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
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
[Idiopathic pulmonary fibrosis].
38;6 ...
Hennion, Nathan +4 more
openaire +6 more sources
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
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
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
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
Macrorheology of cystic fibrosis, chronic obstructive pulmonary disease & normal sputum [PDF]
Background Prior microrheologic assessments of selected, microlitre plugs of cystic fibrosis (CF) sputum suggest no intrinsic rheologic abnormality. However, such analyses may not be representative of CF sputum as a whole.
Young Simon A +7 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
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

