À propos de l’offre de traitement en milieu correctionnel : guérir, soigner, aider

This paper adresses the complex unconscious identificatory processes which may silently be active within the clinician when he proposes treatment either to a child or adolescent in an institution or to an adult in a correctionnal setting. Three representations of treatment serve as a backdrop to analyse different identificatory movements on the part of the clinician and how such unconscious motivations may affect his work

Design e criatividade nos processos de inovação aberta: o caso de distritos industriais italianos

This paper discusses the topic of collaborative innovation as a social process that involves the exchange of knowledge focused on the existence of dense social fabric relationships between potential problem solvers (e.g. designers, suppliers, research institutions) to generate innovation. Innovation networks are developed either through market (traded interdependencies) or not-for-market (untraded interdependencies) relationships, the latter facilitated by spatial proximity. Territories and cities with their local communities are therefore also crucial in collaborative innovation processes. The territories are now recognized as a repository of local knowledge based on the experiences of those who live in that specific context, but shared with producers, workers and end-users. Today a “cognitive role” is attributed to the territory: first, it provides knowledge instrumental to the production system and also promotes, under certain conditions, a converging of talented people, values and social behaviors that determine the very meaning of life and production in the territory. The challenge for companies lies in the ability to combine exogenous factors (architectures of collaborative innovation) with endogenous factors, the latter related to territorial contexts that stimulate, enhance and channel individual expressions of creativity. The cases of some Italian industrial districts specialized in design-oriented products exemplify interesting governance architectures, significantly different from the standard open-innovation of Anglo-Saxon contexts. Key words: territory, industrial district, open innovation, design- driven innovation, creativity.Este artigo discute o tema da inovação colaborativa como um processo social que envolve o intercâmbio de conhecimentos centrado na existência de um denso tecido de relações sociais entre os solucionadores de problemas em potencial (por exemplo, designers, fornecedores, instituições de pesquisa) para gerar inovação. Redes de inovação são desenvolvidas através de relações de o mercado (interdependências negociadas) ou de não mercado (interdependências não-negociadas), este facilitado pela proximidade espacial. Territórios e cidades com suas comunidades locais são, portanto, também cruciais nos processos de inovação colaborativa. Os territórios são agora reconhecidos como um repositório de conhecimento local com base nas experiências das pessoas que vivem nesse contexto específico, mas compartilhadas com os produtores, trabalhadores e usuários finais. Hoje é atribuído um papel cognitivo ao território: primeiro, fornece o conhecimento instrumental para o sistema de produção e promove também, em certas condições, uma convergência de talentos, valores e comportamentos sociais que determinam o próprio sentido da vida e da produção no território. O desafio para as empresas reside na capacidade de combinar fatores exógenos (arquiteturas de inovação colaborativa) com fatores endógenos, estes referentes a contextos territoriais que estimulam, aprimoram e canalizam as expressões da criatividade individual. Os casos de alguns distritos industriais italianos especializados em produtos orientados por design nos servirão para exemplificar arquiteturas de governança significativamente diferentes do padrão de inovação aberta dos contextos anglo-saxões. Palavras-chave: território, distrito industrial, inovação aberta, inovação movida a design, criatividade

A Possible Case of Neurogenic Pulmonary Edema in a Sheep following Intracranial Surgery

A 3-year-old female crossbred sheep weighing 64 kg was anaesthetized for intracranial surgery as a part of a research project. Premedication and induction of anesthesia were uneventful as well as tracheal intubation. Anesthesia was maintained with isoflurane in a 50% mixture of oxygen and air, fentanyl (5-15 µg kg-1h-1) and lidocaine (1.8 mg kg-1h-1). During anesthesia, an increased alveolar-arterial oxygen gradient was calculated on the basis of arterial blood gas analysis: inspiratory fraction of oxygen was increased and a recruitment manoeuvre was performed. After 210 minutes of anesthesia, the sheep was let recover with oxygen supplementation under monitoring of pulse-oxymetry, capnography, inspired and expired oxygen, temperature and invasive blood pressure. At tracheal extubation no signs of regurgitation or aspiration were noticed. Twenty-five minutes later, the sheep showed deterioration of neurological status and clonic seizure responsive to diazepam. After transient tachycardia, blood pressure rose acutely and sinus bradycardia followed. Severe tachypnea started in few minutes accompanied by loud respiratory noises and harsh diffuse crackles on both sides of the thorax. Foamy blood nasal exudates discharged from the nostrils. Neurogenic pulmonary edema as a sequel of increased intracranial pressure was suspected and treated with intravenous mannitol (0.5 gkg-1) and furosemide (4 mgKg-1). Hypoxemia was successfully managed with oxygen supplementation. Motor and cognitive functions improved progressively and were deemed normal within 12 hours from the episode, when arterial partial pressure of oxygen was 11.7 kPa (88 mmHg) at room air

Regulation of cytotoxic lymphocyte effector functions

Studies of patients with inborn errors of immunity provide an important opportunity to understand the human immune system in a natural context. The articles in this thesis are a compilation of such investigations. With our work, we aimed to identify the cause of primary hemophagocytic lymphohistiocytosis (HLH) in an unexplained pediatric patient (paper I), explore the modulation of SAMD9 and SAMD9L gain-of-function (GOF) mutants (paper II), and understand natural killer (NK) cell biology in the context of DEF6 deficiency (paper III). In paper I, we uncovered biallelic loss-of-function variants in RHOG in a 4-monthold patient presenting with HLH and displaying defective lymphocyte exocytosis. Deletion of RHOG in a human NK cell line abrogated exocytosis that could be rescued by constructs expressing wild type RHOG protein. Moreover, we found that MUNC13-4, associated with autosomal recessive familial HLH type 3, required RHOG interactions for recruitment to the plasma membrane during cytotoxic granule exocytosis. Thus, we demonstrated that RHOG is essential in cytotoxic granule exocytosis by human T and NK cells and proposed that biallelic loss-offunction mutations in RHOG are a novel cause for familial HLH. In paper II, we examined a variety of pathogenic SAMD9 and SAMD9L GOF variants associated with syndromes encompassing bone marrow failure, autoinflammation or selective loss of NK cells, B cells, and monocytes. We sought to understand whether viral host range factors, which are known to antagonize wild-type SAMD9 and SAMD9L proteins, could counteract the anti-proliferative and anti-translational activities of these pathogenic variants. SAMD9 or SAMD9L variants and viral factors were overexpressed in a cell line, followed by biochemical and functional analyses. Vaccinia virus K1 exhibited the highest inhibitory capacity but could not antagonize a truncated variant of SAMD9L that lacked the K1 binding site. The other factors (M062, C7 and KI) could interact with all the SAMD9 and SAMD9L mutants but displayed low capacity in antagonizing the anti-translational and anti-proliferative action of SAMD9/9L mutants. This study provided some novel insights into the structure and the regulation of SAMD9/9L proteins. Additionally, it showed that targeting pathogenic GOF SAMD9/L mutants via viral host range factors or, more broadly speaking, by protein-protein interaction is possible. Autosomal recessive mutations in DEF6 are associated with autoimmunity and severe herpes virus infections. While the latter is a hallmark of NK cell deficiencies, this cell type has not been thoroughly studied in DEF6-deficient patients. In paper III, we surveyed the role of DEF6 in NK cell development and function. We analyzed NK cell phenotypes in DEF6-deficient patients from two unrelated families and found a reduction in canonical CD56dimPLZF+ NK cells concomitant with the expansion of CMV-induced adaptive CD56dimPLZF– NK cells. Deletion of DEF6 in induced pluripotent stem cells gave rise to mature NK cells in an in vitro culture system. In primary human NK cells, deletion of DEF6 reduced cell viability, degranulation and IFN-γ production. We therefore postulate that impaired canonical NK cell survival and function may contribute to the viral susceptibility of DEF6 deficient individuals. To conclude, these studies have shed light on several cellular and molecular mechanisms behind IEI. These mechanisms enlarge our understanding of cytotoxic lymphocytes and of the immune system in general. Moreover, they have inspired us to pursue new directions and experimental approaches in our research

Fonctionnement sectaire et violence envers les enfants : le cas de l’Église baptiste de Windsor

Cet article vise, par une analyse de cas unique, à étudier l’évolution d’un groupe religieux minoritaire du Québec, l’Église baptiste de Windsor, vers un fonctionnement sectaire particulier qui l’isola de son environnement social et donna lieu à une grave situation de violence contre des mineurs. Les enfants du groupe y ont été soumis à de sévères punitions corporelles qui furent jugées comme constituant des sévices physiques par les services de protection de l’enfance et par le bureau du procureur général. Grâce à une analyse documentaire exhaustive à laquelle se sont ajoutés des témoignages d’anciens membres du groupe ainsi que des entretiens avec des informateurs clés, la reconstruction de l’histoire du groupe, de sa fondation à sa dissolution, a été rendue possible. Quatre moments dans le développement du groupe ont été identifiés ; leur analyse a permis de cerner les éléments qui ont contribué à la mise en place d’un contexte groupal et doctrinal qui légitimait le recours généralisé aux sévices physiques des enfants y vivant.Based on a case study, this article presents the evolution of a minority religious group of Quebec, the Baptist church of Windsor, towards a sectarian group whom, isolated from its social environment, resorted to severe child physical abuse. The children of the group were indeed subject to severe physical punishment that was considered child battering by the child protection agency and office of the attorney general. Through an exhaustive analysis of documents, complemented with testimonies of former members and interviews with key participants, the existence of the group is analyzed from origin to dissolution. Four stages in the evolution of the group were identified and its analysis highlights the elements contributing to the development of a sectarian and doctrinal context in which the use of severe physical punishment was generally accepted as legitimate.Este artículo tiene como objetivo estudiar, a partir de un caso único, la evolución de un grupo religioso minoritario de Quebec, la Iglesia bautista de Windsor, hacia un funcionamiento sectario y autoritario que lo aisló del ámbito social. Los niños de dicho grupo eran sometidos a severos castigos corporales que fueron considerados como maltrato físico por los servicios de protección de la infancia y por la oficina del procurador general. Gracias a un análisis documental exhaustivo acompañado de testimonios de ex miembros y de entrevistas con informantes clave ha sido posible reconstruir la historia de este grupo de su fundación a su disolución. Cuatro momentos en la evolución del grupo fueron identificados y su análisis permitió identificar los elementos que contribuyeron al surgimiento de un contexto grupal y doctrinal que legitimaba el recurso generalizado al maltrato físico de los menores del grupo

Shock-capturing with discontinuous garlekin methods

A shock capturing strategy for high order Discontinuous Galerkin methods for conservation laws is proposed. We present a method in the one-dimensional case based on the introduction of artificial viscosity into the original equations. With this approach the shock is capture with sharp resolution maintaining high-order accuracy. The ideas for the extension to the two-dimensional case are also set

Entre le retrait et la contestation. Réactions des mormons fondamentalistes à des allégations d’entorses aux lois

L’objectif de cet article est de comprendre les effets sur un groupe de mormons polygames fondamentalistes établis à Bountiful, en Colombie-Britannique, d’allégations d’entorses aux lois. Ces allégations vont du trafic de jeunes femmes entre le Canada et les États-Unis afin de les marier à des hommes polygames plus âgés qu’elles aux sévices physiques et sexuels sur des enfants, des adolescents et des femmes. Des entretiens avec des membres de ce groupe ainsi que l’analyse documentaire du journal de la communauté, des échanges sur le forum de discussion Web du groupe et des principaux journaux et périodiques canadiens et étatsuniens de 1990 à 2006 ont permis de documenter les allégations et de cerner les réactions du groupe. Celles-ci sont distinguées en deux périodes, un premier temps où le groupe a réagi par un retrait silencieux, puis un second où la contestation a dominé. Ces réactions sont décrites, analysées et illustrées à l’aide d’extraits d’entretiens et d’échanges Web.This article seeks to understand the impact of allegations of illegal conduct on a group of polygamous Fundamentalist Mormons established in Bountiful, British Columbia. These allegations include the illegal passage of young women between Canada and the United States in order to provide older polygamous men with young wives, as well as the sexual and physical abuse of children, adolescents and women. Interviews with group members, analysis of the writings of the group and of the discussions held by members of the group on a WEB Forum, as well as a review of the principal Canadian and American periodicals and newspapers from 1990 to 2006 are used to identify the allegations and outline the group’s reactions. Two distinct types of reactions to the allegations are identified, the first being a silent retreat, which was followed by a reaction of contestation. These reactions are described, analysed and illustrated with the help of extracts from interviews and Web exchanges.Este artículo tiene por objetivo comprender el impacto de diversos alegatos relativos a violaciones a la ley sobre un grupo de mormones fundamentalistas polígamos establecidos en Bountiful, Columbia Británica. Dichos alegatos van del tráfico de mujeres jóvenes entre Canadá y Estados Unidos con el fin de casaras con hombres polígamos mayores que ellas a abusos físicos y sexuales sobre menores, adolescentes y mujeres. Entrevistas a miembros de este grupo así como un análisis documental del periódico de la comunidad, de intercambios en el foro de discusión web del grupo y de los principales diarios y periódicos canadienses y estadounidenses de 1990 a 2006 han permitido documentar dichos alegatos e identificar las reacciones del grupo ante ellos. Dos tipos de reacción fueron identificados: en un primer tiempo, un repliegue silencioso del grupo; en un segundo tiempo, una actitud de protesta predominó. Estas reacciones son descritas, analizadas e ilustradas gracias a extractos de entrevistas e intercambios web