Association of FcγRIIa R131H polymorphism with idiopathic pulmonary fibrosis severity and progression [PDF]
Background A significant genetic component has been described for idiopathic pulmonary fibrosis (IPF). The R131H (rs1801274) polymorphism of the IgG receptor FcγRIIa determines receptor affinity for IgG subclasses and is associated with several chronic ...
Hart Simon P +31 more
core +2 more sources
Alveolar cells under mechanical stressed niche: critical contributors to pulmonary fibrosis
Pulmonary fibrosis arises from the repeated epithelial mild injuries and insufficient repair lead to over activation of fibroblasts and excessive deposition of extracellular matrix, which result in a mechanical stretched niche.
Juntang Yang +3 more
doaj +1 more source
Rare and common variants in KIF15 contribute to genetic risk of idiopathic pulmonary fibrosis [PDF]
RATIONALE: Genetic studies of Idiopathic Pulmonary Fibrosis (IPF) have improved our understanding of this disease, but not all causal loci have been identified.
Newton, Chad A +20 more
core +1 more source
Informal caregivers experience of supplemental oxygen in pulmonary fibrosis
Background Patients prescribed supplemental oxygen (O2) therapy face challenges as they adjust to being constantly “tethered” to an oxygen delivery device.
Bridget A. Graney +8 more
doaj +1 more source
Idiopathic pulmonary fibrosis is defined as a chronic progressive fibrosing interstitial pneumonia of unknown etiology. There are intrinsic and extrinsic risk factors that could favor the development of the disease in individuals with a genetic predisposition.
Francisco, León-Román +2 more
openaire +6 more sources
Pirfenidone in idiopathic pulmonary fibrosis:expert panel discussion on the management of drug-related adverse events [PDF]
Pirfenidone is currently the only approved therapy for idiopathic pulmonary fibrosis, following studies demonstrating that treatment reduces the decline in lung function and improves progression-free survival.
Per M. Hellström +46 more
core +1 more source
Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis
Background Obstructive sleep apnea (OSA), nocturnal hypoxemia and excessive daytime sleepiness (EDS) are common comorbidities in people with cystic fibrosis (pwCF). Most of the data showing this originates from children and adolescents.
Matthias Welsner +10 more
doaj +1 more source
Pulmonary rehabilitation in idiopathic pulmonary fibrosis and COPD: a propensity matched real-world study [PDF]
BACKGROUND: The adherence to and clinical efficacy of pulmonary rehabilitation in idiopathic pulmonary fibrosis (IPF), particularly in comparison to people with chronic obstructive pulmonary disease (COPD), remains uncertain.
Ingram, Karen A +21 more
core +1 more source
Surfactant protein a in cystic fibrosis: supratrimeric structure and pulmonary outcome. [PDF]
The state of oligomerization of surfactant associated protein-A (SP-A) monomers differs between individuals. This likely affects SP-A's functional properties and could thereby influence clinical status in patients with lung diseases.
Karl Paul +15 more
core +1 more source
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

