1000 resultados para multiple hereditary
Resumo:
Debido a que en España se han dado diferentes accidentes con múltiples víctimas en diferentes puntos del país, se cree conveniente que cada ciudad tenga un plan de gestión de catástrofes con una serie de espacios habilitados para poder responder en caso de un accidente de estas características. El presente artículo tiene como objetivo presentar la habilitación de espacios aplicable en cualquier capital de provincia de España, tomando como ejemplo la ciudad de Girona. Se ha tomado la ciudad de Girona como modelo por presentar infraestructuras viarias, ferroviarias, portuarias y aeroportuarias cada vez más concurridas
Resumo:
The underlying cause of many human autoimmune diseases is unknown, but several environmental factors are implicated in triggering the self-destructive immune reactions. Multiple Sclerosis (MS) is a chronic autoimmune disease of the central nervous system, potentially leading to persistent neurological deterioration. The cause of MS is not known, and apart from immunomodulatory treatments there is no cure. In the early phase of the disease, relapsing-remitting MS (RR-MS) is characterized by unpredictable exacerbations of the neurological symptoms called relapses, which can occur at different intervals ranging from 4 weeks to several years. Microbial infections are known to be able to trigger MS relapses, and the patients are instructed to avoid all factors that might increase the risk of infections and to properly use antibiotics as well as to take care of dental hygiene. Among those environmental factors which are known to increase susceptibility to infections, high ambient air inhalable particulate matter levels affect all people within a geographical region. During the period of interest in this thesis, the occurrence of MS relapses could be effectively reduced by injections of interferon, which has immunomodulatory and antiviral properties. In this thesis, ecological and epidemiological analyses were used to study the possible connection between MS relapse occurrence, population level viral infections and air quality factors, as well as the effects of interferon medication. Hospital archive data were collected retrospectively from 1986-2001, a period in time ranging from when interferon medication first became available until just before other disease-modifying MS therapies arrived on the market. The grouped data were studied with logistic regression and intervention analysis, and individual patient data with survival analysis. Interferons proved to be effective in the treatment of MS in this observational study, as the amount of MS exacerbations was lower during interferon use as compared to the time before interferon treatment. A statistically significant temporal relationship between MS relapses and inhalable particular matter (PM10) concentrations was found in this study, which implies that MS patients are affected by the exposure to PM10. Interferon probably protected against the effect of PM10, because a significant increase in the risk of exacerbations was only observed in MS patients without interferon medication following environmental exposure to population level specific viral infections and PM10. Apart from being antiviral, interferon could thus also attenuate the enhancement of immune reactions caused by ambient air PM10. The retrospective approach utilizing carefully constructed hospital records proved to be an economical and reliable source of MS disease information for statistical analyses.
Resumo:
Most Finnish periodical magazines have a website, often an online service. The objective of this thesis is to understand the magazines’ resources and capabilities and match them with online strategies’ goals and objectives. The thesis’ theoretical part focuses on explaining and classifying resources, capabilities, goals and objectives, and applying everything into Finnish magazine publishing context. In the empirical part, there is a comparative case study of four magazines. The findings indicate that with cooperating, advertising and community hosting capabilities magazines may utilize their human, brand, content and customer base resources. The resources can be further addressed to reach profitability, customer-centricity and brand congruency goals.
Resumo:
Avhandlingen behandlar entreprenöriella intentioner och individens uppfattningar om entreprenörskap. Om vi vill främja entreprenörskap så räcker det inte att vi förstår vilken nytta samhället kan ha av entreprenörer (arbetsplatser, mera skatteinkomster osv.). Vi måste förstå varför entreprenörskap är intressant och attraktiv ur individens synvinkel. Just den frågan har varit central inom kognitiv entreprenörskapsforskning de senaste 10 åren har vår förståelse för entreprenörer ökat betydligt tack vare den forskningen. Problemet med existerande forskning är att uppfattad genomförbarhet och uppfattad attraktivitet, dvs. de attityder som sägs leda till entreprenöriella intentioner, beskriver enbart vilken attityd individen generellt har till entreprenörskap. Enligt tidigare forskningsresultat så är det skillnad på generella attityder till en handling och attityder till att genomföra just den handlingen. Vill vi veta om individen kan tänka sig starta och driva ett företag så måste vi alltså studera individens attityd till att utföra just den specifika handlingen. Enligt avhandlingens forskningsresultat så kan vi lära oss mera om attityder till entreprenörskap genom att studera också motivation och mål. På så sätt kan vi förstå varför en del väljer att bli entreprenörer medan andra väljer att låta bli, även om de utåt sett har samma möjligheter att bli entreprenörer.
Resumo:
Background. Multiple myeloma (MM) is the second most common hematologic malignancy after lymphomas In Finland: the annual incidence of MM is approximately 200. For three decades the median survival remained at 3 to 4 years from diagnosis until high-dose melphalan treatment supported by autologous stem cell transplantation (ASCT) became the standard of care for newly diagnosed MM since the mid 1990’s and the median survival increased to 5 – 6 years. This study focuses on three important aspects of ASCT, namely 1) stem cell mobilization, 2) single vs. double ASCT as initial treatment, and 3) the role of minimal residual disease (MRD) for longterm outcome. Aim. The aim of this series of studies was to evaluate the outcomes of MM patients and the ASCT procedure at the Turku University Central Hospital, Finland. First, we tried to identify which factors predict unsuccessful mobilization of autologous stem cells. Second, we compared the use of short-acting granulocyte-colony stimulating factor (GCSF) with long-acting G-CSF as mobilization agents. Third, one and two successive ASCTs were compared in 100 patients with MM. Fourth, for patients in complete response (CR) after stem cell transplantation (SCT), patient-specific probes for quantitative allele-specific oligonucleotide polymerase-chain reaction (qASO-PCR) measurements were designed to evaluate MRD and its importance for long-term outcome. Results. The quantity of previous chemotherapy and previous interferon use were significant pre-mobilization factors that predicted mobilization failure, together with some factors related to mobilization therapy itself, such as duration and degree of cytopenias and occurrence of sepsis. Short-acting and long-acting G-CSF combined with chemotherapy were comparable as stem cells mobilizers. The progression free (PFS) and overall survival (OS) tended to be longer after double ASCT than after single ASCT with a median follow-up time of 4 years, but this difference disappeared as the follow-up time increased. qASO-PCR was a good and sensitive divider of the CR patients into two prognostic groups: MRD low/negative (≤ 0.01%) and MRD high (>0.01%) groups with a significant difference in PFS and suggestively also in OS. Conclusions. When the factors prediciting a poor outcome of stem cell mobilization prevail, it is possible to identify those patients who need specific efforts to maximize the mobilization efficacy. Long-acting pegfilgrastim is a practical and effective alternative to short-acting filgrastim for mobilization therapy. There is no need to perform double ASCT on all eligible patients. MRD assessment with qASO-PCR is a sensitive method for evaluation of the depth of the CR response and can be used to predict long-term outcome after ACST.
Resumo:
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy(CADASIL) is the most common hereditary small vessel disease (SVD) leading to vascular dementia. The cause of the disease is mutations in NOTCH3 gene located at chromosome 19p13.1. The gene defect results in accumulation of granular osmiophilic material and extracellular domain of NOTCH3 at vascular smooth muscle cells (VSMCs) with subsequent degeneration of VSMCs. This arteriopathy leads to white matter (WM) rarefaction and multiple lacunar infarctions in both WM and deep grey matter (GM) visible in magnetic resonance imaging. This thesis is focused on the quantitative morphometric analysis of the stenosis and fibrosis in arterioles of the frontal cerebral WM, cortical GM and deep GM (lenticular nucleus (LN), i.e. putamen and globus pallidus). It was performed by assessing four indicators of arteriolar stenosis and fibrosis: (1) diameter of arteriolar lumen, (2) thickness of arteriolar wall, (3) external diameter of arterioles and (4) sclerotic index. These parameters were assessed (a) in 5 elderly CADASIL patients with the mean age of onset 47 years and of death 63 years, (b) in a 32-year-old young CADASIL patient with the first ischemic episode at the age of 29 years and (c) a very old CADASIL patient aged 95 years, who suffered the first stroke at the age of 71 years. These measurements were compared with age-matched controls without stroke, dementia, hypertension, and cerebral amyloid angiopathy. Morphometric analyses disclosed that in all age groups of CADASIL patients compared to corresponding controls there was significant narrowing of arteriolar lumen (stenosis) and fibrotic thickening of the walls (fibrosis) in the WM arterioles, although the significance of stenosis in the very old patient was marginal. In the LN arterioles there was only significant fibrosis without stenosis. These results suggest that the ischemic lesions and lacunar infarcts in the cerebral WM are mainly attributable to the stenosis of arterioles, whereas those in the LN are probably mainly due to hemodynamic changes of the cerebral blood flow. In conclusion: The SVD of CADASIL is characterized by narrowing of lumina and fibrotic thickening of walls predominantly in the cerebral WM arterioles. On the other hand, in the LN the ischemic lesions and lacunar infarcts are most probably hemodynamic due to impaired autoregulation caused by the rigidity of fibrotic arterioles. The pathological cerebral arteriolar alterations begin to develop already at a relatively young age but the onset may be delayed to a remarkably old age. This underlines the well known great variability in the clinical picture of CADASIL. The very late onset of CADASIL may cause its underdiagnosis, because the strokes are common in the elderly and are attributed to common risk factors.
Resumo:
The adoption of a proper traceability system is being incorporated into meat production practices as a method of gaining consumer confidence. The various partners operating in the chain of meat production can be considered a social network, and they have the common goal of generating a communication process that can ensure each characteristic of the product, including safety. This study aimed to select the most appropriate meat traceability system “from farm to fork” that could be applied to Brazilian beef and pork production for international trade. The research was done in three steps. The first used the analytical hierarchy process (AHP) for selecting the best on-farm livestock traceability. In the second step, the actors in the meat production chain were identified to build a framework and defined each role in the network. In the third step, the selection of the traceability system was done. Results indicated that with an electronic traceability system, it is possible to acquire better connections between the links in the chain and to provide the means for managing uncertainties by creating structures that facilitate information flow more efficiently.
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Social media is a rather new phenomenon which has revolutionised the world of online communication. However, academic research on how companies can benefit from social media is lacking. The research objective of this thesis was to examine the use of social media in international brand communication of small Finnish design-intensive companies. Therefore, this research contributes also to the research gap in SME branding. The focus was on communication targeted at consumers. The research was carried out as a mixed methods research employing the questionnaire and multiple case study methods. The questionnaire was used to gather preliminary information on Finnish design-intensive companies and to provide an eligible list of companies for deeper examination. Then, four case companies were studied in more depth. The empirical evidence of the case companies was mainly gathered through theme interviews. The results of the questionnaire shed light on the internationalisation of small Finnish designintensive companies. On average, the companies had internationalised rather quickly after they had been founded. However, the share of exports was rather low in most of the companies. The results revealed also that social media was already used widely in the exporting companies and the use can be expected to grow in future. The findings of the multiple case study suggest that branding activities in small Finnish designintensive companies are constrained by limited resources and skills. In addition, the branding activities are strongly guided by the vision and values of the entrepreneur(s) rather than extensive marketing research. The brand structure was simple in all case companies and they aimed at having a standardised brand image across markets. However, all case companies had faced a need for some adaptation of their international brand communication. Internationally important brand communication channels were international fairs, the internet, word-of-mouth and social media. Social media offered a cost-effective brand communication channel for the case companies. It was used for various purposes, such as creating brand awareness and affecting how the brand is perceived. The entrepreneurs found the use of social media to be rather easy and the case companies had not faced any major challenges. However, the companies had recognised that communication in social media requires consistency and planning. The planning was rather informal and stayed on a general level. Overall, the utilisation of social media in the case companies was limited by a lack of resources. It seemed to affect especially the follow-up of brand communication in social media which stayed rather superficial.
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PURPOSE: To compare obstetric outcomes of induced preterm twin births (under 32 weeks gestation) with those spontaneously conceived. METHODS: Prospective study of twin pregnancies (25 induced and 157 spontaneously conceived) developed over a period of 16 years in a tertiary obstetric center. Demographic factors, obstetric complications, gestational age at delivery, mode of delivery, birth weight and immediate newborn outcome were compared. RESULTS: The analysis of obstetrical complications concerning urinary or other infections, hypertensive disorders of pregnancy, gestational diabetes, fetal malformations, intrauterine fetal death, intrauterine growth restriction and intrauterine discordant growth reveal no significant statistical differences between the two groups. First trimester bleeding was higher in the induced group (24 versus 8.3%, p=0.029). The cesarean delivery rate was 52.2% in spontaneous gestations and 64% in induced gestations. Gestational age at delivery, birth weight, Apgar scores at first and fifth minutes, admissions to Neonatal Intensive Care Unit and puerperal complications show no statistically significant differences between the two groups. These results were independent of chorionicity and induction method. CONCLUSION: The mode of conception did not influence obstetric and neonatal outcomes. Although induced pregnancies have higher risk of first trimester bleeding, significant differences were not observed regarding other obstetric and puerperal complications and neonatal results.
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As traditional advertising is losing its value in corporations’ marketing, new and growing social media marketing channels are increasing their importance. The growing interest in the media and the lack of research on the subject create a clear need for this study. The objectives are to increase understanding about product placement in personal blogs and examine the benefits as well as the downsides created by this marketing channel and present the requirements for success. The study also takes a stand on what kind of things a successful product placement strategy in personal blogs includes and what points should be considered when creating a strategy. The study’s empirical part consists of seven thematic interviews with case companies’ representatives and one agent. The study’s personal blogs were delimited to consist only lifestyle and fashion blogs. The results show that product placement on personal lifestyle and fashion blogs is a strongly growing marketing channel and it is best suited to reach young women. It is also a very good channel to change and improve brand image. Via personal blogs it is possible to reach a large number of consumers with a very cost-efficient manner. Thus utilizing the channel is suitable for all companies regardless of the size of the marketing budget. Of course, companies have to consider the suitability of their product to the themes of blogs. Executing a successful campaign in this media requires good relationship management skills and understanding of this particular media as the final content is not in advertiser’s hands. As a marketing channel, personal blogs differ highly from traditional marketing channels because of their constantly changing nature and multidirectional quality where several parties are in interaction.
Multiple scales analysis of nonlinear oscillations of a portal frame foundation for several machines
Resumo:
An analytical study of the nonlinear vibrations of a multiple machines portal frame foundation is presented. Two unbalanced rotating machines are considered, none of them resonant with the lower natural frequencies of the supporting structure. Their combined frequencies is set in such a way as to excite, due to nonlinear behavior of the frame, either the first anti-symmetrical mode (sway) or the first symmetrical mode. The physical and geometrical characteristics of the frame are chosen to tune the natural frequencies of these two modes into a 1:2 internal resonance. The problem is reduced to a two degrees of freedom model and its nonlinear equations of motions are derived via a Lagrangian approach. Asymptotic perturbation solutions of these equations are obtained via the Multiple Scales Method.
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Weed resistance to herbicides has been a major issue in Brazil, mainly due to the inefficiency of the herbicides used in no-till areas and to the high cost of these herbicide treatments. Failures in controlling the weed Conyza have been reported in Western and Northern grain crop areas in Paraná (Brazil). This work aimed to evaluate the potential occurrence of C. sumatrensis biotypes resistant to the herbicides chlorimuron-ethyl and glyphosate. Experiments were carried out under greenhouse conditions with four biotypes (Cascavel-2, Toledo-4, Tupãssi-6, and Assis Chateaubriand-7) possibly resistant to, as well as a population considered susceptible to chlorimuron-ethyl and glyphosate. To obtain dose-response curves, eight herbicide doses of chlorimuron-ethyl (0, 2.5, 5, 10, 20, 40, 80 and 160 g ha-1) and glyphosate (0, 90, 180, 360, 720, 1,440, 2,880 and 5,760 g e.a. ha-1) were applied and weed control and shoot biomass evaluations were made. Results provided evidence that two biotypes (Cascavel-2 and Tupãssi-6) were resistant to glyphosate and four biotypes (Cascavel-2, Toledo-4, Tupãssi-6 and Assis Chateaubriand-7) were resistant to chlorimuronethyl. Multiple resistance to glyphosate and chlorimuron was confirmed for biotypes Cascavel2 and Tupãssi 6. This is the first report on multiple resistance in Conyza sumatrensis, worldwide.