Esta tese de doutorado trata de um estudo das políticas públicas de inclusão escolar no Rio Grande do Sul, de acordo com o atual cenário nacional de implantação de políticas de educação inclusiva a partir, principalmente, do ano de 2001. Este objetivo primeiro foi complementado pela construção de “mapas de sentidos” sobre este processo no Estado, apontando o “lugar” da modalidade de educação especial na construção de sistemas de ensino inclusivos, revelando aspectos sócio-histórico-políticos e educacionais constitutivos desta área. Esta conjugação de questões de pesquisa exigiu a construção de instrumentos e estratégias de investigação específicas que foram sistematizados através de coleta de dados em dez localidades (municípios), os quais foram considerados amostra dos comportamentos regionais a respeito do processo de inclusão escolar de alunos com deficiência e demais necessidades educacionais especiais no Estado. A sistematização dos dados coletados nas diferentes localidades proporcionou, de forma integrada à teoria, uma análise da atual configuração destas políticas, revelando um fenômeno de características multifacetadas, com diferentes “mapas de sentidos” sobre o assunto; a variação verificada fica por conta das diferenças regionais, sobretudo, das características culturais, das características educacionais e do “modelo” de educação especial vigente. A partir desta constatação, a pesquisa apontou que a viabilidade de construção de um adequado acompanhamento de implementação de políticas públicas de inclusão escolar, por tratar de um fenômeno complexo, exige uma matriz de interpretação que extrapole a simples questão técnica das avaliações. Neste sentido, um acompanhamento que favoreça um processo genuíno de compreensão das configurações locais indica a necessidade de se partir de uma perspectiva cultural e do entendimento da política como um processo de aprendizagem, adotando uma abordagem cognitiva das políticas públicas, perspectiva que se esforça por apreendê-las como matrizes cognitivo-normativas que constituem sistemas de interpretação do real, valorizando os valores, as idéias e as representações locais. O estudo aponta, por fim, um Estado que, apesar das diferentes interpretações verificadas, reúne-se em torno da modalidade de educação especial como “elemento” fundamental ao processo de inclusão escolar, ainda que a sua “localização” e o seu “sentido” na organização dos sistemas de ensino não seja unânime entre os municípios pesquisados. / This doctoral thesis deals with the study of the public policies of school inclusion in Rio Grande do Sul, in accordance with the current national scene of implementation of policies of inclusive education starting, mainly, from the year 2001. This first objective was complemented by the construction of “sense maps” on this process in the State, pointing the “place” of the modality of special education in the construction of inclusive systems of education, disclosing socialhistorical- political and educational aspects constituent of this area. This conjugation of research questions demanded the construction of instruments and specific strategies of investigation that had been systemized through collection of data in ten localities (counties), which had been considered sample of the regional behaviors regarding the process of school inclusion of students with deficiency and others special educational necessities in the State. The systematization of the data collected in the different localities provided, in an integrated way to the theory, an analysis of the current configuration of these policies, disclosing a multifaceted characteristics’ phenomenon, with different “sense maps” about the subject; the verified variation is at the expense of the regional differences, above all, of the cultural characteristics, the educational characteristics, and the valid “model” of special education. From this verification, the research showed that the construction viability of an adequate accompaniment of public policies implementation of school inclusion, by dealing with a complex phenomenon, demands a matrix interpretation that surpasses the simple technical evaluation questions. In this manner, an accompaniment that favors a genuine process of understanding of the local configurations indicates the necessity of starting it from a cultural perspective and from an understanding of the policies as a learning process, adopting a cognitive approach of the public policies, perspective that struggles to apprehend them as first cognitive-normative matrixes that constitute systems of interpretation of what is real, valuing the local values, ideas and representations. The study finally shows, a State that, despite the different verified interpretations, is congregated around the modality of special education as fundamental “element” to the process of school inclusion, despite its “localization” and its “orientation” in the organization of the education systems is not unanimous among the searched counties.

Esta tese de doutorado trata de um estudo das políticas públicas de inclusão escolar no Rio Grande do Sul, de acordo com o atual cenário nacional de implantação de políticas de educação inclusiva a partir, principalmente, do ano de 2001. Este objetivo primeiro foi complementado pela construção de “mapas de sentidos” sobre este processo no Estado, apontando o “lugar” da modalidade de educação especial na construção de sistemas de ensino inclusivos, revelando aspectos sócio-histórico-políticos e educacionais constitutivos desta área. Esta conjugação de questões de pesquisa exigiu a construção de instrumentos e estratégias de investigação específicas que foram sistematizados através de coleta de dados em dez localidades (municípios), os quais foram considerados amostra dos comportamentos regionais a respeito do processo de inclusão escolar de alunos com deficiência e demais necessidades educacionais especiais no Estado. A sistematização dos dados coletados nas diferentes localidades proporcionou, de forma integrada à teoria, uma análise da atual configuração destas políticas, revelando um fenômeno de características multifacetadas, com diferentes “mapas de sentidos” sobre o assunto; a variação verificada fica por conta das diferenças regionais, sobretudo, das características culturais, das características educacionais e do “modelo” de educação especial vigente. A partir desta constatação, a pesquisa apontou que a viabilidade de construção de um adequado acompanhamento de implementação de políticas públicas de inclusão escolar, por tratar de um fenômeno complexo, exige uma matriz de interpretação que extrapole a simples questão técnica das avaliações. Neste sentido, um acompanhamento que favoreça um processo genuíno de compreensão das configurações locais indica a necessidade de se partir de uma perspectiva cultural e do entendimento da política como um processo de aprendizagem, adotando uma abordagem cognitiva das políticas públicas, perspectiva que se esforça por apreendê-las como matrizes cognitivo-normativas que constituem sistemas de interpretação do real, valorizando os valores, as idéias e as representações locais. O estudo aponta, por fim, um Estado que, apesar das diferentes interpretações verificadas, reúne-se em torno da modalidade de educação especial como “elemento” fundamental ao processo de inclusão escolar, ainda que a sua “localização” e o seu “sentido” na organização dos sistemas de ensino não seja unânime entre os municípios pesquisados. / This doctoral thesis deals with the study of the public policies of school inclusion in Rio Grande do Sul, in accordance with the current national scene of implementation of policies of inclusive education starting, mainly, from the year 2001. This first objective was complemented by the construction of “sense maps” on this process in the State, pointing the “place” of the modality of special education in the construction of inclusive systems of education, disclosing socialhistorical- political and educational aspects constituent of this area. This conjugation of research questions demanded the construction of instruments and specific strategies of investigation that had been systemized through collection of data in ten localities (counties), which had been considered sample of the regional behaviors regarding the process of school inclusion of students with deficiency and others special educational necessities in the State. The systematization of the data collected in the different localities provided, in an integrated way to the theory, an analysis of the current configuration of these policies, disclosing a multifaceted characteristics’ phenomenon, with different “sense maps” about the subject; the verified variation is at the expense of the regional differences, above all, of the cultural characteristics, the educational characteristics, and the valid “model” of special education. From this verification, the research showed that the construction viability of an adequate accompaniment of public policies implementation of school inclusion, by dealing with a complex phenomenon, demands a matrix interpretation that surpasses the simple technical evaluation questions. In this manner, an accompaniment that favors a genuine process of understanding of the local configurations indicates the necessity of starting it from a cultural perspective and from an understanding of the policies as a learning process, adopting a cognitive approach of the public policies, perspective that struggles to apprehend them as first cognitive-normative matrixes that constitute systems of interpretation of what is real, valuing the local values, ideas and representations. The study finally shows, a State that, despite the different verified interpretations, is congregated around the modality of special education as fundamental “element” to the process of school inclusion, despite its “localization” and its “orientation” in the organization of the education systems is not unanimous among the searched counties.

Esta tese de doutorado trata de um estudo das políticas públicas de inclusão escolar no Rio Grande do Sul, de acordo com o atual cenário nacional de implantação de políticas de educação inclusiva a partir, principalmente, do ano de 2001. Este objetivo primeiro foi complementado pela construção de “mapas de sentidos” sobre este processo no Estado, apontando o “lugar” da modalidade de educação especial na construção de sistemas de ensino inclusivos, revelando aspectos sócio-histórico-políticos e educacionais constitutivos desta área. Esta conjugação de questões de pesquisa exigiu a construção de instrumentos e estratégias de investigação específicas que foram sistematizados através de coleta de dados em dez localidades (municípios), os quais foram considerados amostra dos comportamentos regionais a respeito do processo de inclusão escolar de alunos com deficiência e demais necessidades educacionais especiais no Estado. A sistematização dos dados coletados nas diferentes localidades proporcionou, de forma integrada à teoria, uma análise da atual configuração destas políticas, revelando um fenômeno de características multifacetadas, com diferentes “mapas de sentidos” sobre o assunto; a variação verificada fica por conta das diferenças regionais, sobretudo, das características culturais, das características educacionais e do “modelo” de educação especial vigente. A partir desta constatação, a pesquisa apontou que a viabilidade de construção de um adequado acompanhamento de implementação de políticas públicas de inclusão escolar, por tratar de um fenômeno complexo, exige uma matriz de interpretação que extrapole a simples questão técnica das avaliações. Neste sentido, um acompanhamento que favoreça um processo genuíno de compreensão das configurações locais indica a necessidade de se partir de uma perspectiva cultural e do entendimento da política como um processo de aprendizagem, adotando uma abordagem cognitiva das políticas públicas, perspectiva que se esforça por apreendê-las como matrizes cognitivo-normativas que constituem sistemas de interpretação do real, valorizando os valores, as idéias e as representações locais. O estudo aponta, por fim, um Estado que, apesar das diferentes interpretações verificadas, reúne-se em torno da modalidade de educação especial como “elemento” fundamental ao processo de inclusão escolar, ainda que a sua “localização” e o seu “sentido” na organização dos sistemas de ensino não seja unânime entre os municípios pesquisados. / This doctoral thesis deals with the study of the public policies of school inclusion in Rio Grande do Sul, in accordance with the current national scene of implementation of policies of inclusive education starting, mainly, from the year 2001. This first objective was complemented by the construction of “sense maps” on this process in the State, pointing the “place” of the modality of special education in the construction of inclusive systems of education, disclosing socialhistorical- political and educational aspects constituent of this area. This conjugation of research questions demanded the construction of instruments and specific strategies of investigation that had been systemized through collection of data in ten localities (counties), which had been considered sample of the regional behaviors regarding the process of school inclusion of students with deficiency and others special educational necessities in the State. The systematization of the data collected in the different localities provided, in an integrated way to the theory, an analysis of the current configuration of these policies, disclosing a multifaceted characteristics’ phenomenon, with different “sense maps” about the subject; the verified variation is at the expense of the regional differences, above all, of the cultural characteristics, the educational characteristics, and the valid “model” of special education. From this verification, the research showed that the construction viability of an adequate accompaniment of public policies implementation of school inclusion, by dealing with a complex phenomenon, demands a matrix interpretation that surpasses the simple technical evaluation questions. In this manner, an accompaniment that favors a genuine process of understanding of the local configurations indicates the necessity of starting it from a cultural perspective and from an understanding of the policies as a learning process, adopting a cognitive approach of the public policies, perspective that struggles to apprehend them as first cognitive-normative matrixes that constitute systems of interpretation of what is real, valuing the local values, ideas and representations. The study finally shows, a State that, despite the different verified interpretations, is congregated around the modality of special education as fundamental “element” to the process of school inclusion, despite its “localization” and its “orientation” in the organization of the education systems is not unanimous among the searched counties.

La douleur chronique est l’un des problèmes les plus complexes auxquels sont confrontées la médecine et les neurosciences. Au fil des siècles, il a été un casse-tête et reste encore un défi pour la recherche étant donné la complexité de sa nature. Parmi la quantité considérable de douleurs chroniques existantes, la douleur du membre fantôme est l’une de plus difficiles à traiter. Les études récentes montrent que les changements corticaux majeurs qui apparaissent après une amputation sont la conséquence de la douleur chronique fantôme. Je soutiens que la cause principale de la douleur fantôme est la non-acceptation de la perte d’une partie du corps, autrement dit, que la douleur mentale provoquée par la transformation de l’image de soi devient une douleur chronique physique, et que c’est l’esprit qui contrôle les réseaux cérébraux : bien qu’il émerge du cerveau, l’esprit arrive à le modifier du fait d’influences exogènes. L’analyse des études que j’ai réalisée pour tester mon hypothèse, m’a permis de constater que la perception douloureuse dépend de diverses influences externes qui sont indépendantes de l'entrée (signal) nociceptive. Je conclus que la plasticité corticale mise en évidence lors de l'expérience douloureuse chronique ne dépend pas seulement de l'action et de l'interaction entre les réseaux neuronaux dynamiques, mais aussi de la communication entre ces réseaux neuronaux (système endogène) et les réseaux environnementaux (système exogène), ces derniers étant capables de moduler la perception de la douleur. Je souligne donc l’importance de la reconnaissance du caractère mental de la douleur chronique et le besoin d’analyser sa dimension émotionnelle afin de la moduler. / Chronic pain is one of the most complex problems facing medicine and neuroscience. Over the centuries, it has been a puzzle and remains a research challenge given the complexity of its nature. Among the large number of existing different kinds of chronic pain, phantom limb pain is one of the most difficult to treat. Recent studies show that major cortical changes that appear after amputation are the result of chronic phantom limb pain. I argue that the main cause of phantom limb pain is the non-acceptance of the loss of a part of the body, that is to say, that the mental pain caused by the transformation of the self-image becomes a chronic physical pain. It is the mind that controls the cérébral networks : even if it emerges from the brain, the mind manages to modify it as a consequence of external influences. The analysis of the studies I performed to test my hypothesis, allowed me to confirm that the perception of pain depends on various external influences that are independent of the nociceptive signals. I conclude that the cortical plasticity highlighted during chronic painful experience does not only depend on the action and interaction between dynamic neural networks, but also on the communication between these neural networks (endogenous system) and environmental networks (exogenous system). These latter networks are capable of modulating the perception of pain. I therefore emphasize the importance of recognizing the mental nature of chronic pain and the need to analyze the emotional dimensions which modulate it.

Le maintien de l'intégrité du génome est essentiel pour la survie cellulaire et la propagation de l'information génétique. Une mauvaise prise en charge des dommages de l’ADN et / ou une aberration de la maintenance de l’intégrité des télomères - les extrémités des chromosomes linéaires - provoquent chez l'homme des pathologies associées à une instabilité génétique. Ainsi, des dysfonctions télomériques sont à l’origine de la Dyskératose Congénitale (DC), et de sa forme rare et sévère, le Syndrome de Hoyeraal-Hreidarsson (HHS). Les DC et HHS se caractérisent principalement par une insuffisance médullaire progressive, des défauts développementaux et une prédisposition à développer des cancers. Par ailleurs, de nombreux syndromes associant déficits immunitaires et anomalies développementales sont causés par des défauts de réparation de l'ADN (cas de déficits immunitaires sévères, de l’Anémie de Fanconi (FA), de l’ataxie télangiectasie (AT), etc …). Au cours de ce travail, nous avons réalisé une étude phénotypique et génétique de patients atteints de deux pathologies aux caractéristiques cliniques distinctes. Ce travail de thèse a permis : 1) d'une part d'identifier des mutations de RTEL1 chez 3 patients atteints de HHS, décrivant ainsi une nouvelle cause moléculaire de cette pathologie. L'analyse des cellules de ces patients a révélé le rôle crucial que joue RTEL1 sur la stabilité du génome et le maintien des télomères dans des cellules humaines. 2) d'autre part, d'identifier un défaut en MYSM1, une histone déubiquitinase, dans un nouveau syndrome immuno-hématologique associé à des défauts de réparation de l’ADN présentant certaines similitudes avec l'anémie de Fanconi. Cette étude démontre pour la première fois, qu'outre son rôle dans la régulation transcriptionnelle, MYSM1 participe également aux mécanismes de réparation des lésions de l'ADN. / Maintaining genome integrity is essential for cell survival and propagation of the genetic information. Improper management of DNA damages and / or aberrations in maintenance of telomere - the ends of linear chromosomes - causes humans disorders associated with genetic instability. Thus, in humans, telomere dysfunction causes Dyskeratosis Congenita (DC), and its rare and severe form, Hoyeraal-Hreidarsson Syndrome (HHS). DC and HHS are mainly characterized by progressive bone marrow failure, developmental defects and predisposition to cancer. In addition, many syndromes involving immunodeficiency and developmental abnormalities are caused by defects in DNA repair (e.g. severe immune deficiencies, Fanconi Anemia (FA), Ataxia Telangiectasia (AT),…). In this work, we performed a phenotypic and genetic study of patients with two syndromes presenting distinct clinical features. This work permitted : 1) on one hand, to identify RTEL1 mutations in patients with HHS and describe a new molecular cause of this disease. The analysis of patients’ cells revealed the crucial role for RTEL1 in genome stability and telomere maintenance in human cells. 2) on the other hand, to identify mutations in MYSM1, a histone deubiquitinase, in a new immuno-hematological syndrome associated with defects in DNA repair and sharing some similarities with Fanconi anemia. This study demonstrates for the first time that, in addition to its role in transcriptional regulation, MYSM1 is required to cope with DNA damages.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by a loss of voluntary movement over time, leading to paralysis and death. While 10% of ALS cases are inherited or familial (FALS), the majority of cases (90%) are sporadic (SALS) with unknown etiology. Approximately 20% of FALS cases are genetically linked to a mutation in the anti-oxidizing enzyme, superoxide dismutase (SOD1). SALS and FALS are clinically indistinguishable, suggesting a common pathogenic mechanism exists for both types. Since such a large number of genetic mutations in SOD1 result in FALS (>170), it is reasonable to suspect that non-genetic modifications to SOD1 induce structural perturbations that result in ALS pathology as well. In fact, misfolded SOD1 lacking any genetic mutation was identified in end stage spinal cord tissues of SALS patients using misfolded SOD1-specific antibodies. In addition, this misfolded WT SOD1 found in SALS tissue inhibits axonal transport in vitro, supporting the notion that misfolded WT SOD1 exhibits toxic properties like that of FALS-linked SOD1. Indeed, aberrant post-translational modifications, such as oxidation, cause WT SOD1 to mimic the toxic properties of FALS-linked mutant SOD1. Based on these data, I hypothesize that modified, misfolded forms of WT SOD1 contribute to SALS disease progression in a manner similar to FALS linked mutant SOD1 in FALS. The work presented in this dissertation supports this hypothesis. Specifically, one common misfolded form of SOD1 is defined and exposure of this toxic region is shown to enhance SOD1 toxicity. Preventing exposure, or perhaps stabilization, of this “toxic” region is a potential therapeutic target for a subset of both familial and sporadic ALS patients. Further, the possibility of exploiting this misfolded SOD1 species as a biomarker is explored. For example, an over-oxidized SOD1 species was identified in peripheral blood mononuclear cells (PBMCs) from SALS patients that is reduced in controls. Moreover, 2-dimensional gel electrophoresis revealed a more negatively charged species of SOD1 in PBMCs of healthy controls greatly reduced in SALS patients. This species is hypothesized to be involved in the degradation of SOD1, further implicating both misfolded SOD1 and altered protein homeostasis in ALS pathogenesis.

Cu, Zn superoxide dismutase (SOD1) is a dimeric, β-sandwich, metalloenzyme responsible for the dismutation of superoxide. Mutations covering nearly 50% of the amino acid sequence of SOD1 have been found to acquire a toxic gain-of-function leading to amyotrophic lateral sclerosis. A hallmark of this disease is the presence of insoluble aggregates containing SOD1 found in the brain and spinal cord. While it is unclear how these aggregates or smaller, precursor oligomeric species may be the source of the toxicity, mutations leading to increased populations of unstable, partially folded species along the folding pathway of SOD1 may be responsible for seeding and propagating aggregation. In an effort to determine the responsible species, we have systematically characterized the stability and folding kinetics of five well studied ALS variants: A4V, L38V, G93A, L106V and S134N. The effect of the amino acid substitutions was determined on a variety of different constructs characterizing the various post-translational maturation steps of SOD1: folding, disulfide bond formation and Zn binding. Zn was found to bind progressively tighter along the folding pathway of SOD1, minimizing populations of monomeric species. In contrast, ALS variants were found to have the greatest perturbation in the equilibrium populations of the folded and unfolded state for the most immature, disulfide-reduced metal-free SOD1. In this species, at physiological temperature, four out of five ALS variants were >50% unfolded. Finally the energetic barriers in the folding and unfolding reaction were studied to investigate the unusually slow folding of SOD1. These results reveal that both unfolding and refolding are dominated by enthalpic barriers which may be explained by the desolvation of the chain and provide insights into the role of sequence in governing the folding pathway and rate.

La vitamine D occupe de nombreuses fonctions dans la régulation de divers systèmes affectant le corps entier. Une déficience de cette hormone peut donc affecter plusieurs systèmes essentiels (ex. squelettique, dentaire, immunitaire) et peut laisser des lésions repérables sur le squelette et les dents. Au cours de la Révolution industrielle, plusieurs facteurs environnementaux ont probablement contribué à l’augmentation des cas de déficience en vitamine D. Ainsi, la majorité des études portant sur la déficience en vitamine D se sont concentrées sur des populations européennes du XIXe siècle. Au Canada, pour la même époque, la fréquence de ce type de maladie métabolique n’a pas été explorée en profondeur. De plus, les possibles interactions entre déficience en vitamine D et autres maladies ont été peu explorées jusqu’à maintenant. Afin de pallier cette lacune, la présente étude se concentre sur une population euroquébécoise (cimetière St-Antoine, Montréal (1799-1854)), explorant la fréquence et prévalence de diverses lésions et conditions pathologiques, en plus d’évaluer leur influence sur les catégories d’âge et de sexe. Plus précisément, cette recherche investigue le possible lien entre la présence de cas de déficience vitamine D durant l’enfance et la détérioration de l’état de santé plus tard au cours de la vie, en plus de l’évolution temporelle des cas au sein du cimetière St-Antoine en lien avec le phénomène d’industrialisation. Au total, 52 individus (8 non adultes, 44 adultes) issus du cimetière urbain St- Antoine à Montréal ont été étudiés. Les données collectées sont de nature empirique et ont été acquises selon les trois phases suivantes : i) l’inventaire squelettique de base (sexe, âge au décès), ii) les observations macroscopiques et radiographiques des lésions pathologiques pour chaque squelette; iii) et l’analyse histologique de tissus dentaires sélectionnés afin d’explorer la présence ou l’absence de dentine interglobulaire (DIG). Les résultats indiquent une proportion élevée de DIG durant l’enfance indiquant une déficience en vitamine D, c’est-à-dire 44% (23/52) à 75% (39/52), excluant et incluant le degré 1 de DIG, respectivement. La majorité de ces épisodes de déficience se sont produits à ou à partir de la naissance, allant jusqu’à trois ans (63%, n=33/52), et 12% des individus (n=6/52) présentent des épisodes prénataux de DIG, ce qui soulève la question de la déficience maternelle et de l’influence des facteurs culturels. La présence de DIG durant l’enfance n’est corrélée ni aux paramètres démographiques ni à une détérioration de l’état de santé plus tard au cours de la vie de l’individu. Un possible lien a été soulevé entre les degrés plus sévères de DIG et l’hypoplasie dentaire. Le nombre d’épisodes de DIG durant l’enfance semble diminuer avec le temps, contrairement à ce qui pourrait être attendu d’une population en voie d’industrialisation. Toutefois, ce résultat doit être considéré avec prudence en raison de la faible portion de l’échantillon provenant des aires plus récentes du cimetière. / Vitamin D has many functions in the regulation of various systems affecting the whole body. A deficiency of this hormone thus affects many essential systems (e.g. skeletal, dental, immune) and can leave visible lesions on the skeleton and teeth. During the Industrial Revolution, various environmental factors probably contributed to an increase in vitamin D deficiency. Therefore, most studies on vitamin D deficiency have been conducted on European skeletal samples dated to the 19th century. However, the frequency of this type of metabolic disease has not been explored extensively in Canada for the same period. Furthermore, in palaeopathology, the possible interactions between vitamin D deficiency and other diseases have only been poorly explored. To fill this gap, the present study focuses on an historic Euro-Quebecois population (St. Antoine cemetery, Montréal (1799-1854), exploring the frequency and prevalence of pathological lesions and conditions as well as their possible influence on the age and sex categories. More precisely, are investigated the possible link between the occurrence of vitamin D deficiency cases during infancy and health deterioration later in life, as well as the temporal evolution of the cases within the St. Antoine cemetery in relation to the industrialization process. In total, 52 individuals (8 nonadults, 44 adults) from the St. Antoine urban cemetery were used for the study. The data collected during the research is of an empiric nature and was acquired following three steps: i) the basic skeletal inventory (sex, age-at-death); ii) the macroscopic and radiographic observations of the pathological lesions on the skeletons; iii) and the histological examination of selected dental tissues to assess the presence of interglobular dentin (IGD). The results indicate a high prevalence of IGD during infancy indicative of vitamin D deficiency, i.e. 44% (23/52) to 75% (39/52), excluding and including the degree 1 of IGD, respectively. The majority of these episodes occurred at or from birth to up to three years-old (63%, n=33/52) and 12% of the individuals (n=6/52) presented prenatal IGD episodes, raising questions about maternal deficiencies and cultural influences. The presence of IGD during infancy was not correlated with demographic parameters, nor with health status deterioration later in life. A possible link was recorded between the presence of more severe degrees of IGD and LEH. The number of IGD cases during infancy seems to decrease with time, contrary to what might be expected of an industrializing population. However, this result must be taken with caution because of the small portion of the sample coming from the more recent parts of the cemetery.

(in English) This Master's Thesis is interested in the support of adoptive families in the Czech Republic. The aim of the thesis is to define possibilities of this support and stress the need to help people in the process of arranging adoption in the 21st century. The thesis describes the possibilities of supporting adoptive families in the chosen regions of the Czech Republic. Suggestions of different ways of helping and supporting adoptive families from experts are researched. The thesis is divided into two parts. The theoretical part of the thesis consists of four chapters. In the first chapter the term adopting is defined and different forms of this type of foster parenthood are introduced. This chapter also brings an overview of the historical development of adoption. The second chapter is interested in the specifics of adoption in the first 21st century, analyzes the needs of adopting families and stresses the importance of supporting the people involved in the different stages of the adoption process. The third chapter identifies the support of adopting families, and specifies the content, aims and the target group of the thesis. The last chapter is interested in describing different ways of supporting adoptive families in the Czech Republic and refers to places where adoptive families can...

The prevalence of Protein-Energy Malnutrition (PEM) in South Africa has been welldescribed in previous research studies and yet little is known about the nutritional status of toddlers in the Vosloorus Township. Using the research questions as the conceptual framework for the study, an exploratory descriptive survey was conducted to determine. • What factors give rise to malnutrition in the Vosloorus Township? • Why do toddlers on the PEM Programme fail to achieve their expected target weight? Data was collected by means of structured interviews from a sample of 50 mothers in the Vosloorus Township whose toddlers were on the PEM Programme. The fmdings indicated that the poor socio-economic conditions of the majority of households, especially unemployment, low levels of education and ignorance, contributed towards the development of malnutrition amongst toddlers and their failure to thrive on the PEM Programme. Recommendations to reduce levels of malnutrition and transform existing nutrition programmes were made. / Health Studies / M.A. (Nursing Science)