POWERbreathe® Inspiratory Muscle Training in Amyotrophic Lateral Sclerosis.

Inspiratory muscle training may benefit respiratory function, cardiocirculatory parameters, quality of life and functionality in neuromuscular diseases. This pilot study aimed to demonstrate the POWERbreathe® inspiratory muscle training effects on maximum inspiratory pressure (PImax), heart rate (HR) and HR variability, as well as the quality of life impairment and functionality in patients with Amyotrophic Lateral Sclerosis (ALS). A pilot single-blinded, non-randomized controlled clinical trial was carried out. A total of 20T ALS patients were enrolled and divided into experimental (n = 10) and control (n = 10) groups. The experimental group received POWERbreathe® inspiratory muscle training in conjunction with usual care, and the control group received only usual care for 8 weeks. PImax (measured by POWERbreathe® KH1), HR and HR variability (evaluated by Polar H7), quality of life impairment [measured by the Amyotrophic Lateral Sclerosis Assessment Questionnaire—40 items (ALSAQ-40)] and functionality [assessed by the ALS Functional Rating Scale Revised (ALSFRS-R)] were collected at baseline and after 8 weeks of intervention. We detected statistically significant differences (p < 0.05) with an effect size ranging from medium to large (Cohen’s d = 0.72–1.37); relative to the control group, the experimental group had an increased PImax (mean difference = 10.80 cm H2O; 95% CI = 3.42–18.17) and ALSFRS-R score (mean difference = 5.30 points; 95% CI = −0.03–10.63) and reduced HR (mean difference = −8.80 beats-per-minute; 95% CI = −20.27–2.67) and R-R interval (mean difference = 78.30 ms; 95% CI = 2.89–153.70). POWERbreathe® inspiratory muscle training, in addition to usual care, may improve inspiratory strength and heart rate in patients with ALS. These results encourage larger and longer trials investigating potential clinically relevant benefits of inspiratory muscle training to these patients over the disease course.post-print6421 K

Elementos de la proyección del nudo electroenergético del citi; Projection elements of city electroenergetical node

<p>Se presentan las experiencias obtenidas en la proyecci&oacute;n del nudo electroenerg&eacute;tico de un objetivo civil llamado Centro de Investigaciones de Tecnolog&iacute;as Integradas (CITI). Este nudo consiste en una subestaci&oacute;n el&eacute;ctrica, un punto de generaci&oacute;n con grupos electr&oacute;genos y un centro general de distribuci&oacute;n (CGD), como complejo de alimentaci&oacute;n a un objetivo que exige m&aacute;xima confiabilidad en el suministro de energ&iacute;a el&eacute;ctrica.<br />Se exponen los an&aacute;lisis de opciones factibles y los esquemas que finalmente se proyectaron, sus caracter&iacute;sticas de operaci&oacute;n y evaluaciones econ&oacute;micas realizadas durante el proceso de dise&ntilde;o y proyecci&oacute;n.</p><p>&nbsp;</p><p>&nbsp;</p><p>The obtained experiences in the projection of Center of Reaserches of Integrated Technologies (CITI) electroenergetical node. This node consist in an electric substation, a generation point with generator groups and a distribution general center (CGD), as a complex feeding of an objective that demands maximum reliability in the electric power supply. The analyses of feasible options and the outlines that finally were projected, are exposed, their operation characteristics and economic evaluations carried out during the design process and projection are also presented.</p

Elementos de la proyección del nudo electroenergético del CITI

The obtained experiences in the projection of Center of Reaserches of Integrated Technologies (CITI) electroenergetical node. This node consist in an electric substation, a generation point with generator groups and a distribution general center (CGD), as a complex feeding of an objective that demands maximum reliability in the electric power supply. The analyses of feasible options and the outlines that finally were projected, are exposed, their operation characteristics and economic evaluations carried out during the design process and projection are also presented.Se presentan las experiencias obtenidas en la proyección del nudo electroenergético de un objetivo civil llamado Centro de Investigaciones de Tecnologías Integradas (CITI). Este nudo consiste en una subestación eléctrica, un punto de generación con grupos electrógenos y un centro general de distribución (CGD), como complejo de alimentación a un objetivo que exige máxima confiabilidad en el suministro de energía eléctrica. Se exponen los análisis de opciones factibles y los esquemas que finalmente se proyectaron, sus características de operación y evaluaciones económicas realizadas durante el proceso de diseño y proyección

Algunos elementos empleados en el diseño de iluminación del CITI; Some elements used in the CITI illumination design

Se presentan elementos de los estudios y proyectos realizados con un sistema de iluminaci&oacute;n novedoso en Cuba y de reciente aparici&oacute;n en un objetivo civil que se construye en la Ciudad Universitaria Jos&eacute; Antonio Echeverr&iacute;a (CUJAE), llamado Centro de Investigaciones de Tecnolog&iacute;as Integradas (CITI). Este sistema redimensiona las bases de dise&ntilde;o y conceptos que se manejan en esta especialidad, reforz&aacute;ndose los relacionados con la funcionalidad, eficiencia y sostenibilidad de cada una de sus partes y componentes como son; fuentes de luz primaria, elementos &oacute;pticos, balastos y sistemas de control.Se exponen sus caracter&iacute;sticas y posibilidades con l&aacute;mparas fluorescentes. Se esboza en qu&eacute; consiste su &ldquo;inteligencia&rdquo; y se ejemplifica con parte de los proyectos realizados, evalu&aacute;ndose las ventajas que se obtienen. La aplicaci&oacute;n de los resultados presentados reduce la potencia total instalada y la densidad de potencia por local.&nbsp;&nbsp;&nbsp;The elements of the studies and projects carried out with a novel illumination system in Cuba of recent appearance in an objective that is built in the Universitary Citi Jos&eacute; Antonio Echeverr&iacute;a (CUJAE), call Center of Reaserches of Integrated Technologies (CITI), are presented. This system resize the design bases and concepts that are managed in this specialty, being reinforced those related with the functionality, and efficiency of each one on their components as; primary light sources, optic elements, ballasts and control systems. Their characteristics and possibilities are exposed with fluorescent lamps. It is explained on what consists its "intelligence" and it is exemplified with part of the carried out projects, being evaluated the advantages that are obtained. The application of the presented results reduces the installed total power and the density of power for room.</p

Algunos elementos empleados en el diseño de iluminación del CITI

The elements of the studies and projects carried out with a novel illumination system in Cuba of recent appearance in an objective that is built in the Universitary Citi José Antonio Echeverría (CUJAE), call Center of Reaserches of Integrated Technologies (CITI), are presented. This system resize the design bases and concepts that are managed in this specialty, being reinforced those related with the functionality, and efficiency of each one on their components as; primary light sources, optic elements, ballasts and control systems. Their characteristics and possibilities are exposed with fluorescent lamps. It is explained on what consists its "intelligence" and it is exemplified with part of the carried out projects, being evaluated the advantages that are obtained. The application of the presented results reduces the installed total power and the density of power for room.Se presentan elementos de los estudios y proyectos realizados con un sistema de iluminación novedoso en Cuba y de reciente aparición en un objetivo civil que se construye en la Ciudad Universitaria José Antonio Echeverría (CUJAE), llamado Centro de Investigaciones de Tecnologías Integradas (CITI). Este sistema redimensiona las bases de diseño y conceptos que se manejan en esta especialidad, reforzándose los relacionados con la funcionalidad, eficiencia y sostenibilidad de cada una de sus partes y componentes como son; fuentes de luz primaria, elementos ópticos, balastos y sistemas de control.Se exponen sus características y posibilidades con lámparas fluorescentes. Se esboza en qué consiste su �inteligencia� y se ejemplifica con parte de los proyectos realizados, evaluándose las ventajas que se obtienen. La aplicación de los resultados presentados reduce la potencia total instalada y la densidad de potencia por local

Recurrent mutations in epigenetic regulators, RHOA and FYN kinase in peripheral T cell lymphomas

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous and poorly understood group of non Hodgkin lymphomas(1,2). Here we combined whole exome sequencing of 12 tumor-normal DNA pairs, RNAseq analysis and targeted deep sequencing to identify new genetic alterations in PTCL transformation. These analyses identified highly recurrent epigenetic factor mutations in TET2, DNMT3A and IDH2 as well as a new highly prevalent RHOA p.Gly17Val (NM_001664) mutation present in 22/35 (67%) of angioimmunoblastic T-cell lymphomas (AITL) and in 8/44 (18%) not otherwise specified PTCL (PTCL NOS) samples. Mechanistically, the RHOA Gly17Val protein interferes with RHOA signaling in biochemical and cellular assays, an effect potentially mediated by the sequestration of activated Guanine Exchange Factor (GEF) proteins. In addition, we describe new and recurrent, albeit less frequent, genetic defects including mutations in FYN, ATM, B2M and CD58 implicating SRC signaling, impaired DNA damage response and escape from immune surveillance mechanisms in the pathogenesis of PTCL

Erratum to: Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (Autophagy, 12, 1, 1-222, 10.1080/15548627.2015.1100356

non present

Paediatric COVID-19 mortality: a database analysis of the impact of health resource disparity

Background The impact of the COVID-19 pandemic on paediatric populations varied between high-income countries (HICs) versus low-income to middle-income countries (LMICs). We sought to investigate differences in paediatric clinical outcomes and identify factors contributing to disparity between countries.Methods The International Severe Acute Respiratory and Emerging Infections Consortium (ISARIC) COVID-19 database was queried to include children under 19 years of age admitted to hospital from January 2020 to April 2021 with suspected or confirmed COVID-19 diagnosis. Univariate and multivariable analysis of contributing factors for mortality were assessed by country group (HICs vs LMICs) as defined by the World Bank criteria.Results A total of 12 860 children (3819 from 21 HICs and 9041 from 15 LMICs) participated in this study. Of these, 8961 were laboratory-confirmed and 3899 suspected COVID-19 cases. About 52% of LMICs children were black, and more than 40% were infants and adolescent. Overall in-hospital mortality rate (95% CI) was 3.3% [=(3.0% to 3.6%), higher in LMICs than HICs (4.0% (3.6% to 4.4%) and 1.7% (1.3% to 2.1%), respectively). There were significant differences between country income groups in intervention profile, with higher use of antibiotics, antivirals, corticosteroids, prone positioning, high flow nasal cannula, non-invasive and invasive mechanical ventilation in HICs. Out of the 439 mechanically ventilated children, mortality occurred in 106 (24.1%) subjects, which was higher in LMICs than HICs (89 (43.6%) vs 17 (7.2%) respectively). Pre-existing infectious comorbidities (tuberculosis and HIV) and some complications (bacterial pneumonia, acute respiratory distress syndrome and myocarditis) were significantly higher in LMICs compared with HICs. On multivariable analysis, LMIC as country income group was associated with increased risk of mortality (adjusted HR 4.73 (3.16 to 7.10)).Conclusion Mortality and morbidities were higher in LMICs than HICs, and it may be attributable to differences in patient demographics, complications and access to supportive and treatment modalities

Respiratory support in patients with severe COVID-19 in the International Severe Acute Respiratory and Emerging Infection (ISARIC) COVID-19 study: a prospective, multinational, observational study

Background: Up to 30% of hospitalised patients with COVID-19 require advanced respiratory support, including high-flow nasal cannulas (HFNC), non-invasive mechanical ventilation (NIV), or invasive mechanical ventilation (IMV). We aimed to describe the clinical characteristics, outcomes and risk factors for failing non-invasive respiratory support in patients treated with severe COVID-19 during the first two years of the pandemic in high-income countries (HICs) and low middle-income countries (LMICs). Methods: This is a multinational, multicentre, prospective cohort study embedded in the ISARIC-WHO COVID-19 Clinical Characterisation Protocol. Patients with laboratory-confirmed SARS-CoV-2 infection who required hospital admission were recruited prospectively. Patients treated with HFNC, NIV, or IMV within the first 24 h of hospital admission were included in this study. Descriptive statistics, random forest, and logistic regression analyses were used to describe clinical characteristics and compare clinical outcomes among patients treated with the different types of advanced respiratory support. Results: A total of 66,565 patients were included in this study. Overall, 82.6% of patients were treated in HIC, and 40.6% were admitted to the hospital during the first pandemic wave. During the first 24 h after hospital admission, patients in HICs were more frequently treated with HFNC (48.0%), followed by NIV (38.6%) and IMV (13.4%). In contrast, patients admitted in lower- and middle-income countries (LMICs) were less frequently treated with HFNC (16.1%) and the majority received IMV (59.1%). The failure rate of non-invasive respiratory support (i.e. HFNC or NIV) was 15.5%, of which 71.2% were from HIC and 28.8% from LMIC. The variables most strongly associated with non-invasive ventilation failure, defined as progression to IMV, were high leukocyte counts at hospital admission (OR [95%CI]; 5.86 [4.83-7.10]), treatment in an LMIC (OR [95%CI]; 2.04 [1.97-2.11]), and tachypnoea at hospital admission (OR [95%CI]; 1.16 [1.14-1.18]). Patients who failed HFNC/NIV had a higher 28-day fatality ratio (OR [95%CI]; 1.27 [1.25-1.30]). Conclusions: In the present international cohort, the most frequently used advanced respiratory support was the HFNC. However, IMV was used more often in LMIC. Higher leucocyte count, tachypnoea, and treatment in LMIC were risk factors for HFNC/NIV failure. HFNC/NIV failure was related to worse clinical outcomes, such as 28-day mortality. Trial registration This is a prospective observational study; therefore, no health care interventions were applied to participants, and trial registration is not applicable