906 results match your criteria: "Centre de Ressources[Affiliation]"

Short-term modification of breathprint by Elexacaftor/Tezacaftor/Ivacaftor in a paediatric cohort.

J Cyst Fibros

January 2025

INSERM U1151, Institut Necker Enfants Malades, Paris, France; Hôpital Necker Enfants Malades, Centre de Référence Maladies Rares Mucoviscidose et Maladies apparentées, Paris, France; Université Paris-Cité, Paris, France; European Reference Network-Lung. Frankfurt, Germany. Electronic address:

Background: The triple combination Elexacaftor/Tezacaftor/Ivacaftor (ETI) translates into major respiratory improvements in adults; yet current clinical endpoints may prove insufficiently sensitive in young children. We hypothesised that ETI rapidly modifies the lungs' metabolism, resulting in changes in breath composition.

Methods: Eleven children with CF were enrolled in a longitudinal pilot study at the paediatric Necker hospital.

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Psychometric Validation of the Hemophilia Functional Ability Scoring Tool (Hemo-FAST).

Thromb Haemost

January 2025

Centre de Ressources et Compétences des Maladies Hémorragiques Constitutionnelles, CHU Clermont-Ferrand, Estaing Hospital, Clermont-Ferrand, France.

Background:  The Hemophilia Functional Ability Scoring Tool (Hemo-FAST), consisting of a patient-reported outcome (PRO) part and a clinician-reported outcome (ClinRO) part, was developed as a rapid and effective tool to assess functional mobility in clinical practice. This study (NCT04731701) aimed to validate the psychometric properties of Hemo-FAST for assessment of joint health in people with haemophilia (PwH).

Methods:  PwH A or B aged ≥18 years completed questionnaires including the PRO part of Hemo-FAST and the short-form 36 health survey (SF-36) during one study visit.

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Introduction: Cellular characteristics of induced sputum (IS) are not investigated in cystic fibrosis (CF) patients.

Objectives: This pilot study, conducted on 17 expectorating CF adolescents, compared sputa obtained the same day, in a stable period, by autogenic drainage (expectorating sputum, ES) and 4 h later after inhaling hypertonic saline (IS).

Results: No difference was noted concerning weight, volume, and percentage of dead cells between the two collection methods.

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Background: We previously reported the safety and immunogenicity data from a randomized trial comparing the booster responses of vaccinees who received monovalent (MV) recombinant protein Beta-variant (MVB.1.351) and MV ancestral protein (MVD614) vaccines with AS03 adjuvant (Sanofi/GSK) to booster response of vaccinees who received mRNA MV ancestral strain BNT162b2 vaccine (Pfizer-BioNTech).

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Cystic and alveolar echinococcosis are severe zoonotic diseases characterized by long asymptomatic periods lasting months or years. Viable Echinococcus spp. eggs released into the environment through the feces of canids can infect humans through accidental ingestion via hand-to-mouth contact or consumption of contaminated food or water.

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Cardiac adaptations in young triathletes: a 9-month longitudinal study during the peak height velocity period.

Eur J Appl Physiol

January 2025

Laboratoire de Pharm-Ecologie Cardiovasculaire (EA 4278), Université d'Avignon, 33 Rue Louis Pasteur, 84000, Avignon, France.

Purpose: The present study examined the influence of endurance training on the morphological and functional heart adaptations in young athletes throughout a longitudinal 9-month follow-up period during the adolescent peak height velocity (PHV).

Methods: Thirty-six 13- to 15-year-old males (twenty-three triathletes and thirteen untrained peers) were evaluated before and after a 9-month period during PHV. Maximal oxygen uptake ( ) and power at were assessed during incremental cycling test.

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Hepatocellular carcinoma (HCC) is the third most common cause of cancer-related death worldwide. Treating HCC is challenging because of the poor drug effectiveness and the lack of tools to predict patient responses. To resolve these issues, we established a patient-centric spheroid model using HepG2, TWNT-1, and THP-1 co-culture, that mimics HCC phenotype.

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The molecular diagnosis of type 1 facioscapulohumeral muscular dystrophy (FSHD1) relies on the detection of a shortened D4Z4 array at the 4q35 locus. Until recently, the diagnosis of FSHD2 relied solely on the absence of a shortened D4Z4 allele in clinically affected patients. It is now established that most FSHD2 cases carry a heterozygous variant in the SMCHD1 gene.

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The development of disease-modifying therapies (DMTs) for the treatment of multiple sclerosis (MS) has been highly successful in recent decades. It is now widely accepted that early initiation of DMTs after disease onset is associated with a better long-term prognosis. However, the question of when and how to de-escalate or discontinue DMTs remains open and critical.

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Background: A fundamental property of cancer cells is their metabolic reprogramming, allowing them to increase glucose uptake and glycolysis. Using a rat colon adenocarcinoma model, we previously showed that blood levels of free methylglyoxal (MG), a side-product of glycolysis, remained normal in animals grafted with a non-growing tumor cell clone, while MG levels were significantly increased and positively correlated with tumor growth in animals grafted with a tumorigenic cell clone issued from the same tumor.

Methods: We measured free MG in the blood of cancerous non-diabetic patients and compared the results to healthy subjects and non-cancerous diabetic patients.

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Epigenetic control of myogenic identity of human muscle stem cells in Duchenne muscular dystrophy.

iScience

December 2024

Institut NeuroMyoGène, Physiopathologie et Génétique du Neurone et du Muscle Université Claude Bernard Lyon 1, CNRS U5261, Inserm U1315, University Lyon, Lyon, France.

In Duchenne muscular dystrophy (DMD), muscle stem cells' (MuSCs) regenerative capacities are overwhelmed leading to fibrosis. Whether MuSCs have intrinsic defects or are disrupted by their environment is unclear. We investigated cell behavior and gene expression of MuSCs from DMD or healthy human muscles.

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Outbreak of multidrug-resistant Staphylococcus haemolyticus ST29 in a French neonatal unit.

J Hosp Infect

December 2024

Hygiène Hospitalière, Centre Hospitalier Universitaire, Besançon, France; UMR 6249 Chrono-environnement, Université de Franche-Comté, Besançon, France. Electronic address:

Background: Staphylococcus haemolyticus is a clinically relevant coagulase-negative staphylococcus frequently responsible for hospital-acquired infections, especially in premature newborns.

Aim: To describe an outbreak of multidrug-resistant S. haemolyticus in a neonatal department.

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Analysis of a shark reveals ancient, Wnt-dependent, habenular asymmetries in vertebrates.

Nat Commun

November 2024

CNRS, Sorbonne Université, UMR7232-Biologie Intégrative des Organismes Marins, Observatoire Océanologique, Banyuls-sur-Mer, France.

The mode of evolution of left-right asymmetries in the vertebrate habenulae remains largely unknown. Using a transcriptomic approach, we show that in a cartilaginous fish, the catshark Scyliorhinus canicula, habenulae exhibit marked asymmetries, in both their medial and lateral components. Comparisons across vertebrates suggest that those identified in lateral habenulae reflect an ancestral gnathostome trait, partially conserved in lampreys, and independently lost in tetrapods and neopterygians.

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Background/objective: Offspring's optimal neurodevelopment depends on maternal dietary lipids supply during the perinatal period. Dairy products are an important source of fat in Western diets. This study aimed at examining the associations between maternal dairy fat intake during pregnancy, evaluated through biomarkers in perinatal biofluids (C15:0 and C17:0) and child's cognitive outcomes.

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HIV-1 budding requires cortical actin disassembly by the oxidoreductase MICAL1.

Proc Natl Acad Sci U S A

November 2024

Membrane Traffic and Cell Division Unit, Institut Pasteur, Université Paris Cité, CNRS UMR3691, Paris F-75015, France.

Many enveloped viruses bud from the plasma membrane that is tightly associated with a dense and thick actin cortex. This actin network represents a significant challenge for membrane deformation and scission, and how it is remodeled during the late steps of the viral cycle is largely unknown. Using superresolution microscopy, we show that HIV-1 buds in areas of the plasma membrane with low cortical F-actin levels.

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Spatial transcriptomics methods provide insight into the cellular heterogeneity and spatial architecture of complex, multicellular systems. Combining molecular and spatial information provides important clues to study tissue architecture in development and disease. Here, we present a comprehensive do-it-yourself (DIY) guide to perform such experiments at reduced costs leveraging open-source approaches.

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The use of immunosuppressive treatment is required to prevent rejection events, even a long time after kidney transplantation despite rare recipients achieving long-term graft stability without the need for immunosuppressive treatment, called operationally tolerant patients (TOLs). We comprehensively investigate the immune system of long-term IS recipients (LTTs) and TOLs to highlight their shared and unique immune features. Blood immune cell phenotyping was performed by spectral cytometry.

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Candida albicans strains adapted to the mouse gut are resistant to bile salts via a Flo8-dependent mechanism.

Fungal Genet Biol

December 2024

Departamento de Microbiología y Parasitología-IRYCIS, Facultad de Farmacia, Universidad Complutense de Madrid, Avda. Ramón y Cajal s/n, 28040 Madrid, Spain. Electronic address:

Candidaalbicans normally colonizes the human gastrointestinal tract as a commensal. Studying fungal factors involved in colonizing the mammalian gastrointestinal tract requires mouse models with altered microbiota. We have obtained strains of C.

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Article Synopsis
  • A study was conducted during the COVID-19 pandemic in France to evaluate the effectiveness of various biomarkers in predicting short-term worsening (STW) of the disease in hospitalized patients.
  • The research involved analyzing data from 511 consecutive COVID-19 patients across 20 hospitals, identifying key blood test markers that could help in severity triage.
  • The results showed that out of the patients analyzed, 11.7% experienced STW, with certain biomarker levels at admission indicating a higher risk of rapid deterioration within seven days.
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Article Synopsis
  • MandibuloAcral Dysplasia associated to MTX2 gene (MADaM) is an accelerated aging disease characterized by skin issues, growth delays, and heart-related problems.
  • Research using mtx-2-deficient C. elegans (a type of worm) shows they exhibit similar aging characteristics to humans with MADaM, including rougher skin texture and poor mitochondrial function.
  • The findings from various analyses support these worms as a promising model to explore MADaM's molecular mechanisms and help develop potential treatments.
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