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idiopathic lung fibrosis

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https://www.readbyqxmd.com/read/29351446/mechanisms-and-consequences-of-oxidative-stress-in-lung-disease-therapeutic-implications-for-an-aging-populace
#1
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/29348469/treprostinil-inhibits-proliferation-and-extracellular-matrix-deposition-by-fibroblasts-through-camp-activation
#2
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/29348182/trends-in-mortality-from-idiopathic-pulmonary-fibrosis-in-the-european-union-an-observational-study-of-the-who-mortality-database-from-2001-2013
#3
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
#4
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
#5
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
#6
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
#7
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/29335860/effectiveness-and-safety-of-chinese-medicine-for-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#8
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
#9
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
https://www.readbyqxmd.com/read/29333842/an-adolescent-with-idiopathic-pleuroparenchymal-fibroelastosis-case-report
#10
Emine Atag, Nilay Bas Ikizoglu, Yasemin Gokdemir, Ela Erdem Eralp, Gursu Kiyan, Dilek Yilmazbayhan, Bulent Karadag
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades. We present a 16 year old patient with a 2-year history of exertional dyspnea, nonproductive cough and weight loss...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29329637/transcriptomic-evidence-of-immune-activation-in-macroscopically-normal-appearing-and-scarred-lung-tissues-in-idiopathic-pulmonary-fibrosis
#11
Irina G Luzina, Mariah V Salcedo, Mónica L Rojas-Peña, Anne E Wyman, Jeffrey R Galvin, Ashutosh Sachdeva, Andrew Clerman, June Kim, Teri J Franks, Edward J Britt, Jeffrey D Hasday, Si M Pham, Allen P Burke, Nevins W Todd, Sergei P Atamas
Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease manifested by overtly scarred peripheral and basilar regions and more normal-appearing central lung areas. Lung tissues from macroscopically normal-appearing (IPFn) and scarred (IPFs) areas of explanted IPF lungs were analyzed by RNASeq and compared with healthy control (HC) lung tissues. There were profound transcriptomic changes in IPFn compared with HC tissues, which included elevated expression of numerous immune-, inflammation-, and extracellular matrix-related mRNAs, and these changes were similar to those observed with IPFs compared to HC...
January 3, 2018: Cellular Immunology
https://www.readbyqxmd.com/read/29327616/nintedanib-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#12
Francesco Varone, Giacomo Sgalla, Bruno Iovene, Teresa Bruni, Luca Richeldi
Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by the progressive loss of pulmonary function, ultimately leading to respiratory failure and death. Two novel compounds, nintedanib and pirfenidone, have shown efficacy in reducing the rate of decline of lung function in IPF patients. The multiple tyrosine kinase inhibitor nintedanib has extensively being studied as a potential angiogenesis inhibitor in clinical against various neoplastic disorders. Afterwards, this compound was successfully tested in IPF...
January 12, 2018: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/29325685/reduced-incidence-of-lung-cancer-in-patients-with-idiopathic-pulmonary-fibrosis-treated-with-pirfenidone
#13
Yukiko Miura, Takefumi Saito, Toru Tanaka, Hiroyuki Takoi, Yohei Yatagai, Minoru Inomata, Takahito Nei, Yoshinobu Saito, Akihiko Gemma, Arata Azuma
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a disease with a worse prognosis than some types of cancer. In patients with IPF, lung cancer is critical because of the associated high mortality rate from its progression and fatal complications from anticancer treatments. Therefore, preventing lung cancer in patients with IPF is primordial. Pirfenidone is an anti-fibrotic agent that reduces the decline in forced vital capacity. This study aimed to assess the effect of pirfenidone in the development of lung cancer in patients with IPF...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325684/respiratory-comorbidities-and-risk-of-mortality-in-hospitalized-patients-with-idiopathic-pulmonary-fibrosis
#14
Keishi Oda, Kazuhiro Yatera, Yoshihisa Fujino, Takashi Kido, Tetsuya Hanaka, Konomi Sennari, Kiyohide Fushimi, Shinya Matsuda, Hiroshi Mukae
BACKGROUND: Respiratory comorbidities are frequently associated with idiopathic pulmonary fibrosis (IPF). However, little is known about their prognostic impact in hospitalized patients with IPF. We examined the impact of respiratory comorbidities on the mortality rates of hospitalized patients with IPF using a Japanese nationwide database. METHODS: We identified 5665 hospitalized patients diagnosed with IPF between April 2010 and March 2013. The primary outcome was defined as the in-hospital mortality at 30 days after admission...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325683/physical-activity-in-daily-life-in-patients-with-idiopathic-pulmonary-fibrosis
#15
Osamu Nishiyama, Ryo Yamazaki, Hiroyuki Sano, Takashi Iwanaga, Yuji Higashimoto, Hiroaki Kume, Yuji Tohda
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is characterized by progressive impairment of lung function and degradation of daily activity; however, this degradation has not been adequately elucidated. The objective of this study was to measure the physical activity of patients with IPF to determine its relationships with physiological parameters and survival rate. METHODS: In total, 31 patients with IPF and 20 age-matched healthy participants were enrolled in this study...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325682/computer-based-quantitative-computed-tomography-image-analysis-in-idiopathic-pulmonary-fibrosis-a-mini-review
#16
REVIEW
Hirotsugu Ohkubo, Hiroaki Nakagawa, Akio Niimi
Idiopathic pulmonary fibrosis (IPF) is the most common type of progressive idiopathic interstitial pneumonia in adults. Many computer-based image analysis methods of chest computed tomography (CT) used in patients with IPF include the mean CT value of the whole lungs, density histogram analysis, density mask technique, and texture classification methods. Most of these methods offer good assessment of pulmonary functions, disease progression, and mortality. Each method has merits that can be used in clinical practice...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325681/is-hypothyroidism-in-idiopathic-pleuroparenchymal-fibroelastosis-a-novel-lung-thyroid-syndrome
#17
Nobuyasu Awano, Takehiro Izumo, Kensuke Fukuda, Mari Tone, Daisuke Yamada, Tamiko Takemura, Soichiro Ikushima, Toshio Kumasaka
BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare type of interstitial pneumonia characterized by fibroelastosis. Patients with IPPFE as well as idiopathic interstitial pneumonia often have autoimmune diseases, which sometimes coincide with hypothyroidism (HypoT). However, there have been no reports on the association between IPPFE and HypoT. The purpose of this study was to evaluate the correlation between IPPFE and HypoT. We also examined the pathological features of the thyroid glands from autopsied cases...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325680/clinico-radio-pathological-characteristics-of-unclassifiable-idiopathic-interstitial-pneumonias
#18
Yasuhiko Nakamura, Keishi Sugino, Masashi Kitani, Akira Hebisawa, Naobumi Tochigi, Sakae Homma
BACKGROUND: The purpose of this study was to clarify the clinico-radio-pathological characteristics and prognostic factors of unclassifiable-idiopathic interstitial pneumonias (U-IIPs) diagnosed by surgical lung biopsy. METHODS: Among 86 patients with interstitial pneumonia who underwent surgical lung biopsy from January 2005 to September 2013, 33 (38.4%; 16 male patients; mean age, 64.4 ± 8.8 years) were diagnosed with U-IIPs. They were subsequently categorized into rapidly progressive (n = 7), slowly progressive (n = 7), and stable (n = 19) groups based on the decrease of the percent predicted forced vital capacity or percent predicted diffusing capacity of the lung carbon monoxide and the occurrence of acute exacerbation...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29321510/a-model-of-human-lung-fibrogenesis-for-the-assessment-of-anti-fibrotic-strategies-in-idiopathic-pulmonary-fibrosis
#19
Katy M Roach, Amanda Sutcliffe, Laura Matthews, Gill Elliott, Chris Newby, Yassine Amrani, Peter Bradding
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with limited therapeutic options. KCa3.1 ion channels play a critical role in TGFβ1-dependent pro-fibrotic responses in human lung myofibroblasts. We aimed to develop a human lung parenchymal model of fibrogenesis and test the efficacy of the selective KCa3.1 blocker senicapoc. 2 mm3 pieces of human lung parenchyma were cultured for 7 days in DMEM ± TGFβ1 (10 ng/ml) and pro-fibrotic pathways examined by RT-PCR, immunohistochemistry and collagen secretion...
January 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29321022/serum-metabolic-profiling-identified-a-distinct-metabolic-signature-in-patients-with-idiopathic-pulmonary-fibrosis-a-potential-biomarker-role-for-lysopc
#20
Barbara Rindlisbacher, Cornelia Schmid, Thomas Geiser, Cédric Bovet, Manuela Funke-Chambour
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease of unknown etiology. Patients present loss of lung function, dyspnea and dry cough. Diagnosis requires compatible radiologic imaging and, in undetermined cases, invasive procedures such as bronchoscopy and surgical lung biopsy. The pathophysiological mechanisms of IPF are not completely understood. Lung injury with abnormal alveolar epithelial repair is thought to be a major cause for activation of profibrotic pathways in IPF...
January 10, 2018: Respiratory Research
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