Contribution to the management of vocational training policies in organizations

Cette thèse s’inscrit dans une ambition de Validation des Acquis de l’Expérience. À ce titre sa première partie décrit un itinéraire professionnel à travers l’exercice de différentes responsabilités dans une carrière essentiellement dédiée à la formation professionnelle. Elle cherche à démontrer que le professionnalisme acquis peut s’assimiler à des compétences d’expertise. La deuxième partie explique comment un praticien de la formation a pu et voulu, pour l’exercice opérationnel de son propre métier, bénéficier des apports de la recherche et des chercheurs. Au point d’instituer une certaine porosité entre leurs activités respectives, facteur objectif de progrès réciproques. La troisième partie témoigne d’une recherche approfondie sur les conditions de pilotage des politiques de formation dans les institutions de travail. À partir du récit des pratiques issues de la Loi française de 1971, elle démontre le caractère unilatéral de la seule logique d’investissement en matière de formation, négligeant, de ce fait la réalité des pratiques effectives. Forte de ce constat, elle propose un nouveau postulat, postulat d’une double logique de pilotage de la formation dans les organisations : celle liée aux risques de dysfonctionnements dans la production du travail et celle d’investissements pour faire face aux risques d’inemployabilité liés aux évolutions des organisations et des technologies... Les modalités de mise en œuvre de la formation qui en résultent y sont présentées dans leur détail. Enfin, ce chapitre se termine sur les conditions actuelles et prospectives pour une mise en œuvre positive de ce pilotage.This thesis is part of an ambition to Validation des Acquis de l’Expérience (Validation of Acquired Experience). As such, its first part describes a career path through the exercise of different responsibilities in a career mainly dedicated to vocational training. It seeks to demonstrate that the professionalism acquired can be assimilated to expertise skills. The second part explains how a training practitioner was able and willing, for the operational exercise of his own profession, to benefit from the contributions of research and researchers. To the point of instituting a certain porosity between their respective activities, objective factor of reciprocal progress. The third part shows an in-depth research on the management conditions of training policies in work institutions. From the narrative of the practices resulting from the French Law of 1971, it demonstrates the unilateral character of the sole logic of investment in training, neglecting, therefore, the reality of the actual practices. On the strength of this observation, it proposes a new postulate: a postulate of a dual logic of training management in organizations, one related to the risks of dysfunctions in the production of work and one related to the investments to cope with the risks of unemployability due to the evolution of organizations and technologies ... The resulting methods of implementing the training are presented in detail. Finally, this chapter ends with the current and prospective conditions for a positive implementation of this pilotage

Aux sciences, citoyens !

Ce livre décrit plus de 25 expériences de consultation et de participation, réalisées au Québec et ailleurs dans le monde, touchant les enjeux scientifiques les plus divers. Couronnées de succès ou pas, ces expériences sont toujours instructives. C'est pourquoi les auteurs proposent aussi un répertoire des méthodes et des moyens utilisés pour en évaluer la pertinence et l'efficacité

SORD-related peripheral neuropathy in a French and Swiss cohort: Clinical features, genetic analyses, and sorbitol dosages.

Biallelic variants in SORD have been reported as one of the main recessive causes for hereditary peripheral neuropathies such as Charcot-Marie-Tooth disease type 2 (CMT2) and distal hereditary motor neuropathy (dHMN) resulting in lower limb (LL) weakness and muscular atrophy. In this study, phenotype and genotype landscapes of SORD-related peripheral neuropathies were described in a French and Swiss cohort. Serum sorbitol dosages were used to classify SORD variants. Patients followed at neuromuscular reference centres in France and Switzerland were ascertained. Sanger sequencing and next generation sequencing were performed to sequence SORD, and mass spectrometry was used to measure patients' serum sorbitol. Thirty patients had SORD peripheral neuropathy associating LL weakness with muscular atrophy, foot deformities (87%), and sometimes proximal LL weakness (20%) or distal upper limb weakness (50%). Eighteen had dHMN, nine had CMT2, and three had intermediate CMT. Most of them had a mild or moderate disease severity. Sixteen carried a homozygous c.757delG (p.Ala253Glnfs*27) variant, and 11 carried compound heterozygous variants, among which four variants were not yet reported: c.403C > G, c.379G > A, c.68_100 + 1dup, and c.850dup. Two unrelated patients with different origins carried a homozygous c.458C > A variant, and one patient carried a new homozygous c.786 + 5G > A variant. Mean serum sorbitol levels were 17.01 mg/L ± 8.9 SD for patients carrying SORD variants. This SORD-inherited peripheral neuropathy cohort of 30 patients showed homogeneous clinical presentation and systematically elevated sorbitol levels (22-fold) compared to controls, with both diagnostic and potential therapeutic implications

Convergence of patient- and physician-reported outcomes in the French National Registry of Facioscapulohumeral Dystrophy

International audienceFacioscapulohumeral muscular dystrophy (FSHD) is among the most prevalent muscular dystrophies and currently has no treatment. Clinical and genetic heterogeneity are the main challenges to a full comprehension of the physiopathological mechanism. Improving our knowledge of FSHD is crucial to the development of future therapeutic trials and standards of care. National FSHD registries have been set up to this end. The French National Registry of FSHD combines a clinical evaluation form (CEF) and a self-report questionnaire (SRQ), filled out by a physician with expertise in neuromuscular dystrophies and by the patient, respectively. Aside from favoring recruitment, our strategy was devised to improve data quality. Indeed, the pairwise comparison of data from 281 patients for 39 items allowed for evaluating data accuracy. Kappa or intra-class coefficient (ICC) values were calculated to determine the correlation between answers provided in both the CEF and SRQ. Results Patients and physicians agreed on a majority of questions common to the SRQ and CEF (24 out of 39). Demographic, diagnosis- and care-related questions were generally answered consistently by the patient and the medical practitioner (kappa or ICC values of most items in these groups were greater than 0.8). Muscle function-related items, i.e. FSHD-specific signs, showed an overall medium to poor correlation between data provided in the two forms; the distribution of agreements in this section was markedly spread out and ranged from poor to good. In particular, there was very little agreement regarding the assessment of facial motricity and the presence of a winged scapula. However, patients and physicians agreed very well on the Vignos and Brooke scores. The report of symptoms not specific to FSHD showed general poor consistency. Conclusions Patient and physician answers are largely concordant when addressing quantitative and objective items. Consequently, we updated collection forms by relying more on patient-reported data where appropriate. We hope the revised forms will reduce data collection time while ensuring the same quality standard. With the advent of artificial intelligence and automated decision-making, high-quality and reliable data are critical to develop top-performing algorithms to improve diagnosis, care, and evaluate the efficiency of upcoming treatments