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Job-exposure matrix (JEM) validity on crystalline silica among systemic sclerosis patients.
Occup Med (Lond)
January 2025
Maine et Loire, Univ Angers, CHU Angers, Univ Rennes, Inserm, EHESP, Irset (Institut de recherche en santé, environnement et travail) - UMR_S 1085, IRSET-ESTER, SFR ICAT, CAPTV CDC, 49000 Angers, France.
Background: Systemic sclerosis (SSc) is the connective tissue disease with the highest individual mortality. Crystalline silica is known to be an occupational risk factor for SSc. To assess past crystalline silica exposure, we aimed to study the validity of a job exposure matrix (JEM) to assess occupational exposure to crystalline silica compared to specific occupational interviews in two populations of SSc patients.
View Article and Find Full Text PDFUndocking of an extensive ciliary network induces proteostasis and cell fate switching resulting in severe primary ciliary dyskinesia.
Sci Transl Med
January 2025
Department of Cell Biology and Physiology, Washington University School of Medicine, Saint Louis, MO 63110, USA.
Primary ciliary dyskinesia is a rare monogenic syndrome that is associated with chronic respiratory disease, infertility, and laterality defects. Although more than 50 genes causative of primary ciliary dyskinesia have been identified, variants in the genes encoding coiled-coil domain-containing 39 (CCDC39) and CCDC40 in particular cause severe disease that is not explained by loss of ciliary motility alone. Here, we sought to understand the consequences of these variants on cellular functions beyond impaired motility.
View Article and Find Full Text PDFEuropean ILD registry algorithm for self-assessment in interstitial lung diseases (eurILDreg ASA-ILD).
PLoS One
January 2025
European IPF/ILD Registry and Biobank (eurIPFreg/bank, eurILDreg/bank), Giessen, Germany.
Background And Aims: Predicting progression and prognosis in Interstitial Lung Diseases (ILD), especially Idiopathic Pulmonary Fibrosis (IPF) and Progressive Pulmonary Fibrosis (PPF), remains a challenge. Integrating patient-centered measurements is essential for earlier and safer detection of disease progression. Home monitoring through e-health technologies, such as spirometry and oximetry connected to smartphone applications, holds promise for early detection of ILD progression or acute exacerbations, enabling timely therapeutic interventions.
View Article and Find Full Text PDFA comparative analysis of risk stratification tools in systemic sclerosis-associated pulmonary arterial hypertension: a EUSTAR analysis.
Rheumatology (Oxford)
January 2025
Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Objectives: The 2022 European Society of Cardiology and European Respiratory Society (ESC/ERS) Guidelines for pulmonary arterial hypertension (PAH) recommend risk stratification to optimize management. However, the performance of generic PAH risk stratification tools in patients with systemic sclerosis (SSc)-associated PAH remains unclear. Our objective was to identify the most accurate approach for risk stratification at SSc-PAH diagnosis.
View Article and Find Full Text PDFEpicardial Pacemaker Lead Related Cardiac Strangulation: The Importance of Early Recognition.
Pediatr Cardiol
January 2025
Cardiothoracic Department, Children's Health Ireland at Crumlin, Dublin, Ireland.
Lead strangulation is a dangerous complication of epicardial pacemaker insertion. This complication has been increasingly highlighted lately. Our institution has recently identified four cases over the past five years.
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