Sphingolipids in pulmonary fibrosis [PDF]
Advances in Biological Regulation, 2015 Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by alveolar epithelial cell injury, accumulation of fibroblasts/myofibroblasts and deposition of extracellular matrix proteins. Levels of sphingosine-1-phosphate (S1P), a naturally occurring bioactive lipid, are elevated in bronchoalveolar fluids and lung tissues from IPF ...
Long Shuang Huang, Viswanathan Natarajan
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Role of growth factors in the pathogenesis of tissue fibrosis in systemic sclerosis. [PDF]
, 2010 The most severe clinical and pathologic manifestations of systemic sclerosis (SSc) are the result of a fibrotic process characterized by the excessive and often progressive deposition of collagen and other connective tissue macromolecules in skin and ...
Castro, Susan V. +2 more
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Idiopathic pulmonary fibrosis [PDF]
Thorax, 2011 Idiopathic pulmonary fibrosis is a progressive lung disease that carries a poor prognosis and for which there are no effective therapies. Although the excessive deposition of extracellular matrix, combined with evidence of recurrent injury to the alveolar epithelium, are well-described there is a pressing need to understand these processes better at a ...
Moira K. B. Whyte, Zhe Hui Hoo
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Medicines, 2021 Current FDA-approved antifibrotic treatments for Idiopathic Pulmonary Fibrosis slow down disease progression but have little impact on symptoms or quality of life in patients.
Neha Shah
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Endothelial to mesenchymal transition (EndoMT) in the pathogenesis of Systemic Sclerosis-associated pulmonary fibrosis and pulmonary arterial hypertension. Myth or reality? [PDF]
, 2016 Systemic Sclerosis (SSc) is a systemic autoimmune disease characterized by progressive fibrosis of skin and multiple internal organs and severe functional and structural microvascular alterations.
Jimenez, Sergio A. +1 more
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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
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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
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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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Design and rationale of a multi-center, pragmatic, open-label randomized trial of antimicrobial therapy - the study of clinical efficacy of antimicrobial therapy strategy using pragmatic design in Idiopathic Pulmonary Fibrosis (CleanUP-IPF) clinical trial [PDF]
, 2020 Compelling data have linked disease progression in patients with idiopathic pulmonary fibrosis (IPF) with lung dysbiosis and the resulting dysregulated local and systemic immune response.
A Mishra +43 more
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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
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