The 1980 earthquake in southern Italy: rescue of trapped victims and mortality.

A retrospective survey was undertaken on the health effects of the 1980 earthquake in southern Italy. The study population included 3619 people living in 7 villages situated near the epicentre of the disaster. The overall casualty rate (dead and injured) was 19.7%. Nearly all the deaths (192/202) occurred among trapped people who died before they could be rescued. Eighty per cent of all the trapped people were extricated within 2 days, mostly without the use of sophisticated means. The probability of survival decreased sharply, the longer the time before extrication. The crude mortality during the 18 months following the earthquake was 19.0 per thousand among the injured people who received treatment, and 14.1 per thousand among non-injured people. After age standardization, there was no significant difference between these two figures and the expected mortality figures for the Italian population in normal times (14.4 per thousand). These results stress the importance of providing rescue activities in the first 48 hours after the impact. Strengthening the self-reliance of the community in disaster preparedness is suggested as the best way to improve the effectiveness of relief operations. In disaster-prone areas, training and education in methods of rescue should be an integral part of any primary health care programme

The V471A polymorphism in autophagy-related gene ATG7 modifies age at onset specifically in Italian Huntington disease patients

The cause of Huntington disease (HD) is a polyglutamine repeat expansion of more than 36 units in the huntingtin protein, which is inversely correlated with the age at onset of the disease. However, additional genetic factors are believed to modify the course and the age at onset of HD. Recently, we identified the V471A polymorphism in the autophagy-related gene ATG7, a key component of the autophagy pathway that plays an important role in HD pathogenesis, to be associated with the age at onset in a large group of European Huntington disease patients. To confirm this association in a second independent patient cohort, we analysed the ATG7 V471A polymorphism in additional 1,464 European HD patients of the “REGISTRY” cohort from the European Huntington Disease Network (EHDN). In the entire REGISTRY cohort we could not confirm a modifying effect of the ATG7 V471A polymorphism. However, analysing a modifying effect of ATG7 in these REGISTRY patients and in patients of our previous HD cohort according to their ethnic origin, we identified a significant effect of the ATG7 V471A polymorphism on the HD age at onset only in the Italian population (327 patients). In these Italian patients, the polymorphism is associated with a 6-years earlier disease onset and thus seems to have an aggravating effect. We could specify the role of ATG7 as a genetic modifier for HD particularly in the Italian population. This result affirms the modifying influence of the autophagic pathway on the course of HD, but also suggests population-specific modifying mechanisms in HD pathogenesis

Identification of genetic variants associated with Huntington's disease progression: a genome-wide association study

Background Huntington's disease is caused by a CAG repeat expansion in the huntingtin gene, HTT. Age at onset has been used as a quantitative phenotype in genetic analysis looking for Huntington's disease modifiers, but is hard to define and not always available. Therefore, we aimed to generate a novel measure of disease progression and to identify genetic markers associated with this progression measure. Methods We generated a progression score on the basis of principal component analysis of prospectively acquired longitudinal changes in motor, cognitive, and imaging measures in the 218 indivduals in the TRACK-HD cohort of Huntington's disease gene mutation carriers (data collected 2008–11). We generated a parallel progression score using data from 1773 previously genotyped participants from the European Huntington's Disease Network REGISTRY study of Huntington's disease mutation carriers (data collected 2003–13). We did a genome-wide association analyses in terms of progression for 216 TRACK-HD participants and 1773 REGISTRY participants, then a meta-analysis of these results was undertaken. Findings Longitudinal motor, cognitive, and imaging scores were correlated with each other in TRACK-HD participants, justifying use of a single, cross-domain measure of disease progression in both studies. The TRACK-HD and REGISTRY progression measures were correlated with each other (r=0·674), and with age at onset (TRACK-HD, r=0·315; REGISTRY, r=0·234). The meta-analysis of progression in TRACK-HD and REGISTRY gave a genome-wide significant signal (p=1·12 × 10−10) on chromosome 5 spanning three genes: MSH3, DHFR, and MTRNR2L2. The genes in this locus were associated with progression in TRACK-HD (MSH3 p=2·94 × 10−8 DHFR p=8·37 × 10−7 MTRNR2L2 p=2·15 × 10−9) and to a lesser extent in REGISTRY (MSH3 p=9·36 × 10−4 DHFR p=8·45 × 10−4 MTRNR2L2 p=1·20 × 10−3). The lead single nucleotide polymorphism (SNP) in TRACK-HD (rs557874766) was genome-wide significant in the meta-analysis (p=1·58 × 10−8), and encodes an aminoacid change (Pro67Ala) in MSH3. In TRACK-HD, each copy of the minor allele at this SNP was associated with a 0·4 units per year (95% CI 0·16–0·66) reduction in the rate of change of the Unified Huntington's Disease Rating Scale (UHDRS) Total Motor Score, and a reduction of 0·12 units per year (95% CI 0·06–0·18) in the rate of change of UHDRS Total Functional Capacity score. These associations remained significant after adjusting for age of onset. Interpretation The multidomain progression measure in TRACK-HD was associated with a functional variant that was genome-wide significant in our meta-analysis. The association in only 216 participants implies that the progression measure is a sensitive reflection of disease burden, that the effect size at this locus is large, or both. Knockout of Msh3 reduces somatic expansion in Huntington's disease mouse models, suggesting this mechanism as an area for future therapeutic investigation

The People's Champions? Populist Communication as a Contextually Dependent Political Strategy

This article conceives of populist communication as a contextually dependent political strategy. We bridge actor- and communication-centered approaches by arguing that the context of issues conditions the extent to which parties employ populist communication. We draw from a content analysis of 2,085 news stories in eight news media outlets and Eurobarometer data connected to 41 EU policy issues and analyze statements from 85 political parties. Our findings show that populist parties are more prone to express populism on salient and polarized issues. Issues important to civil society groups, in contrast, make non-populist parties more inclined to express such communication.</p

Balanced or biased? Interest groups and legislative lobbying in the European news media

This article examines the coverage of legislative lobbying in European news media. The starting point thereby is that lobbying in the crowded European Union (EU)-level interest community is not only a struggle for direct access to policymakers, but that in order to realize policy goals many interest groups rely on political attention generated by the media. Our main research question is how media attention is skewed toward particular interests and which factors explain these varying levels of prominence. Our empirical analysis is based on a set of 125 legislative proposals adopted by the European Commission between 2008 and 2010. For all these cases we identified 379 interest organizations that made public statements, we coded the amount of media attention these organized interests gained, the type of statements they made as well as some key organizational features. While the aggregate levels of attention look pretty balanced, our evidence shows that media prominence is skewed toward particular types of interests; in particular that organized interests which oppose a proposed policy gain significantly higher levels of media attention

The people versus the money: What drives interest group influence in the European Union?

This paper evaluates whether lobbying influence is open to the highest bidder or boosted by congruence with popular opinion. Common wisdom holds that well-endowed organizations prevail in lobbying battles. This perception contrasts with recent observations, which point to the decisive role of public opinion. This paper unites these seemingly contrasting stances by arguing that both economic resources and congruence with public opinion are paramount for lobbying influence. What matters, we argue, is the interplay between the two. Lobby groups that already enjoy substantial economic capacities are expected to benefit most from congruence with public opinion. We test our expectations in the context of European Union policy making. We draw from a sample of 41 policy issues for which public opinion polls were conducted, an extensive content analysis of 2,085 news articles and 183 lobbyists’ survey responses. We demonstrate that interest groups with more economic resources are generally more influential, but only if their policy positions are congruent with a public majorit

The alignment of parties and interest groups in EU legislative politics: A tale of two different worlds?

Political science research on European Union (EU) interest groups and parties represents two separate study fields and both literatures convey a somewhat different image of EU legislative policy-making. While most scholars of party politics endorse the notion that parties politicize EU legislative politics, scholarship of interest groups tends to portray EU policy-making as embedded in a somewhat de-politicized context in which expertise-based exchanges between societal interests and bureaucratic agencies is crucial. By analysing the context of EU legislative lobbying in relation to 54 legislative acts we demonstrate that, in particular on controversial cases, the alignment of parties and interests groups reflects party political cleavages

Lobbying the lobbyists: when and why do policymakers seek to influence advocacy groups in global governance?

Advocacy is typically conceived of as an activity where advocacy groups seek and policymakers grant influence. In this paper, we turn the classic approach to advocacy upside down and ask under what conditions policymakers seek to exert influence on the positions adopted by opposing or allied advocacy groups. Two strategies that policymakers can use in their interactions with advocacy groups are proposed: amplification and persuasion. We build on resource exchange theory and the concept of political opportunity structures to explain which strategy a policymaker uses. The analysis relies on a unique database, which draws from 297 interviews with policymakers from 107 different countries at global climate change and trade conferences. Our results demonstrate that, overall, policymakers seek out advocacy groups more when they are faced with increased levels of political pressures. Namely, elected politicians are more prone to seek out opposing societal interests than non-elected policymakers. Moreover, policymakers from democratically accountable countries, who work on salient issues, are more inclined to mobilize their advocacy group allies