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Undocking 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.
Article Synopsis
- Primary ciliary dyskinesia (PCD) is a rare genetic disorder linked to chronic respiratory issues, infertility, and problems with body asymmetry, primarily caused by mutations in the CCDC39 and CCDC40 genes.
- Researchers used advanced techniques to investigate how these genetic variants impact cellular functions beyond just causing cilia to stop moving.
- They discovered that the absence of CCDC39/CCDC40 creates a significant loss of over 90 ciliary structural proteins, leading to cilia dysfunction and other cellular issues, suggesting that gene therapy could potentially offer a new treatment strategy for PCD.
Systematic Review and Meta-Analysis of the Application of T-PEP in the Therapeutic Management of COPD Patients.
J Clin Med
January 2025
Division of Respiratory Medicine, University Hospital Tor Vergata, 00133 Rome, Italy.
Chronic obstructive pulmonary disease (COPD) is a leading cause of morbidity and mortality worldwide, characterized by chronic mucus hypersecretion (CMH) that exacerbates airway obstruction and accelerates disease progression. Effective airway clearance techniques are essential to improve respiratory function and reduce exacerbations. Temporary Positive Expiratory Pressure (T-PEP) is a novel airway clearance device that has shown promise in managing COPD.
View Article and Find Full Text PDFNon-cystic fibrosis bronchiectasis in pediatrics: A cohort profile of patients with inborn errors of immunity at a referral center in Cali, Colombia.
Biomedica
December 2024
Facultad de Ciencias de la Salud, Departamento de Medicina, Universidad ICESI, Cali, Colombia; Centro de Investigaciones Clínicas, Fundación Valle del Lili, Cali, Colombia; Servicio de Alergología e Inmunología Pediátrica, Departamento de Pediatría, Fundación Valle del Lili, Cali, Colombia.
Introduction. Inborn errors of immunity are frequently associated with bronchiectasis. The diagnostic performance of these inborn errors has improved because the association of some of these entities with progressive airway damage is better known.
View Article and Find Full Text PDFReconsidering the Diagnosis: Abnormal Sweat Chloride Tests in Non-CF Bronchiectasis.
Pediatr Pulmonol
January 2025
Department of Internal Medicine, Division of Pulmonary and Critical Care, University of Virginia, Charlottesville, Virginia, USA.
Introduction: While the diagnosis of cystic fibrosis (CF) is often straightforward and reliant on correlation between genetic testing and clinical signs and symptoms, there is a subset where the distinction is not nearly as clearcut. This has previously been reported in patients identified through newborn screening but not meeting full CF diagnostic criteria, earning the label of CF Screen Positive, Inconclusive Diagnosis (CFSPID) instead. A homologous diagnostic category in adults is named CF Transmembrane Conductance Regulator-Related Disorder (CFTR-RD).
View Article and Find Full Text PDFEosinopenia in bronchiectasis: A novel biomarker for morbidity and mortality.
Chron Respir Dis
January 2025
Pulmonary Institute, Rabin Medical Center - Beilinson Hospital, Petach Tikva, Israel.
Background: The paradigm of bronchiectasis is shifting away from its exclusive characterization as a neutrophilic condition. Patients with bronchiectasis and high eosinophil levels have been found to have a specific phenotype, but the clinical effect of eosinopenia remains unclear.
Method: A retrospective, single-center, observational study was conducted at a tertiary medical center.
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