An international registry on autoinflammatory diseases: the Eurofever experience.

OBJECTIVE: To report on the demographic data from the first 18 months of enrollment to an international registry on autoinflammatory diseases in the context of the Eurofever project. METHODS: A web-based registry collecting baseline and clinical information on autoinflammatory diseases and related conditions is available in the member area of the PRINTO web-site. Anonymised data were collected with standardised forms. RESULTS: 1880 (M:F=916:964) individuals from 67 centers in 31 countries have been entered in the Eurofever registry. Most of the patients (1388; 74%), reside in western Europe, 294 (16%) in the eastern and southern Mediterranean region (Turkey, Israel, North Africa), 106 (6%) in eastern Europe, 54 in Asia, 27 in South America and 11 in Australia. In total 1049 patients with a clinical diagnosis of a monogenic autoinflammatory diseases have been enrolled; genetic analysis was performed in 993 patients (95%): 703 patients have genetically confirmed disease and 197 patients are heterozygous carriers of mutations in genes that are mutated in patients with recessively inherited autoinflammatory diseases. The median diagnosis delay was 7.3 years (range 0.3-76), with a clear reduction in patients born after the identification of the first gene associated with autoinflammatory diseases in 1997. CONCLUSIONS: A shared online registry for patients with autoinflammatory diseases is available and enrollment is ongoing. Currently, there are data available for analysis on clinical presentation, disease course, and response to treatment, and to perform large scale comparative studies between different conditions.

Targeted therapy against multi-resistant bacteria in leukemic and hematopoietic stem cell transplant recipients: guidelines of the 4th European Conference on Infections in Leukemia (ECIL-4, 2011).

The detection of multi-resistant bacterial pathogens, particularly those to carbapenemases, in leukemic and stem cell transplant patients forces the use of old or non-conventional agents as the only remaining treatment options. These include colistin/polymyxin B, tigecycline, fosfomycin and various anti-gram-positive agents. Data on the use of these agents in leukemic patients are scanty, with only linezolid subjected to formal trials. The Expert Group of the 4(th) European Conference on Infections in Leukemia has developed guidelines for their use in these patient populations. Targeted therapy should be based on (i) in vitro susceptibility data, (ii) knowledge of the best treatment option against the particular species or phenotype of bacteria, (iii) pharmacokinetic/pharmacodynamic data, and (iv) careful assessment of the risk-benefit balance. For infections due to resistant Gram-negative bacteria, these agents should be preferably used in combination with other agents that remain active in vitro, because of suboptimal efficacy (e.g., tigecycline) and the risk of emergent resistance (e.g., fosfomycin). The paucity of new antibacterial drugs in the near future should lead us to limit the use of these drugs to situations where no alternative exists.

Preface"11th Plinius Conference on Mediterranean Storms"

The EGU Plinius Conference on Mediterranean Storms was established in 1999 within the framework of the Interdisciplinary Working Group on Natural Hazards (IWG-NH) of the former European Geophysical Society (EGS)- since 2002,European Geosciences Union (EGU). Since its advent, the Plinius Conference series has provided a crucial interdisciplinary forum for improving our understanding of hazardous storms over the Mediterranean basin that are capable of producing strong winds, heavy rains, explosive landslides, devastating flash floods and other related extremes ...

ODiSEA: International Registry on Research Data

El artículo revisa los temas principales en la preservación y reuso de los datos de investigación (beneficios, ciclo de vida, proyectos, normativas ) e identifica la falta de un registro mundial de bancos, repositorios y bibliotecas de datos. Expone la creación de una herramienta web que recoja este tipo de depósitos y los clasifique por áreas disciplinares: ODiSEA International Registry on Research Data. Ofrecemos resultados sobre número y tipología temática de este tipo de depósitos a escala mundial. Esta aportación facilita el descubrimiento de nuevos conjuntos de datos cuya recombinación desde una perspectiva multidisciplinar fomentará la innovación y la rentabilidad de la inversión en ciencia.

Privacy as a Tradeoff: Introducing the Notion of Privacy Calculus for Context-Aware Mobile Applications

Evidences collected from smartphones users show a growing desire of personalization offered by services for mobile devices. However, the need to accurately identify users' contexts has important implications for user's privacy and it increases the amount of trust, which users are requested to have in the service providers. In this paper, we introduce a model that describes the role of personalization and control in users' assessment of cost and benefits associated to the disclosure of private information. We present an instantiation of such model, a context-aware application for smartphones based on the Android operating system, in which users' private information are protected. Focus group interviews were conducted to examine users' privacy concerns before and after having used our application. Obtained results confirm the utility of our artifact and provide support to our theoretical model, which extends previous literature on privacy calculus and user's acceptance of context-aware technology.

On cumulant techniques in speech processing

This paper analyzes applications of cumulant analysis in speech processing. A special focus is made on different second-order statistics. A dominant role is played by an integral representation for cumulants by means of integrals involving cyclic products of kernels.

Improving Pitch Tracking Performance in Hard Noise Conditions by a Preprocessing Based on Mathematical Morphology

In this paper we show how a nonlinear preprocessing of speech signal -with high noise- based on morphological filters improves the performance of robust algorithms for pitch tracking (RAPT). This result happens for a very simple morphological filter. More sophisticated ones could even improve such results. Mathematical morphology is widely used in image processing and has a great amount of applications. Almost all its formulations derived in the two-dimensional framework are easily reformulated to be adapted to one-dimensional context

ESCCAR international congress on Rickettsia and other intracellular bacteria.

The European Society for the study of Chlamydia, Coxiella, Anaplasma and Rickettsia (ESCCAR) held his triennial international meeting in Lausanne. This meeting gathered 165 scientists from 28 countries and all 5 continents, allowing efficient networking and major scientific exchanges. Topics covered include molecular and cellular microbiology, genomics, as well as epidemiology, veterinary and human medicine. Several breakthroughs have been revealed at the meeting, such as (i) the presence of CRISPR (the "prokaryotic immune system") in chlamydiae, (ii) an Anaplasma effector involved in host chromatin remodelling, (iii) the polarity of the type III secretion system of chlamydiae during the entry process revealed by cryo-electron tomography. Moreover, the ESCCAR meeting was a unique opportunity to be exposed to cutting-edge science and to listen to comprehensive talks on current hot topics.