bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Correlation of Skeletal and Cardiac Muscle Dysfunction in Duchenne Muscular Dystrophy
Authors: Posner Andrew D. , Soslow Jonathan H. , Burnette W. Bryan , Bian Aihua , Shintani Ayumi , Sawyer Douglas B. , Markham Larry W. 
Published: March 2016

Background: Duchenne muscular dystrophy (DMD) is characterized by progressive skeletal muscle and cardiac dysfunction. While skeletal muscle dysfunction precedes cardiomyopathy, the relationship between the progressive decline in skeletal and cardiac muscle function is unclear. This relationship is especially important given that the myocardial effects of many developing DMD therapies are largely unknown. Objective: Our objective was to assess the relationship between progression of skeletal muscle weakness and onset of cardiac dysfunction in DMD. Methods: A total of 77 DMD subjects treated at a single referral center were included. Demographic information, quantitative muscle testing (QMT), subjective muscle strength, cardiac function, and current and retrospective medications were collected. A Spearman rank correlation was used to evaluate for an association between subjective strength and fractional shortening. The effects of total QMT and arm QMT on fractional shortening were examined in generalized least square with and without adjustments for age, ambulatory status, and duration of corticosteroids and cardiac specific medications. Results: We found a significant correlation between maintained subjective skeletal muscle arm and leg strength and maintained cardiac function as defined by fractional shortening (rho = 0.47, p = 0.004 and rho = 0.48, p = 0.003, respectively). We also found a significant association between QMT and fractional shortening among non-ambulatory DMD subjects ( p = 0.03), while this association was not significant in ambulatory subjects. Conclusions: Our findings allow us to conclude that in this population, there exists a significant relationship between skeletal muscle and cardiac function in non-ambulatory DMD patients. While this does not imply a causal relationship, a possible association between skeletal and cardiac muscle function suggests that researchers should carefully monitor cardiac function, even when the primary outcome measures are not cardiac in nature.
Aceneuramic Acid Extended Release Administration Maintains Upper Limb Muscle Strength in a 48-week Study of Subjects with GNE Myopathy: Results from a Phase 2, Randomized, Controlled Study
Authors: Argov Zohar , Caraco Yoseph , Lau Heather , Pestronk Alan , Shieh Perry B. , Skrinar Alison , Koutsoukos Tony , Ahmed Ruhi , Martinisi Julia , Kakkis Emil 
Published: March 2016

Background: GNE Myopathy (GNEM) is a progressive adult-onset myopathy likely caused by deficiency of sialic acid (SA) biosynthesis. Objective: Evaluate the safety and efficacy of SA (delivered by aceneuramic acid extended-release [Ace-ER]) as treatment for GNEM. Methods: A Phase 2, randomized, double-blind, placebo-controlled study evaluating Ace-ER 3 g/day or 6 g/day versus placebo was conducted in GNEM subjects ( n = 47). After the first 24 weeks, placebo subjects crossed over to 3 g/day or 6 g/day for 24 additional weeks (dose pre-assigned during initial randomization). Assessments included serum SA, muscle strength by dynamometry, functional assessments, clinician- and patient-reported outcomes, and safety. Results: Dose-dependent increases in serum SA levels were observed. Supplementation with Ace-ER resulted in maintenance of muscle strength in an upper extremity composite (UEC) score at 6 g/day compared with placebo at Week 24 (LS mean difference +2.33 kg, p = 0.040), and larger in a pre-specified subgroup able to walk ≥200 m at Screening (+3.10 kg, p = 0.040). After cross-over, a combined 6 g/day group showed significantly better UEC strength than a combined 3 g/day group (+3.46 kg, p = 0.0031). A similar dose-dependent response was demonstrated within the lower extremity composite score, but was not significant (+1.06 kg, p = 0.61). The GNEM-Functional Activity Scale demonstrated a trend improvement in UE function and mobility in a combined 6 g/day group compared with a combined 3 g/day group. Patients receiving Ace-ER tablets had predominantly mild-to-moderate AEs and no serious adverse events. Conclusions: This is the first clinical study to provide evidence that supplementation with SA delivered by Ace-ER may stabilize muscle strength in individuals with GNEM and initiating treatment earlier in the disease course may lead to better outcomes.
Is There a Consensus when Physicians Evaluate the Relevance of Retrieved Systematic Reviews?
Authors: O’Sullivan Dympna , Kuziemsky Craig , Michalowski Wojtek , Farion Ken , Kukawka Bartosz , Wilk Szymon 
Published: March 2016

SummaryBackground: A significant challenge associated with practicing evidence-based medicine is to provide physicians with relevant clinical information when it is needed. At the same time it appears that the notion of relevance is subjective and its perception is affected by a number of contextual factors.Objectives: To assess to what extent physi -cians agree on the relevance of evidence in the form of systematic reviews for a common set of patient cases, and to identify possible contextual factors that influence their perception of relevance.Methods: A web-based survey was used where pediatric emergency physicians from multiple academic centers across Canada were asked to evaluate the relevance of systematic reviews retrieved automatically for 14 written case vignettes (paper patients). The vignettes were derived from prospective data describing pediatric patients with asthma exacerbations presenting at the emer gency department. To limit the cognitive burden on respondents, the number of reviews associated with each vignette was limited to three.Results: Twenty-two academic emergency physicians with varying years of clinical practice completed the survey. There was no consensus in their evaluation of relevance of the retrieved reviews and physicians’ assessments ranged from very relevant to irrelevant evidence, with the majority of evaluations being somewhere in the middle. This indicates that the study participants did not share a notion of relevance uniformly. Further analysis of commentaries provided by the physicians allowed identifying three possible contextual factors: expected speci ficity of evidence (acute vs chronic condition), the terminology used in the systematic reviews, and the micro environment of clinical setting.Conclusions: There is no consensus among physicians with regards to what constitutes relevant clinical evidence for a given patient case. Subsequently, this finding suggests that evidence retrieval systems should allow for deep customization with regards to physi -cian’s preferences and contextual factors, including differences in the micro environment of each clinical setting.
HOT DUST OBSCURED GALAXIES WITH EXCESS BLUE LIGHT: DUAL AGN OR SINGLE AGN UNDER EXTREME CONDITIONS?
Authors: Assef R. J. , Walton D. J. , Brightman M. , Stern D. , Alexander D. , Bauer F. , Blain A. W. , Diaz-Santos T. , Eisenhardt P. R. M. , Finkelstein S. L. , Hickox R. C. , Tsai C.-W. , Wu J. W. 
Published: March 2016

ABSTRACT
Hot dust-obscured galaxies (Hot DOGs) are a population of hyper-luminous infrared galaxies identified by the Wide-field Infrared Survey Explorer (WISE) mission from their very red mid-IR colors, and characterized by hot dust temperatures (T > 60 K). Several studies have shown clear evidence that the IR emission in these objects is powered by a highly dust-obscured active galactic nucleus (AGN) that shows close to Compton-thick absorption at X-ray wavelengths. Thanks to the high AGN obscuration, the host galaxy is easily observable, and has UV/optical colors usually consistent with those of a normal galaxy. Here we discuss a sub-population of eight Hot DOGs that show enhanced rest-frame UV/optical emission. We discuss three scenarios that might explain the excess UV emission: (i) unobscured light leaked from the AGN by reflection over the dust or by partial coverage of the accretion disk; (ii) a second unobscured AGN in the system; or (iii) a luminous young starburst. X-ray observations can help discriminate between these scenarios. We study in detail the blue excess Hot DOG WISE J020446.13–050640.8, which was serendipitously observed by Chandra/ACIS-I for 174.5 ks. The X-ray spectrum is consistent with a single, hyper-luminous, highly absorbed AGN, and is strongly inconsistent with the presence of a secondary unobscured AGN. Based on this, we argue that the excess blue emission in this object is most likely either due to reflection or a co-eval starburst. We favor the reflection scenario as the unobscured star formation rate needed to power the UV/optical emission would be ≳1000 M
⊙ yr−1. Deep polarimetry observations could confirm the reflection hypothesis.
EXTENDED HEAT DEPOSITION IN HOT JUPITERS: APPLICATION TO OHMIC HEATING
Authors: Ginzburg Sivan , Sari Re’em 
Published: March 2016

ABSTRACT
The observed radii of many giant exoplanets in close orbits exceed theoretical predictions. One suggested origin for this discrepancy is heat deposited deep inside the atmospheres of these “hot Jupiters”. Here, we study extended power sources that distribute heat from the photosphere to the deep interior of the planet. Our analytical treatment is a generalization of a previous analysis of localized “point sources”. We model the deposition profile as a power law in the optical depth and find that planetary cooling and contraction halt when the internal luminosity (i.e., cooling rate) of the planet drops below the heat deposited in the planet’s convective region. A slowdown in the evolutionary cooling prior to equilibrium is possible only for sources that do not extend to the planet’s center. We estimate the ohmic dissipation resulting from the interaction between the atmospheric winds and the planet’s magnetic field, and apply our analytical model to ohmically heated planets. Our model can account for the observed radii of most inflated planets, which have equilibrium temperatures of ≈1500–2500 K and are inflated to a radius of

. However, some extremely inflated planets remain unexplained by our model. We also argue that ohmically inflated planets have already reached their equilibrium phase, and no longer contract. Following Wu & Lithwick, who argued that ohmic heating could only suspend and not reverse contraction, we calculate the time it takes ohmic heating to re-inflate a cold planet to its equilibrium configuration. We find that while it is possible to re-inflate a cold planet, the re-inflation timescales are longer by a factor of

than the cooling time.
The First French Settlement in Texas
Authors: Meyrieux Jacqueline 
Published: March 2016

Abstract
The initial European incursions in the American continent were marked by tenuous and sometimes provocative cultural exchanges, relentless uncertainty and confusion, muddled nostalgia, horrific violence, and immense distances. The recent discovery of the shipwreck The Belle in Matagorda Bay has reinvigorated scholarly interest in the French colony founded by René-Robert Cavelier (Sieur de La Salle) in Texas in the late seventeenth century. The company's attempt to bring European culture and life to the Americas was colored by local customs, class distinctions, long stints of boredom, vengeance, and murder; there were also several important social transitions, including that of men and women who abandoned European life altogether, conflicting in-group etiquettes among the French (including mutiny), and the looming complications of a return home. This article examines the diary of Henri Joutel, one of the members of the La Salle company who survived the ordeal and returned to France. It focuses on how the company affiliates balanced harsh labor and constant uncertainty with home-rituals involving food consumption and religious ceremony, and measured interactions (including military alliances) with Native Americans, which became enormously important to their subsistence.
“No Perfect House”: Destabilized Domesticity by Border Crosser Annie Proulx
Authors: Jocelyn Hannah May 
Published: March 2016

Abstract
To cross the carefully mediated border between the United States and Canada is to become unsettled. As a woman who has lived long-term stints in both countries, contemporary writer Annie Proulx has closely experienced this physical and emotional dislocation. In interviews, she has mentioned feeling rootless, as though a “perpetual outsider” (Lafreniere, 2009). Perhaps engendered by the lingering effect concomitant to border crossing, this rootlessness is represented in her work by transient characters and deteriorating homes. Like Proulx, her fictional characters are outsiders, an illustration of the liminality contracted by border crossing. Their dislocation serves to dismantle myths of the home and home life and to disrupt the historically established gender binary. This paper investigates how Proulx's background as a border crosser allows her the insight to write subtly yet truthfully of being unsettled so that we may read a demystification of domesticity.
“Either feed your belly or nourish your soul”: Work, Artistic Aspiration and Autobiography in Rosa R. Cappiello's Oh Lucky Country
Authors: Carniel Jessica 
Published: March 2016

Abstract
Written during the tentative first decade of multicultural policy in Australia and set in the 1970s, Cappiello's Oh Lucky Country (1984) reveals a view of Australian multiculturalism as exploitative of migrants and their ethnic cultures. The protagonist and narrator, Rosa, struggles with her intersecting identities as a migrant and as a writer within this new multicultural paradigm; her migrant status often thwarts her literary ambitions yet, as a migrant who writes, she presents a desirable cultural resource. Her cultural value as a writer is, however, secondary to her economic value as a worker; Rosa struggles against her working class reality, which she sees as taking time away from her true literary vocation. This paper explores the tension between Rosa's desire to write and her need to work, and the values of these different types of labour within multicultural Australia. It argues that each of Rosa's identities—writer, worker, migrant—all reflect a sense of isolation and marginalisation from both her host society and her ethnic community. It also examines the text's generic categorisation as autobiographical fiction, with a particular consideration of how autobiographical writing by migrants is received popularly and critically.
Omgevingswet: maak de ambities waar, maar maak het niet ingewikkelder dan het al is
Authors: Nijmeijer A.G.A. (Tonny) 
Published: March 2016

Omgevingswet: maak de ambities waar, maar maak het niet ingewikkelder dan het al is
In dit gastredactioneel wordt het advies van de Raad voor de leefomgeving en infrastructuur over de AMvB’s onder de Omgevingswet besproken.
Het omgevingsplan in de Omgevingswet
Authors: van Angeren J.R. (Jan Reinier) 
Published: March 2016

Het omgevingsplan in de Omgevingswet
In dit artikel wordt het omgevingsplan besproken. Na een weergave van de totstandkomingsgeschiedenis van dit instrument wordt de inhoud van het omgevingsplan behandeld. Aan de orde komen de verschillende regels die het omgevingsplan moet bevatten, de bevoegdheid om het omgevingsplan vast te stellen, de omgevingswaarden en de programma’s. Ook wordt ingegaan op de wijze waarop het omgevingsplan als toetsingskader voor omgevingsvergunningen gaat functioneren.
Reactie op ‘Kanttekeningen bij de Utrechtse kantorenaanpak’
Authors: de Vries H.J. (Henk) 
Published: March 2016

Reactie op ‘Kanttekeningen bij de Utrechtse kantorenaanpak’
In deze bijdrage reageert de auteur op de kanttekeningen die door Groothuijse en Korsse zijn geplaatst bij zijn artikel in TO 2015, afl. 3, p. 113-132
De ervaren mogelijkheden om te re-integreren vanuit detentie
Authors: Molleman Toon , Lasthuizen Karin 
Published: March 2016

The experienced possibilites to reintegrate from detention
In this article we examine which factors are correlated to the rehabilitation of prisoners. Central is the prisoners’ willingness during their imprisonment to work on a successful return in society. Assumptions derived from the theory were statistically tested with data from the Dutch Detainee Survey 2014; a large-scale study, in which prisoners held in all Dutch penitentiary institutions (N = 29) were surveyed (n = 2120). The results are in line with the theory and show that detainees are more positive about their (potential for) rehabilitation when they are more satisfied with a) the way they are treated by correctional officers (staff orientation); b) active deployment of the so-called Detention and Re-integration Plan; c) their daily program and activities, and; d) the experienced autonomy and personal decision-making. These outcomes offer fruitful perspectives on how the prison system can promote a successful return of prisoners in our society.
Politie en burgers: van informatie delen naar volwaardige samenwerking
Authors: Kerstholt José , de Vries Arnout , Huis in ’t Veld Mirjam 
Published: March 2016

Police and citizens: from sharing information to full co-production
Community oriented policing (COP) can be seen as a co-production between citizens and police (and other stakeholders) in increasing public safety. There is a broad range of projects to cooperate with citizens. We ordered these projects along two dimensions: level of co-production (informing/consulting, advising and co-creation) and domain (prevention, law and order, investigation and quality of life). Today, most initiatives are at the lowest rungs (informing and consulting), but social media provide new possibilities to involve citizens in a more direct and fast way. The effects of the various projects are mostly limited to psycho-social factors like experienced safety, feelings of control and legitimacy. However recent studies show that these factors may have an indirect effect on crime rate. Furthermore, as reducing crime rate was not the only goal for introducing COP effect studies should not be limited to crime rate but incorporate a broad range of indicators.
Educação Especial, Educação Inclusiva e Pedagogia da Diversidade: Celebrar a diversidade! Exaltar a tolerância! Notabilizar o respeito! Proclamar a solidariedade!
Authors: Lunardi-Lazzarin Márcia Lise , Hermes Simoni Timm 
Published: March 2016

Na articulação da Educação Especial com a Educação Inclusiva, este artigo objetiva problematizar como a Pedagogia da Diversidade tem perpassado a formação continuada das professoras e as práticas curriculares ditas inclusivas na escola contemporânea. A partir dos Estudos Foucaultianos em Educação e dos Estudos Culturais, a materialidade analítica constitui-se por relatos da obra “Atendimento Educacional Especializado no Brasil: relatos da experiência profissional de professores e sua formação”, derivada do Curso de Aperfeiçoamento/Extensão em Atendimento Educacional Especializado, ofertado por uma universidade pública do sul do país, na Rede Nacional de Formação Continuada de Professores na Educação Especial. Nessa materialidade analítica, o direito à educação reveste-se como direito à educação da diversidade, e este direito potencializa as operações do Atendimento Educacional Especializado na escola dita inclusiva. Nesse contexto, a Política Nacional de Educação Especial na Perspectiva da Educação Inclusiva aciona o preparo para o acolhimento e o trato da diversidade, de modo a intensificar a formação continuada das professoras, em detrimento da formação inicial, e produzir a informação, a opinião, a generalização, a rapidez e o pragmatismo do atual serviço da Educação Especial nesses cursos de aperfeiçoamento/extensão. Celebrar a diversidade! Exaltar a tolerância! Notabilizar o respeito! Proclamar a solidariedade! Por meio da tríade da tolerância, do respeito e da solidariedade, essa formação continuada e as práticas curriculares na escola dita inclusiva produzem modos de sociabilidade plurais, inclusivos no registro da governamentalidade neoliberal.
Induction of CCAAT/Enhancer-Binding Protein β Expression With the Phosphodiesterase Inhibitor Isobutylmethylxanthine Improves Myoblast Engraftment Into Dystrophic Muscle
Authors: Lala-Tabbert Neena , Fu Dechen , Wiper-Bergeron Nadine 
Published: March 2016

Abstract

Duchenne muscular dystrophy (DMD), caused by mutations in the dystrophin gene, is the most common muscular dystrophy. Characterized by rounds of muscle degeneration and regeneration, DMD features progressive muscle wasting and is fatal. One approach for treatment is transplantation of muscle progenitor cells to repair and restore dystrophin expression to damaged muscle. However, the success of this approach has been limited by difficulties in isolating large numbers of myogenic progenitors with strong regenerative potential, poor engraftment, poor survival of donor cells, and limited migration in the diseased muscle. We demonstrate that induction of the transcription factor CCAAT/enhancer-binding protein β (C/EBPβ) using the cyclic adenosine monophosphate phosphodiesterase inhibitor isobutylmethylxanthine (IBMX) results in enhanced myoblast expansion in culture and increased satellite cell marker expression. When equal numbers of IBMX-treated cells were transplanted into dystrophic muscle, they contributed to muscle repair more efficiently than did vehicle-treated cells and engrafted into the satellite cell niche in higher numbers, demonstrating improved cell migration from the site of injury and enhanced survival after transplantation. Thus, pharmacologic stimulation of C/EBPβ expression reprograms myoblasts to a more stem cell-like state, promotes expansion in culture, and improves engraftment such that better transplantation outcomes are achieved.

Significance
Duchenne muscular dystrophy is a genetic disorder for which no cure exists. One therapeutic approach is transplantation of myogenic progenitors to restore dystrophin to damaged muscle, but this approach is limited by poor engraftment of cultured myoblasts. Transient upregulation of CCAAT/enhancer-binding protein β in primary myoblasts using the phosphodiesterase isobutylmethylxanthine (IBMX) increases satellite cell marker expression in cultured myoblasts, improves their migration, and increases their survival after transplantation. When transplanted into C57BL/10ScSn-mdx/J mice , IBMX-treated myoblasts restored dystrophin expression and were able to occupy the satellite cell niche more efficiently than controls. A myoblast culture approach that reprograms myoblasts to a more primitive state, resulting in improved transplantation outcomes and reinvigorating research into myoblast transplantation as a viable therapeutic approach, is described.
A Rational Strategy for the Use of Amniotic Epithelial Stem Cell Therapy for Liver Diseases
Authors: Miki Toshio 
Published: March 2016

Summary

Stem cell-based therapies hold the potential to alleviate the burden of many serious diseases, including those of the liver. Among the different types of stem cells, human placenta-derived stem cells are potentially one of the most clinically applicable stem cells because of their tissue-specific advantages. They are a readily available cell source that can be procured in a noninvasive manner, and there are few ethical concerns regarding their use. Recent studies have demonstrated that the amniotic epithelium contains stem cells that possess four unique and advantageous properties; human amniotic epithelial cells (hAECs) have low immunogenicity, secrete several immune regulatory molecules, possess the potential to differentiate into all three germ layers, and contain abundant lysosomes allowing them to secrete lysosomal enzymes. This perspective article provides an overview of the beneficial properties of hAECs and proposes a rational strategy for translating placental stem cells toward clinical application for various liver diseases.

Significance
This article provides an overview of the beneficial properties of one type of human placental stem cell and proposes a rational strategy for translating placental stem cells toward clinical application for various liver diseases.