26 results match your criteria: "Research Institute Valdecilla (IDIVAL)[Affiliation]"

Article Synopsis
  • Spinal muscular atrophy (SMA) is caused by a lack of survival motor neuron (SMN) protein, leading to the degeneration of lower motor neurons and muscle atrophy.
  • Early research in SMNΔ7 mice showed signs of motor dysfunction and myofiber changes before muscle denervation occurred, highlighting that SMN deficiency has direct effects on muscle cells.
  • The study revealed ultrastructural abnormalities in muscle fibers, including disruptions in the myofibrillar structure and altered mitochondrial dynamics, suggesting that muscle changes occur independently of motor neuron loss in SMA.
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Global differences in the management of alcohol-associated hepatitis.

Lancet Gastroenterol Hepatol

November 2024

Department of Gastroenterology, School of Medicine, Pontifical Catholic University of Chile, Santiago, Chile; Observatorio Multicéntrico de Enfermedades Gastrointestinales (OMEGA), Santiago, Chile; Division of Gastroenterology, Hepatology, and Nutrition, Department of Internal Medicine, Virginia Commonwealth University School of Medicine, Richmond, VA 23284, USA. Electronic address:

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Background: Treatment-resistant depression (TRD) is defined as the failure of at least two antidepressants in adequate doses and timing during a major depressive episode. Esketamine intranasal (ESK-IN) has been approved by the Food and Drug Administration and the European Medicines Agency for the treatment of TRD in combination with other antidepressants.

Aims: To assess the effectiveness and tolerability of a sample of TRD patients who received treatment with ESK-IN as part of the compassionate use program.

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Purpose: Metabolic bone disease of prematurity (MBDP) remains a significant cause of morbidity in extremely premature newborns. In high-risk patients, suspected diagnosis and subsequent treatment modifications, with limitations in terms of sensitivity and specificity, rely on low phosphorus levels and/or high levels of alkaline phosphatase (ALP). We investigated the potential of fibroblast growth factor-23 (FGF23) as an early marker for MBDP when measured at 3-4 weeks of life in at-risk patients.

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An artificial intelligence-generated model predicts 90-day survival in alcohol-associated hepatitis: A global cohort study.

Hepatology

November 2024

Departamento de Gastroenterología, Escuela de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

Background And Aims: Alcohol-associated hepatitis (AH) poses significant short-term mortality. Existing prognostic models lack precision for 90-day mortality. Utilizing artificial intelligence in a global cohort, we sought to derive and validate an enhanced prognostic model.

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Supplementation for Performance and Health in Patients with Phenylketonuria: An Exercise-Based Approach to Improving Dietary Adherence.

Nutrients

February 2024

Asociación Española para el Estudio de los Errores Congénitos del Metabolismo (AECOM)-AECOM&Sociedad, 28221 Majadahonda, Spain.

Supplementation is crucial for improving performance and health in phenylketonuria (PKU) patients, who face dietary challenges. Proteins are vital for athletes, supporting muscle growth, minimizing catabolism, and aiding muscle repair and glycogen replenishment post-exercise. However, PKU individuals must limit phenylalanine (Phe) intake, requiring supplementation with Phe-free amino acids or glycomacropeptides.

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Contemporary use of cefazolin for MSSA infective endocarditis: analysis of a national prospective cohort.

Int J Infect Dis

December 2023

Department of Infectious Diseases, Microbiology and Parasitology, Institute of Biomedicine of Seville, University Hospital Virgen del Rocío, Spanish National Research Council, University of Seville, Spain; CIBER de Enfermedades Infecciosas (CIBERINFEC), Instituto de Salud Carlos III, Madrid, Spain.

Objectives: This study aimed to assess the real use of cefazolin for methicillin-susceptible Staphylococcus aureus (MSSA) infective endocarditis (IE) in the Spanish National Endocarditis Database (GAMES) and to compare it with antistaphylococcal penicillin (ASP).

Methods: Prospective cohort study with retrospective analysis of a cohort of MSSA IE treated with cloxacillin and/or cefazolin. Outcomes assessed were relapse; intra-hospital, overall, and endocarditis-related mortality; and adverse events.

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Human milk (HM) offers important nutritional benefits. However, except for phenylketonuria (PKU), there are little data on optimal levels of consumption of HM and a special formula free of disease-related amino acids (SF-AA) in infants with inborn errors of metabolism of amino acids and proteins (IEM-AA-P). We designed a spreadsheet to calculate the amounts of SF-AA and HM required to cover amino acid, protein, and energy needs in patients with the nine main IEM-AA-P in infants aged under 6 months.

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MELD 3.0 adequately predicts mortality and renal replacement therapy requirements in patients with alcohol-associated hepatitis.

JHEP Rep

August 2023

Departamento de Gastroenterología, Escuela de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

Background & Aims: Model for End-Stage Liver Disease (MELD) score better predicts mortality in alcohol-associated hepatitis (AH) but could underestimate severity in women and malnourished patients. Using a global cohort, we assessed the ability of the MELD 3.0 score to predict short-term mortality in AH.

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Background: The pathogenicity of the different genetic variants causing hypertrophic cardiomyopathy (HCM) and the genotype/phenotype correlations are difficult to assess in clinical practice, as most mutations are unique or identified in non-informative families. Pathogenic variants in the sarcomeric gene inherited with an autosomal dominant pattern, whereas incomplete and age-dependent penetrance are the most common causes of HCM.

Methods: We describe the clinical characteristics of a new truncating variant, p.

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Introduction: Topographical memory is crucial for navigation and environmental representation. The Walking Corsi Test (WalCT) has been used to evaluate topographical memory in children from 4 years upward. The present study aims to determine whether adapted versions of the WalCT- by simplifying instructions and increasing motivation- can be adopted to test topographical memory in 2- and 3-year-old toddlers born at term and preterm.

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Background And Aims: Detecting NASH remains challenging, while at-risk NASH (steatohepatitis and F≥ 2) tends to progress and is of interest for drug development and clinical application. We developed prediction models by supervised machine learning techniques, with clinical data and biomarkers to stage and grade patients with NAFLD.

Approach And Results: Learning data were collected in the Liver Investigation: Testing Marker Utility in Steatohepatitis metacohort (966 biopsy-proven NAFLD adults), staged and graded according to NASH CRN.

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Coaxial Synthesis of PEI-Based Nanocarriers of Encapsulated RNA-Therapeutics to Specifically Target Muscle Cells.

Biomolecules

July 2022

Research Group on Food, Nutritional Biochemistry and Health, Universidad Europea del Atlántico, 39011 Santander, Spain.

In this work, we performed a methodological comparative analysis to synthesize polyethyleneimine (PEI) nanoparticles using (i) conventional nanoprecipitation (NP), (ii) electrospraying (ES), and (iii) coaxial electrospraying (CA). The nanoparticles transported antisense oligonucleotides (ASOs), either encapsulated (CA nanocomplexes) or electrostatically bound externally (NP and ES nanocomplexes). After synthesis, the PEI/ASO nanoconjugates were functionalized with a muscle-specific RNA aptamer.

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The map and global disease burden of chronic liver diseases are markedly changing, with nonalcoholic fatty liver disease (NAFLD) becoming the most common cause of liver diseases coinciding with the current epidemics of obesity, type 2 diabetes, and metabolic syndrome. Understanding the incidence and prevalence of NAFLD is critical because of its linkage to a significant economic burden of hospitalization and changing patterns in consequences, such as liver transplantation. Moreover, the long-term average health care expenses of NAFLD patients have exceeded those of other liver diseases.

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A Global Systematic Review of Hepatitis C Elimination Efforts through Micro-Elimination.

Semin Liver Dis

May 2022

Division of Molecular and Clinical Medicine, University of Dundee School of Medicine, Ninewells Hospital, Dundee, United Kingdom.

Microelimination targets specific subpopulations and/or geographic settings for hepatitis C virus (HCV) elimination. This review reports on global HCV microelimination literature published from 2013 to 2020. Data were extracted from publications to report a score based on the four key components defining microelimination.

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Background & Aims: Corticosteroids are the only effective therapy for severe alcohol-associated hepatitis (AH), defined by a model for end-stage liver disease (MELD) score >20. However, there are patients who may be too sick to benefit from therapy. Herein, we aimed to identify the range of MELD scores within which steroids are effective for AH.

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Background And Aims: The Spanish prison population includes two groups: people in prison and those who are serving non-custodial sentences. The latter has not yet been studied. This study aims to describe this population and the results of a test-and-treat strategy for hepatitis C including a holistic health assessment.

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HCV detection is possible during SARS CoV-2 testing; and throughout COVID-19 vaccination?

J Hepatol

August 2021

Gastroenterology and Hepatology Department. Marqués de Valdecilla University Hospital, Santander, Spain; Research Institute Valdecilla (IDIVAL), Santander, Spain. Electronic address:

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The usefulness of serum alkaline phosphatase (ALP) and phosphorous in screening and monitoring of metabolic bone disease of prematurity (MBDP) still has some limitations, especially in preterm infants with concomitant conditions such as cholestasis. We aimed to assess a modification of serum ALP (M-ALP) as a biomarker for MBDP in preterm infants, and the use of ultrasound monitoring for the apparition of knee ossification centers as marker of bone mineralization. Biochemical and clinical registers were taken from 94 preterm newborns <32 weeks.

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Objective: To evaluate the usefulness of a new marker, pentraxin, as a prognostic marker in septic shock patients.

Materials And Methods: Single-centre prospective observational study that included all consecutive patients 18 years or older who were admitted to the intensive care unit (ICU) with septic shock. Serum levels of procalcitonin (PCT), C-reactive protein (CRP) and pentraxin (PTX3) were measured on ICU admission.

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Advanced fibrosis and portal hypertension influence short-term mortality. Lipocalin 2 (LCN2) regulates infection response and increases in liver injury. We explored the role of intrahepatic LCN2 in human alcoholic hepatitis (AH) with advanced fibrosis and portal hypertension and in experimental mouse fibrosis.

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Vitamin D binding protein, but not vitamin D or vitamin D-related peptides, is associated with septic shock mortality.

Enferm Infecc Microbiol Clin (Engl Ed)

April 2019

Health Research Institute Valdecilla-IDIVAL, University of Cantabria, School of Nursing, Avenida de Valdecilla s/n 39008, Santander, Spain.

Background: The aim of this study was to assess the prognostic value of vitamin D, vitamin D binding protein (VDBP) and vitamin D-related peptides in septic shock patients in relation to hospital mortality.

Methods: This is a single-center, prospective, observational study that included all consecutive patients meeting criteria for septic shock who were admitted to the ICU. VDBP, 25-hydroxy vitamin D, 1,25-dihydroxy vitamin D, cathelicidin and beta-defensin levels were determined in blood samples obtained on admission to the ICU.

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This study aimed to assess the effect of rehydration during and after acute aerobic submaximal exercise on total homocysteine (tHcy) concentrations and related parameters in physically active adult males. Twenty trained males (29.4 ± 7.

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