Impact of non-invasive mechanical ventilation (niv) in critical patients with influenza (H1N1) PDM09

The use of non-invasive mechanical ventilation (NIV) in patients with influenza A (H1N1)pdm09 admitted to intensive care units (ICU) has been controversial

L-Arginine Ameliorates Defective Autophagy in GM2 Gangliosidoses by mTOR Modulation.

AIMS: Tay-Sachs and Sandhoff diseases (GM2 gangliosidosis) are autosomal recessive disorders of lysosomal function that cause progressive neurodegeneration in infants and young children. Impaired hydrolysis catalysed by β-hexosaminidase A (HexA) leads to the accumulation of GM2 ganglioside in neuronal lysosomes. Despite the storage phenotype, the role of autophagy and its regulation by mTOR has yet to be explored in the neuropathogenesis. Accordingly, we investigated the effects on autophagy and lysosomal integrity using skin fibroblasts obtained from patients with Tay-Sachs and Sandhoff diseases. RESULTS: Pathological autophagosomes with impaired autophagic flux, an abnormality confirmed by electron microscopy and biochemical studies revealing the accelerated release of mature cathepsins and HexA into the cytosol, indicating increased lysosomal permeability. GM2 fibroblasts showed diminished mTOR signalling with reduced basal mTOR activity. Accordingly, provision of a positive nutrient signal by L-arginine supplementation partially restored mTOR activity and ameliorated the cytopathological abnormalities. INNOVATION: Our data provide a novel molecular mechanism underlying GM2 gangliosidosis. Impaired autophagy caused by insufficient lysosomal function might represent a new therapeutic target for these diseases. CONCLUSIONS: We contend that the expression of autophagy/lysosome/mTOR-associated molecules may prove useful peripheral biomarkers for facile monitoring of treatment of GM2 gangliosidosis and neurodegenerative disorders that affect the lysosomal function and disrupt autophagy

Satisfaction rating in a group of women from Granada on birthing care, support and breastfeeding lenght

Introducción: El análisis de la satisfacción se está utilizando como instrumento para crear diferentes reformas sanitarias para la mejora de la calidad y numerosos estudios apuntan al incremento de la satisfacción de la madre en relación directa con el cuidado en la maternidad. Objetivos: Identificar el grado de satisfacción de la mujer sobre la atención al parto, el acompañamiento durante el nacimiento y la duración de la lactancia materna. Objetivos: Identificar el grado de satisfacción de la mujer sobre la atención al parto, el acompañamiento durante el nacimiento y la duración de la lactancia materna. Material y método: Estudio descriptivo transversal en el Hospital Universitario San Cecilio de Granada (España), durante el periodo de tiempo agosto del 2011 al 2012, se realizo con una segunda fase de seguimeinto prospectivo a una N= 60 madres. Se utilizó un protocolo (anexo 1) a las 24 horas en el hospital y a los 14 días por teléfono. A los 3 meses, se realizó un seguimeinto relacionado con la alimentación del bebe. Resultados: El nivel de satisfacción global sobre el parto es alto en la población estudiada. Se ha demostrado que la lactancia materna (P=0,514) y el parto vía vaginal sin epidural (P=0,320) crea una mayor satisfacción en la madre. Por otro lado, la satisfacción sobre el parto guarda relación con la duración de la lactancia materna. Conclusión: La opinión satisfactoria de las madres relacionadas con la atención al parto y el acompañamiento durante el nacimiento se incrementa en aquellas mujeres cuyo parto ocurrió de forma eutócica sin epidural e iniciaron la lactancia materna precoz

Development of an activity disease score in patients with uveitis (UVEDAI)

To develop a disease activity index for patients with uveitis (UVEDAI) encompassing the relevant domains of disease activity considered important among experts in this field. The steps for designing UVEDAI were: (a) Defining the construct and establishing the domains through a formal judgment of experts, (b) A two-round Delphi study with a panel of 15 experts to determine the relevant items, (c) Selection of items: A logistic regression model was developed that set ocular inflammatory activity as the dependent variable. The construct "uveitis inflammatory activity" was defined as any intraocular inflammation that included external structures (cornea) in addition to uvea. Seven domains and 15 items were identified: best-corrected visual acuity, inflammation of the anterior chamber (anterior chamber cells, hypopyon, the presence of fibrin, active posterior keratic precipitates and iris nodules), intraocular pressure, inflammation of the vitreous cavity (vitreous haze, snowballs and snowbanks), central macular edema, inflammation of the posterior pole (the presence and number of choroidal/retinal lesions, vascular inflammation and papillitis), and global assessment from both (patient and physician). From all the variables studied in the multivariate model, anterior chamber cell grade, vitreous haze, central macular edema, inflammatory vessel sheathing, papillitis, choroidal/retinal lesions and patient evaluation were included in UVEDAI. UVEDAI is an index designed to assess the global ocular inflammatory activity in patients with uveitis. It might prove worthwhile to motorize the activity of this extraarticular manifestation of some rheumatic diseases

TOMO-ETNA experiment at Etna volcano: Activities on land

In the present paper we describe the on-land field operations integrated in the TOMO-ETNA experiment carried out in June-November 2014 at Mt. Etna volcano and surrounding areas. This terrestrial campaign consists in the deployment of 90 short-period portable three-component seismic stations, 17 broadband seismometers and the coordination with 133 permanent seismic station belonging to Italy’s Istituto Nazionale di Geofisica e Vulcanologia (INGV). This temporary seismic network recorded active and passive seismic sources. Active seismic sources were generated by an array of air-guns mounted in the Spanish oceanographic vessel “Sarmiento de Gamboa” with a power capacity of up to 5200 cubic inches. In total more than 26,000 shots were fired and more than 450 local and regional earthquakes were recorded. We describe the whole technical procedure followed to guarantee the success of this complex seismic experiment. We started with the description of the location of the potential safety places to deploy the portable network and the products derived from this search (a large document including full characterization of the sites, owners and indication of how to arrive to them). A full technical description of the seismometers and seismic sources is presented. We show how the portable seismic network was deployed, maintained and recovered in different stages. The large international collaboration of this experiment is reflected in the participation of more than 75 researchers, technicians and students from different institutions and countries in the on-land activities. The main objectives of the experiment were achieved with great success.PublishedS04272SR. VULCANI - Servizi e ricerca per la SocietàJCR Journalope

Long-term follow-up of certolizumab pegol in uveitis due to immune-mediated inflammatory diseases : multicentre study of 80 patients

Objectives To evaluate effectiveness and safety of certolizumab pegol (CZP) in uveitis due to immune-mediated inflammatory diseases (IMID). Methods Multicentre study of CZP-treated patients with IMID uveitis refractory to conventional immunosuppressant. Effectiveness was assessed through the following ocular parameters: best-corrected visual acuity, anterior chamber cells, vitritis, macular thickness and retinal vasculitis. These variables were compared between the baseline, and first week, first, third, sixth months, first and second year. Results We studied 80 (33 men/47 women) patients (111 affected eyes) with a mean age of 41.6±11.7 years. The IMID included were: spondyloarthritis (n=43), Behçet's disease (n=10), psoriatic arthritis (n=8), Crohn's disease (n=4), sarcoidosis (n=2), juvenile idiopathic arthritis (n=1), reactive arthritis (n=1), rheumatoid arthritis (n=1), relapsing polychondritis (n=1), Conclusions CZP seems to be effective and safe in uveitis related to different IMID, even in patients refractory to previous biological drugs

Cystic Echinococcosis in Spain: Current Situation and Relevance for Other Endemic Areas in Europe

Cystic echinococcosis (CE) remains an important health problem in many regions of the world, both where no control measures have been implemented, and where control programs have been incompletely successful with ensuing re-emergence of the disease. In Spain, official data on CE show an increase in the proportion of intermediate hosts with CE during the last few years, and autochthonous pediatric patients have been reported, a sign of active local transmission of disease. A similar picture emerges from data reported to the European Food Safety Authority by other European countries. Nevertheless, several crucial aspects related to CE that would help better understand and control the disease have not been tackled appropriately, in particular the emergence of infection in specific geographical areas. In this respect, while some data are missing, other data are conflicting because they come from different databases. We review the current situation of CE in Spain compared with areas in which similar problems in the CE field exist, and offer recommendations on how to overcome those limitations. Specifically, we believe that the introduction of national registries for CE with online data entry, following the example set by the European Registry for Alveolar Echinococcosis, would help streamline data collection on CE by eliminating the need for evaluating and integrating data from multiple regions, by avoiding duplication of data from patients who access several different health facilities over time, and by providing much needed clinical and epidemiological data that are currently accessible only to clinicians

Renal involvement in mitochondrial cytopathies

Mitochondrial cytopathies constitute a group of rare diseases that are characterized by their frequent multisystemic involvement, extreme variability of phenotype and complex genetics. In children, renal involvement is frequent and probably underestimated. The most frequent renal symptom is a tubular defect that, in most severe forms, corresponds to a complete De Toni-Debré-Fanconi syndrome. Incomplete proximal tubular defects and other tubular diseases have also been reported. In rare cases, patients present with chronic tubulo-interstitial nephritis or cystic renal diseases. Finally, a group of patients develop primarily a glomerular disease. These patients correspond to sporadic case reports or can be classified into two major defects, namely 3243 A>G tRNALEU mutations and coenzyme Q10 biosynthesis defects. The latter group is particularly important because it represents the only treatable renal mitochondrial defect. In this Educational Review, the principal characteristics of these diseases and the main diagnostic approaches are summarized