Severity of lung injury in cyclooxygenase-2-deficient mice is dependent on reduced prostaglandin E-2 production [PDF]
Levels of prostaglandin E-2 (PGE(2)), a potent inhibitor of fibroblast function, are decreased in the lungs of patients with pulmonary fibrosis, which has been shown to be because of limited expression of cyclooxygenase-2 (COX-2).
McAnulty, R J +35 more
core +1 more source
Enhanced IL-1β Release Following NLRP3 and AIM2 Inflammasome Stimulation Is Linked to mtROS in Airway Macrophages in Pulmonary Fibrosis [PDF]
Fibrotic Interstitial lung diseases (ILDs) are complex disorders of variable clinical behaviour. The majority of them cause significant morbidity, whilst Idiopathic Pulmonary Fibrosis (IPF) is recognised as the most relentless.
Mastrodimou, Semeli +9 more
core +1 more source
Treatment of pulmonary exacerbations in cystic fibrosis [PDF]
Purpose of review This review will discuss the challenges of defining a pulmonary exacerbations in cystic fibrosis and the key pathogens, which contribute.
Nadig, Tejaswi +3 more
core +2 more sources
Background CFTR modulator therapy with ivacaftor is a treatment option for Cystic Fibrosis (CF) patients with at least one copy of a R117H-7T mutation in the CFTR gene.
Matthias Welsner +3 more
doaj +1 more source
MicroRNAs in fibrosis: opportunities and challenges [PDF]
MicroRNAs (miRNAs) are small, non-coding RNAs that mediate mRNA cleavage, translational repression or mRNA destabilisation and are around 22-25 nucleotides in length via partial complementary binding to the 3' untranslated region in target transcripts ...
O'Reilly, Steven, Steven O’Reilly
core +1 more source
Quality of life is associated with physical activity and fitness in cystic fibrosis [PDF]
BACKGROUND: Health-related and disease-specific quality of life (HRQoL) has been increasingly valued as relevant clinical parameter in cystic fibrosis (CF) clinical care and clinical trials.
Helge Hebestreit +31 more
core +1 more source
Promoter variants in tissue inhibitor of metalloproteinase-3 (TIMP-3) protect against susceptibility in pigeon breeders' disease. [PDF]
BACKGROUND: Tissue inhibitors of metalloproteinases (TIMPs) play a major role in extracellular matrix turnover in the lung. However, in chronic lung disorders such as idiopathic pulmonary fibrosis (IPF) and pigeon breeders' disease (PBD), TIMPs may ...
Briggs, L +7 more
core +1 more source
Modelling forced vital capacity in idiopathic pulmonary fibrosis: optimising trial design. [PDF]
INTRODUCTION: Forced vital capacity is the only registrational endpoint in idiopathic pulmonary fibrosis clinical trials. As most new treatments will be administered on top of standard of care, estimating treatment response will become more challenging ...
Meyvisch, Paul +34 more
core +1 more source
Cough suppression and HRQoL in adult people with cystic fibrosis: an unexplored correlation
Background Cough suppression assessed by embarrassment about coughing has been shown in adolescents with cystic fibrosis (CF) and negatively affects health-related quality of life (HRQoL) and clinical indicators of disease severity in adolescent females.
Ute Niehammer +7 more
doaj +1 more source
Sputum biomarkers and the prediction of clinical outcomes in patients with cystic fibrosis. [PDF]
Lung function, acute pulmonary exacerbations (APE), and weight are the best clinical predictors of survival in cystic fibrosis (CF); however, underlying mechanisms are incompletely understood.
Rogers, S.L. +84 more
core +1 more source

