X-linked dominant Conradi-Hunermann syndrome is one of a group of disorders with aberrant punctate calcification in cartilage, or chondrodysplasia punctata. This is most prominent around the vertebral column, pelvis and long bones in CPDX2. Additionally, CDPX2 patients may have asymmetric rhizomesomelia, sectorial cataracts, patchy alopecia, ichthyosis and atrophoderma. The phenotype in CDPX2 females ranges from stillborn to mildly affected individuals identified in adulthood. CDPX2 is presumed lethal in males, although a few affected males have been reported. We found increased 8-cholestenol (...) and 8-dehydrocholesterol in tissue samples from seven female probands with CDPX2. This pattern of accumulated cholesterol intermediates suggested a deficiency of 3β-hydroxysteroid- Δ8,Δ7-isomerase, which catalyses an intermediate step in the conversion of lanosterol to cholesterol. A candidate gene encoding a sterol-Δ8-isomerase has been identified and mapped to Xp11.22-p11.23. Using SSCP analysis and sequencing of genomic DNA, we found EBP mutations in all probands. We confirmed the functional significance of two missense alleles by expressing them in a sterol-Δ8- isomerase-deficient yeast strain. Our results indicate that defects in sterol-Δ8-isomerase cause CDPX2 and suggest a role for sterols in bone development. (shrink)

OBJECTIVE: To report and analyse the pattern of end-of-life decision making for terminal Chinese cancer patients. DESIGN: Retrospective descriptive study. SETTING: A cancer clinical trials unit in a large teaching hospital. PATIENTS: From April 1992 to August 1997, 177 consecutive deaths of cancer clinical trial patients were studied. MAIN MEASUREMENT: Basic demographic data, patient status at the time of signing a DNR consent, or at the moment of returning home to die are documented, and circumstances surrounding these events evaluated. RESULTS: (...) DNR orders were written for 64.4% of patients. Patients in pain (odds ratio 0.45, 95% CI 0.22-0.89), especially if requiring opioid analgesia (odds ratio 0.40, 95% CI 0.21-0.77), were factors associated with a higher probability of such an order. Thirty-five patients were taken home to die, a more likely occurrence if the patient was over 75 years (odds ratio 0.12, 95% CI 0.04-0.34), had children (odds ratio 0.14, 95% CI 0.02-0.79), had Taiwanese as a first language (odds ratio 6.74, 95% CI 3.04-14.93), or was unable to intake orally (odds ratio 2.73, 95% CI 1.26-5.92). CPR was performed in 30 patients, none survived to discharge. CONCLUSIONS: DNR orders are instituted in a large proportion of dying Chinese cancer patients in a cancer centre, however, the order is seldom signed by the patient personally. This study also illustrates that as many as 20% of dying patients are taken home to die, in accordance with local custom. (shrink)

Copyright © 2015 Massachusetts Medical Society. BACKGROUND: Routine preoperative testing is not recommended for patients undergoing cataract surgery, because testing neither decreases adverse events nor improves outcomes. We sought to assess adherence to this guideline, estimate expenditures from potentially unnecessary testing, and identify patient and health care system characteristics associated with potentially unnecessary testing. METHODS: Using an observational cohort of Medicare beneficiaries undergoing cataract surgery in 2011, we determined the prevalence and cost of preoperative testing in the month before surgery. (...) We compared the prevalence of preoperative testing and office visits with the mean percentage of beneficiaries who underwent tests and had office visits during the preceding 11 months. Using multivariate hierarchical analyses, we examined the relationship between preoperative testing and characteristics of patients, health system characteristics, surgical setting, care team, and occurrence of a preoperative office visit. RESULTS: Of 440,857 patients, 53% had at least one preoperative test in the month before surgery. Expenditures on testing during that month were $4.8 million higher and expenditures on office visits $12.4 million higher than the mean monthly expenditures during the preceding 11 months. Testing varied widely among ophthalmologists; 36% of ophthalmologists ordered preoperative tests for more than 75% of their patients. A patient's probability of undergoing testing was associated mainly with the ophthalmologist who managed the preoperative evaluation. CONCLUSIONS: Preoperative testing before cataract surgery occurred frequently and was more strongly associated with provider practice patterns than with patient characteristics.. (shrink)

Liz JacksonEducation University of Hong KongGiven how important textbooks continue to be in education, how textbooks are read for learning and research remains poorly understood. As Michael Apple n...

Understanding the association between autonomic nervous system [ANS] function and brain morphology across the lifespan provides important insights into neurovisceral mechanisms underlying health and disease. Resting-state ANS activity, indexed by measures of heart rate [HR] and its variability [HRV] has been associated with brain morphology, particularly cortical thickness [CT]. While findings have been mixed regarding the anatomical distribution and direction of the associations, these inconsistencies may be due to sex and age differences in HR/HRV and CT. Previous studies have been (...) limited by small sample sizes, which impede the assessment of sex differences and aging effects on the association between ANS function and CT. To overcome these limitations, 20 groups worldwide contributed data collected under similar protocols of CT assessment and HR/HRV recording to be pooled in a mega-analysis (N = 1,218 (50.5% female), mean age 36.7 years (range: 12-87)). Findings suggest a decline in HRV as well as CT with increasing age. CT, particularly in the orbitofrontal cortex, explained additional variance in HRV, beyond the effects of aging. This pattern of results may suggest that the decline in HRV with increasing age is related to a decline in orbitofrontal CT. These effects were independent of sex and specific to HRV; with no significant association between CT and HR. Greater CT across the adult lifespan may be vital for the maintenance of healthy cardiac regulation via the ANS-or greater cardiac vagal activity as indirectly reflected in HRV may slow brain atrophy. Findings reveal an important association between CT and cardiac parasympathetic activity with implications for healthy aging and longevity that should be studied further in longitudinal research. (shrink)

© 2015.This document a59Summary of the discussions and conclusions following from Dark Matter @ LHC 2014, held at Merton College, Oxford, on September 25-27, 2014. outlines a set of simplified models for dark matter and its interactions with Standard Model particles. It is intended to summarize the main characteristics that these simplified models have when applied to dark matter searches at the LHC, and to provide a number of useful expressions for reference. The list of models includes both s-channel and (...) t-channel scenarios. For s-channel, spin-0 and spin-1 mediations are discussed, and also realizations where the Higgs particle provides a portal between the dark and visible sectors. The guiding principles underpinning the proposed simplified models are spelled out, and some suggestions for implementation are presented. (shrink)

Workplace vigilantism refers to employees’ self-initiated acts of monitoring and punishing deviant coworkers based on their perceptions of right and wrong. Prior research has primarily adopted an identity perspective to explain this phenomenon. Drawing on an interest-based perspective, we examine how individual differences in individualism and collectivism orientations drive this behavior through a cost–benefit analysis of its anticipated consequences. We propose that an individualism orientation discourages vigilantism by heightening perceived risks, whereas a collectivism orientation promotes it by fostering pro-team motivation. (...) Across an experimental study (n = 104) and a two-wave field study (n = 357) of organizational employees, we found consistent support for these effects. Furthermore, we demonstrated in our field study that high task interdependence moderated the indirect effect of both individualism and collectivism orientations on workplace vigilantism. These findings advance theoretical understanding of workplace vigilantism and offer practical insights for organizations to effectively address employee deviance. (shrink)

Volume 26, Issue 2, February 2026, Page 1-6.

The author manages to work in quite a bit of ethics among numerous examples and illustrations. Both classical and modern views are considered. Great simplicity and clarity.--F. W. N.

After some preliminaries on the logic of change and the logic of action the author develops an extended theory of norms in this companion volume to his The Varieties of Goodness. Although the book will be of particular interest to deontic logicians, its philosophic value is greatly enhanced by a concern for broader issues, including problems in the ontology of norms. The usefulness of the work is very much decreased by numerous undeveloped suggestions and unanswered questions.—F. W. N.

A symposium by twelve English thinkers of various Christian backgrounds. The papers investigate the possibility of incorporating traditional metaphysics and the insights of contemporary continental philosophers into the empirical and analytic tradition. The concept of intuition or immediate apprehension is explored in several of the papers as a possible key to the problem. Though the writers often fail to face up to hard problems, the book offers an important, if cautious, effort at integration.--F. W. N.

A persuasive attack on Ryle's notion that "remember" is an achievement verb, and on Russell's view that all acts of memory might be entirely misleading. Although we can never be sure in any particular case that our memories are veridical, we need not adopt total scepticism. The book suffers from awkwardness of style and unnecessary repetition.--F. W. N.

These twenty-nine essays from a period of thirty-five years cover topics in ethics, critical and speculative philosophy, American philosophy and social philosophy. The late Professor Murphy's concern for the social and political relevance of theoretical philosophical issues is very much in evidence, and something of his humane personality shows through.—F. W. N.

An elementary analysis, both historical and systematic, of the two topics mentioned in the title. Although the book presents difficulties in both phases of its analysis, readers concerned with the topics should find it an interesting presentation of a Catholic view.--F. W. N.

This work has been selected by scholars as being culturally important and is part of the knowledge base of civilization as we know it. This work is in the public domain in the United States of America, and possibly other nations. Within the United States, you may freely copy and distribute this work, as no entity (individual or corporate) has a copyright on the body of the work. Scholars believe, and we concur, that this work is important enough to be (...) preserved, reproduced, and made generally available to the public. To ensure a quality reading experience, this work has been proofread and republished using a format that seamlessly blends the original graphical elements with text in an easy-to-read typeface. We appreciate your support of the preservation process, and thank you for being an important part of keeping this knowledge alive and relevant. (shrink)

In an almost forgotten passage of the Postenor Analytics (Bk I, ch. III) Aristotle argues against 'another school', according to which it is possible to proof things 'by each other and in a circle'. His logical refutation of this opinion became so dominant in the Western philosophical tradition, that the 'vicious circle' has always deemed a crime since. A scientific demonstration has to be built on firm premisses in order to deduce conclusions from them in a straight, ongoing proces, in (...) which one does not have to return to the premisses for a reconsideration of them on the basis of their implications. Although Aristotle does not mention names, he in fact reacts against Plato and the mainstream of Greek thought, which stresses the essential circularity of human argumentation. In the first, the historical part of the treatise the roots of this circular logic are unearthed in the work of Heraclitus and Parmenides, who both defended a ring structure of their metaphysical explication of nature. Truth is described as well-rounded, because you can follow it in a circle, passing through each of its links in turn, back to your startingpoint. The association of circular motion with thought is fully present in the work of Plato, who speeks many times about the 'periforas tès dianoias' and the essential interdependance of our ideas. Not the theory of an anonymous alternative school but Aristotle's own rectilinear formal logic was the exceptional, untraditional standpoint, which, moreover, was in conflict with his explication of divine thinking as 'noèsis noèseôs'. In modern times Hegel, Nietzsche and Heidegger were the main representatives of an anti-Aristotelian attitude. All three reject the logical prohibition of the circular argument, because it is inadequate to the life of concrete scientific thinking. The path of science always turns around towards its origins and results in a correction of its fundamental hypotheses on a higher level. Circularity is considered as a hallmark of sound logical procedure, as a characteristic of excellence in human understanding. The famous 'hermeneutic circle' is interpreted as the modern equivalent of the Hegelian 'Kreislauf and the Greek ' to par'allèlôn'. In the second, the more systematic part of the text the circular nature of our cognitive and scientific behaviour is demonstrated along three different lines. Reflection on the natural language with its finite stock of words, whose meanings are structurally connected, leads us to the so called semiotical circularity. Natural language is a complex network in which circular argumentation is not only unavoidable, but even the only means of explication. We have to work with the material contained in our dictionary, in which the items are defined by each other. The second reason is constituted by the system-orientation of science. In science we try to systematize our knowledge by means of formalisations and abstractions. The development of science can be characterised as a succession of systems, in which every element gets its meaning from the whole organisation. If we accept systematicity as the arbiter of truth, our logic is anti-fundamentalistic and the path of our thought is in fact circular (or better : spiralistic). The third adstruction of our theory is afforded by the coupling, that is realised in a rational proof. We draw conclusions from the coherence of our perceptions, from the reciprocal implications of our ideas. Never any of our ideas is without the repercussions of the movements elsewhere in the field. When we proof something, we also disproof and correct our startingpoints at the same time. The tradition of the circular nature of human thinking is very old, but not antiquated. The accusation of committing a fallacy in the case of circularity in normal and in scientific thinking is unfouded. It is now the formal logicians turn to defend his claim. (shrink)

Vertaling (verkorte versie) van en commentaar op de wijsgerige verhandeling van de Nederlandse filosoof (1632-1677) over de betekenis van het christendom.