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https://www.readbyqxmd.com/read/28642621/variants-of-the-abca3-gene-might-contribute-to-susceptibility-to-interstitial-lung-diseases-in-the-chinese-population
#1
Wei Zhou, Yi Zhuang, Jiapeng Sun, Xiaofen Wang, Qingya Zhao, Lizhi Xu, Yaping Wang
ATP-binding cassette A3 (ABCA3) is a phospholipid carrier that is mainly expressed in the alveolar epithelium. Biallelic mutations of ABCA3 has been associated with fatal respiratory distress syndrome and interstitial lung disease (ILD) in children. However, whether variations in ABCA3 have a role in the development of adult ILD, including idiopathic pulmonary fibrosis (IPF), remains to be addressed. In this study, we screened for germline variants of ABCA3 by exons-sequencing in 30 patients with sporadic IPF and in 30 matched healthy controls...
June 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28642115/anti-tnf%C3%AE-therapy-in-inflammatory-lung-diseases-bn
#2
REVIEW
Rama Malaviya, Jeffrey D Laskin, Debra L Laskin
Increased levels of tumor necrosis factor (TNF) α have been linked to a number of pulmonary inflammatory diseases including asthma, chronic obstructive pulmonary disease (COPD), acute lung injury (ALI)/acute respiratory distress syndrome (ARDS), sarcoidosis, and interstitial pulmonary fibrosis (IPF). TNFα plays multiple roles in disease pathology by inducing an accumulation of inflammatory cells, stimulating the generation of inflammatory mediators, and causing oxidative and nitrosative stress, airway hyperresponsiveness and tissue remodeling...
June 19, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28641719/surfactant-protein-d-inhibits-interleukin-12p40-production-by-macrophages-through-the-sirp%C3%AE-rock-erk-signaling-pathway
#3
Rui Yamaguchi, Arisa Sakamoto, Takatoshi Yamamoto, Yasuji Ishimaru, Shinji Narahara, Hiroyuki Sugiuchi, Yasuo Yamaguchi
OBJECTIVE: Interleukin (IL)-12 has a pivotal profibrotic role in the development of idiopathic pulmonary fibrosis (IPF). Medical research trials based on IPF registry databases have actively recruited patients. Surfactant protein D (SP-D) is a useful biomarker in patients with IPF. SP-D binds to signal regulatory protein α (SIRPα), which acts as an inhibitory receptor, and this SP-D/SIRPα interaction may have an anti-inflammatory effect. Accordingly, the inhibitory effect of SP-D on IL-12p40 production by lipopolysaccharide (LPS)-stimulated macrophages was investigated...
June 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28635908/-idiopatic-pulmonary-fibrosis-a-new-paradigm
#4
S N Avdeev
Idiopathic pulmonary fibrosis ((IPF) is the most common disease from a group of interstitial lung diseases, which occurs mainly in middle-aged and elderly people. Over the past decade, there have been considerable changes in approaches to diagnosing and treating IPF. The paper presents an update on the epidemiology of IPF, the results of new studies of its pathogenesis, and main approaches to diagnosing the disease. In addition, there is new evidence on therapy for IPF.
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28629920/pulmonary-hypertension-in-parenchymal-lung-diseases-any-future-for-new-therapies
#5
REVIEW
Sergio Harari, Davide Elia, Marc Humbert
Pulmonary hypertension (PH) due to chronic lung diseases is associated with a poor prognosis, regardless of the underlying respiratory condition. Updated PH guidelines recommend optimal treatment of the underlying lung disease, including long-term oxygen therapy, in patients with chronic hypoxaemia despite the lack of randomized controlled clinical trials supporting this statement. So far, randomized controlled trials on drugs approved for pulmonary arterial hypertension (PAH) have yielded discouraging results in both interstitial lung diseases (ILD) and chronic obstructive pulmonary diseases (COPD) with PH...
June 16, 2017: Chest
https://www.readbyqxmd.com/read/28629448/azithromycin-for-idiopathic-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-retrospective-single-center-study
#6
Kodai Kawamura, Kazuya Ichikado, Yuko Yasuda, Keisuke Anan, Moritaka Suga
BACKGROUND: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is a fatal condition without an established pharmaceutical treatment. Most patients are treated with high-dose corticosteroids and broad-spectrum antibiotics. Azithromycin is a macrolide with immunomodulatory activity and may be beneficial for treatment of acute lung injury. The objective of this study was to determine the effect of azithromycin on survival of patients with idiopathic AE of IPF. METHODS: We evaluated 85 consecutive patients hospitalized in our department for idiopathic AE of IPF from April 2005 to August 2016...
June 19, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28629363/fibroblast-paracrine-tnf-%C3%AE-signaling-elevates-integrin-a5-expression-in-idiopathic-pulmonary-fibrosis-ipf
#7
Gali Epstein Shochet, Elizabetha Brook, Lilach Israeli-Shani, Evgeny Edelstein, David Shitrit
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis. Inflammatory cytokines play a significant role in IPF pathology. However, the fibroblast itself is also believed to be the primary effector in IPF. We hypothesized that the fibroblasts themselves secrete pro-inflammatory cytokines that could propagate IPF by affecting normal neighboring cells. Thus, we explored the effects of IPF fibroblast derived media on normal fibroblast characteristics...
June 19, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28628389/an-ounce-of-prevention%C3%A2-will-this-be-the-future-for-ipf
#8
Joyce S Lee
No abstract text is available yet for this article.
June 19, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28622199/antifibrotic-drugs-as-treatment-of-nonidiopathic-pulmonary-fibrosis-interstitial-pneumonias-the-time-is-now
#9
Michael Kreuter, Julia Wälscher, Jürgen Behr
PURPOSE OF REVIEW: Therapeutic advances in the management of idiopathic pulmonary fibrosis (IPF) has led to improved outcomes with the use of the antifibrotic agents pirfenidone and nintedanib, with a number of randomized studies demonstrating benefits in slowing disease progression in IPF. However, treatment of other fibrosing interstitial lung diseases (ILD) remains challenging. RECENT FINDINGS: Observational and uncontrolled studies investigating pirfenidone and nintedanib in non-IPF ILDs suggest potential benefits, although the data is weak...
June 15, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28619094/morphologic-and-molecular-study-of-lung-cancers-associated-with-idiopathic-pulmonary-fibrosis-and-other-pulmonary-fibroses
#10
Alice Guyard, Claire Danel, Nathalie Théou-Anton, Marie-Pierre Debray, Laure Gibault, Pierre Mordant, Yves Castier, Bruno Crestani, Gérard Zalcman, Hélène Blons, Aurélie Cazes
BACKGROUND: Primitive lung cancers developed on lung fibroses are both diagnostic and therapeutic challenges. Their incidence may increase with new more efficient lung fibrosis treatments. Our aim was to describe a cohort of lung cancers associated with idiopathic pulmonary fibrosis (IPF) and other lung fibrotic disorders (non-IPF), and to characterize their molecular alterations using immunohistochemistry and next-generation sequencing (NGS). METHODS: Thirty-one cancer samples were collected from 2001 to 2016 in two French reference centers for pulmonary fibrosis - 18 for IPF group and 13 for non-IPF group...
June 15, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28613983/azithromycin-attenuates-myofibroblast-differentiation-and-lung-fibrosis-development-through-proteasomal-degradation-of-nox4
#11
Kazuya Tsubouchi, Jun Araya, Shunsuke Minagawa, Hiromichi Hara, Akihiro Ichikawa, Nayuta Saito, Tsukasa Kadota, Nahoko Sato, Masahiro Yoshida, Yusuke Kurita, Kenji Kobayashi, Saburo Ito, Yu Fujita, Hirofumi Utsumi, Haruhiko Yanagisawa, Mitsuo Hashimoto, Hiroshi Wakui, Yutaka Yoshii, Takeo Ishikawa, Takanori Numata, Yumi Kaneko, Hisatoshi Asano, Makoto Yamashita, Makoto Odaka, Toshiaki Morikawa, Katsutoshi Nakayama, Yoichi Nakanishi, Kazuyoshi Kuwano
Accumulation of profibrotic myofibroblasts is involved in the process of fibrosis development during idiopathic pulmonary fibrosis (IPF) pathogenesis. TGFB (transforming growth factor beta) is one of the major profibrotic cytokines for myofibroblast differentiation and NOX4 (NADPH oxidase 4) has an essential role in TGFB-mediated cell signaling. Azithromycin (AZM), a second-generation antibacterial macrolide, has a pleiotropic effect on cellular processes including proteostasis. Hence, we hypothesized that AZM may regulate NOX4 levels by modulating proteostasis machineries, resulting in inhibition of TGFB-associated lung fibrosis development...
June 14, 2017: Autophagy
https://www.readbyqxmd.com/read/28611471/evaluation-of-the-immature-platelet-fraction-contribute-to-the-differential-diagnosis-of-hereditary-immune-and-other-acquired-thrombocytopenias
#12
F L B Ferreira, M P Colella, S S Medina, C Costa-Lima, M M L Fiusa, L N G Costa, F A Orsi, J M Annichino-Bizzacchi, K Y Fertrin, M F P Gilberti, M C Ozelo, E V De Paula
The differential diagnosis of immune (ITP) and hereditary macrothrombocytopenia (HM) is key to patient management. The immature platelet fraction (IPF) represents the subset of circulating platelets with higher RNA content, and has been shown to distinguish hypo- from hyperproliferative thrombocytopenias. Here we evaluated the diagnostic accuracy of IPF in the differential diagnosis between HM and other thrombocytopenias in a population of patients with post-chemotherapy thrombocytopenia (n = 56), bone marrow failure (n = 22), ITP (n = 105) and HM (n = 27)...
June 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28611390/protective-and-therapeutic-effect-of-felodipine-against-bleomycin-induced-pulmonary-fibrosis-in-mice
#13
Ken-Ichiro Tanaka, Tomomi Niino, Tomoaki Ishihara, Ayaka Takafuji, Takahiro Takayama, Yuki Kanda, Toshifumi Sugizaki, Fumiya Tamura, Shota Kurotsu, Masahiro Kawahara, Tohru Mizushima
Idiopathic pulmonary fibrosis (IPF) involves alveolar epithelial injury and abnormal collagen production caused by activated fibroblasts; transforming growth factor (TGF)-β1 is implicated in this activation. In this study, we screened for chemicals capable of inhibiting TGF-β1-induced collagen production in cultured fibroblasts from medicines already in clinical use. We selected felodipine based on its extent of collagen production inhibition, clinical safety profile, and other pharmacological activity. Felodipine is a dihydropyridine Ca(2+) channel blocker that has been used clinically to treat patients with high blood pressure...
June 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28604659/inference-and-quantification-of-peptidoforms-in-large-sample-cohorts-by-swath-ms
#14
George Rosenberger, Yansheng Liu, Hannes L Röst, Christina Ludwig, Alfonso Buil, Ariel Bensimon, Martin Soste, Tim D Spector, Emmanouil T Dermitzakis, Ben C Collins, Lars Malmström, Ruedi Aebersold
Consistent detection and quantification of protein post-translational modifications (PTMs) across sample cohorts is a prerequisite for functional analysis of biological processes. Data-independent acquisition (DIA) is a bottom-up mass spectrometry approach that provides complete information on precursor and fragment ions. However, owing to the convoluted structure of DIA data sets, confident, systematic identification and quantification of peptidoforms has remained challenging. Here, we present inference of peptidoforms (IPF), a fully automated algorithm that uses spectral libraries to query, validate and quantify peptidoforms in DIA data sets...
June 12, 2017: Nature Biotechnology
https://www.readbyqxmd.com/read/28602096/access-to-palliative-care-for-patients-undergoing-mechanical-ventilation-with-idiopathic-pulmonary-fibrosis-in-the-united-states
#15
Barret Rush, Landon Berger, Leo Anthony Celi
OBJECTIVE: The utilization of palliative care (PC) in patients with end-stage idiopathic pulmonary fibrosis (IPF) is not well understood. METHODS: The Nationwide Inpatient Sample (NIS) was utilized to examine the use of PC in mechanically ventilated (MV) patients with IPF. The NIS captures 20% of all US inpatient hospitalizations and is weighted to estimate 95% of all inpatient care. RESULTS: A total of 55 208 382 hospital admissions from the 2006 to 2012 NIS samples were examined...
January 1, 2017: American Journal of Hospice & Palliative Care
https://www.readbyqxmd.com/read/28598197/hypersensitivity-pneumonitis-perspectives-in-diagnosis-and-management
#16
Martina Vasakova, Ferran Morell, Simon Walsh, Kevin Leslie, Ganesh Raghu
Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease caused by recurrent exposure to one or more offending inducers in genetically susceptible individuals. It mimics other acute and chronic pulmonary diseases and is often misdiagnosed as idiopathic pulmonary fibrosis (IPF) or another idiopathic interstitial pneumonia if the history of exposure to the inducer is not elicited. We propose a new classification of HP based not only on clinical data but also radiologic and histopathologic findings which might have potential to serve as predictors of disease behaviour and therefore guide management...
June 9, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28596432/obstructive-sleep-apnoea-and-related-comorbidities-in-incident-idiopathic-pulmonary-fibrosis
#17
Thomas Gille, Morgane Didier, Marouane Boubaya, Loris Moya, Angela Sutton, Zohra Carton, Fanny Baran-Marszak, Danielle Sadoun-Danino, Dominique Israël-Biet, Vincent Cottin, Frederic Gagnadoux, Bruno Crestani, Marie-Pia d'Ortho, Pierre-Yves Brillet, Dominique Valeyre, Hilario Nunes, Carole Planès
The objectives of this prospective study were: 1) to determine the prevalence and determinants of obstructive sleep apnoea (OSA) in patients with newly diagnosed idiopathic pulmonary fibrosis (IPF); 2) to determine whether OSA was associated with cardiovascular disease (CVD) as well as increased oxidative stress and levels of IPF biomarkers in the blood.A group of 45 patients with newly diagnosed IPF attended polysomnography. The prevalence of CVD and the severity of coronary artery calcification were investigated by high-resolution computed tomography...
June 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28595790/trace-metals-in-fluids-lining-the-respiratory-system-of-patients-with-idiopathic-pulmonary-fibrosis-and-diffuse-lung-diseases
#18
REVIEW
Elena Bargagli, Federico Lavorini, Massimo Pistolesi, Elisabetta Rosi, Antje Prasse, Emilia Rota, Luca Voltolini
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with a poor prognosis and an undefined etiopathogenesis. Oxidative stress contributes to alveolar injury and fibrosis development and, because transition metals are essential to the functioning of most proteins involved in redox reactions, a better knowledge of metal concentrations and metabolism in the respiratory system of IPF patients may provide a valuable complementary approach to prevent and manage a disease which is often misdiagnosed or diagnosed in later stages...
July 2017: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/28591554/nlrp3-participates-in-the-regulation-of-emt-in-bleomycin-induced-pulmonary-fibrosis
#19
Rui Tian, Yong Zhu, Jiayi Yao, Xiaoxiao Meng, Jinfeng Wang, Hui Xie, Ruilan Wang
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and irreversible lung disease. Studies have shown that epithelial-mesenchymal transition (EMT) plays an important role in the development of IPF. The NLRP3 inflammasome is reported to be activated and play an important role in many respiratory diseases. However, whether the NLRP3 inflammasome is activated in alveolar epithelial cells as well as the regulatory role of NLRP3 in EMT have not been reported. In this study, we transfected NLRP3 siRNA into A549 and RLE-6TN cells and treated them with bleomycin (BLM) for 24h...
June 4, 2017: Experimental Cell Research
https://www.readbyqxmd.com/read/28591049/impact-of-serum-sp-a-and-sp-d-levels-on-comparison-and-prognosis-of-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#20
Kai Wang, Qing Ju, Jing Cao, Wenze Tang, Jian Zhang
BACKGROUND AND OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) has a poor prognosis in general; however, it is heterogeneous to detect relative biomarkers for predicting the disease progression. Serum biomarkers can be conveniently collected to detect and help to differentially diagnose IPF and predict IPF prognosis. This meta-analysis aimed to evaluate the use of serum surfactant proteins A and D (SP-A and SP-D) for differential diagnosis and prognosis of IPF. METHODS: Relevant articles were searched in PubMed, Embase, and Chinese National Knowledge Infrastructure databases and reviewed by 2 independent readers...
June 2017: Medicine (Baltimore)
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