bluuebunny/crossref_metadata_2025 huggingface dataset:
Reproducible and Sustained Efficacy of Targeted Therapy With Vemurafenib in Patients With BRAFV600E-Mutated Erdheim-Chester Disease
Authors: Haroche Julien , Cohen-Aubart Fleur , Emile Jean-François , Maksud Philippe , Drier Aurélie , Tolédano Dan , Barete Stéphane , Charlotte Frédéric , Cluzel Philippe , Donadieu Jean , Benameur Neïla , Grenier Philippe A. , Besnard Sophie , Ory Jean-Paul , Lifermann François , Idbaih Ahmed , Granel Brigitte , Graffin Bruno , Hervier Baptiste , Arnaud Laurent , Amoura Zahir
Published: November 2014

Purpose

Histiocytoses are rare disorders with heterogeneous prognosis. BRAFV600E mutations have been observed in half of patients with Langerhans cell histiocytosis (LCH) and in 50% to 100% of patients with Erdheim-Chester disease (ECD) patients. We recently reported short-term efficacy of a BRAF inhibitor (vemurafenib) in three patients with multisystemic ECD.

Patients and Methods

Vemurafenib was given to eight patients with multisystemic ECD with CNS and/or cardiac involvement. All patients were refractory to first-line treatment and harbored a BRAFV600E mutation. Four patients also had LCH lesions. Positron emission tomography (PET) scan response at month 6 was used as the main evaluation criterion. Secondary evaluation criteria were comparison at baseline and at last visit of PET and of cardiovascular and cerebral infiltrations (computed tomography scan and magnetic resonance imaging [MRI]).

Results

All patients were partial metabolic responders at 6 months of vemurafenib, and the median reduction in maximum standardized uptake value was 63.5% (range, 41.3% to 86.9%). Evaluation of cardiac and aortic infiltrations showed that seven patients had a partial response and one patient had stable disease according to surface measurements derived from RECIST criteria. The four patients with infratentorial CNS infiltration had an objective decrease of the lesions on MRI. All patients had an improvement of general symptoms and a persistent response to vemurafenib, with a median follow-up time of 10.5 months (range, 6 to 16 months). Skin adverse effects were frequent and severe.

Conclusion

Vemurafenib has an objective and sustained efficacy in BRAFV600E-mutated ECD as second-line therapy. In contrast to melanoma, no resistance has emerged to date after 6 to 16 months.
Comparison of Breast Cancer Recurrence and Outcome Patterns Between Patients Treated From 1986 to 1992 and From 2004 to 2008
Authors: Cossetti Rachel J.D. , Tyldesley Scott K. , Speers Caroline H. , Zheng Yvonne , Gelmon Karen A.
Published: November 2014

Purpose

To determine whether the patterns of relapse according to estrogen receptor (ER) and human epidermal growth factor receptor 2 (HER2) status changed in the contemporary era.

Patients and Methods

Female patients referred to the British Columbia Cancer Agency with biopsy-proven stage I to III breast cancer (BC), diagnosed between 1986 and 1992 (cohort 1 [C1]) and between mid-2004 and 2008 (cohort 2 [C2]), and with known ER and HER2 status were eligible. Data were prospectively collected. C2 patients were matched to C1 patients for stage, grade, and ER and HER2 status. The primary end point was hazard rate of relapse (HRR) for BC by study cohort according to biomarker status. Secondary outcomes included HRR according to stage, grade, and age and hazard rate of death (HRD).

Results

After matching, 7,178 patients were included (3,589 patients in each cohort). BC subtype distribution was as following ER positive/HER2 negative, 70.8%; ER positive/HER2 positive, 6.9%; ER negative/HER2 positive, 6.6%; and ER negative/HER2 negative, 15.8%. For the overall population, the HRR approximately halved in all yearly intervals to year 9 in C2 compared with C1. Differences in HRR between cohorts were greater in the initial five intervals for HER2-positive and ER-negative/HER2-negative BC. The HRR decreased in C2 compared with C1 for all disease stages and grades. The HRD in C2 also decreased compared with C1, although to a lesser extent.

Conclusion

Although the pattern of relapse remains similar, there has been a significant improvement in BC relapse-free survival. Outcomes have improved for all BC subtypes, especially HER2-positive and ER-negative/HER2-negative BC, with the early spike in disease recurrence markedly decreased. These contemporary hazard rates are important for treatment decisions, patient discussions, and planning clinical trials of early BC.
On the Correspondence between 2D Force Networks and Polyhedral Stress Functions
Authors: Fraternali Fernando , Carpentieri Gerardo
Published: November 2014

We formulate and discuss the relationship between polyhedral stress functions and internally self-equilibrated frameworks in 2D, and a two-mesh technique for the prediction of the stress field associated with such systems. We generalize classical results concerned with smooth Airy stress functions to polyhedral functions associated with arbitrary triangulations of a simply-connected domain. We also formulate a regularization technique that smoothly projects the stress function corresponding to an unstructured force network over a structured triangulation. The paper includes numerical examples dealing with a benchmark problem of plane elasticity, and the stress fields associated with truss models of a cantilever beam and an elliptical membrane.
STEM projects and activities
Authors: Williams Sarah
Published: November 2014

Birches Head Academy prizes its focus on STEM education, including its role as a lead school with the Computing at School initiative, supporting delivery of the computing curriculum. Sarah Williams discusses some of their activities
The Social and Political Identities of the Shi'i Community in Lebanon
Authors: Siklawi Rami
Published: November 2014

Since the early days of the Shi'i presence in Lebanon, the Shi'i community has been considered the most marginalized and underprivileged community. This had been the case of the Shi'ites during the Mamluk period, the Ottoman period, the French mandate period, and again during the post-independence period. This article addresses the Shi'ite presence in Lebanon until the eve of Lebanese Civil War (1975). The article will explore the various aspects of socio-political identities of the Shi'ites and how that changed and developed during the Ottoman period, the French mandate, and the independence period by elaborating on the main actors and processes/phases that shaped this change. It has been argued that the marginalization and the neglect policies that were applied against the Shi'ites opened the wide doors for the Shi'i community to migrate to the urban cities in Lebanon (mainly Beirut) starting from the late nineteenth and early twentieth century, and this had also been expanded to the rest of the world (mainly America and West Africa). This migration played a key role in developing their contemporary social and political identities in which Musa al-Sadr and his movement played an important part.
Commercial sport – Debordian spectacle or Barthesian mythology?
Authors: Møller Verner , Genz Jakob
Published: November 2014

Concern over the corrupting effects of modern sport has been voiced since its birth in the nineteenth century. Its critics have deemed it unhealthy, called it fascist, claimed that it created false conscience, etc. Its advocates, on the other hand, have praised sport’s ability to build character and facilitate understanding and respect between people and nations. But the sport advocates have not been univocal in their appraisal. They have expressed concern that the sporting values have come under threat from professionalization and increased commercialization of sport. In this article, however, we make the case that modern-day sport has qualities that help ground us in the world and, in fact, civilizes. The article starts by exposing the outdatedness of the sociologist John J. MacAloon’s ‘festival nostalgia’. It goes on to take issue with Guy Debord’s criticism of the disempowerment of the people caused by what he calls the ‘society of the spectacle’. Using soccer as the prime example, this is followed by a discussion of the fundamental elements of the fascination exerted by sport and of sport’s significance as modern mythology. We argue that the media-driven commercialization, often looked upon as the root cause of sport’s unfortunate development, is, in fact, part of the engine that propels it forward as a project for the development of popular culture.
Sport and the neo-liberal world order
Authors: Nauright John
Published: November 2014

Sport is a huge global enterprise worth nearly $150 billion annually. As a result international federations and other stakeholders are keen to maintain the veneer of integrity and morality in an idealized vision of sport. The value of sport and what it means to the operation of global sports federations and associated enterprises, most notably the International Olympic Committee (IOC); The International Federation of Football Associations (FIFA); and the World Anti-Doping Agency (WADA), is discussed. In this short viewpoint article I analyse the political economy underpinning the ‘war’ on athletes being waged at present in the name of ‘pure sport’. I argue that ‘pure sport’ and ‘level playing field’ are ideologies used to maintain the power position of major international sporting federations rather than an actual goal of these federations.
Chameleon hair: How hair’s materiality affects its fashionability
Authors: Holmes Helen
Published: November 2014

This article seeks to explore the changeable materiality of hair. Drawing upon the archaeological concept of the palimpsest, as an inimitable material record, it illuminates how hair’s chameleon abilities are the foundation upon which the contemporary hair fashion industry resides. As Nigel Thrift notes, hair ‘is the easiest part of the body to alter. It grows so must be cut’. However, paradoxically, it is hair’s very materiality that also inhibits its conformity to certain fashions. While hair may appear to have chameleon materiality, it has multiple temporalities. And as this article argues, hair is simultaneously changing and changed, but it displays constant features.
As we may publish: Digital scholarship and the future(s) of art education
Authors: Knochel Aaron D. , Patton Ryan M.
Published: November 2014

Qualities of digital scholarship for the arts and humanities are outlined as an ecology of research and pedagogy, expanding the scholarly journal by advancing the possibilities of online publishing. While moving away from paper-based scholarship is the first step, new models of digital scholarship that are multimodal and interactive allow for real-time effects, reflexive research, reconsider intellectual openness through a radical sense of accessibility, and broadly (re)define the textual body in research to one that is transdisciplinary. In this article, we elaborate on the current trends and opportunities in digital scholarship presented by online publications as an extension of research modalities, augmenting what art education research publications look like, presenting new and rich contexts for scholarship. We explore limitations and benefits found in the current state of academic publishing by asking how scholarship can be transformed in a technological ecology.
(Mis)Information highways: A critique of online resources for multicultural art education
Authors: Acuff Joni Boyd
Published: November 2014

This article calls to attention hegemonic online resources for multicultural art education. The author suggests that art educators carefully critique multicultural art lesson plans published online, as the Internet is increasingly a primary resource teachers use to make pedagogical and curricular decisions. The author demonstrates how some multicultural art education resources offered online contribute to an ‘us–other’ dichotomy, and contradict with the current progressive critical multicultural art education scholarship being published by contemporary art education scholars. The author asserts three contentions of support that illustrate how these online curricular resources maintain ‘liberal’ multiculturalism, exoticize cultural groups, produce surface knowledge about difference and fail to question power. This article concludes with a call to action in which art educators are encouraged to explicitly acknowledge, discuss and work with students and peers to build counter-curriculum that work against these damaging online multicultural art education resources.
Integrating Taiwanese culture into design pedagogy
Authors: Hsieh Hsiu Ching Laura
Published: November 2014

Many Taiwanese students enthusiastically accept the consumption cultures of Japan and the United States, but neglect the value of their own culture. Thus, as a design educator, it is necessary to guide the students reflecting on and identifying with their own culture. This pedagogical study adopted qualitative methods and collected and analysed data from interviews, observation on the creation process and related documents. The purposes of this research were: to explore the related research and theories and integrate them as a model of Incorporating Culture into Design Creation; to explore the advantages and challenges of applying the new model to pedagogical practice; and to reflect on the application of the new model to teaching. According to the findings, it is feasible to apply this model to the design pedagogy. The contribution of this study was to encourage the students to recognize the characteristics of their own culture and facilitate their capabilities of incorporating culture into the design creation.
Empathy and aesthetic experience in the art museum
Authors: Arnold Alice , Meggs Susan Martin , Greer Annette G.
Published: November 2014

The purpose of this study was to explore how students’ aesthetic understanding and ability to empathize were impacted by multiple learning experiences in the environment of an art museum. As part of a semester-long class in elementary art methods, education majors experienced a docent-led tour of the local art museum, featuring the work of fibre artist Deidre Scherer. The tour was followed by a series of learning events centred on the exhibit and the study of end-of-life care, the theme of Scherer’s work. Students then answered structured questionnaires prompting them to process the learning events with intentional reflection. A qualitative method of enquiry sought to uncover the lived experiences of the students enrolled in the class. Through these encounters, students developed empathy and understanding as well as the ability to describe the meanings of the art in terms of their own life scenarios.
Role-playing games in arts, research and education
Authors: Cox Jason Matthew
Published: November 2014

Games are increasingly being used as tools to broaden pedagogical options in schools and create interdisciplinary linkages. This article pays particular attention to role-playing games (RPGs), and the applications they have for arts education. RPGs can be used in art classrooms and in arts-based research and as an exciting and useful form of collaborative art-making, interpersonal discourse and reflection. This article is divided into sections describing the games and the manner in which it has been applied to arts education. Interspersed within the article are reflections from the author’s experience in a game that examines the politics of oppression called the The Tribunal, which resulted in introspection on both personal and societal issues. The article concludes that RPGs can help make both personal and communal meaning of experiences and foster the development of empathy.
Review
Authors: Westron Loree
Published: November 2014

Naming the unnameable

‘I Am Your Brother’ Short Story Studies, Charles E. May (2013) Available by print-on-demand, 277 pp., ISBN: 9781490347615, p/bk, £9.35

Morphology, Ra Page (ed.) (2013) Manchester: Comma Press, 204 pp., ISBN: 9781905583485, p/bk, £9.99
Mentoring the contemporary arts student in the university
Authors: Daichendt G. James , Funk Clayton , Swift Jason
Published: November 2014

The roles of professional artists and educators are established and defined by mentors in the field. In addition, they have a large impact on students, and can have a powerful influence on future artists. Our position as mentors becomes one of furthering the knowledge base of the field and passing it down to the next generation. However, we know mentorship to be essential but it is also often misunderstood. Educating, motivating or helping others to become more effective are characteristics that can be developed in university art and art education programmes through the process of mentoring, whether in the classroom, studio, online, or institutions like museums or galleries. Despite the process of mentorship trending outside the traditional classroom, this was not always the case in the history of art education and much can be done through collaboration and effective communication to develop the mentoring process. This article explores the changing landscape of mentoring in the university and considers the deskilled context that favours developing competencies through the concepts of accidental mentoring and mentoring through collaboration.
Art’s asymptotic leadership: Arts leadership, education and the loss of autonomy
Authors: Baldacchino John
Published: November 2014

This article will mostly engage with arts leadership through a discussion that focuses on the arts, leadership and education, and how their convergence might have a direct impact on autonomy. Taking a meta-theoretical approach, the main argument is that arts leadership is an asymptotic state of affairs. Rather than pose art and leadership as antithetical events that necessitate forms of syntheses through identifiable contexts, the context for arts leadership represents a contiguous space where art and leadership continuously seek a mutual way of preserving their integrity in an asymptotic relationship. If this relationship turns into a synthesis, both art’s autonomy and the ability to lead creatively are neutralized. The aim is to question the various implications that bring together the autonomous spheres of the arts, education and leadership, while inviting the reader to draw his or her own conclusions critically and autonomously. To clarify this approach, this article straddles across several horizons, including: arts practice as a sphere of autonomous dispositions and the political implications that follow; education as a horizon that educes – leads out – through the pedagogical exits that are offered by the arts; and art’s anti-systemic pedagogy, where art’s autonomy becomes a possibility of unlearning systems.
Servant leadership in the creative academy
Authors: Hafeli Mary
Published: November 2014

How do the characteristics and behaviours of creative people mesh with their roles as faculty members? How do the opportunities and demands of today’s college environments play into the work lives of arts faculty? And what are the implications of these questions for thinking about how best to lead artists and arts scholars in colleges and universities? This article explores these questions through an examination of servant leadership, an approach that focuses on service as an underlying principle of leadership. Intertwined with a discussion of creative people and optimal characteristics of those who lead them, I describe current conditions of colleges and universities and what being a college leader may be like. I then present characteristics of servant leaders and attempt to draw some parallels between servant leadership behaviours and needs of faculty and students in the arts. Finally, I summarize some potentially positive outcomes of servant leadership as well as some of the model’s possible drawbacks and challenges.
Teaching art where art should not exist: Women’s empowerment in Saudi Arabia
Authors: Congdon Kristin G.
Published: November 2014

Saudi Arabia is perhaps the most conservative country in the Middle East. The display of art is not allowed, or extremely limited, under Sharia law. This article explores a three-week adult art education class taught to women at the Al Baylasan Art Center in Al Khobar, Saudi Arabia. With the goal of empowering women, it challenged two American art educators to develop and present a curriculum that was respectful of the culture but allowed the students to push the limits of their thinking about what might be possible in their changing cultural context.
SummerVision DC: An evolving Professional Learning Community (PLC) explores art museums and nurtures the nurturer
Authors: Sandell Renee , Henry Carole
Published: November 2014

This article discusses the National Art Education Association’s (NAEA) SummerVision DC program, which combines blended learning with ongoing professional development grounded in an intensive four-day expeditionary engagement, exploring diverse museums as vital learning sites while building a professional learning community (PLC). To comprehend the interactive nature of the SummerVision DC experience and its creation of optimal conditions for growing a supportive and professional learning community (PLC) within the museum environment, this article reviews key stages in the evolving process – from virtual preparation/anticipation to actual learning experiences, revealing the interweaving of the preparatory work with museum educators and participants, the hands-on/on-site experiences, the collaboration and relationship building, the time for reflection, the opportunity to apply what one is learning and walk away with lessons that can be used in the classroom, the use of technology to expand and extend the learning (before the event), and the community connections (after the event) that are continued and expanded through social media with ongoing NAEA support for collaborative networking and insightful and creative leadership.
A challenge for art education: Understanding leadership and creating new leaders for public policy development, educational partnerships and teacher training programmes
Authors: Sabol F. Robert
Published: November 2014

The fields of general education and art education are in an unprecedented period of change. Educational leaders at all instructional levels have experienced new challenges, problems and opportunities that required higher levels of creativity, innovation and visionary thinking than ever before. Advances in neuroscience, creativity studies, charter schools, technology, national curriculum standards, high-stake assessments of learning, teacher evaluations and numerous other developments have contributed to making leadership development a priority in all school districts and communities. Opportunities for leadership development and professional learning are limited. In order for the field of art education to meet these growing leadership challenges, the field needs to increase its emphasis on leadership development for all art educators. Those in positions of leadership must engage in and emphasize policy studies in art education. At a fundamental level, art educators and leaders must refine and develop their understanding of the nature and structures of leadership. In order for these things to occur across the nation, creation of professional development opportunities must be made available on an ongoing basis so that art educators can continue to develop and refine their skills as leaders. Among the full array of leadership development needs in art education, there are significant opportunities for leadership growth and involvement in the areas of public policy, educational partnerships and teacher training programmes. Concentrating leadership development in these key areas holds potential for producing leadership outcomes that can positively influence art education in all schools and communities across the country.
O ESTÁGIO COMO ESPAÇO DE INVESTIGAÇÃO DA SOCIALIZAÇÃO DO EXERCÍCIO DOCENTE
Authors: Sambugari Márcia Regina Do Nascimento
Published: November 2014

Este artigo discute o estágio como espaço de investigação da socialização do exercício docente por meio da pesquisa sobre a socialização entre alunos estagiários do curso de Pedagogia de uma instituição pública de ensino superior do interior do estado de Mato Grosso do Sul e professoras da escola básica. Partiu-se da problemática do enfrentamento da formação dos alunos em um percurso anterior, durante o qual já adquiriram modos de pensar e apreciar o exercício docente como alunos em toda a vida familiar, escolar e social, em face do que vão vivenciar nas salas de aula durante o estágio. Apresenta o caminho teórico-metodológico percorrido, que consistiu em exercício analítico na construção do conceito de socialização, sob a perspectiva da sociologia da educação. Os dados foram coletados por meio de questionário para caracterização dos sujeitos, sessões de observação nos momentos de estágio de quatro acadêmicos e as respectivas professoras que os receberam em sua sala de aula, gravação das reuniões de orientação de estágio na Universidade, registro dos diários de estágio e realização de entrevista. A análise apontou o quanto os percursos de cada um marcam suas vidas, traduzindo modos de pensar e agir a partir dessas vivências, provocando diversidades na socialização, ainda que com certa regularidade, advindas dessas características sociais e pessoais. Dessa maneira, as ideias e informações sobre a docência transmitidas pelas professoras aos futuros professores provocaram diversas reações.http://dx.doi.org/10.14572/nuances.v20i21.1099
The etymology of rune
Authors: Mees Bernard
Published: November 2014

The etymology of the term
Die zunehmende Profilierung der Schreibsilbe in der Geschichte des Deutschen
Authors: Fuhrhop Nanna , Schmidt Karsten
Published: November 2014

Concepts like the written syllable, the featural analysis of letter forms and the length hierarchy have led to stunning insights over the last few years. However, they are still very much disputed. The present paper investigates a number of historical developments in the German writing system with regard to the length hierarchy. Many of these changes can be understood (exclusively) as an increasing profiling of the written syllable.
Kritik des Apparats
Authors: Kragl Florian
Published: November 2014

On the occasion of the new ›Laurin‹-edition, this article focuses on the phenomenon of the critical apparatus. Philologists have taken serious efforts in re-examining the standards of editing medieval texts in general over the last decades. However, the apparatus itself has received far less attention. The article discusses the consequences of this disequilibrium for contemporary editions. It proposes a new approach to apparatus design, which takes into account the manifold textual situations we encounter in medieval transmission.
Reflexive differential forms on singular spaces.
Geometry and cohomology
Authors: Greb Daniel , Kebekus Stefan , Peternell Thomas
Published: November 2014

Based on a recent extension theorem for reflexive differential forms, that is,
regular differential forms defined on the smooth locus of a possibly singular
variety, we study the geometry and cohomology of sheaves of reflexive
differentials.

First, we generalise the extension theorem to holomorphic forms on locally
algebraic complex spaces. We investigate the (non-)existence of reflexive
pluri-differentials on singular rationally connected varieties, using a
semistability analysis with respect to movable curve classes. The necessary
foundational material concerning this stability notion is developed in an
appendix to the paper. Moreover, we prove that Kodaira–Akizuki–Nakano
vanishing for sheaves of reflexive differentials holds in certain extreme
cases, and that it fails in general. Finally, topological and Hodge-theoretic
properties of reflexive differentials are explored.
Holomorphic one-forms, integral and rational points on complex hyperbolic surfaces
Authors: Yeung Sai-Kee
Published: November 2014

The first goal of this paper is to study the question of finiteness of integral points on
a cofinite non-compact complex two-dimensional ball quotient defined over a number field. Along the process
we will also consider some negatively curved compact surfaces. Using some fundamental results of Faltings, the question is to reduce to a conjecture of Borel
about existence of virtual holomorphic one-forms on cofinite non-cocompact complex ball quotients. The second goal of this paper is to study the conjecture on such non-compact surfaces.
Semiotic Challenges in India
Authors: Khanwalkar Seema
Published: November 2014

Is India’s legacy of intellectual supremacy at odds with the vast landscape of the everyday? In the context of Modernity, everything social, cultural and political is subject to interpretative filters. How do we make sense of any of these, or all of these? Several slippages of meanings, several residues, and lengthy ‘liminal’ spaces characterize the semiotic landscape of contemporary India. Do we understand negotiations, between the past, the present and the continuous of some of the significant cultural discourses in contemporary India as dialogues? Dialogue is also a genre of discourse and can be seen as the modality itself of thought The roles of the traditional postulates like ‘Maya’, ‘Dharma’ and ‘Rasa’ for example as cosmological schema, readily translatable interpretative frameworks in contemporary discourses has often been questioned by critical theory in modern India. Critics are also of the view that, in Rasa theory, the human onlooker is just a locus and not an active agent in what is a global alchemy of properties and perceptions. The same, according to them, can be said of Dharma as an inhuman theodicy that often exists over and above the movements of detection, discovery, reasoning and proof in modern methods of justice.
Intertextuality in Writing Under Lotman’s Late Text View
Authors: Hu Yuanyan
Published: November 2014

Writing is a dialogic practice of which the essence lies in intertextuality. Taking intertextuality theory and Lotman’s late text view, which is in harmony with the essence of intertextuality theory as the theoretical basis, this paper aims to introduce four types of texts involved during the writing process, namely, intertext, consisting of both schema text and exterior text, reader text, context text and the individualized text referring to the final output of writer and also the offspring of the intertextuality of the aforementioned three texts. Moreover, the paper briefly analyzes the intertextual relationships among these four texts in the hope of raising more interest in intertextual practice and intertextual awareness of writing.
Subjectivity, Suprasubjectivity, and Semiosis
Authors: Deely John
Published: November 2014

That what underlies the possibility of semiosis in the first place at any level is the irreducibility of relations to subjectivity (including intersubjectivity), together with the singularity of relations as the only form of being capable of being positively verified to exist whether the existence is an awarenessdependent or awareness-independent occurrence, remains to be generally understood. Yet just this understanding, along with appreciation of the irreducible triad (significate, representamen, interpretant) unified by any given relation of a semiosic character, is required to explain the vis a prospecto uniqueness of signs as able to transmit through present circumstances an influence of the future rearranging the relevance of past occurrences to what will be even though it may not be yet.
Semiosis between Action and Passion
Authors: Fernández Eliseo
Published: November 2014

All organisms are autonomous, self-organizing wholes separated by semi-permeable boundaries from a surrounding environment. Across these boundaries conveyances of action and passion are channeled through efferent and afferent pathways. I analyze this scheme in terms of two fundamental processes: semiosis and control. I propose a unified account of the functioning of semiosis and of controlling and controlled actions by viewing organisms as systems that separate their responses (actions) from the actions their environment exerts upon them (passions). Semiosis and goal-directed action are seen as complementary forms of causation. Examples from cell physiology and the functioning of efferent and afferent pathways in plants and animals illustrate and expand these ideas.

Based on this interpretation of the relations between semiosis and control I reach a generalized conception of purposeful action, linking the expansion of semiotic capacities throughout biological evolution to a concomitant increase in an organism’s powers for intervention in its environment.

The fruitfulness of these ideas is substantiated through examples showing how they make intelligible phenomena previously deemed disparate. Examples include similarities and differences between signs and instruments, and analogies in the evolution of organisms and artifacts.
Paradigm Inversion
Authors: Pearson Charls
Published: November 2014

I have previously discussed various ways of designing semiotics experiments. One method that plays a very important role is governed by the Principle of Paradigm Inversion. The word “paradigm” here refers not to what Kuhn called the overall scientific paradigm, but to what experimentalists call the experimental paradigm - the design principle of the particular experiment. In this essay, I discuss this unique design method in more detail. How do you invert an experimental paradigm? How do you turn the semiotic sock inside out? When is it possible to invert an experimental paradigm and obtain a semiotics experiment? How do you establish the measurement scale for an inverted paradigm? What kinds of experiments are ripe for inversion? What are the performance parameters of the inverted experimental design? These and other important experimental semiotics questions are discussed in this essay.
Branching processes in a Markov random environment
Authors: Dyakonova Elena E.
Published: November 2014

The paper is concerned with critical branching processes in a Markov random environment. A conditional functional limit theorem for the number of particles in a process and a conditional invariance principle are proved. The asymptotic tail behaviour for the distributions of the maximum and the total number of particles in a process is found.
Properties of Boolean functions without three-argument implicents
Authors: Goloshchapov Vladimir N. , Roldugin Pavel V.
Published: November 2014

Let F
Growth in product replacement graphs of Grigorchuk groups
Authors: Malyshev Anton , Pak Igor
Published: November 2014

The product replacement graph Γ
k
(G) is the graph on the
generating k-tuples of a group G, with edges corresponding to Nielsen moves. We prove the exponential growth of product replacement graphs Γ
k
(𝔾ω) of Grigorchuk groups, for k ≥ 5.
SKENO: Secret key encryption with non-interactive opening
Authors: Chen Jiageng , Emura Keita , Miyaji Atsuko
Published: November 2014

In this paper, we introduce the notion of secret key encryption with non-interactive opening (SKENO). With SKENO, one can make a non-interactive proof π to show that the decryption result of a ciphertext C under a shared secret key K is indeed plaintext M without revealing K itself. SKENO is the secret key analogue of public key encryption with non-interactive opening (PKENO). We give a generic construction of SKENO from verifiable random function (VRF) with certain stronger uniqueness, for example, the Hohenberger–Waters VRF and the Berbain–Gilbert I
V-dependent stream cipher construction. Although the strong primitive VRF is used, by taking advantage of the features of the stream cipher, we can still achieve good performance without sacrificing much of the efficiency. Though our VRF-based SKENO construction does not require random oracles, we show that SKENO can be constructed from weak VRF (which is strictly weaker primitive than VRF) in the random oracle model.
An additive subspace preconditioning method for the iterative solution of some problems with extreme contrasts in coefficients
Authors: Axelsson O.
Published: November 2014

-By use of an additive subspace preconditioning method it is shown how a set of small eigenvalues of a given system operator can be moved to the upper part of the spectrum, thereby significantly improving the spectral condition number and the rate of convergence of the iterative solution method used for the corresponding preconditioned system.
On the piecewise-spectral homotopy analysis method and its convergence: solution of hyperchaotic Lü system
Authors: Motsa S. S. , Nik H. Saberi , Effati S. , Saberi-Nadjafi J.
Published: November 2014

- In this paper, a novel modification of the spectral-homotopy analysis method (SHAM) technique for solving highly nonlinear initial value problems that model systems with chaotic and hyper-chaotic behaviour is presented. The proposed method is based on implementing the SHAM on a sequence of multiple intervals thereby increasing it’s radius of convergence to yield highly accuratemethod which is referred to as the piece-wise spectral homotopy analysis method (PSHAM). We investigate the application of the PSHAM to the L¨u system [20] which is well known to display periodic, chaotic and hyper-chaotic behaviour under carefully selected values of it’s governing parameters. Existence and uniqueness of solution of SHAM that give a guarantee of convergence of SHAM, has been discussed in details. Comparisons are made between PSHAMgenerated results and results from literature and Runge-Kutta generated results and good agreement is observed.
A novel ABCD1 gene mutation in a Chinese patient with X-linked adrenoleukodystrophy
Authors: Cai Yan-na , Jiang Min-yan , Liang Cui-li , Peng Min-zhi , Cheng Jing , Sheng Hui-ying , Fan Li-ping , Chen Xi-qing , Liu Li
Published: November 2014

X-linked adrenoleukodystrophy (X-ALD) (OMIM: 300100) is a recessive neurodegenerative disorder caused by defects in the

We describe an affected boy who developed normally until he was 8 years old then suffered progressive neurological deficits that ultimately led to death.

Diagnosis was based on clinical symptoms, an abnormal very long chain fatty acid profile in plasma, typical CCALD MRI pattern, and molecular analysis.

Direct sequencing of the

We have identified a novel
Mutations in BTD gene causing biotinidase deficiency: a regional report
Authors: Kasapkara Çiğdem Seher , Akar Melek , Özbek Mehmet Nuri , Tüzün Heybet , Aldudak Bedri , Baran Rıza Taner , Tanyalçın Tijen
Published: November 2014

Biotinidase deficiency is an autosomal recessive inborn error of biotin metabolism. Children with biotinidase deficiency cannot cleave biocytin and, therefore, cannot recycle biotin. Untreated individuals become secondarily biotin deficient, which in turn results in decreased activities of the biotin-dependent carboxylases and the subsequent accumulation of toxic metabolites causing clinical symptoms. Biotinidase deficiency is characterized by neurological, cutaneous manifestations and metabolic abnormalities. The worldwide incidence of profound biotinidase deficiency has been estimated at 1:112,271. The human biotinidase gene is located on chromosome 3p25 and consists of four exons with a total length of 1629 base pairs. To date, more than 100 mutations in the biotinidase gene known to cause biotinidase deficiency have been reported. The vast majority of mutations are homozygous or compound heterozygous. Finding known mutations can be correlated with the biochemical enzymatic results. This report summarizes the demographic features of patients identified as biotinidase deficient from August of 2012 through August of 2013 and mutation analysis results for 20 cases in the southeast region of Turkey.
Separation of 134Cs and 137Cs from 125I solution for medical applications
Authors: Ram Ramu , Banerjee Dayamoy , Dash Ashutosh
Published: November 2014

While neutron irradiation of natural Xe gas followed by wet chemical dissolution of activation products constitutes a successful
paradigm for the small scale production 125I, the concomitant production of 134Cs and 137Cs emerged
as the primary impediment which necessitates purification of 125I solution. This paper describes an ion-exchange
chromatographic technique using Resorcinol Formaldehyde (RF) resin to purify 125I solution from 134Cs and
137Cs impurities. A thorough investigation of the adsorption parameters of RF resin was carried out to arrive at the
experimental conditions resulting optimum retention of 134Cs and 137Cs impurities. Based on the experimental
findings, an optimized separation procedure was developed in which the neutron irradiated dissolved products at pH ∼ 13 was passed through
a chromatography column containing RF resin where in 134Cs and 137Cs impurities gets adsorbed leaving behind
125I to appear in the effluent. The overall recovery of 125I was >90% with acceptable purity
amenable for clinical applications.
Studies on extraction of actinides by unsymmetrical diamylbutyl phosphonate
Authors: Brahmmananda Rao C. V. S. , Jayalakshmi S. , Sivaraman N. , Vasudeva Rao P. R.
Published: November 2014

This paper reports the extraction behavior of uranium and thorium by diamylbutyl phosphonate (DABP). This extractant was synthesized,
characterized by various techniques like carbon and hydrogen analysis, IR, NMR (1H, 13C and 31P)
and mass spectra. This paper reports the physicochemical properties like density and viscosity of this ligand. The tracer and macro
extraction of uranium and thorium and stoichiometry of the extracted species of actinides by DABP is presented in this paper. This
compound is an unsymmetrical phosphonate and is compared with symmetrical member of the same family, diamylamyl phosphonate (DAAP) and
a member of trialkyl phosphate family, viz. tributyl phosphate (TBP).
Satzgliedfunktionen im Deutschen: eine realistische Weiterentwicklung
Authors: Neef Martin
Published: November 2014

The concept of grammatical functions has a long and changeful history. It is used in many different theoretical approaches, not least in the grammar that underlies school instruction. However, several problems and inconsistencies are connected with this concept. These problems can be solved if definitions of the individual grammatical functions are homogeneously grounded in formal criteria instead of making recourse to semantics. In this article, an axiomatic approach of this kind is presented which is couched in terms of the paradigm of Linguistic Realism. The set of definitions given affords a basis for the unambiguous analysis of (German) sentences. At the same time, the traditionally distinct concepts of grammatical functions on the one hand and valency of verbs on the other can be merged into a uniform concept. Crucial in this context is a reevaluation of the grammatical function ‘adverbial’.
Crystal growth, structural characterization and high temperature behavior of Na3TmSi3O9
Authors: Kahlenberg Volker , Mörtl Alexander , Krüger Hannes
Published: November 2014

Single-crystals of Na3TmSi3O9 were obtained in the course of systematic studies on the synthesis of rare earth element containing silicates from alkali fluoride flux systems. At 25 °C, the compound has the following basic crystallographic data: space group P212121, a = 15.1681(3) Å, b = 15.0855(3) Å, c = 14.9937(3) Å, V = 3430.83(12) Å3, Z = 16. Structure solution was based on direct methods. The subsequent refinement calculations resulted in a residual of R(∣F∣) = 0.039 for 9159 independent observed reflections with I > 2σ(I) and 579 parameters. From a structural point of view, Na3TmSi3O9 belongs to the group of single-chain silicates. The periodicity of the extremely folded chains has a value of 24. The compound is isostructural with the corresponding yttrium phase. Alternatively, the structure can be described as a mixed octahedral-tetrahedral framework, for which a detailed topological analysis is presented. Furthermore, a distinct pseudo-symmetry was observed. The sub-structure of the silicon and thulium atoms fulfills the symmetry requirements of P41212 within a few tenths of an Ångstrom. The high-temperature behavior of Na3TmSi3O9 was investigated by in-situ single-crystal X-ray diffraction in the range between 25 and 700 °C. No anomalies in the lattice parameters and unit cell volume vs. temperature curves were observed, pointing to the absence of structural phase transitions. From the evolution of the lattice parameters, the thermal expansion tensor αij has been determined. Thermal expansion shows a pronounced anisotropy. In the whole temperature region, the largest values are observed along [010] (parallel to the single-chains).
Key drivers of biomedical innovation in cancer drug discovery
Authors: Huber Margit A , Kraut Norbert
Published: November 2014

Discovery and translational research has led to the identification of a series of “cancer drivers”—genes that, when mutated or otherwise misregulated, can drive malignancy. An increasing number of drugs that directly target such drivers have demonstrated activity in clinical trials and are shaping a new landscape for molecularly targeted cancer therapies. Such therapies rely on molecular and genetic diagnostic tests to detect the presence of a biomarker that predicts response. Here, we highlight some of the key discoveries bringing precision oncology to cancer patients. Large‐scale “omics” approaches as well as modern, hypothesis‐driven science in both academic and industry settings have significantly contributed to the field. Based on these insights, we discuss current challenges and how to foster future biomedical innovation in cancer drug discovery and development.
Body composition and bone mineral density in women with Cushing's syndrome in remission and the association with common genetic variants influencing glucocorticoid sensitivity
Authors: Ragnarsson Oskar , Glad Camilla A M , Bergthorsdottir Ragnhildur , Almqvist Erik G , Ekerstad Eva , Widell Håkan , Wängberg Bo , Johannsson Gudmundur
Published: November 2014

Objective

Adverse body compositional features and low bone mineral density (BMD) are the characteristic of patients with active Cushing's syndrome (CS). The aim of this study was to evaluate body composition and BMD in women with CS in long-term remission and the influence of polymorphisms in genes affecting glucocorticoid (GC) sensitivity on these end-points.

Design, patients and methods

This was a cross-sectional, case–controlled study, including 50 women previously treated for CS and 50 age and gender-matched controls. Median (interquartile range) remission time was 13 (5–19) years. Body composition and BMD were measured with dual-energy X-ray absorptiometry. Five polymorphisms in four genes associated with GC sensitivity were analysed using TaqMan or Sequenom single-nucleotide polymorphism genotyping.

Results

Patients with CS in remission had increased abdominal fat mass (P<0.01), whereas BMD was not significantly different at any site between patients and controls. In patients, theNR3C1Bcl1 polymorphism was associated with reduced total (P<0.05) and femur neck BMD (P<0.05). The polymorphism rs1045642 in theABCB1gene was associated with increased abdominal fat mass (P<0.05) and decreased appendicular skeletal muscle mass (P<0.05). GC replacement was associated with reduced total BMD (P<0.01), BMD at lumbar spine (P<0.05) and increased abdominal fat (P<0.01).

Conclusion

Ongoing GC replacement therapy together with polymorphisms in two genes related with GC sensitivity is associated with abdominal obesity and adverse skeletal health in patients with CS in long-term remission.