25 research outputs found
Red Europe Versus No Europe? The impact of attitudes towards the EU and the economic crisis on radical-left voting
The 2014 European Parliament election saw a relatively large increase in the size of radical left parties (RLPs), particularly in Western Europe. This article aims to provide new ways of thinking about the dynamics of radical-left voting by analyzing the changing role of attitudes towards the European Union (EU) in explaining support for RLPs at European Parliament elections during the Great Recession. It is argued that the Europeanization of economic issues during the financial crisis, together with the particular kind of Euroscepticism advocated by these parties, have enabled them to successfully attract a heterogeneous pool of voters. Using the 2009 and 2014 European Elections Studies, it is shown that the effect of negative opinions about the EU on radical-left voting increased significantly during the crisis. In addition, support for RLPs also increased among voters with positive views of the EU who were nevertheless strongly dissatisfied with the economic situation
Weathering the crisis: Evidence of diffuse support for the EU from a six-wave Dutch panel
Political regimes draw legitimacy from diffuse political support. How diffuse is support for the European Union? By focusing on cross-sectional data, the extant literature fails to demonstrate that support for the European Union displays the key defining characteristic of diffuse support: individual-level stability over a time of crisis. I use a six-wave panel data set from the Netherlands to study stability in support for the European Parliament during the 2008 economic crisis. I argue that public support for the European Parliament is highly diffuse. Using three analytical techniques, I find that individual-level support for the European Parliament remained highly stable from 2007 to 2012. These results suggest that in times of crisis, the European Union can draw on mass public support as a source of resilience
Impact of Vutrisiran on Quality of Life and Physical Function in Patients with Hereditary Transthyretin-Mediated Amyloidosis with Polyneuropathy
INTRODUCTION: Hereditary transthyretin (ATTRv; v for variant) amyloidosis, also known as hATTR amyloidosis, is a progressive and fatal disease associated with rapid deterioration of physical function and patients' quality of life (QOL). Vutrisiran, a subcutaneously administered RNA interference (RNAi) therapeutic that reduces hepatic production of transthyretin, was assessed in patients with ATTRv amyloidosis with polyneuropathy in the pivotal HELIOS-A study. METHODS: The phase 3 open-label HELIOS-A study investigated the efficacy and safety of vutrisiran in patients with ATTRv amyloidosis with polyneuropathy, compared with an external placebo group from the APOLLO study of the RNAi therapeutic patisiran. Measures of QOL and physical function were assessed. RESULTS: At month 18, vutrisiran improved Norfolk Quality of Life-Diabetic Neuropathy (Norfolk QOL-DN) total score (least squares mean difference [LSMD] in change from baseline [CFB]: –21.0; p = 1.84 × 10–10) and Norfolk QOL-DN domain scores, compared with external placebo. This benefit relative to external placebo was evident across all baseline polyneuropathy disability (PND) scores and most pronounced in patients with baseline PND scores I–II. Compared with external placebo, vutrisiran also demonstrated benefit in EuroQoL-Visual Analog Scale (EQ-VAS) score (LSMD in CFB: 13.7; nominal p = 2.21 × 10–7), 10-m walk test (LSMD in CFB: 0.239 m/s; p = 1.21 × 10–7), Rasch-built Overall Disability Score (LSMD in CFB: 8.4; p = 3.54 × 10–15), and modified body mass index (mBMI) (LSMD in CFB: 140.7; p = 4.16 × 10–15) at month 18. Overall, Norfolk QOL-DN, EQ-VAS, and mBMI improved from pretreatment baseline with vutrisiran, whereas all measures worsened from baseline in the external placebo group. At month 18, Karnofsky Performance Status was stable/improved from baseline in 58.2/13.1% with vutrisiran versus 34.7/8.1% with external placebo. CONCLUSION: Vutrisiran treatment provided significant clinical benefits in multiple measures of QOL and physical function in patients with ATTRv amyloidosis with polyneuropathy. Benefits were most pronounced in patients with earlier-stage disease, highlighting the importance of early diagnosis and treatment
Determination of serum ferritin by a one-step immunoenzymoassay, and comparison of four liver-ferritin standards.
Abstract
We describe a one step "sandwich"-type immunoenzymoassay for ferritin in human serum. The solid-phase consists of glutaraldehyde-treated polypropylene tubes coated with rabbit antibody to human ferritin. Liver ferritin is the standard. Peroxidase-conjugated antiserum to ferritin and a sensitive chromogen, o-phenylenediamine, are used. The assay requires 90 min. The standard curve is linear up to 400 micrograms of ferritin per liter of serum. Within- and between-run CVs are less than 6% for low, high, and medium concentrations and are about 13.0% at the decision level for iron deficiency. Results by a two-step "sandwich" procedure (New England Immunology Associates kit) correlated well, r = 0.98. We assessed four liver ferritin standards from different manufacturers with the described method. The mean absorbance for the 40 micrograms/L ferritin standard was 1.5 for that from Diagnostics Biochem and National Institute for Biological Standards and Controls, 1.0 for that from Dako, and 0.4 for that from Sigma. Consequently, to standardize results, all liver ferritin standards should be calibrated vs the National Institute for Biological Standards and Controls reference standard.</jats:p
Neuropathies périphériques au cours des maladies de système : partie I (connectivites et granulomatoses)
Neuropathies périphériques au cours des maladies de système : partie II (vascularites)
Early detection of skin and cardiac amyloid deposits among asymptomatic carriers of hereditary pathogenic transthyretin mutation with normal electroneuromyography
Abstract
Introduction
During the last decade, disease modifying therapies have been proposed for hereditary amyloidogenic transthyretin (ATTRv) amyloidosis. It is essential to screen for disease onset among asymptomatic mutation carriers in order to trigger effective therapy as early as possible. We describe clinical and paraclinical data from ATTRv asymptomatic carriers, as a baseline assessment to detect disease onset and progression.
Methods
We retrospectively collected data of asymptomatic ATTRv carriers with normal electroneuromyography (ENMG), between March 1st 2015 and January 31st 2019, including: demographics, symptoms, physical exam, ENMG (initial and follow-up obtained in 73 patients, 56.2%), neurovegetative tests, skin biopsy (amyloid deposition, denervation), and cardiac evaluation (multimodal imaging, cardiac denervation and arrhythmias).
Results
We included 130 patients, aged 43.6 years (± 13.5), selected in family of a proband with an age of onset of 52.7 years (±15.7), 40.8% male, carrying 20 different variants of the TTR gene, including 63.8% Val30Met. Amyloid deposits on skin biopsy and/or cardiac fixation on bone scintigraphy, characteristic of amyloid infiltration, were found in 22/130 patients (16%). Skin biopsy was positive in 11 patients, and cardiac fixation on bone scintigraphy was positive in 15 patients. Amyloid infiltration was statistically associated with age (p=0.024), age difference from index case (p&lt;0.001), electrophysiological carpal tunnel syndrome (p=0.022), interventricular septum thickness &gt;12 mm (p&lt;0.001), contrast enhancement in cardiac MRI (p=0.002), cardiac denervation using MIBG scintigraphy C/M ratio &lt;1.85 (p=0.044). Multivariate analysis showed that age difference with index case and interventricular septum thickness were predictors for amyloid infiltration (OR=1.11, IC [1,04–1,19] and OR=1,76, IC [1,19–2,60]). Subgroup analysis showed electroneuromyography alteration during follow up in 11 patients (delay 26.9 months, range 10–49) with statistic association with amyloid deposits (55.6 versus 7.1% when ENMG was unchanged, p&lt;0.001) and interventricular septum thickness (p&lt;0.010).
Conclusion
Clinical and paraclinical assessment of asymptomatic carriers of ATTRv mutations shows that skin or cardiac amyloid infiltration may precede ENMG abnormalities in 16% of patients. This may trigger specific therapies in borderline cases. Age close to age of onset among index case and septum interventricular thickness appear as risk factors for developing electroneuromyogram alteration.
Funding Acknowledgement
Type of funding source: None
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Early detection of skin and cardiac amyloid deposits among asymptomatic carriers of hereditary pathogenic transthyretin mutation with normal electroneuromyography
International audienc
Red Europe versus no Europe? The impact of attitudes towards the EU and the economic crisis on radical-left voting
Hijacking Europe: Counter‐European Strategies and Radical Right Mainstreaming during the Humanitarian Crisis Debate 2015–16*
Radical right European contestation is analyzed mainly as claims denouncing European integration. Less focus is put on narratives suggesting how a supposed 'truly European' political process should be and work. This paper explores how radical right actors use such counter-European claims within their competition strategies. Computer-assisted qualitative analysis of communication by Front National, Alternative for Germany and Fidesz during the humanitarian crisis debate 2015-16 demonstrates that radical right counter-Europeanism constitutes a nuanced tactical resource deployed to normalize nativist supply in the name of Europe. Unlike Eurorejectionist FN, Fidesz and AfD redefined Europe in nativist terms, attempting to draw legitimacy from association with European identity and cooperation. Concurrently, both actors justified Euronativism not only as antithetical to current EU values and political principles, but mainly as their more fundamental practice juxtaposed against 'not (truly) European elites', effectively 'hijacking' Europe from within. The findings deepen our understanding of radical right Europe-contesting competition strategies
