Decision making and results analysis of the management of general management department of Brenes company in the Market Sejd1920b

This Final Degree Project aims to show the functioning of the enterprise called Brenes during the simulation process carried out for the period of time between January 2020 and December 2023. Specifically, the development of the department of General Management led by the CEO. In order to do so, first the main characteristics and particularities of the company will be discussed. That is: the company itself, highlighting the foundation of the company and the general aspects such as mission, vision and values; the products they offer and the markets in which Brenes operates. Then, the department managed, the General Management one, will be considered. To do so the structure of the area will be explained. The methodologies used in the decision-making process will be illustrated as well as the objectives and main decisions that need to be made each period by the CEO. Within this section, it will be also reviewed the relationships that the role of the CEO has with the other departments of the company. Afterwards, the results of the company during the period selected will be shown by classifying them into different categories to facilitate their analysis. In addition, an assessment of each decision made will be offered as well as graphical representations of them. To conclude, it will be verified in the last section of the project if the objectives that were intended to be achieved through it, have been achieved.Administración de Empresa

Gene therapy for neuronopathic mucopolysaccharidoses : state of the art

The need for long-lasting and transformative therapies for mucopolysaccharidoses (MPS) cannot be understated. Currently, many forms of MPS lack a specific treatment and in other cases available therapies, such as enzyme replacement therapy (ERT), do not reach important areas such as the central nervous system (CNS). The advent of newborn screening procedures represents a major step forward in early identification and treatment of individuals with MPS. However, the treatment of brain disease in neuronopathic MPS has been a major challenge to date, mainly because the blood brain barrier (BBB) prevents penetration of the brain by large molecules, including enzymes. Over the last years several novel experimental therapies for neuronopathic MPS have been investigated. Gene therapy and gene editing constitute potentially curative treatments. However, despite recent progress in the field, several considerations should be taken into account. This review focuses on the state of the art of in vivo and ex vivo gene therapy-based approaches targeting the CNS in neuronopathic MPS, discusses clinical trials conducted to date, and provides a vision for the future implications of these therapies for the medical community. Recent advances in the field, as well as limitations relating to efficacy, potential toxicity, and immunogenicity, are also discussed

Gene Therapy for Neuronopathic Mucopolysaccharidoses: State of the Art

Virus adeno associat; Lentivirus; Vectors viralsVirus adenoasociado; Lentivirus; Vectores viralesAdeno-associated virus; Lentivirus; Viral vectorsThe need for long-lasting and transformative therapies for mucopolysaccharidoses (MPS) cannot be understated. Currently, many forms of MPS lack a specific treatment and in other cases available therapies, such as enzyme replacement therapy (ERT), do not reach important areas such as the central nervous system (CNS). The advent of newborn screening procedures represents a major step forward in early identification and treatment of individuals with MPS. However, the treatment of brain disease in neuronopathic MPS has been a major challenge to date, mainly because the blood brain barrier (BBB) prevents penetration of the brain by large molecules, including enzymes. Over the last years several novel experimental therapies for neuronopathic MPS have been investigated. Gene therapy and gene editing constitute potentially curative treatments. However, despite recent progress in the field, several considerations should be taken into account. This review focuses on the state of the art of in vivo and ex vivo gene therapy-based approaches targeting the CNS in neuronopathic MPS, discusses clinical trials conducted to date, and provides a vision for the future implications of these therapies for the medical community. Recent advances in the field, as well as limitations relating to efficacy, potential toxicity, and immunogenicity, are also discussed.This research received no external funding

Genomic insights into drug resistance and virulence platforms, CRISPR-Cas systems and phylogeny of commensal E. Coli from wildlife

Commensal bacteria act as important reservoirs of virulence and resistance genes. However, existing data are generally only focused on the analysis of human or human-related bacterial populations. There is a lack of genomic studies regarding commensal bacteria from hosts less exposed to antibiotics and other selective forces due to human activities, such as wildlife. In the present study, the genomes of thirty-eight E. coli strains from the gut of various wild animals were sequenced. The analysis of their accessory genome yielded a better understanding of the role of the mobilome on inter-bacterial dissemination of mosaic virulence and resistance plasmids. The study of the presence and composition of the CRISPR/Cas systems in E. coli from wild animals showed some viral and plasmid sequences among the spacers, as well as the relationship between CRISPR/Cas and E. coli phylogeny. Further, we constructed a single nucleotide polymorphisms-based core tree with E. coli strains from different sources (humans, livestock, food and extraintestinal environments). Bacteria from humans or highly human-influenced settings exhibit similar genetic patterns in CRISPR-Cas systems, plasmids or virulence/resistance genes-carrying modules. These observations, together with the absence of significant genetic changes in their core genome, suggest an ongoing flow of both mobile elements and E. coli lineages between human and natural ecosystems.Funding: This work was partially supported by project SAF2016-76571-R and PID2019-106158RB-I00 from the Agencia Estatal de Investigación (AEI) of Spain and the Fondo Europeo de Desarrollo Regional (FEDER) of EU. During the experimental work of this study, Carla Andrea Alonso Alonso had a predoctoral fellowship FPI from MINECO

Refuerzo de pilares con tejidos de F.C.

Los pilares son los elementos estructurales más sensibles de una estructura, por lo que resulta muy frecuente su refuerzo. En el presente trabajo se realiza una campaña de ensayos de pilares en modelo reducido, para este fin. Se construyeron 30 pilares en modelo reducido, 15 de ellos de sección cuadrada y 15 de sección circular. En cada caso se formaron 3 lotes, 5 pilares sin refuerzo (pilares testigo), 5 pilares con una capa de refuerzo y 5 pilares con dos capas de refuerzo. Los ensayos se realizaron en las instalaciones de GEOCISA. El trabajo comprende un estudio teórico-experimental del comportamiento mecánico de pilares de hormigón armado reforzados por confinamiento mediante la técnica de adhesión de tejidos de fibra de carbono, sometidos a una carga axial. Se describen los modos de fallo de forma comparativa entre los distintos lotes, para plantear finalmente una serie de conclusiones y recomendaciones

Monitoring of killer yeast populations in mixed cultures: influence of incubation temperature of microvinifications samples

Killer yeasts are frequently used to combat and prevent contamination by wild-type yeasts during wine production and they can even dominate the wine fermentation. Stuck and sluggish fermentations can be caused by an unbalanced ratio of killer to sensitive yeasts in the bioreactor, and therefore it is important to determine the proportion of both populations. The aim of this study was to provide a simple tool to monitor killer yeast populations during controlled mixed microvinifications of killer and sensitive Saccharomyces cerevisiae. Samples were periodically extracted during vinification, seeded on Petri dishes and incubated at 25 and 37 °C; the latter temperature was assayed for possible inactivation of killer toxin production. Colonies developed under the described conditions were randomly transferred to killer phenotype detection medium. Significant differences in the killer/sensitive ratio were observed between both incubation temperatures in all microvinifications. These results suggest that 37 °C seems a better option to determine the biomass of sensitive yeasts, in order to avoid underestimation of sensitive cells in the presence of killer yeasts during fermentations. Incubation at a toxin-inhibiting temperature clearly showed the real ratio of killer to sensitive cells in fermentation systems.Fil: Maturano, Yolanda Paola. Universidad Nacional de San Juan. Instituto de Biotecnología; ArgentinaFil: Nally, María Cristina. Universidad Nacional de San Juan. Instituto de Biotecnología; ArgentinaFil: Toro, María Eugenia. Universidad Nacional de San Juan. Instituto de Biotecnología; ArgentinaFil: Castellanos de Figueroa, Lucía Inés. PROIMI; Argentina. Universidad Nacional de Tucumán. FBQyF; ArgentinaFil: Combina, Mariana. Instituto Nacional de Tecnología Agropecuaria (INTA). Estación Experimental Agropecuaria Mendoza; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Vazquez, Fabio. Universidad Nacional de San Juan. Instituto de Biotecnología; Argentin

The Tetrahymena metallothionein gene family: twenty-one new cDNAs, molecular characterization, phylogenetic study and comparative analysis of the gene expression under different abiotic stressors

Background: Ciliate metallothioneins (MTs) are included in family 7 of the MT superfamily. This family has been divided into two main subfamilies: 7a or CdMTs and 7b or CuMTs. All ciliate MTs reported have been isolated from different Tetrahymena species and present unique features with regard to standard MTs. Likewise, an expression analysis has been carried out on some of MT genes under metal stress, corroborating their classification into two subfamilies. Results: We isolated 21 new cDNAs from different Tetrahymena species to obtain a wider view of the biodiversity of these conserved genes. Structural analysis (cysteine patterns) and an updated phylogenetic study both corroborated the previous classification into two subfamilies. A new CuMT from a Tetrahymena-related species Ichthyophthirius multifiliis was also included in this general analysis. We detected a certain tendency towards the presentation of a CdMT tri-modular structure in Borealis group species with respect to Australis group. We report for the first time a semi-complete paralog duplication of a CdMT gene originating a new CdMT gene isoform in T. malaccensis. An asymmetry of the codon usage for glutamine residues was detected between Cd- and CuMTs, and the phylogenetic implications are discussed. A comparative gene expression analysis of several MT genes by qRTPCR revealed differential behavior among them under different abiotic stressors in the same Tetrahymena species. Conclusions: The Tetrahymena metallothionein family represents a quite conserved proteinstructure group with unique features with respect to standard MTs. Both Cd- and CuMT subfamilies present very defined and differentiated characteristics at several levels: cysteine patterns, modular structure, glutamine codon usage and gene expression under metal stress, among others. Gene duplication through evolution seems to be the major genetic mechanism for creating new MT gene isoforms and increasing their functional diversity

Beckwith Wiedemann Syndrome : presentation of a case report

Beckwith Wiedemann Syndrome (BWS) is a rare congenital disease of low prevalence. However, it presents a high prevalence within the genetic pathologies of overgrowth. This syndrome presents typical manifestations such as macroglossia, macrosomy at birth, omphalocele and defects of the anterior abdominal wall. Its origin is known to be genetic, but its mechanism of generation is not clear. This syndrome has been the object of wide studies since investigators have established a relationship between the methods of assisted fertilization (assisted reproduction treatment, ART) and its appearance. Currently, research is oriented towards the improvement of the prenatal diagnostic techniques, which would allow a preparation of the multidisciplinary medical team to treat the pathologies with which these patients are born. Next we present the case of a 1 year-old child who consults this service with a diagnosis of macroglossia associated with BWS

Healthcare Experience and their Relationship with Demographic, Disease and Healthcare-Related Variables: A Cross-Sectional Survey of Patients with Chronic Diseases Using the IEXPAC Scale

Health Administration; Public Health; Quality of Life ResearchAdministración sanitaria; Salud pública; Investigación de calidad de vidaAdministració sanitària; Salut pública; Investigació sobre la qualitat de vidaBackground Patient experience is acknowledged as a principal aspect of quality healthcare delivery, and it has implications with regard to outcomes. Objectives Our objective was to evaluate the healthcare experience of patients with chronic diseases to identify patient-perceived healthcare gaps and to assess the influence of demographic and healthcare-related variables on patient experiences. Methods A cross-sectional survey was delivered to adult patients with chronic diseases: diabetes mellitus (DM), human immunodeficiency virus (HIV) infection, inflammatory bowel disease (IBD) or rheumatic diseases. Patient experiences were assessed with the Instrument for Evaluation of the Experience of Chronic Patients (IEXPAC) questionnaire, with possible scores ranging from 0 (worst) to 10 (best experience). Results Of the 2474 patients handed the survey, 1618 returned it (response rate 65.4%). Patients identified gaps in healthcare related mainly to access to reliable information and services, interaction with other patients and continuity of healthcare after hospital discharge. The mean ± standard deviation (SD) IEXPAC score was 6.0 ± 1.9 and was higher for patients with HIV (6.6 ± 1.7) than for those with rheumatic disease (5.5 ± 2.0), IBD (5.9 ± 2.0) or DM (5.9 ± 1.9) (p < 0.001). In multivariate models, better overall IEXPAC experience was associated with follow-up by the same physician, follow-up by a nurse, receiving healthcare support from others and treatment with subcutaneous or intravenous drugs. The multivariate model that confirmed patients with HIV or DM had better experience than did those with rheumatic diseases. Conclusions Through IEXPAC, patients identified aspects for healthcare quality improvements and circumstances associated with better experience, which may permit greater redirection of healthcare toward patient-centered goals while facilitating improvements in social care and long-term healthcare quality.This project was funded by Merck Sharp and Dohme, Spain