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Background: Consumer and research activity monitors have become popular because of their ability to quantify energy expenditure (EE) in free-living conditions. However, the accuracy of activity trackers in determining EE in people with Huntington’s Disease (HD) is unknown. Research question: Can the ActiGraph wGT3X-B or the Fitbit Charge 4 accurately measure energy expenditure during physical activity, in people with HD compared to Indirect Calorimetry (IC) (Medisoft Ergo Card)? Methods: We conducted a cross-sectional, observational study with fourteen participants with mild-moderate HD (mean age 55.7 ± 11.4 years). All participants wore an ActiGraph and Fitbit during an incremental test, running on a treadmill at 3.2 km/h and 5.2 km/h for three minutes at each speed. We analysed and compared the accuracy of EE estimates obtained by Fitbit and ActiGraph against the EE estimates obtained by a metabolic cart, using with Intra-class correlation (ICC), Bland-Altman analysis and correlation tests. Results: A significant correlation and a moderate reliability was found between ActiGraph and IC for the incremental test (r = 0.667)(ICC=0.633). There was a significant correlation between Fitbit and IC during the incremental test (r = 0.701), but the reliability was poor at all tested speeds in the treadmill walk. Fitbit significantly overestimated EE, and ActiGraph underestimated EE compared to IC, but ActiGraph estimates were more accurate than Fitbit in all tests. Significance: Compared to IC, Fitbit Charge 4 and ActiGraph wGT3X-BT have reduced accuracy in estimating EE at slower walking speeds. These findings highlight the need for population-specific algorithms and validation of activity trackers., The research leading to these results has received funding from “la Caixa” Foundation and Caja de Burgos Foundation, under agreement LCF/PR/PR18/51130008".

Introducción: La enfermedad de Huntington (EH) es un trastorno raro neurodegenerativo. La información fiable del estado nutricional, especialmente de la composición corporal, es crítica en clínica y en investigación. La facilidad de aplicación y portabilidad del análisis de la bioimpedancia de múltiples frecuencias (mfBIA) la convierten en una herramienta atractiva para medirla, pero se desconoce su precisión en la EH. Objetivo: Evaluar la precisión del mfBIA frente a la absorciometría dual de rayos X (DEXA) en la EH. Pacientes y métodos: Estudio transversal, observacional y unicéntrico. La EH se midió con la subescala motora de la escala unificada de valoración de la EH y con la capacidad funcional total. La composición corporal se valoró según la masa libre de grasa (MLG), la masa grasa (MG), el índice de masa libre de grasa (IMLG) y el índice de masa grasa (IMG). Se utilizó el coeficiente de correlación intraclase con intervalos de confianza al 95% y estimaciones de sesgo mediante gráficos de Bland-Altman. Resultados: Se incluyó a 16 pacientes, siete hombres y nueve mujeres, con edad media de 58,5 (32-68) años, capacidad funcional total de 10 (3-13) y escala unificada de valoración de la EH de 31 (7-85). La fiabilidad era alta entre el mfBIA y la DEXA para el IMLG en hombres, 0,88 (intervalo de confianza al 95%: 0,17-0,98), y mujeres, 0,9 (intervalo de confianza al 95%: 0,61-0,98); y para el IMG en hombres, 0,97 (intervalo de confianza al 95%: 0,83-0,99), y mujeres, 0,91 (intervalo de confianza al 95%: 0,68-0,98). El mfBIA sobreestimó ligeramente la MLG, la MG, el IMG y el IMLG en los hombres, pero subestimó el IMLG en las mujeres. Conclusiones: El mfBIA es un método fácil de usar, seguro, no invasivo y preciso para medir la composición corporal y el estado nutricional en pacientes con EH leve-moderada., Introduction: Huntington´s disease (HD) is a rare neurodegenerative disorder. Reliable information about nutritional status, especially body composition from individuals with HD is critical for clinical care and research. The ease of application and portability of multiple frequencies bioelectrical impedance analysis (mfBIA) make it an attractive tool for measuring body composition, but its accuracy in HD is unknown. Aim: To evaluate the accuracy of mfBIA vs. Dual X-ray absorptiometry (DEXA) in HD. Patients and methods: Cross-sectional, observational, and single-center study. HD severity was measured using motor subscale of the unified Huntington´s disease rating scale (m-UHDRS) and the total functional capacity (TFC). Body composition was measured in terms of fat-free mass (FFM), fat mass (FM), fat-free mass index (FFMI), and fat mass index (FMI). Using Bland-Altman plots, we analyzed reliability between DEXA and mfBIA using the Intraclass Correlation Coefficient with 95% confidence intervals (CI) and bias estimates for all. Results: We included 16 patients with HD, 7 men, and 9 women, median age of 58.5 (32;68) years, TFC: 10 (3;13), and m-UHDRS: 31 (7;85). The reliability between mfBIA and DEXA were high for FFMI in men: 0.88 (95% CI 0.17-0.98), and women: 0.90 (95% CI 0.61- 0.98); for FMI, men: 0.97 (95% CI 0.83-0.99), and women: 0.91 (95% CI 0.68-0.98). Compared to DEXA, mfBIA slightly overestimated FFM, FM, FMI and FFMI in men and underestimated FFMI in women. Conclusions: mfBIA is an easy-to-use, safe, non-invasive, accurate method for measuring body composition and nutritional status in patients with mild-moderate HD.

Huntington’s disease (HD) is an autosomal dominant progressive brain disorder, caused by a pathological expansion of a CAG repeat that encodes the huntingtin gene. This genetic neurodegenerative rare disease is characterized by cognitive, motor, and neuropsychiatric manifestations. The aim of the treatment is symptomatic and addresses the hyperkinetic disorders (chorea, dystonia, myoclonus, tics, etc.) and the behavioural and cognitive disturbances (depression, anxiety, psychosis, etc.) associated with the disease. HD is still a complex condition in need of innovative and efficient treatment. The long-term goal of pharmacogenetic studies is to use genotype data to predict the effective treatment response to a specific drug and, in turn, prevent potential undesirable effects of its administration. Chorea, depression, and psychotic symptoms have a substantial impact on HD patients’ quality of life and could be better controlled with the help of pharmacogenetic knowledge. We aimed to carry out a review of the available publications and evidence related to the pharmacogenetics of HD, with the objective of compiling all information that may be useful in optimizing drug administration. The impact of pharmacogenetic information on the response to antidepressants and antipsychotics is well documented in psychiatric patients, but this approach has not been investigated in HD patients. Future research should address several issues to ensure that pharmacogenetic clinical use is appropriately supported, feasible, and applicable., M.S.-R. contract was supported by the Instituto de Salud Carlos III (ISCIII), the Spanish Ministry of Science and Innovation, through the Sara Borrell Program (CD21/00022). A.S.-S. contract was funded by the Fundación HNA, 2nd edition of the Scientific Health Research Award.

Introduction: Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder characterized by motor dysfunction, cognitive impairment, and psychiatric disorders. These symptoms cause functional limitations in occupational performance. This study aimed to describe the difficulties in self-care, productivity, and leisure activities and to analyze the patients’ own perception and satisfaction and the association with sex, age, disability, HD severity, and quality of life. Method: We conducted an observational, retrospective study in 38 patients with HD without cognitive impairment at the Burgos University Hospital. We assessed occupational performance, quality of life, and HD severity with the Canadian Occupational Performance Measure, Short-Form Health Survey 12, Unified Huntington’s Disease Rating Scale, Total Functional Capacity, and the Problems Behavior Assessment. Results: One hundred sixty-five occupations (median 4, range 2–7) were categorized as self-care (50.90%), leisure (30.30%), and productivity (18.79%). Patients reported a higher degree of satisfaction with the activities prioritized than with the degree of performance. Elderly HD patients reported more satisfaction in leisure and women in self-care. Self-ratings of satisfaction in productivity were associated with higher levels of mental wellbeing. Conclusions: HD patients have problems with their daily life occupations, particularly with self-care. This underlines the need for assessment tools that capture the complexity of occupations.

To study how the visual areas of the 2 hemispheres interact in processing visual stimuli we have recorded local field potentials in the callosally connected parts of areas 17 and 18 of the ferret during the presentation of 3 kinds of stimuli: 2.5 degrees squares flashed for 50 ms randomly in the visual field (S1), 4 full-field gratings differing in orientation by 45 degrees and identical in the 2 hemifields (S2) and gratings as above but whose orientation and/or direction of motion differed by 90 degrees in the 2 hemifields (S3). The gratings remained stationary for 0.5 s and then moved in 1 of the 2 directions perpendicular to their orientation for 3 s. We compared the responses in baseline conditions with those obtained whereas the contralateral visual areas were inactivated by cooling. Cooling did not affect the responses to S1 but it modified those to S2 and to S3 generally increasing early components of the response while decreasing later components. These findings indicate that interhemispheric processing is restricted to visual stimuli which achieve spatial summation and that it involves complex inhibitory and facilitatory effects, possibly carried out by interhemispheric pathways of different conduction velocity., Depto. de Análisis Matemático y Matemática Aplicada, Fac. de Ciencias Matemáticas, TRUE, pub

Accelerometers can estimate the intensity, frequency, and duration of physical activity in healthy adults. Although thresholds to distinguish varying levels of activity intensity using the Actigraph wGT3X-B have been established for the general population, their accuracy for Huntington’s disease (HD) is unknown. We aimed to define and cross-validate accelerometer cut-points for different walking speeds in adults with mild to moderate HD. A cross-sectional, multicentre, case-control, observational study was conducted with a convenience sample of 13 symptomatic ambulatory HD participants. The accelerometer was placed around the right hip, and a heart monitor was fitted around the chest to monitor heart rate variability. Participants walked on a treadmill at three speeds with light, moderate and vigorous intensities. Correlation and receiver operation curve analyses were performed between the accelerometer magnitude vector with relative oxygen and heart rate. Optimal cut-points for walking speeds of 3.2 km/h were ≤2852; 5.2 km/h: >2852 to ≤4117, and in increments until their maximum velocity: >4117. Our results support the application of the disease-specific cut-points for quantifying physical activity in patients with mild to moderate HD and promoting healthy lifestyle interventions., The project leading to these results has received funding from “La Caixa” Foundation (ID100010434), under agreement FUI1-PI008.

El trastorno por déficit de atención e hiperactividad (TDAH) es considerado el trastorno del neurodesarrollo más presente en la vida infantil de nuestra sociedad y, actualmente es entendido como aquel que posee más comorbilidades asociadas, ya que casi un 70% de la población con TDAH presenta otro tipo de trastornos asociados. Una de ellas es el denominado trastorno por consumo de sustancias (TCS), ya que este se desarrolla en adolescentes y adultos con TDAH debido a la impulsividad y a la necesidad de buscar nuevas sensaciones. En este escrito se observará cómo mediante el método de la teoría fundamente, se pueden ligar ambos trastornos, partiendo de la impulsividad como factor de riesgo para desarrollar un TCS en personas con TDAH., Attention deficit hyperactivity disorder (ADHD) is considered the neurodevelopmental disorder most present in children's life in our society and is currently understood as the one with the most associated comorbidities, since almost 70% of the population with ADHD has other associated disorders. One of them is the so-called substance use disorder (SUD), since it develops in adolescents and adults with ADHD due to impulsivity and the need to seek new sensations. In this paper we will show how, by means of the grounded theory method, both disorders can be linked, starting from impulsivity as a risk factor for developing SCT in people with ADHD., Departamento de Pedagogía, Máster en Psicopedagogía

Los enterococos son cocos gram positivos, catalasa-negativos y anaerobios facultativos que se presentan de manera individual, en parejas o en cadenas cortas. El género Enterococcus incluye la especie Enterococcus faecalis, uno de los patógenos nosocomiales más importantes actualmente. E. faecalis puede dar lugar a infecciones de heridas, tejidos blandos y el tracto urinario. Dan lugar a cuadros de bacteriemia lo que, en caso de un daño previo de las válvulas cardiacas, puede desembocar en un proceso de endocarditis. Se han identificados varios factores como importantes en la patogénesis de la bacteria, aunque aún no se ha esclarecido el modo en el que esta bacteria pasa del comensalismo a la patogenicidad. Además, esta bacteria suele ser portadora de determinantes de resistencia tanto intrínsecos como adquiridos. Un tratamiento con cefalosporinas, a las que el enterococo es intrínsecamente resistente, supone un factor de riesgo para la adquisición de una infección enterocócica. La resistencia intrínseca a cefalosporinas es una de las más interesantes de las que presenta el enterococo. Se han identificado varios factores importantes para esta resistencia, pero la relación entre ellos y sus efectores permanecen sin dilucidar..., Enterococcus spp. are facultatively anaerobic, catalase-negative Gram-positive cocci, arranged individually, in pairs, or short chains. Enterococci include one of the most important nosocomial pathogens, Enterococcus faecalis. E. faecalis can be responsible for infections of wounds, soft tissue and the urinary tract. In some cases they produce bacteremia which can lead to endocarditis in previously damaged cardiac valves. Several factors have been identified as important in the pathogenesis of the bacteria. Nevertheless, it remains unclear how this commensal bacteria evolved into an insidious pathogen. In addition, Enterococcus faecalis harbours a large amount of both intrinsic and adquired antibiotic resistance determinants. In fact, the administration of cephalosporins is a risk factor for the adquisition of an enterococcal infection. Among the different antibiotic resistance phenotypes that the bacteria display, one of the most fascinating resistance mechanism is the intrinsic resistance to cephalosporins. Various important factors important for this intrinsic resistance have been identified, but the relationships between them and their upstream and downstream effectors remain unknown...

Mitochondria use oxygen as the final acceptor of the respiratory chain, but its incomplete reduction can also produce reactive oxygen species (ROS), especially superoxide. Acute hypoxia produces a superoxide burst in different cell types, but the triggering mechanism is still unknown. Herein, we show that complex I is involved in this superoxide burst under acute hypoxia in endothelial cells. We have also studied the possible mechanisms by which complex I could be involved in this burst, discarding reverse electron transport in complex I and the implication of PTEN-induced putative kinase 1 (PINK1). We show that complex I transition from the active to ‘deactive’ form is enhanced by acute hypoxia in endothelial cells and brain tissue, and we suggest that it can trigger ROS production through its Na/H antiporter activity. These results highlight the role of complex I as a key actor in redox signalling in acute hypoxia.

Streptococcus suis is an emerging zoonotic agent responsible for high-mortality outbreaks among the human population in China. In this species, the ABC transporter SatAB mediates fluoroquinolone resistance when overexpressed. Here, we describe and characterize satR, an open reading frame (ORF) encoding a MarR superfamily regulator that acts as a repressor of satAB. satR is cotranscribed with satAB, and its interruption entails the overexpression of the pump, leading to a clinically relevant increase in resistance to fluoroquinolones., Unión Europea. FP7, Ministerio de Ciencia e Innovación (MICINN), Comunidad de Madrid, Centro de Vigilancia Sanitaria Veterinaria (VISAVET), TRUE, pub

Unión Europea. FP7, Ministerio de Ciencia e Innovación (MICINN), Comunidad de Madrid, Centro de Vigilancia Sanitaria Veterinaria (VISAVET), TRUE, pub

Spread of antibiotic resistance among bacteria responsible for nosocomial and community-acquired infections urges for novel therapeutic or prophylactic targets and for innovative pathogen-specific antibacterial compounds. Major challenges are posed by opportunistic pathogens belonging to the low GC% gram-positive bacteria. Among those, Enterococcus faecalis is a leading cause of hospital-acquired infections associated with life-threatening issues and increased hospital costs. To better understand the molecular properties of enterococci that may be required for virulence, and that may explain the emergence of these bacteria in nosocomial infections, we performed the first large-scale functional analysis of E. faecalis V583, the first vancomycin-resistant isolate from a human bloodstream infection. E. faecalis V583 is within the high-risk clonal complex 2 group, which comprises mostly isolates derived from hospital infections worldwide. We conducted broad-range screenings of candidate genes likely involved in host adaptation (e.g., colonization and/or virulence). For this purpose, a library was constructed of targeted insertion mutations in 177 genes encoding putative surface or stress-response factors. Individual mutants were subsequently tested for their i) resistance to oxidative stress, ii) antibiotic resistance, iii) resistance to opsonophagocytosis, iv) adherence to the human colon carcinoma Caco-2 epithelial cells and v) virulence in a surrogate insect model. Our results identified a number of factors that are involved in the interaction between enterococci and their host environments. Their predicted functions highlight the importance of cell envelope glycopolymers in E. faecalis host adaptation. This study provides a valuable genetic database for understanding the steps leading E. faecalis to opportunistic virulence., Unión Europea. FP6, Ministerio de Ciencia e Innovación (MICINN), Agence Nationale de la Recherche, Fundaçaõ para a Ciência e Tecnologia, Centro de Vigilancia Sanitaria Veterinaria (VISAVET), TRUE, pub