bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mutational analysis of PHEX, FGF23 and CLCN5 in patients with hypophosphataemic rickets
Authors: Guven Ayla , Al‐Rijjal Roua A. , BinEssa Huda A. , Dogan Durmuş , Kor Yılmaz , Zou Minjing , Kaya Namik , Alenezi Anwar F. , Hancili Suna , Tarım Ömer , Baitei Essa Y. , Kattan Walaa E , Meyer Brian F. , Shi Yufei 
Published: April 2017

SummaryContextHypophosphataemic rickets (HR) is a group of rare hereditary renal phosphate wasting disorders caused by mutations in PHEX, FGF23, DMP1, ENPP1, CLCN5 or SLC34A3.ObjectiveTo investigate underlying genetic defects in patients with hypophosphataemic rickets.MethodsWe analysed genomic DNA from nine unrelated families for mutations in the entire coding region of PHEX, FGF23, DMP1, ENPP1, CLCN5 or SLC34A3 by PCR sequencing and copy number analysis.ResultsA total of 14 patients were studied. PHEX mutations were identified in 12 patients from seven families. Five of them were novel mutations present in eight patients: c.154G>T (p.E52*), c.401_402insGCCAAA (p.Q134_K135insPK), c.1600C>T (p.P534S), g.22016715_22056805del (40‐kb deletion including promoter and exons 1‐3) and c.2242_2243delCT (p.L748 fs*48). Four patients had previously reported mutations: c.1768+1G>A and c.1807G>A (p.W602*). Novel CLCN5 (c.1205G>A, p.W402*) and FGF23 (c.526C>G, p.R176G) mutations were found in two patients from the remaining two families. Many of the mutations were de novo: c.154G>T and c.2242_2243delCT in PHEX and c.526C>G in FGF23. Furthermore, we characterized the breakpoint of the novel PHEX g.22016715_22056805del and the c.2242_2243delCT, which is 6 bp from the stop codon, resulting in a frameshift and extension of the reading frame by 42 amino acids.ConclusionsNovel and de novo mutations are frequent and PHEX mutations are still the most common genetic defects in the Turkish population. Gene copy number analysis should be considered in patients with negative results by conventional PCR‐based sequencing analysis. The current study further expands the mutation spectrum underlying HR.
Implantation of two second‐generation trabecular micro‐bypass stents and topical travoprost in open‐angle glaucoma not controlled on two preoperative medications: 18‐month follow‐up
Authors: Berdahl John , Voskanyan Lilit , Myers Jonathan S , Hornbeak Dana M , Giamporcaro Jane Ellen , Katz L Jay , Samuelson Thomas W 
Published: April 2017

AbstractImportanceAdditional data are sought regarding treatment options for glaucoma, a major cause of global blindness.BackgroundThe study assessed outcomes following standalone implantation of two second‐generation trabecular micro‐bypass stents and postoperative topical prostaglandin in eyes with open‐angle glaucoma not controlled on two preoperative medications.DesignThe study design is a prospective, nonrandomized, open‐label study at a tertiary‐care ophthalmology centre.ParticipantsSubjects had open‐angle glaucoma with preoperative intraocular pressure of 18–30 mmHg on two medications, a medication washout phase, and post‐washout intraocular pressure of 22–38 mmHg. All subjects (N = 53) have been followed for 18 months.MethodsOne day following implantation of two second‐generation trabecular micro‐bypass stents, subjects started topical travoprost. Medication washout was repeated at month 12.Main Outcome MeasuresThe main outcome measure was the proportion of eyes with intraocular pressure reduction ≥ 20% versus medicated baseline intraocular pressure with reduction of one medication at 12 months.ResultsAt 12 months, 91% of eyes achieved intraocular pressure reduction ≥ 20% with reduction of one medication. All eyes had intraocular pressure ≤ 18 mmHg with reduction of one medication, and 87% had intraocular pressure ≤ 15 mmHg. Mean intraocular pressure on one medication was ≤ 13.0 mmHg (≥ 34% reduction) through 18 months. Mean post‐washout intraocular pressure at month 13 was 33% lower than preoperative unmedicated intraocular pressure. No adverse events occurred through 18 months.Conclusions and RelevanceIn open‐angle glaucoma eyes on two preoperative medications, treatment with two second‐generation trabecular stents and one postoperative prostaglandin resulted in mean intraocular pressure ≤ 13 mmHg with reduction of one medication, with favourable safety. These findings show the utility of second‐generation trabecular bypass with postoperative prostaglandin in patients with open‐angle glaucoma.
Governors matter
Authors: Sharafutdinova Gulnaz , Steinbuks Jevgenijs 
Published: April 2017

AbstractThis study uses the latest 2011 round of the Business Environment and Enterprise Performance Survey for the Russian Federation to take a closer look at regional‐level factors influencing the business environment in Russia. Specifically, the study explores the role of regional administrations and variables of administrative continuity and governor origin in shaping regional business environment. The findings reveal that regional businesses in Russia are (1) acutely anxious about administrative transitions (as expressed in gubernatorial replacements) and favour administrative continuity, and (2) favour government officials that are locally embedded. The analysis suggests that many localities in Russia have witnessed the emergence of mutually beneficial state‐business arrangements that are inimical to economic competition.
The Free Harmony of the Faculties and the Primacy of Imagination in Kant's Aesthetic Judgment
Authors: Ostaric Lara 
Published: April 2017

AbstractThis essay argues that, contrary to the prevailing view according to which reflection in Kant's aesthetic judgment is interpreted as ‘the logical actus of the understanding’, we should pay closer attention to Kant's own formulation of aesthetic reflection as ‘an action of the power of imagination’. Put differently, I contend in this essay that the rule that governs and orders the manifold in aesthetic judgment is imagination's own achievement, the achievement of the productive synthesis of the ‘fictive power’ (Dichtungsvermögen), entirely independent of the understanding. While this view does not entail that the faculty of the understanding is not necessary in aesthetic reflection, a stronger emphasis on the role of imagination in aesthetic reflection allows us to realize that its schematizing and interpretive activity, while consistent with, goes well beyond the discursive demands of the understanding insofar as it intimates the supersensible ground of freedom that manifests itself as ‘the feeling of life’. Therefore, I show in this essay that the imagination's unique interpretive power has a special role in completing Kant's critical system by facilitating the connection of the sensible to the supersensible, which further helps us appreciate imagination's practical as opposed to merely cognitive significance.
Shifting barriers and phenotypic diversification by hybridisation
Authors: Sefc Kristina M. , Mattersdorfer Karin , Ziegelbecker Angelika , Neuhüttler Nina , Steiner Oliver , Goessler Walter , Koblmüller Stephan 
Published: April 2017

AbstractThe establishment of hybrid taxa relies on reproductive isolation from the parental forms, typically achieved by ecological differentiation. Here, we present an alternative mechanism, in which shifts in the strength and location of dispersal barriers facilitate diversification by hybridisation. Our case study concerns the highly diverse, stenotopic rock‐dwelling cichlids of the African Great Lakes, many of which display geographic colour pattern variation. The littoral habitat of these fish has repeatedly been restructured in the course of ancient lake level fluctuations. Genetic data and an experimental cross support the hybrid origin of a distinct yellow‐coloured variant of Tropheus moorii from ancient admixture between two allopatric, red and bluish variants. Deficient assortative mating preferences imply that reproductive isolation continues to be contingent on geographic separation. Linking paleolimnological data with the establishment of the hybrid variant, we sketch a selectively neutral diversification process governed solely by rearrangements of dispersal barriers.
Shaping the professional landscape through research, advocacy and education – an Australian perspective
Authors: Siemensma Gemma , Ritchie Ann , Lewis Suzanne 
Published: April 2017

AbstractThis article is the first in a new series in this regular feature. The intention of the series is to look at important global developments in health science libraries. Librarians will be invited to share with HILJ readers key initiatives in their country or region. These articles should serve as a road map, describing the key changes in the field and exploring factors driving these changes. We initiate this series with an article by three Australian librarians who use research findings to depict the evolving professional landscape in their country. The starting point of their analysis is a report completed in 2011 which looked into likely future workforce and education requirements for health library professionals. The authors trace the achievements since then, most notably in the areas of research, advocacy and education. Clearly, a great deal has been achieved leading to a greater return on investment. The authors maintain that the key to shaping the profession and enhancing the status of librarians is ongoing professional development. To this end, Australia is promoting a systematic, competency based health specialist certification. Finally, they identify trends impacting on health librarianship, such as the growing importance of research data management and consumer health literacy.JM
The Context and Impact of Being Wrongly Accused of Abuse in Occupations of Trust
Authors: BURNETT ROS , HOYLE CAROLYN , SPEECHLEY NAOMI‐ELLEN 
Published: April 2017

AbstractIn recent years, there has been rising concern that allegations of sexual abuse, particularly non‐recent abuse, have not received an appropriate response. From this has emerged a new determination to correct past and prevent further injustices, with police operations focusing considerable resources on the identification and prosecution of child abusers. Police and other services have reached out to encourage reporting, and developments in the trial process related to the rules of evidence have eroded due process protections for suspects. This article considers this changed legal and social context, and the processes entailed in responding to allegations of abuse, before presenting original empirical data, gathered from the accounts of 30 men and women who were wrongly accused of abuse related to their employment in occupations of trust. It demonstrates the considerable and lasting harms done to those who face allegations of such heinous crimes.
The Case for Activist Directors: A Conversation with Ira M. Millstein
Authors: Millstein Ira M. , Colvin Geoff 
Published: April 2017

In this conversation held at the 2016 Millstein Governance Forum at Columbia Law School, Ira Millstein, a leading authority on corporate governance and founding chair of the Millstein Center for Global Markets and Corporate Ownership, discusses his new book, The Activist Director, with Geoff Colvin of Fortune Magazine. In explaining why he wrote the book, Millstein said that it is important for boards of directors to understand the key role they must play to secure the future of our corporations, and for shareholders to recognize, encourage, and support this role.The role of directors has changed significantly over the years. Yet corporate performance, broadly speaking, has not lived up to expectations, and Millstein attributes this in part to the failure of directors to adapt and evolve quickly and decisively enough—which in turn has helped to fuel the rise of activist investors. Much of the problem stems from the tendency of boards to view themselves as oversight organizations that review and “challenge” management at arm's length, as opposed to truly engaging with management to make better decisions.To address this problem, Millstein makes the case for “activist” directors who will partner with management, think deliberately and critically about the company's strategy, and work for the longterm interest of the corporation. And to provide financial incentives for directors to reinforce their commitment to the corporations they serve, Millstein favors an increase in compensation for directors that is tied to long‐term performance.As an early example of what became an activist board, Millstein describes his own experience with the board of General Motors in the late 1980s and early 1990s when it confronted managerial failure and ended up replacing the CEO. In the current environment of activist investors, activist boards must give serious consideration to shareholder proposals for change, without succumbing to pressure for shortsighted cutbacks in value‐adding investment and while ensuring that management is focused on long‐term growth and innovation. Directors must have the courage and commitment to carry out the course of action they deem to be in the best longterm interests of the corporation.
The Evolution of Executive Pay Policy at General Motors, 1918–2008
Authors: O'Byrne Stephen F. , Young S. David 
Published: April 2017

This article documents the gradual movement of General Motors away from the partnership concept that dominated U.S. corporate pay policy in the first half of the 20th century and toward the “competitive pay” concepts that have prevailed since then. The partnership concept was achieved by paying managers bonuses in the form of GM shares, with the amounts paid out of a single company‐wide bonus pool and based on a fixed share of profit (after subtracting a charge for the cost of capital). Thanks to this “EVA‐like” bonus scheme, GM's managers effectively became “partners” with the company's shareholders, sharing the wealth in good times but also the pain in troubled times. What's more, the authors also show that, from the establishment of the program in 1918 through the 1950s, the directors went to great lengths—including several bouts of innovative (and often complex) problem‐solving—to achieve their compensation objectives while maintaining such fixed‐share bonuses.But the sharing philosophy and associated compensation practices were gradually supplanted by competitive pay practices from the 1960s onward. The authors show that by the late 1970s, GM had a board of directors with modest shareholdings, in contrast to the board in the early post‐war period, whose directors had large stakes. As a consequence, directors began acting less like stewards of capital and more like employees whose financial rewards came not from returns on GM's stock but from the fees they received for their services. This fundamental change in board compensation almost certainly contributed to the gradual abandonment of fixed‐profit sharing for GM's managers. In its place, the board implemented competitive pay policies that, while coming to dominate executive pay policy in the U.S. and abroad, have largely divorced executive pay from changes in shareholder wealth. In the case of GM, this growing separation of pay from performance was accompanied by a significant decline in corporate returns on operating capital as well as stock returns over time.
Corporate Governance and the Investment Efficiency of Diversified Corporate Asset Buyers
Authors: Chen I‐Ju , Chen Sheng‐Syan 
Published: April 2017

Many corporate assets are bought and sold each year in the U.S. and most scholars believe these transactions improve economic efficiency. But given the reality that the interests of corporate managers may diverge from those of their shareholders and reflect empire‐building or other managerial entrenchment strategies—and that such agency problems tend to be worse in highly diversified, multi‐divisional companies—the authors tested the proposition that diversified corporate asset buyers with more effective governance structures can be expected to allocate capital more efficiently, as reflected in higher rates of return on operating capital and more favorable market reactions to the announcements of their purchases.Using a sample of diversified U.S. companies that announced large asset purchases between 1988 and 2006, the authors report finding that the investment allocation process following such asset purchases was more consistent with value creation in the case of diversified buyers with more effective governance structures, which were identified by their greater board independence, higher‐quality audit committees, and higher levels of stock ownership by institutional ownership, directors, and CEOs.
Rationally designed ligand‐independent peptide inhibitors of TREM‐1 ameliorate collagen‐induced arthritis
Authors: Shen Zu T. , Sigalov Alexander B. 
Published: April 2017

AbstractTriggering receptor expressed on myeloid cells 1 (TREM‐1) is critically involved in the pathogenesis of rheumatoid arthritis (RA). In contrast to cytokine blockers, therapeutic blockade of TREM‐1 can blunt excessive inflammation while preserving the capacity for microbial control. However, the nature of the TREM‐1 ligand(s) and mechanisms of TREM‐1 signalling are still not yet well understood, impeding the development of clinically relevant inhibitors of TREM‐1. The aim of this study was to evaluate the anti‐arthritic activity of a novel, ligand‐independent TREM‐1 inhibitory nonapeptide GF9 that was rationally designed using the signalling chain homo oligomerization (SCHOOL) model of cell signalling. Free GF9 and GF9 bound to macrophage‐targeted nanoparticles that mimic human high‐density lipoproteins (GF9‐HDL) were used to treat collagen‐induced arthritis (CIA). We also tested if 31‐mer peptides with sequences from GF9 and helices 4 (GE31) and 6 (GA31) of the major HDL protein, apolipoprotein A‐I, are able to perform three functions: assist in the self‐assembly of GA/E31‐HDL, target these particles to macrophages and block TREM‐1 signalling. We showed that GF9, but not control peptide, ameliorated CIA and protected against bone and cartilage damage. The therapeutic effect of GF9 was accompanied by a reduction in the plasma levels of macrophage colony‐stimulating factor and pro‐inflammatory cytokines such as tumour necrosis factor‐α, interleukin (IL)‐1 and IL‐6. Incorporation of GF9 alone or as a part of GE31 and GA31 peptides into HDL significantly increased its therapeutic efficacy. Collectively, our findings suggest that TREM‐1 inhibitory SCHOOL sequences may be promising alternatives for the treatment of RA.
Signaling by an informed service provider
Authors: Lee Frances Xu , Fong Yuk‐fai 
Published: April 2017

AbstractWe study a service provider, who, at the time of offering a contract, is better informed than the potential client. A service provider that is hired to increase the client's chance of a gain, an “enhancer,” may be better informed of whether the client has a big or small opportunity. A service provider that is hired to reduce the client's chance of a loss, a “problem solver,” may be better informed of whether the client has a big or small problem. We show that an enhancer predominantly offers a contingent contract, while a problem solver predominantly offers a flat fee due to their signaling incentives. This explains the differences in real‐world contracts and also provides a novel explanation for the existence of low‐powered incentive contracts. We evaluate the policy intervention that limits the contingent part of the service providers' contracts.
Effects of a Multicomponent Restraint Reduction Program for Korean Nursing Home Staff
Authors: Kong Eun‐Hi , Song Eunjin , Evans Lois K. 
Published: April 2017

AbstractPurposePhysical restraints are used frequently in Korea, suggesting a growing need for access to programs focused on reduction. The aim of this study was to evaluate the effects of a multicomponent restraint reduction program (MRRP) for nursing staff in Korean nursing homes.DesignA cluster‐randomized, single‐blind, controlled pretest‐posttest design was used. A total of 122 nursing staff (nurses and geriatric care assistants) in two Korean nursing homes participated in this study: 62 in the experimental group (EG) and 60 in the control group (CG).MethodsNursing staff in the EG home received the MRRP comprising three educational sessions (two classroom‐based and one web‐based) and two unit‐based consultations. Three instruments were used to measure nursing staff's knowledge, perceptions, and attitudes regarding physical restraints. Data were collected immediately before and after the intervention, and again 1 and 3 months later.FindingsRepeated measures analysis of variance showed significant differences between groups in knowledge (p < .001), perceptions (p< .001), and attitudes (p = .011) over time. These significant improvements in the MRRP group (EG) were sustained over the 3‐month period.ConclusionsThe MRRP effectively improved the knowledge, perceptions, and attitudes of nursing home staff about restraint use with older adults. Additional studies are recommended to evaluate effects of its components while using larger samples and rigorous research methods and measurements, and the inclusion of boosters or other supports to sustain change.Clinical RelevanceThese results provide valuable knowledge regarding a multicomponent intervention for changing nursing home staff attributes that likely influence clinical practice. Elements of the educational content and methods found useful for nursing home staff may also be effective in vocational and continuing education as well as for families of older nursing home residents.
Autologous platelet‐rich plasma versus readymade growth factors in skin rejuvenation: A split face study
Authors: Gawdat Heba I , Tawdy Amira M , Hegazy Rehab A , Zakaria Mohga M , Allam Ryham S 
Published: April 2017

SummaryBackgroundThe escalating urge for a youthful‐looking skin instigates continuous innovations with minimally invasive procedures. Readymade growth factors and autologous platelet‐rich plasma (PRP) represent such therapeutic interventions.ObjectiveCompare the efficacy and safety of PRP to readymade growth factors in skin rejuvenation.Patients and MethodsTwenty adult females with Fitzpatrick skin types III‐IV and Glogau photoaging types II and III were enrolled in this study. They underwent a split face therapy where each side was randomly assigned to treatment by either readymade growth factors (area A) or autologous PRP (area B). All patients received six sessions at 2‐weeks interval. Evaluation was carried out using Global Aesthetic Improvement Scale (GAIS) and optical coherence tomography (OCT). Patients were followed up for 6 months.ResultsBoth procedures yielded significant improvement regarding both GAIS (skin turgor and overall vitality) and OCT (epidermal and dermal thickness) assessment. Significant negative correlation was detected between patients' age, sun exposure, and GAIS. Burning sensation was significantly higher in area A. Patient satisfaction was significantly higher in area B. Improvement was more sustained in area B on follow‐up.ConclusionPlatelet‐rich plasma is effective and safe for skin rejuvenation, comparable to readymade growth factors with noticeable higher longevity.