Cette thèse porte sur une étude des aspects jusqu’à présent négligés concernant les ressources immédiatement accessibles aux entrepreneurs des jeunes et petites entreprises. En s’appuyant sur l’argument que les ressources immédiatement accessibles aux entrepreneurs sont leur capital humain et social, l’étude est d’abord centrée sur une lecture individuelle, qui met en exergue l’entrepreneur et son capital humain. Ensuite, elle tient compte explicitement de l'enracinement social des entrepreneurs et plus précisément de leur enracinement familial « family embeddedness ». Ainsi, l’étude s’ouvre à l'entrepreneur et son conjoint, donc au couple d’entrepreneurs. Cette thèse s'inscrit dans les domaines de recherche de l'entrepreneuriat et de l'entreprise familiale, et emprunte des concepts et des théories à l'économie et à la sociologie. Chacun des quatre articles qui constituent la thèse est une étude autonome destinée à la publication. Néanmoins, toutes les études se rapportent à une problématique centrale qui traite des aspects encore négligés concernant les liens entre les résultats des petites entreprises, l'entrepreneur et le couple d’entrepreneurs. / This thesis focuses on a study of the hitherto neglected aspects of the resources immediately accessible to entrepreneurs of young and small businesses. Based on the argument that the resources immediately available to entrepreneurs are their human and social capital, the study is primarily centered on individual reading, which highlights the entrepreneur and his human capital. Then, it explicitly takes into account the social roots of entrepreneurs and more specifically their family-embeddedness. Thus, the study opens to the entrepreneur and his spouse, thus to the couple of entrepreneurs. This thesis is part of the research fields of entrepreneurship and the family business, and borrows concepts and theories from economics and sociology. Each of the four articles that make up the thesis is an independent study for publication. Nevertheless, all the studies relate to a central issue that deals with the still neglected aspects of the links between the results of small firms, the entrepreneur and the couple of entrepreneurs.

L’objet de cette thèse est de mettre en lumière la politique dynastique des Wittelsbach dans le premier tiers du XIXe siècle. L’étude part de l’hypothèse qu’il s’agit là d’une politique savamment orchestrée qui, le plus souvent, fut couronnée de succès. De plus, ce travail dégage le concept d’ensemble de cette politique en démontrant qu’elle repose sur les trois piliers de « pouvoir », « parenté » et « amour ». Après avoir présenté les acteurs principaux dans le contexte de leur réseau familial (qui servit de moyen de consolidation de pouvoir), l’étude se penche sur le rôle éminent de l’éducation dispensée aux enfants princiers. Sont ensuite examinées les négociations menées pour marier ces princes et princesses le plus avantageusement possible : ici, l’étude démontre que les idées nouvelles (postulant la primauté du mariage d’inclination sur le mariage de raison) n’ont été prônées que pour voiler le calcul politique. Finalement, l’examen des conséquences de la politique dynastique révèle une grande homogénéité entre les principes transmis par les parents et l’action des enfants. / This thesis analyses the dynastic politics of the House of Wittelsbach in the first third of the 19th century. The study assumes that these politics were well orchestrated and most often successful. Furthermore, the thesis aims at showing that the global concept of these politics was based on the three pillars “power”, “kinship” and “love”. After presenting the main actors in the context of their family network (which served as a medium of consolidating power), the study examines the key role played by the education of the royal children. The thesis then explores the negotiations led in order to marry the princes and princesses to the highest advantage: here it is shown that the new ideas (postulating the primacy of the marriage for love over the marriage of convenience) were only used in order to veil political calculation. Finally, the focus is put on the consequences of these dynastic politics: it is revealed that a great degree of congruence exists between the parents’ principles and the children’s actions.

Introdução: A amiloidose transtiretina é uma doença autossômica dominante decorrente de uma proteína transtiretina (TTR) variante, que sofre uma mudança conformacional e origina um tetrâmero de TTR instável, passo que é decisivo para o início da formação dos depósitos amilóides em diferentes órgãos e tecidos. Na maioria dos pacientes, o sistema nervoso periférico é o alvo principal, resultando na polineuropatia amiloidótica familiar transtiretina (TTR-FAP), classicamente uma neuropatia sensitivo-motora e autonômica progressiva, evoluindo para o óbito em aproximadamente 10 anos. A mutação de ponto mais frequente no mundo, incluindo o Brasil, é a TTRVal30Met, entretanto mais de 100 mutações de ponto diferentes já foram descritas. Objetivos: descrever a epidemiologia mutacional do gene TTR na polineuropatia amiloidótica familiar e correlacionar estas mutações com seus achados clínicos e eletroneuromiográficos. Métodos: estudo de coorte, descritivo e retrospectivo de um grupo de pacientes brasileiros encaminhados para o serviço de neurogenética do HC da FMRP-USP para investigação de neuropatia periférica, cujo estudo genético identificou uma mutação no gene TTR, com posterior análise transversal dos resultados obtidos entre os subgrupos com as diferentes mutações. Resultados: um total de 128 pacientes tiveram uma mutação de ponto no gene TTR identificada, dos quais 12 (9,4%) pacientes apresentaram uma mutação não TTRVal30Met, incluindo 4 patogênicas (6 pacientes, 4,7%) e 2 não patogênicas (6 pacientes, 4,7%). A mutações não TTRVal30Met patogênicas foram TTRAsp38Tyr (2 pacientes), TTRIle107Val (2 pacientes), TTRVal71Ala (1 paciente) e TTRVal122Ile (1 paciente). Dentre as mutações não patogênicas, foram encontradas TTRGly6Ser (5 pacientes) e TTRThr119Thr (1 paciente). A mutação TTRVal30Met estava presente em 116 (90,6%) pacientes, dos quais 52 possuíam dados clínicos e eletroneuromiográficos completos: 39 (75%) tiveram início precoce e 13 (25%), início tardio. O grupo de início precoce apresentou-se como a forma clássica da PAF-TTR, sem predileção de gênero (homens: 53,8%), manifestação inicial como neuropatia de fibras finas e autonômica (82,1%) e história familiar positiva (90,3%). A ENMG estava normal em 36,7% destes pacientes. O envolvimento cardiovascular foi caracterizado mais frequentemente por alterações da condução cardíaca (84,2%), sendo menos prevalente a cardiomiopatia (11,1%). Por outro lado, o grupo de início tardio mostrou uma predominância do sexo masculino (92,3%), presença de sintomas motores na primeira consulta (38,5%), resultando numa neuropatia sensitivo-motora com acometimento de fibras grossas e história familiar negativa (69,2%). Todos apresentaram neuropatia sensitivo-motora na ENMG. Neste grupo, a cardiomiopatia estava presente em 71,4% dos pacientes. Todos os pacientes, em ambos os grupos, tiveram disautonomia em algum momento do seu seguimento clínico. Conclusões: no nosso estudo aproximadamente 5% dos pacientes com FAP-TTR tinham uma mutação não TTRVal30Met, demonstrando a importância do sequenciamento do gene TTR em pacientes com história clínica sugestiva e screening negativo para a mutação TTR Val30Met. Além disso, os pacientes brasileiros com FAP-TTRVal30Met apresentaram achados clínicos e eletroneuromiográficos similares as populações descritas com esta mutação em outros países. / Background: Transthyretin amyloidosis is an autossomal dominant disease caused by variant transthyretin, that is misfolded, originating a unstable transthyretin tetramer, a rate-limiting step in the formation of the amyloid deposits in different organs and tissues. In most patients, the peripheral nervous system is the main target, leading to transtyretin familial amyloid neuropathy (TTR-FAP), classically characterized as a progressive sensory-motor and autonomic neuropathy, that leads to death in about 10 years. TTRVal30Met is the most frequent point mutation worldwide, including Brazil, but more than 100 different point mutations has been described. Objectives: describe the mutational epidemiology of TTR gene in TTR-FAP and characterize its clinical and electrophysiological findings. Methods: a descriptive and retrospective study of a group of Brazilian patients forwarded to the Neurogenetics or Peripheral Nerve Clinics from FMRP-USP whose etiological investigation identified a mutation in the TTR gene. A cross-sectional analysis evaluating the subgroups with different mutations was also carried on. Results: we identified one hundred and twenty eight patients carrying a TTR point mutation, of whom 12 (9,4%) harbored a non-Val30Met mutation, including 4 pathogenic (6 patients, 4,7%) and 2 non-pathogenic abnormalities (6 patients, 4,7%). The non Val30Met pathogenic mutations were TTRAsp38Tyr (2 patients), TTRIle107Val (2 patients), TTRVal71Ala (1 patient) and TTRVal122Ile (1 patient). Among the non-pathogenic mutations, we found the TTRGly6Ser (5 patients) and the TTRThr119Thr (1 patient). The TTRVal30Met mutation was present in 116 (90,6%) patients, of whom 52 had a complete clinical and neurophysiological data: 39 (75%) with early-onset and 13(25%) with late-onset neuropathies. The early-onset group presented as the classic TTRFAP, with no gender predominance (male: 53,8%), the first manifestations were those of a small fiber sensory and autonomic neuropathy (82,1%) and a highly positive family history (90,3%). EMG was normal in 36,7% of these patients. The cardiovascular involvement was characterized by frequent ECG abnormalities (84,2%), less often associated with cardiomayopathy (11,1%). On the other hand, the late-onset TTRVal30Met showed a male predominance (92,3%), presence of motor complaints in the first evaluation (38,5%) resulting in a sensory-motor polyneuropathy with large fiber involvement and a negative family history (69,2%). All patients presented a sensory and motor neuropathy on EMG examination. In this group, cardiomiopathy was frequently associated with the neuropathy (71,4%). All patients, in both groups, had autonomic symptoms at some point in clinical follow up. Conclusions: In our study almost 5% of the patients with TTR-FAP have a non Val30Met pathogenic mutation, highlighting the importance of sequecing the whole TTR gene in patients with a sugestive clinical history and negative screening for TTRVal30Met mutation. In adition, the Brazilian patients we studied with early and late onset TTR-FAP, present similar findings to TTRVal30Met populations from other countries submitted to similar studies.

Le handicap de l’enfant peut avoir des conséquences sur des dimensions de la vie familiale : santé physique et psychologique, relations sociales et familiales, temps de prise en charge, emploi et poids économique. Mieux connaître les facteurs liés à cet impact permettrait d'en limiter les conséquences négatives sur la vie familiale.L’impact familial de la paralysie cérébrale (PC) d’adolescents européens a été étudié en 2009 et des facteurs personnels, sociaux et environnementaux de leurs familles également recueillis. Un questionnaire sur l’impact familial du handicap dans 5 des 7 dimensions identifiées dans la littérature a été utilisé. Etendu aux dimensions manquantes par l’ajout de 4 questions, l’outil amélioré a été validé dans le cadre de cette enquête européenne.Un modèle théorique fondé sur le modèle d’adaptation Double ABCX a été confronté aux données. Sont mis en relation des éléments de perturbations de l’équilibre familial, liées à l’enfant ou à l’environnement (A), des ressources disponibles pour faire face (B), la perception de conséquences négatives (C) et le degré d’adaptation familiale, mesurée par la détresse psychologique (X). La modélisation par équations structurelles a permis de valider un modèle estimant des coefficients de corrélation significatifs entre les variables selon une méthode itérative par moindres carrés pondérés (WLSMV). 57,4% de la variance de la détresse psychologique a été expliquée par des perturbations (A) : âge avancé de l’adolescent (bêta=0.19), absence d’emploi des parents (bêta=0.25), sévérité de la déficience motrice (bêta=0.23), sévérité des troubles du comportement de l’adolescent (bêta=0.45) ; par des ressources (B) : accès à des modes de garde extérieurs (bêta=-0.15), degré de dysfonctionnement familial (bêta=0.40), sentiments positifs du parent par rapport au handicap (bêta=-0.16) ; et enfin par l’impact négatif perçu (C) sur la santé des membres de la famille (bêta=0.45). Des éléments de (A) et de (B) influençaient l’impact négatif perçu dans d’autres dimensions de vie familiale. D’après ce modèle, l’adaptation de la famille face au handicap de l’adolescent semble se jouer plus autour de la qualité du fonctionnement familial que sur la mise en place de compensations physiques ou financières. Il est donc important de mettre en place des actions permettant aux familles d’améliorer les différentes dimensions de ce fonctionnement. L’utilisation d’outils de mesure validés peut aider à évaluer et sélectionner des interventions les plus efficaces pour gérer les troubles du comportement chez l’enfant en situation de handicap, augmenter l’autonomie de l’enfant, favoriser le bon fonctionnement familial, ou promouvoir les attitudes parentales positives face au handicap.L’absence d’emploi influençant le bien-être psychologique, cette situation a été étudiée pour les mères d’enfants en situation de handicap. A partir de données disponibles, nous avons constaté qu’en tenant compte de nombreuses caractéristiques de l’enfant et de la famille, la présence d’une déficience intellectuelle chez l’enfant et un bas niveau d’études de la mère ou le non-emploi du père influençaient significativement le risque de non-emploi de la mère.Par ailleurs, les mères d’enfants en situation de handicap sévère se sont avérées proportionnellement plus nombreuses que la population générale à être sans emploi (47,2% contre 31,4%). En revanche, les mères d’enfants avec PC interviewées lors d’une enquête n’étaient pas plus sans emploi que les mères de la population générale, remettant en question l’usage de la PC comme modèle du handicap, pour l’étude des conséquences familiales.L’étude de l’impact du handicap est encore trop rare en Europe et les effets de possibles interventions mal connues. Il est important de développer la recherche dans ce domaine pour améliorer le quotidien de ces familles et offrir à l’enfant en situation de handicap le meilleur environnement possible pour son développement. / The child's disability could impact family life in several dimensions: physical and psychological health, social and family relationships, time management, employment and economic weight. Knowledge about the factors related to this effect will limit the negative impact on family life.The family impact of the cerebral palsy (CP) in European adolescents was studied in 2009 and the personal, social and environmental factors of these families were also collected. A questionnaire measuring 5 dimensions of the 7 identified in literature was used. Extended to missing dimensions by adding four questions, this improved tool has been validated in the framework of this European survey.A theoretical model based on the Double ABCX model of family adaptation has been compared to the situation observed by these families, linking the stressors related to the child or the family environment (A), the resources available to cope with (B), the negative perceived impact in different dimensions of the family life (C) and the level of family adaptation, measured by the parental psychological distress (X). Structural equation modelling was used to validate a model estimating significant correlation coefficients between variables by an iterative method using weighted least squares (WLSMV). 57.4% of the variance in psychological distress was explained by stressors (A): age of the adolescent (beta=0.19), parental unemployment (beta=0.25), the severity of motor impairment (beta=0.23), the severity of behavioural disorders in adolescents (beta=0.45); by resources (B), the access to support in the childcare (beta=-0.15), the level of family dysfunction (beta=0.40), the parental positive attitude towards disability (beta=-0.16); and finally by the negative perceived impact (C) on the health of family members (beta=0.45). Some elements from (A) and (B) explained the perceived family impact in various dimensions of family life. Based on this model, in adolescence the family adaptation to disability seems to be more played out on quality of family functioning than on physical adaptation or financial compensation. Thus it is important to implement process to enable families to improve different aspects of this functioning. The use of validated measurement tools can help to assess and select the most effective interventions to manage behavioural problems in children with disabilities, to increase the autonomy of the child, to promote family functioning or parental positive attitudes towards disability.A lack of employment affecting the psychological well-being, this has been studied for mothers of children with disabilities. From an analysis of available data, we found that taking into account many characteristics of the child and family, the presence of an intellectual impairment in children and a low level of education of the mother or the father’s nonemployment appeared to significantly influence the risk of non-employment of the mother. On the other hand, the mothers of children with severe disabilities were more likely than the general population to be not employed (47.2% against 31.4%). But in contrast, mothers of children with CP interviewed during a survey were not more unemployed than mothers in the general population; calling into question the use of CP as a model of disability to study family consequences.The study of the impact of disability is still too rare in Europe and the effects of possible interventions little known. It is important to develop research in this area to improve the lives of these families and provide children with disabilities the best possible environment for its development.The study of the impact of disability is too rare in Europe and little is known on the effects of possible interventions. It is important to develop research in this area to improve the life for these families and to provide for disabled children the best possible environment for its development.

<p>Over 20 different plasma proteins have been shown to have the capacity to undergo conformational changes and self-assemble into highly stable and insoluble amyloid fibrils. </p><p>One, transthyretin (TTR), consists of 127 amino acid residues arranged in eight β-strands (named A to H) and is involved in two different clinical forms of amyloidosis. In familial amyloidotic polyneuropathy (FAP), mutated TTR is found in the amyloid deposits while in senile systemic amyloidosis (SSA) only wild type TTR is present in the amyloid deposits.</p><p>In this study, we have identified a novel form of amyloidosis that is caused by the deposition of an N-terminal fragment of apolipoprotein A-IV (apoA-IV). Interestingly, apoA-IV amyloid was found deposited in a patient that also suffered from SSA. Thus, this patient had two biochemically distinct and concurrent forms of amyloidosis that were derived from apoA-IV and TTR. </p><p>We have also discovered that two different morphological deposition patterns (identified as patterns A and B) exist in TTR-derived amyloidosis. Pattern A, observed in all SSA patients studied and in half of the FAP patients examined contained large homogenous deposits that were composed of short randomly oriented fibrils. In contrast, pattern B was observed in the remaining FAP patients and was represented by smaller-sized deposits that consisted of longer fibrils that were arranged in parallel bundles. The predominant TTR component deposited also differed between the two amyloid patterns. Amyloid pattern A contained mainly C-terminal TTR fragments while pattern B amyloid consisted of full-length TTR. Our findings suggest that two different mechanisms of fibril formation may exist in TTR-derived amyloidosis. </p><p>We have found two epitopes, corresponding to strand C and H that are surface-exposed in TTR-derived amyloid fibrils but hidden and part of the hydrophobic core in the native molecular structure. This indicates that TTR undergoes partial unfolding during fibril formation. </p>

Over 20 different plasma proteins have been shown to have the capacity to undergo conformational changes and self-assemble into highly stable and insoluble amyloid fibrils. One, transthyretin (TTR), consists of 127 amino acid residues arranged in eight β-strands (named A to H) and is involved in two different clinical forms of amyloidosis. In familial amyloidotic polyneuropathy (FAP), mutated TTR is found in the amyloid deposits while in senile systemic amyloidosis (SSA) only wild type TTR is present in the amyloid deposits. In this study, we have identified a novel form of amyloidosis that is caused by the deposition of an N-terminal fragment of apolipoprotein A-IV (apoA-IV). Interestingly, apoA-IV amyloid was found deposited in a patient that also suffered from SSA. Thus, this patient had two biochemically distinct and concurrent forms of amyloidosis that were derived from apoA-IV and TTR. We have also discovered that two different morphological deposition patterns (identified as patterns A and B) exist in TTR-derived amyloidosis. Pattern A, observed in all SSA patients studied and in half of the FAP patients examined contained large homogenous deposits that were composed of short randomly oriented fibrils. In contrast, pattern B was observed in the remaining FAP patients and was represented by smaller-sized deposits that consisted of longer fibrils that were arranged in parallel bundles. The predominant TTR component deposited also differed between the two amyloid patterns. Amyloid pattern A contained mainly C-terminal TTR fragments while pattern B amyloid consisted of full-length TTR. Our findings suggest that two different mechanisms of fibril formation may exist in TTR-derived amyloidosis. We have found two epitopes, corresponding to strand C and H that are surface-exposed in TTR-derived amyloid fibrils but hidden and part of the hydrophobic core in the native molecular structure. This indicates that TTR undergoes partial unfolding during fibril formation.

The familial aggregation of Major Depressive Disorder (MDD) has been repeatedly demonstrated. Several studies have investigated associations between various clinical characteristics of MDD in probands and overall rates of MDD in relatives. Few studies, however, have considered the familial aggregation of clinical characteristics of MDD. The aim of the present report is to examine mother–offspring associations of a variety of clinical characteristics of MDD in a general population sample. Data were derived from baseline and 4-year-follow-up data of 933 adolescents and their biological mothers of the Early Developmental Stages of Psychopathology (EDSP) study, a prospective-longitudinal community study. MDD and its characteristics were assessed with the Munich-Composite International Diagnostic Interview. We found that children of mothers who had a lifetime history of severe MDD and high number of symptoms, high impairment and/or melancholia, revealed elevated odds of MDD regarding the same characteristics as their mothers (ORs between 5.2 and 13.9). The observed associations did not differ by the children’s sex. DSM-IV melancholia and severity as well as impairment were found to aggregate within families. This finding can be interpreted as a validation of the DSM-IV MDD severity subtypes as well as of the melancholic specifier. Severe and melancholic MDD reveal a considerable high degree of familiar aggregation making the search for mechanisms involved in the familiar transmission of these forms of MDD particularly promising.

BAKGRUND: Psykisk ohälsa bland unga kvinnor är ett folkhälsoproblem. Anorexia nervosa kategoriseras in som en psykisk sjukdom. Anorexia nervosa drabbar främst unga kvinnor, vilket exempelvis kan bero på tidigare händelser i livet som gett upphov till psykisk obalans i deras vardag. SYFTE: Syftet med studien var att undersöka vilka sociokulturella orsaker som bidrar till utvecklingen av Anorexia nervosa bland flickor och unga kvinnor i åldrarna 12-25 år. METOD: En literaturstudie utfördes, som baseras på 21 olika vetenskapliga artiklar. Databaserna som användes var pubmed och psycinfo. Temaanalys användes och artiklarna kategoriserades in i huvudteman och underteman. RESULTAT: Resultatet i studien delades in i två huvudkategorier, med två underkategorier vardera. Sociala orsaker till uppkomsten av Anorexia nervosa: skola och ungdomskultur, samt bristande familjerelationer. Mediala orsaker till uppkomsten av Anorexia nervosa: exponering av unga kvinnors idealkroppar och negativ inspiration på internet. Olika orsaker bidrar till utvecklingen av Anorexia nervosa. Sociokulturella orsaker, mediala orsaker, familjära orsaker och kulturella orsaker samspelar och bidrar tillsammans till en utveckling av sjukdomen. IMPLIKATION: Exempelvis kan man studera de olika orsakerna på en mer specifik nivå och studera varje enskild orsak för sig. Studien kan också användas som en form av inspiration som stärker betydelsen av att hälsofrämjande åtgärder för psykisk ohälsa sätts i fokus. / BACKGROUND: Mental illness among young woman is a public health problem. Anorexia nervosa is categorized as a mental illness. Young woman affected by Anorexia nervosa, wich for example may be due to earlier life events that gave rise to mental imbalance in their everyday lives. OBJECTIVE: The aim of this study was to describe the socio-cultural reasons that contribute to the development of Anorexia nervosa among girls and young women 12-25 years. METHOD: The study is a literature review, based on 21 differens aricles. The method has been to look up earlier research in the area through various databases on the internet. Databases that have been used is pubmed and psycinfo. The articles were divided into different themes, with main category and subcategory. RESULTS: The results of the study was divided into two main categories each with two subcategories. Social causes for the occurrence of Anorexia nervosa: School and youth culture and the second was lack of family relationship. Media reasons in the emergence of Anorexia nervosa: Media image of the feminine ideal and negative inspiration on the internet. Socio-cultural reasons, medial reasons, family reasons, and cultural reasons. IMPLICATION: For example, one can study the various reason in a more specific level, and studying each factors separately. The study can also be used as a form of inspiration that reinforces the importance of health promotion activities for mental illness is the focus.

It has been proved from several studies that the genetic influence has been the most significant factor for having preeclampsia (PE). Still there are many uncertainties about origin and magnitude of the genetic effects as no specific inheritance patterns have been established. In this study, heritage risk of PE is in both the woman’s family and her partner’s family to her risk of PE is examined, along women and men own history with same and different partners. Moreover it is also examined whether timing of onset of PE is also has any impact on familial clustering of PE. Here, we used the population based Danish birth and multi generation registers to identify a cohort of women who have given birth during 1978 to 2008; which consisted of 1,79,69,28 singleton deliveries. This information is linked with pedigree information from the Danish Family Relation Database to define both maternal and paternal relationships. Risk ratios were estimated comparing women with and without various PE histories. It is found that the recurrence risk of a woman suffering from PE is 12.4 with 95% confidence limits (11.9, 12.8). Woman's recurrence risk diminishes only slightly when she changes partner means that particularly maternal genetic factors play the largest role, compared to male partner whose recurrence risk almost diminishes if he changes his female partner. Women and men from families with PE contribute to risk of PE in pregnancies they are involved in. The woman’s family history is still more important compared to man family history of PE; except for increased rick in pregnancies fathered by men who were born to preeclamptic mothers.  The recurrence risk of a women suffering from PE, if she already has suffered from this condition before 34 weeks is found to be very high (RR=25.4 with 95% confidence limits (21.8, 29.1)) with same male partner. It is found that early-onset PE and later-onset varieties have a clear genetic component but the intensity of early onset is stronger than late onset varieties. There are both maternal and paternal genetic contributions to early-onset PE, with the maternal ones seeming to be stronger.

Basée sur une approche sociologique, cette recherche a pour objet l'analyse et la compréhension du parcours scolaire des élèves de Section d'Enseignement Général et Professionnel Adapté (SEGPA) à l'île de La Réunion. Nous nous sommes principalement intéressé aux élèves en fin de scolarité dans douze SEGPA. Notre approche en termes de processus nous permet de montrer que l'histoire et le parcours scolaires des élèves rencontrés ne se réduisent pas à une aventure individuelle, mais sont le résultat d'un processus global construit dont les dynamiques sont à chercher dans la combinaison et l'interaction complexes d'un grand nombre d'éléments, de phénomènes, d'événements. Ainsi, même s'il apparaît que le fonctionnement institutionnel de l'orientation influence fortement les destins scolaires, il ressort de cette recherche que ce qui rend possibles le parcours scolaire et les sorties sans qualification des élèves de SEGPA, n'est pas réductible aux seules caractéristiques personnelles des élèves, ni à celles de leur cadre familial de socialisation, et encore moins à ce qui se joue dans l'espace scolaire ; nous y voyons plutôt le produit d'un processus global dont les dynamiques interdépendantes se conjuguent, s'imbriquent, se cumulent et s'influencent.