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Idiopathic pulmonary disease

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https://www.readbyqxmd.com/read/28646553/epithelial-mesenchymal-transition-emt-a-spectrum-of-states-role-in-lung-development-homeostasis-and-disease
#1
REVIEW
Mohit Kumar Jolly, Chris Ward, Mathew Suji Eapen, Stephen Myers, Oskar Hallgren, Herbert Levine, Sukhwinder Singh Sohal
Epithelial Mesenchymal Transition (EMT) plays key roles during lung development and many lung diseases such as Chronic Obstructive Pulmonary Disease (COPD), lung cancer and pulmonary fibrosis. Here, integrating morphological observations with underlying molecular mechanisms, we highlight the functional role of EMT in lung development and injury repair, and discuss how it can contribute to pathogenesis of chronic lung disease. We discuss the evidence of manifestation of EMT and its potential driving role in COPD, idiopathic pulmonary fibrosis (IPF), bronchiolitis obliterans syndrome (BOS), and lung cancer, while noting that all cells need not display a full EMT in any of these contexts, i...
June 24, 2017: Developmental Dynamics: An Official Publication of the American Association of Anatomists
https://www.readbyqxmd.com/read/28646122/nintedanib-a-triple-tyrosine-kinase-inhibitor-attenuates-renal-fibrosis-in-chronic-kidney-disease
#2
Feng Liu, Li Wang, Hualin Qi, Jun Wang, Wei Jiang, Yi Wang, Liuqing Xu, Na Liu, Shougang Zhuang
<span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">Nintedanib (BIBF1120) is a triple kinase inhibitor</span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;"> of </span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">platelet derived growth factor receptor (PDGFR), fibroblast growth factor receptors (FGFR)</span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">, </span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">vascular endothelial growth factor receptor (VEGFR)</span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;"> and Src family kinase, </span><span style="margin: 0px; color: black; line-height: 200%; font-family: 'Times New Roman',serif; font-size: 12pt;">that has recently been approved by FDA to treat idiopathic pulmonary fibrosis...
June 23, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28643420/impact-on-survival-of-warfarin-in-patients-with-pulmonary-arterial-hypertension-receiving-subcutaneous-treprostinil
#3
Mona Ascha, Xuan Zhou, Youlan Rao, Omar A Minai, Adriano R Tonelli
INTRODUCTION: Anticoagulation is a common treatment modality in patients with pulmonary arterial hypertension (PAH). Further studies are needed to appropriately assess the risk / benefit ratio of anticoagulation, particularly in PAH patients receiving PAH-specific therapies. AIMS: We use observational long-term data on PAH patients treated with subcutaneous (SQ) treprostinil from a large open-label study. Patients were followed for up to four years. The use of warfarin and bleeding events were recorded...
June 23, 2017: Cardiovascular Therapeutics
https://www.readbyqxmd.com/read/28642621/variants-of-the-abca3-gene-might-contribute-to-susceptibility-to-interstitial-lung-diseases-in-the-chinese-population
#4
Wei Zhou, Yi Zhuang, Jiapeng Sun, Xiaofen Wang, Qingya Zhao, Lizhi Xu, Yaping Wang
ATP-binding cassette A3 (ABCA3) is a phospholipid carrier that is mainly expressed in the alveolar epithelium. Biallelic mutations of ABCA3 has been associated with fatal respiratory distress syndrome and interstitial lung disease (ILD) in children. However, whether variations in ABCA3 have a role in the development of adult ILD, including idiopathic pulmonary fibrosis (IPF), remains to be addressed. In this study, we screened for germline variants of ABCA3 by exons-sequencing in 30 patients with sporadic IPF and in 30 matched healthy controls...
June 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28635908/-idiopatic-pulmonary-fibrosis-a-new-paradigm
#5
S N Avdeev
Idiopathic pulmonary fibrosis ((IPF) is the most common disease from a group of interstitial lung diseases, which occurs mainly in middle-aged and elderly people. Over the past decade, there have been considerable changes in approaches to diagnosing and treating IPF. The paper presents an update on the epidemiology of IPF, the results of new studies of its pathogenesis, and main approaches to diagnosing the disease. In addition, there is new evidence on therapy for IPF.
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28632001/vasodilator-responsiveness-in-idiopathic-pulmonary-arterial-hypertension-identifying-a-distinct-phenotype-with-distinct-physiology-and-distinct-prognosis
#6
David Langleben, Stylianos Orfanos
Within the cohort of patients suffering from idiopathic pulmonary arterial hypertension (IPAH) is a group that responds dramatically (VR-PAH) to an acute vasodilator challenge and that has excellent long-term hemodynamic improvement and prognosis on high dose calcium channel blockers compared with vasodilator non-responders (VN-PAH). For the purposes of diagnosing VR-PAH, there is to date no test to replace the acute vasodilator challenge. However, recent studies have identified markers that may aid in the identification of VR-PAH, including peripheral blood lymphocyte RNA expression levels of desmogelin-2 and Ras homolog gene family member Q, and plasma levels of provirus integration site for Moloney murine leukemia virus...
June 20, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28629920/pulmonary-hypertension-in-parenchymal-lung-diseases-any-future-for-new-therapies
#7
REVIEW
Sergio Harari, Davide Elia, Marc Humbert
Pulmonary hypertension (PH) due to chronic lung diseases is associated with a poor prognosis, regardless of the underlying respiratory condition. Updated PH guidelines recommend optimal treatment of the underlying lung disease, including long-term oxygen therapy, in patients with chronic hypoxaemia despite the lack of randomized controlled clinical trials supporting this statement. So far, randomized controlled trials on drugs approved for pulmonary arterial hypertension (PAH) have yielded discouraging results in both interstitial lung diseases (ILD) and chronic obstructive pulmonary diseases (COPD) with PH...
June 16, 2017: Chest
https://www.readbyqxmd.com/read/28629363/fibroblast-paracrine-tnf-%C3%AE-signaling-elevates-integrin-a5-expression-in-idiopathic-pulmonary-fibrosis-ipf
#8
Gali Epstein Shochet, Elizabetha Brook, Lilach Israeli-Shani, Evgeny Edelstein, David Shitrit
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis. Inflammatory cytokines play a significant role in IPF pathology. However, the fibroblast itself is also believed to be the primary effector in IPF. We hypothesized that the fibroblasts themselves secrete pro-inflammatory cytokines that could propagate IPF by affecting normal neighboring cells. Thus, we explored the effects of IPF fibroblast derived media on normal fibroblast characteristics...
June 19, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28626631/chronic-eosinophilic-pneumonia-adjunctive-therapy-with-inhaled-steroids
#9
Christopher Chan, David DeLapp, Perry Nystrom
Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare form of diffuse parenchymal lung disease first identified by Carrington et al. in 1969. It is characterized by the presence of constitutional and respiratory symptoms with associated peripheral opacities on imaging and elevated serum and/or bronchoalveolar eosinophilia. Although data is limited regarding etiology or prevalence, it is known that ICEP has a 2:1 female: male predominance and typically affects non-smokers. Diagnosis rests on the clinical constellation of respiratory symptoms of at least 2-4 weeks duration, the presence of diffuse pulmonary alveolar consolidation, classically described as the "photographic negative of pulmonary edema", the presence of eosinophils ≥40% on bronchoalveolar lavage or ≥1000/mm(3) eosinophils on peripheral blood and the exclusion of other known causes of eosinophilic lung diseases such as drugs, toxins, fungi, parasites, and collagen-vascular disorders...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28624389/plasma-proteome-analysis-in-patients-with-pulmonary-arterial-hypertension-an-observational-cohort-study
#10
Christopher J Rhodes, John Wharton, Pavandeep Ghataorhe, Geoffrey Watson, Barbara Girerd, Luke S Howard, J Simon R Gibbs, Robin Condliffe, Charles A Elliot, David G Kiely, Gerald Simonneau, David Montani, Olivier Sitbon, Henning Gall, Ralph T Schermuly, H Ardeschir Ghofrani, Allan Lawrie, Marc Humbert, Martin R Wilkins
BACKGROUND: Idiopathic and heritable pulmonary arterial hypertension form a rare but molecularly heterogeneous disease group. We aimed to measure and validate differences in plasma concentrations of proteins that are associated with survival in patients with idiopathic or heritable pulmonary arterial hypertension to improve risk stratification. METHODS: In this observational cohort study, we enrolled patients with idiopathic or heritable pulmonary arterial hypertension from London (UK; cohorts 1 and 2), Giessen (Germany; cohort 3), and Paris (France; cohort 4)...
June 14, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28624000/aneurysms-of-the-azygos-vein
#11
REVIEW
Maximilian Kreibich, Matthias Siepe, Jochen Grohmann, Gregor Pache, Friedhelm Beyersdorf
OBJECTIVE: Azygos vein aneurysms (AVAs) are uncommon and infrequently diagnosed. When confronted with a patient presenting with an AVA, physicians can rely on only a few case reports after an extensive literature search. To date, no guideline, no rule, and no review on the optimal treatment strategy for these patients exist. METHODS: A PubMed and MEDLINE database search for papers and case reports describing AVA was performed. Cases from our own institutions were also reviewed...
July 2017: Journal of Vascular Surgery. Venous and Lymphatic Disorders
https://www.readbyqxmd.com/read/28623539/pneumothorax-after-transbronchial-biopsy-in-pulmonary-fibrosis-lessons-from-the-multicenter-comet-trial
#12
Jonathan A Galli, Nicholas L Panetta, Nathaniel Gaeckle, Fernando J Martinez, Bethany Moore, Thomas Moore, Anthony Courey, Kevin Flaherty, Gerard J Criner
PURPOSE: Some patients with diffuse interstitial lung disease (ILD) undergo bronchoscopy with transbronchial biopsy (TBB) as part of their diagnostic evaluation. It is unclear what the incidence and risk factors for pneumothorax (PTX) following TBB are in this patient population. METHODS: Ninety-seven subjects with pulmonary fibrosis who underwent a research bronchoscopy with TBB as part of the multicenter correlating outcomes with biochemical markers to estimate time-progression in idiopathic pulmonary fibrosis (COMET) trial were retrospectively reviewed...
June 16, 2017: Lung
https://www.readbyqxmd.com/read/28622199/antifibrotic-drugs-as-treatment-of-nonidiopathic-pulmonary-fibrosis-interstitial-pneumonias-the-time-is-now
#13
Michael Kreuter, Julia Wälscher, Jürgen Behr
PURPOSE OF REVIEW: Therapeutic advances in the management of idiopathic pulmonary fibrosis (IPF) has led to improved outcomes with the use of the antifibrotic agents pirfenidone and nintedanib, with a number of randomized studies demonstrating benefits in slowing disease progression in IPF. However, treatment of other fibrosing interstitial lung diseases (ILD) remains challenging. RECENT FINDINGS: Observational and uncontrolled studies investigating pirfenidone and nintedanib in non-IPF ILDs suggest potential benefits, although the data is weak...
June 15, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28617305/fibrotic-hypersensitivity-pneumonitis-key-issues-in-diagnosis-and-management
#14
REVIEW
Vasileios Kouranos, Joseph Jacob, Andrew Nicholson, Elizabetta Renzoni
The diagnosis of hypersensitivity pneumonitis (HP) relies on the clinical evaluation of a number of features, including a history of significant exposure to potentially causative antigens, physical examination, chest CT scan appearances, bronchoalveolar lavage lymphocytosis, and, in selected cases, histology. The presence of fibrosis is associated with higher morbidity and mortality. Differentiating fibrotic HP from the idiopathic interstitial pneumonias can be a challenge. Furthermore, even in the context of a clear diagnosis of fibrotic HP, the disease behaviour can parallel that of idiopathic pulmonary fibrosis in a subgroup, with inexorable progression despite treatment...
June 15, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28615246/src-family-kinases-in-chronic-kidney-disease
#15
Jun Wang, Shougang Zhuang
Src family kinases (SFKs) belong to non-receptor protein tyrosine kinases (PTKs) and have been implicated in the regulation of numerous cellular processes, including cell proliferation, differentiation, migration and invasion, angiogenesis. The role and mechanisms of SFKs in tumorgenesis have been extensively investigated and some of SFK inhibitors are currently under clinical trials for tumor treatment. Recent studies have also demonstrated the importance of SFKs in regulating the development of various fibrosis related chronic diseases (e...
June 14, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28613983/azithromycin-attenuates-myofibroblast-differentiation-and-lung-fibrosis-development-through-proteasomal-degradation-of-nox4
#16
Kazuya Tsubouchi, Jun Araya, Shunsuke Minagawa, Hiromichi Hara, Akihiro Ichikawa, Nayuta Saito, Tsukasa Kadota, Nahoko Sato, Masahiro Yoshida, Yusuke Kurita, Kenji Kobayashi, Saburo Ito, Yu Fujita, Hirofumi Utsumi, Haruhiko Yanagisawa, Mitsuo Hashimoto, Hiroshi Wakui, Yutaka Yoshii, Takeo Ishikawa, Takanori Numata, Yumi Kaneko, Hisatoshi Asano, Makoto Yamashita, Makoto Odaka, Toshiaki Morikawa, Katsutoshi Nakayama, Yoichi Nakanishi, Kazuyoshi Kuwano
Accumulation of profibrotic myofibroblasts is involved in the process of fibrosis development during idiopathic pulmonary fibrosis (IPF) pathogenesis. TGFB (transforming growth factor beta) is one of the major profibrotic cytokines for myofibroblast differentiation and NOX4 (NADPH oxidase 4) has an essential role in TGFB-mediated cell signaling. Azithromycin (AZM), a second-generation antibacterial macrolide, has a pleiotropic effect on cellular processes including proteostasis. Hence, we hypothesized that AZM may regulate NOX4 levels by modulating proteostasis machineries, resulting in inhibition of TGFB-associated lung fibrosis development...
June 14, 2017: Autophagy
https://www.readbyqxmd.com/read/28613935/obstructive-sleep-apnea-and-subclinical-interstitial-lung-disease-in-mesa
#17
John S Kim, Anna J Podolanczuk, Priya Borker, Steven M Kawut, Ganesh Raghu, Joel D Kaufman, Karen D Hinckley Stukovsky, Eric A Hoffman, R Graham Barr, Daniel J Gottlieb, Susan S Redline, David J Lederer
RATIONALE: Obstructive sleep apnea (OSA) has been postulated to contribute to idiopathic pulmonary fibrosis by promoting alveolar epithelial injury via tractional forces and intermittent hypoxia. OBJECTIVE: To determine whether OSA is associated with subclinical interstitial lung disease (ILD) and with biomarkers of alveolar epithelial injury and remodeling. METHODS: We performed cross-sectional analyses of 1,690 community-dwelling adults who underwent 15-channel in-home polysomnography and thoracic computed tomography (CT) imaging in the Multi-Ethnic Study of Atherosclerosis...
June 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28611075/identification-and-functional-characterization-of-hypoxia-induced-endoplasmic-reticulum-stress-regulating-lncrna-hyperlnc-in-pericytes
#18
Florian C Bischoff, Astrid Werner, David John, Jes-Niels Boeckel, Maria-Theodora Melissari, Phillip Grote, Simone F Glaser, Shemsi Demolli, Shizuka Uchida, Katharina M Michalik, Benjamin Meder, Hugo A Katus, Jan Haas, Wei Chen, Soni S Pullamsetti, Werner Seeger, Andreas M Zeiher, Stefanie Dimmeler, Christoph M Zehendner
Rationale: Pericytes are essential for vessel maturation and endothelial barrier function. Long non-coding RNAs (lncRNAs) regulate many cellular functions, but their role in pericyte biology remains unexplored. Objective: Here we investigate the effect of Hypoxia-Induced Endoplasmic Reticulum Stress Regulating lncRNA (HypERlnc, also known as ENSG00000262454) on pericyte function in vitro and its regulation in human heart failure and idiopathic pulmonary arterial hypertension. Methods and Results: RNA sequencing in human primary pericytes (hPCs) identified hypoxia regulated lncRNAs, including HypERlnc...
June 13, 2017: Circulation Research
https://www.readbyqxmd.com/read/28610674/elevated-serum-d-dimer-level-is-associated-with-an-increased-risk-of-acute-exacerbation-in-interstitial-lung-disease
#19
Genta Ishikawa, Samuel O Acquah, Mary Salvatore, Maria L Padilla
BACKGROUND: Early recognition of patients with interstitial lung disease (ILD) who have an increased risk of developing acute exacerbation (AE) or preclinical AE may be clinically useful, since AE is associated with poor outcome and preventive measures would be of interest to ILD researchers. This study evaluated the relationship between elevated serum D-dimer level (≥0.4 mcg/mL) and subsequent AE or preclinical AE in patients with ILD. METHODS: This single-center, retrospective study was performed from October 2009 through September 2015 in patients with ILD who were ≥18 years old and had idiopathic pulmonary fibrosis, other idiopathic interstitial pneumonias, chronic hypersensitivity pneumonitis, ILD related to collagen tissue disease, or combined pulmonary fibrosis/emphysema...
July 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28605770/improving-biocuration-of-micrornas-in-diseases-a-case-study-in-idiopathic-pulmonary-fibrosis
#20
Yalbi Itzel Balderas-Martínez, Fabio Rinaldi, Gabriela Contreras, Hilda Solano-Lira, Mishael Sánchez-Pérez, Julio Collado-Vides, Moisés Selman, Annie Pardo
http://odin.ccg.unam.mx/ODIN/bc2015-miRNA/.
January 1, 2017: Database: the Journal of Biological Databases and Curation
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