El temblor en la fenilcetonuria es un trastorno del movimiento muy prevalente entre pacientes de diagnóstico precoz y tardío. Se trata de un temblor rápido y muy regular, localizado fundamentalmente en extremidades superiores, cuya amplitud aumenta con el movimiento sobre todo en los pacientes de diagnóstico tardío, en quienes puede causar una discapacidad para las tareas cotidianas. Los datos neurofisiológicos sugieren que el temblor está causado por una disfunción de circuitos neuronales a nivel del sistema nervioso central, pudiendo ser considerado como un indicador de daño cerebral. El temblor en la PKU se asocia a un descenso en las concentraciones plasmáticas de CoQ10 en los pacientes con un adecuado control metabólico, así como a una edad tardía de diagnóstico de la enfermedad. Debido a la asociación descrita entre el déficit de CoQ10 y el aumento del estrés oxidativo en la PKU, nuestros resultados sugieren que el déficit de coenzima Q10 podría actuar induciendo un daño oxidativo a nivel de los circuitos implicados en la fisiopatología del temblor en estos enfermos. Los pacientes fenilcetonúricos en tratamiento dietético presentan alteraciones significativas en el volumen de la sustancia blanca y sustancia gris, cuya aparición está relacionada con la duración y el adecuado cumplimiento del tratamiento dietético. Existe una distribución específica de esta pérdida de volumen a nivel de las áreas motoras corticales, ganglios basales y sustancia blanca periventricular, cuyo significado clínico deberá ser ampliado en futuros trabajos. En cuanto a las nuevas opciones terapéuticas en la PKU, la prueba de sobrecarga combinada con Phe/BH4 es una herramienta muy útil para la selección de los pacientes candidatos a iniciar un tratamiento con BH4. Considerando lo difícil que resulta predecir la respuesta a la BH4 a partir del genotipo, recomendamos realizar la prueba de sobrecarga a todos los pacientes PKU, exceptuando solo aquellos pacientes con mutaciones nulas en ambos alelos. Finalmente, consideramos que el tratamiento con BH4 es seguro y eficaz en un grupo seleccionado de pacientes PKU con fenotipos leves y moderados. La utilización de dosis bajas de BH4 permite liberalizar la dieta y retirar la fórmula especial, garantizando un desarrollo nutricional y cognitivo adecuados sin que se hayan observado efectos indeseados. En conclusión, pese a un adecuado control metabólico, los pacientes fenilcetonúricos en tratamiento dietético presentan signos clínicos de disfunción neurológica así como anomalías estructurales en el parénquima cerebral. En su origen están implicados múltiples factores; en primer lugar relacionados con el inicio, la duración y el adecuado cumplimiento dietético, pero también alteraciones metabólicas secundarias como el déficit de CoQ10 y el aumento del estrés oxidativo. El tratamiento con tetrahidrobiopterina representa una alternativa terapéutica a la dieta restrictiva en Phe en un grupo seleccionado de pacientes con fenotipos leves y moderados, en quienes permite optimizar el control metabólico y garantizar un adecuado desarrollo cognitivo y nutricional. / BRAIN INJURY IN PHENYLKETONURIA: PATHOPHYSIOLOGY AND NOVEL THERAPEUTIC APPROACH” Tremor is a common sign in phenylketonuric patients under dietary treatment. It is more frequent and severe among late-treated PKU patients. The cause of tremor is unknown, but neurophysiological data suggest that it is more dependen ton abnormalities in central nervous system neural networks than on peripheral circuits involving mechanical oscillations. If such is the case, tremor may be an index of cerebral damage in PKU patients. Tremor is associated with low concentrations of CoQ10 in patients under dietary treatment, potentially leading to increased oxidative stress damage in central neural networks. Treated patients may show significant gray and white matter volume changes related to the duration and strict observation of dietary treatment. Voxel-based maps revealed a significant gray and white matter reduction in motor and premotor cortex, thalamus and periventricular white matter. Further studies are needed to investigate whether the presence of neurologic symptoms may be explained by specific anatomic alterations. Regarding new therapeutic options in PKU, we found that Phe/BH4 loeading should be recommended for al PKU patients before starting BH4 therapy considering the difficulties in predicting a good response on the basis of patients genotype. Finally, our data confirm that BH4 is a safe and effective therapy in a selected group of mild and moderate PKU patients. Low doses of BH4 in monoteraphy allow for withdrawal of the special formula and guarantee a good clinical and nutritional outcome with no adverse side effects in PKU patients.

This thesis aims to explore the role and significance of the aesthetic experience in Kant&rsquo / s philosophy. To accomplish this aim / firstly, the role of aesthetic power of judgment is discovered in subject&rsquo / s production of truths about the sensible world which is attributed to the cognitive power of understanding. Secondly, the role of aesthetic power of judgment in subject&rsquo / s representation of the good and in formation of moral judgments is demonstrated. Aesthetic power of judgment which enables both the reception and production of the beauty as a necessary harmony and unity brings an aesthetic and intuitive determinability to the acknowledged transcendent field for knowledge. The thesis is concluded by the affirmation that aesthetic power of judgment as an orienting interpretative power is a necessary condition for the subject, who is limited in knowledge and sensibly conditioned in the realization of moral purposes, in order to know and to have a moral life.

In this dissertation, focusing on the problem of &ldquo / aesthetic form&rdquo / and its relation to the distinction between natural and artistic beauty, it is argued that the Japanese philosopher Kitar

Photorealism was a movement which focused on meticulously reproducing the quotidian photograph on a large scale. Despite its popularity from between 1967-1977, it was often considered by critics to be vacuous and anachronistic. This was due to several factors, most significant of which were its focus on the outmoded practice of pictorial realism, the banality of its subjects and its apparent plagiaristic approach to the photograph-as-source. This neutral and cool aesthetic was misunderstood as superficial and served to render the movement the very antithesis of aesthetic creativity. This thesis seeks to reconsider such apparent objectivity in Photorealist painting by presenting a semiotic reading of Photorealist practice that will form the basis of the central argument in this thesis. This argument posits that there are evidential layers of signification embedded within the making of a Photorealist work which enact perceptual, conventional and historical conditions that deny the project of visual neutrality. This argument will prove that Photorealism was not a clear continuation of traditional pictorial realism, but a more conceptual movement which questioned conceptions of the phenomenological real. It will also show how, in Photorealism’s transmutation of the photographic aesthetic, it instigated the development of a hybrid visual language – a ‘route to meaning’ – which served to occupy the vacant space between the painting and the photograph. To reveal the development of such a language, and its inherently subjectivist foundations, I will inquire into the Photorealist painting from its very beginnings – from perceptual conception to conclusion – within the realm of the artist. The methodology for such an inquiry will involve the development and application of a heuristic model, informed by a semiotic approach, which will show how reality in Photorealism is mediated and transformed in the process of (re)-presenting the photograph. This model will categorise the key stages of activity and influence in the Photorealist process, showing at each stage how visual language is engendered and compounded. By adopting such an approach this thesis will show that Photorealist paintings, contrary to much criticism, managed to establish a unique and significant manner of depicting the real that is now worthy of contemporary reappraisal.

Inductors and transformers are electrical devices critical to power conversion systems. The current-voltage (I-V) behavior of these devices is very nonlinear as a result of the magnetic cores used in their construction. However, in the design of these power conversion systems the present state of Spice-based models is limited to low frequency and room temperature effects. An addition to the present model found in many Spice type environments, Jiles-Atherton, is the subject of this work. Chapter 1 of this dissertation introduces the source of these nonlinearities as being a result of the relationship between the magnetic flux density, B, and the applied magnetic field, H. Chapter two then derives the original mathematical model used in the Spice, Jiles-Atherton, to provide a physical basis of the addition to the model. The original derivation as it can be found in the referenced literature shows that a temperature and frequency dependence on the model does not exist. This work will seek to add such a dependence on temperature first followed by frequency. Using this approach, the temperature dependence can be modeled without the core experiencing self-heating. The model therefore must be capable of being further modified after the temperature dependence is added.

Bakgrund: De flesta kvinnor oavsett bh-storlek, belastar via sin bh:s axelband nacke, axlar och dess muskulatur med bystens tyngd hela dagen, vilket skapar ett statiskt tryck under en längre tid utan vila. För att belastningsskador inte ska uppstå bör belastning begränsas i tid (AFS 1998:1). Syfte: Syftet med denna studie var att beskriva hur kvinnor i arbetsför ålder med diagnosen fibromyalgi upplever och hanterar smärtan i nacke och axlar utifrån bh-bandens statiska belastning, samt försöka förstå om och hur detta även påverkar arbetsförmågan. Metod: Med en kvalitativ forskningsansats som grund genomfördes en gruppdiskussion utifrån fokusgruppsintervjuns arbetssätt, samt två enskilda intervjuer där respondenterna fick delge sina erfarenheter och upplevelser inom ämnesområdet. Resultat: För tre av studiens fyra deltagare visade resultatet på att bh-bandens belastning hade betydelse för en utökad smärtupplevelse i nacke och axlar och att detta även påverkade arbetsförmågan negativt när deltagarna upplevde försämrad ork, koncentration och kraftlöshet. Slutsats: Bh-bandens statiska belastning kan ha negativ inverkan på smärta i nacke och axlar och för att i framtiden kunna underlätta hanteringen, minska belastningen och öka arbetsförmågan hos berörd målgrupp men även hos andra kvinnor med liknande problem, är information och vidare forskning inom området nödvändig. / <p>2013-06-04</p>

This thesis tries to distinguish kitsch and art with the help of philosophical hermeneutics by reading it in the light of Derrida&rsquo / s interpretation of Hamlet in Specters of Marx. It defines kitsch as experience in order to go beyond both of the two main approaches, namely, the one that sees kitsch as an object and the one that reduces it to the behaviour of the subject. It tries to show that kitsch is not just an aesthetic problem, but also, a political one.

Litteratur visar på att bysthållare (BH) är ett mycket komplext plagg och endast småjusteringar kan förändra passformen. I denna studie har BH konstruerats med syfte attundersöka vad olika avskärningar över kupan gör med passformen. På modeföretaget därstudien utförts fanns det ett önskemål om bra BH-grunder som underlag för att undvika attleverantören gör konstruktionerna, då måttlista och skiss inte anses som tillräckligt braunderlag att skicka. Genom att själva göra konstruktionerna anser sig företaget få merkontroll över BH:ns passform. Konstruktionerna i denna studie har utvecklats i CADprogramvaranModaris. Detta följt av uppsömnad och avprovning, där BH:arna testas påolika provmodeller med mått motsvarande företagets målgrupp. Under avprovningarna haravprovningsprotokoll förts, där frågor relaterade till litteraturen ställs till de medverkande.Det visar sig att passformen förändras när avskärningen gör det. Detta på grund av attfördelningen av vidden ändras, vilket innebär att kupan får en ny form. För att undvikapassformsproblem på företag kan en idé vara att utveckla ett flertal olika grundkonstruktionermed olika avskärningar, så att endast mindre justeringar krävs, och färre justeringar behövsgöras från grunden. / Literature shows that bras are a very complex piece of clothing and only small adjustmentscan change the fit. In this study, bras has been constructed with the purpose to examine whatdifferent cuts on the bras cup make with the fit. The fashion brand where this study has beenperformed, wanted a basic pattern for bras, since communication with vendors using onlysketches and measurement lists was not enough to get a good result. The patterns in thisstudy have been developed in CAD software Modaris. This followed by sewing and fittingsessions on fitting models with body measurement corresponding to the brands target group.During the fittings, a protocol related to literature is written. After analysing the fit of thebras, it came to show that shape is changed even though the width of the cup is the same.This is because the width is divided differently which gives the cup a new shape. To avoidfitting problems it could be a good idea that companies have more than one basic pattern forbras with different cuts to base their models on, to decrease the proportion of major fittingchanges.

This study analyses the invisible forms of repression in the Bolivian art system by interpreting Roberto Valcárcel’s artwork in the light of Herbert Marcuse’s ideas on repression and liberation as expounded in Eros and Civilization. It considers, on the one hand, Valcárcel’s artwork in relation to the liberating role that Marcuse attributes to art (via phantasy, polymorphous eroticism, and Orphic paradigm). On the other hand, it explores the strategies devised by Valcárcel against repression, such as self-promotion, multiple texts, play, humour and unmasking certain repressive truths. The reading of Valcárcel’s work via Marcuse is supported by archival research from contemporary newspapers, exhibition documentation and Bolivian art history, which provide relevant information about the sorts of latent repression to which Valcárcel’s artworks responds. The dissertation is organised in five chapters in which examples of repressive beliefs are unveiled. Chapter One examines El Movimiento Erótico (The Erotic Movement, 1983) and the manifold strategies used by Valcárcel to escape the traps of a presumed type of sexual liberation (sexist and genital oriented) and capitalism’s culture industry. Chapter Two discusses artworks where the intentional construction of open meaning challenges the norm of a univocal creation and consumption of art. Chapter Three studies some of Valcárcel’s humorous identities in contrast with the dramatic, and overly serious self-perception of Bolivians artists. Chapter Four explores Valcárcel’s use of play, black humour and deceit as effective devices to escape hidden authoritarianism in society during dictatorial regimes. Chapter Five analyses how Valcárcel’s work unveils the latent repression in the idealisation of indigenous heritage through play and anti-thesis. The dissertation introduces a new topic into the study of art in Bolivia – veiled repression – at the same time that it sheds light on the potential of the artwork of Roberto Valcárcel to open new ways of historicizing and thinking about art in Bolivia.

I denna studie är målet att arbeta fram en sport BH som ger extra stöd under sportaktivitet och samtidigt är bekväm. Företaget som har samarbetats med är en större sportkedja. De vill utöka sitt sortiment med en sport BH med en sydd kupa, där konstruktionen gör formen, då det endast i sortimentet finns sport BH i stretchtyg med formpressade kupor eller lösa vadderade kupor. En mjuk (utan bygel) BH grund har konstruerats och sytts upp och provats av i storlek 75C. Denna har sedan vidareutvecklats till en sport BH. Till hjälp för detta har gjorts en litteraturstudie om vad som viktigt för utformandet av en sport BH, och tillsammans med sportföretaget har modellen beslutats. Avprovningar på 3 personer har genomförts, först av BH grunden och sedan under produktutvecklingsfasen till en sport BH, för att säkerställa storlek och passform. Resultatet visar att vid produktutveckling från en mjuk BH till en sport BH, är det en del förändringar som måste göras. Några av dessa är bredare och vadderade axelband, plattare kupa för att stabilisera bysten och ökning av vidden för kupan. Samtidigt visar studien att BH grunden fungerade att utgå från för att konstruera en sport BH. / In this study, the goal is to make a sports bra that provides extra support during sports activity and at the same time is comfortable. The company that has collaborated with is a major sports company. They want to expand their range with a sports bra with a stitched cup, where the construction makes the shape, since only in the range is sports bra in stretch fabric with molded cups or loose padded cups. A soft (wireless) bra base has been constructed and sewn and tested for size 75C. This has been further developed into a sports bra. For knowledge, a literature study has been made, to find out what is important for the design of a sports bra, and together with the sports company, the model has been decided. Fitting of 3 people have been completed, first of the BH foundation and then during the product development phase of a sports bra, to ensure size and fit. The result shows that during product development from a soft bra to a sports bra, there are some changes that must be made. Some of these are wider and padded shoulder straps, flatter cup to stabilize the bust and increase the width for the cup. At the same time, the study shows that the BH foundation worked to start from, to make a construction for a sports bra.