Muscle activation during gait in children with Duchenne muscular dystrophy

The aim of this prospective study was to investigate changes in muscle activity during gait in children with Duchenne muscular Dystrophy (DMD). Dynamic surface electromyography recordings (EMGs) of 16 children with DMD and pathological gait were compared with those of 15 control children. The activity of the rectus femoris (RF), vastus lateralis (VL), medial hamstrings (HS), tibialis anterior (TA) and gastrocnemius soleus (GAS) muscles was recorded and analysed quantitatively and qualitatively. The overall muscle activity in the children with DMD was significantly different from that of the control group. Percentage activation amplitudes of RF, HS and TA were greater throughout the gait cycle in the children with DMD and the timing of GAS activity differed from the control children. Significantly greater muscle coactivation was found in the children with DMD. There were no significant differences between sides. Since the motor command is normal in DMD, the hyper-activity and co-contractions likely compensate for gait instability and muscle weakness, however may have negative consequences on the muscles and may increase the energy cost of gait. Simple rehabilitative strategies such as targeted physical therapies may improve stability and thus the pattern of muscle activity

Effect of Hydrocortisone on Mortality and Organ Support in Patients With Severe COVID-19: The REMAP-CAP COVID-19 Corticosteroid Domain Randomized Clinical Trial.

Importance: Evidence regarding corticosteroid use for severe coronavirus disease 2019 (COVID-19) is limited. Objective: To determine whether hydrocortisone improves outcome for patients with severe COVID-19. Design, Setting, and Participants: An ongoing adaptive platform trial testing multiple interventions within multiple therapeutic domains, for example, antiviral agents, corticosteroids, or immunoglobulin. Between March 9 and June 17, 2020, 614 adult patients with suspected or confirmed COVID-19 were enrolled and randomized within at least 1 domain following admission to an intensive care unit (ICU) for respiratory or cardiovascular organ support at 121 sites in 8 countries. Of these, 403 were randomized to open-label interventions within the corticosteroid domain. The domain was halted after results from another trial were released. Follow-up ended August 12, 2020. Interventions: The corticosteroid domain randomized participants to a fixed 7-day course of intravenous hydrocortisone (50 mg or 100 mg every 6 hours) (n = 143), a shock-dependent course (50 mg every 6 hours when shock was clinically evident) (n = 152), or no hydrocortisone (n = 108). Main Outcomes and Measures: The primary end point was organ support-free days (days alive and free of ICU-based respiratory or cardiovascular support) within 21 days, where patients who died were assigned -1 day. The primary analysis was a bayesian cumulative logistic model that included all patients enrolled with severe COVID-19, adjusting for age, sex, site, region, time, assignment to interventions within other domains, and domain and intervention eligibility. Superiority was defined as the posterior probability of an odds ratio greater than 1 (threshold for trial conclusion of superiority >99%). Results: After excluding 19 participants who withdrew consent, there were 384 patients (mean age, 60 years; 29% female) randomized to the fixed-dose (n = 137), shock-dependent (n = 146), and no (n = 101) hydrocortisone groups; 379 (99%) completed the study and were included in the analysis. The mean age for the 3 groups ranged between 59.5 and 60.4 years; most patients were male (range, 70.6%-71.5%); mean body mass index ranged between 29.7 and 30.9; and patients receiving mechanical ventilation ranged between 50.0% and 63.5%. For the fixed-dose, shock-dependent, and no hydrocortisone groups, respectively, the median organ support-free days were 0 (IQR, -1 to 15), 0 (IQR, -1 to 13), and 0 (-1 to 11) days (composed of 30%, 26%, and 33% mortality rates and 11.5, 9.5, and 6 median organ support-free days among survivors). The median adjusted odds ratio and bayesian probability of superiority were 1.43 (95% credible interval, 0.91-2.27) and 93% for fixed-dose hydrocortisone, respectively, and were 1.22 (95% credible interval, 0.76-1.94) and 80% for shock-dependent hydrocortisone compared with no hydrocortisone. Serious adverse events were reported in 4 (3%), 5 (3%), and 1 (1%) patients in the fixed-dose, shock-dependent, and no hydrocortisone groups, respectively. Conclusions and Relevance: Among patients with severe COVID-19, treatment with a 7-day fixed-dose course of hydrocortisone or shock-dependent dosing of hydrocortisone, compared with no hydrocortisone, resulted in 93% and 80% probabilities of superiority with regard to the odds of improvement in organ support-free days within 21 days. However, the trial was stopped early and no treatment strategy met prespecified criteria for statistical superiority, precluding definitive conclusions. Trial Registration: ClinicalTrials.gov Identifier: NCT02735707

Les effets de l’invitation européenne à agir sur la conciliation emploi-famille: une analyse comparée de la négociation collectiveen France et au Luxembourg

International audienceL’article s’interroge sur l’effectivité de l’invitation faite par les instances européennes aux États membres et aux partenaires sociaux d’agir en faveur d’une meilleureconciliation de la vie professionnelle et de la vie familiale, notamment à travers le dialogue social. Il étudie plus précisément la réponse des partenaires sociaux dansle cadre de deux pays, la France et le Luxembourg, présentant une convention de genre et un système de relations professionnelles bien différenciés. Après avoir explicitéla nature de l’invitation européenne et ses déclinaisons institutionnelles en France et au Luxembourg, l’article procède à une analyse exploratoire du produit dela négociation collective aux niveaux de la branche et de l’entreprise sur la base d’un échantillon comparable en France et au Luxembourg. La question qui est posée estplus précisément d’apprécier sous quelle forme et avec quel contenu la thématique de la conciliation emploi-famille a été négociée dans cet échantillon restreint, pourmieux en saisir la portée, en rapportant ces résultats à la diversité des dispositifs institutionnels existants

Architectural reorganization of the nuclei upon transfer into oocytes accompanies genome reprogramming

International audienceThe ability of cloned embryos to sustain full-term development depends on the ability of the recipient ooplasm to reprogram the donor cell genome. As the nuclear architecture has recently emerged as a key-factor in the regulation of gene expression, we questioned whether early embryos obtained from transfer of ES metaphasic chromosomes into mouse ooplasm would adopt the somatic or embryonic type of nuclear organization. We have particularly focused on the arrangement of chromosomal territories with respect to the nucleolar compartment, and the pericentric heterochromatin domains called chromocenters. We found that nuclear transfer triggers profound chromatin rearrangements including the dispersion of the donor cell chromocenters components. These rearrangements lead to a typical 1-cell pronuclear organization, namely a radial arrangement of the chromosome territories with centromeres attached to the nucleoli, which adopt the compact fibrillar structure of nucleolar precursor bodies (NPBs). Subsequently, during the second cycle, the cloned embryos undergo further reorganization with the establishment of new chromocenters, clustered in one part of the nucleus, as during normal embryogenesis. We could also establish that the adequate distribution of chromosomal territories at the pronuclear stage seems important for the development until blastocyst

Outils et leviers pour favoriser le développement d'une génétique adaptée aux enjeux de l'agro-écologie pour les élevages de ruminants

National audienceDans le cadre du Projet Agro-Ecologique pour la France, la présente étude a été conduite afin de déterminer les « Outils et leviers pour favoriser le développement d’une génétique animale adaptée aux enjeux de l’agro-écologie (AE) ». Après une étude bibliographique portant sur les critères de performance au regard des enjeux de l’AE, et un état des lieux des évolutions passées ou en cours de l’offre génétique, une enquête téléphonique auprès de 37 éleveurs et un groupe d’éleveurs, en ruminants et engagés dans une démarche AE sur leur exploitation, a été réalisée afin de recueillir leur vision de la génétique, leurs attentes et besoins, et leurs propositions pour l’avenir. Une synthèse de ces éléments a conduit à des recommandations. Les dispositifs de sélection actuels apportent déjà des réponses aux éleveurs de ruminants engagés dans des démarches AE (3/4 des enquêtés considèrent que l’offre génétique actuelle leur permet une conduite d’élevage en adéquation avec les principes de l’AE), grâce à la diversité génétique des reproducteurs disponibles, et à l’évolution des objectifs de sélection. Néanmoins ces dispositifs doivent évoluer significativement en intégrant : 1) une orientation vers plus de robustesse, santé et efficacité alimentaire, en encourageant une approche multicritères ; 2) une meilleure connaissance et une prise en compte de l’adaptation des génotypes à différents milieux d’élevage, dans les différentes races et croisements, 3) un certain degré de « personnalisation » du tri des reproducteurs afin de proposer le meilleur compromis entre conditions d’élevage – animaux – objectifs de l’éleveur et de sa filièr

From congenial paralysis to post-early brain injury developmental condition: Where does cerebral palsy actually stand?

Cerebral palsy (CP), an umbrella term for a developmental motor disorder caused by early brain injury (EBI)/interference, remains debated. In this essay, we present a narrative, beginning with the original anatomical-clinical description of the so-called paralysie congéniale (congenial paralysis) by the French psychiatrist Jean-Baptiste Cazauvieilh. We then discuss how the concept has evolved over the last 2 centuries. We aim to illustrate these ideas with the biopsychosocial model of health, especially in light of the current neuroscientific and sociological knowledge of human development. We endeavour to integrate 3 connected but distinct entities: (1) the EBI as a seminal turning point of the individual's story; (2) the clinical findings we call CP, when motor impairment and activity limitation related to post-EBI (or other early non-progressive brain interference) appears, and; (3) a post-EBI developmental condition that encompasses the overall consequences of an EBI. This framework should guide individual, familial and collective care discussions and research strategies beyond the scope of CP

Review: Towards the agroecological management of ruminants, pigs and poultry through the development of sustainable breeding programmes. II. Breeding strategies

Agroecology uses ecological processes and local resources rather than chemical inputs to develop productive and resilient livestock and crop production systems. In this context, breeding innovations are necessary to obtain animals that are both productive and adapted to a broad range of local contexts and diversity of systems. Breeding strategies to promote agroecological systems are similar for different animal species. However, current practices differ regarding the breeding of ruminants, pigs and poultry. Ruminant breeding is still an open system where farmers continue to choose their own breeds and strategies. Conversely, pig and poultry breeding is more or less the exclusive domain of international breeding companies which supply farmers with hybrid animals. Innovations in breeding strategies must therefore be adapted to the different species. In developed countries, reorienting current breeding programmes seems to be more effective than developing programmes dedicated to agroecological systems that will struggle to be really effective because of the small size of the populations currently concerned by such systems. Particular attention needs to be paid to determining the respective usefulness of cross-breeding v. straight breeding strategies of welladapted local breeds. While cross-breeding may offer some immediate benefits in terms of improving certain traits that enable the animals to adapt well to local environmental conditions, it may be difficult to sustain these benefits in the longer term and could also induce an important loss of genetic diversity if the initial pure-bred populations are no longer produced. As well as supporting the value of within-breed diversity, we must preserve between-breed diversity in order to maintain numerous options for adaptation to a variety of production environments and contexts. This may involve specific public policies to maintain and characterize local breeds (in terms of both phenotypes and genotypes), which could be used more effectively if they benefited from the scientific and technical resources currently available for more common breeds. Last but not least, public policies need to enable improved information concerning the genetic resources and breeding tools available for the agroecological management of livestock production systems, and facilitate its assimilation by farmers and farm technicians

Review: Towards the agroecological management of ruminants, pigs and poultry through the development of sustainable breeding programmes: I-selection goals and criteria

Agroecology uses natural processes and local resources rather than chemical inputs to ensure production while limiting the environmental footprint of livestock and crop production systems. Selecting to achieve a maximization of target production criteria has long proved detrimental to fitness traits. However, since the 1990s, developments in animal breeding have also focussed on animal robustness by balancing production and functional traits within overall breeding goals. We discuss here how an agroecological perspective should further shift breeding goals towards functional traits rather than production traits. Breeding for robustness aims to promote individual adaptive capacities by considering diverse selection criteria which include reproduction, animal health and welfare, and adaptation to rough feed resources, a warm climate or fluctuating environmental conditions. It requires the consideration of genotype × environment interactions in the prediction of breeding values. Animal performance must be evaluated in low-input systems in order to select those animals that are adapted to limiting conditions, including feed and water availability, climate variations and diseases. Finally, we argue that there is no single agroecological animal type, but animals with a variety of profiles that can meet the expectations of agroecology. The standardization of both animals and breeding conditions indeed appears contradictory to the agroecological paradigm that calls for an adaptation of animals to local opportunities and constraints in weakly artificialized systems tied to their physical environment

Outils et leviers pour favoriser le développement d'une génétique animale adaptée aux enjeux de l'agroécologie: Rapport final de l’étude n° SSP-2014-061

Le néologisme « agro-écologie » est apparu dans la littérature dans les années 30, d’abord pour désigner une discipline scientifique au carrefour de l’écologie et de l’agronomie, puis à partir des années 70 un ensemble de pratiques et un mouvement social en réaction à l’intensification de l’agriculture (Wezel et al., 2009). Francis et al. (2003) ont encore élargi cette définition en considérant l’ensemble des dimensions environnementales et socio-économiques à l’échelle des filières. Dans son acception scientifique, qui est l’angle choisi dans cette étude, l’agro-écologie se définit comme l’application des concepts et principes de l’écologie à la conception et à la gestion d’agroécosystèmes durables. La question du bouclage des cycles pour limiter les intrants et les pollutions par une meilleure réutilisation des sorties des différents ateliers est au coeur de ces problématiques. On vise également un recours accru à des régulations biologiques et écologiques que l’on cherche à piloter au mieux des attendus de l’élevage. Ceci implique de prendre en compte la diversité biologique à tous ses niveaux d'organisation, de comprendre et d’utiliser les capacités adaptatives des animaux et les interactions biotiques dans les agroécosystèmes, en considérant les systèmes de culture ou les systèmes d'élevage comme des écosystèmes particuliers (Dumont et al. 2013). Ainsi, l’agro-écologie s’inscrit-elle dans un mouvement de fond autour du « Comment produire autrement ? » dans des systèmes de production dont on cherche à concilier les performances techniques, environnementales, économiques et sociales. Lancé le 18 décembre 2012, le Projet Agro-Ecologique pour la France (PAEF) vise à conjuguer l’ensemble de ces performances tant au niveau de l'exploitation qu’au niveau collectif (territoires, filières, etc.). Il s’agit d’engager une évolution des systèmes de production, qui remette l’agronomie au coeur des pratiques, pour combiner ces différentes performances. La Loi n° 2014-1170 du 13 octobre 2014 dite Loi d'avenir pour l'agriculture, l'alimentation et la forêt en donne la définition suivante : « Les politiques publiques visent à promouvoir et à pérenniser les systèmes de production agro-écologiques, dont le mode de production biologique, qui combinent performance économique, sociale, notamment au travers d’un haut niveau de protection sociale, environnementale et sanitaire. Ces systèmes privilégient l'autonomie des exploitations agricoles et l'amélioration de leur compétitivité, en maintenant ou en augmentant la rentabilité économique, en améliorant la valeur ajoutée des productions et en réduisant la consommation d'énergie, d'eau, d'engrais, de produits phytopharmaceutiques et de médicaments vétérinaires, en particulier les antibiotiques. Ils sont fondés sur les interactions biologiques et l'utilisation des services écosystémiques et des potentiels offerts par les ressources naturelles, en particulier les ressources en eau, la biodiversité, la photosynthèse, les sols et l'air, en maintenant leur capacité de renouvellement du point de vue qualitatif et quantitatif. Ils contribuent à l'atténuation et à l'adaptation aux effets du changement climatique