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Pulmonary fibrosis

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https://www.readbyqxmd.com/read/29330966/static-hyperinflation-is-associated-with-ventilatory-limitation-and-exercise-tolerance-in-adult-cystic-fibrosis
#1
Daniel Stevens
INTRODUCTION: Lung hyperinflation is a potential mechanism limiting exercise tolerance. However, available data on the impact of static hyperinflation on exercise performance in adult cystic fibrosis is lacking. Furthermore, the relative contribution of both static and dynamic hyperinflation to exercise performance is unknown. OBJECTIVES: To determine the impact of static hyperinflation on exercise tolerance and lung dynamics in adult cystic fibrosis. METHODS: Clinical data of 107 adult patients with cystic fibrosis, including pulmonary function, lung volumes, and cardiopulmonary exercise from the Toronto Cystic Fibrosis database were collected and analyzed...
January 13, 2018: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/29330249/is-it-idiopathic-pulmonary-fibrosis-or-not
#2
REVIEW
Mary Salvatore, Genta Ishikawa, Maria Padilla
Pulmonary fibrosis is not uncommon. Usual interstitial pneumonitis (UIP)/idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic pulmonary fibrotic diseases and has the worst prognosis with a mean life expectancy of 3.8 years. The American Thoracic Society has provided guidelines for the accurate diagnosis of IPF.In 2014, 2 antifibrotic medications were approved in the United States that target the multiple fibrotic pathways of UIP, which increased the need for early and accurate diagnosis of IPF...
January 2018: Journal of the American Board of Family Medicine: JABFM
https://www.readbyqxmd.com/read/29329637/transcriptomic-evidence-of-immune-activation-in-macroscopically-normal-appearing-and-scarred-lung-tissues-in-idiopathic-pulmonary-fibrosis
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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/29325675/mycophenolate-mofetil-as-a-therapeutic-agent-for-interstitial-lung-diseases-in-systemic-sclerosis
#9
REVIEW
Takahiro Ueda, Takuro Sakagami, Toshiaki Kikuchi, Toshinori Takada
Systemic sclerosis (SSc) is an intractable disease that causes fibrosis in all organs. Approximately 40% of patients with SSc have some degree of interstitial lung disease (ILD). One third of patients with SSc and ILD, approximately 15% of all patients, have pulmonary lesions, which slowly progress to respiratory failure resistant to corticosteroid and other treatments. A randomized controlled trial conducted in the United States indicated that one year of treatment with oral cyclophosphamide in patients with SSc-ILD had a significant but modest beneficial effect on lung function, dyspnea, thickening of the skin, and health-related quality of life...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325044/adaptation-of-staphylococcus-aureus-to-the-airways-of-cystic-fibrosis-patients-by-the-up-regulation-of-superoxide-dismutase-m-and-iron-scavenging-proteins
#10
Janina Treffon, Desiree Block, Martin Moche, Swantje Reiß, Stephan Fuchs, Susanne Engelmann, Dörte Becher, Lars Langhanki, Alexander Mellmann, Georg Peters, Barbara C Kahl
Staphylococcus aureus represents an important pathogen in cystic fibrosis (CF) caused by early and persistent colonization of airways of these patients for extended periods. Although the median predicted survival age of CF patients has increased during the last decade, pulmonary infections are still a challenge.In this study we investigated the adaptation of S. aureus, which was cultured from the airways of an individual CF patient, by comparing the proteome of the first and latest available isolate recovered 13 years apart...
January 6, 2018: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29323796/treatment-of-chronic-rhinosinusitis-with-dornase-alfa-in-patients-with-cystic-fibrosis-a-systematic-review
#11
Gopi B Shah, Linde De Keyzer, Joy A Russell, Ashleigh Halderman
BACKGROUND: A major component of sputum in cystic fibrosis (CF) patients is polymerized DNA, a byproduct of degraded neutrophils. Dornase alfa (dornase) selectively cleaves extracellular DNA and reduces the viscosity of sputum. It improves mucociliary clearance and pulmonary function. The benefit of dornase on CF-associated sinusitis is less clear. Therefore, the objective of this study was to systematically review the use of dornase on chronic rhinosinusitis (CRS) in CF patients. METHODS: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement was followed for this systematic review...
January 11, 2018: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/29323786/pulmonary-endoplasmic-reticulum-stress-scars-smoke-and-suffocation
#12
REVIEW
Jennifer A Dickens, Elke Malzer, Joseph E Chambers, Stefan J Marciniak
Protein misfolding within the endoplasmic reticulum (ER stress) can be a cause or consequence of pulmonary disease. Mutation of proteins restricted to the alveolar type II pneumocyte can lead to inherited forms of pulmonary fibrosis, but even sporadic cases of pulmonary fibrosis appear to be strongly associated with activation of the unfolded protein response (UPR) and/or the integrated stress response (ISR). Inhalation of smoke can impair protein folding and may be an important cause of pulmonary ER stress...
January 11, 2018: FEBS Journal
https://www.readbyqxmd.com/read/29323734/inhibitory-effect-of-circulating-fibrocytes-on-injury-repair-in-acute-lung-injury-acute-respiratory-distress-syndrome-mice-model
#13
Wenlin Tai, Jinyu Li, Boyang Zheng, Hanxin Wu, Jiawei Ding, Ling Gao, Zhaoxin Dong
The study was aimed to explore the functions of circulating fibrocytes (CFs) on injury repair in acute lung injury/acute respiratory distress syndrome (ALI/ARDS) mice model and its clinical value as a biomarker for ALI/ARDS. ALI/ARDS mice model was established by intratracheal instillation of lipopolysaccharide (LPS). Mononuclear cells were isolated from peripheral blood of ALI/ARDS model and flow cytometry was used to measure CFs defined as cells positive for CD45 and collagen-1. Histological changes of lung tissues were evaluated by H&E staining and Masson's trichrome staining...
January 11, 2018: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/29322707/knowledge-attitudes-beliefs-and-practices-of-physicians-regarding-idiopathic-pulmonary-fibrosis-and-the-impact-of-a-continuing-medical-education-program
#14
Sahajal Dhooria, Inderpaul Singh Sehgal, Ritesh Agarwal, Ashutosh Nath Aggarwal, Digambar Behera
Background: Significant deficiencies have been identified previously in the knowledge of physicians regarding the current diagnosis and management of idiopathic pulmonary fibrosis (IPF). Whether a continuing medical education (CME) program helps in overcoming these deficiencies has never been studied. Methods: This was a questionnaire-based study performed to assess the knowledge, attitudes, beliefs and practices of physicians regarding IPF before and after attending a CME program at a tertiary care teaching Institute in northern India...
November 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29321510/a-model-of-human-lung-fibrogenesis-for-the-assessment-of-anti-fibrotic-strategies-in-idiopathic-pulmonary-fibrosis
#15
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/29321495/tanshinone-iia%C3%A2-sulfonate-protects-against-cigarette-smoke-induced-copd-and-down-regulation-of-cftr-in-mice
#16
Defu Li, Jian Wang, Dejun Sun, Xuefang Gong, Hua Jiang, Jiaze Shu, Ziyi Wang, Zhen Long, Yiguan Chen, Zili Zhang, Liang Yuan, Ruijuan Guan, Xue Liang, Ziying Li, Hongwei Yao, Nanshan Zhong, Wenju Lu
Chronic obstructive pulmonary disease (COPD) is a chronic lung disease characterized by abnormal inflammation, persistent and progressive lung function decline. The anti-inflammatory actions of tanshinone IIA, which is the most important active component from Chinese herbal medicine Danshen, have been well studied. However, it remains unknown whether sodium tanshinone IIA sulfonate (STS) protects against the development of COPD. Here we found that STS inhalation (5 mg/kg, 30 min per session, twice a day) significantly attenuated lung function decline, airspace enlargement, mucus production, bronchial collagen deposition, inflammatory responses and oxidative stress caused by cigarette smoke (CS) and lipopolysaccharide (LPS) exposures in mice...
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
#17
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
https://www.readbyqxmd.com/read/29321018/management-and-attitudes-about-ipf-idiopathic-pulmonary-fibrosis-among-physicians-from-latin-america
#18
Iván Cherrez-Ojeda, Vincent Cottin, Juan Carlos Calderón, César Delgado, Erick Calero, Daniel Simanca-Racines, Silvia Quadrelli, Annia Cherrez
BACKGROUND: The aim of our study was to assess current practice patterns and attitudes towards diagnosis and management of idiopathic pulmonary fibrosis (IPF) patients in Latin America. METHODS: A Cross-sectional survey was developed and up to 455 physicians were enrolled. We used a rigorous method of validation using the translated version of the AIR Survey. RESULTS: Mean age was 47.5 years (SD 12.6) with 20.4 years (SD 12.3) of practice...
January 10, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29320941/progressive-delayed-respiratory-complications-of-sulfur-mustard-poisoning-in-43-iranian-veterans-three-decades-after-exposure
#19
E Darchini-Maragheh, M Balali-Mood, M Malaknezhad, S R Mousavi
The most common delayed complication of sulfur mustard (SM) poisoning has been observed in the respiratory tracts. It was thus aimed to investigate the delayed respiratory complications in SM-exposed patients around 25 years before the study. Forty-three veterans with more than 25% disability of due to SM poisoning were investigated. Clinical examinations as well as pulmonary function test (PFT) were performed. High-resolution computed tomography (HRCT) of the lungs was done as clinically indicated. Triad of chronic cough, dyspnea, and expectoration were the most common symptoms that were recorded in 88...
February 2018: Human & Experimental Toxicology
https://www.readbyqxmd.com/read/29320807/overlap-of-interstitial-pneumonia-with-autoimmune-features-with-undifferentiated-connective-tissue-disease-and-contribution-of-uip-to-mortality
#20
Bryan T Kelly, Teng Moua
BACKGROUND AND OBJECTIVE: Criteria for interstitial pneumonia with autoimmune features (IPAF) were recently established for research purposes in a joint statement from the European Respiratory Society (ERS) and American Thoracic Society (ATS). We reviewed the utility of these criteria in patients previously diagnosed as broadly defined undifferentiated connective tissue disease (UCTD) and noted overlapping IPAF findings. Additional review was given to IPAF patients with usual interstitial pneumonia (UIP) on histopathology or radiology in terms of survival and outcome...
January 10, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
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