Cystic fibrosis patients exhibit lung disease consistent with a failure of innate airway defense mechanisms. The link between abnormal ion transport and disease initiation and progression is not fully understood, but airway mucus dehydration seems paramount in the initiation of CF lung disease. New therapies are currently in development that target the ion transport defects in CF with the intention of rehydrating airway surfaces.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2430075 | PMC |
| http://dx.doi.org/10.1016/j.ddmec.2007.09.001 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Infection by Clonally Related Isolates: The Role of Drinking Water.
Am J Respir Crit Care Med
March 2025
The University of Queensland, Children's Health Research Centre, Faculty of Medicine , Brisbane, Queensland, Australia.
Rationale: group bacteria (MABS) cause lethal infections in people with chronic lung diseases. Transmission mechanisms remain poorly understood; the detection of dominant circulating clones (DCCs) has suggested potential for person-to-person transmission.
Objectives: This study aimed to determine the role of drinking water in the transmission of MABS.
-Induced Liver Damage Through Ferroptosis in Rat Model.
Cells
February 2025
College of Veterinary Medicine, Jilin University, Changchun 130062, China.
(1) Background: (CE) is an -induced worldwide parasitic zoonosis and is a recognized public health and socio-economic concern. The liver is the major target organ for CE's infective form protoscolex (PSCs), which causes serious liver damage and endangers the host's life. Reports show that PSC infection causes liver cell Fe metabolism disorder and abnormal deposition of Fe in liver cells and results in liver cell death.
View Article and Find Full Text PDFBackground: People with cystic fibrosis (pwCF) often have multifactorial peripheral muscle abnormalities attributed to, for example, malnutrition, steroid use, altered redox balance and, potentially, CF-specific intrinsic alterations. Malnutrition in CF now includes an increasing prevalence of overweight and obesity, particularly in those receiving CF transmembrane conductance regulator (CFTR) modulator therapy (CFTRm). We aimed to characterise peripheral muscle function and body composition in pwCF on Elexacaftor/Tezacaftor/Ivacaftor (ETI) CFTRm, compared to healthy controls.
View Article and Find Full Text PDFThe Frequency and Potential Implications of HFE Genetic Variants in Children With Cystic Fibrosis.
Pediatr Pulmonol
March 2025
Department of Pediatrics, University of Wisconsin - Madison, Madison, Wisconsin, USA.
Background: Genetic modifiers have been identified that increase the risks of lung disease and other complications, such as diabetes in people with cystic fibrosis (CF). Variants in the hemochromatosis gene (HFE) were reported in a study of adults to be associated with worse lung disease.
Objectives: To ascertain the frequency of HFE variants, particularly C282Y (c.
Pulmonary nontuberculous mycobacterial infections among women with cystic fibrosis and non-cystic fibrosis bronchiectasis.
Ther Adv Respir Dis
March 2025
Department of Medicine, National Jewish Health, Denver, CO, USA.
Nontuberculous mycobacteria (NTM) are ubiquitous, opportunistic pathogens that can cause lung disease in people with non-cystic fibrosis bronchiectasis (NCFB) and cystic fibrosis (CF). The incidence of NTM pulmonary infections and lung disease has continued to increase worldwide over the last decade among both groups. Notably, women with NCFB NTM pulmonary disease (NTM-PD) bear a disproportionate burden with NTM rates increasing in this population as well as having consistently higher incidence of NTM-PD compared to men.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!