Mucopolysaccharidosis type I (MPS I) is a rare lysosomal storage disorder treated with bone marrow transplantation or enzyme replacement therapy with laronidase, a high-cost orphan drug. Laronidase was approved by the US Food and Drug Administration and the European Medicines Agency in 2003 and by the Brazilian National Health Surveillance Agency in 2005. Many Brazilian MPS I patients have been receiving laronidase despite the absence of a governmental policy regulating access to the drug. Epidemiological and treatment data concerning MPS I (...) are scarce. This study aims to present a demographic profile of Brazilian patients with MPS I, describe the routes of access to laronidase in Brazil, and discuss associated ethical issues relating to public funding of orphan drugs. (shrink)

Listening to someone from some distance in a crowded room you may experience the following phenomenon: when looking at them speak, you may both hear and see where the source of the sounds is; but when your eyes are turned elsewhere, you may no longer be able to detect exactly where the voice must be coming from. With your eyes again fixed on the speaker, and the movement of her lips a clear sense of the source of the sound will (...) return. This ‘ventriloquist’ effect reflects the ways in which visual cognition can dominate auditory perception. And this phenomenological observation is one what you can verify or disconfirm in your own case just by the slightest reflection on what it is like for you to listen to someone with or without visual contact with them. (shrink)

In _Violence and Civility_, Étienne Balibar boldly confronts the insidious causes of violence, racism, nationalism, and ethnic cleansing worldwide, as well as mass poverty and dispossession. Through a novel synthesis of theory and empirical studies of contemporary violence, the acclaimed thinker pushes past the limits of political philosophy to reconceive war, revolution, sovereignty, and class. Through the pathbreaking thought of Derrida, Balibar builds a topography of cruelty converted into extremism by ideology, juxtaposing its subjective forms and its objective manifestations. Engaging (...) with Marx, Hegel, Hobbes, Clausewitz, Schmitt, and Luxemburg, Balibar introduces a new, productive understanding of politics as antiviolence and a fresh approach to achieving and sustaining civility. Rooted in the principles of transformation and empowerment, this theory brings hope to a world increasingly divided even as it draws closer together. (shrink)

More than 1.5 billion students experienced disruption to education as a result of COVID-19, representing the most substantial interruption to global education in modern history. Many educational institutions transitioned to emergency remote teaching (ERT) overnight, which has presented an array of distinct challenges for educators. Using virtual interviews and an experiential approach to thematic analysis, the study examined Scottish primary teachers’ (n = 10) lived experiences of adapting to ERT practice. Findings demonstrated three main themes; ‘Meeting Learners’ Needs,’ ‘Influencing Engagement’, (...) and ‘The Impact of ERT on Teachers’. Key findings suggested that differentiating learning, engaging, and communicating with learners is more difficult during ERT than face-to-face teaching. Parental engagement is vital, however, is influenced by several mitigating factors. Finally, ERT is likely to have substantial effects on the wellbeing of those involved. Implications of these findings are considered and discussed. (shrink)

Two major functions of the visual system are discussed and contrasted. One function of vision is the creation of an internal model or percept of the external world. Most research in object perception has concentrated on this aspect of vision. Vision also guides the control of object-directed action. In the latter case, vision directs our actions with respect to the world by transforming visual inputs into appropriate motor outputs. We argue that separate, but interactive, visual systems have evolved for the (...) perception of objects on the one hand and the control of actions directed at those objects on the other. This 'duplex' approach to high-level vision suggests that Marrian or 'reconstructive' approaches and Gibsonian or 'purposive-animate-behaviorist' approaches need not be seen as mutually exclusive, but rather as complementary in their emphases on different aspects of visual function. (shrink)

Do we directly perceive physical objects? What is the significance of the qualification ‘directly’ here? Austin famously denied that there was a unique interpretation by which we could make sense of the traditional debate in the philosophy of perception. I look here at Thompson Clarke’s discussion of G. E. Moore and surface perception to answer Austin’s scepticism.

When we dream, it is often assumed, we are isolated from the external environment. It is also commonly believed that dreams can be, at times, accurate, convincing replicas of waking experience. Here I analyse some of the implications of this view for an enactive theory of conscious experience. If dreams are, as described by the received view, “inactive”, or “cranially envatted” whilst replicating the experience of being awake, this would be problematic for certain extended conscious mind theories. Focusing specifically on (...) Alva Noë’s enactive view, according to which the vehicles of perceptual experience extend beyond the brain, I argue that dreams are a quandary. Noë’s view is that dreaming is consistent with enactivism because even if dreams are inactive and shut off from the external environment, they are not “full-blown” perceptual consciousness, and also, there is some reason to reject the inactive claim. However, this view rests on an unjustified and reductive account of dreams which is not supported... (shrink)

A long-standing theme in discussion of perception and thought has been that our primary cognitive contact with individual objects and events in the world derives from our perceptual contact with them. When I look at a duck in front of me, I am not merely presented with the fact that there is at least one duck in the area, rather I seem to be presented withthisthing (as one might put it from my perspective) in front of me, which looks to (...) me to be a duck. Furthermore, such a perception would seem to put me in a position not merely to make the existential judgment that there is some duck or other present, but rather to make a singular, demonstrative judgment, that that is a duck. My grounds for an existential judgment in this case derives from my apprehension of the demonstrative thought and not vice versa. (shrink)

Trust among current and future patients is essential for the success of biobank research. The submission of an informed consent is an act of trust by a patient or a research subject, but a strict application of the rule of informed consent may not be sensitive to the multiplicity of patient interests at stake, and could thus be detrimental to trust. According to a recently proposed law on “genetic integrity” in Sweden, third parties will be prohibited from requesting or seeking (...) genetic information about an individual. Cumbersome restrictions on research may be lifted, thus creating a more favourable climate for medical research. (shrink)

Decompressive craniectomy is a technically straightforward procedure whereby a large section of the cranium is temporarily removed in cases where the intracranial pressure is dangerously high. While its use has been described for a number of conditions, it is increasingly used in the context of severe head injury. As the use of the procedure increases, a significant number of patients may survive a severe head injury who otherwise would have died. Unfortunately some of these patients will be left severely disabled; (...) a condition likened to the RUB, an acronym for the Risk of Unacceptable Badness. Until recently it has been difficult to predict this outcome, however an accurate prediction model has been developed and this has been applied to a large cohort of patients in Western Australia. It is possible to compare the predicted outcome with the observed outcome at 18 months within this cohort. By using predicted and observed outcome data this paper considers the ethical implications in three cases of differing severity of head injury in view of the fact that it is possible to calculate the RUB for each case. (shrink)

OBJECTIVES: Cognitive and sensory difficulties frequently jeopardize informed consent of frail elderly patients This study is the first to test whether preliminary research experience could enhance geriatric patients' capacity to consent. DESIGN/SETTING: A step-wise consent procedure was introduced in a study on fluid balance in geriatric patients. Eligible patients providing verbal consent participated in a try-out of a week, during which bioelectrical impedance and weight measurements were performed daily. Afterwards, written informed consent was requested. Comprehension, risk and inconvenience scores (ranges: (...) 0-10) were obtained before and after the try-out by asking ten questions about the study's essentials and by asking for a risk and inconvenience assessment on a ten-points rating scale. SUBJECTS AND RESULTS: Seventy of the 78 eligible subjects started the try-out and 53 (68%) provided written consent. The comprehension score increased from 5.0 (+/- 2.3) to 7.0 (+/- 1.9) following the try-out (P < 0.001). The number of subjects capable of weighing risks and inconveniences increased from 32 to 48 (P < 0.001). CONCLUSIONS: Research experience improved the capacity to consent, still enabling an acceptable participation rate. Therefore, experienced consent seems a promising tool to optimize informed consent in frail elderly subjects. (shrink)

The state-of-art research in the field of life’s organization confronts the need to investigate a number of interacting components, their properties and conditions of sustainable behaviour within a natural system. In biology, ecology and life sciences, the performance of such stable system is usually related to homeostasis, a property of the system to actively regulate its state within a certain allowable limits. In our previous work, we proposed a deterministic model for systems’ homeostasis. The model was based on dynamical system’s (...) theory and pairwise relationships of competition, amensalism and antagonism taken from theoretical biology and ecology. However, the present paper proposes a different dimension to our previous results based on the same model. In this paper, we introduce the influence of inter-component relationships in a system, wherein the impact is characterized by direction (neutral, positive, or negative) as well as its (absolute) value, or strength. This makes the model stochastic which, in our opinion, is more consistent with real-world elements affected by various random factors. The case study includes two examples from areas of hydrobiology and medicine. The models acquired for these cases enabled us to propose a convincing explanation for corresponding phenomena identified by different types of natural systems. (shrink)

Traditionally, surgical practice has been experiential and based on the contemporary understanding of basic mechanisms of disease. It was both a science and an art and depended to far too great an extent on the individualism and self belief of its main exponents. “Evidence based medicine” emerged in the 1980s and a new gospel of “Rules of Evidence” was introduced. There is no doubt that the net effect of EBM has been beneficial, but over reliance on randomised controlled trials and (...) the lack of generalisability of scientific evidence to individual patients has perhaps led to less enthusiasm for its tenets among surgeons. There are valid and spurious reasons for this that are discussed. The situation is improving but inevitable tensions remain between the surgeon committed to the individual patient here and now, and the clinical researcher whose focus is the benefit of future patients in the larger community. (shrink)

I discuss the treatment by Chisholm of the problem posed by the fact that one can produce some neuro-physiological changes by moving a limb, namely the ones which cause the motions. I concentrate largely on the treatment Chisholm gave to this question before Person and Object, and I compare it with von Wright's discussion of it, I conclude that there are correct elements about both but that both are unsatisfactory, Chisholm's because it entails that we must know something which we (...) manifestly need not know when we move. (shrink)

This study examines the specificvalues held by consumers towards organic andconventionally produced meat, with particularreference to moral issues surrounding foodanimal production. A quota sample of 30 femalesfrom both a rural and an urban area of Scotland, were interviewed. Overall, there was lowcommitment towards the purchase of organicmeats and little concern for ethical issues.Price and product appearance were the primarymeat selection criteria, the latter being usedas a predictor of eating quality. Manyattitude-behavior anomalies were identified,mainly as a result of respondents' cognitivedissonance and (...) lack of understanding regardingmeat production criteria underpinning meatquality marks, e.g., Soil Association label.Responsibilities for ethical issues appeared tobe delegated by the consumer to the meatretailer or government. This raises issuesabout educating consumers and bringingconsumers closer to understanding meatproduction systems. A conceptual framework isproposed that illustrates the significance ofconsumer involvement in how meat-purchasingdecisions are approached in terms of theevaluation of tangible and or intangiblequality attributes. The results also point tothe need for further research into thoseaspects of quality that individuals tend toaddress at the level of the citizen,rather than at the point of purchase. (shrink)