Heterologous matrix metalloproteinase gene promoter activity allows In Vivo real-time imaging of Bleomycin-induced Lung fibrosis in transiently transgenized mice [PDF]
, 2017 Idiopathic pulmonary fibrosis is a very common interstitial lung disease derived from chronic inflammatory insults, characterized by massive scar tissue deposition that causes the progressive loss of lung function and subsequent death for respiratory ...
Donofrio, Gaetano +8 more
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Increased expression of 5-hydroxytryptamine(2A/B) receptors in idiopathic pulmonary fibrosis: a rationale for therapeutic intervention [PDF]
, 2010 Background Idiopathic pulmonary fibrosis (IPF) has a poor prognosis and limited responsiveness to available treatments. It is characterised by epithelial cell injury, fibroblast activation and proliferation and extracellular matrix deposition ...
Amarie, Oana Veronica +8 more
core +1 more source
BMC Pulmonary Medicine, 2019 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
ROCKing pulmonary fibrosis [PDF]
Journal of Clinical Investigation, 2013 Clinical vignette: A 76-year-old man consults you for increasing shortness of breath over the past two years and an increasing requirement for home oxygen. A video-assisted thoracoscopic lung biopsy shows findings of usual interstitial pneumonitis, and he has no identifiable cause for pulmonary fibrosis, so he is considered to have idiopathic pulmonary
openaire +4 more sources
Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis [PDF]
, 2018 BACKGROUND: In children with cystic fibrosis (CF), recovery from growth faltering within 2 years of diagnosis (Responders) is associated with better growth and less lung disease at age 6 years.
Farrell, Philip M. +3 more
core +1 more source
Lung ultrasound in systemic sclerosis: correlation with high-resolution computed tomography, pulmonary function tests and clinical variables of disease [PDF]
, 2015 Interstitial lung disease (ILD) is a hallmark of systemic sclerosis (SSc). Although high-resolution computed tomography (HRCT) is the gold standard to diagnose ILD, recently lung ultrasound (LUS) has emerged in SSc patients as a new promising technique ...
Amoroso, Antonio +8 more
core +1 more source
Cough suppression and HRQoL in adult people with cystic fibrosis: an unexplored correlation
Health and Quality of Life Outcomes, 2022 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
Protein kinase Cδ and c-Abl kinase are required for transforming growth factor β induction of endothelial-mesenchymal transition in vitro. [PDF]
, 2011 OBJECTIVE: The origin of the mesenchymal cells responsible for the intimal fibrosis in systemic sclerosis (SSc) has not been fully identified. The present study was undertaken to investigate whether subendothelial mesenchymal cells may emerge through ...
Jimenez, Sergio A., Li, zhaodong
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Development of a Consensus Statement for the Definition, Diagnosis, and Treatment of Acute Exacerbations of Idiopathic Pulmonary Fibrosis Using the Delphi Technique. [PDF]
, 2015 © 2015, The Author(s).Introduction: There is a lack of agreed and established guidelines for the treatment of acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF).
Bennett, BM +8 more
core +2 more sources
A prospective observational study of mycophenolate mofetil treatment in progressive diffuse cutaneous systemic sclerosis of recent onset. [PDF]
, 2012 OBJECTIVE: A prospective observational study of mycophenolate mofetil (MMF) treatment in patients with diffuse progressive cutaneous systemic sclerosis (SSc) of recent onset.
Jimenez, Sergio A. +3 more
core +3 more sources