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Pulmonary Fibrosis in Children [PDF]
Journal of Clinical Medicine, 2019 Pulmonary fibrosis (PF) is a very rare condition in children, which may be observed in specific forms of interstitial lung disease. None of the clinical, radiological, or histological descriptions used for PF diagnosis in adult patients, especially in situations of idiopathic PF, can apply to pediatric situations.
Nathan, Nadia +10 more
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The Lancet, 2017 Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease. Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death.
Luca Richeldi +3 more
+12 more sources
BMC Pulmonary Medicine, 2017 Background Patients with pulmonary fibrosis (PF) have impaired quality of life, and research suggests that dyspnea and physical activity are primary drivers. As PF progresses, some patients notice the disease “shrinks their worlds”. The objective of this
Elisabeth Dowling Root +10 more
doaj +1 more source
Tracking dyspnea up to supplemental oxygen prescription among patients with pulmonary fibrosis
BMC Pulmonary Medicine, 2017 Background Dyspnea is the hallmark symptom of pulmonary fibrosis. Supplemental oxygen (O2) is prescribed to many patients with pulmonary fibrosis in hopes of alleviating dyspnea and improving physical functioning.
Amy L. Olson +10 more
doaj +1 more source
Tuftelin1 drives experimental pulmonary fibrosis progression by facilitating stress fiber assembly
Respiratory Research, 2023 Background Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease (ILD) with unknown etiology, characterized by sustained damage repair of epithelial cells and abnormal activation of fibroblasts, the underlying mechanism of the ...
Caoyuan Niu +13 more
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Alveolar cells under mechanical stressed niche: critical contributors to pulmonary fibrosis
Molecular Medicine, 2020 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
Obstructive sleep apnea and nocturnal hypoxemia in adult patients with cystic fibrosis
BMC Pulmonary Medicine, 2022 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
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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Informal caregivers experience of supplemental oxygen in pulmonary fibrosis
Health and Quality of Life Outcomes, 2017 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
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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