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idiopathic fibrosis pulmonary

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https://www.readbyqxmd.com/read/29355549/air-pollution-exposure-is-associated-with-lower-lung-function-but-not-changes-in-lung-function-in-patients-with-idiopathic-pulmonary-fibrosis
#1
Kerri A Johannson, Eric Vittinghoff, Julie Morisset, Paul J Wolters, Elizabeth M Noth, John R Balmes, Harold R Collard
BACKGROUND: Air pollution exposure is associated with acute exacerbation, disease progression and mortality in patients with idiopathic pulmonary fibrosis (IPF). The objective of this study was to describe the impact of air pollution exposures on disease severity, and changes in lung function, in patients with IPF. METHODS: Using home spirometers and symptom diaries, 25 patients with IPF prospectively recorded forced vital capacity (FVC) weekly for up to 40 weeks...
January 17, 2018: Chest
https://www.readbyqxmd.com/read/29355514/a-shared-pattern-of-%C3%AE-catenin-activation-in-bronchopulmonary-dysplasia-and-idiopathic-pulmonary-fibrosis
#2
Jennifer M S Sucre, Gail H Deutsch, Christopher Jetter, Namasivayam Ambalavanan, John T Benjamin, Linda A Gleaves, Bryan A Millis, Lisa R Young, Timothy S Blackwell, Jonathan A Kropski, Susan H Guttentag
Wnt/β-catenin signaling is necessary for normal lung development, and abnormal Wnt signaling contributes to the pathogenesis of both bronchopulmonary dysplasia (BPD) and idiopathic pulmonary fibrosis (IPF), fibrotic lung diseases that occur during infancy and aging, respectively. Using a library of human normal and diseased human lung samples, we identified a distinct signature of nuclear accumulation of β-catenin phosphorylated at tyrosine 489 and epithelial cell cytosolic localization of β-catenin phosphorylated at tyrosine 654 in early normal lung development and fibrotic lung diseases BPD and IPF...
January 16, 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29355493/elongation-factor-2-kinase-acts-downstream-of-p38-mapk-to-regulate-proliferation-apoptosis-and-autophagy-in-human-lung-fibroblasts
#3
Yanni Wang, Guojin Huang, Zhixia Wang, Huiping Qin, Biwen Mo, Changming Wang
Idiopathic pulmonary fibrosis (IPF) is a chronic, fatal and progressive fibro-proliferative lung disease, and fibroblast-to-myofibroblast differentiation is a crucial process in the development of IPF. Elongation factor-2 kinase (eEF2K) has been reported to play an important role in various disease types, but the role of eEF2K in IPF is unknown. In this study, we investigated the role of eEF2K in normal lung fibroblast (NHLF) proliferation, differentiation, apoptosis, and autophagy as well as the interaction between eEF2K and p38 MAPK signaling through in vitro experiments...
January 17, 2018: Experimental Cell Research
https://www.readbyqxmd.com/read/29354175/open-lung-biopsy-performed-in-idiopathic-pulmonary-fibrosis-is-a-safe-procedure
#4
Tomasz Marjański, Joanna Halman, Sonia Taniewska, Natalia Burzyńska, Anna Piekarska, Wioletta Sawicka
Introduction: Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with a fatal prognosis. The diagnosis is made on the basis of high-resolution computed tomography and histological examination in selected cases. Aim: To determine the risk of complications of open lung biopsy performed in patients with IPF. Material and methods: We performed a retrospective analysis of 51 patients who underwent diagnostic excision of pulmonary parenchyma due to IPF in the period 1995-2014...
December 2017: Kardiochirurgia i Torakochirurgia Polska, Polish Journal of Cardio-Thoracic Surgery
https://www.readbyqxmd.com/read/29352073/right-ventricular-myofilament-functional-differences-in-humans-with-systemic-sclerosis-associated-versus-idiopathic-pulmonary-arterial-hypertension
#5
Steven Hsu, Kristen M Kokkonen-Simon, Jonathan A Kirk, Todd M Kolb, Rachel L Damico, Stephen C Mathai, Monica Mukherjee, Ami A Shah, Fredrick M Wigley, Kenneth B Margulies, Paul M Hassoun, Marc K Halushka, Ryan J Tedford, David A Kass
Background -Patients with systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) have a far worse prognosis than those with idiopathic PAH (IPAH). In the intact heart, SSc-PAH exhibits depressed rest and reserve right ventricular (RV) contractility as compared to IPAH. We tested whether this disparity involves underlying differences in myofilament function. Methods -Cardiac myocytes were isolated from RV septal endomyocardial biopsies from patients with SSc-PAH, IPAH, or SSc with exertional dyspnea but no resting PAH (SSc-d); control RV septal tissue was obtained from non-diseased donor hearts (6-7/group)...
January 19, 2018: Circulation
https://www.readbyqxmd.com/read/29351446/mechanisms-and-consequences-of-oxidative-stress-in-lung-disease-therapeutic-implications-for-an-aging-populace
#6
Louise Hecker
The rapid expansion of the elderly population has led to the recent epidemic of age-related diseases, including increased incidence and mortality of chronic and acute lung diseases. Numerous studies have implicated aging and oxidative stress in the pathogenesis of various pulmonary diseases; however, despite recent advances in these fields, the specific contributions of aging and oxidative stress remain elusive. This review will discuss the consequences of aging on lung morphology and physiology, and how redox imbalance with aging contributes to lung disease susceptibility...
December 14, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29351129/impact-of-pulmonary-rehabilitation-on-exercise-tolerance-and-quality-of-life-in-patients-with-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#7
Mansueto Gomes-Neto, Cassio Magalhães Silva, Diego Ezequiel, Cristiano Sena Conceição, Micheli Saquetto, Adelmir Souza Machado
BACKGROUND: Patients with pulmonary fibrosis are living longer and present with an increasing number of comorbidities over time. Pulmonary rehabilitation, as a nonpharmacological approach, may be promising in these patients, although there is limited information on the impact of pulmonary rehabilitation on exercise tolerance and quality of life. Thus, conducting a systematic review and meta-analysis, the purpose of this study was to determine the effects of pulmonary rehabilitation on exercise tolerance and quality of life in patients with idiopathic pulmonary fibrosis...
January 18, 2018: Journal of Cardiopulmonary Rehabilitation and Prevention
https://www.readbyqxmd.com/read/29348469/treprostinil-inhibits-proliferation-and-extracellular-matrix-deposition-by-fibroblasts-through-camp-activation
#8
Christopher Lambers, Michael Roth, Peter Jaksch, Gabriella Muraközy, Michael Tamm, Walter Klepetko, Bahil Ghanim, Feng Zhao
Idiopathic pulmonary fibrosis (IPF) is characterized by peripheral lung fibrosis and increased interstitial extracellular matrix (ECM) deposition. In IPF, tumor growth factor (TGF)-β1 which is the major stimulus of ECM deposition, and platelet derived growth factor (PDGF)-BB is a potent stimulus of fibrosis. Thus, the effect of Treprostinil on TGF-ß1 and PDGF-induced fibroblast proliferation and ECM deposition was investigated. Human peripheral lung fibroblasts of seven IPF patients and five lung donors were stimulated by PDGF, or TGF-β1, or the combination...
January 18, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29348188/the-unmet-medical-need-of-pulmonary-hypertension-in-idiopathic-pulmonary-fibrosis
#9
EDITORIAL
Vincent Cottin, Laura C Price, Claudia Valenzuela
No abstract text is available yet for this article.
January 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29348187/the-increasing-mortality-of-idiopathic-pulmonary-fibrosis-fact-or-fallacy
#10
EDITORIAL
Christopher J Ryerson, Martin Kolb
No abstract text is available yet for this article.
January 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29348182/trends-in-mortality-from-idiopathic-pulmonary-fibrosis-in-the-european-union-an-observational-study-of-the-who-mortality-database-from-2001-2013
#11
Dominic C Marshall, Justin D Salciccioli, Barry S Shea, Praveen Akuthota
Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias and is characterised by progressive accumulation of scar tissue in the lungs. The objective of this study was to describe the current mortality rates due to IPF in Europe, based on the World Health Organization (WHO) mortality database.We used country-level data for IPF mortality, identified in the WHO mortality database using International Classification of Diseases 10th Edition (ICD-10) codes, for the period 2001-2013...
January 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29348178/a-scoring-system-to-predict-the-elevation-of-mean-pulmonary-arterial-pressure-in-idiopathic-pulmonary-fibrosis
#12
Taiki Furukawa, Yasuhiro Kondoh, Hiroyuki Taniguchi, Mitsuaki Yagi, Toshiaki Matsuda, Tomoki Kimura, Kensuke Kataoka, Takeshi Johkoh, Masahiko Ando, Naozumi Hashimoto, Koji Sakamoto, Yoshinori Hasegawa
Elevated mean pulmonary arterial pressure (MPAP; ≥21 mmHg) is sometimes seen in patients with idiopathic pulmonary fibrosis (IPF) and has an adverse impact upon survival. Although early diagnosis is crucial, there is no established screening tool that uses a combination of noninvasive examinations.We retrospectively analysed IPF patients at initial evaluation from April 2007 to July 2015 and, using logistic regression analysis, created a screening tool to identify elevated MPAP. Internal validation was also assessed for external validity using a bootstrap method...
January 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29346191/the-four-corners-sign-a-specific-imaging-feature-in-differentiating-systemic-sclerosis-related-interstitial-lung-disease-from-idiopathic-pulmonary-fibrosis
#13
Lara Walkoff, Darin B White, Jonathan H Chung, Dennis Asante, Christian W Cox
PURPOSE: Differentiating between systemic sclerosis-related interstitial lung disease (SSc-ILD) and idiopathic pulmonary fibrosis (IPF) is important because of the differences in workup, prognosis, and treatment. However, there is much overlap in the appearance of these 2 entities on high-resolution computed tomography. We propose that inflammation and/or fibrosis focally or disproportionately involving the bilateral anterolateral upper lobes and posterosuperior lower lobes ["Four Corners" Sign (FCS)] is specific for SSc-ILD...
January 16, 2018: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/29345973/r-spondin-2-is-upregulated-in-idiopathic-pulmonary-fibrosis-and-affects-fibroblasts-behavior
#14
Adrián Munguía-Reyes, Yalbi I Balderas-Martínez, Carina Becerril, Marco Checa, Remedios Ramírez, Blanca Ortíz-Quintero, Jorge Meléndez-Zajgla, Annie Pardo, Moisés Selman
Idiopathic pulmonary fibrosis (IPF) is characterized by the expansion of the myofibroblasts population, excessive extracellular matrix accumulation, and destruction of the lung parenchyma. The R-spondins family (RSPO) comprises a group of proteins essential for development. From them, RSPO2 is expressed primarily in the lungs and its mutations cause severe defects in the respiratory tract. Interestingly, RSPO2 participates in the canonical WNT pathway, a critical route in the pathogenesis of IPF. Thus, the aim of this study was to examine the expression and putative role of RSPO2 in this disease...
January 18, 2018: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/29343236/barriers-to-timely-diagnosis-of-interstitial-lung-disease-in-the-real-world-the-intensity-survey
#15
Gregory P Cosgrove, Pauline Bianchi, Sherry Danese, David J Lederer
BACKGROUND: The diagnosis of idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILD) presents significant clinical challenges. To gain insights regarding the diagnostic experience of patients with ILD and to identify potential barriers to a timely and accurate diagnosis, we developed an online questionnaire and conducted a national survey of adults with a self-reported diagnosis of ILD. METHODS: A pre-specified total of 600 subjects were recruited to participate in a 40-question online survey...
January 17, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29341047/german-guideline-for-idiopathic-pulmonary-fibrosis-update-on-pharmacological-therapies-2017
#16
Jürgen Behr, Andreas Günther, Francesco Bonella, Klaus Geißler, Dirk Koschel, Michael Kreuter, Antje Prasse, Nicolas Schönfeld, Helmut Sitter, Joachim Müller-Quernheim, Ulrich Costabel
Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease with a median survival of 2 - 4 years after diagnosis. Since the publication of the German IPF guideline in 2013 new treatment trials have been published, necessitating an update of the pharmacological therapy of IPF. Different from the previous guideline, the GRADE system was discarded and replaced by the Oxford evidence classification system which allows a more differentiated judgement. The following pharmacological therapies were rated not suitable for the treatment of IPF patients (recommendation A; evidence 1-b): triple therapy with prednisolone, azathioprine and acetyl-cysteine; imatinib; ambrisentan; bosentan; macitentan...
January 16, 2018: Pneumologie
https://www.readbyqxmd.com/read/29340493/mortality-from-idiopathic-pulmonary-fibrosis-a-temporal-trend-analysis-in-brazil-1979-2014
#17
Eduardo Algranti, Cézar Akiyoshi Saito, Diego Rodrigues Mendonça E Silva, Ana Paula Scalia Carneiro, Marco Antonio Bussacos
OBJECTIVE: To analyze mortality from idiopathic pulmonary fibrosis (IPF) in Brazil over the period 1979-2014. METHODS: Microdata were extracted from the Brazilian National Ministry of Health Mortality Database. Only deaths for which the underlying cause was coded as International Classification of Diseases version 9 (ICD-9) 515 or 516.3 (until 1995) or as ICD version 10 (ICD-10) J84.1 (from 1996 onward) were included in our analysis. Standardized mortality rates were calculated for the 2010 Brazilian population...
November 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/29340483/idiopathic-pulmonary-fibrosis-in-brazil-challenges-for-epidemiological-characterization-and-management
#18
EDITORIAL
Bruno Guedes Baldi
No abstract text is available yet for this article.
November 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/29335860/effectiveness-and-safety-of-chinese-medicine-for-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#19
Qi Wu, Yao Zhou, Fan-Chao Feng, Xian-Mei Zhou
OBJECTIVE: To evaluate the effectiveness and safety of Chinese medicine (CM) for Idiopathic pulmonary fifibrosis (IPF) patients. METHODS: To screened relevant articles, PubMed, Cochrane Library, Excerpta Medica Datase (EMBASE), China National Knowledge Infrastructure (CNKI), Chinese VIP Information (VIP), Wanfang Database and Chinese Biomedical Database (CBM) were searched in English or Chinese until December 2015 for randomized controlled trials, which compared CM treatment (CM group) with Western medicine or placebo (control group) on IPF...
January 15, 2018: Chinese Journal of Integrative Medicine
https://www.readbyqxmd.com/read/29335764/pulmonary-18f-fdg-uptake-helps-refine-current-risk-stratification-in-idiopathic-pulmonary-fibrosis-ipf
#20
Thida Win, Nicholas J Screaton, Joanna C Porter, Balaji Ganeshan, Toby M Maher, Francesco Fraioli, Raymondo Endozo, Robert I Shortman, Lynn Hurrell, Beverley F Holman, Kris Thielemans, Alaleh Rashidnasab, Brian F Hutton, Pauline T Lukey, Aiden Flynn, Peter J Ell, Ashley M Groves
PURPOSE: There is a lack of prognostic biomarkers in idiopathic pulmonary fibrosis (IPF) patients. The objective of this study is to investigate the potential of 18F-FDG-PET/ CT to predict mortality in IPF. METHODS: A total of 113 IPF patients (93 males, 20 females, mean age ± SD: 70 ± 9 years) were prospectively recruited for 18F-FDG-PET/CT. The overall maximum pulmonary uptake of 18F-FDG (SUVmax), the minimum pulmonary uptake or background lung activity (SUVmin), and target-to-background (SUVmax/ SUVmin) ratio (TBR) were quantified using routine region-of-interest analysis...
January 16, 2018: European Journal of Nuclear Medicine and Molecular Imaging
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