Bone mineral density in patients with inherited bone marrow failure syndromes.

BackgroundPatients with inherited bone marrow failure syndromes (IBMFS) may have several risk factors for low bone mineral density (BMD). We aimed to evaluate the prevalence of low BMD in IBMFS and determine the associated risk factors.MethodsPatients with IBMFS with at least one dual-energy X-ray absorptiometry (DXA) scan were evaluated. Diagnosis of each IBMFS, Fanconi anemia (FA), dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome was confirmed by syndrome-specific tests. Data were gathered on age, height, and clinical history. DXA scans were completed at the lumbar spine, femoral neck, and forearm. BMD was adjusted for height (HAZ) in children (age ≤20 years). Low BMD was defined as a BMD Z-score and HAZ ≤-2 in adults and children, respectively, in addition to patients currently on bisphosphonate therapy.ResultsNine of thirty-five adults (26%) and eleven of forty children (27%) had low BMD. Adults with FA had significantly lower BMD Z-scores than those with other diagnoses; however, HAZ did not vary significantly in children by diagnosis. Risk factors included hypogonadism, iron overload, and glucocorticoid use.ConclusionsAdults and children with IBMFS have high prevalence of low BMD. Prompt recognition of risk factors and management are essential to optimize bone health

Health information portals: How can we improve the user\u27s search experience?

Health information portals (HIP) are now common place. Governments and other health agencies are using HIPs extensively to reduce costs and distribute information more effectively. Generally, HIPs are not very technically sophisticated specifically in terms of options for improving searching. There are many ways in which searching and retrieving relevant information can be improved. This paper presents an exploratory study which investigated five HIPs. Each HIP offered a range of features and functionality to assist with searching. Our research explored the features and functionality of each HIP. Through usability evaluations we compared the response of users to each HIP and identified users’ preferences for improved searching. We found that HIPs with improved search functionality and other features that assisted searching were better received by the users. Users regarded these portals as easier to understand, easier to use, required fewer steps in retrieving information and were more likely to say they would return. Comments from users are provided to illustrate further the importance of providing effective functionality. The paper concludes with recommendations for Health Information Portal builders on what is needed to improve the user search experience

Political economics, collective action and wicked socio-ecological problems: A practice story from the field

Empowering integrative, sustainable and equitable approaches to wicked socio-ecological problems requires multiple disciplines and ways of knowing. Following calls for greater attention to political economics in this transdisciplinary work, we offer a practitioner perspective on political economy and collective action and their influences on our community engagement practice and public policy. Our perspective is grounded in a pervasive wicked problem in Australia, invasive rabbits, and the emergence of the Victorian Rabbit Action Network. The network grew out of a publically funded research project to support community-led action in rabbit management. Victorian residents and workers affected by rabbits – public and private land managers, scientists, government officers and others – were invited to engage in a participatory planning process to generate sustainable strategies to address the rabbit problem. Each stage in the process, which involved interviews, a workshop and consultations, was designed to nurture the critical enquiry, listening and learning skills of participants, advance understandings of the problem from multiple perspectives, generate collective options to guide decision-making, and encourage community-led collective action. We reflect on our understanding of these processes using the language and lens of political economics and, in particular, the context of democratic professionalism. In so doing, we define terms and refer to information resources that have enabled us to bring a practical working knowledge of political economics to our professional practice. Our intent is to motivate academics, community members, government officials, and scientists alike, to draw on their knowledge and field experiences and to share practice stories through the lens of political economics and collective action. This is an opportunity to engage each other in small ‘p’ politics of how we understand and act on wicked problems, to negotiate and connect across disciplines, practical experiences and human difference, so that people may work more creatively and effectively together to address the challenging issues of our time. &nbsp

Comment on ``Protective measurements of the wave function of a single squeezed harmonic-oscillator state''

Alter and Yamamoto [Phys. Rev. A 53, R2911 (1996)] claimed to consider ``protective measurements'' [Phys. Lett. A 178, 38 (1993)] which we have recently introduced. We show that the measurements discussed by Alter and Yamamoto ``are not'' the protective measurements we proposed. Therefore, their results are irrelevant to the nature of protective measurements.Comment: 2 pages LaTe

Russellian Monism and Mental Causation

© 2019 Wiley Periodicals, Inc.According to Russellian monism, consciousness is constituted at least partly by quiddities: intrinsic properties that categorically ground dispositional properties described by fundamental physics. If the theory is true, then consciousness and such dispositional properties are closely connected. But how closely? The contingency thesis says that the connection is contingent. For example, on this thesis the dispositional property associated with negative charge might have been categorically grounded by a quiddity that is distinct from the one that actually grounds it. Some argue that Russellian monism entails the contingency thesis and that this makes its consciousness‐constituting quiddities epiphenomenal—a disastrous outcome for a theory that is motivated partly by its prospects for integrating consciousness into physical causation. We consider two versions of that argument, a generic version and an intriguing version developed by Robert J. Howell, which he bases on Jaegwon Kim's well‐known “exclusion argument.” We argue that neither succeeds.Peer reviewe

Measurement Methods of Burnup and Heavy Element Isotopic Composition in Irradiated Power Reactor Fuels

Methods for determining the heavy element isotopic compositions and atom percent fission for reactor fuels are briefly described, and their accuracies are discussed. (D.C.W.

The noise in gravitational-wave detectors and other classical-force measurements is not influenced by test-mass quantization

It is shown that photon shot noise and radiation-pressure back-action noise are the sole forms of quantum noise in interferometric gravitational wave detectors that operate near or below the standard quantum limit, if one filters the interferometer output appropriately. No additional noise arises from the test masses' initial quantum state or from reduction of the test-mass state due to measurement of the interferometer output or from the uncertainty principle associated with the test-mass state. Two features of interferometers are central to these conclusions: (i) The interferometer output (the photon number flux N(t) entering the final photodetector) commutes with itself at different times in the Heisenberg Picture, [N(t), N(t')] = 0, and thus can be regarded as classical. (ii) This number flux is linear in the test-mass initial position and momentum operators x_o and p_o, and those operators influence the measured photon flux N(t) in manners that can easily be removed by filtering -- e.g., in most interferometers, by discarding data near the test masses' 1 Hz swinging freqency. The test-mass operators x_o and p_o contained in the unfiltered output N(t) make a nonzero contribution to the commutator [N(t), N(t')]. That contribution is cancelled by a nonzero commutation of the photon shot noise and radiation-pressure noise, which also are contained in N(t). This cancellation of commutators is responsible for the fact that it is possible to derive an interferometer's standard quantum limit from test-mass considerations, and independently from photon-noise considerations. These conclusions are true for a far wider class of measurements than just gravitational-wave interferometers. To elucidate them, this paper presents a series of idealized thought experiments that are free from the complexities of real measuring systems.Comment: Submitted to Physical Review D; Revtex, no figures, prints to 14 pages. Second Revision 1 December 2002: minor rewording for clarity, especially in Sec. II.B.3; new footnote 3 and passages before Eq. (2.35) and at end of Sec. III.B.

Introduction: Policymaking, Learning and Devolution

This collection explores the idea that devolution in European states can provide ‘laboratories of democracy' as states experiment in different ways to address social and economic problems and engage their citizens. While there is a substantial literature on policy diffusion and learning among US states, and on transfer and learning between countries, there is not much on learning among European federated and devolved governments. This collection fills that gap with a series of studies based primarily on experiences in Germany, Spain and the UK (including a particular focus on Northern Ireland). The introduction sets up the analytical framework, discussing the concepts of learning and transfer, the direction of transfer (for example, from the centre to the devolved territory), the mechanisms involved (from voluntary to coercive) and the degree of transfer

Dominant cognitive frames and the innovative power of social networks

In this paper, we illustrate the link between social network structures, dominant cognitive frames on network purpose and the innovative power of a network, through a mixed-method comparative analysis of two Knowledge Translation Networks (KTNs)in the English National Health Service (NHS). Our findings illustrate several challenges for networked forms of organization linked to different manifestations of social networks (centralized/decentralized) and dominant cognitive frames (polarizing/loosely clustered). Our paper contributes a better understanding of how dominant frames on network purpose emerge alongside the development of network structure itself, and explores how this interplay between dominant frames and social networks impacts upon the collaborative work that supports the networks’ innovative power

Heart rate variability and target organ damage in hypertensive patients

Background: We evaluated the association between linear standard Heart Rate Variability (HRV) measures and vascular, renal and cardiac target organ damage (TOD). Methods: A retrospective analysis was performed including 200 patients registered in the Regione Campania network (aged 62.4 ± 12, male 64%). HRV analysis was performed by 24-h holter ECG. Renal damage was assessed by estimated glomerular filtration rate (eGFR), vascular damage by carotid intima-media thickness (IMT), and cardiac damage by left ventricular mass index. Results: Significantly lower values of the ratio of low to high frequency power (LF/HF) were found in the patients with moderate or severe eGFR (p-value < 0.001). Similarly, depressed values of indexes of the overall autonomic modulation on heart were found in patients with plaque compared to those with a normal IMT (p-value <0.05). These associations remained significant after adjustment for other factors known to contribute to the development of target organ damage, such as age. Moreover, depressed LF/HF was found also in patients with left ventricular hypertrophy but this association was not significant after adjustment for other factors. Conclusions: Depressed HRV appeared to be associated with vascular and renal TOD, suggesting the involvement of autonomic imbalance in the TOD. However, as the mechanisms by which abnormal autonomic balance may lead to TOD, and, particularly, to renal organ damage are not clearly known, further prospective studies with longitudinal design are needed to determine the association between HRV and the development of TOD