942 resultados para Oinonen, Eriikka: Finnish and Spanish families in converging Europe
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Whilst shopping malls have been explored at length by critical urban studies, there has been little exploration of their role in restructuring the practice of urban and spatial planning. This article uses the shopping mall as an object of study in the light of the neoliberal trends and post-metropolisation in Southern Europe, with the aim of exploring challenges for urban governance and planning practice and with a focus on the role of the ongoing economic crisis. A threefold exploratory framework – the ‘lost-in-time scenario’, the ‘messianic mall model’ and the ‘(im)mature planning explanation’ – is used to make sense of the local versions of shopping mall development in Lisbon (Portugal) and Palermo (Southern Italy). According to findings, we highlight the clash between the multi-scalar nature of shopping malls and the dominance of the municipal scale in regulatory planning frameworks, and the risk that shopping mall development (at least in Southern Europe) may replicate uneven development patterns, reproducing the pre-conditions of the crisis without helping to overcome it.
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Pessimistic Malthusian verdicts on the capacity of pre-industrial European economies to sustain a degree of real economic growth under conditions of population growth are challenged using current reconstructions of urbanisation ratios, the real wage rates of building and agricultural labourers, and GDP per capita estimated by a range of methods. Economic growth is shown to have outpaced population growth and raised GDP per capita to in excess of $1,500 (1990 $ international at PPP) in Italy during its twelfth- and thirteenth-century commercial revolution, Holland during its fifteenth- and sixteenth-century golden age, and England during the seventeenth- and eighteenth-century runup to its industrial revolution. During each of these Smithian growth episodes expanding trade and commerce sustained significant output and employment growth in the manufacturing and service sectors. These positive developments were not necessarily reflected by trends in real wage rates for the latter were powerfully influenced by associated changes in relative factor prices and the per capita supply of labour as workers varied the length of the working year in order to consume either more leisure or more goods. The scale of the divergence between trends in real wage rates and GDP per capita nevertheless varied a great deal between countries for reasons which have yet to be adequately explained.
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The European summer of 2013 was marked by hot and dry conditions in Western Europe associated with a northward shifted Atlantic storm track and a positive phase of the SNAO. Model results suggest that, relative to a 1964–93 reference period, changes in SST/SIE explain 63% (±26%) of the area-averaged warming signal over Western Europe, with the remaining 37% (±29%) explained by the direct impact of changes in anthropogenic radiative forcings from GHG and aerosols. The results further suggest that the anomalous atmospheric circulation, and associated low rainfall, were also influenced both by changes in SST/SIE and by the direct impact of changes in radiative forcings; however, the magnitude of the forced signals in these variables is much less, relative to internal variability, than for surface air temperature. Further evidence suggests that changes in North Atlantic SST were likely an important factor in explaining the striking contrast between the European summers of 2013 and that of 2012. A major area for further work is to understand more completely the mechanisms that explain these influences.
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Familial hypomagnesemia with hypercalciuria and nephrocalcinosis is an autosomal recessive tubular disorder characterized by excessive renal magnesium and calcium excretion and chronic kidney failure. This rare disease is caused by mutations in the CLDN16 and CLDN19 genes. These genes encode the tight junction proteins claudin-16 and claudin-19, respectively, which regulate the paracellular ion reabsorption in the kidney. Patients with mutations in the CLDN19 gene also present severe visual impairment. Our goals in this study were to examine the clinical characteristics of a large cohort of Spanish patients with this disorder and to identify the disease causing mutations. We included a total of 31 patients belonging to 27 unrelated families and studied renal and ocular manifestations. We then analyzed by direct DNA sequencing the coding regions of CLDN16 and CLDN19 genes in these patients. Bioinformatic tools were used to predict the consequences of mutations. Clinical evaluation showed ocular defects in 87% of patients, including mainly myopia, nystagmus and macular colobomata. Twenty two percent of patients underwent renal transplantation and impaired renal function was observed in another 61% of patients. Results of the genetic analysis revealed CLDN19 mutations in all patients confirming the clinical diagnosis. The majority of patients exhibited the previously described p.G20D mutation. Haplotype analysis using three microsatellite markers showed a founder effect for this recurrent mutation in our cohort. We also identified four new pathogenic mutations in CLDN19, p.G122R, p.I41T, p.G75C and p.G75S. A strategy based on microsequencing was designed to facilitate the genetic diagnosis of this disease. Our data indicate that patients with CLDN19 mutations have a high risk of progression to chronic renal disease.
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OBJECTIVE: To identify the genetic causes underlying early-onset autosomal recessive retinitis pigmentosa (arRP) in the Spanish population and describe the associated phenotype. DESIGN: Case series. PARTICIPANTS: A total of 244 unrelated families affected by early-onset arRP. METHODS: Homozygosity mapping or exome sequencing analysis was performed in 3 families segregating arRP. A mutational screening was performed in 241 additional unrelated families for the p.Ser452Stop mutation. Haplotype analysis also was conducted. Individuals who were homozygotes, double heterozygotes, or carriers of mutations in RP1 underwent an ophthalmic evaluation to establish a genotype-phenotype correlation. MAIN OUTCOME MEASURES: DNA sequence variants, homozygous regions, haplotypes, best-corrected visual acuity, visual field assessments, electroretinogram responses, and optical coherence tomography images. RESULTS: Four novel mutations in RP1 were identified. The new mutation p.Ser542Stop was present in 11 of 244 (4.5%) of the studied families. All chromosomes harboring this mutation shared the same haplotype. All patients presented a common phenotype with an early age of onset and a prompt macular degeneration, whereas the heterozygote carriers did not show any signs of retinitis pigmentosa (RP). CONCLUSIONS: p.Ser542Stop is a single founder mutation and the most prevalent described mutation in the Spanish population. It causes early-onset RP with a rapid macular degeneration and is responsible for 4.5% of all cases. Our data suggest that the implication of RP1 in arRP may be underestimated. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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Inherited retinal dystrophies present extensive phenotypic and genetic heterogeneity, posing a challenge for patients' molecular and clinical diagnoses. In this study, we wanted to clinically characterize and investigate the molecular etiology of an atypical form of autosomal recessive retinal dystrophy in two consanguineous Spanish families. Affected members of the respective families exhibited an array of clinical features including reduced visual acuity, photophobia, defective color vision, reduced or absent ERG responses, macular atrophy and pigmentary deposits in the peripheral retina. Genetic investigation included autozygosity mapping coupled with exome sequencing in the first family, whereas autozygome-guided candidate gene screening was performed by means of Sanger DNA sequencing in the second family. Our approach revealed nucleotide changes in CDHR1; a homozygous missense variant (c.1720C > G, p.P574A) and a homozygous single base transition (c.1485 + 2T > C) affecting the canonical 5' splice site of intron 13, respectively. Both changes co-segregated with the disease and were absent among cohorts of unrelated control individuals. To date, only five mutations in CDHR1 have been identified, all resulting in premature stop codons leading to mRNA nonsense mediated decay. Our work reports two previously unidentified homozygous mutations in CDHR1 further expanding the mutational spectrum of this gene.
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Abundant research has shown that poverty has negative influences on young child academic and psychosocial development, and unfortunately, disparities in school readiness between low and high income children can be seen as early the first year of life. The largest federal early care and education intervention for these vulnerable children is Early Head Start (EHS). To diminish these disparate child outcomes, EHS seeks to provide community based flexible programming for infants and toddlers and their families. Given how relatively recent these programs have been offered, little is known about the nuances of how EHS impacts infant and toddler language and psychosocial development. Using a framework of Community Based Participatory Research (CBPR) this paper had 5 goals: 1) to characterize the associations between domain specific and cumulative risk and child outcomes 2) to validate and explore these risk-outcome associations separately for Children of Hispanic immigrants (COHIs), 3) to explore relationships among family characteristics, multiple environmental factors, and dosage patterns in different EHS program types, 4) to examine the relationship between EHS dosage and child outcomes, and 5) to examine how EHS compliance impacts child internalizing and externalizing behaviors and emerging language abilities. Results of the current study showed that risks were differentially related to child outcomes. Poor maternal mental health was related to child internalizing and externalizing behaviors, but not related to emerging child language skills. Although child language skills were not related to maternal mental health, they were related to economic hardship. Additionally, parent level Spanish use and heritage orientation were associated with positive child outcomes. Results also showed that these relationships differed when COHIs and children with native-born parents were examined separately. Further, unique patterns emerged for EHS program use, for example families who participated in home-based care were less likely to comply with EHS attendance requirements. These findings provide tangible suggestions for EHS stakeholders: namely, the need to develop effective programming that targets engagement for diverse families enrolled in EHS programs.
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Issue for 1889 includes French, Spanish, Portuguese, Finnish, German, Dutch, Swedish, Norwegian-Danish, Bohemian, Welsh, and Italian sections.
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Background De-institutionalization of psychiatric patients has led to a greater emphasis on family management in the community, and family members are often overwhelmed by the demands that caring for a patient with schizophrenia involves. Most studies of family burden in schizophrenia have taken place in developed countries. The current study examined family burden and its correlates in a regional area of a medium income country in South America. Method Sixty-five relatives of patients with schizophrenia who were attending a public mental health out-patient service in the province of Arica, Chile, were assessed on Spanish versions of the Zarit Caregiver Burden Scale and SF-36 Health Survey (SF-36). Results Average levels of burden were very high, particularly for mothers, carers with less education, carers of younger patients and carers of patients with more hospitalisations in the previous 3 years. Kinship and number of recent hospitalisations retained unique predictive variance in a multiple regression. Burden was the strongest predictor of SF-36 subscales, and the prediction from burden remained significant after entry of other potential predictors. Conclusions In common with families in developed countries, family members of schizophrenia patients in regional Chile reported high levels of burden and related functional and health impact. The study highlighted the support needs of carers in contexts with high rates of poverty and limited health and community resources.
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P>Context Congenital generalized lipodystrophy, or Berardinelli-Seip syndrome, is a rare autosomal recessive disease caused by mutations in either the BSCL2 or AGPAT2 genes. This syndrome is characterized by an almost complete loss of adipose tissue usually diagnosed at birth or early infancy resulting in apparent muscle hypertrophy. Common clinical features are acanthosis nigricans, hepatomegaly with or without splenomegaly and high stature. Acromegaloid features, cardiomyopathy and mental retardation can also be present. Design We investigated 11 kindreds from different geographical areas of Brazil (northeast and southeast). All coding regions as well as flanking intronic regions of both genes were examined. Polymerase chain reaction (PCR) amplifications were performed using primers described previously and PCR products were sequenced directly. Results Four AGPAT2 and two BSCL2 families harboured the same set of mutations. BSCL2 gene mutations were found in the homozygous form in four kindreds (c.412C > T c.464T > C, c.518-519insA, IVS5-2A > G), and in two kindreds compound mutations were found (c.1363C > T, c.424A > G). In the other four families, one mutation of the AGPAT2 gene was found (IVS3-1G > C and c.299G > A). Conclusions We have demonstrated four novel mutations of the BSCL2 and AGPAT2 genes responsible for Berardinelli-Seip syndrome and Brunzell syndrome (AGPAT2-related syndrome).
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Carbon isotope composition (delta C-13), oxygen isotope composition (delta O-18), and nitrogen concentration (N-mass) of branchlet tissue at two canopy positions were assessed for glasshouse seedlings and 9-year-old hoop pine (Araucaria cunninghamii Ait. ex D. Don) trees from 22 open-pollinated families grown in 5 blocks of a progeny test at a water-limited and nitrogen-deficient site in southeastern Queensland, Australia. Significant variations in canopy delta C-13, delta O-18, and N-mass existed among the 9-year-old hoop pine families, with a heritability estimate of 0.72 for branchlet delta C-13 from the upper inner canopy position. There was significant variation in canopy delta C-13 of glasshouse seedlings between canopy positions and among the families, with a heritability estimate of 0.66. The canopy delta C-13 was positively related to canopy N-mass only for the upper outer crown in the field (R = 0.62, p < 0.001). Phenotypic correlations existed between tree height and canopy delta C-13 (R = 0.37-0.41, p < 0.001). Strong correlations were found between family canopy delta C-13 at this site and those at a wetter site and between field canopy delta C-13 and glasshouse seedling delta C-13. The mechanisms of the variation in canopy delta C-13 are discussed in relation to canopy photosynthetic capacity as reflected in the N-mass and stomatal conductance as indexed by canopy delta O-18.
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This paper will focus on some aspects of translation based on blending distinct linguistic domains such as the vocabulary of Hotel Industry, of Enology and Gastronomy in Spanish by tertiary level students (2nd year) of the course of Hotel Management. Portuguese students, most of the times, rely on a L1 (Portuguese) general language, namely using false cognates in the above mentioned areas in the Spanish and English classes in, at a first sight helpful but misleading way, hoping to succeed by using the word that seems correct to the context, when there isn’t, because: •they choose a word suitable to the context in L2, but the choice of that word is often misleading, by relying in a false L1 reality that is going to adulterate reality in the L2 domain, •but it seems that the opposite is also true, and takes place too; The difficulty in making such type of distinctions is due to: •the lack of linguistic and lexical knowledge; • the need to study the cause of these chromaticisms, by: • being in touch with specific literature; . working, not only with their peers, but also with their language teacher to develop strategies to diminish and, if possible, to eradicate this type of linguistic and, mainly translation problem, that causes so many learning constraints.
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ABSTRACT OBJECTIVE To analyze the relations between the meanings of working and the levels of doctors work well-being in the context of their working conditions. METHOD The research combined the qualitative methodology of textual analysis and the quantitative one of correspondence factor analysis. A convenience, intentional, and stratified sample composed of 305 Spanish and Latin American doctors completed an extensive questionnaire on the topics of the research. RESULTS The general meaning of working for the group located in the quartile of malaise included perceptions of discomfort, frustration, and exhaustion. However, those showing higher levels of well-being, located on the opposite quartile, associated their working experience with good conditions and the development of their professional and personal competences. CONCLUSIONS The study provides empirical evidence of the relationship between contextual factors and the meanings of working for participants with higher levels of malaise, and of the importance granted both to intrinsic and extrinsic factors by those who scored highest on well-being.
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In this paper we check whether generator's bid behavior at the Spanish whosale electricity market is consistent with the hypothesis of profit maximization on their residual demands. Using OMEL data, we find the arc-elacticity of the residual demand around the system marginal price. The results suggest thet the larger firms are not actually profit-msximization. We argue how the regulatory environment may drive these results. Finally, we repeat the analysis for the first session of the intra-day market where presumably firms may not have the same incentives as in the day-ahead market.