Peatlands in the Toledo Mountains (central Spain): characterisation and conservation status.

We have limited knowledge about the biodiversity and vulnerability of peatlands in the lower-altitude mountains of the Iberian Peninsula, largely because peatlands have not been routinely differentiated from other habitat types in Spain. Understanding is now developing about the ecology of peatlands in central Spain, but they are already under severe threat of conversion and degradation. In this article we describe the results of a field survey and literature review study to characterise and describe the current condition of peatlands in the Toledo Mountains in terms of their typology, the representation of habitat types protected by European (EU) designations, threats, endangered plant species, and conservation status. Our results suggest that the principal threats are overgrazing by domestic animals and ungulates (red deer and wild boar), fire, expansion of cereal crops, drying-out and erosion. Disturbance is most severe in areas that are not protected by nature conservation designations, where peatlands are especially vulnerable to damage arising from human activities

Peatlands in the Toledo Mountains (central Spain): characterisation and conservation status

SUMMARY We have limited knowledge about the biodiversity and vulnerability of peatlands in the lower-altitude mountains of the Iberian Peninsula, largely because peatlands have not been routinely differentiated from other habitat types in Spain. Understanding is now developing about the ecology of peatlands in central Spain, but they are already under severe threat of conversion and degradation. In this article we describe the results of a field survey and literature review study to characterise and describe the current condition of peatlands in the Toledo Mountains in terms of their typology, the representation of habitat types protected by European (EU) designations, threats, endangered plant species, and conservation status. Our results suggest that the principal threats are overgrazing by domestic animals and ungulates (red deer and wild boar), fire, expansion of cereal crops, drying-out and erosion. Disturbance is most severe in areas that are not protected by nature conservation designations, where peatlands are especially vulnerable to damage arising from human activities

SEOM clinical guideline for treatment of kidney cancer (2017)

The goal of this article is to provide recommendations about the management of kidney cancer. Based on pathologic and molecular features, several kidney cancer variants were described. Nephron-sparing techniques are the gold standard of localized disease. After a randomized trial, sunitinib could be considered in adjuvant treatment in high-risk patients. Patients with advanced disease constitute a heterogeneous population. Prognostic classification should be considered. Both sunitinib and pazopanib are the standard options for first-line systemic therapy in advanced renal cell carcinoma. Based on the results of two randomized trials, both nivolumab and cabozantinib should be considered the standard for second and further lines of therapy. Response evaluation for present therapies is a challenge

Escala AUTODDIS: Evaluación de la autodeterminación de jóvenes y adultos con discapacidad intelectual. Manual de aplicación y corrección

[ES]La Escala AUTODDIS que se presenta en este manual permite evaluar la autodeterminación de jóvenes y adultos con discapacidad intelectual mediante un heteroinforme; es decir, a través de la información proporcionada por una tercera persona que conoce bien a la persona con discapacidad intelectual evaluada. La utilización de la Escala AUTODDIS permitirá el desarrollo de iniciativas ajustadas orientadas a la promoción de su autodeterminación, fomentando así el aumento necesario de prácticas basadas en evidencias empíricas

Homozygous R136S mutation in PRNP gene causes inherited early onset prion disease

Altres ajuts: Fundació la Marató de TV3/201821-31Background: More than 40 pathogenic heterozygous PRNP mutations causing inherited prion diseases have been identified to date. Recessive inherited prion disease has not been described to date. Methods: We describe the clinical and neuropathological data of inherited early-onset prion disease caused by the rare PRNP homozygous mutation R136S. In vitro PrP propagation studies were performed using recombinant-adapted protein misfolding cyclic amplification technique. Brain material from two R136S homozygous patients was intracranially inoculated in TgMet129 and TgVal129 transgenic mice to assess the transmissibility of this rare inherited form of prion disease. Results: The index case presented symptoms of early-onset dementia beginning at the age of 49 and died at the age of 53. Neuropathological evaluation of the proband revealed abundant multicentric PrP plaques and Western blotting revealed a ~ 8 kDa protease-resistant, unglycosylated PrP fragment, consistent with a Gerstmann-Sträussler-Scheinker phenotype. Her youngest sibling suffered from progressive cognitive decline, motor impairment, and myoclonus with onset in her late 30s and died at the age of 48. Genetic analysis revealed the presence of the R136S mutation in homozygosis in the two affected subjects linked to homozygous methionine at codon 129. One sibling carrying the heterozygous R136S mutation, linked to homozygous methionine at codon 129, is still asymptomatic at the age of 74. The inoculation of human brain homogenates from our index case and an independent case from a Portuguese family with the same mutation in transgenic mice expressing human PrP and in vitro propagation of PrP studies failed to show disease transmissibility. Conclusion: In conclusion, biallelic R136S substitution is a rare variant that produces inherited early-onset human prion disease with a Gerstmann-Sträussler-Scheinker neuropathological and molecular signature. Even if the R136S variant is predicted to be "probably damaging", heterozygous carriers are protected, at least from an early onset providing evidence for a potentially recessive pattern of inheritance in human prion diseases

Altered innate immune profile in blood of systemic mastocytosis patients

[Background]: Mast cells (MC) from systemic mastocytosis (SM) patients release MC mediators that lead to an altered microenvironment with potential consequences on innate immune cells, such as monocytes and dendritic cells (DC). Here we investigated the distribution and functional behaviour of different populations of blood monocytes and DC among distinct diagnostic subtypes of SM. [Methods]: Overall, we studied 115 SM patients - 45 bone marrow mastocytosis (BMM), 61 indolent SM (ISM), 9 aggressive SM (ASM)- and 32 healthy donors (HD). Spontaneous and in vitro-stimulated cytokine production by blood monocytes, and their plasma levels, together with the distribution of different subsets of blood monocytes and DCs, were investigated. [Results]: SM patients showed increased plasma levels and spontaneous production by blood monocytes of IL1β, IL6, IL8, TNFα and IL10, associated with an exhausted ability of LPS + IFNγ-stimulated blood monocytes to produce IL1β and TGFβ. SM (particularly ISM) patients also showed decreased counts of total monocytes, at the expense of intermediate monocytes and non-classical monocytes. Interestingly, while ISM and ASM patients had decreased numbers of plasmacytoid DC and myeloid DC (and their major subsets) in blood, an expansion of AXL+ DC was specifically encountered in BMM cases. [Conclusion]: These results demonstrate an altered distribution of blood monocytes and DC subsets in SM associated with constitutive activation of functionally impaired blood monocytes and increased plasma levels of a wide variety of inflammatory cytokines, reflecting broad activation of the innate immune response in mastocytosis.This study has been funded by Instituto de Salud Carlos III (ISCIII) (grant number PI19/01166; and Centro de Investigación Biomédica en Red de Cáncer [CIBERONC] programme, grant number CB16/12/00400) and co-funded by the European Union (EU). We thank the support of the Spanish National DNA Bank Carlos III (www.bancoadn.org; biobank ID B.0000716; supported by ISCIII and co-founded by EU [grant number PT20/00085]) for providing plasma samples. APP was supported by a grant of the Government of Castilla y León (Orden EDU/556/2019), Spain; co-financed with the “European Regional Development Fund” (BDNS, Identif.:422058). We thank the support of the Spanish Association of Mastocytosis and Related Diseases

Mating and aggregative behaviors among basal hexapods in the Early Cretaceous

Among the many challenges in paleobiology is the inference and reconstruction of behaviors that rarely, if ever, leave a physical trace on the environment that is suitable for fossilization. Of particular significance are those behaviors tied to mating and courtship, individual interactions critical for species integrity and continuance, as well as those for dispersal, permitting the taxon to expand its distribution as well as access new habitats in the face of local or long-term environmental change. In this context, two recently discovered fossils from the Early Cretaceous amber of Spain (ca. 105 mya) give a detailed view of otherwise fleeting ethologies in Collembola. These occurrences are phylogenetically spaced across the class, and from species representing the two major clades of springtailsÐSymphypleona and Entomobryomorpha. Specifically, we report unique evidence from a symphypleonan male (Pseudosminthurides stoechus SaÂnchez-GarcõÂa & Engel, 2016) with modified antennae that may have functioned as a clasping organ for securing females during mating on water's surface, and from an aggregation of entomobryomorphan individuals (Proisotoma communis Sánchez-García & Engel, 2016) purportedly representing a swarming episode on the forest floor. We demonstrate that the mating behavioral repertoire in P. stoechus, which is associated with considerable morphological adaptations, likely implied elaborate courtship and maneuvering for guarantee sperm transfer in an epineustic species. These discoveries reveal significant behaviors consistent with modern counterparts and a generalized stasis for some ancient hexapod ethologies associated with complex mating and courtship and social or pre-social aggregations, so critical to specific constancy and dispersal

MAPPING COMMUNITY INTEREST HABITATS IN THE COLUMBRETES ARCHIPELAGO, AN EXTRAORDINARY HOT SPOT OF BIODIVERSITY

The Columbretes Archipelago and their submerged surroundings are part of an unusual, Pleistocene volcanic field located in the Western Mediterranean designated as a Site of Community Importance (SCI) of the Natura 2000 Network. In the present study, 4 benthic habitats of community interest (1110, 1170, 1180 and 8330) have been identified by analyzing several sources of information. Generalized additive models (GAMs) have been used to model the potential distribution of reefs (1170) and maërl beds (1110). Our results highlight the diversity and extent of these habitats and allow comparisons to other marine SCIs of Spain. This can be attributed to the variability of the environment of this site. The Columbretes Islands combine a relatively shallow environment with volcanic structures, hydrothermalism with active degassing, current-driven sedimentary lobes and the influence of inland flows. Understanding high biodiversity spots is crucial as they offer natural laboratories to describe how ecosystems respond to the effects of global change. The knowledge obtained will be of paramount importance for the conservation of species and habitats. Furthermore, it will establish a baseline for future monitoring and assist in the development of effective management plans

Overview of recent TJ-II stellarator results

The main results obtained in the TJ-II stellarator in the last two years are reported. The most important topics investigated have been modelling and validation of impurity transport, validation of gyrokinetic simulations, turbulence characterisation, effect of magnetic configuration on transport, fuelling with pellet injection, fast particles and liquid metal plasma facing components. As regards impurity transport research, a number of working lines exploring several recently discovered effects have been developed: the effect of tangential drifts on stellarator neoclassical transport, the impurity flux driven by electric fields tangent to magnetic surfaces and attempts of experimental validation with Doppler reflectometry of the variation of the radial electric field on the flux surface. Concerning gyrokinetic simulations, two validation activities have been performed, the comparison with measurements of zonal flow relaxation in pellet-induced fast transients and the comparison with experimental poloidal variation of fluctuations amplitude. The impact of radial electric fields on turbulence spreading in the edge and scrape-off layer has been also experimentally characterized using a 2D Langmuir probe array. Another remarkable piece of work has been the investigation of the radial propagation of small temperature perturbations using transfer entropy. Research on the physics and modelling of plasma core fuelling with pellet and tracer-encapsulated solid-pellet injection has produced also relevant results. Neutral beam injection driven Alfvénic activity and its possible control by electron cyclotron current drive has been examined as well in TJ-II. Finally, recent results on alternative plasma facing components based on liquid metals are also presented. ISSN:0029-5515 ISSN:1741-432

SARS-CoV-2 viral load in nasopharyngeal swabs is not an independent predictor of unfavorable outcome

The aim was to assess the ability of nasopharyngeal SARS-CoV-2 viral load at first patient’s hospital evaluation to predict unfavorable outcomes. We conducted a prospective cohort study including 321 adult patients with confirmed COVID-19 through RT-PCR in nasopharyngeal swabs. Quantitative Synthetic SARS-CoV-2 RNA cycle threshold values were used to calculate the viral load in log10 copies/mL. Disease severity at the end of follow up was categorized into mild, moderate, and severe. Primary endpoint was a composite of intensive care unit (ICU) admission and/or death (n = 85, 26.4%). Univariable and multivariable logistic regression analyses were performed. Nasopharyngeal SARS-CoV-2 viral load over the second quartile (≥ 7.35 log10 copies/mL, p = 0.003) and second tertile (≥ 8.27 log10 copies/mL, p = 0.01) were associated to unfavorable outcome in the unadjusted logistic regression analysis. However, in the final multivariable analysis, viral load was not independently associated with an unfavorable outcome. Five predictors were independently associated with increased odds of ICU admission and/or death: age ≥ 70 years, SpO2, neutrophils > 7.5 × 103/µL, lactate dehydrogenase ≥ 300 U/L, and C-reactive protein ≥ 100 mg/L. In summary, nasopharyngeal SARS-CoV-2 viral load on admission is generally high in patients with COVID-19, regardless of illness severity, but it cannot be used as an independent predictor of unfavorable clinical outcome