Non-alcoholic fatty liver in hereditary fructose intolerance

Background: Non-alcoholic fatty liver disease (NAFLD) is characterized by fat accumulation affecting >5% of the liver volume that is not explained by alcohol abuse. It is known that fructose gives rise to NAFLD and it has been recently described that the ingestion of fructose in low amounts in aldolase B deficient mice is associated with the development of fatty liver. Therefore, it is reasonable that patients with HFI (Hereditary Fructose Intolerance) present fatty liver at diagnosis, but its prevalence in patients treated and with adequate follow-up is not well documented in the literature. The aim of this study is to analyze the association between HFI and NAFLD in treated patients. Methods: A cross-sectional observational study was conducted. The population comprised 16 genetically diagnosed HFI patients aged from 3 years to 48 and in dietary treatment of fructose, sorbitol and sacarose exclusion at least for two years. Blood samples were obtained for analytical studies and anthropometric measurements of each patient were performed. Results: Patients presented a Body Mass Index (BMI) of 17.9 ± 2.9 kg/m 2 . The HOMA index and Quick index were in normal range for our population. The S-adenosyl-methionine (SAM)/S-adenosyl-L-homocysteine (SAH) ratio was increased in the patients in whom this analysis was performed. By imaging techniques it was observed that 9 of the 16 patients presented fatty liver (7 by hepatic MRI). Of these 9 patients, only 3 presented hepatomegaly. 7 of 9 patients affected by the c.448G > C mutation had fatty infiltration, of which three of them presented in addition hepatomegaly. Conclusions: There is a high prevalence of fatty liver in HFI patients and it is not related to obesity and insulin resistance. The diagnosis of fatty liver in HFI patients and, above all, the identification of new therapeutic approaches, can positively impact the quality of life of these patients

Nueva metodología y recursos online para la adaptación de las prácticas de laboratorio de Óptica a escenarios docentes con diferente grado de presencialidad

Memoria ID-040 Ayudas de la Universidad de Salamanca para la innovación docente, curso 2020-2021

Palbociclib in combination with endocrine therapy versus capecitabine in hormonal receptor-positive, human epidermal growth factor 2-negative, aromatase inhibitor-resistant metastatic breast cancer: a phase III randomised controlled trial—PEARL

Background: Palbociclib plus endocrine therapy (ET) is the standard treatment of hormone receptor-positive and human epidermal growth factor receptor 2-negative, metastatic breast cancer (MBC). However, its efficacy has not been compared with that of chemotherapy in a phase III trial. Patients and methods: PEARL is a multicentre, phase III randomised study in which patients with aromatase inhibitor (AI)-resistant MBC were included in two consecutive cohorts. In cohort 1, patients were randomised 1 : 1 to palbociclib plus exemestane or capecitabine. On discovering new evidence about estrogen receptor-1 (ESR1) mutations inducing resistance to AIs, the trial was amended to include cohort 2, in which patients were randomised 1 : 1 between palbociclib plus fulvestrant and capecitabine. The stratification criteria were disease site, prior sensitivity to ET, prior chemotherapy for MBC, and country of origin. Co-primary endpoints were progression-free survival (PFS) in cohort 2 and in wild-type ESR1 patients (cohort 1 + cohort 2). ESR1 hotspot mutations were analysed in baseline circulating tumour DNA. Results: From March 2014 to July 2018, 296 and 305 patients were included in cohort 1 and cohort 2, respectively. Palbociclib plus ET was not superior to capecitabine in both cohort 2 [median PFS: 7.5 versus 10.0 months; adjusted hazard ratio (aHR): 1.13; 95% confidence interval (CI): 0.85-1.50] and wild-type ESR1 patients (median PFS: 8.0 versus 10.6 months; aHR: 1.11; 95% CI: 0.87-1.41). The most frequent grade 3-4 toxicities with palbociclib plus exemestane, palbociclib plus fulvestrant and capecitabine, respectively, were neutropenia (57.4%, 55.7% and 5.5%), hand/foot syndrome (0%, 0% and 23.5%), and diarrhoea (1.3%, 1.3% and 7.6%). Palbociclib plus ET offered better quality of life (aHR for time to deterioration of global health status: 0.67; 95% CI: 0.53-0.85). Conclusions: There was no statistical superiority of palbociclib plus ET over capecitabine with respect to PFS in MBC patients resistant to AIs. Palbociclib plus ET showed a better safety profile and improved quality of life

Acute stroke care during the COVID-19 pandemic. Ictus Madrid Program recommendations

La pandemia por COVID-19 ha obligado a una reorganización de los sistemas sanitarios y ha comportado una saturación excepcional de sus recursos. En este contexto es vital asegurar la atención al ictus agudo y optimizar los procesos asistenciales del código ictus para reducir el riesgo de contagios y racionalizar el uso de recursos hospitalarios. Para ello, desde el Grupo Multidisciplinar Ictus Madrid proponemos una serie de recomendaciones. Métodos Revisión bibliográfica no sistemática de las publicaciones disponibles con los términos «stroke» y «COVID-19» o «coronavirus» o «SARS-CoV-2», así como otras conocidas por los autores. En base a esta se redacta un documento de recomendaciones que es sometido a consenso por el Grupo Multidisciplinar Ictus Madrid y su Comité de Neurología. Resultados Las recomendaciones se estructuran en cinco líneas fundamentales: 1) coordinar la actuación para garantizar el acceso a la asistencia hospitalaria de los pacientes con ictus; 2) reconocer a los pacientes con ictus potencialmente infectados por COVID-19, 3) organización adecuada para garantizar la protección de los profesionales sanitarios frente al riesgo de contagio por COVID-19, 4) en la realización de neuroimagen y otros procedimientos que conlleven contactos de riesgo de infección COVID-19 hay que procurar reducirlos y asegurar la protección, y 5) alta y seguimiento seguros procurando optimizar la ocupación hospitalaria. Resumimos el procedimiento de forma esquemática con el acrónimo CORONA (COordinar, Reconocer, Organizar, Neuroimagen, Alta). Conclusiones Estas recomendaciones pueden servir de apoyo para la organización del sistema sanitario en la atención al ictus agudo y la optimización de sus recursos, garantizando la protección de sus profesionalesThe COVID-19 pandemic has forced a reorganization of healthcare systems and an exceptional saturation of their resources. In this context, it is vital to ensure acute stroke care and optimize the care processes of the stroke code to reduce the risk of contagion and rationalize the use of hospital resources. To do this, the Ictus Madrid Multidisciplinary Group proposes a series of recommendations. Methods Non-systematic bibliographic review of the available publications with the terms «stroke» and «COVID-19» or «coronavirus» or «SARS-CoV-2», as well as other already known for the authors. We provide a document of recommendations as a result of the consensus of the Ictus Madrid Multidisciplinary Group and its Neurology Committee. Results Our recommendations are structured on five lines: (1) coordinate to guarantee the access to hospital care for stroke patients, (2) recognize potentially COVID-19 infected stroke patients, (3) organize to ensure the protection of healthcare professionals from COVID-19 infections, (4) neuroimaging and other procedures potentially associated to risks for COVID-19 infection should be reduced and secured to avoid contagion, and (5) at home as soon as possible and supported follow-up to optimize hospital occupancy. The procedure is shown summarized under the acronym CORONA (COordinate, Recognize, Organize, Neuroimaging, At home). Conclusions These recommendations can support the organization of healthcare services for acute stroke care and the optimization of their resources, guaranteeing the protection of healthcare professional

PRIMAGE project : predictive in silico multiscale analytics to support childhood cancer personalised evaluation empowered by imaging biomarkers

PRIMAGE is one of the largest and more ambitious research projects dealing with medical imaging, artificial intelligence and cancer treatment in children. It is a 4-year European Commission-financed project that has 16 European partners in the consortium, including the European Society for Paediatric Oncology, two imaging biobanks, and three prominent European paediatric oncology units. The project is constructed as an observational in silico study involving high-quality anonymised datasets (imaging, clinical, molecular, and genetics) for the training and validation of machine learning and multiscale algorithms. The open cloud-based platform will offer precise clinical assistance for phenotyping (diagnosis), treatment allocation (prediction), and patient endpoints (prognosis), based on the use of imaging biomarkers, tumour growth simulation, advanced visualisation of confidence scores, and machine-learning approaches. The decision support prototype will be constructed and validated on two paediatric cancers: neuroblastoma and diffuse intrinsic pontine glioma. External validation will be performed on data recruited from independent collaborative centres. Final results will be available for the scientific community at the end of the project, and ready for translation to other malignant solid tumours

A review of the combination among global change factors in forests, shrublands and pastures of the Mediterranean Region : beyond drought effects

Research in CRAG is also supported byCERCA institution (Generalitat de Catalunya).Climate change, alteration of atmospheric composition, land abandonment in some areas and land use intensification in others, wildfires and biological invasions threaten forests, shrublands and pastures all over the world. However, the impacts of the combinations between global change factors are not well understood despite its pressing importance. Here we posit that reviewing global change factors combination in an exemplary region can highlight the necessary aspects in order to better understand the challenges we face, warning about the consequences, and showing the challenges ahead of us. The forests, shrublands and pastures of the Mediterranean Basin are an ideal scenario for the study of these combinations due to its spatial and temporal heterogeneity, increasing and diverse human population and the historical legacy of land use transformations. The combination of multiple global change factors in the Basin shows different ecological effects. Some interactions alter the effects of a single factor, as drought enhances or decreases the effects of atmospheric components on plant ecophysiology. Several interactions generate new impacts: drought and land use changes, among others, alter water resources and lead to land degradation, vegetation regeneration decline, and expansion of forest diseases. Finally, different factors can occur alone or simultaneously leading to further increases in the risk of fires and biological invasions. The transitional nature of the Basin between temperate and arid climates involves a risk of irreversible ecosystem change towards more arid states. However, combinations between factors lead to unpredictable ecosystem alteration that goes beyond the particular consequences of drought. Complex global change scenarios should be studied in the Mediterranean and other regions of the world, including interregional studies. Here we show the inherent uncertainty of this complexity, which should be included in any management strategy

Guide to the alien and invasive species of rivers, lakes and estuaries in the Iberian Peninsula.

Guide to the alien and invasive species of rivers, lakes and estuaries in the Iberian Peninsula

Translocated LPS Might Cause Endotoxin Tolerance in Circulating Monocytes of Cystic Fibrosis Patients

Cystic Fibrosis (CF) is an inherited pleiotropic disease that results from abnormalities in the gene codes of a chloride channel. The lungs of CF patients are chronically infected by several pathogens but bacteraemia have rarely been reported in this pathology. Besides that, circulating monocytes in CF patients exhibit a patent Endotoxin Tolerance (ET) state since they show a significant reduction of the inflammatory response to bacterial stimulus. Despite a previous description of this phenomenon, the direct cause of ET in CF patients remains unknown. In this study we have researched the possible role of microbial/endotoxin translocation from a localized infection to the bloodstream as a potential cause of ET induction in CF patients. Plasma analysis of fourteen CF patients revealed high levels of LPS compared to healthy volunteers and patients who suffer from Chronic Obstructive Pulmonary Disease. Experiments in vitro showed that endotoxin concentrations found in plasma of CF patients were enough to induce an ET phenotype in monocytes from healthy controls. In agreement with clinical data, we failed to detect bacterial DNA in CF plasma. Our results suggest that soluble endotoxin present in bloodstream of CF patients causes endotoxin tolerance in their circulating monocytes