Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers.

The objective of this work was to develop and validate a set of clinical criteria for the classification of patients affected by periodic fevers. Patients with inherited periodic fevers (familial Mediterranean fever (FMF); mevalonate kinase deficiency (MKD); tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS); cryopyrin-associated periodic syndromes (CAPS)) enrolled in the Eurofever Registry up until March 2013 were evaluated. Patients with periodic fever, aphthosis, pharyngitis and adenitis (PFAPA) syndrome were used as negative controls. For each genetic disease, patients were considered to be 'gold standard' on the basis of the presence of a confirmatory genetic analysis. Clinical criteria were formulated on the basis of univariate and multivariate analysis in an initial group of patients (training set) and validated in an independent set of patients (validation set). A total of 1215 consecutive patients with periodic fevers were identified, and 518 gold standard patients (291 FMF, 74 MKD, 86 TRAPS, 67 CAPS) and 199 patients with PFAPA as disease controls were evaluated. The univariate and multivariate analyses identified a number of clinical variables that correlated independently with each disease, and four provisional classification scores were created. Cut-off values of the classification scores were chosen using receiver operating characteristic curve analysis as those giving the highest sensitivity and specificity. The classification scores were then tested in an independent set of patients (validation set) with an area under the curve of 0.98 for FMF, 0.95 for TRAPS, 0.96 for MKD, and 0.99 for CAPS. In conclusion, evidence-based provisional clinical criteria with high sensitivity and specificity for the clinical classification of patients with inherited periodic fevers have been developed.

Phenotypic and genotypic characteristics of cryopyrin-associated periodic syndrome: a series of 136 patients from the Eurofever Registry.

OBJECTIVE: To evaluate genetic, demographic and clinical features in patients with cryopyrin-associated periodic syndrome (CAPS) from the Eurofever Registry, with a focus on genotype-phenotype correlations and predictive disease severity markers. METHODS: A web-based registry retrospectively collected data on patients with CAPS. Experts in the disease independently validated all cases. Patients carrying NLRP3 variants and germline-mutation-negative patients were included. RESULTS: 136 patients were analysed. The median age at disease onset was 9 months, and the median duration of follow-up was 15 years. Skin rash, musculoskeletal involvement and fever were the most prevalent features. Neurological involvement (including severe complications) was noted in 40% and 12% of the patients, respectively, with ophthalmological involvement in 71%, and neurosensory hearing loss in 42%. 133 patients carried a heterozygous, germline mutation, and 3 patients were mutation-negative (despite complete NLRP3 gene screening). Thirty-one different NLRP3 mutations were recorded; 7 accounted for 78% of the patients, whereas 24 rare variants were found in 27 cases. The latter were significantly associated with early disease onset, neurological complications (including severe complications) and severe musculoskeletal involvement. The T348M variant was associated with early disease onset, chronic course and hearing loss. Neurological involvement was less strongly associated with V198M, E311 K and A439 V alleles. Early onset was predictive of severe neurological complications and hearing loss. CONCLUSIONS: Patients carrying rare NLRP3 variants are at risk of severe CAPS; onset before the age of 6 months is associated with more severe neurological involvement and hearing loss. These findings may have an impact on treatment decisions.

Broadband prices in the European Union : competition and commercial strategies

This paper analyses the determinants of broadband Internet access prices in a group of 15 EU countries between 2008 and 2011. Using a rich panel dataset of broadband plans, we show the positive effect of downstream speed on prices, and report that cable and fibre-to-the-home technologies are available at lower prices per Mbps than x DSL technology. Operators’marketing strategies are also analysed as we show how much prices rise when the broadband service is offered in a bundle with voice telephony and/or television, and how much they fall when download volume caps are included. The most insightful results of this study are provided by a group of metrics that represent the situation of competition and entry patterns in the broadband market. We show that consumer segmentation positively affects prices. On the other hand, broadband prices are higher in countries where entrants make greater use of bitstream access and lower when they use more intensively direct access -local loop unbundling-. However, we do not find a significant effect of inter-platform competition on prices.

Liderazgo y dirección de empresas en el s. XXI

Peer-reviewed

La Gestió per competències als llocs de treball de comandament de les policies locals

Les policies són potser un dels sectors de l’administració que més incidència directa té sobre la ciutadania en les seves decisions quotidianes i que, per tant, més han d’evolucionar cap aquests models descentralitzats i horitzontals de decisió i de funcionament en xarxa. Aquesta responsabilitat de millora i adaptació és competència immediata dels comandaments intermedis i als Caps d’aquests Cossos, que hauran de disposar de les capacitats, habilitats i coneixements necessaris (competències) per dur-los a terme. Caldria, doncs, plantejar-nos si un sistema basat en la gestió per competències podria ser idoni per ser aplicat de forma integral: des del reclutament o selecció passant per la definició de perfils competencials per als llocs de treball, per posteriorment poder fer l’avaluació d’acompliment del lloc i atribuir en aquest resultat alguna mena de recompensa o reconeixement en forma de carrera professional vertical o horitzontal. En aquest treball ens centrarem en les dues primeres etapes, és a dir, estudiarem si la gestió per competències podria millorar els mètodes de reclutament i, de forma especial, en la definició del perfil professional idoni, per als Caps i els comandaments intermedis de la Policia Local, per a la qual cosa es durà a terme un treball de camp amb els responsables polítics i comandaments de diferents policies locals de Catalunya.

A evolução dos reagentes químicos comerciais através dos rótulos e frascos

This work presents an evolution profile of labels and containers of commercial chemicals employed in laboratories since 1870. Most chemicals were made in Germany before the Second World War, after which many other manufacturers arrived on the Brazilian market. North-american products were dominant in the 1940s, but Brazilian chemicals have increased their participation along time. Labels presented increasingly more information, from originally simple names of the compounds at the beginning of the XXth century to the data presented today such as chemical formulae, safety regards and detailed chemical analysis. The raw material for container manufacturing also changed: glass was dominant until the 1950s, but nowadays plastic flasks are preferred whenever possible. Cork covers were replaced by screw caps. The diversity of commercial products also sharply increased with time, especially after the 1950s, following the many new and specific applications of chemicals for research and commercial purposes.

Projecte de legalització d'una activitat dedicada a l'explotació ramadera i a la formatgeria artesanal

La integració en una mateixa activitat d'una explotació ramadera ecològica basada en el bestiar cabrú de llet i d'una formatgeria artesanal basada en varietats autòctones de formatge de cabra, permet ocupar un nínxol de mercat encara poc explotat actualment a Catalunya. D'igual manera, la dificultat que tenen actualment molts dels formatgers artesans del país per trobar llet de cabra en quantitats suficients i amb unes mínimes garanties higiènico-sanitàries dificulten l'avanç d'un sector amb molt bones perspectives de futur com és el de la formatgeria artesanal i justifica la necessitat de crear noves explotacions (o reconvertir-ne de ja existents) orientant-les de ple vers l'aprofitament lleter del bestiar cabrú, que és actualment molt reduït en l'àmbit Català. A més d'això, les actuals exigències en el camp del benestar animal així com en el camp de la qualitat i de la seguretat alimentaria, exigeixen a les explotacions ramaderes i a les indústries alimentàries el compliment d'una sèrie de requisits, tan de disseny dels locals i de les instal•lacions, com de control de les activitats i dels productes elaborats, que garanteixin en tot moment la seva correcte gestió i funcionament en aquestes matèries. L'objectiu d'aquest projecte, doncs, és la legalització d'una activitat, que anomenarem “Mas Peirot, S.L.”, dedicada a l'explotació ramadera ecològica de bestiar cabrú de llet i a la formatgeria artesanal de formatge de cabra. És important mencionar que tot i tractar-se d'un projecte de legalització d'una activitat,, també s'hi han contemplat aspectes i detalls relacionats amb l'execució de la mateixa, al tractar-se d'unes obres d'una certa complexitat i considerant que així es tracten amb més detall i rigorositat les actuacions projectades. En concret, en aquest projecte es planifiquen les operacions de rehabilitació de dues naus ramaderes actualment en desús, per tal que puguin destinar-se a l'explotació ramadera de bestiar cabrú i a la formatgeria artesanal, projectant-se així mateix totes les instal•lacions necessàries per tal que a les mencionades naus s'hi puguin desenvolupar les activitats citades. Com a conclusions més importants d'aquest projecte podem dir que amb un cens de 100 caps de bestiar cabrú és possible obtenir una producció mitjana de llet de 32.500 litres anuals, que es corresponen a un volum total de 3.823 quilograms de formatge a l'any (de les tipologies Formatge Garrotxa i Formatge de cabra amb oli, ambdues varietats tradicionals catalanes) obtenint un benefici de 21.389,47 Euros/any durant els primers 15 anys i un benefici de 33.386,39 Euros/any a partir del 16è any, cosa que permetrà recuperar la inversió inicial de 172.716,76 Euros que valen les naus i instal•lacions projectades, en un termini de 8 anys i permetrà, com s'ha dit, ocupar un nínxol de mercat encara poc explotat per la indústria agroalimentària a Catalunya.

Estudi tècnic-econòmic per a la instal·lació d'una granja d'ànecs per a producció de foie-gras situada en el terme municipal de Fontanals de Cerdanya

Inversors però, principalment professionals de l'àmbit agrari, per tal de buscar una diversificació de l'activitat agrícola i ramadera que desenvolupen a les seves finques, sobretot joves emprenedors, poden estar interessats en produccions alternatives com la producció d'ànecs per a foie-gras, per tal de complementar la seva renda agrària. Actualment cal augmentar la competitivitat de les finques agrícoles per tal que aquestes siguin econòmicament viables. L’objectiu de l'estudi és determinar la viabilitat econòmica d’una explotació ramadera d’ànecs per a la producció de foie-gras, en el terme municipal de Fontanals de Cerdanya, analitzant les diverses alternatives en quant a dimensionament i disseny de les instal·lacions, segons el cicle productiu de l’animal. L'alternativa escollida per realitzar l'estudi es la de semi-integració (cria, engreix i embocat), de règim semi-intensiu (durant l'etapa de l'engreix), amb animals mascles de la raça híbrida Mulard. Les instal·lacions tindran una capacitat de 6.500 animals, amb lots de 250 caps. Aquest dimensionament permet, durant la fase d'embocat, en la qual es requereix més dedicació, que la demanda de mà d'obra pugui ser satisfeta per un únic treballador. La principal conclusió que es pot extreure de l'estudi és que la inversió projectada és massa elevada i que aquesta econòmicament no és viable o rendible, ja que la despesa és massa elevada. Es mouen uns fluxos de caixa molt elevats i el marge comercial és molt reduït. Únicament si l'objectiu del promotor és obtenir una remuneració per la seva feina, sempre i quan és produeixi un augment del preu actual de la carn i una disminució del preu del pinso, es podria recomanar que es portés a terme la implantació de l'explotació, intentant, però, reduir la despesa d'execució de construccions i instal·lacions.

Determinação espectrofotométrica indireta de capsaicinoides em pimentas Capsicum a partir da reação com o complexo de Co(II) com 4-(2-piridilazo) resorcinol

Capsaicinoids (CAPS) are substances responsible for pungency in Capsicum. It is important to quantify these types of compounds owing to their broad application in food, pharmaceuticals, cosmetics and chemical weapons. In this work, we developed an indirect spectrophotometric method based on the colorimetric reaction between CAPS, Co(II) 3.10×10-5 mol L-1 and 4-(2-pyridylazo) resorcinol (PAR) 6.23×10-5 and, in cachaça:water 92:8v/v solutions, for quantification of total CAPS in Capsicum peppers. The product of the reaction is CoPAR2CAPS2 and its absorption in aquo-ethanolic solution at 510 nm is proportional to the total CAPS concentration from 0.60 to 17.94 mg L-1. The values of limit of detection and limit of quantification were 0.0004 and 0.001 mg of CAPS/g of pepper, respectively, with 4% relative standard deviation. The developed method yielded similar results to those obtained from high performance liquid chromatography, with 95% of confidence.

The Performance of European Small Cap Equity Funds

The thesis examines the risk-adjusted performance of European small cap equity funds between 2008 and 2013. The performance is measured using several measures including Sharpe ratio, Treynor ratio, Modigliani measure, Jensen alpha, 3-factor alpha and 4-factor alpha. The thesis also addresses the issue of persistence in mutual fund performance. Thirdly, the relationship between the activity of fund managers and fund performance is investigated. The managerial activity is measured using tracking error and R-squared obtained from a 4-factor asset pricing model. The issues are investigated using Spearman rank correlation test, cross-sectional regression analysis and ranked portfolio tests. Monthly return data was provided by Morningstar and consists of 88 mutual funds. Results show that small cap funds earn back a significant amount of their expenses, but on average loose to their benchmark index. The evidence of performance persistence over 12-month time period is weak. Managerial activity is shown to positively contribute to fund performance

Essential oil of Lippia alba and its main constituent citral block the excitability of rat sciatic nerves

Lippia alba is empirically used for infusions, teas, macerates, and hydroalcoholic extracts because of its antispasmodic, analgesic, sedative, and anxiolytic effects. Citral is a mixture of trans-geranial and cis-neral and is the main constituent of L. alba essential oil and possesses analgesic, anxiolytic, anticonvulsant, and sedative effects. The present study evaluated the effects of the essential oil of L. alba (EOLa) and citral on compound action potentials (CAPs) in Wistar rat sciatic nerves. Both drugs inhibited CAP in a concentration-dependent manner. The calculated half-maximal inhibitory concentrations (IC50) of peak-to-peak amplitude were 53.2 µg/mL and 35.00 µg/mL (or 230 µM) for EOLa and citral, respectively. Peak-to-peak amplitude of the CAP was significantly reduced by 30 µg/mL EOLa and 10 µg/mL citral. EOLa and citral (at 60 and 30 µg/mL, values close to their respective IC50 for CAP blockade) significantly increased chronaxy and rheobase. The conduction velocity of the first and second CAP components was statistically reduced to ∼86% of control with 10 µg/mL EOLa and ∼90% of control with 3 µg/mL citral. This study showed that EOLa inhibited nerve excitability and this effect can be explained by the presence of citral in its composition. Both EOLa and citral showed inhibitory actions at lower concentrations compared with other essential oils and constituents with local anesthetic activity. In conclusion, these data demonstrate that EOLa and citral are promising agents in the development of new drugs with local anesthetic activity.

A designação id quod summum omnium e os "nomes divinos" em Anselmo de Cantuária

Anselmo de Cantuária investiga no Proslogion (caps. 5-12) se o conteúdo de nossas palavras se refere de modo adequado à substância criadora. Essa obra de Anselmo pode ser considerada como uma meditação realizada por um espírito que busca entender aquilo que inicialmente crê a respeito do ser divino. O Proslogion nos oferecerá um caminho para pensar o sentido da busca de razões no domínio exclusivo da fé, do esforço da palavra humana para encontrar aquilo que já fora dito por outra palavra. Este artigo visa a explicitar o sentido dessa racionalidade, naquilo que diz respeito aos "nomes divinos". A designação id quod summum omnium (Proslogion, cap.5) retoma um tema já presente na reflexão anselmiana e impõe ao teólogo um desafio: articular, sob a dialética do melius, o modo propriamente humano de a criatura referir-se a Deus, reconhecido como maius e distante de qualquer relação de continuidade com o mundo.

HG 198.20-198.35 Thin Section

Coarse grained brown sediment with angular-sub-angular grains. Contains mainly medium sized clasts with a few smaller aggregates. A dark organic rich domain can be seen within this sample. Grain crushing (edge-to-edge and into one another) can be seen alongside with grain stacks, and silt caps.

Receipt from Andrews and Aikine

Receipt from Andrews and Aikine, Hats, Caps, Furs and Gloves, St. Catharines for clothing, April 9, 1887.

Le recyclage du déchet dans l'art du XXIe siècle : trois études de cas

Pour respecter les droits d'auteur, la version électronique de ce mémoire a été dépouillée de ses documents visuels et audio-visuels. La version intégrale du mémoire a été déposée au Service de la gestion des documents et des archives de l'Université de Montréal.