33 research outputs found

    Recommandations pédagogiques pour pallier le manque de sommeil associé aux stages de soir chez les étudiantes en soins infirmiers

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    Comprend des références bibliographiques.Le rapport de recherche PAREA PA-2015-018 "Effets des stages en soirée sur l'apprentissage d'étudiantes en soins infirmiers et recommandations pédagogiques" est également disponible sur support électronique dans EDUQ.info et sur support papier au CD

    Effets des stages en soirée sur l'apprentissage d'étudiantes en soins infirmiers et recommandations pédagogiques

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    La présente recherche a été subventionnée par le ministère de l'Éducation et de l'Enseignement supérieur dans le cadre du Programme d'aide à la recherche sur l'enseignement et l'apprentissage (PAREA).Comprend des références bibliographiques

    The effects of a 12-week strength training program on skeletal muscle impairments and physical limitations in men with myotonic dystrophy type 1

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    Background/rationale: Myotonic dystrophy type 1 (DM1) is a genetic multisystemic degenerative disease and represents the most prevalent myopathy in adults. Skeletal muscles are particularly affected, as demonstrated by muscle weakness and atrophy experienced by affected people, which limit their social participation. Purpose/research objectives: The aim of this project is to determine the effects of a 12-week strength training program on skeletal muscle impairments and physical limitations in men with DM1. Relevance: Strength training has been shown to be safe in this population, but it remains unknown if it can trigger muscle hypertrophy process, thus slowing or reversing the significant muscle impairments that characterize this disease. Methods: In this before-after study, a 12-week strength training program (twice a week) of 6 to 8 maximal repetitions (RM) of five different lower limb exercises was completed by 11 men with DM1. The evaluation included: 10 meter walk test (comfortable and maximal speed), 30 second sit-to-stand test, quantitative muscle strength assessment of knee extensors muscle group, 1-RM test for all exercises and an interview about perceived changes. Results: Results showed significant maximal muscle strength increases as well as improvement in all functional tests (p<0.05). Patients also reported many positive changes after the training program such as an improved confidence in their legs and that they had ceased falling. Conclusion: Many positive changes have resulted from this training program showing that a well standardized strength training is an efficient and promising treatment option to reduce skeletal muscle impairments and physical limitations in people with DM1

    Becoming a research participant : decision-making needs of individuals with neuromuscular diseases

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    BACKGROUND: Research has shown that some people with neuromuscular diseases may have a lower level of education due to lower socioeconomic status and possibly compromised health literacy. In view of these data, it appears important to document their decision-making needs to ensure better support when faced with the decision to participate or not in research projects. OBJECTIVES: 1) To document the decision-making needs of individuals with neuromuscular diseases to participate in research; 2) To explore their preferences regarding the format of knowledge translation tools related to research participation. METHODS: This qualitative study is based on the Ottawa Decision Support Framework. A two-step descriptive study was conducted to capture the decision-making needs of people with neuromuscular diseases related to research participation: 1) Individual semi-directed interviews (with people with neuromuscular diseases) and focus groups (with healthcare professionals); 2) Synthesis of the literature. RESULTS: The semi-directed interviews (n = 11), the two focus groups (n = 11) and the literature synthesis (n = 50 articles) identified information needs such as learning about ongoing research projects, scientific advances and research results, the potential benefits and risks associated with different types of research projects, and identified values surrounding research participation: helping other generations, trust, obtaining better clinical follow-up, and socialization. CONCLUSION: This paper provides useful recommendations to support researchers and clinicians in developing material to inform individuals with neuromuscular diseases about research participation

    Patient-reported outcome measures in neuromuscular diseases : a scoping review

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    Patient-reported outcome measures (PROMs) are valuable in comprehensively understanding patients' health experiences and informing healthcare decisions in research and clinical care without clinicians' input. Until now, no central resource containing information on all PROMS in neuromuscular diseases (NMD) is available, hindering the comparison and choice of PROMs used to monitor NMDs and appropriately reflect the patient’s voice. This scoping review aimed to present a comprehensive assessment of the existing literature on using PROMs in children and adults with NMD. A scoping methodology was followed using Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) and COnsensus-based Standards for the selection of health Measurement Instruments (COSMIN) guidelines to assess the literature on PROMs in NMDs. Eligibility criteria encompassed articles describing psychometric development or evaluation of generic or disease-specific PROM-based instruments for adults and children with specific NMDs. The data charting process involved extracting measurement properties of included PROMs, comprising validity, reliability, responsiveness, and interpretability information. The review identified 190 PROMs evaluated across 247 studies in individuals with NMDs. The majority of PROMs were disease specific. The physical functioning domain was most assessed. Validity was the most frequently investigated measurement property, with a limited number of PROMs sufficiently evaluated for a range of psychometric characteristics. There is a strong need for further research on the responsiveness and interpretability of PROMs and the development of PROMs on social functioning in NMD

    A STEP fault in Central Betics, associated with lateral lithospheric tearing at the northern edge of the Gibraltar arc subduction system

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    We study the crustal and lithospheric mantle structure under central Betics in the westernmost Mediterranean region by migrating P-receiver functions along a dense seismic profile (∼2 km interstation distance). The profile, North–South oriented, probes the crustal structure of different geological units, from the Alboran domain in the south with metamorphic rocks, through the External Zones with sedimentary rocks to the Variscan terrains of the Iberian Massif in the north. From north to south, the Moho depth increases from ∼30 km to ∼46 km underneath the Guadix basin, due to the underthrusting of the Iberian crust below the Alboran crust, and suddenly shallows to ∼30 km underneath the Internal Zones with a step of 17 km. This sharp Moho step correlates well with a lithospheric step of ∼40 km, where the thickness of the lithosphere changes abruptly from ∼100 km in the north to ∼50 km in the south. We interpret this sharp and prominent lithospheric step as the termination of the Iberian lithosphere caused by a near-vertical STEP (Subduction-Transform-Edge-Propagator) fault that continues towards the surface as a positive flower tectonic structure of crustal scale. This STEP fault is located at the northern edge of the narrow Westernmost Mediterranean subduction system facilitating the slab rollback motion towards the west. The sharp termination of the Iberian lithosphere occurs under the contact between the Alpujarride and the Nevado-Filabride complexes of the Alboran domain in an ENE-WSW right-lateral transpressive shear zone. The thickest crust and lithosphere do not correlate with the highest topography along the profile suggesting that this high topography is a combined effect of the positive flower structure, and the push up of the asthenosphere produced by the removal of the Iberian lithosphere.This work was sup-ported by the projects: CGL2015-67130-C2-2-R, GCL2012-31472 (TRANSCORBE), HIRE (GFZ Potsdam) and PP2012-PIJD003 (Granada University). We acknowledge work on free softwares SAC and GMT

    Consensus-based care recommendations for adults with myotonic dystrophy type 1

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    Purpose of review Myotonic dystrophy type 1 (DM1) is a severe, progressive genetic disease that affects between 1 in 3,000 and 8,000 individuals globally. No evidence-based guideline exists to inform the care of these patients, and most do not have access to multidisciplinary care centers staffed by experienced professionals, creating a clinical care deficit. Recent findings The Myotonic Dystrophy Foundation (MDF) recruited 66 international clinicians experienced in DM1 patient care to develop consensus-based care recommendations. MDF created a 2-step methodology for the project using elements of the Single Text Procedure and the Nominal Group Technique. The process generated a 4-page Quick Reference Guide and a comprehensive, 55-page document that provides clinical care recommendations for 19 discrete body systems and/or care considerations. Summary The resulting recommendations are intended to help standardize and elevate care for this patient population and reduce variability in clinical trial and study environments. Described as “one of the more variable diseases found in medicine,” myotonic dystrophy type 1 (DM1) is an autosomal dominant, triplet-repeat expansion disorder that affects somewhere between 1:3,000 and 1:8,000 individuals worldwide.1 There is a modest association between increased repeat expansion and disease severity, as evidenced by the average age of onset and overall morbidity of the condition. An expansion of over 35 repeats typically indicates an unstable and expanding mutation. An expansion of 50 repeats or higher is consistent with a diagnosis of DM1. DM1 is a multisystem and heterogeneous disease characterized by distal weakness, atrophy, and myotonia, as well as symptoms in the heart, brain, gastrointestinal tract, endocrine, and respiratory systems. Symptoms may occur at any age. The severity of the condition varies widely among affected individuals, even among members of the same family. Comprehensive evidence-based guidelines do not currently exist to guide the treatment of DM1 patients. As a result, the international patient community reports varied levels of care and care quality, and difficulty accessing care adequate to manage their symptoms, unless they have access to multidisciplinary neuromuscular clinics. Consensus-based care recommendations can help standardize and improve the quality of care received by DM1 patients and assist clinicians who may not be familiar with the significant variability, range of symptoms, and severity of the disease. Care recommendations can also improve the landscape for clinical trial success by eliminating some of the inconsistencies in patient care to allow more accurate understanding of the benefit of potential therapies

    IMPACT DES FACTEURS GÉNÉTIQUES, FONCTIONNELS, PSYCHOPATHOLOGIQUES ET NEUROPSYCHOLOGIQUES DANS L'ADAPTATION A LA DYSTROPHIE MYOTONIQUE DE STEINERT

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    Background: adaptation to a chronically and progressive disease is such a complex phenomena, which require a multidimensional approach. We believe that Steinert myotonic dystrophy (DM1), as a pathology involving physiological and neurological modifications as well as social deprivation, represents a suitable model for testing an integrative approach of disease adjustment. Method: we have assessed the role and the impact of genetic, clinical, cognitive, emotional, affective and social factors on quality of life, in patients with MD1 (41), facioscapulo-humeral dystrophy (FSHD: 19) and 20 population controls. Results: DM1 patients had more frequent and more intense apathy than FSHD patients and controls. About 22% of DM1 patients met current major depressive disorder criteria. As a group, DM1 patients had a mild to moderate depression. The depression severity was significantly correlated to functional impairment, emotion-focused coping strategies, but was independent from CTG repeats and neuropsychological assessment. Thus, depression, in DM1, seems to be a “reactional” process. Within the DM1 group, patients with, or intellectual or executive functions pathological scores, used significantly more emotion-focused coping strategies. These results emphasize the link between emotion and cognition into the adaptive process. Conclusion: our results highlight the importance of working on coping strategies, among stress management, in neuromuscular disease. Besides, it is relevant that family helpers and professionals must take a special care on disease evolution critical periods.L'adaptation à la maladie chronique et évolutive est un phénomène complexe mettant en jeu des facteurs physiologiques, psychologiques et sociaux. L'évaluation d'un tel processus chez un sujet, ou au sein d'une population donnée, requiert une approche intégrative. Nous pensons que la dystrophie myotonique de Steinert (DM1) constitue un modèle pertinent pour aborder l'approche biopsychosociale de l'ajustement à l'adversité, car cette pathologie neuromusculaire d'origine génétique a des conséquences sur ces trois dimensions. Sur la base des données de la littérature, nous avons posé l'hypothèse que le trouble de l'humeur dépressive, dans la DM1, était un phénomène réactionnel à la maladie progressivement invalidante, en raison de l'utilisation de stratégies de « coping » inopérantes. De plus, nous avons postulé que les sujets atteints de DM1 présenteraient une altération significative, sur le plan intellectuel et au niveau des fonctions exécutives, que des sujets atteints d'une autre maladie neuromusculaire sans atteinte connue du système nerveux central (la myopathie facio-scapulo-humérale, FSH), et que des sujets contrôles issus de la population générale. Enfin, considérant l'impact des troubles thymiques et des altérations cognitives sur les capacités d'adaptation des sujets DM1, nous avons fait l'hypothèse que les sujets DM1 estimeraient que leur qualité de vie est diminuée, autant sur la sphère physique que la sphère psychologique. Nous avons donc analysé le rôle des facteurs génétiques, cliniques, cognitifs, émotionnels, affectifs et sociaux, et leur influence sur la qualité de vie, auprès de 41 sujets atteints de dystrophie myotonique de Steinert, et de 19 sujets atteints de myopathie facio-scapulo-humérale. Nous avons comparé ces évaluations avec les résultats d'un groupe contrôle, constitué de 20 sujets sans maladie chronique, issu de la population générale. Nos résultats mettent en avant, d'une part, que les sujets DM1 ont, en moyenne, un niveau intellectuel global qui se situe dans la norme inférieure, et qui est significativement inférieur à celui de la population générale et du groupe témoin FSH ; ainsi que des altérations spécifiques des capacités d'attention/concentration. De plus, les sujets DM1 adultes présentent une apathie plus fréquente et plus intense que les deux autres groupes. Nos résultats permettent de faire l'hypothèse que l'apathie, caractéristique spécifique des sujets DM1, serait d'origine organique dans cette pathologie. Il s'agit d'une piste de réflexion qui mérite d'être approfondie dans de prochains travaux, notamment en y associant l'utilisation de techniques modernes d'imagerie. D'autre part, nous observons, dans la DM1, que la dépression est un symptôme fréquent. Près de 22% des sujets DM1 de notre étude remplissent les critères d'un épisode dépressif majeur actuel. L'humeur y est le plus souvent modifiée, et certains sujets présentent même des idées suicidaires. Néanmoins, l'intensité de cette symptomatologie est, si nous considérons l'ensemble du groupe DM1, légère à modérée. On retrouve des modifications émotionnelles de type « émoussement affectif » et « dyscontrôle et hyper-émotionnalité ». L'intensité dépressive est corrélée à l'augmentation du handicap fonctionnel. Elle est indépendante, statistiquement, des facteurs génétiques et neuropsychologiques. Enfin, elle est associée à l'utilisation de stratégies de coping « centrées sur l'émotion ». Ce type de coping est connu, par ailleurs, pour être peu opérant dans l'adaptation aux maladies chroniques. Inversement, le coping « centré sur le problème » est corrélé à une meilleure estime de soi et à des degrés moindres de dépression. Ces résultats sont en faveur de l'hypothèse que les troubles thymiques sont, dans la DM1, la conséquence secondaire d'une affection chronique incurable et évolutive, en raison de l'utilisation de stratégies d'adaptation non opérantes. Dans la DM1, les troubles anxieux et dépressifs, la diminution de l'estime de soi et l'augmentation de la fatigue subjective sont des facteurs inter-corrélés et associés à une moins bonne adaptation sociale et une moins bonne qualité de vie dans les domaines physique et psychologique. Enfin, au-delà de l'opposition classique entre facteurs primaires et secondaires, ou celle d'une origine neurologique versus origine psychique, nous observons, à travers nos résultats, les interactions entre émotions, cognition et adaptation au stress causé par une maladie évolutive. En effet, alors que les sujets atteints de FSH modifient leur utilisation du coping, au fur et à mesure que la maladie évolue, les sujets DM1, eux, utilisent toujours les mêmes types de stratégies, quelle que soit l'étape de leur maladie. Au sein de ce groupe de sujets atteints de DM1, ce sont les personnes qui présentent une altération cognitive significative (intellectuelle et exécutive) qui utilisent préférentiellement un coping de type émotionnel, associé à des perturbations thymiques. Ainsi, nous pensons que lorsque les substrats cognitifs, qui sous-tendent les capacités d'appréciation et de flexibilité des ressources nécessaires à un ajustement opérant sont altérés, les sujets présentent une plus grande vulnérabilité au développement de troubles affectifs. Les résultats de notre étude soulignent l'importance d'un travail de remédiation sur les stratégies de coping et les modes de réactivité au stress, selon les déterminants propres au sujet (comme par exemple l'anxiété-trait). Ils témoignent aussi de la nécessité d'être attentif, dans la prise en charge des patients DM1, aux périodes critiques d'évolution de la maladie. Il est important, aussi bien pour les aidants et les familles, que pour les cliniciens, d'apporter, dans ces périodes, d'autant plus de présence, d'écoute et d'attention
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