1,201 research outputs found

    The Musculoskeletal System in Pompe disease

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    The Musculoskeletal System in Pompe disease

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    Health and well-being in the private garden : examples of social sustainability in Rostorp yesterday, today and in the future

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    TrĂ€dgĂ„rden innebĂ€r en plats för Ă„terhĂ€mtning och platser för vĂ€lmĂ„ende vĂ€rderas högt. Behovet har ökat pĂ„ grund av samhĂ€llets restriktioner och nedstĂ€ngningar under Covid-19 pandemin. Dagens samhĂ€llsutmaningar med klimatförĂ€ndringar och debatten om förtĂ€tning i stĂ€der tillsammans med pandemins pĂ„verkan kan komma att förĂ€ndra vĂ„r hĂ€lsa och livskvalitet. Stadsplanerare uppmanas skapa gröna infrastrukturpolicys som gynnar hĂ€lsa, vĂ€lbefinnande och biologisk mĂ„ngfald. HĂ€r kan utformningen av Rostorps trĂ€dgĂ„rdar med dess restaurerande funktioner och biodiversitet belysas som en viktig resurs till diskussionen om social hĂ„llbar utveckling i stĂ€der. Rostorps egnahemsomrĂ„de i Malmö byggdes i början 1900-talet för att lösa dĂ„tidens samhĂ€llsproblem. Det var ett sĂ€tt att tillhandahĂ„lla mat, en hĂ€lsosam livsmiljö som ett sĂ€tt att förbĂ€ttra livsvillkoren för den vĂ€xande stadsbefolkningen. Syftet med detta examensarbete Ă€r att kunna planera för vĂ€lmĂ„ende, som t ex gynnad fysisk och mental hĂ€lsa, samt gemenskap, i framtidens privata trĂ€dgĂ„rdar och bostadsomrĂ„den. Resultaten frĂ„n intervjuer, formulĂ€r som tagits fram av en jubileumsgrupp och egen dokumenteringen av vĂ€xtmaterial i 8 trĂ€dgĂ„rdar har visat pĂ„ hög mĂ„ngfald och artrikedom av traditionella och gamla kulturvĂ€xter, vĂ€xter som gynnar pollinatörer, en viss grad av sjĂ€lvförsörjning och ett ökat trĂ€dgĂ„rds- och odlingsintresse i Rostorp. Resultaten visar Ă€ven pĂ„ en hög social hĂ„llbarhet med gemenskap och delaktighet i omrĂ„dets aktiviteter. Med 100 Ă„rs erfarenheter frĂ„n Rostorp tillsammans med material som dokumenterats i samband med 100 Ă„rs jubileet 2022 Ă€r studien en vĂ€rdefull resurs för framtidens stadsplanering och byggande.The garden means a place for recovery and places for well-being are highly valued. The need has increased due to society's restrictions and shutdowns during the Covid-19 pandemic. Today's societal challenges with climate change and the densification debate together with the impact of the pandemic may change our health and quality of life. Urban planners are encouraged to create green infrastructure policies that promote health, well-being and biodiversity. Here, the design of Rostorp's gardens with its restorative functions and biodiversity can be highlighted as an important resource for the discussion about socially sustainable development in cities. Rostorp's ‘own-home’ housing area in Malmö was built at the beginning of the 20th century to solve the social problems of the time. It was a way of providing food, a healthy living environment as a way of improving the living conditions of the growing urban population. The purpose of this thesis is to be able to plan for well-being, such as favored physical and mental health, as well as community, in the private gardens and residential areas of the future. The results from interviews, forms produced by an anniversary group and own documentation of plant material in 8 gardens have shown high diversity and species richness of traditional and old cultivated plants, plants that benefit pollinators, a certain degree of self-sufficiency and an increased interest in gardening and cultivation in Rostorp. The results also show a high social sustainability with community and participation in the area's activities. With 100 years of experience from Rostorp together with material documented in connection with the 100th anniversary in 2022, the study is a valuable resource for future urban planning and construction

    Superconductivity in ternary rare earth transition metal silicides and germanides with the Sc5Co4Si10-type structure

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    A systematic study of the superconducting and normal state properties of some ternary rare earth transition metal silicides and germanides of the Sc(,5)Co(,4)Si(,10)-type is reported in this work. Low temperature heat capacity measurements indicate the presence of a complicated phonon density of states in these structurally complex compounds. A better description of the phonon spectrum of the high T(,c) materials, Sc(,5)Rh(,4)Si(,10), Sc(,5)Ir(,4)Si(,10), and Y(,5)Os(,4)Ge(,10), given by a model proposed by Junod et al.(\u271), is presented and discussed. The large values of (DELTA)C/(gamma)(,n)T(,c) and the electron-phonon coupling constant for these high T(,c) compounds indicate that they are strong-coupled superconductors. Relative to other ternary superconductors, many of these materials have large Debye temperatures. The BSC theory does not seem to afford an adequate description of the supercon- ducting state in these compounds;DC electrical resistivity measurements on these compounds show resistivity behaviors deviating from those exhibited by simple metals. The (rho)(T) data for Y(,5)Ir(,4)Si(,10), Lu(,5)Rh(,4)Si(,10), Lu(,5)Ir(,4)Si(,10), and Y(,5)Os(,4)Ge(,10), indicate the presence of anomalies;Static molar magnetic susceptibility measurements performed on these compounds indicate (1) a small effective magnetic moment of 0.26(mu)(,B) on the Co atom and (2) anomalous behaviors in the Lu(,5)Rh(,4)Si(,10), Lu(,5)Ir(,4)Si(,10), Y(,5)Ir(,4)Si(,10), Lu(,5)Ir(,4)Ge(,10), and Y(,5)Rh(,4)Ge(,10) data. It is suggested that the same mechanism, namely, the forma- tion of a charge- or spin-density wave, is causing the anomalous behaviors in both the resistivity and susceptibility data;Lastly, upper critical magnetic field measurements were performed on Sc(,5)Co(,4)Si(,10), Sc(,5)Rh(,4)Si(,10), Sc(,5)Ir(,4)Si(,10), Lu(,5)Rh(,4)Si(,10), Lu(,5)Ir(,4)Si(,10), and Y(,5)Os(,4)Ge(,10). Relative to the other five samples, Y(,5)Os(,4)Ge(,10) exhibits very high values for (-dH(,c2)/dT)(,Tc) = 10.2 kOe/ K and H(,c2)(0) = 60.4 kOe. Comparing the value of (-dH(,c2)/dT)(,Tc) gained from the fit of the data to the WHH theory to the calculated (-dH(,c2)/dT)(,Tc) yields various degrees of agreement for these com- pounds. Indications are also that (1) there seems to be little or no;Pauli limiting and (2) the spin-orbit effect appears to be negligible in these compounds; *DOE Report IS-T-1215. This work was performed under contract No. W-7405-Eng-82 with the U. S. Department of Energy. (\u271)A. Junod, D. Bichsel, and J. Muller, Helv. Phys. Acta 52, 580 (1979)

    Co-receptor CD8-mediated modulation of T-cell receptor functional sensitivity and epitope recognition degeneracy

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    The interaction between T-cell receptors (TCRs) and peptide epitopes is highly degenerate: a TCR is capable of interacting productively with a wide range of different peptide ligands, involving not only cross-reactivity proper (similar epitopes elicit strong responses), but also polyspecificity (ligands with distinct physicochemical properties are capable of interacting with the TCR). Degeneracy does not gainsay the fact that TCR recognition is fundamentally specific: for the vast majority of ligands, the functional sensitivity of a given TCR is virtually null whereas this TCR has an appreciable functional sensitivity only for a minute fraction of all possible ligands. Degeneracy can be described mathematically as the probability that the functional sensitivity, of a given TCR to a randomly selected ligand, exceeds a set value. Variation of this value generates a statistical distribution that characterizes TCR degeneracy. This distribution can be modeled on the basis of a Gaussian distribution for the TCR/ligand dissociation energy. The kinetics of the TCR and the MHCI molecule can be used to transform this underlying Gaussian distribution into the observed distribution of functional sensitivity values. In the present paper, the model is extended by accounting explicitly for the kinetics of the interaction between the co-receptor and the MHCI molecule. We show that T-cells can modulate the level of degeneracy by varying the density of co-receptors on the cell surface. This could allow for an analog of avidity maturation during incipient T-cell responses

    The Swedish European elections will be held in the shadow of September’s national election, but European issues will play a key role

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    Sweden will not only hold European elections in May, but will also hold national, regional and municipal elections in September. Linda Berg and Jonathan Polk write on the effect this ‘super-election’ year will have on the vote for the European Parliament. They note that while the two largest parties, the Social Democrats and Moderates, will likely have one eye on the national elections, European issues will nevertheless play a substantial role in the campaign

    (Un-)veiling the west : Burkini-gate, Princess Hijab and dressing as struggle for postsecular integration

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    The ban of the burkini in the summer of 2016 in France is the latest stage in a long political history, where the French depreciation or fear of the veil, and of Islam, has come to play a more significant role since the end of the cold war. Unveiling female bodies at the beach in Nice expose conditioned values of the French republic. In this context, drawing black veils on public advertisements becomes a performative act commenting on consumerism, religion, secularity, and the imagined Muslim woman. In this article we discuss freedom and integration in “third spaces” via an analysis of “hijabisation” in street art and the official reactions against certain types of beachwear. In line with Talal Asad (2006) we want to raise the issue on how the secular state addresses the pain of people who are obliged to give up part of their religious identity to become acceptable. Race-thinking was once an explicit part of celebrated values like modernity, secularity, democracy and human rights. However, the fact that the idea of races has been erased from articulations of Western nations and international bodies does not mean that traces of race-thinking in the heritage from the enlightenment are gone. By following Princess Hijab and the “Burkini-gate” a nationalist fantasy intertwined with the idea of the secular state reveals itself and acts of un/dressing emerge as signs of integration revealing a challenged imperialist paradigm

    The Musculoskeletal System in Pompe Disease: Pathology, consequences and treatment options

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    __Abstract__ Pompe disease, also known as glycogen storage disorder type II and acid maltase deficiency, is a rare metabolic myopathy. It is caused by a deficiency of lysosomal acid α-glucosidase which results in the accumulation of glycogen in cells, especially muscle cells. The disease presents as a broad clinical spectrum with progressive muscle weakness as prominent symptom and can hence be categorized as a lysosomal storage disorder, a glycogen storage disorder and also as a neuromuscular disorder. The broad term “neuromuscular disorders” encompasses many different syndromes and diseases that either directly or indirectly impair the function of the skeletal muscles, the muscles that enables to move the limbs and trunk. Myopathies, diseases of the muscle itself, are a subgroup of neuromuscular disorders and include muscular dystrophies, inflammatory disorders of the muscle and metabolic diseases of the muscle. Pompe disease was the first myopathy for which treatment became available. Enzyme replacement therapy (ERT) is the registered treatment for Pompe disease. It was shown to elicit positive effects first in babies with the most severe form of the disease1-5 and later also in children and adults with more slowly progressive forms of the disease.6-16 Despite improvements in skeletal muscle strength, walking distance, respiratory function and survival, not all patients respond equally well to treatment and not all muscle damage and functional impairment is resolved.14,17,18 Long-term clinical programs should therefore also focus on comorbidities and additional strategies to improve patients’ functioning. This thesis explores the muscle pathology in Pompe disease across the clinical spectrum, the interplay between skeletal muscle dysfunction and bone structure and the effect of enzyme therapy on it, as well as the use of exercise training to improve patients’ functioning. This introductory chapter gives an overview of the history, pathophysiology, clinical characteristics, diagnosis and treatment of Pompe disease and reviews the use of exercise training programs in myopathies

    Menneskehandelens ofre - fra utnytting til myndiggjĂžring

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    Regjeringen lanserte sin fÞrste handlingsplan mot handel med kvinner og barn i 2003. Handlingsplanen mot menneskehandel har som formÄl Ä forebygge og bekjempe menneskehandel, spesielt handel med kvinner og barn, samt beskytte ofrene. Et av tiltakene i handlingsplanen var etableringen av ROSA-prosjektet. Denne evalueringen av ROSA-prosjektet er gjort pÄ oppdrag fra Justisdepartementet og er gjennomfÞrt i perioden november 2007 til juni 2008. HovedformÄlet med evalueringen har vÊrt Ä vurdere en permanent organisering av trygge bosteder for ofre for menneskehandel. Selve evalueringen bygger pÄ intervjuer med sentrale og lokale aktÞrer, gjennomgang av relevante dokumenter, samt deltakelse pÄ mÞter i den tverrdepartementale gruppa som er opprettet for Ä fÞlge opp regjeringens handlingsplan mot menneskehandel. Rapporten gir fÞlgende anbefalinger for det videre arbeidet: 1. ROSA-prosjektet bÞr viderefÞres. 2. Krisesenter er en god lÞsning i tidlig fase, men er lite egnet for langtidsopphold. 3. Innholdet i refleksjonsperioden mÄ tydeliggjÞres. 4. Systemet rundt rÄdgiving i forbindelse med retur mÄ forbedres. 5. Arbeidsdelingen mellom de ulike kompetansemiljÞer pÄ feltet tydeliggjÞre

    Defining Excellence in Graduate Studies

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    This article provides an analysis of definitions of excellence in graduate study provided by Master’s degree and doctoral candidates, identified by their department as “excellent,” and by chairs of graduate programs (n = 43) at two western Canadian universities. Faculty members’ definitions tended to focus primarily on external markers of success rather than on personal characteristics of graduate students. Both graduate faculty respondents (n = 20) and graduate student interview participants (n = 23) mentioned the importance of visibility in the department and the community. The graduate student participants made infrequent mention of external indicators, such as grades and ability to garner funding, and attributed their identification as excellent to their own actions and internal attributes. External factors frequently mentioned by graduate students were the cutting edge nature of their research and the importance of the supervisory relationship. Further exploration is needed to develop a working definition of “excellence” in graduate study and explore the factors involved in its attainment. This paper offers a rudimentary framework for how excellence in graduate study is currently defined, makes preliminary recommendations, and discusses possible directions for future research into excellence in graduate studies
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