Evolution of coastal zone vulnerability to marine inundation in a global change context. Application to Languedoc Roussillon (France)

The coastal system is likely to suffer increasing costal risk in a global change context. Its management implies to consider those risks in a holistic approach of the different vulnerability components of the coastal zone, by improving knowledge of hazard and exposure as well as analyzing and quantifying present day and future territory vulnerability. The ANR/VMC2007/MISEEVA project (2008-2011) has applied this approach on Languedoc Roussillon region in France. MISEEVA approach relies on several scenarios for 2030 and 2100, in terms of meteorology (driver of coastal hazard), sea level rise, and also considering further trends in demography and economy, and possible adaption strategies Hazard has been modeled (SWAN, MARS and SURFWB), on the base of the presentday situation, sea level rise hypotheses, and existing or modeled data, of extreme meteorological driving f. It allowed to assess the possible surges ranges and map coastal zone exposure to: - a permanent inundation (considering sea level rise in 2030 and 2100, - a recurrent inundation (considering sea level rise and extreme tidal range) - an exceptional inundation (adding extreme storm surge to sea level rise and tidal range). In 2030, exposure will be comparable to present day exposure. In 2100, extreme condition will affect a larger zone. Present days social and economic components of the coastal zone have been analyzed in terms of vulnerability and potential damaging. Adaptation capacity was approached by public inquiries and interviews of stakeholders and policy makers, based on existing planning documents The knowledge of the present day system is then compared to the possible management strategies that could be chosen in the future, so to imagine what would be the evolution of vulnerability to marine inundation, in regards to these possible strategies

Traitement par sulfamides hypoglycémiants dans le diabète néonatal : étude de l’efficacité thérapeutique selon le génotype puis utilisation dans le diabète néonatal transitoire par anomalie du chromosome 6

Neonatal diabetes (ND) can be due to KCNJ11 or ABCC8 mutations (coding for K-ATP channels). Sulfonylurea (SU) can treat monogenic ND. Methods: we made a meta-analysis of articles describing patients treated with SU for a ND and articles studying in vitro SU-sensitivity of mutated channels to determine success rate of SU in ND. We reported 3 cases of patients treated with SU for a ND due to chromosome 6 abnormalities, to determine with a specific literature review, the success rate of SU in patients with ND due to chromosome 6 abnormalities. Results: 409/473 patients obtained a good glycemic control with SU treatment, including 329/373 KCNJ11 and 64/76 ABCC8 mutations carriers. 42 different KCNJ11 or ABCC8 mutations are coding for SU-sensitive mutated KATP-channels. 11/11 published patients and 2/3 of our reported cases had successful SU treatment of ND due to chromosome 6 abnormalities. Conclusion: SU are efficient treatment of ND due to KCNJ11, ABCC8 or chromosome 6 mutations. The availability of a pediatric dosage form would benefit the treatment of neonates and infants.Contexte : le diabète néonatal (DN) peut être dû à des mutations de canaux potassiques ATP-dépendants par mutation des gènes KCNJ11 ou ABCC8. Les sulfamides hypoglycémiants (SU) peuvent traiter le DN par stimulation de l’insulinosécrétion. Méthodes : nous avons conduit une méta-analyse des articles décrivant les patients traités par SU pour un DN monogénique ainsi que les analyses de sensibilité in vitro aux SU des canaux potassiques mutés, afin de déterminer le taux de succès thérapeutique des SU chez ces patients. En parallèle de la revue de la littérature, nous avons décrit 3 cas cliniques de patients atteints de DN par anomalie du chromosome 6 pour déterminer l’efficacité des SU chez les patients ayant un diabète néonatal par anomalie du chromosome 6. Résultats : 409/473 patients ont obtenu contrôle glycémique satisfaisant sous SU parmi lesquels 329/373 porteurs de mutations KCNJ11 et 64/76 porteurs de mutation ABCC8. 42 mutations différentes des gènes KCNJ11 et ABCC8 codent pour des canaux potassiques sensibles in vitro aux SU. 11/11 patients de la littérature ayant un DN par anomalie du chromosome 6 ont été traités avec succès ainsi que 2/3 des cas que nous avons rapportés. Conclusion : les SU constituent un traitement efficace et sécuritaire du diabète néonatal monogénique par mutation des gènes KCNJ11, ABCC8 mais aussi par anomalies du chromosome 6. La production d’une forme galénique adaptée à la population pédiatrique est à promouvoir

Neonatal diabetes due to potassium channel mutation: Response to sulfonylurea according to the genotype

Objective A precision medicine approach is used to improve treatment of patients with monogenic diabetes. Herein, we searched SU efficiency according to the genotype-phenotype correlation, dosage used, and side effects. Research Design and Methods Systematic review conducted according the PRISMA control criteria identifying relevant studies evaluating the in vivo and in vitro sensitivity of ATP-dependent potassium channels according to the characteristics of genetic mutation. Results Hundred and three selected articles with complete data in 502 cases in whom 413 (82.3%) had mutations inKCNJ11(#64) and 89 inABCC8(# 56). Successful transfer from insulin to SU was achieved in 91% and 86.5% patients, respectively, at a mean age of 36.5 months (0-63 years). Among patients withKCNJ11andABCC8mutations 64 and 46 were associated with constant success, 5 and 5 to constant failure, and 10 and 4 to variable degrees of reported success rate, respectively. The glibenclamide dosage required for each genotype ranged from 0.017 to 2.8 mg/kg/day. Comparing both the in vivo and in vitro susceptibility results, some mutations appear more sensitive than others to sulfonylurea treatment. Side effects were reported in 17/103 of the included articles: mild gastrointestinal symptoms and hypoglycaemia were the most common. One premature patient had an ulcerative necrotizing enterocolitis which association with SU is difficult to ascertain. Conclusions Sulfonylureas are an effective treatment for monogenic diabetes due toKCNJ11andABCC8genes mutations. The success of the treatment is conditioned by differences in pharmacogenetics, younger age, pharmacokinetics, compliance, and maximal dose used."Societe Francophone du diabete" (2012) European Society for Pediatric Endocrinology Societe Francophone du Diabet

Treatable Hyperkinetic Movement Disorders Not to Be Missed

International audienceHyperkinetic movement disorders are characterized by the presence of abnormal involuntary movements, comprising most notably dystonia, chorea, myoclonus, and tremor. Possible causes are numerous, including autoimmune disorders, infections of the central nervous system, metabolic disturbances, genetic diseases, drug-related causes and functional disorders, making the diagnostic process difficult for clinicians. Some diagnoses may be delayed without serious consequences, but diagnosis delays may prove detrimental in treatable disorders, ranging from functional disabilities, as in dopa-responsive dystonia, to death, as in Whipple's disease. In this review, we focus on treatable disorders that may present with prominent hyperkinetic movement disorders

The “Neurospeed” game: a fun tool to learn the neurological semiology

International audienceBackground: Neurological semiology is often considered by medical students as particularly difficult to learn. Finding alternative teaching methods may improve students' motivation and understanding of this field. Methods: We developed the "Neurospeed", a game to learn neurological syndromes. We assessed its efficiency on short-term learning of neurological syndromes in third-year medical students, through Multiple Choice Questions (MCQs) before and after the game session. Students' satisfaction was evaluated by a satisfaction survey. Results: Out of the 199 third-year medical students of the Faculty of Medicine Sorbonne Paris Nord, 180 attended the Neurospeed in December 2020, and 148 answered 20 Multiple Choice Questions before and after the game, with significant improvement of their score (p < 0.001). Most of the participants agreed that the game was playful, stimulating, and helpful to learn neurological semiology. Conclusions: Overall, our results show that the Neurospeed game is an interesting tool as a complement to traditional lectures. Further studies are necessary to compare the efficacy of different types of serious games on short-term and long-term learning of neurological semiology

Chapitre VIII. Dynamique fluviale holocène de la Loire moyenne (val d’Orléans, France)

Introduction La gestion du risque fluvial actuel et futur nécessite une bonne compréhension de la complexité de la réponse fluviale aux changements du climat et de l’occupation du bassin-versant par les sociétés. Dans le contexte actuel de changement global, les reconstitutions des dynamiques fluviales, survenues durant le passé en réponse aux changements climatiques et sociaux, constituent des clés pour une meilleure perception des dynamiques actuelles et futures (Gregory et al., 2006). Les ..

Chapter 8. Holocene fluvial dynamics of the middle Loire River (Val d’Orléans, France)

Introduction Current and future fluvial risk management requires a good understanding of the complexity of fluvial responses to climate and societal changes. In the current context of global change, the reconstruction of fluvial dynamics in response to climate and societal changes during the past, are key for a better perception of current and future dynamics (Gregory et al., 2006). During the last few decades, several works on European catchments have revealed important variability in fluvia..

Two critical brain networks for generation and combination of remote associations

International audienceRecent functional imaging findings in humans indicate that creativity relies on spontaneous and controlled processes, possibly supported by the default mode and the fronto-parietal control networks, respectively. Here, we examined the ability to generate 10 and combine remote semantic associations, in relation to creative abilities, in patients with focal frontal lesions. Voxel-based lesion-deficit mapping, disconnection-deficit mapping and network-based lesion-deficit approaches revealed critical prefrontal nodes and connections for distinct mechanisms related to creative cognition. Damage to the right medial prefrontal region, or its potential disrupting effect on the default mode network, affected the ability to generate remote ideas, likely by altering the organization of semantic associations. Damage to the left rostrolateral prefrontal region and its connections, or its potential disrupting effect on the 15 left fronto-parietal control network, spared the ability to generate remote ideas but impaired the ability to appropriately combine remote ideas. Hence, the current findings suggest that damage to specific nodes within the default mode and fronto-parietal control networks led to a critical loss of verbal creative abilities by altering distinct cognitive mechanisms