Introduction: Congenital myasthenic syndromes refer to a heterogenic group of neuromuscular transmission disorders. CMS-subtypes are diverse regarding exercise intolerance and muscular weakness, varying from mild symptoms to life-limiting forms with neonatal onset. Long-term follow-up studies on disease progression and treatment-response in pediatric patients are rare.Patients and Methods: We analyzed retrospective clinical and medication data in a cohort of 32 CMS-patients including the application of a standardized, not yet validated test to examine muscular strength and endurance in 21 patients at the (...) last follow-up. Findings obtained in our cohort were compared with long-term follow-up studies of CMS-cohorts from the literature by considering the underlying molecular mechanisms. Outcomes of CMS-ST were compared to results of normal clinical assessment.Results: Thirty-two pediatric patients with defects in eight different CMS-genes were followed by a median time of 12.8 years. Fifty-nine percentage of patients manifested with first symptoms as neonates, 35% as infants. While 53% of patients presented a reduced walking distance, 34% were wheelchair-bound. Even under adequate therapy with pyridostigmine and 3,4-diaminopyridine, CHAT-mutations led to the progression of muscular weakness partly in combination with persistent respiratory and bulbar symptoms. RAPSN, CHRND, and CHRNB1 patients with neonatal manifestation, early respiratory problems, and bulbar symptoms showed a good and maintained treatment response. CHAT and CHRNE patients required higher PS dosages, whereas RAPSN patients needed a lower mean dosage at the last follow-up. The benefits of short-term medication and long-term progression of symptoms were highly dependent on the specific genetic defect. CMS-ST was carried out in 17/21 patients, determined affected muscle groups including bulbar and ocular symptoms, some of which were not reported by the patients.Conclusions: Our findings and comparison with the literature- suggest a better treatment-response and less severe progression of symptoms present in patients suffering from mutations in CMS-genes directly associated with receptor deficiency, while patients with defects leading to synaptopathy and presynaptic defects tend to have worse outcomes. Assessment of affected muscular groups and clinical symptoms by CMS-ST may be a useful tool for optimal therapeutic management of the patients, especially for future clinical studies. (shrink)

Perceptual experiences seem to in some sense have singular contents. For example, a perceptual experience of a dog as fluffy seems to represent some particular dog as being fluffy. There are important phenomenological, intuitive, and semantic considerations for thinking that perceptual experiences represent singular contents, but there are also important phenomenological, epistemic, and metaphysical considerations for thinking that they do not. This paper proposes a two-tier picture of the content of singular perceptual experiences that is based on phenomenal intentionality theories (...) of intentionality combined with self-ascriptivism about derived representation, a combination of views that allows mental states to have two types of contents: phenomenal contents and derived contents. On the proposed picture, singular perceptual experiences represent singular phenomenal contents, which do not involve worldly objects, as well as singular derived contents, which do involve worldly objects. This picture accommodates and reconciles the considerations for and against thinking that perceptual experiences have singular contents. (shrink)

Little has been studied on the relationship between affect and school problems related with attendance. This study aims to identify different affective profiles and to determine whether these profiles differ from each other based on the four functional conditions of school refusal behavior. Participants comprised 1,816 Spanish adolescents aged 15–18 years. The Positive and Negative Affect Schedule for Children-Short Form and the School Refusal Assessment Scale-Revised for Children were administered. Latent profile analysis revealed five affective profiles: low affective profile, self-fulfilling (...) profile, low positive affect profile, self-destructive profile, and high affective profile. The self-destructive profile revealed the highest average scores in the first three factors of the SRAS-R-C, whereas the high affective profile reached the highest average score in the fourth factor. On the contrary, the self-fulfilling profile obtained the lowest average scores in the first two factors of the SRAS-R-C, whereas the low affective profile revealed the lowest average scores in the last two factors. Findings suggest the relevance of developing more adaptative affective profiles, such as the self-fulfilling profile, which would contribute to diminishing school attendance problems. (shrink)

The question which this paper examines is that of the correct scope of the claim that extra-linguistic factors (such as gender and social status) can block the proper workings of natural language. The claim that this is possible has been put forward under the apt label of silencing in the context of Austinian speech act theory. The ‘silencing’ label is apt insofar as when one’s ability to exploit the inherent dynamic of language is ‘blocked’ by one’s gender or social status (...) then one might justly be said to be silenced. The notion that factors independent of any person’s linguistic competence might block her ability to exploit the inherent dynamic of language is of considerable social as well as theoretical significance. I shall defend the claim that factors independent of a person’s linguistic competence can indeed block her ability to do things with words but I will show that the cases that have been previously considered to be cases of illocutionary failure are instances of rhetic or locutionary act failure instead. I shall refine the silencing claim as previously advanced in the debate in at least one fundamental respect. I also show that considering the metaphysics of speech acts clarifies many of the issues previously appearing as thorny bones of contention between those who hold that the only notion of silencing that is coherent is that of physically preventing someone from speaking or writing and those who hold the opposite sort of claim sketched above. (shrink)

ABSTRACT As doctoral students from New Zealand and Australia, advised by supervision teams with a diversity of critical realist experience from limited to none, we came independently to the 2018 Critical Realism conference – primed to seek increased understanding, confidence, motivation, and reassurance. We certainly found these things from the pre-conference, presentations, and individuals within the critical realist community. We also found each other, and a virtual writing group was born. This article is a description of what we did, why, (...) and the outcomes we experienced over the final two years completing our theses. It identifies the structures, contexts, motivations, and mechanisms from which our emotional, critical realist and writing-related outcomes emerged. We outline the roles of serendipitous timing, culture, different disciplinary approaches, administrative structures, and types of interactions on the social learning we developed. We anchor our discussion in recent theoretical literature about the role of writing groups in doctoral education. (shrink)

We previously reported a high level of information on the Austrian organ donation law in medical and non-medical students, patients and ICU nurses, whereby ICU nurses at University Hospital in Graz (n = 185) were very well informed and also had the most critical view of the Austrian organ donation law.

This paper presents a framework for the design of human-centric identity management systems. Whilst many identity systems over the past few years have been labelled as _human-centred,_ we argue that the term has been appropriated by technologists to claim moral superiority of their products, and by system owners who confuse administrative convenience with benefits for users. The framework for human-centred identity presented here identifies a set of design properties that can impact the lived experience of the individuals whose identity is (...) being managed. These properties were identified through an analysis of public response to 15 historic national identity systems. They capture the practical design aspects of an identity system, from structural aspects that affect the flow of information - _Control Points, Subject Engagement, Identity Exposure, Population Coverage_—to the metrical aspects that considers how information is used and perceived—_Expert Interpretation, Population Comprehension, Information Accuracy, Information Stability, Subject Coupling, Information Polymorphism_. Any identity system can be described in terms of these fundamental properties, which affect individuals’ lived experience, and therefore help to determine the acceptance or rejection of such systems. We first apply each individual property within the context of two national identity systems—the UK DNA Database and the Austrian Citizen Card, and then also demonstrate the applicability of the framework within the contexts of two non-government identity platforms—Facebook and Phorm. Practitioners and researchers would make use of this framework by analysing an identity system in terms of the various properties, and the interactions between these properties within the context of use, thus allowing for the development of the potential impacts that the system has on the lived experience. (shrink)

The widespread adoption of radioisotopes as tools in biomedical research and therapy became one of the major consequences of the "physicists' war" for postwar life science. Scientists in the Manhattan Project, as part of their efforts to advocate for civilian uses of atomic energy after the war, proposed using infrastructure from the wartime bomb project to develop a government-run radioisotope distribution program. After the Atomic Energy Bill was passed and before the Atomic Energy Commission was formally established, the Manhattan Project (...) began shipping isotopes from Oak Ridge. Scientists and physicians put these reactor-produced isotopes to many of the same uses that had been pioneered with cyclotron-generated radioisotopes in the 1930s and early 1940s. The majority of early AEC shipments were radioiodine and radiophosphorus, employed to evaluate thyroid function, diagnose medical disorders, and irradiate tumors. Both researchers and politicians lauded radioisotopes publicly for their potential in curing diseases, particularly cancer. However, isotopes proved less successful than anticipated in treating cancer and more successful in medical diagnostics. On the research side, reactor-generated radioisotopes equipped biologists with new tools to trace molecular transformations from metabolic pathways to ecosystems. The U.S. government's production and promotion of isotopes stimulated their consumption by scientists and physicians, such that in the postwar period isotopes became routine elements of laboratory and clinical use. In the early postwar years, radioisotopes signified the government's commitment to harness the atom for peace, particularly through contributions to biology, medicine, and agriculture. (shrink)

Ten years ago, I stopped work as a junior doctor at a provincial New Zealand hospital and enrolled in a creative writing degree. I finished on a night shift—quiet, but marred by a particularly upsetting case of domestic violence. I remember getting changed at the end of the night into my own clothes, stuffing the scrubs I’d been wearing into the laundry bag that hung outside the doctor’s lounge, and leaving the hospital to pack for the move to a new (...) city. It was a strange way to finish, on a Sunday morning, without the colleagues in the Woman, Child and Family service with whom I’d spent so much time.For the next year, instead of ward rounds, night shifts, and on-call, I read, wrote poetry, and trekked up the hill.. (shrink)

Chez Aristophane, le public est impliqué dans le jeu comique, grâce à la disposition physique de l’espace théâtral. Il est raisonnable de penser que les femmes faisaient partie du public. Ce travail analyse les vv. 42 ss. de Lysistrata, où il semble que Cléonice s’adresse aux femmes assises dans la cavea.

Abstract In slogan form, the thesis of this paper is that beliefs are self-verifying fictions: We make them up, but in so doing, they come to exist, and so the fiction of belief is in fact true. This picture of belief emerges from a combination of three independently motivated views: (1) a phenomenal intentionalist picture of intentionality, on which phenomenal consciousness is the basis of intentionality; (2) what I will call a “self-ascriptivist” picture of derived representation, on which non-fundamental representational (...) features are a matter of our ascribing contents to ourselves or our mental states or contents; and (3) a representationalist picture of the attitudes, on which the attitude components of mental states (e.g., the “belief” bit of a belief that P) are represented contents. This paper outlines and motivates the view of beliefs as self-verifying fictions, compares the view to alternative views of belief, and contrasts beliefs on the resulting picture to other belief-like mental states. (shrink)

En el primer apartado del presente artículo busca evidenciarse la importancia del cuerpo en la práctica política a través del estudio de la antinomia vida-política, que desemboca en la configuración del Estado moderno. En este, el cuerpo individual padece sufrimiento y dolor como resultado de la lógica política de dicho contexto, cuyas estrategias se solidifican mediante las técnicas de poder denominadas anatomopolítica y biopolítica. Posteriormente, se reflexiona sobre el dolor como práctica de dominación política del gobierno neoliberal sobre la corporalidad (...) del sujeto a través de la lógica del hacer vivir dentro de lo que se concibe como el ejercicio de la normalización. Esta última se consigue a través de la vigilancia jerárquica y la sanción normalizadora, que, en oposición a Marx, Foucault no localiza en el Estado sino en la microfísica de poder. Ello permitirá concluir con la invitación a repensar la anatomopolítica como aspecto fundamental de la política y los sujetos y sujetas que la constituyen. (shrink)

The signal functions of infant crying cannot be understood properly without due attention to their ontogenetic development. Based on our own research on the development of infant cries, we argue that the controversies in cry literature will not be solved by static models, but that progress will made only when considering ontogenetic changes in interpreting cry data.

This paper describes the content and evolution of a fourth-year course for medical students on teaching pathographies of mental illness. (It is a follow-up to Nathan Carlin’s Pathographies of Mental Illness that appeared as an Element in the Bioethics and Neuroethics series published by Cambridge University Press.) The course originally centered on classic (and some contemporary) memoirs; however, responding to student evaluations, newer material now ensures more diversity, with material written by women and people of color, and describes the difference (...) that can make. (shrink)

La idea social de infancia, y la propia experiencia de ser niño o niña está interpelada en nuestros días por las mediaciones digitales en las relaciones. Es usual referirse a las nuevas generaciones como “digitales”, o particularmente como nativos digitales. Se habla de nuevas infancias en virtud del modo en que manejan las tecnologías y se plantea la idea de que la diferencia entre las generaciones adultas y jóvenes está epocalmente trastocada o invertida en una época definida como “era digital”. (...) El mandato pedagógico es, a nivel discursivo, “incluir” o “incorporar” tecnologías en la enseñanza. En este artículo nos interesa explorar la relación entre infancias y tecnologías más allá del territorio demarcado por estos discursos instrumentalistas y de cierto determinismo tecnológico, que nos proponemos poner en cuestión a partir de los avances iniciales de una investigación en curso. Nos planteamos como preguntas orientadoras: ¿cómo se re-configura la experiencia de ser niño o niña en una cultura atravesada por las tecnologías y medios digitales? ¿Cómo puede pensarse el lugar de la escuela en el marco de estas transformaciones? Se concluye subrayando algunas formas de resistencia ante los aspectos alienantes de la cultura digital que pueden pensarse como uno de los desafíos pedagógicos y políticos que se abren ante la escuela en nuestros días. (shrink)

The recent historiography of molecular biology features key technologies, instruments and materials, which offer a different view of the field and its turning points than preceding intellectual and institutional histories. Radioisotopes, in this vein, became essential tools in postwar life science research, including molecular biology, and are here analyzed through their use in experiments on bacteriophage. Isotopes were especially well suited for studying the dynamics of chemical transformation over time, through metabolic pathways or life cycles. Scientists labeled phage with phosphorus-32 (...) in order to trace the transfer of genetic material between parent and progeny in virus reproduction. Initial studies of this type did not resolve the mechanism of generational transfer but unexpectedly gave rise to a new style of molecular radiobiology based on the inactivation of phage by the radioactive decay of incorporated phosphorus-32. These ‘suicide experiments’, a preoccupation of phage researchers in the mid-1950s, reveal how molecular biologists interacted with the traditions and practices of radiation geneticists as well as those of biochemists as they were seeking to demarcate a new field. The routine use of radiolabels to visualize nucleic acids emerged as an enduring feature of molecular biological experimentation. (shrink)

Parental participation has gained significant attention in environmental psychology, which has revealed a need for an instrument that can measure parental participation with children regarding environmental issues. The present study met this need by validating the parental participation in the environment (PPE) scale. This process began with 45 Chinese parents participating in an individual interview and group discussions, which helped generate a list of eighteen parent-child environmental activities. The activities were then modified and validated in the current study with a (...) diverse group of 969 parents recruited from six major Chinese cities. Both score structure evidence and generalizability evidence were obtained within this sample, and psychometric tests suggested a single factor construct with nine items. Once the PPE scale was revised, it showed measurement invariance across the parent who responded to the items (mother vs. father), across the child’s primary caregiver (mother vs. father vs. grandparent), across the family’s living region (North China vs. South China), as well as across the family’s income group. Finally, evidence based on relations to other variables showed a relationship among parents’ PPE, pro-environmental behavior, and connectedness with nature. As a result, the study provided a novel measure to assess pro-environmental socializationviaparental participation. (shrink)

We distinguish between different problems of “aboutness”: the “hard” problem of explaining the everyday phenomenon of intentionality and three less challenging “easy” sets of problems concerning the posits of folk psychology, the notions of representation invoked in the mind‐brain sciences, and the intensionality (with an “s”) of mental language. The problem of intentionality is especially hard in that, as is the case with the hard problem of phenomenal consciousness, there is no clear path to a solution using current methods. We (...) argue that naturalistic theories of mental representation do not address the hard problem—either they are only intended to address the easy problems, or the claims they make help address the problem of intentionality only under undefended andprima facieimplausible assumptions to the effect that the hard problem reduces to some combination of the easy problems. We offer a positive account of what would be required to properly face up to the problem of intentionality. (shrink)

La investigación humeana acerca de los principios de la moral parte de la pasión como motivo de la acción y al mismo tiempo pretende que su valoración ha de guiarse por la utilidad. Si bien son dos afirmaciones difíciles de combinar, la tesis del presente estudio es que la posibilidad de tender un puente entre la aspiración a la felicidad privada y el interés por el bienestar público está anclada, en su origen, en el sentimiento de benevolencia que el autor (...) descubre como principio original de la mente, pero logra sus efectos solamente en virtud de la fuerza de la simpatía y del espectáculo de la vida social y la conversación. Así, la moralidad -como posibilidad de realización de distinciones morales, de aprobar o censurar desde el punto devista del espectador- se articula y afina en el flujo incesante decontaminación y contagio de afecciones. (shrink)

This chapter for our edited volume (Character: New Directions from Philosophy, Psychology, and Theology) provides background material on what we consider to be several of the fundamental questions about character, such as whether character traits exist, what their makeup is, and how they can be improved.

Increasing the willingness to reduce animal product consumption has the potential to contribute to ameliorating the impact of animal agriculture on the environment, as well as foster healthier diets and improve the lives of farmed and wild animals. Reduction of animal product consumption is a prosocial behaviour (PSB), and factors that are considered to influence it are empathy and selfishness. In this research, animal-oriented empathy examined empathy specifically for animals. Animal oriented empathy and three types of selfishness: adaptive, egoistic, and (...) pathological were measured to determine if they could predict willingness to reduce animal product consumption. PSB is also influenced by motivations and motivations can lead to willingness. The three most common motivations to reduce animal product consumption: animal welfare, the environment, and health were examined to determine whether they predict willingness. A sample of 492 Australian adults completed questionnaires via the Zoho survey platform, and the data underwent a hierarchical regression. Higher pathological selfishness predicted a greater willingness to reduce animal product consumption, as did environmental and animal welfare motivations. However, higher health motivation predicted a lower willingness to reduce animal product consumption. Interpretation of the perplexing results in relation to pathological selfishness suggested further research. The practical value of utilising environmental and animal motivations to increase willingness to reduce animal products whilst bringing attention to the health issues was also discussed. (shrink)

This book discusses 'how' to respectfully and responsibly include pregnant women in clinical research. In sharp contrast, the existing literature predominantly focuses on the reasons 'why' the inclusion of pregnant women in clinical research is necessary - viz., to develop effective treatments for women during pregnancy, to promote fetal safety, to reduce harm to women and fetuses from suboptimal care, and to allow access to the benefits of research participation. This book supports the shift to a new default position, whereby (...) pregnant women are included in clinical research unless researchers argue convincingly for their exclusion. This shift raises many as yet unexplored ethical and policy questions about existing barriers to the equitable inclusion of pregnant women in research. This book is original in three key ways. First, it presents an unparalleled depth of analysis of the ethics of research with pregnant women, bringing together many of the key authors in this field as well as experts in research ethics and in vulnerability who have not previously applied their work to pregnant women. Second, it includes innovative theoretical work in ethics and disease specific case studies that highlight the current complexity and future challenges of research involving pregnant women. Third, the book brings together authors who argue both for and against including more pregnant women in formal clinical trials. (shrink)

Esta investigación tiene como finalidad responder, desde la filosofía y literatura de Camus, la pregunta de si el hombre puede ser feliz sin Dios. Para llevar a cabo este trabajo, se utiliza un método hermenéutico, pues se hace un acercamiento dialógico y holístico a las obras del pensador argelino para descubrir su pensamiento respecto a Dios y a la felicidad humana. Los principales resultados de la investigación concluyen que el hombre puede ser feliz sin Dios en la medida en que, (...) en primer lugar, acepte el absurdo y viva su rebeldía, y, en segundo lugar, comprenda la felicidad como una vocación ontológica más allá de un estado. (shrink)

The upbringing of infants in native communities is the concern of authorities and researchers for being a vulnerable population segment. The purpose of the study was to analyze child rearing styles of an Amuesha community in the Peruvian jungle. The phenomenological design allowed interviewing amuesha mothers from Oxapampa in Pasco, until reaching saturation. It was found as results that child rearing practices conform to sociocultural patterns, such as identity and communal heritage culture, which are in extinction due to the presence (...) of exotic factors; in addition to a demand to the State for better education and health services. (shrink)

Alexander of Aphrodisias, Aristotle’s commentator par excellence, rarely engages with Plato. In the present paper, however, we see him at work as an exegete of a passage in the _Phaedrus_ (245c5-9), in which Plato argues for the immortality of the soul based on its self-motion. In this paper, I focus on two ways in which Alexander deals with the passage. In his commentary on Aristotle’s _Prior Analytics_ (CAG II 1), Alexander employs the same approach to the _Phaedrus_ that he uses (...) with Aristotle’s treatises: he puts an informal argument expressed in ordinary language into the more formal shape of a syllogism. This does not mean that Alexander gives his approval to Plato’s theory. In fact, in his commentary on Aristotle’s _Topics_ (CAG II 2), Alexander introduces a polemical discussion of the same Platonic argument. Commenting on Aristotle’s denial that the soul is a self-moving substance (_Top_. IV 1.120b21-29), Alexander spells out the implicit reference to the _Phaedrus_ and criticizes Plato’s argument for not taking into account the difference between an accidental property of the soul and the essential characteristics by which it is defined. Plato’s theory of self-motion is partly accepted by Alexander and partly criticized. This paves the way for how the theory will be applied in the Aristotelian tradition, not to the soul, as in Plato, but to the ensouled body, either of the living being or of the heavens. (shrink)