Switching to Glycerol Phenylbutyrate in 48 Patients with Urea Cycle Disorders: Clinical Experience in Spain

Background and objectives: Glycerol phenylbutyrate (GPB) has demonstrated safety and efficacy in patients with urea cycle disorders (UCDs) by means of its clinical trial program, but there are limited data in clinical practice. In order to analyze the efficacy and safety of GPB in clinical practice, here we present a national Spanish experience after direct switching from another nitrogen scavenger to GPB. Methods: This observational, retrospective, multicenter study was performed in 48 UCD patients (age 11.7 ± 8.2 years) switching to GPB in 13 centers from nine Spanish regions. Clinical, biochemical, and nutritional data were collected at three different times: prior to GPB introduction, at first follow-up assessment, and after one year of GPB treatment. Number of related adverse effects and hyperammonemic crisis 12 months before and after GPB introduction were recorded. Results: GPB was administered at a 247.8 ± 102.1 mg/kg/day dose, compared to 262.6 ± 126.1 mg/kg/day of previous scavenger (46/48 Na-phenylbutyrate). At first follow-up (79 ± 59 days), a statistically significant reduction in ammonia (from 40.2 ± 17.3 to 32.6 ± 13.9 μmol/L, p < 0.001) and glutamine levels (from 791.4 ± 289.8 to 648.6 ± 247.41 μmol/L, p < 0.001) was observed. After one year of GPB treatment (411 ± 92 days), we observed an improved metabolic control (maintenance of ammonia and glutamine reduction, with improved branched chain amino acids profile), and a reduction in hyperammonemic crisis rate (from 0.3 ± 0.7 to less than 0.1 ± 0.3 crisis/patients/year, p = 0.02) and related adverse effects (RAE, from 0.5 to less than 0.1 RAEs/patients/year p < 0.001). Conclusions: This study demonstrates the safety of direct switching from other nitrogen scavengers to GPB in clinical practice, which improves efficacy, metabolic control, and RAE compared to previous treatments.This study was funded by AECOM (Spanish Association for the Study of Inborn Errors of Metabolism). Immedica Pharma Spain funded medical writing support and article processing charges

Clonal chromosomal mosaicism and loss of chromosome Y in elderly men increase vulnerability for SARS-CoV-2

The pandemic caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2, COVID-19) had an estimated overall case fatality ratio of 1.38% (pre-vaccination), being 53% higher in males and increasing exponentially with age. Among 9578 individuals diagnosed with COVID-19 in the SCOURGE study, we found 133 cases (1.42%) with detectable clonal mosaicism for chromosome alterations (mCA) and 226 males (5.08%) with acquired loss of chromosome Y (LOY). Individuals with clonal mosaic events (mCA and/or LOY) showed a 54% increase in the risk of COVID-19 lethality. LOY is associated with transcriptomic biomarkers of immune dysfunction, pro-coagulation activity and cardiovascular risk. Interferon-induced genes involved in the initial immune response to SARS-CoV-2 are also down-regulated in LOY. Thus, mCA and LOY underlie at least part of the sex-biased severity and mortality of COVID-19 in aging patients. Given its potential therapeutic and prognostic relevance, evaluation of clonal mosaicism should be implemented as biomarker of COVID-19 severity in elderly people. Among 9578 individuals diagnosed with COVID-19 in the SCOURGE study, individuals with clonal mosaic events (clonal mosaicism for chromosome alterations and/or loss of chromosome Y) showed an increased risk of COVID-19 lethality

Hyperoxemia and excess oxygen use in early acute respiratory distress syndrome : Insights from the LUNG SAFE study

Publisher Copyright: © 2020 The Author(s). Copyright: Copyright 2020 Elsevier B.V., All rights reserved.Background: Concerns exist regarding the prevalence and impact of unnecessary oxygen use in patients with acute respiratory distress syndrome (ARDS). We examined this issue in patients with ARDS enrolled in the Large observational study to UNderstand the Global impact of Severe Acute respiratory FailurE (LUNG SAFE) study. Methods: In this secondary analysis of the LUNG SAFE study, we wished to determine the prevalence and the outcomes associated with hyperoxemia on day 1, sustained hyperoxemia, and excessive oxygen use in patients with early ARDS. Patients who fulfilled criteria of ARDS on day 1 and day 2 of acute hypoxemic respiratory failure were categorized based on the presence of hyperoxemia (PaO2 > 100 mmHg) on day 1, sustained (i.e., present on day 1 and day 2) hyperoxemia, or excessive oxygen use (FIO2 ≥ 0.60 during hyperoxemia). Results: Of 2005 patients that met the inclusion criteria, 131 (6.5%) were hypoxemic (PaO2 < 55 mmHg), 607 (30%) had hyperoxemia on day 1, and 250 (12%) had sustained hyperoxemia. Excess FIO2 use occurred in 400 (66%) out of 607 patients with hyperoxemia. Excess FIO2 use decreased from day 1 to day 2 of ARDS, with most hyperoxemic patients on day 2 receiving relatively low FIO2. Multivariate analyses found no independent relationship between day 1 hyperoxemia, sustained hyperoxemia, or excess FIO2 use and adverse clinical outcomes. Mortality was 42% in patients with excess FIO2 use, compared to 39% in a propensity-matched sample of normoxemic (PaO2 55-100 mmHg) patients (P = 0.47). Conclusions: Hyperoxemia and excess oxygen use are both prevalent in early ARDS but are most often non-sustained. No relationship was found between hyperoxemia or excessive oxygen use and patient outcome in this cohort. Trial registration: LUNG-SAFE is registered with ClinicalTrials.gov, NCT02010073publishersversionPeer reviewe

Mapping local patterns of childhood overweight and wasting in low- and middle-income countries between 2000 and 2017

A double burden of malnutrition occurs when individuals, household members or communities experience both undernutrition and overweight. Here, we show geospatial estimates of overweight and wasting prevalence among children under 5 years of age in 105 low- and middle-income countries (LMICs) from 2000 to 2017 and aggregate these to policy-relevant administrative units. Wasting decreased overall across LMICs between 2000 and 2017, from 8.4% (62.3 (55.1–70.8) million) to 6.4% (58.3 (47.6–70.7) million), but is predicted to remain above the World Health Organization’s Global Nutrition Target of <5% in over half of LMICs by 2025. Prevalence of overweight increased from 5.2% (30 (22.8–38.5) million) in 2000 to 6.0% (55.5 (44.8–67.9) million) children aged under 5 years in 2017. Areas most affected by double burden of malnutrition were located in Indonesia, Thailand, southeastern China, Botswana, Cameroon and central Nigeria. Our estimates provide a new perspective to researchers, policy makers and public health agencies in their efforts to address this global childhood syndemic

Ingles 3-HU59-201500

Today's world demands skilled professionals and English is the language of choice for communication in the global marketplace. Responding to the needs of a rapidly changing world we invite our students to learn and develop the four basic skills required in a foreign language: listening speaking reading and writing. By building these skills and exploring new ideas students will be able to understand speak read and write in English at their current level. After completion of the intermediate level students will be able to manage diverse academic texts as well as make successful oral presentations in English

Extraction and characterization of the polar lipid fraction of blackberry and passion fruit seeds oils using supercritical fluid extraction

The study of the phytochemical composition of seed oils is of upmost importance for the food and cosmetic industries, mainly considering their associated biological properties. Extraction of seed oils using supercritical fluids (SFE) is an ecological and green alternative to conventional extraction processes since it is able to provide with potent bioactive extracts, avoiding degradation and transformation of the compounds present originally in the raw material. The objective of the present work was the extraction of pure fractions of polar lipids and their chemical characterization using chromatographic techniques such as GC-MS and LC-DAD-MS/MS of blackberry (Rubus glaucus) and passion fruit (Passiflora edulis) seed oils extracted by supercritical carbon dioxide. Oleamides derived from oleic acid were identified as the main compounds in both samples; in particular, 9-octadecenamide was the major identified oleamide. Besides, the extract obtained from passion fruit showed to be a source of linoleic acid, while the SFE extract from blackberry presented important concentrations of vanillin. The chemical composition of these seed oils can be of high interest for their further use in cosmetics and food industry.Open Access funding enabled and organized by Projekt DEAL. The authors acknowledge funding from the 2014CD0021 project (I-COOP-AGROFOOD 2014) and AGL2017-89417-R project (Spanish Ministry of Science) and Laboratory Department of Universidad de Nariño (Pasto, Colombia).Peer reviewe

Ingles 1-HU57-201602

Inglés 1 es un curso general de carácter práctico ofrecido por el Área de Inglés dirigido a los estudiantes de todas las carreras y ciclos. El curso busca desarrollar las competencias comunicativas orales y escritas en el idioma inglés a nivel básico como parte del programa de cumplimiento del requisito de idioma en la universidad. El mundo de hoy exige profesionales calificados y el idioma inglés es el escogido para comunicarse en el mercado global. Respondiendo a las necesidades de un mundo que cambia rápidamente el curso invita a nuestros estudiantes a aprender y desarrollar las cuatro habilidades básicas necesarias en un idioma extranjero: escuchar hablar leer y escribir. Con la construcción de estas habilidades y la exploración de nuevas ideas los estudiantes serán capaces de entender hablar leer y escribir en inglés según el nivel en el que se encuentren

Ingles 5 (Blended)-HU285-201500

Today's world demands skilled professionals and English is the language of choice for communication in theglobal marketplace. Responding to the needs of a rapidly changing world we invite our students to learn anddevelop the four basic skills required in a foreign language: listening speaking reading and writing. By building these skills and exploring new ideas students will be able to understand speak read and write inEnglish at their current level. After completion of the intermediate level students will be able to manage diverse academic texts as well as make successful oral presentations in English

Ingles 4 Intensivo (Online)-HE37-201801

Inglés 4 es un curso general ofrecido por el Área de Inglés de la Carrera de Traducción e Interpretación Profesional. Tiene carácter práctico y está dirigido a los estudiantes de todas las carreras y ciclos. El curso busca desarrollar dos principales competencias de la UPC: Comunicación Oral y Comunicación Escrita en una segunda lengua.En la actualidad una de las competencias exigidas a los profesionales es el dominio de lenguas extranjeras y el inglés surge como la lengua más importante de comunicación en un mercado cada vez más globalizado que requiere profesionales capaces de entender hablar leer y escribir en este idioma. Este curso está diseñado para que los estudiantes a través de la exploración de nuevas ideas aprendan y desarrollen las cuatro habilidades básicas para poder comunicarse en inglés: comprensión oral comprensión de lectura expresión oral yproducción de textos al comienzo del nivel intermedio (B1) de acuerdo al Marco Común Europeo de Referencia para las Lenguas para que puedan producir diversos textos escritos y hacer presentaciones orales exitosas en inglés

Ingles 5 Intensivo (Online)-HE38-201801

Inglés 5 es un curso general ofrecido por el Área de Inglés de la Carrera de Traducción e Interpretación Profesional. Tiene carácter práctico y está dirigido a los estudiantes de todas las carreras y ciclos. El curso busca desarrollar dos principales competencias de la UPC: Comunicación Oral y Comunicación Escrita en una segunda lengua. En la actualidad una de las competencias exigidas a los profesionales es el dominio de lenguas extranjeras y el inglés surge como la lengua más importante de comunicación en un mercado cada vez más globalizado que requiere profesionales capaces de entender hablar leer y escribir en este idioma. Este curso está diseñado para que los estudiantes a través de la exploración de nuevas ideas aprendan y desarrollen las cuatro habilidades básicas para poder comunicarse en inglés: comprensión oral comprensión de lectura expresión oral y producción de textos al nivel intermedio (B1) de acuerdo al Marco Común Europeo de Referencia para las Lenguas para que puedan producir diversos textos escritos y hacer presentaciones orales exitosas en inglés