241 resultados para Semmler, Willi
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Kirjallisuusarvostelu
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Esta disertación busca estudiar los mecanismos de transmisión que vinculan el comportamiento de agentes y firmas con las asimetrías presentes en los ciclos económicos. Para lograr esto, se construyeron tres modelos DSGE. El en primer capítulo, el supuesto de función cuadrática simétrica de ajuste de la inversión fue removido, y el modelo canónico RBC fue reformulado suponiendo que des-invertir es más costoso que invertir una unidad de capital físico. En el segundo capítulo, la contribución más importante de esta disertación es presentada: la construcción de una función de utilidad general que anida aversión a la pérdida, aversión al riesgo y formación de hábitos, por medio de una función de transición suave. La razón para hacerlo así es el hecho de que los individuos son aversos a la pérdidad en recesiones, y son aversos al riesgo en auges. En el tercer capítulo, las asimetrías en los ciclos económicos son analizadas junto con ajuste asimétrico en precios y salarios en un contexto neokeynesiano, con el fin de encontrar una explicación teórica de la bien documentada asimetría presente en la Curva de Phillips.
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Prader-Willi syndrome (PWS) was originally described less than 50 y ago,1 although reference to children with characteristics of the syndrome are to be found in other literature previous to this.2 Until relatively recently the diagnosis was made upon the clinical features as outlined by Holm,3 which include severe muscular hypotonia in the neonatal period leading to feeding difficulties and undernutrition, hypogonadism and later hyperphagia and obesity. Latterly the syndrome has been identified as being associated with an interstitial deletion of the q11-13 region on chromosome 15.4 In the majority of cases the deletion is in the paternally derived chromosome. In the remainder of cases there seems to be a failure to inherit the entire paternal chromosome and as a consequence both the chromosomes inherited are maternal, thus leading to maternal disomy.
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Four cases-of congenital dysfunction of the major salivary glands as well as of Prader-Willi, congenital rubella, and Sjogren's syndromes-were identified in a series of 500 patients referred for excessive tooth wear. Although there was evidence of consumption of highly acidic drinks, some occlusal parafunction, and unacceptable toothbrushing habits, salivary dysfunction was the salient factor predisposing a patient to tooth wear in these syndromal cases. The 500 subjects have been characterized either as having medical conditions and medications that predispose them to xerostomia or lifestyles in which workplace- and sports-related dehydration lead to reduced salivary flow. Normal salivation, by buffering capacity, clearance by swallowing, pellicle formation, and capacity for remineralization of demineralized enamel, protects the teeth from extrinsic and intrinsic acids that initiate dental erosion. Thus, the syndromes, unrelated in many respects, underline the importance of normal salivation in the protection of teeth against tooth wear by erosion, attrition, and abrasion.
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A síndrome de Prader-Willi tem uma prevalência aproximada de 1:25000 nascimentos. No período neonatal há hipotonia severa, atraso de crescimento e dificuldade alimentar que persistem durante o primeiro ano de vida. O quadro clínico inicial contrasta com a bulimia que se evidencia mais tarde e que, não controlada, pode conduzir à obesidade mórbida. Descrevem-se as características clínicas, o diagnóstico genético e os cuidados específicos a ter na promoção da saúde a propósito de cinco crianças com síndrome de Prader Willi, cujo diagnóstico foi feito no período neonatal.
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OBJECTIVE: To compare the mechanical external work (Wext ) and pendular energy transduction (Rstep ) at spontaneous walking speed (Ss ) in individuals with Prader-Willi syndrome (PWS) versus subjects with nonsyndromal obesity (OB) to investigate whether the early onset of obesity allows PWS subjects to adopt energy conserving gait mechanics. DESIGN AND METHODS: Wext and Rstep were computed using kinematic data acquired by an optoelectronic system and compared in 15 PWS (BMI = 39.5 ± 1.8 kg m(-2) ; 26.7 ± 1.5 year) and 15 OB (BMI = 39.3 ± 1.0 kg m(-2) ; 28.7 ± 1.9 year) adults matched for gender, age and BMI and walking at Ss . RESULTS: Ss was significantly lower in PWS (0.98 ± 0.03 m s(-1) ) than in OB (1.20 ± 0.02 m s(-1) ; P < 0.001). There were no significant differences in Wext per kilogram between groups (PWS: 0.37 ± 0.04 J kg(-1) m(-1) ; OB: 0.40 ± 0.05 J kg(-1) m(-1) ; P = 0.66) and in Rstep (PWS: 69.9 ± 2.9%; OB: 67.7 ± 2.4%; P = 0.56). However, Rstep normalized to Froude number (Rstep /Fr) was significantly greater in PWS (6.0 ± 0.6) than in OB (3.8 ± 0.2; P = 0.001). Moreover, Rstep /Fr was inversely correlated with age of obesity onset (r = -0.49; P = 0.006) and positively correlated with obesity duration (r = 0.38; P = 0.036). CONCLUSION: Individuals with PWS seem to alter their gait to improve pendular energy transduction as a result of precocious and chronic adaptation to loading.
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Puhe
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OBJECTIVE: To explore how foot growth relates to musculoskeletal loading in children with Prader-Willi syndrome (PWS). STUDY DESIGN: In 37 children with PWS, foot length (FL) before and after 6 years of growth hormone therapy (GHT) was retrospectively evaluated with parental and sibling's FL, height, and factors reflecting musculoskeletal loading, such as weight for height (WfH), lean body mass (LBM; dual energy X-ray absorptiometry, deuterium labeled water), physical activity (accellerometry), and walk age. Because of the typically biphasic evolution of body mass and the late walk age in PWS, 2 age groups were separated (group 1, >2.5 years; group 2, < or =2.5 years). RESULTS: Children with PWS normalized height, but not FL after 6 years of GHT. Parental FL correlation with PWS's FL was lower than with sibling's FL. In group 1, FL positively correlated with WfH, LBM, and physical activity. In group 2, FL negatively correlated with age at onset of independent ambulation. Foot catch-up growth with GHT was slower in group 2 compared with group 1. CONCLUSION: In PWS, FL is positively associated with musculoskeletal loading. Small feet in children with PWS before and during long-term GHT may be more than just another dysmorphic feature, but may possibly reflect decreased musculoskeletal loading influencing foot growth and genetic and endocrine factors.
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On 10 February 1927, the "First International Congress against Imperialism and Colonialism" in Brussels marked the establishment of the anti-imperialist organisation, the League against Imperialism and for National Independence (LAI, 1927-37). The complex preparations for the congress were though initiated already in 1925 by Willi Münzenberg, a German communist and General Secretary of the communist mass organisation, Internationale Arbeiterhilfe (IAH, 1921-35), together with the Communist International (Comintern, 1919-43). Berlin was the centre for the LAI and its International Secretariat (1927-33), a city serving the intentions of the communists to find colonial émigré activists in the Weimar capital, acting as representatives for the anti-colonial movement in Europe after the Great War. With the ascendancy to power of the Nazi Party (NSDAP) on 30 January 1933, the LAI reached an abrupt, but nonetheless, expected end in Berlin. This doctoral thesis examines the role, pu rpose and functions of a sympathising communist organization (LAI): to act as an intermediary for the Comintern to the colonies. The analysis evaluates the structure and activities of the LAI, and by doing so, establish a complex understanding on one of the most influential communist organisations during the interwar period, which, despite its short existence, assumed a nostalgic reference and historical bond for anti-colonial movements during the transition from colonialism to post-colonialism after the Second World War, e.g. the Afro-Asian Conference in Bandung, Indonesia in 1955. Fredrik Petersson’s study, based on archives in Moscow, Berlin, Amsterdam, London, and Stockholm, uncovers why the Comintern established and supported the LAI and its anti-imperialist agenda, disclosing a complicated undertaking, characterised by conflict and the internal struggle for power, involving structural constraints and individual ambitions defined by communist ideology and strategy.
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The aim of the study is to write the first comprehensive history of the Internationale Arbeiterhilfe (International Workers’ Relief) and its message of international solidarity during the Weimar Republic, 1921–1933. The Arbeiterhilfe was the Communist International’s (Comintern) primary international solidarity organisation of the time. The work is identified as a contribution to the transnational history of the interwar period as its main focus is not on governmental politics or intra-state relations, but is focused on the transnational world of an international organisation. The history of the Arbeiterhilfe provides the main springboard from which to write a contextually-based analysis of international solidarity during the Weimar Republic. The study highlights for the first time the importance of the German communist Willi Münzenberg (1889–1940), as the leader of the Arbeiterhilfe, in the history of international solidarity. The main question of this study is how an explicit use of language coupled with the visualisation and practices of solidarity were created through the Arbeiterhilfe. How was solidarity actually envisaged, organised and brought to life by the Arbeiterhilfe in Weimar Germany? How did its expressions of solidarity change over time? Throughout the thesis, the changing and complex character of solidarity is analysed. How was the Arbeiterhilfe’s message of solidarity created and changed in relation to the Comintern and the Soviet Union’s policies? How did the Arbeiterhilfe create a new culture of international solidarity thought film, cinema, illustrated newspapers and the organising of mass spectacles of international solidarity? The Arbeiterhilfe had its international headquarters in Berlin which functioned as the base, one could argue, for some of the inter-war period’s most spectacular solidarity campaigns. The Arbeiterhilfe constitutes a significant case study of an early international organisation as it was one of the first international organisations for global (albeit not universal) international solidarity which had unparalleled prospects to develop new transnational identifications and social ties. It could consequently be suggested that the Arbeiterhilfe in several ways could be perceived as a predecessor to several post-1945 transnational solidarity organisations and International Non-Governmental Organisations (INGOs).
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Monosomy 1p36 is the most common subtelomeric microdeletion syndrome with an incidence rate estimated to be 1 in 5000 births. A hypothesis of a similarity between patients with 1p36 deletion and those with Prader-Willi syndrome and the existence of two different phenotypes for 1p36 microdeletion has been suggested. The main objective of the present study was to determine the existence of 1p36 microdeletion in a sample of patients with mental retardation, obesity and hyperphagia who tested negative by the methylation test for Prader-Willi syndrome. Sixteen patients (7 females, 9 males), 16-26 years old, were evaluated with high-resolution cytogenetic analysis at 550-850 band levels and with 11 polymorphic microsatellite markers located in the 1p36 region. All patients had normal cytogenetic and molecular results. The results obtained by high-resolution cytogenetic methodology were confirmed by the molecular analyses. We did not detect a 1p36 microdeletion in 16 subjects with the Prader-Willi-like phenotype, which reinforces that no correlation seems to exist between Prader-Willi-like phenotype and the 1p36 microdeletion syndrome.
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Se trata de una gu??a dirigida a familias y profesionales que ofrece informaci??n sobre las caracter??sticas de los menores afectados por el S??ndrome de Prader-Willi, una enfermedad poco conocida que afecta a 1 de cada 15.000 nacidos. Esta gu??a, elaborada conjuntamente por expertos y especialistas en la materia, analiza: los s??ntomas, el perfil de desarrollo, la importancia de la dieta en el crecimiento y maduraci??n, la conducta en general de los enfermos y sus necesidades en el ??mbito familiar, escolar y social.
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O objetivo deste estudo foi descrever o percurso e os resultados da terapia fonoaudiológica na síndrome de Prader-Willi, por meio do estudo longitudinal do caso de uma criança de 8 anos de idade, do gênero masculino, ao longo de quatro anos de terapia fonoaudiológica em uma clínica-escola. Foram realizadas filmagens de sessões de terapia e análise documental de informações dos prontuários referentes à anamnese, avaliação e relatórios terapêuticos fonoaudiológicos e avaliações multidisciplinares. A criança apresentou características típicas da síndrome de Prader-Willi como obesidade, hiperfagia, ansiedade, problemas de comportamento e auto-agressões. em avaliação fonoaudiológica foram observados hipotonia orofacial, sialorréia, voz hipernasal, alterações cognitivas, dificuldades de compreensão oral, comunicação por meio de gestos e produção de palavras isoladas ininteligíveis. Inicialmente, a terapia fonoaudiológica teve o objetivo principal de promover o desenvolvimento da linguagem com ênfase na interação social por meio de atividades lúdicas. Com a evolução do caso o direcionamento principal passou a ser o desenvolvimento de habilidades conversacionais e narrativas. Foram observadas evoluções quanto à manutenção da atenção, brincadeira simbólica, contato social e comportamento. Além disso, houve aumento do vocabulário, evolução quanto à compreensão oral e desenvolvimento de habilidades narrativas. Dessa maneira, a intervenção fonoaudiológica em caso de síndrome de Prader-Willi foi eficaz em diferentes níveis, no que se refere às habilidades fonológicas, sintáticas, lexicais e pragmáticas da linguagem
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The aim of this study was to describe the trajectory and the outcomes of speech-language therapy in Prader-Willi syndrome through a longitudinal study of the case of an 8 year-old boy, along four years of speech-language therapy follow-up. The therapy sessions were filmed and documental analysis of information from the child's records regarding anamnesis, evaluation and speech-language therapy reports and multidisciplinary evaluations were carried out. The child presented typical characteristics of Prader-Willi syndrome, such as obesity, hyperfagia, anxiety, behavioral problems and self aggression episodes. Speech-language pathology evaluation showed orofacial hypotony, sialorrhea, hypernasal voice, cognitive deficits, oral comprehension difficulties, communication using gestures and unintelligible isolated words. Initially, speech-language therapy had the aim to promote the language development emphasizing social interaction through recreational activities. With the evolution of the case, the main focus became the development of conversation and narrative abilities. It were observed improvements in attention, symbolic play, social contact and behavior. Moreover, there was an increase in vocabulary, and evolution in oral comprehension and the development of narrative abilities. Hence, speech-language pathology intervention in the case described was effective in different linguistic levels, regarding phonological, syntactic, lexical and pragmatic abilities.
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This investigation evaluates the possibility of constructing new ways of playing for a child with Prader-Willi syndrome, by means of occupational therapy. It is a qualitative study which makes use of the case study methodology, whose starting point is the clinical intervention as data collect field. It also presents a short revision of the literature to subside discussions and reflections. It was observed that through the playing experience the occupational therapist led the child to know his own limitations and possibilities, by making him discover new ways of doing activities. Observing the therapist and learning with her, the patient experienced different situations throughout the therapeutic relationship, what enabled him to experiment them in his everyday life. Finally, this study aims at showing the clinical reasoning of an occupational therapist with a view to demonstrate Brazilian therapeutical conduct.