A central characteristic of people with Prader-Willi Syndrome (PWS) is an apparent insatiable appetite leading to severe overeating and the potential for marked obesity and associated serious health problems and premature death. This behaviour may be due to the effects of the genetic defect resulting from the chromosome 15 abnormalities associated with the syndrome. We examine the ethical and legal dilemmas that can arise in the care of people with PWS. A tension exists between a genetic (...) deterministic perspective and that of individual choice. We conclude that the determination of the capacity of a person with PWS to make decisions about his/her eating behaviour and to control that behaviour is of particular importance in resolving this dilemma. If the person is found to lack capacity, the common law principles of acting in a person's "best interests" using the "least restrictive alternative" may be helpful. Allowing serious weight gain in the absence of careful consideration of these issues is an abdication of responsibility. (shrink)

Prader-Willi syndrome (PWS) is one of the 25 syndromic forms of obesity, in which patients present—in addition to different degrees of obesity—intellectual disability, endocrine disturbs, hyperphagia and/or other signs of hypothalamic dysfunction. In front of a severe/extreme obesity and the failure of non-invasive treatments, bariatric surgery is proposed as a therapeutic option. The complexity of the clinical condition, which could affect the long-term effects of bariatric surgery, and the frequent association with a mild to severe intellectual disability (...) raise some ethical concerns in the treatment of obese PWS adolescents. This article analyses these issues referring to the principles of healthcare ethics: beneficence/non-maleficence (proportionality of treatments; minimisation of risks); respect of autonomy; justice. Based on these principles, three hypothetical scenarios are defined: (1) obese PWS adolescent, capable of making an autonomous decision; (2) obese PWS adolescent with a severe intellectual disability, whose parents agree with bariatric surgery; (3) obese PWS adolescent with a life-threatening condition and a severe intellectual disability, whose parents do not agree with bariatric surgery. The currently available evidence on efficacy and safety of bariatric surgery in PWS adolescents with extreme or severe obesity and the lack of adequate long-term follow-up suggests great caution even in a very life-threatening condition. Clinicians must always obtain a full IQ assessment of patients by psychologists. A multidisciplinary team is needed to analyse the clinical, psychological, social and ethical aspects and organise support for patient and parents, involving also the hospital ethical committee or, if necessary, legal authorities. (shrink)

Genomic imprinting is an epigenetic mechanism resulting in the preferential expression of the maternal or paternal alleles of a specific subset of genes in the mammalian genome. A key but relatively unexplored question is how imprints are established in the germline. New observations(1) on two classical imprinting disorders, the Prader‐Willi (PWS) and Angleman (AS) syndromes, offer the first genetic insight into this process. Molecular analysis of imprinting mutations that interfere with the appropriate establishment of the maternal and paternal (...) epigenotypes has led to the identification of imprinted transcripts that could be involved in switching imprints in the germlines. (shrink)

This article focusses upon defining those factors which may contribute to the pathogenesis of cancer. The molecular basis of tumour etiology is discussed with reference to cancer predisposing syndromes, and in particular to the human inherited disease, Bloom's sysdrome. In Bloom's syndrome, patients are predisposed to a wide variety of malignant disease. We propose a model in which overexpression of the ubiquitous c‐myc proto‐oncogene contributes to this process.

Recent experimental evidence suggests that most of the genome is transcribed into non‐coding RNAs. The initial transcripts undergo further processing generating shorter, metabolically stable RNAs with diverse functions. Small nucleolar RNAs (snoRNAs) are non‐coding RNAs that modify rRNAs, tRNAs, and snRNAs that were considered stable. We review evidence that snoRNAs undergo further processing. High‐throughput sequencing and RNase protection experiments showed widespread expression of snoRNA fragments, known as snoRNA‐derived RNAs (sdRNAs). Some sdRNAs resemble miRNAs, these can associate with argonaute proteins and (...) influence translation. Other sdRNAs are longer, form complexes with hnRNPs and influence gene expression. C/D box snoRNA fragmentation patterns are conserved across multiple cell types, suggesting a processing event, rather than degradation. The loss of expression from genetic loci that generate canonical snoRNAs and processed snoRNAs results in diseases, such as Prader‐Willi Syndrome, indicating possible physiological roles for processed snoRNAs. We propose that processed snoRNAs acquire new roles in gene expression and represent a new class of regulatory RNAs distinct from canonical snoRNAs.Also watch the Video Abstract. (shrink)

Clinical research is a necessity if effective and safe treatments are to be developed. However, this may well include the need for research that is best described as ‘invasive’ in that it may be associated with some discomfort or inconvenience. Limitations in the undertaking of invasive research involving people with intellectual disabilities (ID) are perhaps related to anxieties within the academic community and among ethics committees; however, the consequence of this neglect is that innovative treatments specific to people with ID (...) may not be developed. Such concerns are likely to continue while there is limited published knowledge regarding the actual experiences of people with ID who have participated in invasive clinical research. As part of a pilot study trialling the novel use of a surgically inserted device to curb overeating in people with Prader–Willi syndrome (PWS) we have investigated the experience of research through semistructured qualitative interviews involving three participants and their carers. Thematic analysis revealed that the adults with PWS and their family carers rated their participation positively, seeing it as a rewarding and enriching experience. This brief report discusses findings from our interview data in order to highlight strategies which may ensure that research is acceptable to participants, meets the necessary ethical standards and is able to achieve the aims set out by the researchers. To our knowledge, this is the first study to record experiences directly from people with PWS and their carers regarding their involvement in invasive clinical research. (shrink)

The basic premise of the host‐defense theory is that genomic imprinting, the parent‐of‐origin expression of a subset of mammalian genes, derives from mechanisms originally dedicated to silencing repeated and retroviral‐like sequences that deeply colonized mammalian genomes. We propose that large clusters of tandemly‐repeated C/D‐box small nucleolar RNAs (snoRNAs) or microRNAs represent a novel category of sequences recognized as “genomic parasites”, contributing to the emergence of genomic imprinting in a subset of chromosomal regions that contain them. Such a view is supported (...) by evidence derived from studies of the imprinted snoRNA‐ and/or miRNA‐encoding Dlk1‐Dio3, Snurf‐Snrpn, Sfbmt2, and C19MC domains. While adding a new piece to the challenging puzzle of mammalian genome history, this hypothesis also reinforces the notion that dissecting the features and molecular mechanisms that discriminate between “foreign” and “endogenous” sequences is of crucial importance in the field of mammalian epigenetics. (shrink)

In the target article, Crespi & Badcock (C&B) propose a novel hypothesis based on observations that a large set of phenotypic traits exhibit diametrically opposite phenotypes in autism-spectrum versus psychotic-spectrum conditions. They propose that development of these conditions is mediated in part by alterations in This hypothesis is based on the model of the Prader-Willi and Angelman syndromes. The authors have produced a masterful discussion of the differences between psychosis and autism. Of course, another article could be written (...) on the similarities. (shrink)

This reconsideration of Hume’s classic essay “Of Miracles” via the lens of American pragmatist ways of thinking about history and power shifts our attention from Hume’s epistemic concerns about the legitimacy of witnesses and testimony to his distaste for sacred history, his critical stance regarding the social force of revelation, and his disdain for religious authority. To view Hume’s essay both as an articulation of a critical philosophy of history and as an exercise in moral dynamism (social power or, authority, (...) is a result of epistemic transactions), is to intervene in both Hume studies and Contemporary Pragmatism. (shrink)

An examination of David Hume's philosophy of religion that situates his conception "true religion" within the context of his overall science of human nature, his rejection of popular religion, and his Ciceronian influence"--Provided by publisher.

"First Published in 1999, Routledge is an imprint of Taylor & Francis, an informa company.".

Argues for the primacy of the phylogenetic system as the general reference system in biology. This book, first published in 1966, generated significant controversy and opened possibilities for evolutionary biology.

Ethicists regularly turn to virtue in order to negotiate features of climate change that seem to overwhelm moral agency. Appeals to virtue in climate ethics differ by how they connect individual flourishing with collective responsibilities and by how they interpret Anthropocene relations. Differences between accounts of climate virtue help critique proposals to reframe global ecological problems in terms of resilience and planetary stewardship, the intelligibility of which depends on connecting what would be good for the species with what would be (...) good for an individual life. A pragmatic way of establishing that connection may need a strong role for respect of nature. (shrink)

The fields of environmental ethics and of religion and ecology have been shaped by Lynn White Jr.'s thesis that the roots of ecological crisis lie in religious cosmology. Independent critical movements in both fields, however, now question this methodological legacy and argue for alternative ways of inquiry. For religious ethics, the twin controversies cast doubt on prevailing ways of connecting environmental problems to religious deliberations because the criticisms raise questions about what counts as an environmental problem, how religious traditions change, (...) and whether ethicists should approach problems and traditions with reformist commitments. This article examines the critiques of White's legacy and presents a pluralist alternative that focuses religious ethics on the contextual strategies produced by moral communities as they confront environmental problems. (shrink)

In this study, we argue that contemporary nursing care has been overtaken by new public management strategies aimed at curtailing budgets in the public hospital sector in Australia. Drawing on qualitative interviews with 15 nurses from one public acute hospital with supporting documentary evidence, we demonstrate what happens to nursing work when management imposesroundingas a risk reduction strategy. In the case study outlined rounding was introduced across all wards in response to missed care, which in turn arose as a result (...) of work intensification produced by efficiency, productivity, effectiveness and accountability demands. Rounding is a commercially sponsored practice consistent with new public management. Our study illustrates the impact that new public management strategies such as rounding have on how nurses work, both in terms of work intensity and in who controls their labour. (shrink)

Ethics in the anthropocene -- Atmospheric powers: climate change and moral incompetence -- Christian ethics and unprecedented problems -- Global ethics: moral pluralism and planetary problems -- Sustainability science and the ethics of wicked problems -- Toxic wombs and the ecology of justice -- Impoverishment and the economy of desire -- Intergenerational risk and the future of love -- Sustaining grace.

From an informal group of a dozen faculty and graduate students at Temple University, the Jean Piaget Society grew in seven years to 500 members who have interests in the application of genetic epistemology to their own disciplines and professions. At the outset Piaget endorsed the concept of a society which bore his name and presented a major address on equilibration at the society's first symposium in May, 1971. Had he not done so the society would no doubt have remained (...) a small parochial group, like so many others throughout the country, interested in Piaget and his theory. With the encouragement of Genevans and the leadership of its first four presidents, Lois Macomber, Barbara Press eisen, Marilyn Appel, and John Mickelson, the society undertook a number of programs to collect and disseminate the results of scholarly work in genetic epistemology. Particular emphasis was placed upon applications of Piaget's theory to developmental psychology, philos ophy, and education. One of these programs was the publication of an annual series on the development of knowing, of which this volume is the first. In 1973, the society asked Hans Furth with the assistance of Willis Overton and Jeanette Gallagher to initiate and plan a series of yearbooks with the result that in addition to this volume, a second volume on education was commissioned, and a third one on the decalage issue was planned. (shrink)

Laudato si' attempts simultaneously to disrupt prevailing global environmental discourse and to reorient central concepts in Catholic moral tradition by requalifying the meaning of dominion and by ecologically expanding human dignity. The image of Earth crying out to humans from within a kinship relation plays a central role in both arguments. However, the political consequences of those shifts remain vague because the “voice” of Earth remains silent in crucial loci of the encyclical's argument.

Within the context of employee rights and management social responsibility, this paper identifies and explores three ethical dimensions of downsizing. Using ANOVA and Scheffe post-hoc statistical techniques, groups involved in the downsizing decision making process were compared with groups affected by the process on each ethical dimension. Results indicated that those affected by the process attached greater ethical significance to these dimensions than those who were involved in formulating and implementing/communicating downsizing decisions.

From an informal group of a dozen faculty and graduate students at Temple University, the Jean Piaget Society grew in seven years to 500 members who have interests in the application of genetic epistemology to their own disciplines and professions. At the outset Piaget endorsed the concept of a society which bore his name and presented a major address on equilibration at the society's first symposium in May, 1971. Had he not done so the society would no doubt have remained (...) a small parochial group, like so many others throughout the country, interested in Piaget and his theory. With the encouragement of Genevans and the leadership of its first four presidents, Lois Macomber, Barbara Press eisen, Marilyn Appel, and John Mickelson, the society undertook a number of programs to collect and disseminate the results of scholarly work in genetic epistemology. Particular emphasis was placed upon applications of Piaget's theory to developmental psychology, philos ophy, and education. One of these programs was the publication of an annual series on the development of knowing, of which this volume is the first. In 1973, the society asked Hans Furth with the assistance of Willis Overton and Jeanette Gallagher to initiate and plan a series of yearbooks with the result that in addition to this volume, a second volume on education was commissioned, and a third one on the decalage issue was planned. (shrink)

Problems that overwhelm moral agency challenge methods of ethics that prioritize social practices. This essay explains how climate change exceeds moral competencies, criticizes climate ethics for eliding the difficulties, and the attempts to vindicate a practice-based approach by arguing for the possibility of doing ethics from incompetent projects. However, because incompetence easily becomes the excuse of injustice, I illustrate the argument with an indigenous peoples' climate justice project that both exemplifies the creativity my approach needs and bears a strong critique (...) of its method. (shrink)

Despite acknowledgment that performance failure among new salespeople is a prevalent issue for organizations, researchers do not fully understand the consequences of repetitive periods of failure on new salespeople’s unethical selling behaviors. Further, little is known about how a sales force’s reward structure and managerial attempts to intervene following failure affect new salespeople’s behavior. Combining an experiment with longitudinal growth models, we show that repetitive periods of failure increase unethical behaviors, and interventions intended to remind the salesperson to behave in (...) the customer’s best interests attenuate this effect under a non-contingent reward structure. However, counter to managerial assumptions, under a contingent reward structure these customer-oriented interventions actually backfire by amplifying the original relationship between repetitive failure and unethical behaviors. The results have potentially important managerial implications for those who manage new salespeople learning how to sell or during other failure-prone periods. (shrink)