PFAPA syndrome is not sporadic disease

Résumé de thèseLe syndrome de PFAPA est une maladie fébrile récurrente décrite pour la première fois en 1987 par Marshall et col. Elle est caractérisée par une fièvre périodique, une stomatite aphteuse, une pharyngite et des adénopathies. Ce syndrome débute dans les premières années de vie et est connu pour disparaître spontanément en principe avant l'adolescence. Hormis un traitement de prednisone en début de crise, aucun traitement n'a pu montrer une efficacité thérapeutique ou curative.L'origine et l'étiologie de cette maladie sont encore inconnues à ce jour et le diagnostic reste un diagnostic d'exclusion qui repose sur des critères définis par différents groupes depuis 1987. Dans le cadre du Working Party periodic fever de la Société Européenne de Rhumatologie pédiatrique (PreS), un groupe a été établi et celui-ci a mis en place un registre de patients atteints de PFAPA afin d'analyser cette maladie et de mieux définir les critères diagnostic. Le Dr Michael Hofer a été nommé chairman de ce groupe et a introduit rapidement les patients romands dans cet outil de travail.L'introduction des patients romands dans la base de données ainsi créée, nous a suggéré une susceptibilité familiale qui nous a poussés à investiguer ce point de manière plus approfondie. Nous avons donc regroupé tous les patients lausannois et ceux de collègues bordelais ayant un diagnostic avéré de PFAPA. Nous avons ensuite interrogé, au cours d'un entretien téléphonique, les familles de ces enfants grâce à un questionnaire standardisé. Celui-ci a été testé et validé sur des patients sains d'une consultation de pédiatrie générale.Nous avons ensuite réunie toutes ces informations et séparés les patients en deux groupes AF+ (anamnèse familiale positive pour une fièvre récurrente) et AF- (anamnèse familiale négative pour une fièvre récurrente). Nous avons établi des comparaisons entre les 2 différents groupes en reprenant les caractéristiques de ces patients depuis le registre PFAPA dans lequel ils sont tous inclus. Les analyses ont été contrôlées et validées par le centre d'épidémiologie clinique grâce aux méthodes statistiques reconnues.Les résultats obtenus et qui sont détaillés dans l'article, permettent de suspecter une origine familiale et par là même, potentiellement génétique, à cette maladie d'étiologie inconnue. Jusqu'à présent aucune prépondérance familiale n'avait pu être mise en évidence dans les autres études sur le sujet. Pourtant cette maladie fait partie du groupe des fièvres récurrentes qui ont pour beaucoup déjà un diagnostic génétique.Notre étude ouvre donc des perspectives non seulement de recherche sur l'éventuelle cause génétique mais pourrait également permettre une meilleure compréhension de la maladie, de ses diverses présentations ainsi que par la suite de nouvelles possibilités thérapeutiques.

Political alliances and organisational change in political parties: a framework for analysis

This article sets out a theoretical framework for the study of organisational change within political alliances. To achieve this objective it uses as a starting point a series of premises, the most notable of which include the definition of organisational change as a discrete, complex and focussed phenomenon of changes in power within the party. In accordance with these premises, it analyses the synthetic model of organisational change proposed by Panebianco (1988). After examining its limitations, a number of amendments are proposed to adapt it to the way political alliances operate. The above has resulted in the design of four new models. In order to test its validity and explanatory power in a preliminary manner, the second part looks at the organisational change of the UDC within the CiU alliance between 1978 and 2001. The discussion and conclusions reached demonstrate the problems of determinism of the Panebianco model and suggest, tentatively, the importance of the power balance within the alliance as a key factor.

Devolution & Entrenched Household Poverty: Is Scotland less mobile?

The Scottish National Party led Scottish Government has identified household poverty as a key focus for its anti-poverty strategy. The government’s ‘Solidarity Target’ seeks to both increase wealth and increase the share of total income gained by these three deciles. The ability to demonstrate the advantages of policy divergence within Scotland, relative to the other parts of the United Kingdom, is central to the Government’s aim of gaining support for increased powers for the devolved government. This paper seeks to provide evidence on one aspect of the government’s anti- poverty strategy; the degree to which Scotland differs from the rest of the UK over levels of entrenched poverty. The paper demonstrates that not only does Scotland have greater entrenched poverty but that the changes in mobility since the 1990s have impacted on Scotland to a lesser degree than the rest of the UK.

Electoral Uncertainty and the Deficit Bias in a New Keynesian Economy

Recent attempts to incorporate optimal fiscal policy into New Keynesian models subject to nominal inertia, have tended to assume that policy makers are benevolent and have access to a commitment technology. A separate literature, on the New Political Economy, has focused on real economies where there is strategic use of policy instruments in a world of political conflict. In this paper we combine these literatures and assume that policy is set in a New Keynesian economy by one of two policy makers facing electoral uncertainty (in terms of infrequent elections and an endogenous voting mechanism). The policy makers generally share the social welfare function, but differ in their preferences over fiscal expenditure (in its size and/or composition). Given the environment, policy shall be realistically constrained to be time-consistent. In a sticky-price economy, such heterogeneity gives rise to the possibility of one policy maker utilising (nominal) debt strategically to tie the hands of the other party, and influence the outcome of any future elections. This can give rise to a deficit bias, implying a sub-optimally high level of steady-state debt, and can also imply a sub-optimal response to shocks. The steady-state distortions and inflation bias this generates, combined with the volatility induced by the electoral cycle in a sticky-price environment, can significantly

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The use of the Internet now has a specific purpose: to find information. Unfortunately, the amount of data available on the Internet is growing exponentially, creating what can be considered a nearly infinite and ever-evolving network with no discernable structure. This rapid growth has raised the question of how to find the most relevant information. Many different techniques have been introduced to address the information overload, including search engines, Semantic Web, and recommender systems, among others. Recommender systems are computer-based techniques that are used to reduce information overload and recommend products likely to interest a user when given some information about the user's profile. This technique is mainly used in e-Commerce to suggest items that fit a customer's purchasing tendencies. The use of recommender systems for e-Government is a research topic that is intended to improve the interaction among public administrations, citizens, and the private sector through reducing information overload on e-Government services. More specifically, e-Democracy aims to increase citizens' participation in democratic processes through the use of information and communication technologies. In this chapter, an architecture of a recommender system that uses fuzzy clustering methods for e-Elections is introduced. In addition, a comparison with the smartvote system, a Web-based Voting Assistance Application (VAA) used to aid voters in finding the party or candidate that is most in line with their preferences, is presented.

A bayesian spatial individual effects probit model of the 2010 UK general election

The Conservative Party emerged from the 2010 United Kingdom General Election as the largest single party, but their support was not geographically uniform. In this paper, we estimate a hierarchical Bayesian spatial probit model that tests for the presence of regional voting effects. This model allows for the estimation of individual region-specic effects on the probability of Conservative Party success, incorporating information on the spatial relationships between the regions of the mainland United Kingdom. After controlling for a range of important covariates, we find that these spatial relationships are significant and that our individual region-specic effects estimates provide additional evidence of North-South variations in Conservative Party support.

Mafia in the ballot box

We study the impact of organized crime on electoral competition. Assuming that the mafia is able to bring votes to the supported party in exchange of money, we show that (i) the strongest party is willing to pay the highest price to secure mafia services; (ii) the volume of electoral trade with the mafia increases with political competition and with the efficiency of the mafia. Studying in detail parliamentary elections in Sicily for the period 1946- 1992, we document the significant support given by the Sicilian Mafia to the Christian Democratic party, starting at least from the 1970s. This is consistent with our theoretical predictions, as political competition became much tighter during the 1970s and the Sicilian mafia experienced an extensive centralization process towards the end of the 1960s, which increased substantially its control of the territory. We also provide evidence that in exchange for its electoral support the mafia got economic advantages for its activities in the construction industry.

Determinants of public education expenditure: Evidence from Indian states

Public education expenditure varies significantly across Indian states. Using data on sixteen Indian states from 2001-2010, the paper tries to identify the determinants of per capita education expenditure of state governments in India. The econometric findings indicate that richer states spend more on education compared to the poorer states. A lower share of child population (0-14 years) is found to significantly enhance education expenditure at the state level. We do not find any evidence that political factors such as political ideology of the ruling party and level of corruption affect education expenditure of state governments.

Authoritarian Responses to Foreign Pressure: Spending, Repression, and Sanctions

This paper explores how international sanctions affect authoritarian rulers’ decisions concerning repression and public spending composition, and how different authoritarian rulers respond to foreign pressure. If sanctions are assumed to increase the price of loyalty to the regime, then rulers whose budgets are not severely constrained by sanctions will tend to increase spending in those categories that most benefit their core support groups. In contrast, when constraints are severe due to reduced aid and trade, dictators are expected to greatly increase their levels of repression. Using data on regime types, public expenditures and spending composition (1970–2000) as well as on repression levels (1976–2001), we show that the empirical patterns conform well to our theoretical expectations. Single-party regimes, when targeted by sanctions, increase spending on subsidies and transfers which largely benefit more substantial sectors of the population and especially the urban classes. Likewise, military regimes increase their expenditures on goods and services, which include military equipment and soldiers’ and officers’ wages. Conversely, personalist regimes reduce spending in all categories, especially capital expenditures, while increasing repression much more than other regime types when targeted by sanctions.

Comparative cost-effectiveness analyses of cardiovascular magnetic resonance and coronary angiography combined with fractional flow reserve for the diagnosis of coronary artery disease.

BACKGROUND: According to recent guidelines, patients with coronary artery disease (CAD) should undergo revascularization if significant myocardial ischemia is present. Both, cardiovascular magnetic resonance (CMR) and fractional flow reserve (FFR) allow for a reliable ischemia assessment and in combination with anatomical information provided by invasive coronary angiography (CXA), such a work-up sets the basis for a decision to revascularize or not. The cost-effectiveness ratio of these two strategies is compared. METHODS: Strategy 1) CMR to assess ischemia followed by CXA in ischemia-positive patients (CMR + CXA), Strategy 2) CXA followed by FFR in angiographically positive stenoses (CXA + FFR). The costs, evaluated from the third party payer perspective in Switzerland, Germany, the United Kingdom (UK), and the United States (US), included public prices of the different outpatient procedures and costs induced by procedural complications and by diagnostic errors. The effectiveness criterion was the correct identification of hemodynamically significant coronary lesion(s) (= significant CAD) complemented by full anatomical information. Test performances were derived from the published literature. Cost-effectiveness ratios for both strategies were compared for hypothetical cohorts with different pretest likelihood of significant CAD. RESULTS: CMR + CXA and CXA + FFR were equally cost-effective at a pretest likelihood of CAD of 62% in Switzerland, 65% in Germany, 83% in the UK, and 82% in the US with costs of CHF 5'794, euro 1'517, £ 2'680, and $ 2'179 per patient correctly diagnosed. Below these thresholds, CMR + CXA showed lower costs per patient correctly diagnosed than CXA + FFR. CONCLUSIONS: The CMR + CXA strategy is more cost-effective than CXA + FFR below a CAD prevalence of 62%, 65%, 83%, and 82% for the Swiss, the German, the UK, and the US health care systems, respectively. These findings may help to optimize resource utilization in the diagnosis of CAD.

Impact of voting advice applications on voters's decision-making

So-called online Voting Advice Applications (VAAs) have become very popular all over Europe. Millions of voters are using them as an assistance to make up their minds for which party they should vote. Despite this popularity there are only very few studies about the impact of these tools on individual electoral choice. On the basis of the Swiss VAA smartvote we present some first findings about the question whether VAAs do have a direct impact on the actual vote of their users. In deed, we find strong evidence that Swiss voters were affected by smartvote. However, our findings are somewhat contrary to the results of previous studies from other countries. Furthermore, the quality of available data for such studies needs to be improved. Future studies should pay attention to both: the improvement of the available data, as well as the explanation of the large variance of findings between the specific European countries.

Are parties domocratizing themselves? the evolution of leardership selection in Spain (1977-2008)

The aim of this paper is to verify, for the Spanish case, whether between 1977 and 2008 has increased the internal democracy of the major political parties (PSOE, AP / PP, PCE / IU, PNV and CDC). To do this, we will focus on their leadership selection processes, one of the key elements associated with intra-party democracy. The paper is going to introduce data on four different dimensions of leadership selection: the certification process, the voting procedure, the inclusiveness of the selectorate and, finally, the degree of competitiveness. The results will show that have been few changes in the leadership selection processes of the Spanish political parties since 1977. However, the results of the Spanish case will also be used to suggest some preliminary links between the four dimensions.

Civil war and foreign influence

We study a symmetric information bargaining model of civil war where a third (foreign) party can affect the probabilities of winning the conflict and the size of the post conflict spoils. We show that the possible alliance with a third party makes peaceful agreements difficult to reach and might lead to new commitment problems that trigger war. Also, we argue that the foreign party is likely to induce persistent informational asymmetries which might explain long lasting civil wars. We explore both political and economic incentives for a third party to intervene. The explicit consideration of political incentives leads to two predictions that allow for identifying the influence of foreign intervention on civil war incidence. Both predictions are confirmed for the case of the U.S. as a potential intervening nation: (i) civil wars around the world are more likely under Republican governments and (ii) the probability of civil wars decreases with U.S. presidential approval rates.

Diagnosi ambiental de la riera de Santa Coloma entre Santa Coloma de Farners i Riudarenes

A l’actualitat trobem nombrosos sistemes aquàtics alterats per diferents efectes d’origen antropogènic. Per tal d’evitar i/o disminuir aquests efectes va sorgir la Directiva Marc de l’Aigua (2000/60/CE) essent aquest un dels seus objectius. Aquest article descriu el funcionament hidrogeològic i l’estat ecològic de la riera de Santa Coloma, afluent de la Tordera (NE Catalunya), des de pràcticament el seu inici a Santa Coloma de Farners (Girona) fins a Riudarenes (Girona). S’intenta establir les possibles influències del funcionament hidrogeològic en l’estat ecològic a partir de dades piezomètriques, de cabal, fisicoquímiques i biològiques. Per aquesta última part s’han utilitzat indicadors biològics com l’índex d’hàbitat fluvial (IHF), avaluant l’hàbitat físic; l’índex de Qualitat del Bosc de Ribera (QBR), per determinar la qualitat ecològica de la zona de ribera; l’índex Biological Monitorig Working Party per a conques internes de Catalunya (BMWPC), per avaluar les diferents famílies de macroinvertebrats que hi viuen i l’índex ECOSTRIMED, una síntesi dels dos índex anteriors.

L'impacte dels sistemes multinivell en els sistemes de partits

Imaginemos que un observador quiere analizar el sistema político de los países de Europa Occidental. Si compara el sistema de partidos que hay a nivel nacional con los presentes en cada uno de los distritos de esta elección, apreciará notables diferencias entre países. Por ejemplo, si repara en Alemania, verá como los mismos cinco partidos presentes en el Bundestag compiten también en todos los distritos. Si por otra parte se fija en España, comprobará como en las Elecciones Generales españolas algunos partidos se presentan en todas las circunscripciones pero otros sólo lo hacen en una o en un puñado de ellas. Por lo tanto, mientras que en el primer caso tenemos que el sistema político nacional se reproduce de manera idéntica en cada distrito, en el segundo hay diferencias entre los partidos a nivel nacional y los presentes en determinados distritos. ¿Por qué existen estas divergencias? ¿Qué es lo que explica que esta situación sea estática o que cambie a lo largo del tiempo? En este anexo presento una memoria para justificar los principales avances en la investigación doctoral durante el periodo en que he disfrutado de la beca FI.