learning tool that permits health workers to practice selecting personal protective equipment. This tool was originally developed in collaboration with the countries from the Caribbean region and the Pan American Health Organization (PAHO). Research from these experiences led to strengthened focus on building capacity of health and safety committees, and new modules are thus being created, informed by that work.The products developed have been widely heralded as innovative and interactive, leading to their inclusion into "toolkits" used internationally. The tools used in Canada were substantially improved from the collaborative adaptation process for South and Central America and South Africa. This international collaboration between occupational

  18. International Collaboration and Spatial Dynamics of US Patenting in Central and Eastern Europe 1981-2010

    Science.gov (United States)

    Lengyel, Balázs; Leskó, Mariann

    2016-01-01

    How did post-socialist transition and a parallel shift in international labor division restructure regional innovation systems in Central and Eastern Europe? This question is increasingly important, because current EU innovation policy is combined with regional development in Smart Specialization Strategies; however, spatial trends of innovation in Central and Eastern Europe are not fully understood which might lead to less than perfectly efficient policy. In this paper we describe the spatial dynamics of inventor activity in the Czech Republic, Hungary, Poland and Slovakia between 1981 and 2010 –a period that covers both the late socialist era and the post-socialist transition. Cleaning and analyzing the publicly available data from the United States Patent and Trademark Office we illustrate that Central and Eastern European patents made in international co-operations with partners outside the region receive more citations than those Central and Eastern European patents that lack international co-operation. Furthermore, the technological portfolio of the former patents has become increasingly independent from the technological portfolio of the latter class. A town-level analysis of the applicant-inventor ties reveals that inventors have started to work for foreign assignees in those towns where no innovation activity had been recorded before. However, the positive effect does not last long and patenting seems to be only periodic in the majority of these towns. Therefore, innovation policy in Central and Eastern European countries, as well as in other less developed regions, shall foster synergies between international and domestic collaborations in order to decrease regional disparities in patenting. PMID:27846288

  19. International Collaboration and Spatial Dynamics of US Patenting in Central and Eastern Europe 1981-2010.

    Science.gov (United States)

    Lengyel, Balázs; Leskó, Mariann

    2016-01-01

    How did post-socialist transition and a parallel shift in international labor division restructure regional innovation systems in Central and Eastern Europe? This question is increasingly important, because current EU innovation policy is combined with regional development in Smart Specialization Strategies; however, spatial trends of innovation in Central and Eastern Europe are not fully understood which might lead to less than perfectly efficient policy. In this paper we describe the spatial dynamics of inventor activity in the Czech Republic, Hungary, Poland and Slovakia between 1981 and 2010 -a period that covers both the late socialist era and the post-socialist transition. Cleaning and analyzing the publicly available data from the United States Patent and Trademark Office we illustrate that Central and Eastern European patents made in international co-operations with partners outside the region receive more citations than those Central and Eastern European patents that lack international co-operation. Furthermore, the technological portfolio of the former patents has become increasingly independent from the technological portfolio of the latter class. A town-level analysis of the applicant-inventor ties reveals that inventors have started to work for foreign assignees in those towns where no innovation activity had been recorded before. However, the positive effect does not last long and patenting seems to be only periodic in the majority of these towns. Therefore, innovation policy in Central and Eastern European countries, as well as in other less developed regions, shall foster synergies between international and domestic collaborations in order to decrease regional disparities in patenting.

  20. Enzyme replacement and substrate reduction therapy for Gaucher disease.

    Science.gov (United States)

    Shemesh, Elad; Deroma, Laura; Bembi, Bruno; Deegan, Patrick; Hollak, Carla; Weinreb, Neal J; Cox, Timothy M

    2015-03-27

    Gaucher disease, a rare disorder, is caused by inherited deficiency of the enzyme glucocerebrosidase. It is unique among the ultra-orphan disorders in that four treatments are currently approved by various regulatory authorities for use in routine clinical practice. Hitherto, because of the relatively few people affected worldwide, many of whom started therapy during a prolonged period when there were essentially no alternatives to imiglucerase, these treatments have not been systematically evaluated in studies such as randomized controlled trials now considered necessary to generate the highest level of clinical evidence. To summarize all available randomized controlled study data on the efficacy and safety of enzyme replacement therapies and substrate reduction therapy for treating Gaucher disease. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Inborn Errors of Metabolism Trials Register. Additional searches were conducted on ClinicalTrials.gov for any ongoing studies with potential interim results, and through PubMed. We also searched the reference lists of relevant articles and reviews.Date of last search: 07 August 2014. All randomized and quasi-randomized controlled studies (including open-label studies and cross-over studies) assessing enzyme replacement therapy or substrate reduction therapy, or both, in all types of Gaucher disease were included. Two authors independently assessed the risk of bias in the included studies, and extracted relevant data. Of the 488 studies retrieved by the electronic searches, eight met the inclusion criteria and were analysed (300 participants). Response parameters were restricted to haemoglobin concentration, platelet count, spleen and liver volume and serum biomarkers (chitotriosidase and CCL18). Only one publication reported a 'low risk of bias' score in all parameters assessed, and all studies included were randomized.Four studies reported the responses to enzyme replacement therapy of previously

  1. Nuclear Measurements, Evaluations and Applications (NEMEA-7) Collaborative International Evaluated Library Organisation (CIELO). Workshop Proceedings

    International Nuclear Information System (INIS)

    Chadwick, Mark; Plompen, Arjan; ); Emmeric Dupont; )

    2014-01-01

    The 7. workshop on Nuclear Measurements, Evaluations and Applications (NEMEA) focused on international collaboration in nuclear data by hosting the kick-off meeting of the pilot project of the Collaborative International Evaluated Library Organisation (CIELO). CIELO aims at fostering nuclear data advances by using the joint expertise of the nuclear data community under the auspices of the OECD Nuclear Energy Agency. The workshop aimed at status reviews of planned and completed contributions and related developments for the CIELO pilot isotopes. The workshop further sought to facilitate in-depth discussions on nuclear data issues which are being addressed in the framework of European Commission projects like ERINDA, EUFRAT, ANDES and CHANDA. The 7. workshop on Nuclear Measurements, Evaluations and Applications (NEMEA) provided an opportunity for the Collaborative International Evaluated Library Organisation (CIELO) to meet and advance its objectives to improve our understanding of neutron reactions on key isotopes that are especially important in nuclear applications, especially in the area of criticality safety and reactors. CIELO is focusing initially on six nuclides ( 1 H, 16 O, 56 Fe, 235 U, 238 U and 239 Pu). These nuclides are important in the aforementioned applications, and despite decades of work many open questions remain to be solved. In some cases, the existing evaluations need improvement because the underlying experimental measurements are either lacking or contradictory. In other cases, nuclear theory work is needed to better advance predictions. In yet other cases, information from cross-section measurements is proving difficult to reconcile with information from integral nuclear criticality experiments or neutron shielding experiments. The main challenges to be faced are as follows: For oxygen, new work is needed to better define the total and elastic cross-sections at lower energies and neutron scattering angular distributions. An outstanding

  2. Improving Access to Pediatric Cardiology in Cape Verde via a Collaborative International Telemedicine Service.

    Science.gov (United States)

    Lapão, Luís Velez; Correia, Artur

    2015-01-01

    This paper addresses the role of international telemedicine services in supporting the evacuation procedures from Cape Verde to Portugal, enabling better quality and cost reductions in the management of the global health system. The Cape Verde, as other African countries, health system lacks many medical specialists, like pediatric cardiologists, neurosurgery, etc. In this study, tele-cardiology shows good results as diagnostic support to the evacuation decision. Telemedicine services show benefits while monitoring patients in post-evacuation, helping to address the lack of responsive care in some specialties whose actual use will help save resources both in provision and in management of the evacuation procedures. Additionally, with tele-cardiology collaborative service many evacuations can be avoided whereas many cases will be treated and followed locally in Cape Verde with remote technical support from Portugal. This international telemedicine service enabled more efficient evacuations, by reducing expenses in travel and housing, and therefore contributed to the health system's improvement. This study provides some evidence of how important telemedicine really is to cope with both the geography and the shortage of physicians.

  3. Exploring the patient journey to diagnosis of Gaucher disease from the perspective of 212 patients with Gaucher disease and 16 Gaucher expert physicians.

    Science.gov (United States)

    Mehta, Atul; Belmatoug, Nadia; Bembi, Bruno; Deegan, Patrick; Elstein, Deborah; Göker-Alpan, Özlem; Lukina, Elena; Mengel, Eugen; Nakamura, Kimitoshi; Pastores, Gregory M; Pérez-López, Jordi; Schwartz, Ida; Serratrice, Christine; Szer, Jeffrey; Zimran, Ari; Di Rocco, Maja; Panahloo, Zoya; Kuter, David J; Hughes, Derralynn

    2017-11-01

    Gaucher disease (GD) is a rare hereditary disorder caused by a deficiency of the lysosomal enzyme β-glucocerebrosidase. Diagnosis is challenging owing to a wide variability in clinical manifestations and severity of symptoms. Many patients may experience marked delays in obtaining a definitive diagnosis. The two surveys reported herein aimed to explore the patient journey to diagnosis of GD from the perspectives of Gaucher expert physicians and patients. Findings from the surveys revealed that many patients experienced diagnostic delays and misdiagnoses, with nearly 1 in 6 patients stating that they were not diagnosed with GD for 7years or more after first consulting a doctor. Physicians and patients both reported multiple referrals to different specialties before a diagnosis of GD was obtained, with primary care, haematology/haematology-oncology and paediatrics the main specialties to which patients first presented. Splenomegaly, thrombocytopenia, anaemia and bone pain were reported as the most common medical problems at first presentation in both surveys. These findings support a clear need for straightforward and easy-to-follow guidance designed to assist non-specialists to identify earlier patients who are at risk of GD. Copyright © 2017 The Authors and Shire HGT Inc. Published by Elsevier Inc. All rights reserved.

  4. Unbalanced international collaboration affects adversely the usefulness of countries' scientific output as well as their technological and social impact.

    Science.gov (United States)

    Zanotto, Sonia R; Haeffner, Cristina; Guimarães, Jorge A

    The unbalanced international scientific collaboration as cause of misleading information on the country's contribution to the scientific world output was analyzed. ESI Data Base (Thomson Reuters' InCites), covering the scientific production of 217 active countries in the period 2010-2014 was used. International collaboration implicates in a high percentage (33.1 %) of double-counted world articles, thus impacting qualitative data as citations, impact and impact relative to word. The countries were divided into three groups, according to their individual contribution to the world publications: Group I (24 countries, at least 1 %) representing 83.9 % of the total double-counted world articles. Group II (40 countries, 0.1-0.99 % each). Group III, 153 countries (70.5 %) with international collaboration were: Group I, 43.0 %; Group II, 55.8 % and Group III, 85.2 %. We concluded that very high and unbalanced international collaboration, as presented by many countries, misrepresent the importance of their scientific production, technological and social outputs. Furthermore, it jeopardizes qualitative outputs of the countries themselves, artificially increasing their scientific impact, affecting all fields and therefore, the whole world. The data confirm that when dealing with the qualitative contribution of countries, it is necessary to take in consideration the level of international cooperation because, as seen here, it can and in fact it does create false impression of the real contribution of countries.

  5. Sustaining a Global Geoscience Workforce-The Case for International Collaboration

    Science.gov (United States)

    Leahy, P. P.; Keane, C. M.

    2013-05-01

    Maintaining an adequate global supply of qualified geoscientists is a major challenge facing the profession. With global population expected to exceed 9 billion by midcentury, the demand for geoscience expertise is expected to dramatically increase if we are to provide to society the resource base, environmental quality, and resiliency to natural hazards that is required to meet future global demands. The American Geoscience Institute (AGI) has for the past 50 years tracked the supply of geoscientists and their various areas of specialty for the US. However, this is only part of the necessary workforce analysis, the demand side must also be determined. For the past several years, AGI has worked to acquire estimates for workforce demand in the United States. The analysis suggests that by 2021 there will be between 145,000 to 202,000 unfilled jobs in the US. This demand can be partially filled with an increase in graduates (which is occurring at an insufficient pace in the US to meet full demand), increased migration of geoscientists internationally to the US (a challenge since demands are increasing globally), and more career placement of bachelor degree recipients. To understand the global workforce dynamic, it is critical that accurate estimates of global geoscience supply, demand and retirement be available. Although, AGI has focused on the US situation, it has developed international collaborations to acquire workforce data. Among the organizations that have contributed are UNESCO, the International Union of Geological Sciences (IUGS), the Young Earth-Scientists Network, and the Geological Society of Africa. Among the areas of international collaboration, the IUGS Task Group on Global Geoscience Workforce enables the IUGS to take a leadership role in raising the quality of understanding of workforce across the world. During the course of the taskforce's efforts, several key understandings have emerged. First, the general supply of geoscientists is quantifiable

  6. Collaborative study to assess the suitability of a candidate International Standard for yellow fever vaccine.

    Science.gov (United States)

    Ferguson, Morag; Heath, Alan

    2004-12-01

    Yellow fever vaccines are routinely assayed by plaque assay. However, the results of these assays are then converted into mouse LD(50) using correlations/conversion factors which, in many cases, were established many years ago. The minimum required potency in WHO Recommendations is 10(3) LD(50)/dose. Thirteen participants from 8 countries participated in a collaborative study whose aim was to assess the suitability of two candidate preparations to serve as an International Standard for yellow fever vaccine. In addition, the study investigated the relationship between the mouse LD(50) test and plaque forming units with a view to updating the WHO recommendations. Plaque assays were more reproducible than mouse assays, as expected. Differences in sensitivities of plaque assays were observed between laboratories but these differences appear to be consistent within a laboratory for all samples and the expression of potency relative to the candidate standard vaccine improved the reproducibility of assays between laboratories. However, the use of potencies had little effect on the between laboratory variability in mouse LD(50) assays. There appears to be a consistent relationship between overall mean LD(50) and plaques titre for all study preparations other than sample E. The slope of the correlation curve is >1 and it would appear that 10(3) LD(50) is approximately equivalent to 10(4) plaque forming units (PFU), based on the overall means of all laboratory results. The First International Standard for yellow fever vaccine, NIBSC Code 99/616, has been established as the First International Standard for yellow fever vaccine by the Expert Committee of Biological Standards of the World Health Organisation. The International Standard has been arbitrarily assigned a potency of 10(4.5) International Units (IU) per ampoule. Manufacturers and National Control Laboratories are including the First International Standard for yellow fever vaccine in routine assays so that the minimum

  7. Virtual Interactive Presence in Global Surgical Education: International Collaboration Through Augmented Reality.

    Science.gov (United States)

    Davis, Matthew Christopher; Can, Dang D; Pindrik, Jonathan; Rocque, Brandon G; Johnston, James M

    2016-02-01

    Technology allowing a remote, experienced surgeon to provide real-time guidance to local surgeons has great potential for training and capacity building in medical centers worldwide. Virtual interactive presence and augmented reality (VIPAR), an iPad-based tool, allows surgeons to provide long-distance, virtual assistance wherever a wireless internet connection is available. Local and remote surgeons view a composite image of video feeds at each station, allowing for intraoperative telecollaboration in real time. Local and remote stations were established in Ho Chi Minh City, Vietnam, and Birmingham, Alabama, as part of ongoing neurosurgical collaboration. Endoscopic third ventriculostomy with choroid plexus coagulation with VIPAR was used for subjective and objective evaluation of system performance. VIPAR allowed both surgeons to engage in complex visual and verbal communication during the procedure. Analysis of 5 video clips revealed video delay of 237 milliseconds (range, 93-391 milliseconds) relative to the audio signal. Excellent image resolution allowed the remote neurosurgeon to visualize all critical anatomy. The remote neurosurgeon could gesture to structures with no detectable difference in accuracy between stations, allowing for submillimeter precision. Fifteen endoscopic third ventriculostomy with choroid plexus coagulation procedures have been performed with the use of VIPAR between Vietnam and the United States, with no significant complications. 80% of these patients remain shunt-free. Evolving technologies that allow long-distance, intraoperative guidance, and knowledge transfer hold great potential for highly efficient international neurosurgical education. VIPAR is one example of an inexpensive, scalable platform for increasing global neurosurgical capacity. Efforts to create a network of Vietnamese neurosurgeons who use VIPAR for collaboration are underway. Copyright © 2016 Elsevier Inc. All rights reserved.

  8. Identification of a biomarker in cerebrospinal fluid for neuronopathic forms of Gaucher disease.

    Science.gov (United States)

    Zigdon, Hila; Savidor, Alon; Levin, Yishai; Meshcheriakova, Anna; Schiffmann, Raphael; Futerman, Anthony H

    2015-01-01

    Gaucher disease, a recessive inherited metabolic disorder caused by defects in the gene encoding glucosylceramidase (GlcCerase), can be divided into three subtypes according to the appearance of symptoms associated with central nervous system involvement. We now identify a protein, glycoprotein non-metastatic B (GPNMB), that acts as an authentic marker of brain pathology in neurological forms of Gaucher disease. Using three independent techniques, including quantitative global proteomic analysis of cerebrospinal fluid (CSF) in samples from Gaucher disease patients that display neurological symptoms, we demonstrate a correlation between the severity of symptoms and GPNMB levels. Moreover, GPNMB levels in the CSF correlate with disease severity in a mouse model of Gaucher disease. GPNMB was also elevated in brain samples from patients with type 2 and 3 Gaucher disease. Our data suggest that GPNMB can be used as a marker to quantify neuropathology in Gaucher disease patients and as a marker of treatment efficacy once suitable treatments towards the neurological symptoms of Gaucher disease become available.

  9. Identification of a biomarker in cerebrospinal fluid for neuronopathic forms of Gaucher disease.

    Directory of Open Access Journals (Sweden)

    Hila Zigdon

    Full Text Available Gaucher disease, a recessive inherited metabolic disorder caused by defects in the gene encoding glucosylceramidase (GlcCerase, can be divided into three subtypes according to the appearance of symptoms associated with central nervous system involvement. We now identify a protein, glycoprotein non-metastatic B (GPNMB, that acts as an authentic marker of brain pathology in neurological forms of Gaucher disease. Using three independent techniques, including quantitative global proteomic analysis of cerebrospinal fluid (CSF in samples from Gaucher disease patients that display neurological symptoms, we demonstrate a correlation between the severity of symptoms and GPNMB levels. Moreover, GPNMB levels in the CSF correlate with disease severity in a mouse model of Gaucher disease. GPNMB was also elevated in brain samples from patients with type 2 and 3 Gaucher disease. Our data suggest that GPNMB can be used as a marker to quantify neuropathology in Gaucher disease patients and as a marker of treatment efficacy once suitable treatments towards the neurological symptoms of Gaucher disease become available.

  10. Experimenting with International Collaborative Governance for Climate Change Mitigation by Private Actors: Scaling up Dutch Co-Regulation

    OpenAIRE

    TELESETSKY, Anastasia

    2011-01-01

    For the past two decades, international climate policy has been handled as a matter for State to State deliberation. Non-state actors have played at best marginal roles in making and implementing international policy. This paper argues that climate change remains an intractable transnational problem because State to State deliberations failed to acknowledge that both climate mitigation and adaptation require ongoing collaborative governance with non-State actors to shift normative behavior. T...

  11. International Students in Their Own Country: Motivation of Vietnamese Graduate Students to Attend a Collaborative Transnational University

    Science.gov (United States)

    Yao, Christina W.; Garcia, Crystal E.

    2018-01-01

    Higher education institutions in Vietnam have embraced opportunities to collaborate internationally to address specific educational needs that have emerged as a result of an accelerated economic and political society. The shift to a global market-driven economy has resulted in the need to produce better prepared graduates, advance in technology,…

  12. Economic evaluation of lupus nephritis in the Systemic Lupus International Collaborating Clinics inception cohort using a multistate model approach

    DEFF Research Database (Denmark)

    Barber, Megan R W; Hanly, John G; Su, Li

    2018-01-01

    OBJECTIVE: Little is known about the long-term costs of lupus nephritis (LN). These were compared between patients with and without LN based on multistate modelling. METHODS: Patients from 32 centres in 11 countries were enrolled in the Systemic Lupus International Collaborating Clinics (SLICC...

  13. The role of international collaboration in knowledge development in creation of TSO

    Energy Technology Data Exchange (ETDEWEB)

    Jackowski, Tomasz Marian; Skrzypek, Elena; Spirzewski, Michal [National Centre for Nuclear Research, Otwock (Poland)

    2014-12-15

    National Centre for Nuclear Research has been created on September the 1{sup st} 2011 by the decree of the Polish Government with a clear goal to form Technical Support Organization for Polish regulator and public administration. It was done by merging two institutes, the former Institute of Atomic Energy POLATOM and the former Andrzej Soltan Institute for Nuclear Studies, which worldwide reputation and successful research in various fields of nuclear power-related studies are well known. The fields of activity are widely focused on the nuclear physics, cosmology, electronics as well as detectors, accelerators, material research and many more. The main factor of National Center for Nuclear Research development was the participation in the European Structural Founds program ''Swierk Computing Centre'' from the beginning. In the frame of this particular program, the collaboration with the IAEA, NEA OECD, Euratom and research and TSO organizations from different countries began. The international collaboration plays the key role in the development of the Technical Support Organization expertise, which aim is to become the institution able to provide experts' support for decision-makers in nuclear power industry in Poland. Expertise and knowledge is expanded by the engagement in the code users international trainings, by being involved in various projects and benchmarks. The National Centre for Nuclear Research participates in, among the others, EURATOM projects such as NURESAFE (creation of BE Codes platform). Moreover NCBJ is involved in EURATOM FP7 projects such as NC2I-R (Cogeneration Initiative), ASAMPSAE (Advanced PSA), and ALLIANCE as part of the ALLEGRO project. In most of these groups our centre is taking active part in development as well as in management activities. Thanks to experiences gained and with work on research we are effectively expanding knowledge, experience and expertise to meet future's demands as a Technical

  14. The role of international collaboration in knowledge development in creation of TSO

    International Nuclear Information System (INIS)

    Jackowski, Tomasz Marian; Skrzypek, Elena; Spirzewski, Michal

    2014-01-01

    National Centre for Nuclear Research has been created on September the 1 st 2011 by the decree of the Polish Government with a clear goal to form Technical Support Organization for Polish regulator and public administration. It was done by merging two institutes, the former Institute of Atomic Energy POLATOM and the former Andrzej Soltan Institute for Nuclear Studies, which worldwide reputation and successful research in various fields of nuclear power-related studies are well known. The fields of activity are widely focused on the nuclear physics, cosmology, electronics as well as detectors, accelerators, material research and many more. The main factor of National Center for Nuclear Research development was the participation in the European Structural Founds program ''Swierk Computing Centre'' from the beginning. In the frame of this particular program, the collaboration with the IAEA, NEA OECD, Euratom and research and TSO organizations from different countries began. The international collaboration plays the key role in the development of the Technical Support Organization expertise, which aim is to become the institution able to provide experts' support for decision-makers in nuclear power industry in Poland. Expertise and knowledge is expanded by the engagement in the code users international trainings, by being involved in various projects and benchmarks. The National Centre for Nuclear Research participates in, among the others, EURATOM projects such as NURESAFE (creation of BE Codes platform). Moreover NCBJ is involved in EURATOM FP7 projects such as NC2I-R (Cogeneration Initiative), ASAMPSAE (Advanced PSA), and ALLIANCE as part of the ALLEGRO project. In most of these groups our centre is taking active part in development as well as in management activities. Thanks to experiences gained and with work on research we are effectively expanding knowledge, experience and expertise to meet future's demands as a Technical Support Organization for first Nuclear

  15. Academic performance and personal experience of local, international, and collaborative exchange students enrolled in an Australian pharmacy program.

    Science.gov (United States)

    Davey, Andrew K; Grant, Gary D; Anoopkumar-Dukie, Shailendra

    2013-09-12

    To assess the academic performance and experiences of local, international, and collaborative exchange students enrolled in a 4-year Australian bachelor of pharmacy degree program. Survey instruments exploring the demographics, background, and academic and cultural experiences of students during the program were administered in 2005 to students in all 4 years. Additionally, grades from each semester of the program for students (406 local, 70 international, 155 exchange) who graduated between 2002 and 2006 were analyzed retrospectively. The main differences found in the survey responses among the 3 groups were in students' motivations for choosing the degree program and school, with international and collaborative exchange students having put more thought into these decisions than local students. The average grades over the duration of the program were similar in all 3 demographic groups. However, local students slightly outperformed international students, particularly at the start of the year, whereas collaborative exchange students' grades mirrored those of local students during the 2 years prior to leaving their home country of Malaysia but more closely mirrored those of international students in the final 2 years after arriving on campus in Australia. Despite differences in academic backgrounds and culture, international and exchange students can perform well compared to local students in a bachelor of pharmacy program and were actually more satisfied than local students with the overall experience. Studying in a foreign country can negatively influence academic grades to a small extent and this is probably related to adjusting to the new environment.

  16. Mesenteric mass in a young girl - an unusual site for Gaucher's disease

    International Nuclear Information System (INIS)

    Lim, Adrian K.P.; Vellodi, Ashok; McHugh, Kieran

    2002-01-01

    We report the first case of a child with Gaucher's disease and a large mesenteric mass, confirmed histologically to be Gaucher's cell infiltrates. We describe the radiological findings and discuss further management. The advent of enzyme replacement therapy has prolonged survival and the emergence of previously undocumented manifestations of the disease is being observed. The radiologist and clinician should be alert to the possible development of these new problems and the fact that in Gaucher's disease a palpable right upper-quadrant mass need not necessarily represent hepatomegaly. (orig.)

  17. An Adult Form of Gaucher Disease Associated with Portal Hypertension: A Case

    Directory of Open Access Journals (Sweden)

    Ahmet Dulger

    2013-04-01

    Full Text Available Gaucher disease (GD is an inborn error of metabolism that affects the recycling of cellular glycolipids. Glucosylceramide (also called glucocerebroside accumulate within the lysosomes of cells. Gaucher%u2019s disease is most common lysosomal storage disease and its incidence is 1/75.000. Three types of this disease have been defined. During the course of disease, it was reported that hepatosplenomegaly, portal hypertension, hyperferritinemia, splenic infarcts and splenic nodules might develop. Therefore, as in our case; Gaucher%u2019s disease must be remembered in the setting of hepatosplenomegaly, portal hypertension, hyperferritinemia, splenic infarcts and splenic nodules of unknown etiology.

  18. Imaging features of skeletal changes in children with Gaucher disease

    International Nuclear Information System (INIS)

    Zhang Ningning; Duan Xiaomin; Duan Yanlong

    2011-01-01

    Objective: To discuss the imaging features of skeletal changes in children with Gaucher disease on X-ray and MRI images. Methods: One hundred and nine children with Gaucher disease were enrolled in this study. They all received routine X-ray for spine with anterior-posterior (A-P) and lateral view and bilateral femurs with A-P view. Among them, 18 patients received X-ray for pelvic with A-P view, 14 patients received X-ray for left wrist with A-P view, and 14 patients received MRI scan for femur. The MRI scan included T 1 -weighted imaging, T 2 -weighted imaging and fat-suppressed T 2 -weighted imaging with short tau inversion recovery (STIR) sequence. The imaging features of the X-ray and MRI images were analyzed retrospectively. Results: The most common feature is osteoporosis, which presented in 91 cases (83.5%). Besides this, decreased density of metaphysis occurred in 86 cases (78.9%), erlenmeyer flask deformity of metaphysis occurred in 89 patients (81.7%), thinner cortex occurred in 69 cases (63.3%), osteolytic destruction occurred in. 31 cases (28.4%), pathological fractures occurred in 26 cases (23.9%), osteosclerosis occurred in 12 cases (11.0%). cystic degeneration of bone occurred in 16 cases (14.7%), and dislocation of the hip occurred in 4 cases. All 14 patients received MRI presented abnormal signals. Among them, 4 patients presented low signal intensity both on T 1 -weighted and T 2 -weighted images in bone marrow, the other ten presented high signal intensity mixed in low signal intensity areas on T 2 - weighted and fat-suppressed T 2 -weighted images. Conclusions: The imaging features of skeletal changes in children with Gaucher disease are of some characteristics, which could provide useful information for the clinical treatment. (authors)

  19. Achievement of Therapeutic Goals with Low-Dose Imiglucerase in Gaucher Disease: A Single-Center Experience

    Directory of Open Access Journals (Sweden)

    Irina Tukan

    2013-01-01

    Full Text Available Gaucher disease, a lysosomal storage disorder, is a multisystem disorder with variable and unpredictable onset and severity. Disease-specific enzyme replacement therapy (ERT has been shown to reverse or ameliorate disease-specific hepatosplenomegaly and anemia and thrombocytopenia. ERT also impacts bone manifestations, including bone crises, bone pain, and appearance of new osteonecrosis, and improves bone mineral density to varying degrees. The objective of this study was to assess achievement of predefined therapeutic goals based on international registry outcomes for Israeli patients with Gaucher disease receiving imiglucerase for four consecutive years on a low-dose regimen followed in a single center. All data were taken from patient files. The therapeutic goals were taken from standards published in the literature for disease-specific clinical parameters. Among 164 patients at baseline, values for spleen and liver volumes, hemoglobin and platelet counts, and Z-scores for lumbar spine and femoral were significantly different from the goal. After four years ERT, there was a significant improvement ( in each of the therapeutic goal parameters from baseline. 15.2% of these patients achieved all hematology-visceral goals. In children, there was achievement of linear growth and puberty. This survey highlights the good overall response in symptomatic patients receiving low-dose ERT with imiglucerase in Israel.

  20. Collaboration between specialties for respiratory allergies in the International Classification of Diseases (ICD)-11.

    Science.gov (United States)

    Tanno, Luciana Kase; Calderon, Moises; Linzer, Jeffrey F; Chalmers, Robert J G; Demoly, Pascal

    2017-02-10

    The International Classification of Diseases (ICD) has been grouping the allergic and hypersensitivity disorders involving the respiratory tract under topographic distribution, regardless of the underlying mechanisms, triggers or concepts currently in use for allergic and hypersensitivity conditions. In order to strengthen awareness and deliberate the creation of the new "Allergic or hypersensitivity disorders involving the respiratory tract" section of the ICD-11, we here propose make the building process public. The new frame has been constructed to cover the gaps previously identified and was based on consensus academic reports and ICD-11 principles. Constant and bilateral discussion was kept with relevant groups representing specialties and resulted in proposals submission into the ICD-11 online platform. The "Allergic or hypersensitivity disorders involving the respiratory tract" section covers 64 entities distributed across five main categories. All the 79 proposals submitted resulted from an intensive collaboration of the Allergy working group, relevant Expert working groups and the WHO ICD governance. The establishment of the ICD-11 "Allergic or hypersensitivity disorders involving the respiratory tract" section will allow the dissemination of the updated concepts to be used in clinical practice by many different specialties and health professionals.

  1. The IceCube Collaboration: contributions to the 30th International Cosmic Ray Conference (ICRC 2007)

    International Nuclear Information System (INIS)

    IceCube Collaboration; Ackermann, M.

    2007-01-01

    This paper bundles 40 contributions by the IceCube collaboration that were submitted to the 30th International Cosmic Ray Conference ICRC 2007. The articles cover studies on cosmic rays and atmospheric neutrinos, searches for non-localized, extraterrestrial ν e , ν μ and ν τ signals, scans for steady and intermittent neutrino point sources, searches for dark matter candidates, magnetic monopoles and other exotic particles, improvements in analysis techniques, as well as future detector extensions. The IceCube observatory will be finalized in 2011 to form a cubic-kilometer ice-Cherenkov detector at the location of the geographic South Pole. At the present state of construction, IceCube consists of 52 paired IceTop surface tanks and 22 IceCube strings with a total of 1426 Digital Optical Modules deployed at depths up to 2350 m. The observatory also integrates the 19 string AMANDA subdetector, that was completed in 2000 and extends IceCube's reach to lower energies. Before the deployment of IceTop, cosmic air showers were registered with the 30 station SPASE-2 surface array. IceCube's low noise Digital Optical Modules are very reliable, show a uniform response and record waveforms of arriving photons that are resolvable with nanosecond precision over a large dynamic range. Data acquisition, reconstruction and simulation software are running in production mode and the analyses, profiting from the improved data quality and increased overall sensitivity, are well under way

  2. The Cognitive Behavioral Assessment (CBA Project: Presentation and Proposal for International Collaboration

    Directory of Open Access Journals (Sweden)

    Ezio Sanavio

    2013-11-01

    Full Text Available Aims: The main aim of this paper is to describe almost 30 years of work on psychological assessment using CBA, a research team, and to propose collaboration with Latin countries. Methods: The acronym CBA stands for Cognitive Behavioural Assessment and indicates both an overall approach to clinical assessment and a series of tests. Five general principles formed the basis on which the team developed their questionnaires: (1 assessment is not a passive collection of information, but an active process similar to problem-solving; (2 horizontal integration of questionnaires with other assessment methods; (3 vertical integration and hierarchical structure of assessment questionnaires; (4 idiographic perspective; (5 computer support. Results: The paper briefly presents the most important tests: CBA-2.0, a broad-spectrum Battery for patients who need counselling and/or psychotherapy; CBA-H (Hospital for both in-patients and out-patients suffering from physical illnesses; CBA-SPORT for professional athletes; CBA-Y (young people for adolescents and young adults; CBD-VE (treatment benefits to assess the effectiveness of psychological treatment. Conclusion: These questionnaires have produced over 100 research works, published in Italian journals or presented in conferences. In the near future, we expect important, radical changes and hope to create an international research milieu.

  3. CERN’s model for international scientific collaboration to be discussed at UNOG

    CERN Multimedia

    Antonella Del Rosso

    2015-01-01

    On 2 November, on the occasion of the 70th anniversary of the United Nations, CERN and UNOG will co-host a one-day symposium, with the support of Switzerland and France. The event will bring together policy-makers, scientists and members of civil society to debate how to construct synergies across communities as a means to drive global objectives. CERN people are invited to the Palais des Nations to take part.   CERN's seat at the General Assembly of the United Nations in New York. How does CERN work? How are goals achieved in such a complex environment where diverse communities work together in the interests of science? CERN’s model for international scientific collaboration is being looked at with growing interest by an increasingly large community of experts in various fields. Scientific advances and accomplishments are testament to the effectiveness of the model and prove that ambitious scientific programmes can be carried out only by communities c...

  4. [Development of a French-language online health policy course: an international collaboration].

    Science.gov (United States)

    Hébert, Réjean; Coppieters, Yves; Pradier, Christian; Williams-Jones, Bryn; Brahimi, Cora; Farley, Céline

    2017-01-01

    To present the process and challenges of developing an online competency-based course on public health policy using a collaborative international approach. Five public health experts, supported by an expert in educational technology, adopted a rigorous approach to the development of the course: a needs analysis, identification of objectives and competencies, development of a pedagogical scenario for each module and target, choice of teaching methods and learning activities, material to be identified or developed, and the responsibilities and tasks involved. The 2-credit (90-hour) graduate course consists of six modules including an integration module. The modules start with a variety of case studies: tobacco law (neutral packaging), supervised injection sites, housing, integrated services for the frail elderly, a prevention programme for mothers from disadvantaged backgrounds, and the obligatory use of bicycle helmets. In modules 1, 3, 4 and 5, students learn about different stages of the public policy development process: emergence, formulation and adoption, implementation and evaluation. Module 2 focuses on the importance of values and ideologies in public policy. The integration module allows the students to apply the knowledge learned and addresses the role of experts in public policy and ethical considerations. The course has been integrated into the graduate programmes of the participating universities and allows students to follow, at a distance, an innovative training programme.

  5. Unifying a fragmented effort: a qualitative framework for improving international surgical teaching collaborations.

    Science.gov (United States)

    Fallah, Parisa Nicole; Bernstein, Mark

    2017-09-07

    Access to adequate surgical care is limited globally, particularly in low- and middle-income countries (LMICs). To address this issue, surgeons are becoming increasingly involved in international surgical teaching collaborations (ISTCs), which include educational partnerships between surgical teams in high-income countries and those in LMICs. The purpose of this study is to determine a framework for unifying, systematizing, and improving the quality of ISTCs so that they can better address the global surgical need. A convenience sample of 68 surgeons, anesthesiologists, physicians, residents, nurses, academics, and administrators from the U.S., Canada, and Norway was used for the study. Participants all had some involvement in ISTCs and came from multiple specialties and institutions. Qualitative methodology was used, and participants were interviewed using a pre-determined set of open-ended questions. Data was gathered over two months either in-person, over the phone, or on Skype. Data was evaluated using thematic content analysis. To organize and systematize ISTCs, participants reported a need for a centralized/systematized process with designated leaders, a universal data bank of current efforts/progress, communication amongst involved parties, full-time administrative staff, dedicated funds, a scholarly approach, increased use of technology, and more research on needs and outcomes. By taking steps towards unifying and systematizing ISTCs, the quality of ISTCs can be improved. This could lead to an advancement in efforts to increase access to surgical care worldwide.

  6. The Tourette International Collaborative Genetics (TIC Genetics) study, finding the genes causing Tourette syndrome

    DEFF Research Database (Denmark)

    Dietrich, Andrea; Fernandez, Thomas V; King, Robert A

    2015-01-01

    Tourette syndrome (TS) is a neuropsychiatric disorder characterized by recurrent motor and vocal tics, often accompanied by obsessive-compulsive disorder and/or attention-deficit/hyperactivity disorder. While the evidence for a genetic contribution is strong, its exact nature has yet to be clarif......Tourette syndrome (TS) is a neuropsychiatric disorder characterized by recurrent motor and vocal tics, often accompanied by obsessive-compulsive disorder and/or attention-deficit/hyperactivity disorder. While the evidence for a genetic contribution is strong, its exact nature has yet......, it is clear that large patient cohorts and open-access repositories will be essential to further advance the field. To that end, the large multicenter Tourette International Collaborative Genetics (TIC Genetics) study was established. The goal of the TIC Genetics study is to undertake a comprehensive gene...... discovery effort, focusing both on familial genetic variants with large effects within multiply affected pedigrees and on de novo mutations ascertained through the analysis of apparently simplex parent-child trios with non-familial tics. The clinical data and biomaterials (DNA, transformed cell lines, RNA...

  7. Prevalence of autoantibodies in the course of Gaucher disease type 1: A multicenter study comparing Gaucher disease patients to healthy subjects.

    Science.gov (United States)

    Serratrice, Christine; Bensalah, Nesma; Penaranda, Guillaume; Bardin, Nathalie; Belmatoug, Nadia; Masseau, Agathe; Rose, Christian; Lidove, Olivier; Camou, Fabrice; Maillot, François; Leguy, Vanessa; Magy-Bertrand, Nadine; Marie, Isabelle; Cherin, Patrick; Bengherbia, Monia; Carballo, Sebastian; Boucraut, José; Serratrice, Jacques; Berger, Marc; Verrot, Denis

    2018-01-01

    Type 1 Gaucher disease may be related to the presence of autoantibodies. Their clinical significance is questioned. Primary endpoint was to compare the prevalence of autoantibodies in type 1 Gaucher disease patients with healthy subjects, seeking correlations with autoimmune characteristics. Secondary endpoints were to determine whether patients with autoantibodies reported autoimmunity-related symptoms and if genotype, splenectomy or treatment influenced autoantibodies presence. Type 1 Gaucher disease patients and healthy volunteers were included in this national multicenter exploratory study. Autoantibodies presence was compared in both groups and assessed regarding to genotype, splenectomy, Gaucher disease treatment and autoimmunity-related symptoms. Twenty healthy subjects and 40 type 1 Gaucher disease patients were included. Of the studied group: 15 patients undergone splenectomy, 37 were treated either with enzyme replacement therapy (34) or with substrate reduction therapy (3), 25 were homozygous/heterozygous for the N370S mutation. In type 1 Gaucher disease group (studied group), 52% had positive autoantibodies versus 26% in control group. Antiphospholipid antibodies were more frequent in the studied group (30% vs. 5%), but without correlation to thrombosis, osteonecrosis or bone infarcts. In the studied group, antinuclear antibodies were more frequent (25% vs. 16%). None of the patients with autoantibodies had clinical manifestations of autoimmune diseases. Autoantibodies were not correlated with treatment, genotype, or splenectomy, except for anticardiolipid, more frequent in splenectomized patients. In type 1 Gaucher disease, autoantibodies were more frequent compared to a healthy population. However, they were not associated with an increased prevalence of clinical active autoimmune diseases. Copyright © 2016 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  8. Case report 387: Gaucher disease affecting the skeleton (left femur)

    International Nuclear Information System (INIS)

    Tabas, J.H.; Daffner, R.H.; Hartsock, R.J.; Blakley, J.B.

    1986-01-01

    A case is described of a non-Jewish (Italian) 49-year-old man who presented to the hospital with pain in the left hip. Radionuclide studies showed decreased tracer activity with 99m Tc MDP over a lytic area in the subtrochanteric region of the left femur. Increased activity, however, was present in the right temporal bone, low anterior rib cage and right tenth posterior rib. The presence of subendosteal sclerosis with some cortical thickening adjacent to the femoral lesion, suggested the possibility of malignant neoplasm, (e.g. chondrosarcoma). Biopsy of the bone marrow showed the presence of Gaucher disease. (orig./SHA)

  9. Case report 387: Gaucher disease affecting the skeleton (left femur)

    Energy Technology Data Exchange (ETDEWEB)

    Tabas, J.H.; Daffner, R.H.; Hartsock, R.J.; Blakley, J.B.

    1986-08-01

    A case is described of a non-Jewish (Italian) 49-year-old man who presented to the hospital with pain in the left hip. Radionuclide studies showed decreased tracer activity with /sup 99m/Tc MDP over a lytic area in the subtrochanteric region of the left femur. Increased activity, however, was present in the right temporal bone, low anterior rib cage and right tenth posterior rib. The presence of subendosteal sclerosis with some cortical thickening adjacent to the femoral lesion, suggested the possibility of malignant neoplasm, (e.g. chondrosarcoma). Biopsy of the bone marrow showed the presence of Gaucher disease. (orig./SHA).

  10. Increasing Regional Anesthesia Use in a Serbian Teaching Hospital through an International Collaboration

    Directory of Open Access Journals (Sweden)

    Curtis L. Baysinger

    2017-06-01

    Full Text Available Many low- and middle-income countries (LMICs report low rates of regional anesthesia (RA use for cesarean delivery (CD, despite its association with lower maternal major morbidity and mortality. Also, the prevalence of neuraxial analgesia for labor (NAL is often low in LMICs. We report on the results of a collaboration in clinical education over a multi-year period between Kybele Inc., an international non-profit organization, and Klinicki Centar Vojvodine (CCV, a teaching hospital in Novi Sad, Serbia, to increase RA use for CD and NAL at CCV. From late 2011 through 2015, teams from Kybele participated in annual to biannual didactic conferences and week-long bedside teaching efforts involving obstetric and anesthesia staff from CCV and surrounding hospitals. Ongoing contact occurred at least weekly between Kybele and the host to discuss progress. De-identified quality improvement data on total deliveries, numbers of elective and non-elective CDs, number of vaginal deliveries, type of anesthesia for CD, and the number of NALs were collected. RA use for CD increased to 25% in year 2015 versus 14% in base year 2011 [odds ratio (OR: 2.05; 95% confidence interval (CI: 1.73,2.42; p < 0.001]. NAL increased to 10.5% of laboring women in 2015 versus 1.2% in 2011 (OR: 9.6; 95% CI: 7.2, 12.8; p < 0.001. Greater increases for RA use during non-elective CD were observed between 2011 and 2015 (1.4 versus 7.5% of total CD; OR: 5.52; 95% CI: 2.63, 8.41; p < 0.001 relative to elective CD (12.5 versus 17.5% of total CD; OR: 1.48; 95% CI: 1.23, 1.77; p < 0.001. Overall, RA for CD increased during the 4 year collaboration but was not as great as reported in other countries with similar health-care demographics utilizing a similar program. Detailed descriptions of program interventions and barriers to change at CCV are presented.

  11. Increasing Regional Anesthesia Use in a Serbian Teaching Hospital through an International Collaboration.

    Science.gov (United States)

    Baysinger, Curtis L; Pujic, Borislava; Velickovic, Ivan; Owen, Medge D; Serafin, Joanna; Shotwell, Matthew S; Braveman, Ferne

    2017-01-01

    Many low- and middle-income countries (LMICs) report low rates of regional anesthesia (RA) use for cesarean delivery (CD), despite its association with lower maternal major morbidity and mortality. Also, the prevalence of neuraxial analgesia for labor (NAL) is often low in LMICs. We report on the results of a collaboration in clinical education over a multi-year period between Kybele Inc., an international non-profit organization, and Klinicki Centar Vojvodine (CCV), a teaching hospital in Novi Sad, Serbia, to increase RA use for CD and NAL at CCV. From late 2011 through 2015, teams from Kybele participated in annual to biannual didactic conferences and week-long bedside teaching efforts involving obstetric and anesthesia staff from CCV and surrounding hospitals. Ongoing contact occurred at least weekly between Kybele and the host to discuss progress. De-identified quality improvement data on total deliveries, numbers of elective and non-elective CDs, number of vaginal deliveries, type of anesthesia for CD, and the number of NALs were collected. RA use for CD increased to 25% in year 2015 versus 14% in base year 2011 [odds ratio (OR): 2.05; 95% confidence interval (CI): 1.73,2.42; p  < 0.001]. NAL increased to 10.5% of laboring women in 2015 versus 1.2% in 2011 (OR: 9.6; 95% CI: 7.2, 12.8; p  < 0.001). Greater increases for RA use during non-elective CD were observed between 2011 and 2015 (1.4 versus 7.5% of total CD; OR: 5.52; 95% CI: 2.63, 8.41; p  < 0.001) relative to elective CD (12.5 versus 17.5% of total CD; OR: 1.48; 95% CI: 1.23, 1.77; p  < 0.001). Overall, RA for CD increased during the 4 year collaboration but was not as great as reported in other countries with similar health-care demographics utilizing a similar program. Detailed descriptions of program interventions and barriers to change at CCV are presented.

  12. The ESWN network as a platform to increase international collaboration between women in the Earth Sciences

    Science.gov (United States)

    Braker, Gesche; Wang, Yiming; Glessmer, Mirjam; Kirchgaessner, Amelie

    2014-05-01

    The Earth Science Women's Network (ESWN; ESWNonline.org) is an international peer-mentoring network of women in the Earth Sciences, many in the early stages of their careers. ESWN's mission is to promote career development, build community, provide opportunities for informal mentoring and support, and facilitate professional collaborations. This has been accomplished via email and a listserv, on Facebook, at in-person networking events, and at professional development workshops. In an effort to facilitate international connections among women in the Earth Sciences, ESWN has developed a password protected community webpage supported by AGU and a National Science Foundation ADVANCE grant where members can create an online presence and interact with each other. For example, groups help women to connect with co-workers or center around a vast array of topics ranging from research interests, funding opportunities, work-life balance, teaching, scientific methods, and searching for a job to specific challenges faced by women in the earth sciences. Members can search past discussions and share documents like examples of research statements, useful interview materials, or model recommendation letters. Over the last 10 years, ESWN has grown by word of mouth to include more than 1600 members working on all 7 continents. ESWN also offers professional development workshops at major geologic conferences around the world and at ESWN-hosted workshops mostly exclusively throughout the United States. In 2014, ESWN offers a two day international workshop on communication and networking skills and career development. Women working in all disciplines of Earth Sciences from later PhD level up to junior professors in Europe are invited to the workshop that will be held in Kiel, Germany. The workshop offers participants an individual personality assessment and aims at providing participants with improved communication and networking skills. The second focus will be to teach them how to

  13. Collaboration under the International Partnership for the Hydrogen Economy (IPHE) and the Carbon Sequestration Leadership Forum (CSLF)

    Energy Technology Data Exchange (ETDEWEB)

    Neff, H.J. [Forschungszentrum Juelich (Germany)

    2005-06-01

    The objectives and achievements of the International Partnership for the Hydrogen Economy (IPHE) and the Carbon Sequestration Leadership Forum (CSLF) will be described. Both are agreements between governments and aim at identifying and promoting potential areas of bilateral and multilateral collaboration on new and advanced energy technologies. The IPHE has analysed priorities for international collaboration in research, development, demonstration and utilisation of hydrogen equipment in five areas: hydrogen production, fuel cells, hydrogen storage, codes and standards, socio-economic research. A report on such options is available and a series of IPHE conferences and workshops will pave the way to concrete collaboration projects. The CSLF is focused on development of improved cost-effective technologies for the cost-efficient capture and safe, long-term storage of carbon dioxide (CO{sub 2}) for fossil power plants. The mission of the CSLF is to facilitate the development and deployment of such technologies via collaborative efforts that address key technical issues, as well as economic, and environmental challenges. The CSLF also promotes awareness and champion legal, regulatory, financial, and institutional environments conducive to such technologies. The CSLF has worked out a Technology Roadmap as a guide for the CSLF and its Members that describes possible routes to future CO2 capture, transport and storage needs. Included are modules on the current status of these technologies, ongoing activities in CO{sub 2} capture, transport and storage, and identification of technology gaps and non-technology needs that should be addressed over the next decade. The Technology Roadmap indicates areas where the CSLF can add value through international collaborative effort. Both, hydrogen technologies and CO2 sequestration, are closely connected and will serve an overall strategic framework with clean fossil fuels as a key element of a sustainable energy portfolio

  14. Disposal R&D in the Used Fuel Disposition Campaign: A Discussion of Opportunities for Active International Collaboration

    Energy Technology Data Exchange (ETDEWEB)

    Birkholzer, J.T.

    2011-06-01

    For DOE's Used Fuel Disposition Campaign (UFDC), international collaboration is a beneficial and cost-effective strategy for advancing disposal science with regards to multiple disposal options and different geologic environments. While the United States disposal program focused solely on Yucca Mountain tuff as host rock over the past decades, several international programs have made significant progress in the characterization and performance evaluation of other geologic repository options, most of which are very different from the Yucca Mountain site in design and host rock characteristics. Because Yucca Mountain was so unique (e.g., no backfill, unsaturated densely fractured tuff), areas of direct collaboration with international disposal programs were quite limited during that time. The decision by the U.S. Department of Energy to no longer pursue the disposal of high-level radioactive waste and spent fuel at Yucca Mountain has shifted UFDC's interest to disposal options and geologic environments similar to those being investigated by disposal programs in other nations. Much can be gained by close collaboration with these programs, including access to valuable experience and data collected over recent decades. Such collaboration can help to efficiently achieve UFDC's long-term goals of conducting 'experiments to fill data needs and confirm advanced modeling approaches' (by 2015) and of having a 'robust modeling and experimental basis for evaluation of multiple disposal system options' (by 2020). This report discusses selected opportunities of active international collaboration, with focus on both Natural Barrier System (NBS) and Engineered Barrier System (EBS) aspects and those opportunities that provide access to field data (and respective interpretation/modeling) or allow participation in ongoing field experiments. This discussion serves as a basis for the DOE/NE-53 and UFDC planning process for FY12 and beyond.

  15. Disposal R and D in the Used Fuel Disposition Campaign: A Discussion of Opportunities for Active International Collaboration

    International Nuclear Information System (INIS)

    Birkholzer, J.T.

    2011-01-01

    For DOE's Used Fuel Disposition Campaign (UFDC), international collaboration is a beneficial and cost-effective strategy for advancing disposal science with regards to multiple disposal options and different geologic environments. While the United States disposal program focused solely on Yucca Mountain tuff as host rock over the past decades, several international programs have made significant progress in the characterization and performance evaluation of other geologic repository options, most of which are very different from the Yucca Mountain site in design and host rock characteristics. Because Yucca Mountain was so unique (e.g., no backfill, unsaturated densely fractured tuff), areas of direct collaboration with international disposal programs were quite limited during that time. The decision by the U.S. Department of Energy to no longer pursue the disposal of high-level radioactive waste and spent fuel at Yucca Mountain has shifted UFDC's interest to disposal options and geologic environments similar to those being investigated by disposal programs in other nations. Much can be gained by close collaboration with these programs, including access to valuable experience and data collected over recent decades. Such collaboration can help to efficiently achieve UFDC's long-term goals of conducting 'experiments to fill data needs and confirm advanced modeling approaches' (by 2015) and of having a 'robust modeling and experimental basis for evaluation of multiple disposal system options' (by 2020). This report discusses selected opportunities of active international collaboration, with focus on both Natural Barrier System (NBS) and Engineered Barrier System (EBS) aspects and those opportunities that provide access to field data (and respective interpretation/modeling) or allow participation in ongoing field experiments. This discussion serves as a basis for the DOE/NE-53 and UFDC planning process for FY12 and beyond.

  16. The absence of later wave components in auditory brainstem responses as an initial manifestation of type 2 Gaucher disease.

    Science.gov (United States)

    Okubo, Yusuke; Goto, Masahiro; Sakakibara, Hiroshi; Terakawa, Toshiro; Kaneko, Takashi; Miyama, Sahoko

    2014-12-01

    Type 2 Gaucher disease is the most severe neuronopathic form of Gaucher disease and is characterized by severe neurodegeneration with brainstem involvement and organ failure. Prediction or diagnosis of type 2 Gaucher disease before the development of neurological complications is difficult. A 5-month-old female infant presented with deafness without other neurological abnormalities. Auditory brainstem response analysis revealed the absence of later wave components. Two months later, muscular rigidity became evident, followed by the development of opisthotonus and strabismus. Rapid progression of splenomegaly led to the diagnosis of type 2 Gaucher disease. Abnormal auditory brainstem response findings may already exist before the development of severe brainstem abnormalities such as muscular rigidity and opisthotonus in type 2 Gaucher disease. When patients present with deafness and absent later wave components on auditory brainstem response, type 2 Gaucher disease should be included in the differential diagnosis even in the absence of other neurological abnormalities. Copyright © 2014 Elsevier Inc. All rights reserved.

  17. Manifestações esqueléticas da doença de gaucher Skeletal manifestations in Gaucher's disease

    Directory of Open Access Journals (Sweden)

    Vinicius França de Mendonça

    2001-06-01

    Full Text Available A doença de Gaucher é manifestação genética causada pela deficiência da enzima glicocerebrosidase, resultando no acúmulo secundário de glicocerebrosídeos nos órgãos do sistema reticuloendotelial. Apresenta-se sob três formas clínicas distintas, podendo ser rapidamente fatal ou crônica com poucos sintomas. O presente trabalho tem o objetivo de analisar os achados da radiografia simples do esqueleto em 32 pacientes comprovadamente portadores da doença, de ambos os sexos e em diferentes faixas etárias. Foram observadas as seguintes alterações: osteopenia difusa em todos os casos, deformidade em "frasco de Erlenmeyer" em 93,7%, alterações articulares em 40,6%, necrose da cabeça do fêmur e lesões líticas em 28,1%, respectivamente, fratura patológica em 9,3% e necrose da cabeça do úmero em 6,2% dos casos. Estes resultados encontram-se de acordo com as descrições da literatura, demonstrando a importância da radiologia convencional como método complementar no diagnóstico desta enfermidade.Gaucher's disease has a genetic background and is characterized by the deficiency of enzyme glucocerebrosidase, resulting in secondary accumulation of glucocerebrosides in the reticuloendothelial organs. The objective of the present study is to evaluate the x-ray findings in the skeleton of a group of 32 male and female patients of different ages, with biochemical diagnosis of Gaucher's disease. The following bone lesions were observed: diffuse osteopenia (100% of the patients, "Erlenmeyer flask" deformities (93.7% of the patients, abnormalities of the joints (40.6% of the patients, necrosis of the femoral head (28.1% of the patients, lytic lesions (28.1% of the patients, pathological fractures (9.3% of the patients and necrosis of the humeral head (6.2% of the patients. These results are concordant with the literature, and demonstrate the importance of conventional x-ray as a complementary method in the diagnosis of Gaucher's disease.

  18. Internal and External Regulation to Support Knowledge Construction and Convergence in Computer Supported Collaborative Learning (CSCL)

    Science.gov (United States)

    Romero, Margarida; Lambropoulos, Niki

    2011-01-01

    Computer Supported Collaborative Learning (CSCL) activities aim to promote collaborative knowledge construction and convergence. During the CSCL activity, the students should regulate their learning activity, at the individual and collective level. This implies an organisation cost related to the coordination of the activity with the team-mates…

  19. Discursive Construction of Social Presence and Identity Positions in an International Bilingual Collaboration

    Science.gov (United States)

    Walker, Ute

    2017-01-01

    This article examines the discursive construction of social presence and identity in a bilingual collaboration between tertiary distance learners of German in New Zealand and Academic English students in Germany. Drawing on positioning theory, this small-scale study investigated the collaborative practices of a group of students, whose synchronous…

  20. Glucocerebrosidase genotype of Gaucher patients in The Netherlands: limitations in prognostic value

    NARCIS (Netherlands)

    Boot, R. G.; Hollak, C. E.; Verhoek, M.; Sloof, P.; Poorthuis, B. J.; Kleijer, W. J.; Wevers, R. A.; van Oers, M. H.; Mannens, M. M.; Aerts, J. M.; van Weely, S.

    1997-01-01

    Gaucher disease is a recessively inherited lysosomal storage disorder that is caused by a deficiency in glucocerebrosidase activity. The clinical expression is markedly heterogeneous with respect to age of onset, progression, severity, and neurological involvement. The relative incidence of

  1. [Progressive pulmonary hypertension in a patient with type 1 Gaucher disease].

    Science.gov (United States)

    Ponomarev, R V; Model, S V; Averbukh, O M; Gavrilov, A M; Galstyan, G M; Lukina, E A

    Gaucher disease is the most common form of hereditary enzymopathies combined into a group of lysosomal storage diseases. The basis for the disease is a hereditary deficiency of the activity of acid β-glucosidase, a lysosomal enzyme involved in the catabolism of lipids, which results in the accumulation of nonutilized cellular metabolism products in the macrophage lysosomes. The main clinical manifestations of type 1 Gaucher disease are cytopenia, hepatomegaly, and splenomegaly, and bone lesion. One of the atypical clinical manifestations of Gaucher disease is damage to the lungs with the development of pulmonary hypertension, which is usually considered within the underlying disease - the development of pneumosclerosis due to macrophage dysfunction. The paper describes a case of progressive pulmonary hypertension in a patient with type 1 Gaucher disease.

  2. Four Gaucher disease type II patients with three novel mutations: a single centre experience from Turkey.

    Science.gov (United States)

    Bulut, Fatma Derya; Kör, Deniz; Şeker-Yılmaz, Berna; Hergüner, Özlem; Ceylaner, Serdar; Özkınay, Ferda; Kılavuz, Sebile; Önenli-Mungan, Neslihan

    2018-04-14

    Gaucher disease is the most common lysosomal storage disorder due to glucosylceramidase enzyme deficiency. There are three subtypes of the disease. Neurological involvement accompanies visceral and haematological findings only in type II and type III Gaucher patients. Type II is the acute progressive neuronopathic form which is the most severe and rare subtype. Clinical findings are recognized prenatally or in the first months of life and followed by death within the first two years of age. Among our 81 Gaucher patients, we identified 4 (4,9%) type II patients in our metabolic centre. This rate is significantly higher than the rate reported in the literature (Gaucher patients with three novel mutations and one perinatal lethal form with generalized ichthyosis which is a very rare disorder. Additionally, we would like to highlight the phenotypic heterogeneity not only between the subtypes, also even in the same type.

  3. Impaired IL-10 transcription and release in animal models of Gaucher disease macrophages.

    Science.gov (United States)

    Kacher, Yaacov; Futerman, Anthony H

    2009-01-01

    A number of studies have shown altered cytokine levels in serum from Gaucher disease patients, including changes in levels of the anti-inflammatory cytokine, interleukin-10 (IL-10). However, the source of IL-10, or the mechanisms leading to changes in IL-10 serum levels are not known. We now show that mouse macrophages treated with an active site-directed inhibitor of glucocerebrosidase, or macrophages from a mouse model of Gaucher disease, the L444P mouse, release significantly less IL-10 than their untreated counterparts, but that TNFalpha release is unaffected. These changes are due to reduced transcription of IL-10 mRNA in macrophages. The reduction in IL-10 secretion observed in animal models of Gaucher disease macrophages may be of relevance to explain the increase in inflammation that is often observed in Gaucher disease.

  4. Clinical and genetic assessment of pediatric patients with Gaucherâ ...

    African Journals Online (AJOL)

    Tahia H. Saleem

    2016-09-24

    Sep 24, 2016 ... were squint, seizures and delayed mental development. Five different .... or miscarriages or genetically affected siblings with GD was also included. ..... with Gaucher disease and relation to disease phenotypes. World J.

  5. The biology of the Gaucher cell: the cradle of human chitinases

    NARCIS (Netherlands)

    Bussink, Anton P.; van Eijk, Marco; Renkema, G. Herma; Aerts, Johannes M.; Boot, Rolf G.

    2006-01-01

    Gaucher disease (GD) is the most common lysosomal storage disorder and is caused by inherited deficiencies of glucocerebrosidase, the enzyme responsible for the lysosomal breakdown of the lipid glucosylceramide. GD is characterized by the accumulation of pathological, lipid laden macrophages,

  6. Collaborative international research: ethical and regulatory issues pertaining to human biological materials at a South African institutional research ethics committee.

    Science.gov (United States)

    Sathar, Aslam; Dhai, Amaboo; van der Linde, Stephan

    2014-12-01

    Human Biological Materials (HBMs) are an invaluable resource in biomedical research. To determine if researchers and a Research Ethics Committee (REC) at a South African institution addressed ethical issues pertaining to HBMs in collaborative research with developed countries. Ethically approved retrospective cross-sectional descriptive audit. Of the 1305 protocols audited, 151 (11.57%) fulfilled the study's inclusion criteria. Compared to other developed countries, a majority of sponsors (90) were from the USA (p = 0.0001). The principle investigators (PIs) in all 151 protocols informed the REC of their intent to store HBMs. Only 132 protocols informed research participants (P ethical and regulatory issues pertaining to HBMs. There was a lack of congruence between the ethical guidelines of developed countries and their actions which are central to the access to HBMs in collaborative research. HBMs may be leaving South Africa without EPs and MTAs during the process of international collaborative research. © 2013 John Wiley & Sons Ltd.

  7. Reduced cerebral vascularization in experimental neuronopathic Gaucher disease.

    Science.gov (United States)

    Smith, Nicholas Jc; Fuller, Maria; Saville, Jennifer T; Cox, Timothy M

    2018-01-01

    The glycosphingolipidosis, Gaucher disease, in which a range of neurological manifestations occur, results from a deficiency of acid β-glucocerebrosidase, with subsequent accumulation of β-glucocerebroside, its upstream substrates, and the non-acylated congener β-glucosylsphingosine. However, the mechanisms by which end-organ dysfunction arise are poorly understood. Here, we report strikingly diminished cerebral microvascular density in a murine model of disease, and provide a detailed analysis of the accompanying cerebral glycosphingolipidome in these animals, with marked elevations of β-glucosylsphingosine. Further in vitro studies confirmed a concentration-dependent impairment of endothelial cytokinesis upon exposure to quasi-pathological concentrations of β-glucosylsphingosine. These findings support a premise for pathogenic disruption of cerebral angiogenesis as an end-organ effect, with potential for therapeutic modulation in neuronopathic Gaucher disease. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  8. Splenomegaly, Cardiomegaly, and Osteoporosis in a Child with Gaucher Disease

    Directory of Open Access Journals (Sweden)

    J. J. Sheth

    2011-01-01

    Full Text Available A 15-month-old girl, born to the consanguineous parents, was referred with the sign of massive splenomegaly associated with thrombocytopenia and anemia. Plasma Chitotriosidase estimation was carried out as a screening test and was found to be normal with reduced activity of β-glucosidase in leucocytes suggestive of Gaucher disease. At the age of 4 years, severe osteoporosis and cardiomegaly with pulmonary congestion were observed in the child. Molecular analysis for GBA gene has revealed homozygous status for L444P (c.1448C in the proband, whereas parents and two elder sisters were found to be heterozygote. Prenatal study during the fourth pregnancy was carried out from cultured chorionic villi for β-glucosidase, which was in the carrier range. Further confirmation of the carrier status was carried out from amniotic fluid DNA and was found to be heterozygous for L444P (c.1448C in the GBA gene. This case demonstrates that children with the sign of splenomegaly with anemia and thrombocytopenia need to be screened for Gaucher disease, and molecular study can further help to confirm the heterozygous status, where prenatal study by enzyme investigation demonstrate heterozygous condition.

  9. Hampered Vitamin B12 Metabolism in Gaucher Disease?

    Directory of Open Access Journals (Sweden)

    Luciana Hannibal PhD

    2017-02-01

    Full Text Available Untreated vitamin B 12 deficiency manifests clinically with hematological abnormalities and combined degeneration of the spinal cord and polyneuropathy and biochemically with elevated homocysteine (Hcy and methylmalonic acid (MMA. Vitamin B 12 metabolism involves various cellular compartments including the lysosome, and a disruption in the lysosomal and endocytic pathways induces functional deficiency of this micronutrient. Gaucher disease (GD is characterized by dysfunctional lysosomal metabolism brought about by mutations in the enzyme beta-glucocerebrosidase (Online Mendelian Inheritance in Man (OMIM: 606463; Enzyme Commission (EC 3.2.1.45, gene: GBA1 . In this study, we collected and examined available literature on the associations between GD, the second most prevalent lysosomal storage disorder in humans, and hampered vitamin B 12 metabolism. Results from independent cohorts of patients show elevated circulating holotranscobalamin without changes in vitamin B 12 levels in serum. Gaucher disease patients under enzyme replacement therapy present normal levels of Hcy and MMA. Although within the normal range, a significant increase in Hcy and MMA with normal serum vitamin B 12 was documented in treated GD patients with polyneuropathy versus treated GD patients without polyneuropathy. Thus, a functional deficiency of vitamin B 12 caused by disrupted lysosomal metabolism in GD is a plausible mechanism, contributing to the neurological form of the disorder but this awaits confirmation. Observational studies suggest that an assessment of vitamin B 12 status prior to the initiation of enzyme replacement therapy may shed light on the role of vitamin B 12 in the pathogenesis and progression of GD.

  10. Risk Profile of the RET A883F Germline Mutation: An International Collaborative Study.

    Science.gov (United States)

    Mathiesen, Jes Sloth; Habra, Mouhammed Amir; Bassett, John Howard Duncan; Choudhury, Sirazum Mubin; Balasubramanian, Sabapathy Prakash; Howlett, Trevor A; Robinson, Bruce G; Gimenez-Roqueplo, Anne-Paule; Castinetti, Frederic; Vestergaard, Peter; Frank-Raue, Karin

    2017-06-01

    The A883F germline mutation of the rearranged during transfection (RET) proto-oncogene causes multiple endocrine neoplasia 2B. In the revised American Thyroid Association (ATA) guidelines for the management of medullary thyroid carcinoma (MTC), the A883F mutation has been reclassified from the highest to the high-risk level, although no well-defined risk profile for this mutation exists. To create a risk profile for the A883F mutation for appropriate classification among the ATA risk levels. Retrospective analysis. International collaboration. Included were 13 A883F carriers. The intervention was thyroidectomy. Earliest age of MTC, regional lymph node metastases, distant metastases, age-related penetrance of MTC and pheochromocytoma (PHEO), overall and disease-specific survival, and biochemical cure rate. One and three carriers were diagnosed at age 7 to 9 years (median, 7.5 years) with a normal thyroid and C-cell hyperplasia, respectively. Nine carriers were diagnosed with MTC at age 10 to 39 years (median, 19 years). The earliest age of MTC, regional lymph node metastasis, and distant metastasis was 10, 20, and 20 years, respectively. Fifty percent penetrance of MTC and PHEO was achieved by age 19 and 34 years, respectively. Five- and 10-year survival rates (both overall and disease specific) were 88% and 88%, respectively. Biochemical cure for MTC at latest follow-up was achieved in 63% (five of eight carriers) with pertinent data. MTC of A883F carriers seems to have a more indolent natural course compared with that of M918T carriers. Our results support the classification of the A883F mutation in the ATA high-risk level. Copyright © 2017 Endocrine Society

  11. A Comparison of Internal Dispositions and Career Trajectories after Collaborative versus Apprenticed Research Experiences for Undergraduates.

    Science.gov (United States)

    Frantz, Kyle J; Demetrikopoulos, Melissa K; Britner, Shari L; Carruth, Laura L; Williams, Brian A; Pecore, John L; DeHaan, Robert L; Goode, Christopher T

    2017-01-01

    Undergraduate research experiences confer benefits on students bound for science, technology, engineering, and mathematics (STEM) careers, but the low number of research professionals available to serve as mentors often limits access to research. Within the context of our summer research program (BRAIN), we tested the hypothesis that a team-based collaborative learning model (CLM) produces student outcomes at least as positive as a traditional apprenticeship model (AM). Through stratified, random assignment to conditions, CLM students were designated to work together in a teaching laboratory to conduct research according to a defined curriculum led by several instructors, whereas AM students were paired with mentors in active research groups. We used pre-, mid-, and postprogram surveys to measure internal dispositions reported to predict progress toward STEM careers, such as scientific research self-efficacy, science identity, science anxiety, and commitment to a science career. We are also tracking long-term retention in science-related career paths. For both short- and longer-term outcomes, the two program formats produced similar benefits, supporting our hypothesis that the CLM provides positive outcomes while conserving resources, such as faculty mentors. We discuss this method in comparison with course-based undergraduate research and recommend its expansion to institutional settings in which mentor resources are scarce. © 2017 K. J. Frantz et al. CBE—Life Sciences Education © 2017 The American Society for Cell Biology. This article is distributed by The American Society for Cell Biology under license from the author(s). It is available to the public under an Attribution–Noncommercial–Share Alike 3.0 Unported Creative Commons License (http://creativecommons.org/licenses/by-nc-sa/3.0).

  12. StarPals International Young Astronomers' Network Collaborative Projects for IYA

    Science.gov (United States)

    Kingan, Jessi

    2008-09-01

    StarPals is a nascent non-profit organization with the goal of providing opportunities for international collaboration between students of all ages within space science research. We believe that by encouraging an interest in the cosmos, the one thing that is truly Universal, from a young age, students will not only further their knowledge of and interest in science but will learn valuable teamwork and life skills. The goal is to foster respect, understanding and appreciation of cultural diversity among all StarPals participants, whether students, teachers, or mentors. StarPals aims to inspire students by providing opportunities in which, more than simply visualizing themselves as research scientists, they can actually become one. The technologies of robotic telescopes, videoconferencing, and online classrooms are expanding the possibilities like never before. In honor of IYA2009, StarPals would like to encourage 400 schools to participate on a global scale in astronomy/cosmology research on various concurrent projects. We will offer in-person or online workshops and training sessions to teach the teachers. We will be seeking publication in scientific journals for some student research. For our current project, the Double Stars Challenge, students use the robotic telescopes to take a series of four images of one of 30 double stars from a list furnished by the US Naval Observatory and then use MPO Canopus software to take distance and position angle measurements. StarPals provides students with hands-on training, telescope time, and software to complete the imaging and measuring. A paper will be drafted from our research data and submitted to the Journal of Double Star Observations. The kids who participate in this project may potentially be the youngest contributors to an article in a vetted scientific journal. Kids rapidly adapt and improve their computer skills operating these telescopes and discover for themselves that science is COOL!

  13. Investigation of original multivalent iminosugars as pharmacological chaperones for the treatment of Gaucher disease.

    Science.gov (United States)

    Laigre, Eugénie; Hazelard, Damien; Casas, Josefina; Serra-Vinardell, Jenny; Michelakakis, Helen; Mavridou, Irene; Aerts, Johannes M F G; Delgado, Antonio; Compain, Philippe

    2016-06-24

    Multivalent iminosugars conjugated with a morpholine moiety and/or designed as prodrugs have been prepared and evaluated as new classes of pharmacological chaperones for the treatment of Gaucher disease. This study further confirms the interest of the prodrug concept and shows that the addition of a lysosome-targeting morpholine unit into iminosugar cluster structures has no significant impact on the chaperone activity on Gaucher cells. Copyright © 2016 Elsevier Ltd. All rights reserved.

  14. Studies in the use of liposomes in the development of an animal model of Gaucher's disease

    OpenAIRE

    Weereratne, Elaine Annmarie Hishani

    1982-01-01

    Gaucher's disease is characterised by a malfunction of the enzyme glucocerebrosidase which hydrolyses the glycolipid glucocerebroside. Mammalian tissues contain at least two beta-glucosidases capable of releasing glucose from the synthetic substrate 4-methylumbelliferyl-beta-D-glucopyranoside (4-MUGlc). One of these enzymes, a membrane bound 'acid' form, also hydrolyses glucocerebroside and is deficient in patients with Gaucher's disease. The synthetic substrate is used to measure this latter...

  15. The ESWN webpage as a tool to increase international collaboration in the Earth Sciences

    Science.gov (United States)

    Glessmer, Mirjam S.; Adams, Manda; de Boer, Agatha M.; Hastings, Meredith; Kontak, Rose

    2013-04-01

    The Earth Science Women's Network (ESWN; ESWNonline.org) is an international peer-mentoring network of women in the Earth Sciences, many in the early stages of their careers. ESWN's mission is to promote career development, build community, provide opportunities for informal mentoring and support, and facilitate professional collaborations. This has been accomplished via email and a listserv, on Facebook, at in-person networking events, and at professional development workshops. Over the last 10 years, ESWN has grown by word of mouth to include more than 1600 members working on all 7 continents. In an effort to facilitate international connections among women in the Earth Sciences, ESWN has developed a password protected community webpage where members can create an online presence and interact with each other. For example, regional groups help women to connect with co-workers at the same employer, in the same city or the same country, or with women at the place where they are considering taking a new job, will attend a conference or will start working soon. Topical groups center around a vast array of topics ranging from research interests, funding opportunities, work-life balance, teaching, scientific methods, and searching for a job to specific challenges faced by women in the earth sciences. Members can search past discussions and share documents like examples of research statements, useful interview materials, or model recommendation letters. The new webpage also allows for more connectivity among other online platforms used by our members, including LinkedIn, Facebook, and Twitter. Built in Wordpress with a Buddypress members-only section, the new ESWN website is supported by AGU and a National Science Foundation ADVANCE grant. While the ESWN members-only community webpage is focused on providing a service to women geoscientists, the content on the public site is designed to be useful for institutions and individuals interested in helping to increase, retain

  16. Is there a role for scintigraphic imaging of bone manifestations in Gaucher disease? A review of the literature

    International Nuclear Information System (INIS)

    Mikosch, P.; State Hospital Klagenfurt; Kohlfuerst, S.; Gallowitsch, H.J.; Kresnik, E.; Lind, P.; Mehta, A.B.; Hughes, D.A.

    2008-01-01

    Gaucher disease is the most prevalent inherited, lysosomal storage disease and is caused by deficient activity of the enzyme β-glucocerebrosidase. Bone and bone marrow alterations are frequent in the most prevalent non-neuronopathic form of Gaucher disease. Imaging of bone manifestations in Gaucher disease is performed by a variety of imaging methods, conventional X-ray and MRI as the most frequently and most important ones. However, different modalities of scintigraphic imaging have also been used. This article gives an overview on scintigraphic imaging with respect to bone manifestations in Gaucher disease discussing the advantages and limitations of scintigraphic imaging in comparison to other imaging methods. (orig.)

  17. A case of improved hearing with cochlear implantation in Gaucher disease type 1.

    Science.gov (United States)

    Endo, Shiori; Mizuta, Kunihiro; Yamatodani, Takashi; Nakanishi, Hiroshi; Hosokawa, Kumiko; Misawa, Kiyoshi; Hosokawa, Seiji; Mineta, Hiroyuki

    2018-06-01

    Gaucher disease is a lysosomal storage disorder that is caused by congenital defective function of the enzyme glucocerebrosidase. Glucocerebroside that is not hydrolyzed by glucocerebrosidase mainly accumulates in the reticular tissue. We describe a Japanese boy with Gaucher disease type 1 who developed bilateral profound sensorineural hearing loss within approximately 4years. We performed cochlear implantation initially on his right ear and again on his left ear 5 months later. The cochlear implants were successfully utilized with a speech discrimination score of 95% on a Japanese sentence recognition test. There are many reports of central hearing loss in Gaucher disease type 2 or 3. However, to the best of our knowledge, this is the first report of profound inner ear hearing loss with Gaucher disease. It also appears to be the first record of cochlear implantation for Gaucher disease. Cochlear implants may be useful for sensorineural hearing loss in patients with Gaucher disease without neurological symptoms other than hearing loss. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. A rare form of Gaucher disease resulting from saposin C deficiency.

    Science.gov (United States)

    Kang, Lulu; Zhan, Xia; Ye, Jun; Han, Lianshu; Qiu, Wenjuan; Gu, Xuefan; Zhang, Huiwen

    2018-02-01

    Gaucher disease is mainly caused by the deficiency of lysosomal acid β-glucosidase. Gaucher disease caused by the deficiency of saposin C is rare. Here we report a patient mainly presenting with hepatosplenomegaly, thrombocytopenia and anemia. EEG examination revealed increased theta waves. Gaucher cells identified in his bone marrow and the highly elevated plasma chitotriosidase activity and glucosylsphingosine supported a diagnosis of Gaucher disease. However, the leukocyte β-glucosidase activity was in a normal range. Sanger sequencing revealed a novel maternal exonic mutation c.1133C>G (p.Pro378Arg) in exon 10 of the PSAP gene, which codes the Sap C domain of PSAP protein. To search for other underlying mutations in this patient, whole genome sequencing was applied and revealed a deletion involving exon 2 to 7 of PSAP gene. The deletion appears as a de novo event on paternal chromosome. We concluded that biallelic mutations of PSAP gene were the cause of this patient's Gaucher disease. Our finding expands the mutation spectrum of Gaucher disease with saposin C deficiency. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. Macrophage models of Gaucher disease for evaluating disease pathogenesis and candidate drugs.

    Science.gov (United States)

    Aflaki, Elma; Stubblefield, Barbara K; Maniwang, Emerson; Lopez, Grisel; Moaven, Nima; Goldin, Ehud; Marugan, Juan; Patnaik, Samarjit; Dutra, Amalia; Southall, Noel; Zheng, Wei; Tayebi, Nahid; Sidransky, Ellen

    2014-06-11

    Gaucher disease is caused by an inherited deficiency of glucocerebrosidase that manifests with storage of glycolipids in lysosomes, particularly in macrophages. Available cell lines modeling Gaucher disease do not demonstrate lysosomal storage of glycolipids; therefore, we set out to develop two macrophage models of Gaucher disease that exhibit appropriate substrate accumulation. We used these cellular models both to investigate altered macrophage biology in Gaucher disease and to evaluate candidate drugs for its treatment. We generated and characterized monocyte-derived macrophages from 20 patients carrying different Gaucher disease mutations. In addition, we created induced pluripotent stem cell (iPSC)-derived macrophages from five fibroblast lines taken from patients with type 1 or type 2 Gaucher disease. Macrophages derived from patient monocytes or iPSCs showed reduced glucocerebrosidase activity and increased storage of glucocerebroside and glucosylsphingosine in lysosomes. These macrophages showed efficient phagocytosis of bacteria but reduced production of intracellular reactive oxygen species and impaired chemotaxis. The disease phenotype was reversed with a noninhibitory small-molecule chaperone drug that enhanced glucocerebrosidase activity in the macrophages, reduced glycolipid storage, and normalized chemotaxis and production of reactive oxygen species. Macrophages differentiated from patient monocytes or patient-derived iPSCs provide cellular models that can be used to investigate disease pathogenesis and facilitate drug development. Copyright © 2014, American Association for the Advancement of Science.

  20. Coenzyme Q10 partially restores pathological alterations in a macrophage model of Gaucher disease.

    Science.gov (United States)

    de la Mata, Mario; Cotán, David; Oropesa-Ávila, Manuel; Villanueva-Paz, Marina; de Lavera, Isabel; Álvarez-Córdoba, Mónica; Luzón-Hidalgo, Raquel; Suárez-Rivero, Juan M; Tiscornia, Gustavo; Sánchez-Alcázar, José A

    2017-02-06

    Gaucher disease (GD) is caused by mutations in the GBA1 gene which encodes lysosomal β-glucocerebrosidase (GCase). In GD, partial or complete loss of GCase activity causes the accumulation of the glycolipids glucosylceramide (GlcCer) and glucosylsphingosine in the lysosomes of macrophages. In this manuscript, we investigated the effects of glycolipids accumulation on lysosomal and mitochondrial function, inflammasome activation and efferocytosis capacity in a THP-1 macrophage model of Gaucher disease. In addition, the beneficial effects of coenzyme Q 10 (CoQ) supplementation on cellular alterations were evaluated. Chemically-induced Gaucher macrophages were developed by differentiateing THP-1 monocytes to macrophages by treatment with phorbol 12-myristate 13-acetate (PMA) and then inhibiting intracellular GCase with conduritol B-epoxide (CBE), a specific irreversible inhibitor of GCase activity, and supplementing the medium with exogenous GlcCer. This cell model accumulated up to 16-fold more GlcCer compared with control THP-1 cells. Chemically-induced Gaucher macrophages showed impaired autophagy flux associated with mitochondrial dysfunction and increased oxidative stress, inflammasome activation and impaired efferocytosis. All abnormalities were partially restored by supplementation with CoQ. These data suggest that targeting mitochondria function and oxidative stress by CoQ can ameliorate the pathological phenotype of Gaucher cells. Chemically-induced Gaucher macrophages provide cellular models that can be used to investigate disease pathogenesis and explore new therapeutics for GD.

  1. Collaborative translational research leading to multicenter clinical trials in Duchenne muscular dystrophy: the Cooperative International Neuromuscular Research Group (CINRG).

    Science.gov (United States)

    Escolar, Diana M; Henricson, Erik K; Pasquali, Livia; Gorni, Ksenija; Hoffman, Eric P

    2002-10-01

    Progress in the development of rationally based therapies for Duchenne muscular dystrophy has been accelerated by encouraging multidisciplinary, multi-institutional collaboration between basic science and clinical investigators in the Cooperative International Research Group. We combined existing research efforts in pathophysiology by a gene expression profiling laboratory with the efforts of animal facilities capable of conducting high-throughput drug screening and toxicity testing to identify safe and effective drug compounds that target different parts of the pathophysiologic cascade in a genome-wide drug discovery approach. Simultaneously, we developed a clinical trial coordinating center and an international network of collaborating physicians and clinics where those drugs could be tested in large-scale clinical trials. We hope that by bringing together investigators at these facilities and providing the infrastructure to support their research, we can rapidly move new bench discoveries through animal model screening and into therapeutic testing in humans in a safe, timely and cost-effective setting.

  2. Investigation of osteosarcoma genomics and its impact on targeted therapy: an international collaboration to conquer human osteosarcoma

    OpenAIRE

    Yang, Ji-Long

    2014-01-01

    Osteosarcoma is a genetically unstable malignancy that most frequently occurs in children and young adults. The lack of progress in managing this devastating disease in the clinic has prompted international researchers to collaborate to profile key genomic alterations that define osteosarcoma. A team of researchers and clinicians from China, Finland, and the United States investigated human osteosarcoma by integrating transcriptome sequencing (RNA-seq), high-density genome-wide array comparat...

  3. The Text of an Agreement for Collaboration in an International Programme on Irradiation of Fruit and Fruit Juices

    International Nuclear Information System (INIS)

    1965-01-01

    The text of the Agreement between the Agency, the Osterreichische Studiengesellschaft fuer Atomenergie GmbH and the Organisation for Economic Co-operation and Development for Collaboration in an International Programme on Irradiation of Fruit and Fruit Juices, which was signed on 16 September 1964 and entered into force on 1 January 1965, is reproduced in this document for the information of all Members of the Agency,

  4. The Text of an Agreement for Collaboration in an International Programme on Irradiation of Fruit and Fruit Juices

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1965-08-12

    The text of the Agreement between the Agency, the Osterreichische Studiengesellschaft fuer Atomenergie GmbH and the Organisation for Economic Co-operation and Development for Collaboration in an International Programme on Irradiation of Fruit and Fruit Juices, which was signed on 16 September 1964 and entered into force on 1 January 1965, is reproduced in this document for the information of all Members of the Agency,.

  5. CMS Collaboration

    International Nuclear Information System (INIS)

    Faridah Mohammad Idris; Wan Ahmad Tajuddin Wan Abdullah; Zainol Abidin Ibrahim

    2013-01-01

    Full-text: CMS Collaboration is an international scientific collaboration located at European Organization for Nuclear Research (CERN), Switzerland, dedicated in carried out research on experimental particle physics. Consisting of 179 institutions from 41 countries from all around the word, CMS Collaboration host a general purpose detector for example the Compact Muon Solenoid (CMS) for members in CMS Collaboration to conduct experiment from the collision of two proton beams accelerated to a speed of 8 TeV in the LHC ring. In this paper, we described how the CMS detector is used by the scientist in CMS Collaboration to reconstruct the most basic building of matter. (author)

  6. Collaborative International Evaluated Library Organisation Pilot Project. CIELO meeting, NEA Headquarters, 18-20 May 2015

    International Nuclear Information System (INIS)

    Mattoon, Caleb; Brown, David; Trkov, Andrej; Plompen, Arjan; Hawari, Ayman I.; Roubtsov, Danila; Kim, Do Heon; Bauge, Eric; Palmiotti, Giuseppe; Kessedjian, Gregoire; Conlin, Jeremy Lloyd; Qian, Jing; Leal, Luiz Carlos; Chadwick, Mark; Herman, Michal Wladyslaw; White, Morgan C.; Cabellos, Oscar; Romain, Pascal; Schillebeeckx, Peter; Ichou, Raphaelle; Jacqmin, Robert; Hilaire, Stephane; Danon, Yaron; Ge, Zhigang; Malvagi, Fausto; Kahler, Albert C. Skip; Morillon, Benjamin; Mcnabb, Dennis P.; Oleynik, Dmitry S.; Wu, Haicheng; Marquez Damian, Jose Ignacio; Yokoyama, Kenji; Dunn, Michael; Cho, Young-Sik; Pignet, Sophie; Ignatyuk, Anatoly V.; Leeb, Helmut; Wang, Wenming; Ruan, Xichao

    2015-05-01

    WPEC subgroup 40-CIELO (Collaborative International Evaluated Library Organization) provides a new working paradigm to facilitate evaluated nuclear reaction data advances. It brings together experts from across the international nuclear reaction data community to identify and document discrepancies among existing evaluated data libraries, measured data, and model calculation interpretations, and aims to make progress in reconciling these discrepancies to create more accurate ENDF-formatted files. SG40-CIELO focusses on 6 important isotopes: "1H, "1"6O, "5"6Fe, "2"3"5","2"3"8U, "2"3"9Pu. This document is the proceedings of the CIELO meeting, held at the NEA Headquarters on 18-20 May 2015. It comprises all the available presentations (slides) given by the participants: A - CIELO project: - 1: Status of Cross Section Progress (M. Chadwick); - 2: Update on CIELO Related Measurements at RPI (Y. Danon); - 3: IAEA-NDS and the CIELO Project (A. Trkov); - 4: LANL Criticality Data Testing using CIELO Candidate Evaluations (S. Kahler); - 5: ENDF/B-VII.1 vs. CIELO (R. Cullen); B - O"1"6: - 6: n+"1"6O (A. Plompen); - 7: Resonance Evaluations for "1"6O for the CIELO Project (L. Leal); - 8: Validation of Leal and Hale O-16 Evaluations against FNS/JAEA Liquid Oxygen ToF Benchmark (I. Kodeli); - 9: Cierjacks 1968, Cierjacks 1980 and RPI 2015 (C.R. Lubitz); - 10: O"1"6 Items (C.R. Lubitz); C - Fe"5"6: - 11: Iron in fast neutron range, beta-0 evaluation for "5"6Fe (M. Herman); - 12: Data Evaluation at ORNL (L. Leal); - 13: IAEA-NDS and the CIELO Project Fe-56 (A. Trkov); - 14: The evaluation of experimental data in fast range for "5"6Fe (Z. Ge); D - H1 - 15: Reactivity effect of New Light and Heavy Water TSL on Critical Systems (J.I. Marquez); E - Big3: - 16: Resonance Evaluations of "2"3"5U for the CIELO Project (L. Leal); - 17: IAEA-NDS and the CIELO Project U-235 (A. Trkov); - 18: Status of "2"3"5U CIELO evaluation (B. Morillon); - 19: U"2"3"5 Items (C.R. Lubitz); - 20: Fission

  7. Creating a charter of collaboration for international university partnerships: the Elmina Declaration for Human Resources for Health.

    Science.gov (United States)

    Anderson, Frank; Donkor, Peter; de Vries, Raymond; Appiah-Denkyira, Ebenezer; Dakpallah, George Fidelis; Rominski, Sarah; Hassinger, Jane; Lou, Airong; Kwansah, Janet; Moyer, Cheryl; Rana, Gurpreet K; Lawson, Aaron; Ayettey, Seth

    2014-08-01

    The potential of international academic partnerships to build global capacity is critical in efforts to improve health in poorer countries. Academic collaborations, however, are challenged by distance, communication issues, cultural differences, and historical context. The Collaborative Health Alliance for Reshaping Training, Education, and Research project (funded by the Bill and Melinda Gates Foundation and implemented through academic medicine and public health and governmental institutions in Michigan and Ghana) took a prospective approach to address these issues. The project had four objectives: to create a "charter for collaboration" (CFC), to improve data-driven policy making, to enhance health care provider education, and to increase research capacity. The goal of the CFC was to establish principles to guide the course of the technical work. All participants participated at an initial conference in Elmina, Ghana. Nine months later, the CFC had been revised and adopted. A qualitative investigation of the CFC's effects identified three themes: the CFC's unique value, the influence of the process of creating the CFC on patterns of communication, and the creation of a context for research and collaboration. Creating the CFC established a context in which implementing technical interventions became an opportunity for dialogue and developing a mutually beneficial partnership. To increase the likelihood that research results would be translated into policy reforms, the CFC made explicit the opportunities, potential problems, and institutional barriers to be overcome. The process of creating a CFC and the resulting document define a new standard in academic and governmental partnerships.

  8. Committee for international collaborative research of medical and welfare apparatus; Iryo fukushi kiki kokusai kyodo kenkyu jigyo

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1996-03-01

    The paper summarized activities for an investigational study on the international collaborative research project on the medical and welfare apparatus implemented in fiscal 1995. As investigation activities, the second meeting of information exchanges with E.U. and three north European countries was held following the meeting in fiscal 1994, and at the same time, information exchanges were made with organizations/institutions of industry/government/university in Germany, France and Canada. The study made it clear that Europe is also taking a direction of low-degree action diagnosis/care as Japan is. Further, concrete exchanges of information advanced and an awareness of the common issues was made clear such as the necessity of developing apparatus which meets the apparatus market and users` needs. As international collaborative activities, new methods of information exchanges were adopted such as the satellite meeting of the International MR Society, meetings with researchers who visited Japan. The satellite meeting of the International MR Society was favorably accepted by participants, and it was pointed out that it is important to continue the meeting in view of the materialization of themes, etc. 4 refs., 32 figs., 5 tabs.

  9. Establishment of the international collaboration and licensing preparation planning for the specific design of a prototype SFR

    International Nuclear Information System (INIS)

    Kim, Y. G.; Joo, H. K.; Cho, C. H.; Yoo, J. W.; Lee, D. U.; Ahn, K. S.; Hwang, Y. S.

    2013-05-01

    The conceptual design of prototype of Gen IV SFR (PGSFR) will be early determined through the review of the international experts. After this, the technology demonstration plan and validation of fuel design will be determined in more detail. The project will be accomplished efficiently by introducing the proven technology already validated from the international collaboration. The conceptual design and its requirements of PGSFR will be reviewed by ANL, who has a lot of design experiences in the metal fueled SFR development. The collaboration with ANL has been done through Work For Others (WFO) contract, and the MOU was signed between SFRA and Terra Power(USA), and SFRA and IGCAR. The licensing issues raised during PFBR and FBTR licensing in India will be discussed and reflected into the PGSFR design by inviting the high level expert from India, for example Dr. Chetal in IGCAR. The specific design, technology validation plan and fuel development plan will be established in more detail through the annual International Technical Review Meeting (ITRM) and experimental facilities available from the international institute and companies, which will be the basis for shortening the project period and to reduce the development cost

  10. Hyperferritinemia and iron metabolism in Gaucher disease: Potential pathophysiological implications.

    Science.gov (United States)

    Regenboog, Martine; van Kuilenburg, André B P; Verheij, Joanne; Swinkels, Dorine W; Hollak, Carla E M

    2016-11-01

    Gaucher disease (GD) is characterized by large amounts of lipid-storing macrophages and is associated with accumulation of iron. High levels of ferritin are a hallmark of the disease. The precise mechanism underlying the changes in iron metabolism has not been elucidated. A systematic search was conducted to summarize available evidence from the literature on iron metabolism in GD and its potential pathophysiological implications. We conclude that in GD, a chronic low grade inflammation state can lead to high ferritin levels and increased hepcidin transcription with subsequent trapping of ferritin in macrophages. Extensive GD manifestations with severe anemia or extreme splenomegaly can lead to a situation of iron-overload resembling hemochromatosis. We hypothesize that specifically this latter situation carries a risk for the occurrence of associated conditions such as the increased cancer risk, metabolic syndrome and neurodegeneration. Copyright © 2016 Elsevier Ltd. All rights reserved.

  11. Bone turnover markers in patients with type 1 Gaucher disease

    Directory of Open Access Journals (Sweden)

    Gaetano Giuffrida

    2012-11-01

    Full Text Available Bone complications occur frequently in Gaucher disease (GD and reduce the quality of life of these patients. Skeletal involvement is an important indication for treatment to ameliorate symptoms and reduce the risk of irreversible and debilitating disease. Bone biomarkers have been used to assess disease status and the response to therapy in a number of bone disorders. Here, we examine the literature for evidence of abnormalities in bone turnover markers in patients with type 1 GD to assess whether they might be useful for the assessment of bone involvement in GD. We have found that bone biomarkers in GD show highly variable results which do not currently support their routine use for clinical assessment of bone status, as an indication for therapy initiation, or for monitoring the response to therapy. A greater understanding of bone markers and their relation to the bone manifestations of GD is required.

  12. Isofagomine in vivo effects in a neuronopathic Gaucher disease mouse.

    Directory of Open Access Journals (Sweden)

    Ying Sun

    2011-04-01

    Full Text Available The pharmacological chaperone, isofagomine (IFG, enhances acid β-glucosidase (GCase function by altering folding, trafficking, and activity in wild-type and Gaucher disease fibroblasts. The in vivo effects of IFG on GCase activity, its substrate levels, and phenotype were evaluated using a neuronopathic Gaucher disease mouse model, 4L;C* (V394L/V394L + saposin C-/- that has CNS accumulation of glucosylceramide (GC and glucosylsphingosine (GS as well as progressive neurological deterioration. IFG administration to 4L;C* mice at 20 or 600 mg/kg/day resulted in life span extensions of 10 or 20 days, respectively, and increases in GCase activity and protein levels in the brain and visceral tissues. Cerebral cortical GC and GS levels showed no significant reductions with IFG treatment. Increases of GC or GS levels were detected in the visceral tissues of IFG treated (600 mg/kg/day mice. The attenuations of brain proinflammatory responses in the treated mice were evidenced by reductions in astrogliosis and microglial cell activation, and decreased p38 phosphorylation and TNFα levels. Terminally, axonal degeneration was present in the brain and spinal cord from untreated and treated 4L;C* mice. These data demonstrate that IFG exerts in vivo effects by enhancing V394L GCase protein and activity levels, and in mediating suppression of proinflammation, which led to delayed onset of neurological disease and extension of the life span of 4L;C* mice. However, this was not correlated with a reduction in the accumulation of lipid substrates.

  13. Inhibition of UDP-glucosylceramide synthase in mice prevents Gaucher disease-associated B-cell malignancy

    NARCIS (Netherlands)

    Pavlova, Elena V.; Archer, Joy; Wang, Susan; Dekker, Nick; Aerts, Johannes Mfg; Karlsson, Stefan; Cox, Timothy M.

    2015-01-01

    Clonal B-cell proliferation is a frequent manifestation of Gaucher disease - a sphingolipidosis associated with a high risk of multiple myeloma and non-Hodgkin lymphoma. Gaucher disease is caused by genetic deficiency of acid β-glucosidase, the natural substrates of which (β-d-glucosylceramide and

  14. Dose-response relationships for enzyme replacement therapy with imiglucerase/alglucerase in patients with Gaucher disease type 1

    NARCIS (Netherlands)

    Grabowski, Gregory A.; Kacena, Katherine; Cole, J. Alexander; Hollak, Carla E. M.; Zhang, Lin; Yee, John; Mistry, Pramod K.; Zimran, Ari; Charrow, Joel; vom Dahl, Stephan

    2009-01-01

    Purpose: To determine whether enzyme therapy with imiglucerase/ alglucerase demonstrates dose-response relationships with doses and disease parameters used in routine clinical practice for Gaucher disease type 1 patients. Methods: Analyses included all patients with Gaucher disease type 1 on enzyme

  15. Plasma glucosylceramide and ceramide in type 1 Gaucher disease patients: correlations with disease severity and response to therapeutic intervention

    NARCIS (Netherlands)

    Groener, J. E. M.; Poorthuis, B. J. H. M.; Kuiper, S.; Hollak, C. E. M.; Aerts, J. M. F. G.

    2008-01-01

    The concentrations of plasma glucosylceramide (GlcCer) and ceramide (Cer) were determined in a cohort of type 1 Gaucher disease patients. In plasma of untreated patients, GlcCer concentrations were on average 3-fold increased (median Gaucher: 17.5 nmol/ml, range: 6.5-45.5 (n=27); median control: 5.9

  16. Effects of switching from a reduced dose imiglucerase to velaglucerase in type 1 Gaucher disease: clinical and biochemical outcomes

    NARCIS (Netherlands)

    van Dussen, Laura; Cox, Timothy M.; Hendriks, Erik J.; Morris, Elizabeth; Akkerman, Erik M.; Maas, Mario; Groener, Johanna E. M.; Aerts, Johannes M. F. G.; Deegan, Patrick B.; Hollak, Carla E. M.

    2012-01-01

    This paper describes the effects of a switch to velaglucerase alfa in a group of adult patients with type 1 Gaucher disease, all of whom had previously had their dose reduced as a consequence of the worldwide imiglucerase shortage. Thirty-two patients from two large European Gaucher centers switched

  17. Networking support for collaborative virtual reality projects in national, european and international context

    OpenAIRE

    Hommes, F.; Pless, E.

    2004-01-01

    The report describes experiences from networking support for two three years virtual reality projects. Networking requirements depending on the virtual reality environment and the planned distributed scenarios are specified and verified in the real network. Networking problems especially due to the collaborative, distributed character of interaction via the Internet are presented.

  18. Grading Innovation in an International Marketing Course: Promoting Student Collaboration and Individual Accountability

    Science.gov (United States)

    Chelminski, Piotr

    2017-01-01

    In this article, the author proposes an innovative, exam-based homework grading method to facilitate both collaboration among students and individual accountability while learning a complex theory and applying it to solve a problem. Results from this novel approach to grading a "comparative advantage theory" homework assignment, using an…

  19. Space orbits of collaboration. [international cooperation and the U.S.S.R. space program

    Science.gov (United States)

    Petrov, B.

    1978-01-01

    The U.S.S.R. cooperative space efforts with other Socialist countries dating back to 1957 are reviewed. The Interkosmos program, which is divided into three series of satellites (solar, ionospheric and magnetospheric), is discussed as well as the Prognoz, Kosmos, Soyuz, and Molniya spacecraft. Collaboration with France, India, Sweden, and the United States is mentioned.

  20. Developing Communication Confidence and Professional Identity in Chemistry through International Online Collaborative Learning

    Science.gov (United States)

    Skagen, Darlene; McCollum, Brett; Morsch, Layne; Shokoples, Brandon

    2018-01-01

    The use of online collaborative assignments (OCAs) between two flipped organic chemistry classrooms, one in Canada and the other in the United States, was examined for impact on learners. The intervention was designed to support content mastery, aid in increasing students' communication skills through chemistry drawing and verbalization,…

  1. 17 September 2013 - Estonian Minister of Education and Research J. Aaviksoo signing the guest book with CERN Director-General R- Heuer; visiting the TOTEM facility with TOTEM Collaboration Spokesperson S. Giani; in the LHC tunnel at Point 5 with International Relations Adviser T. Kurtyka and visiting the CMS cavern with CMS Collaboration Spokesperson J. Incandela. International Relations Adviser R. Voss present.

    CERN Multimedia

    Anna Pantelia

    2013-01-01

    17 September 2013 - Estonian Minister of Education and Research J. Aaviksoo signing the guest book with CERN Director-General R- Heuer; visiting the TOTEM facility with TOTEM Collaboration Spokesperson S. Giani; in the LHC tunnel at Point 5 with International Relations Adviser T. Kurtyka and visiting the CMS cavern with CMS Collaboration Spokesperson J. Incandela. International Relations Adviser R. Voss present.

  2. 30 January 2012 - Ecuadorian Ambassador Gallegos Chiriboga, Permanent Representative to the United Nations Office and other International Organisations at Geneva and San Francisco de Quito University Vice Chancellor C. Montùfar visiting CMS surface facilities and underground experimental area with CMS Collaboration L. Sulak and Collaboration Deputy Spokesperson T. Camporesi, throughout accompanied by Head of International Relations F. Pauss.

    CERN Multimedia

    Michael Hoch

    2012-01-01

    30 January 2012 - Ecuadorian Ambassador Gallegos Chiriboga, Permanent Representative to the United Nations Office and other International Organisations at Geneva and San Francisco de Quito University Vice Chancellor C. Montùfar visiting CMS surface facilities and underground experimental area with CMS Collaboration L. Sulak and Collaboration Deputy Spokesperson T. Camporesi, throughout accompanied by Head of International Relations F. Pauss.

  3. Impact of Early Valve Surgery on Outcome of Staphylococcus aureus Prosthetic Valve Infective Endocarditis: Analysis in the International Collaboration of Endocarditis–Prospective Cohort Study

    OpenAIRE

    Chirouze, Catherine; Alla, François; Fowler, Vance G.; Sexton, Daniel J.; Corey, G. Ralph; Chu, Vivian H.; Wang, Andrew; Erpelding, Marie-Line; Durante-Mangoni, Emanuele; Fernández-Hidalgo, Nuria; Giannitsioti, Efthymia; Hannan, Margaret M.; Lejko-Zupanc, Tatjana; Miró, José M.; Muñoz, Patricia

    2014-01-01

    Using appropriate analytical methods to examine data from the International Collaboration on Endocarditis–Prospective Cohort Study, we found that early valve surgery was not associated with reduced 1-year mortality in Staphylococcus aureus prosthetic valve infective endocarditis.

  4. Lessons Learned from GOSAT; Instrument Design, Calibration, Operation, Data Processing, and International Collaboration

    Science.gov (United States)

    Kuze, A.; Suto, H.; Shiomi, K.; Nakajima, M.

    2012-12-01

    we target sun glint (specular reflection) points, where the surface reflectance is not uniform or randomly distributed. Over the desert area, where surface reflectance is high and dust layers extend to high altitude, we have to use lower gain to avoid saturation and multiple scattering is complicated. For both ocean high albedo targets, validation data of XCO2 and XCH4 are limited. Validation by airplane become accurate but extrapolation is needed above flight attitude. Comparison of surface pressure between retrieved data and a priori model forecast fields is useful. For such kind of consistency, measurement must be very linear within wide dynamic range. After launch, we are re-evaluating the linearity of the detector, analog circuit and AD converters using a lab-model on the ground. Since launch, we have received feedback about the radiance spectra data from many data users of diverse institutes. We have gathered many calibration and validation data from international collaboration. We have modified the Level 1 algorithm and instrument calibrations several times. We are currently measuring the grid point of the Earth's surface, but the sampling for source and sink retrieval has not been optimized yet. We will present how we have solved problems, which portions of the instrument design we should modify, and items that we have not understood well yet.

  5. Humans on the International Space Station-How Research, Operations, and International Collaboration are Leading to New Understanding of Human Physiology and Performance in Microgravity

    Science.gov (United States)

    Ronbinson, Julie A.; Harm, Deborah L.

    2009-01-01

    As the International Space Station (ISS) nears completion, and full international utilization is achieved, we are at a scientific crossroads. ISS is the premier location for research aimed at understanding the effects of microgravity on the human body. For applications to future human exploration, it is key for validation, quantification, and mitigation of a wide variety of spaceflight risks to health and human performance. Understanding and mitigating these risks is the focus of NASA s Human Research Program. However, NASA s approach to defining human research objectives is only one of many approaches within the ISS international partnership (including Roscosmos, the European Space Agency, the Canadian Space Agency, and the Japan Aerospace Exploration Agency). Each of these agencies selects and implements their own ISS research, with independent but related objectives for human and life sciences research. Because the science itself is also international and collaborative, investigations that are led by one ISS partner also often include cooperative scientists from around the world. The operation of the ISS generates significant additional data that is not directly linked to specific investigations. Such data comes from medical monitoring of crew members, life support and radiation monitoring, and from the systems that have been implemented to protect the health of the crew (such as exercise hardware). We provide examples of these international synergies in human research on ISS and highlight key early accomplishments that derive from these broad interfaces. Taken as a whole, the combination of diverse research objectives, operational data, international sharing of research resources on ISS, and scientific collaboration provide a robust research approach and capability that no one partner could achieve alone.

  6. The Integrating Role of the LBA and the LPB Programs as an Example of Cyberinfrastructures in International Scientific Collaboration

    Science.gov (United States)

    Dias, P. L.

    2007-05-01

    International science collaboration is a key component of research programs such as the The Large Scale Biosphere Atmosphere Interaction Program (LBA) and the La Plata Basin Project (LPB). Both are programs with crosscutting science questions permeating different areas of knowledge related to the functioning of the natural and agricultural ecosystems in the Amazon system (LBA) and the change in the hydrological, agricultural and social systems of the Plata Basin (LPB) ecosystem under natural climatic variability and climate change. Both programs are strongly related to GEWEX, CLIVAR and IGBP and are based on extensive use of data information system (LBA/LPB/DIS) with mirror sites in the US, Europe and South America. These international programs have a significant impact in building up regional scientific capabilities at all levels of education and triggered the establishment of new research groups located in remote areas of South America. The cyberinfrastructure has been fundamental to promote the integration of the research groups, and a remarkable feedback with the operational forecasting systems has been detected. The LBA/LPB should be used as examples on how to promote international scientific and operational collaboration.

  7. Gaucher disease: plasmalogen levels in relation to primary lipid abnormalities and oxidative stress.

    Science.gov (United States)

    Moraitou, Marina; Dimitriou, Evangelia; Dekker, Nick; Monopolis, Ioannis; Aerts, Johannes; Michelakakis, Helen

    2014-01-01

    Plasmalogens represent a unique class of phospholipids. Reduced red blood cell plasmalogen levels in Gaucher disease patients were reported, correlating to total disease burden. The relation between plasmalogen abnormalities in Gaucher disease patients and primary glycosphingolipid abnormalities, malonyldialdehyde levels, an indicator of lipid peroxidation, and the total antioxidant status was further investigated. Significant reduction of C16:0 and C18:0 plasmalogens in red blood cells of Gaucher disease patients was confirmed. In parallel, a significant increase in the glucosylceramide/ceramide ratio in red blood cell membranes, as well as an average 200-fold increase in plasma glucosylsphingosine levels was observed. Red blood cell malonyldialdehyde levels were significantly increased in patients, whereas their total antioxidant status was significantly reduced. A negative correlation between plasmalogen species and glucosylceramide, ceramide, glucosylceramide/ceramide ratio, glucosylsphingosine and malonyldialdehyde, significant for the C16:0 species and all the above parameters with the exception of malonyldialdehyde levels, was found along with a positive non-significant correlation with the total antioxidant status. Our results indicate that increased lipid peroxidation and reduced total antioxidant status exist in Gaucher disease patients. They demonstrate a clear link between plasmalogen levels and the primary glycolipid abnormalities characterizing the disorder and an association with the increased oxidative stress observed in Gaucher disease patients. Copyright © 2014 Elsevier Inc. All rights reserved.

  8. Are transient and shear wave elastography useful tools in Gaucher disease?

    Science.gov (United States)

    Webb, Muriel; Zimran, Ari; Dinur, Tama; Shibolet, Oren; Levit, Stella; Steinberg, David M; Salomon, Ophira

    2018-02-01

    Up to now, there are no reliable biochemical markers or imaging that could reveal early tissue damage in Gaucher disease. Therefore, we addressed whether elastography technique can serve as a tool for evaluating patients with Gaucher disease. The study included 42 patients with Gaucher disease type I and 33 patients with liver cirrhosis as well as 22 healthy volunteers. Ultrasound and Doppler examination was performed on each participant prior to apply transient and 2D shear wave elastography. In Gaucher disease the median stiffness of the spleen as assessed by transient elastography (TE) and shear wave elastography (SWE) was 35KPa and 22KPa respectively in contrast to the median stiffness of healthy controls (16.95 and 17.5KPa, p=0.0028 and p=0.0002, respectively) and of patients with cirrhosis (45KPa and 34.5KPa, p=0.015 and pGaucher disease from healthy controls and among those with splenomegaly from cirrhotic patients. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Glycosphingolipid analysis in a naturally occurring ovine model of acute neuronopathic Gaucher disease.

    Science.gov (United States)

    Karageorgos, Litsa; Hein, Leanne; Rozaklis, Tina; Adams, Melissa; Duplock, Stephen; Snel, Marten; Hemsley, Kim; Kuchel, Tim; Smith, Nicholas; Hopwood, John J

    2016-07-01

    Gaucher disease arises from mutations in the β-glucocerebrosidase gene which encodes an enzyme required for the lysosomal catabolism of glucosylceramide. We have identified a naturally occurring mutation in the β-glucocerebrosidase gene in sheep that leads to Gaucher disease with acute neurological symptoms. Here we have examined the clinical phenotype at birth and subsequently quantified lipids in Gaucher lamb brain, in order to characterise the disorder. Enzyme activity assessments showed that a reduction in β-glucocerebrosidase activity to 1-5% of wild-type occurs consistently across newborn Gaucher lamb brain regions. We analyzed glucosylceramide, glucosylsphingosine, bis(monoacylglycero)phosphate and ganglioside profiles in brain, liver, and spleen, and observed 30- to 130-fold higher glucosylceramide, and 500- to 2000-fold higher glucosylsphingosine concentrations in Gaucher diseased lambs compared to wild-type. Significant increases of bis(monoacylglycero)phosphate and gangliosides [GM1, GM2, GM3] concentrations were also detected in the brain. As these glycosphingolipids are involved in many cellular events, an imbalance or disruption of the cell membrane lipid homeostasis would be expected to impair normal neuronal function. To our knowledge, this is the first detailed analysis of glycosphingolipids in various brain regions in a large animal model of neuronal disease, which permits the mechanistic investigation of lipid deregulation and their contribution to neurodegenerative process. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Generation of a conditional knockout of murine glucocerebrosidase: utility for the study of Gaucher disease.

    Science.gov (United States)

    Sinclair, Graham B; Jevon, Gareth; Colobong, Karen E; Randall, Derrick R; Choy, Francis Y M; Clarke, Lorne A

    2007-02-01

    Gaucher disease is a disorder of sphingolipid metabolism resulting from an inherited deficiency of the lysosomal hydrolase glucocerebrosidase. Affected individuals present with a spectrum of clinical symptoms ranging from hepatosplenomegaly, haematological abnormalities, and bone pain in type 1 disease, to severe neurodegeneration and premature death in types 2 and 3 disease. Although the basic biochemical defect is well characterized, there remains a poor understanding of the underlying pathophysiology of disease. In vitro studies suggest that macrophage glucocerebroside storage leads to tissue dysfunction through complex mechanisms involving altered intracellular calcium homeostasis and apoptosis. In order to study the pathogenic roles of these complex interactions, a viable animal model for Gaucher disease is needed. The complexity of this single gene disorder has been emphasized by the varied results of previous murine Gaucher models, ranging from perinatal lethality to phenotypically and biochemically asymptomatic animals. Recognizing the need to modulate the biochemical phenotype in mice to produce a relevant model, we have created a murine strain with key exons of the glucocerebrosidase gene flanked by loxP sites. We show that expression of Cre-recombinase in cells of hematopoietic and endothelial origin results in deficiency of glucocerebrosidase in the liver, spleen, bone marrow, and peripheral white cells. Glucocerebroside storage in this model leads to progressive splenomegaly with Gaucher cell infiltration and modest storage in the liver by 26 weeks of age. These results indicate the utility of this loxP GBA targeted murine strain for understanding the complex pathophysiology of Gaucher disease.

  11. A specific and potent inhibitor of glucosylceramide synthase for substrate inhibition therapy of Gaucher disease.

    Science.gov (United States)

    McEachern, Kerry Anne; Fung, John; Komarnitsky, Svetlana; Siegel, Craig S; Chuang, Wei-Lien; Hutto, Elizabeth; Shayman, James A; Grabowski, Gregory A; Aerts, Johannes M F G; Cheng, Seng H; Copeland, Diane P; Marshall, John

    2007-07-01

    An approach to treating Gaucher disease is substrate inhibition therapy which seeks to abate the aberrant lysosomal accumulation of glucosylceramide. We have identified a novel inhibitor of glucosylceramide synthase (Genz-112638) and assessed its activity in a murine model of Gaucher disease (D409V/null). Biochemical characterization of Genz-112638 showed good potency (IC(50) approximately 24nM) and specificity against the target enzyme. Mice that received drug prior to significant accumulation of substrate (10 weeks of age) showed reduced levels of glucosylceramide and number of Gaucher cells in the spleen, lung and liver when compared to age-matched control animals. Treatment of older mice that already displayed significant amounts of tissue glucosylceramide (7 months old) resulted in arrest of further accumulation of the substrate and appearance of additional Gaucher cells in affected organs. These data indicate that substrate inhibition therapy with Genz-112638 represents a viable alternate approach to enzyme therapy to treat the visceral pathology in Gaucher disease.

  12. Effective drinking water collaborations are not accidental: interagency relationships in the international water utility sector.

    Science.gov (United States)

    Jalba, D I; Cromar, N J; Pollard, S J T; Charrois, J W; Bradshaw, R; Hrudey, S E

    2014-02-01

    The role that deficient institutional relationships have played in aggravating drinking water incidents over the last 30 years has been identified in several inquiries of high profile drinking water safety events, peer-reviewed articles and media reports. These indicate that collaboration between water utilities and public health agencies (PHAs) during normal operations, and in emergencies, needs improvement. Here, critical elements of these interagency collaborations, that can be integrated within the corporate risk management structures of water utilities and PHAs alike, were identified using a grounded theory approach and 51 semi-structured interviews with utility and PHA staff. Core determinants of effective interagency relationships are discussed. Intentionally maintained functional relationships represent a key ingredient in assuring the delivery of safe, high quality drinking water. © 2013.

  13. Establishment of the foundation for international collaborating research with US NASA FTCSC to develop space, military and special purpose food

    International Nuclear Information System (INIS)

    Jo, Chul Hun; Byun, Myung Woo; Lee, Ju Wun; Kim, Dong Ho; Kim, Kyung Pho; Kim, Jang Ho; Kwon, Jung Ho

    2005-08-01

    In the space era of 21st century, the advancement of aerospace field is essential for ensuring the national security and raising the national status. Internationally spacefood and space life support system is considered as an limitedly developed technology area. Establishment of the foundation for collaborating study with NASA FTCSC to develop space, military, and special food. Acquirement of the basis of the technology development for safe, long-term preservation of military and special purpose food to ensure national security as well as health and welfare

  14. Cardiovascular events prior to or early after diagnosis of systemic lupus erythematosus in the systemic lupus international collaborating clinics cohort

    DEFF Research Database (Denmark)

    Urowitz, M B; Gladman, D D; Anderson, N M

    2016-01-01

    OBJECTIVE: To describe the frequency of myocardial infarction (MI) prior to the diagnosis of systemic lupus erythematosus (SLE) and within the first 2 years of follow-up. METHODS: The systemic lupus international collaborating clinics (SLICC) atherosclerosis inception cohort enters patients within......% CI 2.38 to 23.57) remained significant risk factors. CONCLUSIONS: In some patients with lupus, MI may develop even before the diagnosis of SLE or shortly thereafter, suggesting that there may be a link between autoimmune inflammation and atherosclerosis....

  15. Rationale for the prevention of oral diseases in primary health care: an international collaborative study in oral health education.

    Science.gov (United States)

    Bourgeois, Denis M; Phantumvanit, Prathip; Llodra, Juan Carlos; Horn, Virginie; Carlile, Monica; Eiselé, Jean-Luc

    2014-10-01

    Ensuring that members of society are healthy and reaching their full potential requires the prevention of oral diseases through the promotion of oral health and well-being. The present article identifies the best policy conditions of effective public health and primary care integration and the actors who promote and sustain these efforts. In this review, arguments and recommendations are provided to introduce an oral health collaborative promotion programme called Live.Learn.Laugh. phase 2, arising from an unique partnership between FDI World Dental Federation, the global company Unilever plc and an international network of National Dental Associations, health-care centres, schools and educators populations. © 2014 FDI World Dental Federation.

  16. The virtual library in action: Collaborative international control of high-energy physics pre-print

    International Nuclear Information System (INIS)

    Kreitz, P.A.; Addis, L.; Galic, H.; Johnson, T.

    1996-02-01

    This paper will discuss how control of the grey literature in high-energy physics pre-prints developed through a collaborative effort of librarians and physicists. It will highlight the critical steps in the development process and describe one model of a rapidly evolving virtual library for high-energy physics information. In conclusion, this paper will extend this physics model to other areas of grey literature management

  17. Effective drinking water collaborations are not accidental: Interagency relationships in the international water utility sector

    International Nuclear Information System (INIS)

    Jalba, D.I.; Cromar, N.J.; Pollard, S.J.T.; Charrois, J.W.; Bradshaw, R.; Hrudey, S.E.

    2014-01-01

    The role that deficient institutional relationships have played in aggravating drinking water incidents over the last 30 years has been identified in several inquiries of high profile drinking water safety events, peer-reviewed articles and media reports. These indicate that collaboration between water utilities and public health agencies (PHAs) during normal operations, and in emergencies, needs improvement. Here, critical elements of these interagency collaborations, that can be integrated within the corporate risk management structures of water utilities and PHAs alike, were identified using a grounded theory approach and 51 semi-structured interviews with utility and PHA staff. Core determinants of effective interagency relationships are discussed. Intentionally maintained functional relationships represent a key ingredient in assuring the delivery of safe, high quality drinking water. - Highlights: • Qualitative analysis of current water sector practices on interagency relations • Identification of suboptimal approaches to working with public health agencies • Effective strategies for developing and maintaining institutional collaborations • Supporting the implementation of WHO guidelines for drinking water quality

  18. Effective drinking water collaborations are not accidental: Interagency relationships in the international water utility sector

    Energy Technology Data Exchange (ETDEWEB)

    Jalba, D.I. [School of Medicine, Flinders University, GPO 2100, Adelaide, SA 5001 (Australia); Cromar, N.J., E-mail: nancy.cromar@flinders.edu.au [School of the Environment, Flinders University, GPO 2100, Adelaide, SA 5001 (Australia); Pollard, S.J.T. [Cranfield Water Science Institute, Cranfield University, Bedfordshire, MK43 0AL (United Kingdom); Charrois, J.W. [Curtin Water Quality Research Centre, Curtin University, GPO Box U1987, Perth, WA 6845 (Australia); Bradshaw, R. [Cranfield Water Science Institute, Cranfield University, Bedfordshire, MK43 0AL (United Kingdom); Hrudey, S.E. [Analytical and Environmental Toxicology Division, Faculty of Medicine and Dentistry, 10-102 Clinical Sciences Building, University of Alberta, Edmonton, AB T6G 2G3 (Canada)

    2014-02-01

    The role that deficient institutional relationships have played in aggravating drinking water incidents over the last 30 years has been identified in several inquiries of high profile drinking water safety events, peer-reviewed articles and media reports. These indicate that collaboration between water utilities and public health agencies (PHAs) during normal operations, and in emergencies, needs improvement. Here, critical elements of these interagency collaborations, that can be integrated within the corporate risk management structures of water utilities and PHAs alike, were identified using a grounded theory approach and 51 semi-structured interviews with utility and PHA staff. Core determinants of effective interagency relationships are discussed. Intentionally maintained functional relationships represent a key ingredient in assuring the delivery of safe, high quality drinking water. - Highlights: • Qualitative analysis of current water sector practices on interagency relations • Identification of suboptimal approaches to working with public health agencies • Effective strategies for developing and maintaining institutional collaborations • Supporting the implementation of WHO guidelines for drinking water quality.

  19. Diagnosis features of pediatric Gaucher disease patients in the era of enzymatic therapy, a national-base study from the Spanish Registry of Gaucher Disease.

    Science.gov (United States)

    Andrade-Campos, Marcio; Alfonso, Pilar; Irun, Pilar; Armstrong, Judith; Calvo, Carmen; Dalmau, Jaime; Domingo, Maria-Rosario; Barbera, Jose-Luis; Cano, Horacio; Fernandez-Galán, Maria-Angeles; Franco, Rafael; Gracia, Inmaculada; Gracia-Antequera, Miguel; Ibañez, Angela; Lendinez, Francisco; Madruga, Marcos; Martin-Hernández, Elena; O'Callaghan, Maria Del Mar; Del Soto, Alberto Pérez; Del Prado, Yolanda Ruiz; Sancho-Val, Ignacio; Sanjurjo, Pablo; Pocovi, Miguel; Giraldo, Pilar

    2017-05-03

    The enzymatic replacement therapy (ERT) availability for Gaucher disease (GD) has changed the landscape of the disease, several countries have screening programs. These actions have promoted the early diagnosis and avoided many complications in pediatric patients. In Spain ERT has been available since 1993 and 386 patients have been included in the Spanish Registry of Gaucher Disease (SpRGD). The aim of this study is to analyze the impact of ERT on the characteristics at time of diagnosis and initial complications in pediatric Gaucher disease patients. To analyze the impact of ERT on the characteristics at time of diagnosis and initial complications in pediatric Gaucher disease patients. A review of data in SpRGD from patients' diagnosed before 18 years old was performed. The cohort was split according the year of diagnosis (≤1994, cohort A; ≥1995, cohort B). A total of 98 pediatric patients were included, GD1: 80, GD3: 18; mean age: 7.2 (0.17-16.5) years, 58 (59.2%) males and 40 (40.8%) females. Forty-five were diagnosed ≤ 1994 and 53 ≥ 1995. Genotype: N370S/N370S: 2 (2.0%), N370S/L444P: 27 (27.5%), N370S/other: 47 (48%), L444P/L444P: 7 (7.1%), L444P/D409H: 2 (2.0%), L444P/other: 3 (6.2%), other/other: 10 (10.2%). The mean age at diagnosis was earlier in patients diagnosed after 1995 (p Gaucher disease in the era of ERT availability has permitted to reduce the incidence of severe and irreversible initial complication in pediatric patients, and this has permitted better development of these patients. This is the largest pediatric cohort from a national registry.

  20. Study on collaborative operation in Xi'an international inland port and airport

    Science.gov (United States)

    Jia, Guoling

    2017-10-01

    Xi 'an international inland port and airport are the important fulcrums for Shaanxi province to implement the strategy of "One Belt One Road" and to develop its export-oriented economy. Based on the general development situation of Xi 'an international inland port and airport and analyzing their similarities and differences, the external cause and internal cause of synergy are discussed. The contents of synergy from the strategy level, tactics level and business level are explained respectively.

  1. The Role of Atomic Energy in the Promotion of International Collaboration

    Science.gov (United States)

    Rabi, I. I.

    1959-10-31

    A brief survey is presented of the international cooperation which made a success of the First Geneva Conference and which has initiated many international scientific meetings since that time. The policy of the United States in this respect is discussed. (J.S.R.)

  2. Interdisciplinary Collaborative Learning: Using Decision Analysts to Enhance Undergraduate International Management Education

    Science.gov (United States)

    Palocsay, Susan W.; White, Marion M.; Zimmerman, D. Kent

    2004-01-01

    This article describes an experiential learning activity designed to promote the development of decision-making skills in international management students at the undergraduate level. Students from an undergraduate management science course in decision analysis served as consultants on a case assigned to teams in an international management class.…

  3. Pathogenesis of Bone Alterations in Gaucher Disease: The Role of Immune System

    Directory of Open Access Journals (Sweden)

    Juan Marcos Mucci

    2015-01-01

    Full Text Available Gaucher, the most prevalent lysosomal disorder, is an autosomal recessive inherited disorder due to a deficiency of glucocerebrosidase. Glucocerebrosidase deficiency leads to the accumulation of glucosylceramide primarily in cells of mononuclear-macrophage lineage. Clinical alterations are visceral, hematological, and skeletal. Bone disorder in Gaucher disease produces defects on bone metabolism and structure and patients suffer from bone pain and crisis. Skeletal problems include osteopenia, osteoporosis, osteolytic lesions, and osteonecrosis. On the other hand a chronic stimulation of the immune system is a well-accepted hallmark in this disease. In this review we summarize the latest findings in the mechanisms leading to the bone pathology in Gaucher disease in relationship with the proinflammatory state.

  4. Meta-analysis of individual registry results enhances international registry collaboration.

    Science.gov (United States)

    Paxton, Elizabeth W; Mohaddes, Maziar; Laaksonen, Inari; Lorimer, Michelle; Graves, Stephen E; Malchau, Henrik; Namba, Robert S; Kärrholm, John; Rolfson, Ola; Cafri, Guy

    2018-03-28

    Background and purpose - Although common in medical research, meta-analysis has not been widely adopted in registry collaborations. A meta-analytic approach in which each registry conducts a standardized analysis on its own data followed by a meta-analysis to calculate a weighted average of the estimates allows collaboration without sharing patient-level data. The value of meta-analysis as an alternative to individual patient data analysis is illustrated in this study by comparing the risk of revision of porous tantalum cups versus other uncemented cups in primary total hip arthroplasties from Sweden, Australia, and a US registry (2003-2015). Patients and methods - For both individual patient data analysis and meta-analysis approaches a Cox proportional hazard model was fit for time to revision, comparing porous tantalum (n = 23,201) with other uncemented cups (n = 128,321). Covariates included age, sex, diagnosis, head size, and stem fixation. In the meta-analysis approach, treatment effect size (i.e., Cox model hazard ratio) was calculated within each registry and a weighted average for the individual registries' estimates was calculated. Results - Patient-level data analysis and meta-analytic approaches yielded the same results with the porous tantalum cups having a higher risk of revision than other uncemented cups (HR (95% CI) 1.6 (1.4-1.7) and HR (95% CI) 1.5 (1.4-1.7), respectively). Adding the US cohort to the meta-analysis led to greater generalizability, increased precision of the treatment effect, and similar findings (HR (95% CI) 1.6 (1.4-1.7)) with increased risk of porous tantalum cups. Interpretation - The meta-analytic technique is a viable option to address privacy, security, and data ownership concerns allowing more expansive registry collaboration, greater generalizability, and increased precision of treatment effects.

  5. The CIELO collaboration: Progress in international evaluations of neutron reactions on Oxygen, Iron, Uranium and Plutonium

    Directory of Open Access Journals (Sweden)

    Chadwick M.B.

    2017-01-01

    Full Text Available The CIELO collaboration has studied neutron cross sections on nuclides that significantly impact criticality in nuclear technologies – 16O, 56Fe, 235,8U and 239Pu – with the aim of improving the accuracy of the data and resolving previous discrepancies in our understanding. This multi-laboratory pilot project, coordinated via the OECD/NEA Working Party on Evaluation Cooperation (WPEC Subgroup 40 with support also from the IAEA, has motivated experimental and theoretical work and led to suites of new evaluated libraries that accurately reflect measured data and also perform well in integral simulations of criticality.

  6. The CIELO Collaboration: Progress in International Evaluations of Neutron Reactions on Oxygen, Iron, Uranium and Plutonium

    Energy Technology Data Exchange (ETDEWEB)

    Chadwick, M. B.; Capote, R.; Trkov, A.; Kahler, A. C.; Herman, M. W.; Brown, D. A.; Hale, G. M.; Pigni, M.; Dunn, M.; Leal, L.; Plompen, A.; Schillebeecks, P.; Hambsch, F. -J.; Kawano, T.; Talou, P.; Jandel, M.; Mosby, S.; Lestone, J.; Neudecker, D.; Rising, M.; Paris, M.; Nobre, G. P. A.; Arcilla, R.; Kopecky, S.; Giorginis, G.; Cabellos, O.; Hill, I.; Dupont, E.; Danon, Y.; Jing, Q.; Zhigang, G.; Tingjin, L.; Hanlin, L.; Xichao, R.; Haicheng, W.; Sin, M.; Bauge, E.; Romain, P.; Morillon, B.; Salvatores, M.; Jacqmin, R.; Bouland, O.; De Saint Jean, C.; Pronyaev, V. G.; Ignatyuk, A.; Yokoyama, K.; Ishikawa, M.; Fukahori, T.; Iwamoto, N.; Iwamoto, O.; Kuneada, S.; Lubitz, C. R.; Palmiotti, G.; Kodeli, I.; Kiedrowski, B.; Roubtsov, D.; Thompson, I.; Quaglioni, S.; Kim, H. I.; KLee, Y. O.; Koning, A. J.; Carlson, A.; Fischer, U.

    2016-11-01

    The CIELO collaboration has studied neutron cross sections on nuclides that significantly impact criticality in nuclear technologies - 16O, 56Fe, 235,8U and 239Pu - with the aim of reducing uncertainties and resolving previous discrepancies in our understanding. This multi-laboratory pilot project, coordinated via the OECD/NEA Working Party on Evaluation Cooperation (WPEC) Subgroup 40 with support also from the IAEA, has motivated experimental and theoretical work and led to suites of new evaluated libraries that accurately reflect measured data and also perform well in integral simulations of criticality.

  7. Joining forces: collaborating internationally to deliver high-quality, online postgraduate education in pain management.

    Science.gov (United States)

    Devonshire, Elizabeth; Siddall, Philip

    2011-01-01

    The effective management of pain is a complex and costly global issue, requiring a range of innovative educational strategies to enable culturally appropriate and high-quality health care provision. In response to this issue, the Pain Management Research Institute at the University of Sydney (Sydney, Australia) has established several strategic alliances with other overseas universities to deliver online postgraduate education in pain management. The present article discusses the rationale for joining forces, and the approach adopted in creating and maintaining these alliances. It also provides insights into the benefits, challenges and opportunities associated with collaborative educational initiatives of this nature, from institutional, academic and student perspectives.

  8. CIELO Collaboration Summary Results: International Evaluations of Neutron Reactions on Uranium, Plutonium, Iron, Oxygen and Hydrogen

    Science.gov (United States)

    Chadwick, M. B.; Capote, R.; Trkov, A.; Herman, M. W.; Brown, D. A.; Hale, G. M.; Kahler, A. C.; Talou, P.; Plompen, A. J.; Schillebeeckx, P.; Pigni, M. T.; Leal, L.; Danon, Y.; Carlson, A. D.; Romain, P.; Morillon, B.; Bauge, E.; Hambsch, F.-J.; Kopecky, S.; Giorginis, G.; Kawano, T.; Lestone, J.; Neudecker, D.; Rising, M.; Paris, M.; Nobre, G. P. A.; Arcilla, R.; Cabellos, O.; Hill, I.; Dupont, E.; Koning, A. J.; Cano-Ott, D.; Mendoza, E.; Balibrea, J.; Paradela, C.; Durán, I.; Qian, J.; Ge, Z.; Liu, T.; Hanlin, L.; Ruan, X.; Haicheng, W.; Sin, M.; Noguere, G.; Bernard, D.; Jacqmin, R.; Bouland, O.; De Saint Jean, C.; Pronyaev, V. G.; Ignatyuk, A. V.; Yokoyama, K.; Ishikawa, M.; Fukahori, T.; Iwamoto, N.; Iwamoto, O.; Kunieda, S.; Lubitz, C. R.; Salvatores, M.; Palmiotti, G.; Kodeli, I.; Kiedrowski, B.; Roubtsov, D.; Thompson, I.; Quaglioni, S.; Kim, H. I.; Lee, Y. O.; Fischer, U.; Simakov, S.; Dunn, M.; Guber, K.; Márquez Damián, J. I.; Cantargi, F.; Sirakov, I.; Otuka, N.; Daskalakis, A.; McDermott, B. J.; van der Marck, S. C.

    2018-02-01

    The CIELO collaboration has studied neutron cross sections on nuclides that significantly impact criticality in nuclear technologies - 235,238U, 239Pu, 56Fe, 16O and 1H - with the aim of improving the accuracy of the data and resolving previous discrepancies in our understanding. This multi-laboratory pilot project, coordinated via the OECD/NEA Working Party on Evaluation Cooperation (WPEC) Subgroup 40 with support also from the IAEA, has motivated experimental and theoretical work and led to suites of new evaluated libraries that accurately reflect measured data and also perform

  9. Institutional Framework for Collaborative Urban Planning in Afghanistan in view of the Transferring Process of International Urban Planning Systems

    Directory of Open Access Journals (Sweden)

    Habib Ahmad Javid

    2015-09-01

    Full Text Available This article provides an overview of Afghanistan’s urban planning institutional change in certain historical periods, particular dilemmas within the current urban planning system and its gradual shift from totalitarian urban planning approaches practiced during 1960s - 1980s to a different form of planning being practiced by the current government. In addition, it will seek to analyze the ease and tension caused by the three recent phenomena that have emerged after the establishment of a new democratic government in Afghanistan since 2001, such as private sector-led urban development, international funding community’s and NGOs’ role in planning and the delegation of certain roles given to different tires of the government. Another purpose of this work is to analyze the collaboration among urban planning institutions, private sector, international funding community, NGOs and civil society within the current urban planning arena of Afghanistan and to identify the roles, responsibilities and functions of urban planning institutions in different levels of urban governance. Finally find out what possible and necessary institutional changes and framework are needed in order to foster grassroots based inter-institutional collaboration and partnership among various tires of government. The methodological approach to the research is based on qualitative data analysis. For the analysis purpose, government urban planning data and in-depth, semi-structured, face-to-face interviews with Afghanistan’s urban planning officials were thematically used, which provided in-depth information about involved actors in urban planning and their roles and relationships.

  10. PRINCESS: Privacy-protecting Rare disease International Network Collaboration via Encryption through Software guard extensionS.

    Science.gov (United States)

    Chen, Feng; Wang, Shuang; Jiang, Xiaoqian; Ding, Sijie; Lu, Yao; Kim, Jihoon; Sahinalp, S Cenk; Shimizu, Chisato; Burns, Jane C; Wright, Victoria J; Png, Eileen; Hibberd, Martin L; Lloyd, David D; Yang, Hai; Telenti, Amalio; Bloss, Cinnamon S; Fox, Dov; Lauter, Kristin; Ohno-Machado, Lucila

    2017-03-15

    We introduce PRINCESS, a privacy-preserving international collaboration framework for analyzing rare disease genetic data that are distributed across different continents. PRINCESS leverages Software Guard Extensions (SGX) and hardware for trustworthy computation. Unlike a traditional international collaboration model, where individual-level patient DNA are physically centralized at a single site, PRINCESS performs a secure and distributed computation over encrypted data, fulfilling institutional policies and regulations for protected health information. To demonstrate PRINCESS' performance and feasibility, we conducted a family-based allelic association study for Kawasaki Disease, with data hosted in three different continents. The experimental results show that PRINCESS provides secure and accurate analyses much faster than alternative solutions, such as homomorphic encryption and garbled circuits (over 40 000× faster). https://github.com/achenfengb/PRINCESS_opensource. shw070@ucsd.edu. Supplementary data are available at Bioinformatics online. © The Author 2016. Published by Oxford University Press. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com

  11. Evaluation of neopterin as a biomarker for the monitoring of Gaucher disease patients.

    Science.gov (United States)

    Drugan, Cristina; Drugan, Tudor C; Miron, Nicolae; Grigorescu-Sido, Paula; Naşcu, Ioana; Cătană, Cristina

    2016-07-01

    Biomarker research is an important area of investigation in Gaucher disease, caused by an inherited deficiency of a lysosomal enzyme, glucocerebrosidase. We evaluated the usefulness of neopterin, as a novel biomarker reflecting chronic inflammation and immune system activation in Gaucher disease and analysed its evolution in response to enzyme replacement therapy (ERT). Circulating plasma neopterin levels in 31 patients with non-neuronopathic Gaucher disease were measured before and after the onset of ERT and were compared with those of 18 healthy controls. Plasma chitotriosidase activity was also monitored, as a reference biomarker, against which we evaluated the evolution of neopterin. Neopterin levels were significantly increased in treatment-naïve patients (mean 11.90 ± 5.82 nM) compared with controls (6.63 ± 5.59 nM, Mann-Whitney U test P = 0.001), but returned to normal levels (6.92 ± 4.66 nM) following ERT. Investigating the diagnostic value of neopterin by receiver operating characteristic analysis, we found a cut-off value of 7.613 nM that corresponds to an area under the curve of 0.780 and indicates a good discrimination capacity, with a sensitivity of 0.774 and a specificity of 0.778. Our results suggest that measurement of circulating neopterin may be considered as a novel test for the confirmation of diagnosis and monitoring of the efficacy of therapeutic intervention in Gaucher disease. Plasma neopterin levels reflect the global accumulation and activation of Gaucher cells and the extent of chronic immune activation in this disorder. Neopterin may be an alternative storage cell biomarker in Gaucher disease, especially in chitotriosidase-deficient patients.

  12. Novel insertion mutation in a non-Jewish Caucasian type 1 Gaucher disease patient

    Energy Technology Data Exchange (ETDEWEB)

    Choy, F.Y.M.; Humphries, M.L. [Univ. of Victoria, British Columbia (Canada); Ferreira, P. [Univ. of Alberta, Edmonton (Canada)

    1997-01-20

    Gaucher disease is the most prevalent lysosomal storage disorder. It is autosomal recessive, resulting in lysosomal glucocerebrosidase deficiency. Three clinical forms of Gaucher disease have been described: type 1 (nonneuronopathic), type 2 (acute neuronopathic), and type 3 (subacute neuronopathic). We performed PCR-thermal cycle sequence analysis of glucocerebrosidase genomic DNA and identified a novel mutation in a non-Jewish type 1 Gaucher disease patient. It is a C insertion in exon 3 at cDNA nucleotide position 122 and genomic nucleotide position 1626. This mutation causes a frameshift and, subsequently, four of the five codons immediately downstream of the insertion were changed while the sixth was converted to a stop codon, resulting in premature termination of protein translation. The 122CC insertion abolishes a Cac81 restriction endonuclease cleavage site, allowing a convenient and reliable method for detection using RFLP analysis of PCR-amplified glucocerebrosidase genomic DNA. The mutation in the other Gaucher allele was found to be an A{r_arrow}G substitution at glucocerebrosidase cDNA nucleotide position 1226 that so far has only been reported among type 1 Gaucher disease patients. Since mutation 122CC causes a frameshift and early termination of protein translation, it most likely results in a meaningless transcript and subsequently no residual glucocerebrosidase enzyme activity. We speculate that mutation 122CC may result in a worse prognosis than mutations associated with partial activity. When present in the homozygous form, it could be a lethal allele similar to what has been postulated for the other known insertion mutation, 84GG. Our patient, who is a compound heterozygote 122CC/1226G, has moderately severe type 1 Gaucher disease. Her clinical response to Ceredase{reg_sign} therapy that began 31 months ago has been favorable, though incomplete. 30 refs., 3 figs., 2 tabs.

  13. Report on International Collaboration Involving the FE Heater and HG-A Tests at Mont Terri

    Energy Technology Data Exchange (ETDEWEB)

    Houseworth, Jim [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Rutqvist, Jonny [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Asahina, Daisuke [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Chen, Fei [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Vilarrasa, Victor [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Liu, Hui-Hai [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States); Birkholzer, Jens [Lawrence Berkeley National Lab. (LBNL), Berkeley, CA (United States)

    2013-11-01

    Nuclear waste programs outside of the US have focused on different host rock types for geological disposal of high-level radioactive waste. Several countries, including France, Switzerland, Belgium, and Japan are exploring the possibility of waste disposal in shale and other clay-rich rock that fall within the general classification of argillaceous rock. This rock type is also of interest for the US program because the US has extensive sedimentary basins containing large deposits of argillaceous rock. LBNL, as part of the DOE-NE Used Fuel Disposition Campaign, is collaborating on some of the underground research laboratory (URL) activities at the Mont Terri URL near Saint-Ursanne, Switzerland. The Mont Terri project, which began in 1995, has developed a URL at a depth of about 300 m in a stiff clay formation called the Opalinus Clay. Our current collaboration efforts include two test modeling activities for the FE heater test and the HG-A leak-off test. This report documents results concerning our current modeling of these field tests. The overall objectives of these activities include an improved understanding of and advanced relevant modeling capabilities for EDZ evolution in clay repositories and the associated coupled processes, and to develop a technical basis for the maximum allowable temperature for a clay repository.

  14. An International Collaboration in Nursing Education Viewed through the Lens of Critical Social Theory.

    Science.gov (United States)

    Ekstrom, David N.; Sigurdsson, Hrafn Oli

    2002-01-01

    An international educational exchange program involving nursing students was examined using Habermas' theory of communicative action. Politics and economics were found to inhibit active communication and the potential benefits of shared understanding through interaction. (Contains 20 references.) (SK)

  15. An international collaborative family-based whole genome quantitative trait linkage scan for myopic refractive error

    DEFF Research Database (Denmark)

    Abbott, Diana; Li, Yi-Ju; Guggenheim, Jeremy A

    2012-01-01

    To investigate quantitative trait loci linked to refractive error, we performed a genome-wide quantitative trait linkage analysis using single nucleotide polymorphism markers and family data from five international sites....

  16. DEVELOPMENT ACQUIREMENTS AND WORKING LIFE COLLABORATION OF INTERNATIONAL HIGHER EDUCATION STUDENTS : Case: Digital Business Development

    OpenAIRE

    Tuukkanen, Laura; Pudas, Sini

    2014-01-01

    This thesis is commissioned by Mikkeli University of Applied Sciences, Business Department. The research is a case study of an intensive study module Digital Business Development held in August 2014. The purpose of the thesis was to investigate international higher education students’ development acquirements during a three week Digital Business Development course. The research also investigated the success of partnership between Mamk, MPY and international higher education students in th...

  17. Combination therapy in a patient with chronic neuronopathic Gaucher disease: a case report.

    Science.gov (United States)

    Ceravolo, Ferdinando; Grisolia, Michele; Sestito, Simona; Falvo, Francesca; Moricca, Maria Teresa; Concolino, Daniela

    2017-01-20

    The variants of neuronopathic Gaucher disease may be viewed as a clinical phenotypic continuum divided into acute and chronic forms. The chronic neuronopathic form of Gaucher disease is characterized by a later onset of neurological symptoms and protracted neurological and visceral involvement. The first-choice treatment for nonneuronopathic Gaucher disease is enzyme replacement therapy with recombinant analogues of the deficient human enzyme glucocerebrosidase. Enzyme replacement therapy has been shown to improve hematological and bone manifestations associated with Gaucher disease, but, as with most proteins, recombinant enzymes cannot cross the blood-brain barrier, which prevents effects on neurological manifestations. Substrate reduction therapy with miglustat (N-butyldeoxynojirimycin) inhibits glucosylceramide synthase, which catalyzes the first step in glycosphingolipid synthesis. Because miglustat can cross the blood-brain barrier, it has been suggested that, combined with enzyme replacement therapy, it might be effective in treating neurological symptoms in patients with neuronopathic Gaucher disease. We report observed effects of combined enzyme replacement therapy and substrate reduction therapy in a 7-year-old Caucasian boy with neuronopathic Gaucher disease who was homozygous for L444P mutations. He had received enzyme replacement therapy from the age of 18 months, and concomitant miglustat treatment was commenced, with dosing according to body surface area uptitrated over 1 month with dietary modifications when he reached the age of 30 months. He experienced mild diarrhea after commencing miglustat therapy, which decreased in frequency/severity over time. His splenomegaly was reduced, and his hematological values and plasma angiotensin-converting enzyme activity normalized. Plasma chitotriosidase also showed substantial and sustained decreases. After 5 years of combination therapy, the patient showed no signs of neurological impairment. This case

  18. Hemorrhage associated with 'bone crisis' in Gaucher's disease identified by magnetic resonance imaging

    International Nuclear Information System (INIS)

    Horev, G.; Kornreich, L.; Hadar, H.; Katz, K.

    1991-01-01

    Children suffering from Gaucher's disease were examined by magnetic resonance imaging (MRI) during a characteristic episode of 'bone crisis'. An unexpectedly high intramedullary as well as subperiosteal signal was observed on both the T1- and T2-weighted sequences in 5 patients, suggesting a subacute hemorrhage or hematoma. It is conceivable that such a painful hemorrhage is an important component of the 'bone crisis' phenomenon. Furthermore, in these cases this is a specific sign which may enable differentiation of bone crises from other types of bone pain associated with Gaucher's disease. (orig.)

  19. Gaucher disease in the liver on hepatocyte specific contrast agent enhanced MR imaging

    International Nuclear Information System (INIS)

    Ayyala, Rama S.; Teot, Lisa A.; Perez Rossello, Jeanette M.

    2017-01-01

    Gaucher disease is a hereditary lipid storage disorder that affects the enzyme beta glucocerebrosidase, causing accumulation of glucocerebroside in macrophages of the reticuloendothelial system. Accumulation can occur in the liver and spleen, manifesting as hepatosplenomegaly, as well as within the bone marrow. Hepatic involvement is usually diffuse but can occasionally manifest as focal liver lesions. We present a case of a 2-year-old boy with Gaucher disease and an infiltrating liver lesion detected on imaging, which was pathologically shown to be focal changes related to the disease. Imaging characteristics of this lesion using hepatocyte specific contrast agent enhanced MRI, which have not been previously discussed in the literature, are described. (orig.)

  20. Gaucher disease in the liver on hepatocyte specific contrast agent enhanced MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Ayyala, Rama S. [Morgan Stanley Children' s Hospital, Department of Radiology, Columbia University Medical Center, New York, NY (United States); Teot, Lisa A. [Boston Children' s Hospital, Department of Pathology, Harvard Medical School, Boston, MA (United States); Perez Rossello, Jeanette M. [Boston Children' s Hospital, Department of Radiology, Harvard Medical School, Boston, MA (United States)

    2017-04-15

    Gaucher disease is a hereditary lipid storage disorder that affects the enzyme beta glucocerebrosidase, causing accumulation of glucocerebroside in macrophages of the reticuloendothelial system. Accumulation can occur in the liver and spleen, manifesting as hepatosplenomegaly, as well as within the bone marrow. Hepatic involvement is usually diffuse but can occasionally manifest as focal liver lesions. We present a case of a 2-year-old boy with Gaucher disease and an infiltrating liver lesion detected on imaging, which was pathologically shown to be focal changes related to the disease. Imaging characteristics of this lesion using hepatocyte specific contrast agent enhanced MRI, which have not been previously discussed in the literature, are described. (orig.)

  1. The International Charter for Human Values in Healthcare: an interprofessional global collaboration to enhance values and communication in healthcare.

    Science.gov (United States)

    Rider, Elizabeth A; Kurtz, Suzanne; Slade, Diana; Longmaid, H Esterbrook; Ho, Ming-Jung; Pun, Jack Kwok-hung; Eggins, Suzanne; Branch, William T

    2014-09-01

    The human dimensions of healthcare--core values and skilled communication necessary for every healthcare interaction--are fundamental to compassionate, ethical, and safe relationship-centered care. The objectives of this paper are to: describe the development of the International Charter for Human Values in Healthcare which delineates core values, articulate the role of skilled communication in enacting these values, and provide examples showing translation of the Charter's values into action. We describe development of the Charter using combined qualitative research methods and the international, interprofessional collaboration of institutions and individuals worldwide. We identified five fundamental categories of human values for every healthcare interaction--Compassion, Respect for Persons, Commitment to Integrity and Ethical Practice, Commitment to Excellence, and Justice in Healthcare--and delineated subvalues within each category. We have disseminated the Charter internationally and incorporated it into education/training. Diverse healthcare partners have joined in this work. We chronicle the development and dissemination of the International Charter for Human Values in Healthcare, the role of skilled communication in demonstrating values, and provide examples of educational and clinical programs integrating these values. The Charter identifies and promotes core values clinicians and educators can demonstrate through skilled communication and use to advance humanistic educational programs and practice. Copyright © 2014. Published by Elsevier Ireland Ltd.

  2. The International Cancer Benchmarking Partnership: an international collaboration to inform cancer policy in Australia, Canada, Denmark, Norway, Sweden and the United Kingdom.

    Science.gov (United States)

    Butler, John; Foot, Catherine; Bomb, Martine; Hiom, Sara; Coleman, Michel; Bryant, Heather; Vedsted, Peter; Hanson, Jane; Richards, Mike

    2013-09-01

    The International Cancer Benchmarking Partnership (ICBP) was initiated by the Department of Health in England to study international variation in cancer survival, and to inform policy to improve cancer survival. It is a research collaboration between twelve jurisdictions in six countries: Australia (New South Wales, Victoria), Canada (Alberta, British Columbia, Manitoba, Ontario), Denmark, Norway, Sweden, and the United Kingdom (England, Northern Ireland, Wales). Leadership is provided by policymakers, with academics, clinicians and cancer registries forming an international network to conduct the research. The project currently has five modules examining: (1) cancer survival, (2) population awareness and beliefs about cancer, (3) attitudes, behaviours and systems in primary care, (4) delays in diagnosis and treatment, and their causes, and (5) treatment, co-morbidities and other factors. These modules employ a range of methodologies including epidemiological and statistical analyses, surveys and clinical record audit. The first publications have already been used to inform and develop cancer policies in participating countries, and a further series of publications is under way. The module design, governance structure, funding arrangements and management approach to the partnership provide a case study in conducting international comparisons of health systems that are both academically and clinically robust and of immediate relevance to policymakers. Copyright © 2013 The Authors. Published by Elsevier Ireland Ltd.. All rights reserved.

  3. Clinical evaluation of chitotriosidase enzyme activity in Gaucher and Niemann Pick A/B diseases: A retrospective study from India.

    Science.gov (United States)

    Kadali, Srilatha; Kolusu, Anusha; Sunkara, Satish; Gummadi, Maheshwar Reddy; Undamatla, Jayanthi

    2016-06-01

    Plasma chitotriosidase originates from activated macrophages and is reported to be elevated in many Lysosomal Storage Disorders. Measurement of this enzyme activity has been an available tool for monitoring therapy of Gaucher disease. The degree of elevation of chitotriosidase is useful for differential diagnosis of Gaucher disease and Niemann Pick A/B. However the potential utility of this chitotriosidase assay depends on the frequency of deficient chitotriosidase activity in a particular population. We therefore aim to study the clinical utility of this assay Gaucher and Niemann Pick A/B diseases in the backdrop of chitotriosidase deficiency in our population. The study comprises 173 patients with clinical suspicion of either Gaucher disease (n=108) or Niemann Pick A/B (n=65) and 92 healthy controls. The plasma samples of controls, Gaucher disease, and Niemann Pick A/B showed chitotriosidase deficiency of 12%, 25% and 27% respectively. The degree of elevation of chitotriosidase in Gaucher disease and Niemann Pick A/B patients is 40-326 (11,325.7±6395.4nmol/h/ml) and 7-22 folds (1192.5±463.0nmol/h/ml) respectively. In view of these findings of distinguishable fold elevation of chitotriosidase in Gaucher disease or Niemann Pick A/B, it can be a potential surrogate differential diagnostic marker for these groups of diseases, except in the patients in whom this enzyme is deficient. Copyright © 2016 Elsevier B.V. All rights reserved.

  4. Gaucher disease: MR evaluation of bone marrow features during treatment with enzyme replacement; Morbus Gaucher: Analyse der Knochenmarkveraenderungen in der MRT waehrend Enzymersatztherapie

    Energy Technology Data Exchange (ETDEWEB)

    Poll, L.W.; Koch, J.A.; Boerner, D.; Cohnen, M.; Jung, G.; Scherer, A.; Moedder, U. [Duesseldorf Univ. (DF). Inst. fuer Diagnostische Radiologie; Dahl, S. vom; Haeussinger, D. [Duesseldorf Univ. (Germany). Klinik fuer Gastroenterologie, Hepatologie und Infektiologie; Willers, R. [Rechenzentrum, Heinrich-Heine-Univ. Duesseldorf (Germany); Niederau, C. [Innere Abt., St. Josef-Hospital Oberhausen, Akademisches Lehrkrankenhaus der Univ. Essen (Germany)

    2001-10-01

    Purpose: Enzyme replacement therapy (ERT) arrests and reverses the hematological and visceral symptoms of adult Gaucher disease, the most frequent lysosomal storage disorder. There are only a few studies available evaluating bone disease during ERT. The aim of this study was to investigate the features of bone marrow (bm) by magnetic resonance imaging (MRI) in these patients during ERT. Materials and Methods: MRI was performed prospectively in thirty adult type I Gaucher patients before and during ERT with a mean follow-up of 3 years. Spin-echo sequences (T{sub 1}/T{sub 2}) of the lower extremities were obtained and the reconversion (response) or lack of reconversion (non-response) to fatty marrow during treatment was analyzed. The morphological features of bm involvement, a homogeneous or non-homogeneous distribution of bm changes and focal bone lesions surrounded by a rim of reduced signal intensity (SI), were analyzed. Results: Infiltration of bm by Gaucher cells is characterized by a reduction of Sl on both T{sub 1}- and T{sub 2}-weighted sequences. Bone marrow responses were seen in 19 patients (63%) during treatment. Focal bone lesions, surrounded by a rim of reduced Sl, did not respond to ERT and correlated with a non-homogenous distribution of bone involvement and splenectomy. (orig.) [German] Ziel: Unter Enzymersatztherapie (enzyme replacement therapy = ERT) zeigen Patienten mit adulter Form des Morbus Gaucher, der haeufigsten lysosomalen Speicherkrankheit, eine deutliche Besserung der haematologischen und visceralen Symptome. Bislang liegen nur wenige Untersuchungen zur Analyse der Knochenveraenderungen waehrend der ERT vor. Ziel war es, die Knochenmarkveraenderungen bei Gaucher-Patienten waehrend der Enzymersatztherapie mit Alglucerase/Imiglucerase in der Magnetresonanztomographie (MRT) zu evaluieren. Material und Methoden: In einer prospektiven Untersuchung wurden 30 adulte Patienten mit gesichertem Morbus Gaucher vor und waehrend der ERT in der MRT

  5. SEMINAR ABOUT SERIOUS GAMES AND VIRTUAL WORLDS: An Experience of International Collaboration And Reflection

    Directory of Open Access Journals (Sweden)

    Wolfram LAASER

    2011-08-01

    Full Text Available The educational possibilities of ICT, dizzying and exponentially growing every day, offer multiple alternatives of mediation for teaching, learning and communication.Thus, the inclusion of video games and virtual worlds into educational context represents a qualitative leap that claims to significantly boost ways of communication and knowledge representation of the scenarios involved. Aware of this reality, in the framework of the Master of Technology Enhanced Learning at the National University of Cordoba, Argentina, a virtual seminar was offered to students to address the issue on the basis of invited lectures of worldwide recognized experts. The format chosen for the seminar allowed the treatment of subjects not only through reading assignments and web quests to be discussed collaboratively but also included the state of the art experience of developers working in the field. The paper describes didactic design and technical solutions of the seminar format.

  6. International Collaboration on Building Local Technical Capacities for Monitoring Volcanic Activity at Pacaya Volcano, Guatemala.

    Science.gov (United States)

    Escobar-Wolf, R. P.; Chigna, G.; Morales, H.; Waite, G. P.; Oommen, T.; Lechner, H. N.

    2015-12-01

    Pacaya volcano is a frequently active and potentially dangerous volcano situated in the Guatemalan volcanic arc. It is also a National Park and a major touristic attraction, constituting an important economic resource for local municipality and the nearby communities. Recent eruptions have caused fatalities and extensive damage to nearby communities, highlighting the need for risk management and loss reduction from the volcanic activity. Volcanic monitoring at Pacaya is done by the Instituto Nacional de Sismologia, Vulcanologia, Meteorologia e Hidrologia (INSIVUMEH), instrumentally through one short period seismic station, and visually by the Parque Nacional Volcan de Pacaya y Laguna de Calderas (PNVPLC) personnel. We carry out a project to increase the local technical capacities for monitoring volcanic activity at Pacaya. Funding for the project comes from the Society of Exploration Geophysicists through the Geoscientists Without Borders program. Three seismic and continuous GPS stations will be installed at locations within 5 km from the main vent at Pacaya, and one webcam will aid in the visual monitoring tasks. Local educational and outreach components of the project include technical workshops on data monitoring use, and short thesis projects with the San Carlos University in Guatemala. A small permanent exhibit at the PNVPLC museum or visitor center, focusing on the volcano's history, hazards and resources, will also be established as part of the project. The strategy to involve a diverse group of local collaborators in Guatemala aims to increase the chances for long term sustainability of the project, and relies not only on transferring technology but also the "know-how" to make that technology useful. Although not a primary research project, it builds on a relationship of years of joint research projects at Pacaya between the participants, and could be a model of how to increase the broader impacts of such long term collaboration partnerships.

  7. Inhibition of UDP-glucosylceramide synthase in mice prevents Gaucher disease-associated B-cell malignancy.

    Science.gov (United States)

    Pavlova, Elena V; Archer, Joy; Wang, Susan; Dekker, Nick; Aerts, Johannes Mfg; Karlsson, Stefan; Cox, Timothy M

    2015-01-01

    Clonal B-cell proliferation is a frequent manifestation of Gaucher disease - a sphingolipidosis associated with a high risk of multiple myeloma and non-Hodgkin lymphoma. Gaucher disease is caused by genetic deficiency of acid β-glucosidase, the natural substrates of which (β-d-glucosylceramide and β-d-glucosylsphingosine) accumulate, principally in macrophages. Mice with inducible deficiency of β-glucosidase [Gba(tm1Karl/tm1Karl)Tg(MX1-cre)1Cgn/0] serve as an authentic model of human Gaucher disease; we have recently reported clonal B-cell proliferation accompanied by monoclonal serum paraproteins and cognate tumours in these animals. To explore the relationship between B-cell malignancy and the biochemical defect, we treated Gaucher mice with eliglustat tartrate (GENZ 112638), a potent and selective inhibitor of the first committed step in glycosphingolipid biosynthesis. Twenty-two Gaucher mice received 300 mg/kg of GENZ 112638 daily for 3-10 months from 6 weeks of age. Plasma concentrations of β-d-glucosylceramide and the unacylated glycosphingolipid, β-d-glucosylsphingosine, declined. After administration of GENZ 112638 to Gaucher mice for 3-10 months, serum paraproteins were not detected and there was a striking reduction in the malignant lymphoproliferation: neither lymphomas nor plasmacytomas were found in animals that had received the investigational agent. In contrast, 14 out of 60 Gaucher mice without GENZ 112638 treatment developed these tumours; monoclonal paraproteins were detected in plasma from 18 of the 44 age-matched mice with Gaucher disease that had not received GENZ 112638. Long-term inhibition of glycosphingolipid biosynthesis suppresses the development of spontaneous B-cell lymphoma and myeloma in Gaucher mice. Copyright © 2014 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  8. Color Discrimination in Patients with Gaucher Disease and Parkinson Disease.

    Science.gov (United States)

    Simon-Tov, Shlomi; Dinur, Tama; Giladi, Nir; Bar-Shira, Anat; Zelis, Mayaan; Zimran, Ari; Elstein, Deborah

    2015-01-01

    Poor color discrimination among patients with Parkinson disease (PD) has long been recognized. It has been shown that carrying one or two mutations in the β-glucocerebrosidase gene (GBA) for the autosomal disease Gaucher disease (GD), as based initially on clinical evidence, is a genetic risk factor for early-onset PD. The purpose of this study was to assess color discrimination in patients with one or two GBA mutations relative to healthy controls to ascertain whether this function is affected when persons with GD or even one GBA mutation develop PD. The Farnsworth-Munsell 100 hue test (FMHT) was evaluated among patients with GD+PD compared to patients with GD only, obligate GBA carriers with and without PD, patients with PD only, and healthy controls. FMHT outcome include computer-generated TES (Total Error Score) and values recommended by Vingrys & King-Smith. Six groups of 10 persons were tested. Significant differences were seen for male GD+PD and for age in PD. The highest mean TES was in the PD only group, the lowest in the GD only group. There was a significant difference because of PD in groups with GD and GBA carriers. GD+PD means were between GD only and PD only mean scores. These findings confirm that PD impacts color discrimination, more in males with GD+PD but nonetheless, GD+PD patients (but not GBA carriers) had better scores than PD only patients.

  9. Gauchers disease--a reappraisal of hematopoietic stem cell transplantation.

    Science.gov (United States)

    Ito, Sawa; Barrett, A John

    2013-03-01

    Hematopoietic stem cell transplantation (HSCT), first performed in 1984, was the first treatment approach for Gaucher's disease (GD) which had curative intent. The early successes in HSCT were soon eclipsed by the introduction of a highly effective enzyme replacement therapy (ERT), which has remained the single most widely used treatment. Experience with HSCT is limited to about 50 reported cases, mainly performed in the last century, with an overall survival around 85%. HSCT typically achieves complete correction of visceral and bony changes and can fully stabilize neurological features in otherwise progressive type II and III GD. ERT, in contrast, is completely safe and effective, but is limited by cost, incomplete resolution of visceral, hematological, and bony features in some patients, and lack of neurological correction in type II and III disease. In this review, we summarize and compare HSCT and ERT. With 20 years of experience of ERT, its limitations as well as its advantages are now well delineated. Meanwhile progress in HSCT over the last decade suggests that transplantation would today represent a very safe curative approach for GD offering one time complete correction of the disease, contrasting with the lifelong need for ERT with its associated expense and dependence on sophisticated drug manufacture. Additionally, unlike ERT, HSCT can be beneficial for neurological forms of GD. We conclude that the time has come to re-evaluate HSCT in selected patients with GD where ERT is less likely to fully eradicate symptoms of the disease.

  10. International energy technology collaboration and climate change mitigation. Case study 2. Cooperation in Agriculture. R and D on High-Yielding Crop Varieties

    Energy Technology Data Exchange (ETDEWEB)

    Gagnon-Lebrun, F. [Global and Structural Policies Division, Organisation for Economic Co-operation and Development OECD, Paris (France)

    2004-07-01

    Mitigating climate change and achieving stabilisation of greenhouse gas atmospheric concentrations will require deep reductions in global emissions of energy-related carbon dioxide emissions. Developing and disseminating new, low-carbon energy technology will thus be needed. Two previous AIXG papers have focused on possible drivers for such a profound technological change: Technology Innovation, Development and Diffusion, released in June 2003, and International Energy Technology Collaboration and Climate Change Mitigation, released in June 2004. The first of these papers assesses a broad range of technical options for reducing energy-related CO2 emissions. It examines how technologies evolve and the role of research and development efforts, alternative policies, and short-term investment decisions in making long-term options available. It considers various policy tools that may induce technological change, some very specific, and others with broader expected effects. Its overall conclusion is that policies specifically designed to promote technical change, or 'technology push', could play a critical role in making available and affordable new energy technologies. However, such policies would not be sufficient to achieve the Convention's objective in the absence of broader policies. First, because there is a large potential for cuts that could be achieved in the short run with existing technologies; and second, the development of new technologies requires a market pull as much as a technology push. The second paper considers the potential advantages and disadvantages of international energy technology collaboration and transfer for promoting technological change. Advantages of collaboration may consist of lowering R and D costs and stimulating other countries to invest in R and D; disadvantage may include free-riding and the inefficiency of reaching agreement between many actors. This paper sets the context for further discussion on the role of

  11. Ties That Bind International Research Teams: A Network Multilevel Model of Interdisciplinary Collaboration

    Science.gov (United States)

    Kollasch, Aurelia Wiktoria

    2012-01-01

    Today large research projects require substantial involvement of researchers from different organizations, disciplines, or cultures working in groups or teams to accomplish a common goal of producing, sharing, and disseminating scientific knowledge. This study focuses on the international research team that was launched in response to pressing…

  12. Nomograms for predicting survival and recurrence in patients with adenoid cystic carcinoma. An international collaborative study

    DEFF Research Database (Denmark)

    Ganly, Ian; Amit, Moran; Kou, Lei

    2015-01-01

    BACKGROUND: Due to the rarity of adenoid cystic carcinoma (ACC), information on outcome is based upon small retrospective case series. The aim of our study was to create a large multiinstitutional international dataset of patients with ACC in order to design predictive nomograms for outcome. METH...

  13. A Collaborative Programming and Outreach Model for International Student Support Offices

    Science.gov (United States)

    Briggs, Peter; Ammigan, Ravichandran

    2017-01-01

    Increasing international student enrollment has been a key priority for many institutions of higher education in the United States. Such recruitment efforts, however, are often carried out without much consideration for providing sufficient support services to these students once they arrive to campus. This article proposes a model for structuring…

  14. An update on electronic records at CERN (internal developments, collaboration and outsourcing)

    CERN Document Server

    Hollier, A

    2008-01-01

    This paper, presented at the "Future Proof IV" International Conference on scientific archives (Royal Swedish Academy of Sciences, 23-25 April 2008), gives an update on some activities related to the long-term preservation of electronic records at CERN.

  15. The Rise of Global Science and the Emerging Political Economy of International Research Collaborations

    Science.gov (United States)

    Peters, Michael A.

    2006-01-01

    This article charts the rise of global science and a global science infrastructure as part of the emerging international knowledge system exemplifying a geography of knowledge and the importance of new info-communications networks. The article theorises the rise of global science, which still strongly reflects a Western bias and is highly…

  16. Imiglucerase in the management of Gaucher disease type 1: an evidence-based review of its place in therapy

    Science.gov (United States)

    Serratrice, Christine; Carballo, Sebastian; Serratrice, Jacques; Stirnemann, Jérome

    2016-01-01

    Introduction Gaucher disease is the first lysosomal disease to benefit from enzyme replacement therapy, thus serving as model for numerous other lysosomal diseases. Alglucerase was the first glucocerebrosidase purified from placental extracts, and this was then replaced by imiglucerase – a Chinese hamster ovary cell-derived glucocerebrosidase. Aim The aim was to review the evidence underlying the use of imiglucerase in Gaucher disease type 1 Evidence review Data from clinical trials and Gaucher Registries were analyzed. Conclusion Imiglucerase has been prescribed and found to have an excellent efficacy and safety profile. We report herein the evidence-based data published for 26 years justifying the use of imiglucerase. PMID:27790078

  17. The Tourette International Collaborative Genetics (TIC Genetics) study, finding the genes causing Tourette syndrome: objectives and methods.

    Science.gov (United States)

    Dietrich, Andrea; Fernandez, Thomas V; King, Robert A; State, Matthew W; Tischfield, Jay A; Hoekstra, Pieter J; Heiman, Gary A

    2015-02-01

    Tourette syndrome (TS) is a neuropsychiatric disorder characterized by recurrent motor and vocal tics, often accompanied by obsessive-compulsive disorder and/or attention-deficit/hyperactivity disorder. While the evidence for a genetic contribution is strong, its exact nature has yet to be clarified fully. There is now mounting evidence that the genetic risks for TS include both common and rare variants and may involve complex multigenic inheritance or, in rare cases, a single major gene. Based on recent progress in many other common disorders with apparently similar genetic architectures, it is clear that large patient cohorts and open-access repositories will be essential to further advance the field. To that end, the large multicenter Tourette International Collaborative Genetics (TIC Genetics) study was established. The goal of the TIC Genetics study is to undertake a comprehensive gene discovery effort, focusing both on familial genetic variants with large effects within multiply affected pedigrees and on de novo mutations ascertained through the analysis of apparently simplex parent-child trios with non-familial tics. The clinical data and biomaterials (DNA, transformed cell lines, RNA) are part of a sharing repository located within the National Institute for Mental Health Center for Collaborative Genomics Research on Mental Disorders, USA, and will be made available to the broad scientific community. This resource will ultimately facilitate better understanding of the pathophysiology of TS and related disorders and the development of novel therapies. Here, we describe the objectives and methods of the TIC Genetics study as a reference for future studies from our group and to facilitate collaboration between genetics consortia in the field of TS.

  18. International Collaboration: the Virtuous Cycle of Low Carbon Innovation and Diffusion. An Analysis of Solar Photovoltaic, Concentrating Solar Power and Carbon Capture and Storage

    International Nuclear Information System (INIS)

    Dominique, Katheen

    2010-01-01

    International collaboration can be leveraged to accelerate the innovation and diffusion of low carbon technologies required to realize the shift to a low carbon trajectory. A collaborative approach to innovation has the potential to capture several benefits, including: pooling risks and achieving scale; knowledge sharing that accommodates competition and cooperation; the creation of a global market; facilitation of policy learning and exchange; and the alignment of technology, finance and policy. International Collaboration: the Virtuous Cycle of Low Carbon Innovation and Diffusion An Analysis of Solar Photovoltaic, Concentrating Solar Power and Carbon Capture and Storage A range of obstacles to the diffusion of low carbon technologies provides ample opportunity for international collaboration in global market creation and capacity building, expanding beyond conventional modes of technology transfer. Current collaborative efforts for carbon capture and storage, solar photovoltaic and concentrating solar power technologies are active in all stages of innovation and diffusion and involve a wide range of actors. Yet, current efforts are not sufficient to achieve the necessary level of emission mitigation at the pace required to avoid catastrophic levels of atmospheric destabilization. This analysis sets forth recommendation to scale up current endeavors and create new ones. The analysis begins by describing the fundamental characteristics of innovation and diffusion processes that create opportunities for international collaboration. It then illustrates a broad array of on-going collaborative activities, depicting how these efforts contribute to innovation and diffusion. Finally, highlighting the gap between the current level of collaborative activities and technology targets deemed critical for emission mitigation, the report sets forth several recommendations to build on current efforts and construct new endeavors

  19. Ethical challenges for international collaborative research partnerships in the context of the Zika outbreak in the Dominican Republic: a qualitative case study.

    Science.gov (United States)

    Canario Guzmán, Julio Arturo; Espinal, Roberto; Báez, Jeannette; Melgen, Ricardo Elias; Rosario, Patricia Antonia Pérez; Mendoza, Eddys Rafael

    2017-09-25

    The establishment of international collaborative research partnerships in times of infectious disease outbreaks of international importance has been considered an ethical imperative. Frail health research systems in low- and middle-income countries can be an obstacle to achieve the goal of knowledge generation and the search for health equity before, during and after infectious disease outbreaks. A qualitative case study was conducted to identify the challenges and opportunities facing the Dominican Republic with regards to developing international collaborative research partnerships in the context of the Zika outbreak and its ethical implications. Researchers conducted 34 interviews (n = 30 individual; n = 4 group) with 39 participants (n = 23 males; n = 16 females) representing the government, universities, international donor agencies, non-governmental organisations, community-based organisations and medical societies, in two metropolitan cities. Five international collaborative research projects related to the Zika virus were identified. Major ethical challenges were linked to the governance of health research, training of human resources, the institutionalisation of scientific activity, access to research funds and cultural aspects. Capacity-building was not necessarily a component of some partnership agreements. With few exceptions, local researchers were merely participating in data collection and less on defining the problem. Opportunities for collaborative work included the possibility of participation in international research consortiums through calls for proposals. The Dominican government and research stakeholders can contribute to the international response to the Zika virus through active participation in international collaborative research partnerships; however, public recognition of the need to embrace health research as part of public policy efforts is warranted. A working group led by the government and formed by national and

  20. International collaborative project to compare and track the nutritional composition of fast foods

    Directory of Open Access Journals (Sweden)

    2012-07-01

    Full Text Available Abstract Background Chronic diseases are the leading cause of premature death and disability in the world with over-nutrition a primary cause of diet-related ill health. Excess quantities of energy, saturated fat, sugar and salt derived from fast foods contribute importantly to this disease burden. Our objective is to collate and compare nutrient composition data for fast foods as a means of supporting improvements in product formulation. Methods/design Surveys of fast foods will be done in each participating country each year. Information on the nutrient composition for each product will be sought either through direct chemical analysis, from fast food companies, in-store materials or from company websites. Foods will be categorized into major groups for the primary analyses which will compare mean levels of saturated fat, sugar, sodium, energy and serving size at baseline and over time. Countries currently involved include Australia, New Zealand, France, UK, USA, India, Spain, China and Canada, with more anticipated to follow. Discussion This collaborative approach to the collation and sharing of data will enable low-cost tracking of fast food composition around the world. This project represents a significant step forward in the objective and transparent monitoring of industry and government commitments to improve the quality of fast foods.