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https://www.readbyqxmd.com/read/28343230/idiopathic-pulmonary-fibrosis-in-switzerland-diagnosis-and-treatment
#1
Manuela Funke-Chambour, Andrea Azzola, Dan Adler, Constance Barazzone-Argiroffo, Christian Benden, Annette Boehler, Pierre-Olivier Bridevaux, Martin Brutsche, Christian F Clarenbach, Katrin Hostettler, Rebekka Kleiner-Finger, Laurent P Nicod, Paola M Soccal, Michael Tamm, Thomas Geiser, Romain Lazor
Idiopathic pulmonary fibrosis (IPF) is a severe progressive and irreversible lung disease. Novel antifibrotic drugs that slow disease progression are now available. However, many issues regarding patient management remain unanswered, such as the choice between available drugs, their use in particular subgroups and clinical situations, time of treatment onset, termination, combination or switch, or nonpharmacologic management. To guide Swiss respiratory physicians in this evolving field still characterized by numerous areas of uncertainty, the Swiss Working Group for interstitial and rare lung diseases of the Swiss Respiratory Society provides a position paper on the diagnosis and treatment of IPF...
March 25, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28339739/unabated-occupational-risk-in-a-patient-with-rheumatoid-pulmonary-fibrosis
#2
V K Holden, S J Kligerman, T G Hastings, S E Hines
Background: This case highlights the importance of considering hypersensitivity pneumonitis (HP) in the differential diagnosis of interstitial lung disease (ILD) and of obtaining an occupational history so that remediable risk factors may be identified and managed. Aims: To report a case of a chicken sexer with severe rheumatoid arthritis (RA) who developed progressively worsening dyspnoea and restrictive lung disease associated with pulmonary fibrosis. Methods: Clinical investigation included physical examination, occupational history, pulmonary function tests (PFTs), chest imaging and bronchoalveolar lavage (BAL), as well as serological tests including standard IgE bird feather mixture and local IgG precipitin preparation to chicken excrement...
February 23, 2017: Occupational Medicine
https://www.readbyqxmd.com/read/28339528/the-utility-of-comprehensive-autoantibody-testing-to-differentiate-connective-tissue-disease-associated-and-idiopathic-interstitial-lung-disease-subgroup-cases
#3
Caroline V Cotton, Lisa G Spencer, Robert P New, Robert G Cooper
Interstitial lung disease (ILD) comprises many heterogeneous disease groups, the largest being CTD-associated and those labelled as idiopathic out of necessity. The mechanisms causing ILD are poorly understood, but most CTD- and idiopathic-ILD cases can respond to immunosuppression, clearly suggesting a pathological role for inflammation. By contrast, corticosteroid immunosuppression causes harm without benefit in the feared idiopathic pulmonary fibrosis, suggesting that inflammation plays little pathological role, and where ILD progresses rapidly to lethal outcome even with anti-fibrotic drug use...
October 20, 2016: Rheumatology
https://www.readbyqxmd.com/read/28339466/in-silico-search-for-modifier-genes-associated-with-pancreatic-and-liver-disease-in-cystic-fibrosis
#4
Pascal Trouvé, Emmanuelle Génin, Claude Férec
Cystic Fibrosis is the most common lethal autosomal recessive disorder in the white population, affecting among other organs, the lung, the pancreas and the liver. Whereas Cystic Fibrosis is a monogenic disease, many studies reveal a very complex relationship between genotype and clinical phenotype. Indeed, the broad phenotypic spectrum observed in Cystic Fibrosis is far from being explained by obvious genotype-phenotype correlations and it is admitted that Cystic Fibrosis disease is the result of multiple factors, including effects of the environment as well as modifier genes...
2017: PloS One
https://www.readbyqxmd.com/read/28339089/hmgb1-induces-lung-fibroblast-to-myofibroblast-differentiation-through-nf%C3%A2-%C3%AE%C2%BAb%C3%A2-mediated-tgf%C3%A2-%C3%AE-1-release
#5
Qiong Wang, Jun Wang, Junfang Wang, Shanchao Hong, Feifei Han, Jingyu Chen, Guoqian Chen
The proinflammatory factor high‑mobility group box protein 1 (HMGB1) has been implicated in the pathogenesis of lung fibrosis; however, the role of HMGB1 in lung fibrosis remains unclear. It has previously been reported that nuclear factor (NF)‑κB and transforming growth factor (TGF)‑β1 may be involved in lung fibrosis. Therefore, the present study aimed to examine the potential molecular mechanisms that underlie HMGB1‑induced lung fibrosis via the regulation of NF‑κB and TGF‑β1. The results demonstrated that HMGB1 stimulation increased the activation of NF‑κB and the release of TGF‑β1, as well as the expression of α‑smooth muscle actin (α‑SMA) and collagen I in human lung fibroblasts in vitro...
March 23, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28337871/cytokine-modulation-in-patients-with-idiopathic-pulmonary-fibrosis-undergoing-treatment-with-steroids-immunosuppressants-and-ifn-%C3%AE-1b
#6
S Marinari, V De Iuliis, V Dadorante, S Colella, A Marino, A Nunziata, V Flati, M Caruso, A Pennelli, F De Benedetto, S Matera, S Capodifoglio, S Martinotti, S Caputi, E Toniato
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown etiology and pathogenic mechanisms. From an etiopathogenic point of view, alveolar macrophages play a key role in accumulation of fibroblasts and deposition of collagen and extracellular matrix by releasing specific cytokines and inflammatory mediators. IPF seems to be also associated with circulating fibrocytes, which might be involved with an abnormal pulmonary vascular repair and remodeling. Based on its hypothesized pathologic mechanisms, anti-inflammatory, anti-fibrotic and immunosuppressive therapies are often used...
January 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28337719/an-early-health-economic-analysis-of-the-potential-cost-effectiveness-of-an-adherence-intervention-to-improve-outcomes-for-patients-with-cystic-fibrosis
#7
Paul Tappenden, Susannah Sadler, Martin Wildman
BACKGROUND: Cystic fibrosis (CF) negatively impacts upon health-related quality of life and survival. Adherence to nebulised treatments is low; improving adherence is hypothesised to reduce rates of exacerbation requiring intravenous antibiotics and lung function decline. OBJECTIVE: A state transition model was developed to assess the cost effectiveness of an intervention aimed at increasing patient adherence to nebulised and inhaled antibiotics compared with current CF care, in advance of the forthcoming CFHealthHub randomised controlled trial (RCT)...
March 23, 2017: PharmacoEconomics
https://www.readbyqxmd.com/read/28337699/lung-ultrasound-is-comparable-with-chest-roentgenogram-for-diagnosis-of-community-acquired-pneumonia-in-hospitalised-children
#8
Krishna Kumar Yadav, Shally Awasthi, Anit Parihar
OBJECTIVE: To evaluate the accuracy of lung ultrasound (LUS) in comparison to chest roentgenogram (CXR) in hospitalised children with community-acquired pneumonia (CAP). METHODS: This study was a hospital based prospective observational study, conducted between January 2014 and December 2014. Hospitalised children aged 2 to 59 mo with community-acquired pneumonia were included in the study. The informed written consent was taken from parents (or legal guardian) before recruitment...
March 24, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28337441/pentoxifylline-regulates-plasminogen-activator-inhibitor-1-expression-and-protein-kinase-a-phosphorylation-in-radiation-induced-lung-fibrosis
#9
Jong-Geol Lee, Sehwan Shim, Min-Jung Kim, Jae Kyung Myung, Won-Suk Jang, Chang-Hwan Bae, Sun-Joo Lee, Kyeong Min Kim, Young-Woo Jin, Seung-Sook Lee, Sunhoo Park
Purpose. Radiation-induced lung fibrosis (RILF) is a serious late complication of radiotherapy. In vitro studies have demonstrated that pentoxifylline (PTX) has suppressing effects in extracellular matrix production in fibroblasts, while the antifibrotic action of PTX alone using clinical dose is yet unexplored. Materials and Methods. We used micro-computed tomography (micro-CT) and histopathological analysis to evaluate the antifibrotic effects of PTX in a rat model of RILF. Results. Micro-CT findings showed that lung density, volume loss, and mediastinal shift are significantly increased at 16 weeks after irradiation...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28337301/endoplasmic-reticulum-stress-a-new-wrestler-in-the-pathogenesis-of-idiopathic-pulmonary-fibrosis
#10
Lei Zhang, Yi Wang, Nuruliarizki Shinta Pandupuspitasari, Guorao Wu, Xudong Xiang, Quan Gong, Weining Xiong, Cong-Yi Wang, Ping Yang, Boxu Ren
Idiopathic pulmonary fibrosis (IPF) has attracted extensive attention for its unexplained progressive lung scarring, short median survival and its unresponsiveness to traditional therapies. Despite extensive studies, the mechanisms underlying IPF pathoetiologies, however, remain poorly understood. Recent advances delineated a potential function of endoplasmic reticulum (ER) stress in meeting the need of fibrotic response, which pinpointed a critical role for the unfolded protein response (UPR) pathways in IPF pathogenesis...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28337251/pentaerythritol-tetranitrate-in-vivo-treatment-improves-oxidative-stress-and-vascular-dysfunction-by-suppression-of-endothelin-1-signaling-in-monocrotaline-induced-pulmonary-hypertension
#11
Sebastian Steven, Matthias Oelze, Moritz Brandt, Elisabeth Ullmann, Swenja Kröller-Schön, Tjebo Heeren, Lan P Tran, Steffen Daub, Mobin Dib, Dirk Stalleicken, Philip Wenzel, Thomas Münzel, Andreas Daiber
Objective. Oxidative stress and endothelial dysfunction contribute to pulmonary arterial hypertension (PAH). The role of the nitrovasodilator pentaerythritol tetranitrate (PETN) on endothelial function and oxidative stress in PAH has not yet been defined. Methods and Results. PAH was induced by monocrotaline (MCT, i.v.) in Wistar rats. Low (30 mg/kg; MCT30), middle (40 mg/kg; MCT40), or high (60 mg/kg; MCT60) dose of MCT for 14, 28, and 42 d was used. MCT induced endothelial dysfunction, pulmonary vascular wall thickening, and fibrosis, as well as protein tyrosine nitration...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28336963/radiological-and-clinical-features-associated-with-epidermal-growth-factor-receptor-mutation-status-of-exon-19-and-21-in-lung-adenocarcinoma
#12
Zhang Shi, Xuan Zheng, Ruifeng Shi, Changen Song, Runhong Yang, Qianwen Zhang, Xinrui Wang, Jianping Lu, Yongwei Yu, Qi Liu, Tao Jiang
The exon 19 and 21 in Epidermal Growth Factor Receptor (EGFR) mutation are the most common subtype of lung adenocarcinoma, and the strongest predictive biomarker for progression-free survival and tumor response. Although some studies have shown differences in radiological features between cases with and without EFGR mutations, they lacked necessary stratification. This article is to evaluate the association of CT features between the wild type and the subtype (exon 19 and 21) of EGFR mutations in patients with lung adenocarcinoma...
March 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28336945/x-ray-dark-field-radiography-facilitates-the-diagnosis-of-pulmonary-fibrosis-in-a-mouse-model
#13
Katharina Hellbach, Andre Yaroshenko, Konstantin Willer, Thomas M Conlon, Margarita B Braunagel, Sigrid Auweter, Ali Ö Yildirim, Oliver Eickelberg, Franz Pfeiffer, Maximilian F Reiser, Felix G Meinel
The aim of this study was to evaluate whether diagnosing pulmonary fibrosis with projection radiography can be improved by using X-ray dark-field radiograms. Pulmonary X-ray transmission and dark-field images of C57Bl/6N mice, either treated with bleomycin to induce pulmonary fibrosis or PBS to serve as controls, were acquired with a prototype grating-based small-animal scanner. Two blinded readers, both experienced radiologists and familiar with dark-field imaging, had to assess dark-field and transmission images for the absence or presence of fibrosis...
March 23, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28336812/tgf-%C3%AE-1-stimulates-hdac4-nucleus-to-cytoplasm-translocation-and-nadph-oxidase4-derived-reactive-oxygen-species-in-normal-human-lung-fibroblasts
#14
Weichao Guo, Shigeki Saito, Cecilia G Sanchez, Yan Zhuang, Rafael E Gongora Rosero, Bin Shan, Fayong Luo, Joseph A Lasky
Myofibroblasts are important mediators of fibrogenesis, thus blocking fibroblast to myofibroblast differentiation (FMD) may be an effective strategy to treat pulmonary fibrosis (PF). Previously we reported that HDAC4 activity is necessary for TGF-β1-induced human lung FMD. Here we show that TGF-β1 increases NOX4 mRNA and protein expression in normal human lung fibroblasts (NHLFs) and causes nuclear export of HDAC4. Application of the NOX family inhibitor, diphenyleneiodonium chloride (DPI), reduces TGF-β1-induced HDAC4 nuclear export, expression of the myofibroblast marker α-smooth muscle actin (α-SMA), and α-SMA fiber formation...
March 23, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28336132/complete-genome-sequence-of-sequential-pandoraea-apista-isolates-from-the-same-cystic-fibrosis-patient-supports-a-model-of-chronic-colonization-with-in-vivo-strain-evolution-over-time
#15
Alexander L Greninger, Jessica Streithorst, Jeffrey A Golden, Charles Y Chiu, Steve Miller
Pandoraea apista in the family Burkholderiaceae is an emerging opportunistic pathogen in cystic fibrosis patients. Here, we describe a case from which 3 separate isolates of P. apista were recovered over a 1-year period. Using a combination of first-, second-, and third-generation sequencing technologies, we sequenced and de novo assembled the complete genomes of these 3 P. apista isolates. The genome of P. apista TF81F4 sequenced in this study was 5.58 Mb with a GC% of 62.3%, differed in sequence from other Pandoraea species by >20%, and included a number of previously undescribed loci...
January 2017: Diagnostic Microbiology and Infectious Disease
https://www.readbyqxmd.com/read/28335088/further-characterization-of-computed-tomographic-and-clinical-features-for-staging-and-prognosis-of-idiopathic-pulmonary-fibrosis-in-west-highland-white-terriers
#16
Florence Thierry, Ian Handel, Gawain Hammond, Lesley G King, Brendan M Corcoran, Tobias Schwarz
Idiopathic pulmonary fibrosis is an interstitial lung disease of unknown etiology resulting in progressive interstitial fibrosis, with a known predilection in West Highland white terriers. In humans, computed tomography (CT) is a standard method for providing diagnostic and prognostic information, and plays a major role in the idiopathic pulmonary fibrosis staging process. Objectives of this retrospective, analytical, cross-sectional study were to establish descriptive criteria for reporting CT findings and test correlations among CT, clinical findings and survival time in West Highland white terriers with idiopathic pulmonary fibrosis...
March 23, 2017: Veterinary Radiology & Ultrasound
https://www.readbyqxmd.com/read/28333981/lipopolysaccharide-induced-endotoxemia-in-corn-oil-preloaded-mice-causes-an-extended-course-of-lung-injury-and-repair-and-pulmonary-fibrosis-a-translational-mouse-model-of-acute-respiratory-distress-syndrome
#17
Chaomin Wu, Colin E Evans, Zhiyu Dai, Xiaojia Huang, Xianming Zhang, Hua Jin, Guochang Hu, Yuanlin Song, You-Yang Zhao
Acute respiratory distress syndrome (ARDS) is characterized by acute hypoxemia respiratory failure, bilateral pulmonary infiltrates, and pulmonary edema of non-cardiac origin. Effective treatments for ARDS patients may arise from experimental studies with translational mouse models of this disease that aim to delineate the mechanisms underlying the disease pathogenesis. Mouse models of ARDS, however, can be limited by their rapid progression from injured to recovery state, which is in contrast to the course of ARDS in humans...
2017: PloS One
https://www.readbyqxmd.com/read/28332341/risk-factors-for-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#18
Meihua Qiu, Yuqing Chen, Qiao Ye
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic andprogressive fibrotic disease limited to the lungs.The course of disease varies widely, with some patients experiencing acute respiratory deterioration, a conditioncalled acute exacerbations of IPF (AE-IPF). The risk factors contributing to AE-IPF are unclear. This systematic review and meta-analysis investigatedthe risk factors for AE-IPF. METHODS: Studies ofrisk factors for AE-IPF were identified in Medline, EMBASE and Cochrane databases...
March 23, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28330773/a-3-year-prognostic-score-for-adults-with-cystic-fibrosis
#19
L Nkam, J Lambert, A Latouche, G Bellis, P R Burgel, M N Hocine
BACKGROUND: Therapeutic progress in patients with cystic fibrosis (CF) has resulted in improved prognosis over the past decades. We aim to reevaluate prognostic factors of CF and provide a prognostic score to predict the risk of death or lung transplantation (LT) within a 3-year period in adult patients. METHODS: We developed a logistic model using data from the French CF Registry and combined the coefficients into a prognostic score. The discriminative abilities of the model and the prognostic score were assessed by c-statistic...
March 18, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28330499/a-novel-multi-network-approach-reveals-tissue-specific-cellular-modulators-of-fibrosis-in-systemic-sclerosis
#20
Jaclyn N Taroni, Casey S Greene, Viktor Martyanov, Tammara A Wood, Romy B Christmann, Harrison W Farber, Robert A Lafyatis, Christopher P Denton, Monique E Hinchcliff, Patricia A Pioli, J Matthew Mahoney, Michael L Whitfield
BACKGROUND: Systemic sclerosis (SSc) is a multi-organ autoimmune disease characterized by skin fibrosis. Internal organ involvement is heterogeneous. It is unknown whether disease mechanisms are common across all involved affected tissues or if each manifestation has a distinct underlying pathology. METHODS: We used consensus clustering to compare gene expression profiles of biopsies from four SSc-affected tissues (skin, lung, esophagus, and peripheral blood) from patients with SSc, and the related conditions pulmonary fibrosis (PF) and pulmonary arterial hypertension, and derived a consensus disease-associate signature across all tissues...
March 23, 2017: Genome Medicine
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