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Idiopathic pulmonary disease

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https://www.readbyqxmd.com/read/28441449/effectiveness-and-safety-of-mycophenolate-mofetil-in-idiopathic-pulmonary-fibrosis
#1
Anoop M Nambiar, Antonio R Anzueto, Jay I Peters
BACKGROUND: Currently available antifibrotic treatments may slow down disease progression in idiopathic pulmonary fibrosis (IPF), but are associated with potentially significant side effects and are costly. Mycophenolate mofetil (MMF) is well known for its potent immunosuppressive properties and possesses important antiproliferative and antifibrotic effects. The safety and effectiveness of MMF in IPF is unknown. METHODS: We performed a retrospective multicohort analysis of IPF patients treated with MMF compared to those treated with either ineffective/harmful treatments or no treatment...
2017: PloS One
https://www.readbyqxmd.com/read/28440731/express-pulmonary-venous-remodeling-in-chronic-obstructive-pulmonary-disease-and-idiopathic-pulmonary-arterial-hypertension
#2
Kasper H Andersen, Claus Bøgelund Andersen, Finn Gustafsson, Joern Carlsen
No abstract text is available yet for this article.
January 1, 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28440556/direct-hemoperfusion-with-polymyxin-b-immobilized-fibre-treatment-for-acute-exacerbation-of-interstitial-pneumonia
#3
Haruhiko Furusawa, Makiko Sugiura, Chieko Mitaka, Naohiko Inase
BACKGROUND AND OBJECTIVE: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is recognized as an important cause of mortality. AE has also been reported in patients with other interstitial lung diseases such as idiopathic non-specific interstitial pneumonia (NSIP) and interstitial pneumonia associated with collagen vascular disease (CVD). Current therapies such as high-dose corticosteroid with immunosuppressive agents have provided little benefit for AE. Direct hemoperfusion (DHP) with a polymyxin B-immobilized fibre column (PMX) was originally developed for the treatment of endotoxaemia...
April 25, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28440314/the-peripheral-blood-proteome-signature-of-idiopathic-pulmonary-fibrosis-is-distinct-from-normal-and-is-associated-with-novel-immunological-processes
#4
David N O'Dwyer, Katy C Norman, Meng Xia, Yong Huang, Stephen J Gurczynski, Shanna L Ashley, Eric S White, Kevin R Flaherty, Fernando J Martinez, Susan Murray, Imre Noth, Kelly B Arnold, Bethany B Moore
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial pneumonia. The disease pathophysiology is poorly understood and the etiology remains unclear. Recent advances have generated new therapies and improved knowledge of the natural history of IPF. These gains have been brokered by advances in technology and improved insight into the role of various genes in mediating disease, but gene expression and protein levels do not always correlate. Thus, in this paper we apply a novel large scale high throughput aptamer approach to identify more than 1100 proteins in the peripheral blood of well-characterized IPF patients and normal volunteers...
April 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28435843/mmp-7-is-a-predictive-biomarker-of-disease-progression-in-patients-with-idiopathic-pulmonary-fibrosis
#5
Yasmina Bauer, Eric S White, Simon de Bernard, Peter Cornelisse, Isabelle Leconte, Adele Morganti, Sebastien Roux, Oliver Nayler
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with poor prognosis, which is characterised by destruction of normal lung architecture and excessive deposition of lung extracellular matrix. The heterogeneity of disease progression in patients with IPF poses significant obstacles to patient care and prevents efficient development of novel therapeutic interventions. Blood biomarkers, reflecting pathobiological processes in the lung, could provide objective evidence of the underlying disease...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28435277/role-of-pirfenidone-in-the-management-of-pulmonary-fibrosis
#6
REVIEW
Keith C Meyer, Catherine A Decker
Pulmonary fibrosis is associated with a number of specific forms of interstitial lung disease (ILD) and can lead to progressive decline in lung function, poor quality of life, and, ultimately, early death. Idiopathic pulmonary fibrosis (IPF), the most common fibrotic ILD, affects up to 1 in 200 elderly individuals and has a median survival that ranges from 3 to 5 years following initial diagnosis. IPF has not been shown to respond to immunomodulatory therapies, but recent trials with novel antifibrotic agents have demonstrated lessening of lung function decline over time...
2017: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/28430774/is-nintedanib-effective-for-idiopathic-pulmonary-fibrosis
#7
Alejandro Jeldres, Gonzalo Labarca
Idiopathic pulmonary fibrosis has poor prognosis and effective therapies are scarce. In the search for treatments that can modify the course of the disease, nintedanib (BIBF 1120), a tyrosine kinase inhibitor, has emerged as an alternative. However, its role is still unclear. To answer this question, we searched in Epistemonikos database, which is maintained by screening multiple sources of information. We identified seven systematic reviews including seven randomized trials overall. We extracted data, conducted a meta-analysis and generated a summary of findings table using the GRADE approach...
April 10, 2017: Medwave
https://www.readbyqxmd.com/read/28430622/serious-adverse-events-of-cell-therapy-for-respiratory-diseases-a-systematic-review-and-meta-analysis
#8
REVIEW
Runzhen Zhao, Zhenlei Su, Jing Wu, Hong-Long Ji
BACKGROUND: Cell therapy holds the most promising for acute and chronic deleterious respiratory diseases. However, the safety and tolerance for lung disorders are controversy. METHODS: We undertook a systematic review and meta-analyses of all 23 clinical studies of cell therapy. The outcomes were odds ratio (OR), risk difference (RD), Peto OR, relative risk, and mean difference of serious adverse events. RESULTS: 342 systemic infusions and 57 bronchial instillations (204 recipients) of cells were analyzed for acute respiratory distress syndrome (ARDS), bronchopulmonary dysplasia, pulmonary arterial hypertension, silicosis, sarcoidosis, extensively drug-resistant tuberculosis, chronic obstructive pulmonary diseases (COPD), and idiopathic pulmonary fibrosis...
February 16, 2017: Oncotarget
https://www.readbyqxmd.com/read/28427553/familial-pulmonary-fibrosis-clinical-and-radiological-characteristics-and-progression-analysis-in-different-high-resolution-ct-patterns
#9
David Bennett, Maria Antonietta Mazzei, Nevada Cioffi Squitieri, Elena Bargagli, Rosa Metella Refini, Antonella Fossi, Luca Volterrani, Paola Rottoli
BACKGROUND: Familial pulmonary fibrosis (FPF) is defined as an idiopathic diffuse parenchymal lung disease affecting two or more members of the same primary biological family. The aim of the present study was to contribute to the clinical, functional and radiological characterisation of FPF with particular regards to disease progression and survival. METHODS: Baseline clinical, functional and radiological data of a FPF population (n = 46 patients) were retrospectively collected and analysed according to the 2011 IPF guidelines HRCT classification...
May 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28425346/ambrisentan-a-review-of-its-use-in-pulmonary-arterial-hypertension
#10
Belinda N Rivera-Lebron, Michael G Risbano
Pulmonary arterial hypertension (PAH) is a progressive disease defined by an elevation in pulmonary arterial pressure that can lead to right heart failure and death. Ambrisentan is a selective endothelin receptor antagonist approved for the treatment of idiopathic, heritable PAH and connective tissue disease-associated PAH. Ambrisentan has been shown to improve exercise capacity and hemodynamics with an acceptable side-effect profile. It has also proven to be safely used in combination with other PAH-specific medications, especially with phosphodiesterase-5 inhibitors...
April 1, 2017: Therapeutic Advances in Respiratory Disease
https://www.readbyqxmd.com/read/28424250/unifying-mechanism-for-different-fibrotic-diseases
#11
Gerlinde Wernig, Shih-Yu Chen, Lu Cui, Camille Van Neste, Jonathan M Tsai, Neeraja Kambham, Hannes Vogel, Yaso Natkunam, D Gary Gilliland, Garry Nolan, Irving L Weissman
Fibrotic diseases are not well-understood. They represent a number of different diseases that are characterized by the development of severe organ fibrosis without any obvious cause, such as the devastating diseases idiopathic pulmonary fibrosis (IPF) and scleroderma. These diseases have a poor prognosis comparable with endstage cancer and are uncurable. Given the phenotypic differences, it was assumed that the different fibrotic diseases also have different pathomechanisms. Here, we demonstrate that many endstage fibrotic diseases, including IPF; scleroderma; myelofibrosis; kidney-, pancreas-, and heart-fibrosis; and nonalcoholic steatohepatosis converge in the activation of the AP1 transcription factor c-JUN in the pathologic fibroblasts...
April 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28422760/cannabinoid-cb1-receptor-overactivity-contributes-to-the-pathogenesis-of-idiopathic-pulmonary-fibrosis
#12
Resat Cinar, Bernadette R Gochuico, Malliga R Iyer, Tony Jourdan, Tadafumi Yokoyama, Joshua K Park, Nathan J Coffey, Hadass Pri-Chen, Gergő Szanda, Ziyi Liu, Ken Mackie, William A Gahl, George Kunos
Idiopathic pulmonary fibrosis (IPF) is a life-threatening disease without effective treatment, highlighting the need for identifying new targets and treatment modalities. The pathogenesis of IPF is complex, and engaging multiple targets simultaneously might improve therapeutic efficacy. To assess the role of the endocannabinoid/cannabinoid receptor 1 (endocannabinoid/CB1R) system in IPF and its interaction with inducible nitric oxide synthase (iNOS) as dual therapeutic targets, we analyzed lung fibrosis and the status of the endocannabinoid/CB1R system and iNOS in mice with bleomycin-induced pulmonary fibrosis (PF) and in lung tissue and bronchoalveolar lavage fluid (BALF) from patients with IPF, as well as controls...
April 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28416592/-wnt-er-is-coming-wnt-signalling-in-chronic-lung-diseases
#13
H A Baarsma, M Königshoff
Chronic lung diseases represent a major public health problem with only limited therapeutic options. An important unmet need is to identify compounds and drugs that target key molecular pathways involved in the pathogenesis of chronic lung diseases. Over the last decade, there has been extensive interest in investigating Wingless/integrase-1 (WNT) signalling pathways; and WNT signal alterations have been linked to pulmonary disease pathogenesis and progression. Here, we comprehensively review the cumulative evidence for WNT pathway alterations in chronic lung pathologies, including idiopathic pulmonary fibrosis, pulmonary arterial hypertension, asthma and COPD...
April 17, 2017: Thorax
https://www.readbyqxmd.com/read/28415591/the-purinergic-receptor-subtype-p2y2-mediates-chemotaxis-of-neutrophils-and-fibroblasts-in-fibrotic-lung-disease
#14
Tobias Müller, Susanne Fay, Rodolfo Paula Vieira, Harry Karmouty-Quintana, Sanja Cicko, Korcan Ayata, Gernot Zissel, Torsten Goldmann, Giuseppe Lungarella, Davide Ferrari, Francesco Di Virgilio, Bernard Robaye, Jean-Marie Boeynaems, Michael R Blackburn, Marco Idzko
Idiopathic pulmonary fibrosis (IPF) is a devastating disease with few available treatment options. Recently, the involvement of purinergic receptor subtypes in the pathogenesis of different lung diseases has been demonstrated. Here we investigated the role of the purinergic receptor subtype P2Y2 in the context of fibrotic lung diseases.The concentration of different nucleotides was measured in the broncho-alveolar lavage (BAL) fluid derived from IPF patients and animals with bleomycin-induced pulmonary fibrosis...
March 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28414242/discovery-of-2-2-ethyl-6-4-2-3-hydroxyazetidin-1-yl-2-oxo-ethyl-piperazin-1-yl-8-methyl-imidazo-1-2-a-pyridin-3-yl-methyl-amino-4-4-fluorophenyl-thiazole-5-carbonitrile-glpg1690-a-first-in-class-autotaxin-inhibitor-undergoing-clinical-evaluation-for-the-treatment
#15
Nicolas Desroy, Christopher Housseman, Xavier Bock, Agnès Joncour, Natacha Bienvenu, Laëtitia Cherel, Virginie Labeguere, Emilie Rondet, Christophe Peixoto, Jean-Marie Joël Grassot, Olivier Picolet, Denis Annoot, Nicolas Triballeau, Alain Monjardet, Emanuelle Wakselman, Veronique Roncoroni, Sandrine Le Tallec, Roland Blanque, Celine Cottereaux, Nele Vandervoort, Thierry Christophe, Patrick Mollat, Marieke B A C Lamers, Marielle Auberval, Boska Hrvacic, Jovica Ralic, Line Oste, Ellen Van der Aar, Reginald Brys, Bertrand Heckmann
Autotaxin is a circulating enzyme with a major role in the production of lysophosphatic acid (LPA) species in blood. A role for the autotaxin/LPA axis has been suggested in many disease areas including pulmonary fibrosis. Structural modifications of the known autotaxin inhibitor lead compound 1, to attenuate hERG inhibition, remove CYP3A4 time-dependent inhibition and improve pharmacokinetic properties, led to the identification of clinical candidate GLPG1690 (11). Compound 11 was able to cause a sustained reduction of LPA levels in plasma in vivo and was shown to be efficacious in a bleomycin-induced pulmonary fibrosis model in mice, and in reducing extra-cellular matrix deposition in the lung whilst also reducing LPA 18:2 content in bronchoalveolar lavage fluid...
April 17, 2017: Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28407046/rage-mediated-extracellular-matrix-proteins-accumulation-exacerbates-hysu-induced-pulmonary-hypertension
#16
Daile Jia, Yuhu He, Qian Zhu, Huan Liu, Caojian Zuo, Guilin Chen, Ying Yu, Ankang Lu
Aims: Extracellular matrix (ECM) proteins accumulation contributes to the progression of pulmonary arterial hypertension (PAH), a rare and fatal cardiovascular condition defined by high pulmonary arterial pressure, whether primary, idiopathic, or secondary to other causes. The receptor for advanced glycation end products (RAGE) is constitutively expressed in the lungs and plays an important role in ECM deposition. Nonetheless, the mechanisms by which RAGE mediates ECM deposition/formation in pulmonary arteries and its roles in PAH progression remain unclear...
April 11, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28404649/the-microbiome-in-respiratory-medicine-current-challenges-and-future-perspectives
#17
REVIEW
Rosa Faner, Oriol Sibila, Alvar Agustí, Eric Bernasconi, James D Chalmers, Gary B Huffnagle, Chaysavanh Manichanh, Philip L Molyneaux, Roger Paredes, Vicente Pérez Brocal, Julia Ponomarenko, Sanjay Sethi, Jordi Dorca, Eduard Monsó
The healthy lung has previously been considered to be a sterile organ because standard microbiological culture techniques consistently yield negative results. However, culture-independent techniques report that large numbers of microorganisms coexist in the lung. There are many unknown aspects in the field, but available reports show that the lower respiratory tract microbiota: 1) is similar in healthy subjects to the oropharyngeal microbiota and dominated by members of the Firmicutes, Bacteroidetes and Proteobacteria phyla; 2) shows changes in smokers and well-defined differences in chronic respiratory diseases, although the temporal and spatial kinetics of these changes are only partially known; and 3) shows relatively abundant non-cultivable bacteria in chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, cystic fibrosis and bronchiectasis, with specific patterns for each disease...
April 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28400116/the-role-of-infection-in-interstitial-lung-diseases-a-review
#18
REVIEW
Natalya Azadeh, Andrew H Limper, Eva M Carmona, Jay H Ryu
Interstitial lung diseases (ILD) comprise an array of heterogeneous parenchymal lung diseases that are associated with a spectrum of pathologic, radiologic and clinical manifestations. There are ILDs with known etiologies and those that are idiopathic, making treatment strategies challenging. Prognosis can vary according to the type of ILD but many exhibit gradual progression with an unpredictable clinical course in individual patients as seen in idiopathic pulmonary fibrosis and the phenomenon of "acute exacerbation"(AE)...
April 8, 2017: Chest
https://www.readbyqxmd.com/read/28399537/antacid-therapy-and-disease-progression-in-patients-with-idiopathic-pulmonary-fibrosis-who-received-pirfenidone
#19
Michael Kreuter, Paolo Spagnolo, Wim Wuyts, Elisabetta Renzoni, Dirk Koschel, Francesco Bonella, Toby M Maher, Martin Kolb, Derek Weycker, Klaus-Uwe Kirchgässler, Ulrich Costabel
BACKGROUND: Gastroesophageal reflux disease is a potential risk factor for idiopathic pulmonary fibrosis (IPF) progression; however, the impact of antacid therapy (AAT) is under debate. OBJECTIVE: To evaluate the effect of AAT on IPF progression in pirfenidone-treated patients. METHODS: This post hoc analysis included patients with IPF who received pirfenidone in 3 trials (CAPACITY [PIPF-004/PIPF-006] and ASCEND [PIPF-016]). Pulmonary function, exercise tolerance, survival, hospitalizations, and adverse events (AEs) over 52 weeks were analyzed by baseline AAT use...
April 12, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28395683/pulmonary-scar-carcinoma-in-south-africa
#20
N Jenkins, E M Irusen, C F N Koegelenberg
BACKGROUND: The association between lung scarring and the subsequent development of cancer remains controversial. South Africa has one of the highest incidences of tuberculosis in the world, and resultant scarring may predispose to malignancy. The country also carries a very high burden of smoking and smoking-related diseases that may be synergistic in malignant transformation. OBJECTIVE: To assess the frequency of pulmonary scarring in patients with lung cancer...
March 29, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
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