Freins et leviers à la diffusion des associations variétales de blé: Etude de cas en France et au Danemark

National audienc

Freins et leviers socio-économiques à la diffusion des mélanges variétaux pour la production de blé : Une comparaison entre France et Danemark

L’adoption des mélanges variétaux par les agriculteurs français est aujourd’hui relativement faible. Dans cette étude, nous cherchons à déterminer les freins et leviers à une diffusion plus large de cette pratique. L’analyse d’entretiens menés auprès des acteurs amont et aval de la filière blé tendre française, ainsi qu’une étude comparée avec la filière orge du Danemark, mettent en évidence trois freins principaux : 1) un frein réglementaire, les mélanges n’étant à ce jour pas autorisés à la commercialisation en France, 2) un frein lié aux standards de qualité exigés par certains débouchés aval comme la meunerie et surtout 3) un frein lié à un manque de connaissances sur les avantages et les effets des mélanges, ainsi que sur les règles de conception des mélanges

Algorithm for the early diagnosis and treatment of patients with cross reactive immunologic material-negative classic infantile pompe disease: a step towards improving the efficacy of ERT.

ObjectiveAlthough enzyme replacement therapy (ERT) is a highly effective therapy, CRIM-negative (CN) infantile Pompe disease (IPD) patients typically mount a strong immune response which abrogates the efficacy of ERT, resulting in clinical decline and death. This study was designed to demonstrate that immune tolerance induction (ITI) prevents or diminishes the development of antibody titers, resulting in a better clinical outcome compared to CN IPD patients treated with ERT monotherapy.MethodsWe evaluated the safety, efficacy and feasibility of a clinical algorithm designed to accurately identify CN IPD patients and minimize delays between CRIM status determination and initiation of an ITI regimen (combination of rituximab, methotrexate and IVIG) concurrent with ERT. Clinical and laboratory data including measures of efficacy analysis for response to ERT were analyzed and compared to CN IPD patients treated with ERT monotherapy.ResultsSeven CN IPD patients were identified and started on the ITI regimen concurrent with ERT. Median time from diagnosis of CN status to commencement of ERT and ITI was 0.5 months (range: 0.1-1.6 months). At baseline, all patients had significant cardiomyopathy and all but one required respiratory support. The ITI regimen was safely tolerated in all seven cases. Four patients never seroconverted and remained antibody-free. One patient died from respiratory failure. Two patients required another course of the ITI regimen. In addition to their clinical improvement, the antibody titers observed in these patients were much lower than those seen in ERT monotherapy treated CN patients.ConclusionsThe ITI regimen appears safe and efficacious and holds promise in altering the natural history of CN IPD by increasing ERT efficacy. An algorithm such as this substantiates the benefits of accelerated diagnosis and management of CN IPD patients, thus, further supporting the importance of early identification and treatment initiation with newborn screening for IPD

Algorithm for the early diagnosis and treatment of patients with cross reactive immunologic material-negative classic infantile pompe disease: a step towards improving the efficacy of ERT.

OBJECTIVE: Although enzyme replacement therapy (ERT) is a highly effective therapy, CRIM-negative (CN) infantile Pompe disease (IPD) patients typically mount a strong immune response which abrogates the efficacy of ERT, resulting in clinical decline and death. This study was designed to demonstrate that immune tolerance induction (ITI) prevents or diminishes the development of antibody titers, resulting in a better clinical outcome compared to CN IPD patients treated with ERT monotherapy. METHODS: We evaluated the safety, efficacy and feasibility of a clinical algorithm designed to accurately identify CN IPD patients and minimize delays between CRIM status determination and initiation of an ITI regimen (combination of rituximab, methotrexate and IVIG) concurrent with ERT. Clinical and laboratory data including measures of efficacy analysis for response to ERT were analyzed and compared to CN IPD patients treated with ERT monotherapy. RESULTS: Seven CN IPD patients were identified and started on the ITI regimen concurrent with ERT. Median time from diagnosis of CN status to commencement of ERT and ITI was 0.5 months (range: 0.1-1.6 months). At baseline, all patients had significant cardiomyopathy and all but one required respiratory support. The ITI regimen was safely tolerated in all seven cases. Four patients never seroconverted and remained antibody-free. One patient died from respiratory failure. Two patients required another course of the ITI regimen. In addition to their clinical improvement, the antibody titers observed in these patients were much lower than those seen in ERT monotherapy treated CN patients. CONCLUSIONS: The ITI regimen appears safe and efficacious and holds promise in altering the natural history of CN IPD by increasing ERT efficacy. An algorithm such as this substantiates the benefits of accelerated diagnosis and management of CN IPD patients, thus, further supporting the importance of early identification and treatment initiation with newborn screening for IPD

Determinants of Quality of Life in Children with Inborn Errors of Metabolism Receiving a Restricted Diet

International audienceObjective To investigate the determinants of quality of life (QoL) in children with inborn errors of metabolism with restricted diet (IEMRDs) using a single theory-based multidimensional model. Study design In this multicenter cross-sectional study, data from children aged 8-17 years with IEMRDs (except phenylketonuria) and their parents were collected from January 2015 to December 2017. Measurements included a child's self-reported QoL, self-rated behavioral problems and anxiety, and parental anxiety. Based on hypotheses from a literature-built theoretical model linking demographic, clinical, family environment, and psychosocial characteristics to QoL either directly or indirectly, associations of these factors with a child's self-rated QoL were examined using a structural equation modeling approach. Results A total of 312 children (mean [SD] age, 12.2 [2.6] years; 51% boys [n = 160]) were included. Higher levels of trait anxiety and behavioral problems in children were the most important factors associated with poorer QoL (standardized path coefficients, À0.71 and À0.23, respectively). In addition, higher parent trait anxiety, younger age at diagnosis, and a disease requiring an emergency diet were associated with poorer QoL in these children. The final model fit the data closely according to conventional goodness-of-fit statistics and explained 86% of the QoL variance

Health Status of French Young Patients with Inborn Errors of Metabolism with Lifelong Restricted Diet

International audienc

Cultiver et protéger autrement: Alternatives aux phytosanitaires : mobiliser les leviers de l'agroécologie, du biocontrôle et de la prophylaxie pour une agriculture performante et durable. Propositions pour un Programme Prioritaire de Recherche.

Frédérique Vidal, Ministre de l’Enseignement supérieur, de la recherche et de l’Innovation et Guillaume Boudy, Secrétaire général pour l’investissement, se sont vus remettre par Philippe Mauguin, Président directeur général de l’Inra le rapport définissant les lignes directrices du programme prioritaire de recherche intitulé « Cultiver et Protéger Autrement ». Ce programme dont le principe avait été annoncé en juillet 2018 sera doté de 30 millions d’euros provenant du 3e Programme d’investissement d’avenir. Le rapport propose une feuille de route claire et ambitieuse pour que la communauté scientifique élabore avec les acteurs du monde agricole de nouvelles pratiques alternatives à l’utilisation des produits phytosanitaires. Les connaissances scientifiques sur les plantes cultivées, sur leurs interactions avec le sol, avec leurs adventices, avec leurs agresseurs : insectes, champignons, virus… seront approfondies et mobilisées afin d’utiliser désormais les services de la biodiversité pour stimuler les mécanismes de défense naturels au niveau de la plante, de la parcelle, du paysage et pour conduire les cultures. Pour compléter ces actions de recherche très innovantes, le rapport préconise la mise en place d’outils structurants. Il est envisagé par exemple de coupler capteurs biologiques et intelligence artificielle pour suivre en temps réel l’apparition et le développement des bioagresseurs et aider ainsi à la conduite des cultures. Pour accompagner la transition vers des pratiques agricoles sans pesticides, des infrastructures de référence seront également développées ou créées pour caractériser au mieux l’exposition des travailleurs agricoles et de la population et contribuer à garantir l’innocuité des substances qui pourraient être amenées à se substituer aux phytosanitaires les plus préoccupants. Le Ministère de l’enseignement supérieur, de la recherche et de l’innovation, le Secrétariat général aux investissements et l’Agence nationale de la recherche vont désormais travailler avec l’Inra et les acteurs de la recherche publique et privée pour identifier, définir et séquencer l’appel à projets qui sera lancé en juin 2019. Le pilotage scientifique du programme sera assuré par l’Inra pour le compte de l’ensemble de la communauté scientifique française et en interaction avec elle, en s’appuyant sur un comité scientifique international. Les actions du programme conforteront les initiatives nationales de recherche et recherche appliquée visant à réduire l’usage des pesticides notamment les appels à projet dédiés de l’Agence Nationale de la recherche et le plan Ecophyto 2+ porté par les ministères de la Transition Écologique et Solidaire, des Solidarités et de la Santé, de l’Enseignement supérieur, de la Recherche et de l’Innovation, de l’Agriculture et de l’Alimentation ainsi que les initiatives européennes de programmation de la recherche, notamment celles qui seront lancées dans le programme cadre Horizon-Europe

Individual and Family Determinants for Quality of Life in Parents of Children with Inborn Errors of Metabolism Requiring a Restricted Diet: A Multilevel Analysis Approach

International audienceObjective The objective of this study was to compare the quality of life (QoL) for parents of children with inborn errors of metabolism (IEMs) requiring a restricted diet with French population norms and investigate parental QoL determinants