bluuebunny/crossref_metadata_2025_split huggingface dataset:
Characteristics of Japanese Patients with Becker Muscular Dystrophy and Intermediate Muscular Dystrophy in a Japanese National Registry of Muscular Dystrophy (Remudy): Heterogeneity and Clinical Variation
Authors: Mori-Yoshimura Madoka , Mitsuhashi Satomi , Nakamura Harumasa , Komaki Hirofumi , Goto Kanako , Yonemoto Naohiro , Takeuchi Fumi , Hayashi Yukiko K. , Murata Miho , Takahashi Yuji , Nishino Ichizo , Takeda Shin’ichi , Kimura En 
Published: March 2018

Background: Obtaining an adequate number of patients to conduct a natural history study for rare diseases such as Becker muscular dystrophy (BMD) is difficult. Objectives: The present study used data from Remudy, a national registry for neuromuscular diseases in Japan, to conduct a phenotypic analysis of BMD. Methods: We analyzed Remudy data of participants with dystrophinopathy. All participants who were aged 17 and older and were ambulant at age 13 were included in this study. Participants were divided into two groups: those with BMD who were ambulant at age 17, and those with intermediate muscular dystrophy (IMD) who lost ambulation by age 17. Frequent mutations were analyzed by age at ambulation, cardiopulmonary function, and genotype. For clinical comparisons, participants who were administered steroids were excluded. Results: From July 2009 through September 2015, 192 participants had registered with Remudy. Mean participant age was 34.80±13.3 (range, 17–78) years, and 52.1% of participants were ambulant. Of the entire study population, 50.5% had cardiomyopathy and 35.9% had respiratory failure. Three participants required invasive ventilation and 30 required non-invasive ventilation. Nineteen of the 30 non-invasive ventilator users were part-time users. In total, 138 (71.9%) had BMD and 54 (28.1%) had IMD. The most frequent mutation was ex45_ex47del (36 participants). Among participants with frequent in-frame mutations, those with the ex45-49del mutation lost their ambulation earlier than those with the ex45_ex47del mutation. A total of 67 different exon deletions and duplications were identified in the study population. Conclusion: We clarified the clinical phenotypes of Japanese patients with BMD/IMD using data from Remudy. Our results suggest that not only IMD but also BMD are associated with risk of respiratory dysfunction.
Problems in Comparing Jitter Values Obtained with Voluntary Activation and Electrical Stimulation
Authors: Stålberg Erik , Kouyoumdjian João Aris , Paiva Gabriel Pina , Yanaze Larissa Luri , Sanders Donald B. 
Published: March 2018

Comparing results from jitter studies performed with voluntary and electrical activation is difficult to perform quantitatively, particularly in complex signals as seen in reinnervation. High jitter values in individual spikes in these multispike signals can be missed with both activation methods, which introduces a bias towards more normal values. With voluntary activation, triggering on a spike from an abnormal end-plate in multispike potentials will overestimate individual jitter values and the number of abnormal jitter values. With electrical stimulation, artefactually-increased jitter may be caused by subliminal stimulation, which causes uncertainty at the stimulation point. Electrical stimulation also may activate many axons, causing signal summation, with erroneous or impossible jitter estimation. Awareness of such pitfalls can improve the correct performance and interpretation of jitter recordings. Quantitative comparisons of results can be made between studies in reinnervated muscle performed with the same activation method, but not between results obtained with different activation methods.
Treatment Algorithm for Infants Diagnosed with Spinal Muscular Atrophy through Newborn Screening
Authors: Glascock Jacqueline , Sampson Jacinda , Haidet-Phillips Amanda , Connolly Anne , Darras Basil , Day John , Finkel Richard , Howell R. Rodney , Klinger Katherine , Kuntz Nancy , Prior Thomas , Shieh Perry B. , Crawford Thomas O. , Kerr Douglas , Jarecki Jill 
Published: March 2018

Background: Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by the degeneration of alpha motor neurons in the spinal cord, leading to muscular atrophy. SMA is caused by deletions or mutations in the survival motor neuron 1 gene ( SMN1). In humans, a nearly identical copy gene, SMN2, is present. Because SMN2 has been shown to decrease disease severity in a dose-dependent manner, SMN2 copy number is predictive of disease severity. Objective: To develop a treatment algorithm for SMA-positive infants identified through newborn screening based upon SMN2 copy number. Methods: A working group comprised of 15 SMA experts participated in a modified Delphi process, moderated by a neutral third-party expert, to develop treatment guidelines. Results: The overarching recommendation is that all infants with two or three copies of SMN2 should receive immediate treatment ( n = 13). For those infants in which immediate treatment is not recommended, guidelines were developed that outline the timing and appropriate screens and tests to be used to determine the timing of treatment initiation. Conclusions: The identification SMA affected infants via newborn screening presents an unprecedented opportunity for achievement of maximal therapeutic benefit through the administration of treatment pre-symptomatically. The recommendations provided here are intended to help formulate treatment guidelines for infants who test positive during the newborn screening process.
Curcumin supplementation and motor-cognitive function in healthy middle-aged and older adults
Authors: Santos-Parker Jessica R. , Lubieniecki Kara L. , Rossman Matthew J. , Van Ark Hannah J. , Bassett Candace J. , Strahler Talia R. , Chonchol Michel B. , Justice Jamie N. , Seals Douglas R. 
Published: March 2018

BACKGROUND: Recent studies suggest curcumin is a promising nutraceutical for improving important clinical and physiological markers of healthy aging, including motor and cognitive function. OBJECTIVE: To determine if curcumin supplementation improves motor and cognitive function in healthy middle-aged and older adults. METHODS: 39 healthy men and postmenopausal women (45–74 yrs) were randomized to 12 weeks of placebo ( n = 19) or curcumin supplementation (2000 mg/day Longvida®; n = 20) with motor and cognitive function assessed at week 0 and 12. RESULTS: Using measures of the NIH Toolbox and other standardized tests, there were no changes in muscle strength and rate of torque development, dexterity, fatigability, mobility, endurance, and balance between the placebo and curcumin groups after 12 weeks (all P > 0.05). Additionally, there were no changes after 12 weeks of placebo and curcumin supplementation in measures of fluid cognitive ability, a cognitive domain that declines with age, including processing speed, executive function, working memory, and episodic memory (all P > 0.3). There were marginal changes in language, a measure of crystallized cognitive ability that is stable with age, following the intervention, wherein reading decoding increased 3% in the curcumin group (post: 2428±35 vs. pre: 2357±34, P = 0.003), but was unchanged in the placebo group (post: 2334±39 vs. pre: 2364±40, P = 0.07). CONCLUSIONS: Overall, 12 weeks of curcumin supplementation does not improve motor and cognitive functions in healthy middle-aged and older adults. It is possible that curcumin may enhance these functions in groups with greater baseline impairments than those studied here, including adults greater than 75 years of age and/or patients with clinical disorders.
Evolution of Magnetic Rayleigh–Taylor Instability into the Outer Solar Corona and Low Interplanetary Space
Authors: Mishra Sudheer K. , Singh Talwinder , Kayshap P. , Srivastava A. K. 
Published: March 2018

Abstract
We analyze the observations from Solar TErrestrial RElations Observatory (STEREO)-A and B/COR-1 of an eruptive prominence in the intermediate corona on 2011 June 7 at 08:45 UT, which consists of magnetic Rayleigh–Taylor (MRT) unstable plasma segments. Its upper-northward segment shows spatio-temporal evolution of MRT instability in form of finger structures up to the outer corona and low interplanetary space. Using the method of Dolei et al., It is estimated that the density in each bright finger is greater than the corresponding dark region lying below it in the surrounding intermediate corona. The instability is evolved due to wave perturbations that are parallel to the magnetic field at the density interface. We conjecture that the prominence plasma is supported by tension component of the magnetic field against gravity. Through the use of linear stability theory, the magnetic field is estimated as 21–40 mG to suppress growth of MRT instability in the observed finger structures. In the southward plasma segment, a horn-like structure is observed at 11:55 UT in the intermediate corona that also indicates MRT instability. Falling blobs are also observed in both of the plasma segments. In the outer corona, up to 6–13 solar radii, the mushroom-like plasma structures have been identified in the upper-northward MRT unstable plasma segment using STEREO-A/COR-2. These structures most likely grew due to the breaking and twisting of fingers at large spatial scales in weaker magnetic fields. In the lower interplanetary space up to 20 solar radii, these structures are fragmented into various small-scale localized plasma spikes, most likely due to turbulent mixing.
Ultraviolet Detection of the Binary Companion to the Type IIb SN 2001ig
Authors: Ryder Stuart D. , Dyk Schuyler D. Van , Fox Ori D. , Zapartas Emmanouil , Mink Selma E. de , Smith Nathan , Brunsden Emily , Bostroem K. Azalee , Filippenko Alexei V. , Shivvers Isaac , Zheng WeiKang 
Published: March 2018

Abstract
We present HST/WFC3 ultraviolet imaging in the F275W and F336W bands of the Type IIb SN 2001ig at an age of more than 14 years. A clear point source is detected at the site of the explosion, with m
F275W = 25.39 ± 0.10 and m
F336W = 25.88 ± 0.13 mag. Despite weak constraints on both the distance to the host galaxy NGC 7424 and the line-of-sight reddening to the supernova, this source matches the characteristics of an early B-type main-sequence star with 19,000 < T
eff < 22,000 K and

. A BPASS v2.1 binary evolution model, with primary and secondary masses of 13 M
⊙ and 9 M
⊙, respectively, is found to simultaneously resemble, in the Hertzsprung–Russell diagram, both the observed location of this surviving companion, and the primary star evolutionary endpoints for other Type IIb supernovae. This same model exhibits highly variable late-stage mass loss, as expected from the behavior of the radio light curves. A Gemini/GMOS optical spectrum at an age of 6 years reveals a narrow He ii
λ4686 emission line, indicative of continuing interaction with a dense circumstellar medium at large radii from the progenitor. We review our findings on SN 2001ig in the context of binary evolution channels for stripped-envelope supernovae. Owing to the uncrowded nature of its environment in the ultraviolet, this study of SN 2001ig represents one of the cleanest detections to date of a surviving binary companion to a Type IIb supernova.
Determining the Nature of White Dwarfs from Low-frequency Gravitational Waves
Authors: Han Wen-Biao , Fan Xi-Long 
Published: March 2018

Abstract
An extreme-mass-ratio system composed of a white dwarf (WD) and a massive black hole can be observed by low-frequency gravitational wave detectors, such as the Laser Interferometer Space Antenna (LISA). When the mass of the black hole is around 104 ∼ 105
M
⊙, the WD will be disrupted by the tidal interaction at the final inspiraling stage. The event position and time of the tidal disruption of the WD can be accurately determined by the gravitational wave signals. Such position and time depend upon the mass of the black hole and especially on the density of the WD. We present the theory that by using LISA-like gravitational wave detectors, the mass–radius relation and the equations of state of WDs could be strictly constrained (accuracy up to 0.1%). We also point out that LISA can accurately predict the disruption time of a WD and forecast the electromagnetic follow-up of this tidal disruption event.
The [O iii] Profiles of Infrared-selected Active Galactic Nuclei: More Powerful Outflows in the Obscured Population
Authors: DiPompeo M. A. , Hickox R. C. , Carroll C. M. , Runnoe J. C. , Mullaney J. R. , Fischer T. C. 
Published: March 2018

Abstract
We explore the kinematics of ionized gas via the [O iii] λ5007 emission lines in active galactic nuclei (AGNs) selected on the basis of their mid-infrared (IR) emission, and split into obscured and unobscured populations based on their optical−IR colors. After correcting for differences in redshift distributions, we provide composite spectra of spectroscopically and photometrically defined obscured/Type 2 and unobscured/Type 1 AGNs from 3500 to 7000 Å. The IR-selected obscured sources contain a mixture of narrow-lined Type 2 AGNs and intermediate sources that have broad Hα emission and significantly narrower Hβ. Using both [O iii] luminosities and AGN luminosities derived from optical−IR spectral energy distribution fitting, we find evidence for enhanced large-scale obscuration in the obscured sources. In matched bins of luminosity we find that the obscured population typically has broader, more blueshifted [O iii] emission than in the unobscured sample, suggestive of more powerful AGN-driven outflows. This trend is not seen in spectroscopically classified samples, and is unlikely to be entirely explained by orientation effects. In addition, outflow velocities increase from small to moderate AGN E(B − V) values, before flattening out (as traced by FWHM) and even decreasing (as traced by blueshift). While difficult to fully interpret in a single physical model, due to both the averaging over populations and the spatially averaged spectra, these results agree with previous findings that simple geometric unification models are insufficient for the IR-selected AGN population, and may fit into an evolutionary model for obscured and unobscured AGNs.
Sun-to-Earth MHD Simulation of the 2000 July 14 “Bastille Day” Eruption
Authors: Török Tibor , Downs Cooper , Linker Jon A. , Lionello R. , Titov Viacheslav S. , Mikić Zoran , Riley Pete , Caplan Ronald M. , Wijaya Janvier 
Published: March 2018

Abstract
Solar eruptions are the main driver of space-weather disturbances at Earth. Extreme events are of particular interest, not only because of the scientific challenges they pose, but also because of their possible societal consequences. Here we present a magnetohydrodynamic (MHD) simulation of the 2000 July 14 “Bastille Day” eruption, which produced a very strong geomagnetic storm. After constructing a “thermodynamic” MHD model of the corona and solar wind, we insert a magnetically stable flux rope along the polarity inversion line of the eruption’s source region and initiate the eruption by boundary flows. More than 1033 erg of magnetic energy is released in the eruption within a few minutes, driving a flare, an extreme-ultraviolet wave, and a coronal mass ejection (CME) that travels in the outer corona at ≈1500 km s−1, close to the observed speed. We then propagate the CME to Earth, using a heliospheric MHD code. Our simulation thus provides the opportunity to test how well in situ observations of extreme events are matched if the eruption is initiated from a stable magnetic equilibrium state. We find that the flux-rope center is very similar in character to the observed magnetic cloud, but arrives ≈8.5 hr later and ≈15° too far to the north, with field strengths that are too weak by a factor of ≈1.6. The front of the flux rope is highly distorted, exhibiting localized magnetic field concentrations as it passes 1 au. We discuss these properties with regard to the development of space-weather predictions based on MHD simulations of solar eruptions.
Quelle place pour les « économies populaires » dans le « développement réel » du XXI e siècle ? Une question ouverte en lieu de postface
Authors: Peemans Jean-Philippe 
Published: March 2018

Ayant rappelé comment l’évolution de la conceptualisation des « réalités économiques informelles ou populaires » a participé aux débats qui ont traversé les discours sur le développement, cette postface montre comment ces changements ont contribué à changer, ou non, les « réalités populaires » et tente de vérifier si la violence au cœur du « développement réel » du début du XXI e siècle n’a pas contribué à éroder, ou à dissoudre, ce qui faisait l’originalité des « secteurs populaires ». Enfin, on pose la question des capacités de résilience des « acteurs populaires » dans ce contexte de changements rapides.
La promotion de l’allaitement maternel chez les femmes en situation de précarité en Haute-Normandie
Authors: Dornier Aurélie 
Published: March 2018

Introduction : En France, la prévalence de l’allaitement maternel chez les femmes en situation de précarité est inférieure au reste de la population. La Haute-Normandie est une région fortement touchée par la précarité, en particulier chez les femmes. Objectif principal : Déterminer l’offre en matière de promotion de l’allaitement maternel à destination des femmes en situation de précarité en Haute-Normandie. Objectifs secondaires : Décrire la prévalence de l’allaitement maternel et identifier les freins à l’allaitement maternel dans cette population. Méthodes : Cette étude observationnelle transversale a été menée sur cette région de mars à juin 2015, en trois volets : 1) envoi de questionnaires aux acteurs accompagnant les femmes en situation de précarité ; 2) analyse des certificats de santé obligatoires chez l’enfant et 3) entretiens sociologiques chez 15 femmes en situation de précarité. Résultats : Parmi les 29 structures ayant répondu favorablement, seules quatre avaient mis en place des actions d’accompagnement à l’allaitement maternel auprès des femmes en situation de précarité, avec des retours positifs. À peine la moitié des femmes en situation de précarité avait initié un allaitement maternel en Haute-Normandie (Eure = 50 %, Seine-Maritime = 54 %). Enfin, les facteurs habituellement connus pour influencer l’allaitement et sa durée ont été retrouvés chez les femmes en situation de précarité. Conclusion : Des pistes d’actions de soutien de l’allaitement maternel auprès des mères en situation de précarité ont pu être élaborées, orientant ainsi les politiques locales en matière de diminution des inégalités sociales de santé sur le territoire de santé de Haute-Normandie.
Les ordonnances collectives et la qualité des soins au triage des urgences : synthèse des connaissances
Authors: Lacroix Marie-Claude , Borgès Da Silva Roxane 
Published: March 2018

Introduction : Le débordement des départements d’urgence est un problème général dans les pays occidentaux. Il engendre des résultats négatifs chez les patients et influence la qualité des soins. Les ordonnances collectives sont une piste envisageable pour améliorer la qualité des soins et donc la performance du système de santé. Le but de l’article est de documenter, par une revue intégrative, les effets sur la qualité des soins des ordonnances collectives initiées par les infirmières au triage dans les départements d’urgence. Méthodes : Les dimensions de la qualité des soins selon l’Institute of Medicine (2001) ont été utilisées comme cadre de référence. Une revue intégrative a été effectuée par le repérage d’articles à partir de Cochrane , CINALH , EMBASE , Medline , PubMed et Google Scholar . Au total, 23 articles ont été retenus et analysés. Résultats : La revue intégrative a permis de documenter les effets des ordonnances collectives initiées au triage par les infirmières sur les six dimensions de la qualité des soins : l’efficience, les soins axés sur le patient, l’efficacité, les soins opportuns, la sécurité et l’équité. Les ordonnances collectives présentent un potentiel pour améliorer l’efficience des soins offerts en diminuant, entre autres, le délai avant les traitements et les examens diagnostiques et la durée de séjour des patients aux urgences. Conclusion : Les ordonnances collectives initiées par les infirmières au triage dans les départements d’urgence peuvent avoir des effets positifs sur la qualité des soins prodigués au patient. D’autres recherches avec des devis de recherche plus robustes sont nécessaires.
Facteurs associés à la force de la culture organisationnelle dans un hôpital du Bénin
Authors: Sopoh Ghislain Emmanuel , Kouckodila Nzingoula Michael Florian , Sossa Jérôme Charles , Hessou Ahahanzo-Glèlè Yolaine , Damienne Agueh Victoire , Tinoaga Ouédraogo Laurent , Makoutodé Michel 
Published: March 2018

Introduction : La culture organisationnelle, concept souvent ignoré, influe sur la satisfaction au travail et la productivité des organisations. Objectif : Déterminer les facteurs associés à la force de la culture organisationnelle (CO) au Centre Hospitalier Départemental (CHD) Mono/Couffo à Lokossa au Bénin. Méthodes : Il s’agit d’une étude transversale descriptive et analytique qui a porté sur 121 travailleurs du CHD Mono/Couffo de Lokossa en mars 2015. La collecte des données sur la force de la CO a été réalisée à l’aide d’un questionnaire élaboré selon l’outil de Cameron et Quinn (2006). La nature des relations entre les variables indépendantes et la culture organisationnelle a été explorée par l’estimation des Odds ratios avec la régression logistique. Résultats : Parmi les sujets enquêtés, 62 % avaient une perception positive de la CO. Chez les sujets qui estimaient ne pas avoir le soutien moral de la hiérarchie, le risque de percevoir une force de la CO faible était six fois plus important [OR = 3,78, 95 % IC (1,08 – 13,22)] que chez ceux qui percevaient ce soutien moral. Chez les sujets qui estimaient que les relations avec l’équipe dirigeante n’étaient pas cordiales, le risque de percevoir une force de CO faible était plus élevé [OR = 14,32, 95 % IC (4,35 – 47,11)] par rapport à ceux qui qualifiaient ces relations de cordiales. Conclusion : Les facteurs managériaux et le type de relation avec l’équipe dirigeante étaient associés à la force de la CO. Les dirigeants des hôpitaux devraient accorder plus d’attention à ces facteurs afin d’améliorer la performance institutionnelle.