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https://www.readbyqxmd.com/read/28332341/risk-factors-for-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#1
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/28326312/a-comparison-of-published-multidimensional-indices-to-predict-outcome-in-idiopathic-pulmonary-fibrosis
#2
Charles Sharp, Huzaifa I Adamali, Ann B Millar
Idiopathic pulmonary fibrosis (IPF) has an unpredictable course and prognostic factors are incompletely understood. We aimed to identify prognostic factors, including multidimensional indices from a significant IPF cohort at the Bristol Interstitial Lung Disease Centre in the UK. Patients diagnosed with IPF between 2007 and 2014 were identified. Longitudinal pulmonary physiology and exercise testing results were collated, with all-cause mortality used as the primary outcome. Factors influencing overall, 12- and 24-month survival were identified using Cox proportional hazards modelling and receiver operating characteristic curve analysis...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28326180/are-physicians-in-primary-health-care-able-to-recognize-pulmonary-fibrosis
#3
Minna Purokivi, Ulla Hodgson, Marjukka Myllärniemi, Eija-Riitta Salomaa, Riitta Kaarteenaho
Background: The early diagnosis of idiopathic pulmonary fibrosis (IPF) has become increasingly important due to evolving treatment options. IPF patients experience a significant delay in receiving an accurate diagnosis, thus delayed access to tertiary care is associated with higher mortality independently from disease severity. Objective: The aims were to evaluate whether there had been a delay in the referral process, and to determine whether the referring doctors had suspected IPF or other interstitial lung disease (ILD) already during the time of referral...
2017: European Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28321059/arterial-carboxyhemoglobin-measurement-is-useful-for-evaluating-pulmonary-inflammation-in-subjects-with-interstitial-lung-disease
#4
Yu Hara, Masaharu Shinkai, Soichiro Kanoh, Yuji Fujikura, Bruce K Rubin, Akihiko Kawana, Takeshi Kaneko
Objective The arterial concentration of carboxyhemoglobin (CO-Hb) in subjects with inflammatory pulmonary disease is higher than that in healthy individuals. We retrospectively analyzed the relationship between the CO-Hb concentration and established markers of disease severity in subjects with interstitial lung disease (ILD). Methods The CO-Hb concentration was measured in subjects with newly diagnosed or untreated ILD and the relationships between the CO-Hb concentration and the serum biomarker levels, lung function, high-resolution CT (HRCT) findings, and the uptake in gallium-67 ((67)Ga) scintigraphy were evaluated...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28315487/xpln-is-modulated-by-hdac-inhibitors-and-negatively-regulates-sparc-expression-by-targeting-mtorc2-in-human-lung-fibroblasts
#5
Koichiro Kamio, Arata Azuma, Jiro Usuki, Kuniko Matsuda, Minoru Inomata, Nobuhiko Nishijima, Shioto Itakura, Hiroki Hayashi, Takeru Kashiwada, Nariaki Kokuho, Kenichiro Atsumi, Tomoyoshi Yamaguchi, Kazue Fujita, Yoshinobu Saito, Shinji Abe, Kaoru Kubota, Akihiko Gemma
Pathogenesis of idiopathic pulmonary fibrosis (IPF) remains unclear. Secreted protein acidic and rich in cysteine (SPARC) is a matricellular protein that participates in the assembly and turnover of the extracellular matrix, whose expression is regulated by transforming growth factor (TGF)-β1 through activation of mammalian target of rapamycin complex 2 (mTORC2). Exchange factor found in platelets, leukemic, and neuronal tissues (XPLN) is an endogenous inhibitor of mTORC2. However, whether XPLN modulates SPARC expression remains unknown...
March 14, 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28314802/an-ex-vivo-model-to-induce-early-fibrosis-like-changes-in-human-precision-cut-lung-slices
#6
Hani N Alsafadi, Claudia A Staab-Weijnitz, Mareike Lehmann, Michael Lindner, Britta Peschel, Melanie Königshoff, Darcy Elizabeth Wagner
IPF is a devastating chronic interstitial lung disease (ILD) characterized by lung tissue scarring and high morbidity. Lung epithelial injury, myofibroblast activation, and deranged repair are believed to be key processes involved in disease onset and progression but the exact molecular mechanisms behind IPF remain unclear. Several drugs have been shown to slow disease progression, but treatments which halt or reverse IPF progression have not been identified. Ex vivo models of human lung have been proposed for drug discovery, one of which is precision-cut lung slices (PCLS)...
March 17, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28300574/sex-steroid-receptor-expression-in-idiopathic-pulmonary-fibrosis
#7
Mitra Mehrad, Humberto E Trejo Bittar, Samuel A Yousem
Usual interstitial pneumonia (UIP) is characterized by progressive scarring of the lungs and is associated with high morbidity and mortality despite therapeutic interventions. Sex steroid receptors have been demonstrated to play an important role in chronic lung conditions; however, their significance is unknown in patients with UIP. We retrospectively reviewed 40 idiopathic UIP cases for the expression of hormonal receptors. Forty cases including 10 normal lung, 10 cryptogenic organizing pneumonia (COP), 10 idiopathic organizing diffuse alveolar damage (DAD), 7 hypersensitivity pneumonitis (HP) and 3 nonspecific interstitial pneumonitis (NSIP) served as controls...
March 11, 2017: Human Pathology
https://www.readbyqxmd.com/read/28300570/idiopathic-interstitial-pneumonia-associated-with-autoantibodies-a-large-case-series-followed-over-one-year
#8
Bridget F Collins, Charles F Spiekerman, Megan A Shaw, Lawrence A Ho, Jennifer Hayes, Carolyn A Spada, Caroline M Stamato, Ganesh Raghu
BACKGROUND: Some patients with autoimmune characteristics and idiopathic interstitial pneumonia (IIP), particularly usual interstitial pneumonia (UIP), do not fit neatly into the category of connective tissue disease associated interstitial lung disease (CTD-ILD), idiopathic pulmonary fibrosis (IPF) or recently proposed yet to be validated criteria for interstitial pneumonia with autoimmune features (IPAF). Outcomes of these patients are unknown. METHODS: Retrospective single center study; ANOVA analyses compared differences in mean change in forced vital capacity (FVC) and diffusion capacity (DLCO) over 1-year among 124 well defined patients (20 AI-ILD [positive autoantibodies with or without symptoms of CTD], 15 IPAF, 36 CTD-ILD, 53 "Lone-IPF" [patients with IPF without any autoantibodies]) RESULTS: 75% of patients with AI-ILD, 33% IPAF, and 33% CTD-ILD had UIP...
March 11, 2017: Chest
https://www.readbyqxmd.com/read/28297158/unclassifiable-interstitial-lung-diseases-clinical-characteristics-and-survival
#9
Charlotte Hyldgaard, Elisabeth Bendstrup, Athol U Wells, Ole Hilberg
BACKGROUND AND OBJECTIVE: Unclassifiable disease in chronic interstitial lung disease (ILD) is a common and challenging problem but has been insufficiently studied. The 2013 update of the international multidisciplinary classification of the idiopathic interstitial pneumonias presented a classification based on observed disease behaviour with the purpose of providing guidance to clinicians in the management of these patients. The aim of this study was to apply the new disease behaviour classification (DBC) and the previously validated ILD-gender age physiology (GAP) score to a cohort of unclassifiable ILD patients and to assess the prognostic value of these two composite approaches...
April 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28294974/mir-30a-as-potential-therapeutics-by-targeting-tet1-through-regulation-of-drp-1-promoter-hydroxymethylation-in-idiopathic-pulmonary-fibrosis
#10
Songzi Zhang, Huizhu Liu, Yuxia Liu, Jie Zhang, Hongbo Li, Weili Liu, Guohong Cao, Pan Xv, Jinjin Zhang, Changjun Lv, Xiaodong Song
Several recent studies have indicated that miR-30a plays critical roles in various biological processes and diseases. However, the mechanism of miR-30a participation in idiopathic pulmonary fibrosis (IPF) regulation is ambiguous. Our previous study demonstrated that miR-30a may function as a novel therapeutic target for lung fibrosis by blocking mitochondrial fission, which is dependent on dynamin-related protein1 (Drp-1). However, the regulatory mechanism between miR-30a and Drp-1 is yet to be investigated...
March 15, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28292882/cthrc1-lowers-pulmonary-collagen-associated-with-bleomycin-induced-fibrosis-and-protects-lung-function
#11
Andrew P Binks, Megyn Beyer, Ryan Miller, Renee J LeClair
Idiopathic pulmonary fibrosis (IPF) involves collagen deposition that results in a progressive decline in lung function. This process involves activation of Smad2/3 by transforming growth factor (TGF)-β and Wnt signaling pathways. Collagen Triple Helix Repeat-Containing-1 (Cthrc1) protein inhibits Smad2/3 activation. To test the hypothesis that Cthrc1 limits collagen deposition and the decline of lung function, Cthrc1 knockout (Cthrc1(-/-)) and wild-type mice (WT) received intratracheal injections of 2.5 U/kg bleomycin or saline...
March 2017: Physiological Reports
https://www.readbyqxmd.com/read/28288384/bulk-production-and-evaluation-of-high-specific-activity-186g-re-for-cancer-therapy-using-enriched-186-wo3-targets-in-a-proton-beam
#12
Tara Mastren, Valery Radchenko, Hong T Bach, Ethan R Balkin, Eva R Birnbaum, Mark Brugh, Jonathan W Engle, Matthew D Gott, James Guthrie, Heather M Hennkens, Kevin D John, Alan R Ketring, Marina Kuchuk, Joel R Maassen, Cleo M Naranjo, F Meiring Nortier, Tim E Phelps, Silvia S Jurisson, D Scott Wilbur, Michael E Fassbender
INTRODUCTION: Rhenium-186g (t1/2 = 3.72 d) is a β(-) emitting isotope suitable for theranostic applications. Current production methods rely on reactor production by way of the reaction (185)Re(n,γ)(186g)Re, which results in low specific activities limiting its use for cancer therapy. Production via charged particle activation of enriched (186)W results in a (186g)Re product with a higher specific activity, allowing it to be used more broadly for targeted radiotherapy applications. This targets the unmet clinical need for more efficient radiotherapeutics...
March 3, 2017: Nuclear Medicine and Biology
https://www.readbyqxmd.com/read/28287347/predicting-life-expectancy-for-pirfenidone-in-idiopathic-pulmonary-fibrosis
#13
Mark Fisher, Steven D Nathan, Christian Hill, Jade Marshall, Fred Dejonckheere, Per-Olof Thuresson, Toby M Maher
BACKGROUND: Conducting an adequately powered survival study in idiopathic pulmonary fibrosis (IPF) is challenging due to the rare nature of the disease and the need for extended follow-up. Consequently, registration trials of IPF treatments have not been designed to estimate long-term survival. OBJECTIVE: To predict life expectancy for patients with IPF receiving pirfenidone versus best supportive care (BSC) in a population that met the inclusion criteria of patients enrolled in the ASCEND and CAPACITY trials...
March 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28287346/systematic-review-and-network-meta-analysis-of-idiopathic-pulmonary-fibrosis-treatments
#14
Kelly Fleetwood, Rachael McCool, Julie Glanville, Susan C Edwards, Sandro Gsteiger, Monica Daigl, Mark Fisher
BACKGROUND: The antifibrotics pirfenidone and nintedanib are both approved for the treatment of idiopathic pulmonary fibrosis (IPF) by regulatory agencies and are recommended by health technology assessment bodies. Other treatments such as N-acetylcysteine are used in clinical practice but have not received regulatory approval. No head-to-head trials have been conducted to directly compare the efficacy of these therapies in IPF. OBJECTIVE: To compare the efficacy of treatments for IPF...
March 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28286407/pulmonary-hypertension-associated-with-idiopathic-pulmonary-fibrosis-current-and-future-perspectives
#15
REVIEW
Scott D Collum, Javier Amione-Guerra, Ana S Cruz-Solbes, Amara DiFrancesco, Adriana M Hernandez, Ankit Hanmandlu, Keith Youker, Ashrith Guha, Harry Karmouty-Quintana
Pulmonary hypertension (PH) is commonly present in patients with chronic lung diseases such as Chronic Obstructive Pulmonary Disease (COPD) or Idiopathic Pulmonary Fibrosis (IPF) where it is classified as Group III PH by the World Health Organization (WHO). PH has been identified to be present in as much as 40% of patients with COPD or IPF and it is considered as one of the principal predictors of mortality in patients with COPD or IPF. However, despite the prevalence and fatal consequences of PH in the setting of chronic lung diseases, there are limited therapies available for patients with Group III PH, with lung transplantation remaining as the most viable option...
2017: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/28283478/annexin-a2-contributes-to-lung-injury-and-fibrosis-by-augmenting-factor-xa-fibrogenic-activity
#16
Michael Schuliga, Jade Jaffar, Asres Berhan, Shenna Langenbach, Trudi Harris, David Waters, Peter Vee Sin Lee, Christopher Grainge, Glen Westall, Darryl Knight, Alastair G Stewart
In lung injury and disease, including idiopathic pulmonary fibrosis (IPF), extravascular factor X is converted into factor Xa (FXa), a coagulant protease with fibrogenic actions. Extracellular annexin A2 binds to FXa, augmenting activation of the protease activated receptor-1 (PAR-1). In this study, the contribution of annexin A2 in lung injury and fibrosis was investigated. Annexin A2 immunoreactivity was observed in regions of fibrosis, including associated with fibroblasts in lung tissue of IPF patients...
March 10, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28274539/the-clinical-impact-of-major-comorbidities-on-idiopathic-pulmonary-fibrosis
#17
REVIEW
Atsushi Suzuki, Yasuhiro Kondoh
Idiopathic pulmonary fibrosis (IPF) is a progressive and devastating disease with a median survival time of 2-3 years after diagnosis. Patients with IPF exhibit distinct patterns of disease progression, and some patients display a more rapidly progressive clinical course. The clinical course of IPF may also include periods of acute deterioration, which are termed acute exacerbations. Patients with IPF frequently experience various comorbidities, such as pulmonary infection, emphysema, pulmonary hypertension, lung cancer, gastroesophageal reflux, cardiovascular disease, diabetes mellitus, and obstructive sleep apnea...
March 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28274188/extracellular-matrix-remodeling-in-idiopathic-pulmonary-fibrosis-it-is-the-bed-that-counts-and-not-the-sleepers
#18
Ioannis P Tomos, Argyrios Tzouvelekis, Vassilis Aidinis, Effrosyni D Manali, Evangelos Bouros, Demosthenes Bouros, Spyros A Papiris
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease characterized by irreversible fibrosis. Current disease pathogenesis assumes an aberrant wound healing process in response to repetitive injurious stimuli leading to apoptosis of epithelial cells, activation of fibroblasts and accumulation of extracellular matrix (ECM). Particularly, lung ECM is a highly dynamic structure that lies at the core of several physiological and developmental pathways. The scope of this review article is to summarize current knowledge on the role of ECM in the pathogenesis of IPF, unravel novel mechanistic data and identify future more effective therapeutic targets...
March 8, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28273952/comparative-analysis-of-lysyl-oxidase-like-family-members-in-pulmonary-fibrosis
#19
Verena Aumiller, Benjamin Strobel, Merrit Romeike, Michael Schuler, Birgit E Stierstorfer, Sebastian Kreuz
Extracellular matrix (ECM) composition and stiffness are major driving forces for the development and persistence of fibrotic diseases. Lysyl oxidase (LOX) and LOX-like (LOXL) proteins play crucial roles in ECM remodeling due to their collagen crosslinking and intracellular functions. Here, we systematically investigated LOX/L expression in primary fibroblasts and epithelial cells under fibrotic conditions, Bleomycin (BLM) induced lung fibrosis and in human IPF tissue. Basal expression of all LOX/L family members was detected in epithelial cells and at higher levels in fibroblasts...
December 2017: Scientific Reports
https://www.readbyqxmd.com/read/28273432/p53-and-mir-34a-feedback-promotes-lung-epithelial-injury-and-pulmonary-fibrosis
#20
Shwetha K Shetty, Nivedita Tiwari, Amarnath S Marudamuthu, Bijesh Puthusseri, Yashodhar P Bhandary, Jian Fu, Jeffrey Levin, Steven Idell, Sreerama Shetty
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease. The pathogenesis of interstitial lung diseases, including its most common form, IPF, remains poorly understood. Alveolar epithelial cell (AEC) apoptosis, proliferation, and accumulation of myofibroblasts and extracellular matrix deposition results in progressive loss of lung function in IPF. We found induction of tumor suppressor protein, p53, and apoptosis with suppression of urokinase-type plasminogen activator (uPA) and the uPA receptor in AECs from the lungs of IPF patients, and in mice with bleomycin, cigarette smoke, silica, or sepsis-induced lung injury...
March 5, 2017: American Journal of Pathology
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