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idiopathic lung fibrosis

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https://www.readbyqxmd.com/read/29785264/delay-and-inequalities-in-the-treatment-of-idiopathic-pulmonary-fibrosis-the-case-of-two-nordic-countries
#1
Ida Pesonen, Lisa Carlson, Nicola Murgia, Riitta Kaarteenaho, Carl Magnus Sköld, Marjukka Myllärniemi, Giovanni Ferrara
Background: Idiopathic pulmonary fibrosis (IPF) is characterized by progressive loss of lung function with high mortality within the first 5 years from diagnosis. In 2011-2014, two drugs, pirfenidone and nintedanib, have been approved worldwide for prevention of IPF progression. National IPF-registries have been established in both Finland and Sweden. Our study explored potential differences in the care of IPF in these two countries. Methods: Patients included consecutively in the Finnish and Swedish IPF-registries from January 1, 2014 through December 31, 2016 were included in the study...
2018: Multidisciplinary Respiratory Medicine
https://www.readbyqxmd.com/read/29782549/sphingosine-1-phosphate-receptor-2-facilitates-pulmonary-fibrosis-through-potentiating-il-13-pathway-in-macrophages
#2
Juanjuan Zhao, Yasuo Okamoto, Yuya Asano, Kazuhiro Ishimaru, Sho Aki, Kazuaki Yoshioka, Noriko Takuwa, Takashi Wada, Yutaka Inagaki, Chiaki Takahashi, Takumi Nishiuchi, Yoh Takuwa
Idiopathic pulmonary fibrosis is a devastating disease with poor prognosis. The pathogenic role of the lysophospholipid mediator sphingosine-1-phosphate and its receptor S1PR2 in lung fibrosis is unknown. We show here that genetic deletion of S1pr2 strikingly attenuated lung fibrosis induced by repeated injections of bleomycin in mice. We observed by using S1pr2LacZ/+ mice that S1PR2 was expressed in alveolar macrophages, vascular endothelial cells and alveolar epithelial cells in the lung and that S1PR2-expressing cells accumulated in the fibrotic legions...
2018: PloS One
https://www.readbyqxmd.com/read/29774214/the-expression-of-aqp1-is-modified-in-lung-of-patients-with-idiopathic-pulmonary-fibrosis-addressing-a-possible-new-target
#3
Ana Galán-Cobo, Elena Arellano-Orden, Rocío Sánchez Silva, José Luis López-Campos, César Gutiérrez Rivera, Lourdes Gómez Izquierdo, Nela Suárez-Luna, María Molina-Molina, José A Rodríguez Portal, Miriam Echevarría
Activation of the epithelial-mesenchymal transition process (EMT) by which alveolar cells in human lung tissue undergo differentiation giving rise to a mesenchymal phenotype (fibroblast/miofibroblasts) has been well recognized as a key element in the origin of idiopathic pulmonary fibrosis (IPF). Here we analyzed expression of AQP1 in lung biopsies of patients diagnosed with IPF, and compared it to biopsies derived from patients with diverse lung pneumonies, such as hypersensitivity pneumonitis, sarcoidosis or normal lungs...
2018: Frontiers in Molecular Biosciences
https://www.readbyqxmd.com/read/29773608/what-s-in-a-name-that-which-we-call-ipf-by-any-other-name-would-act-the-same
#4
Athol U Wells, Kevin K Brown, Kevin R Flaherty, Martin Kolb, Victor J Thannickal
Idiopathic pulmonary fibrosis (IPF) remains a truly idiopathic fibrotic disease, with a modest genetic predilection and candidate triggers but no overall explanation for the development of disease in non-familial cases. Agreement on terminology has contributed to major clinical and translational advances since the millennium. It is likely that the entity currently captured by the term "IPF" will be radically reclassified over the next decade, either through "splitting" (into IPF subgroups responding selectively to individual disease-modifying agents) or through "lumping" of IPF with other forms of progressive fibrotic lung disease (with shared pathogenetic mechanisms and IPF-like disease behaviour)...
May 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29772024/use-of-human-amniotic-epithelial-cells-in-mouse-models-of-bleomycin-induced-lung-fibrosis-a-systematic-review-and-meta-analysis
#5
Fang He, Aiting Zhou, Shuo Feng
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) urgently requires effective treatment. Bleomycin-induced lung injury models are characterized by initial inflammation and secondary fibrosis, consistent with the pathological features of IPF. Human amniotic epithelial cells (hAECs) exhibit good differentiation potential and paracrine activity and are thus ideal for cell-based clinical therapies. The therapeutic effects of hAECs on lung fibrosis are attributed to many factors. We performed a systematic review of preclinical studies investigating the treatment of pulmonary fibrosis with hAECs to provide suggestions for their clinical use...
2018: PloS One
https://www.readbyqxmd.com/read/29769446/ubiquitination-and-deubiquitination-emerge-as-players-in-idiopathic-pulmonary-fibrosis-pathogenesis-and-treatment
#6
REVIEW
Shuang Li, Jing Zhao, Dong Shang, Daniel J Kass, Yutong Zhao
Idiopathic pulmonary fibrosis (IPF) is a fatal fibrotic lung disease that is associated with aberrant activation of TGF-β, myofibroblast differentiation, and abnormal extracellular matrix (ECM) production. Proper regulation of protein stability is important for maintenance of intracellular protein homeostasis and signaling. Ubiquitin E3 ligases mediate protein ubiquitination, and deubiquitinating enzymes (DUBs) reverse the process. The role of ubiquitin E3 ligases and DUBs in the pathogenesis of IPF is relatively unexplored...
May 17, 2018: JCI Insight
https://www.readbyqxmd.com/read/29763900/c-prosp-b-a-possible-biomarker-for-pulmonary-diseases
#7
Nicolas Kahn, Anne-Kathrin Rossler, Katrin Hornemann, Thomas Muley, Ekkehard Grünig, Werner Schmidt, Felix J F Herth, Michael Kreuter
BACKGROUND: Detection of surfactant proteins A and D (SP-A and SP-D) in the serum of patients with pulmonary diseases is thought to reflect an injury of the alveolar epithelial barrier and as such serve as a biomarker for these diseases. However, the data for SP-B are limited. OBJECTIVES: The aim of this feasibility study was to assess whether immature SP-B pre-proteins might have value as a possible biomarker for pulmonary diseases. METHODS: In serum samples from patients with different chronic lung diseases (interstitial lung diseases [ILDs], chronic obstructive pulmonary disease, asthma, lung cancer, pulmonary hypertension, inflammation, patients on ventilator support; total n = 283), C-proSP-B was measured using an electrochemiluminescence immunoassay based on mouse monoclonal anti-C-proSP-B antibodies...
May 15, 2018: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29761229/idiopathic-pulmonary-fibrosis-among-young-patients-challenges-in-diagnosis-and-management
#8
Gabriela Leuschner, Fredrik Reiter, Florian Stocker, Alexander Crispin, Nikolaus Kneidinger, Tobias Veit, Friederike Klenner, Felix Ceelen, Gregor Zimmermann, Hanno Leuchte, Simone Reu, Julien Dinkel, Jürgen Behr, Claus Neurohr
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is considered a disease of older patients, being rare in patients ≤ 50 years. Still, IPF can occur in younger patients, but this particular patient group is not well characterised so far. The aim of this study was to compare the diagnostic certainty, clinical features, comorbidities and survival in young versus older IPF patients. METHODS: We reviewed our medical records from February 2011 until February 2015, to identify IPF patients, who were then classified as young (≤ 50 years) or older IPF (> 50 years)...
May 14, 2018: Lung
https://www.readbyqxmd.com/read/29758336/grand-rounds-hepatic-manifestations-of-telomere-biology-disorders
#9
REVIEW
Mrinal M Patnaik, Patrick S Kamath, Douglas A Simonetto
Clinical Case A 51 year old Caucasian male was referred for evaluation of variceal bleeding. Laboratory tests were remarkable for mild thrombocytopenia and moderate alkaline phosphatase elevation. Synthetic liver function was well preserved. Abdominal computed tomography scan revealed moderate splenomegaly, gastric varices, and normal hepatic contour. A transjugular liver biopsy was performed revealing findings of nodular regenerative hyperplasia (NRH) with no significant fibrosis or necroinflammatory activity...
May 11, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29755982/clinical-genetics-in-interstitial-lung-disease
#10
REVIEW
Chad A Newton, Philip L Molyneaux, Justin M Oldham
Interstitial lung disease (ILD) comprises a heterogeneous group of diffuse parenchymal lung processes with overlapping clinical, radiographic, and histopathologic features. Among the most common and deadly ILDs are idiopathic pulmonary fibrosis (IPF) and chronic hypersensitivity pneumonitis (CHP). As the name implies, the cause of IPF remains elusive, but a variety of genetic and infectious risk factors have been identified. CHP results from chronic inhalation of an organic antigen, usually of avian or mold origin, and may occur in patients with a genetic predisposition...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29754320/pathophysiology-and-genetics-of-bronchiectasis-unrelated-to-cystic-fibrosis
#11
REVIEW
Aleksandra Nikolic
Bronchiectasis is characterized by deregulated inflammatory response and recurrent bacterial infection resulting in progressive lung damage and an irreversible dilatation of bronchi and bronchioles. Generally accepted model of the development of bronchiectasis is the "vicious cycle hypothesis" that proposes compromising of the mucociliary clearance by an initial event, which leads to the infection of the respiratory tract followed by further impairment of mucociliary function, bacterial proliferation, and more inflammation...
May 12, 2018: Lung
https://www.readbyqxmd.com/read/29751748/underlying-and-immediate-causes-of-death-in-patients-with-idiopathic-pulmonary-fibrosis
#12
Miia Kärkkäinen, Hanna Nurmi, Hannu-Pekka Kettunen, Tuomas Selander, Minna Purokivi, Riitta Kaarteenaho
BACKGROUND: The most common cause of death of patients with idiopathic pulmonary fibrosis (IPF) has been reported to be the lung disease itself and mortality from IPF appears to be increasing. However, the causes of death in patients with IPF taking into account differences between genders and smoking histories as well as disease progression, have not been previously explored. METHODS: Retrospective data from hospital register and death certificates from national database of IPF patients treated in Kuopio University Hospital (KUH) from 2002 to 2012 were collected...
May 11, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29747640/fibrocytes-are-increased-in-lung-and-peripheral-blood-of-patients-with-idiopathic-pulmonary-fibrosis
#13
P Heukels, J A C van Hulst, M van Nimwegen, C E Boorsma, B N Melgert, L M van den Toorn, K A T Boomars, M S Wijsenbeek, H Hoogsteden, J H von der Thüsen, R W Hendriks, M Kool, B van den Blink
BACKGROUND: Fibrocytes are implicated in Idiopathic Pulmonary Fibrosis (IPF) pathogenesis and increased proportions in the circulation are associated with poor prognosis. Upon tissue injury, fibrocytes migrate to the affected organ. In IPF patients, circulating fibrocytes are increased especially during exacerbations, however fibrocytes in the lungs have not been examined. Therefore, we sought to evaluate if fibrocytes can be detected in IPF lungs and we compare percentages and phenotypic characteristics of lung fibrocytes with circulating fibrocytes in IPF...
May 10, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29747538/protective-effect-of-standardized-extract-of-myrtus-communis-l-myrtle-on-experimentally-bleomycin-induced-pulmonary-fibrosis-biochemical-and-histopathological-study
#14
Mitra Samareh Fekri, Ali Mandegary, Fariba Sharififar, Hamid Reza Poursalehi, Mohammad Hadi Nematollahi, Atefe Izadi, Mohammad Mehdipour, Amir Asadi, Masoomeh Samareh Fekri
CONTEXT: Myrtle (Myrtus communis L) has been used widely in traditional medicine for different respiratory disorders. Idiopathic pulmonary fibrosis (IPF) is an inflammatory disease characterized by progressive loss of lung function with poor prognosis. The pathogenesis of disease has not been completely elucidated, but probably persistent epithelial damages are involved. OBJECTIVE: Evaluation of biochemical and histopathological effect of preventive and therapeutic doses of myrtle against bleomycin (BLM)-induced pulmonary fibrosis (PF) in animal model...
May 11, 2018: Drug and Chemical Toxicology
https://www.readbyqxmd.com/read/29747274/-real-life-experience-with-pirfenidone-in-idiopathic-pulmonary-fibrosis
#15
Y J Yan, Y L Fan, S W Yu, Q Ye
Objective: To evaluate the efficacy and safety of pirfenidone (PFD) in idiopathic pulmonary fibrosis (IPF) in real-life world. Methods: 47 consecutive patients with IPF taking PFD for at least 12 months (PFD group) were included for analysis, with 47 patients with IPF who did not take PFD as controls. Data were collected from clinical charts to evaluate the lung function parameters and the adverse reactions of PFD. Results: In the PFD group, the percent predicted forced vital capacity (FVC%) and diffusing capacity of carbon monoxide (D(L)CO%) increased by (3...
May 12, 2018: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29731719/rhace2-therapy-modifies-bleomycin-induced-pulmonary-hypertension-via-rescue-of-vascular-remodeling
#16
Anandharajan Rathinasabapathy, Andrew J Bryant, Toshio Suzuki, Christy Moore, Sheila Shay, Santhi Gladson, James D West, Erica J Carrier
Background: Pulmonary hypertension (PH) is a progressive cardiovascular disease, characterized by endothelial and smooth muscle dysfunction and vascular remodeling, followed by right heart failure. Group III PH develops secondarily to chronic lung disease such as idiopathic pulmonary fibrosis (IPF), and both hastens and predicts mortality despite of all known pharmacological interventions. Thus, there is urgent need for development of newer treatment strategies. Objective: Angiotensin converting enzyme 2 (ACE2), a member of the renin angiotensin family, is therapeutically beneficial in animal models of pulmonary vascular diseases and is currently in human clinical trials for primary PH...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29730443/therapeutic-antibodies-a-new-era-in-the-treatment-of-respiratory-diseases
#17
REVIEW
T Sécher, L Guilleminault, K Reckamp, I Amanam, L Plantier, N Heuzé-Vourc'h
Respiratory diseases affect millions of people worldwide, and account for significant levels of disability and mortality. The treatment of lung cancer and asthma with therapeutic antibodies (Abs) is a breakthrough that opens up new paradigms for the management of respiratory diseases. Antibodies are becoming increasingly important in respiratory medicine; dozens of Abs have received marketing approval, and many more are currently in clinical development. Most of these Abs target asthma, lung cancer and respiratory infections, while very few target chronic obstructive pulmonary disease - one of the most common non-communicable causes of death - and idiopathic pulmonary fibrosis...
May 3, 2018: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29727583/protein-tyrosine-phosphatase-n13-ptpn13-promotes-myofibroblast-resistance-to-apoptosis-in-idiopathic-pulmonary-fibrosis
#18
Alison Bamberg, Elizabeth F Redente, Steve D Groshong, Rubin M Tuder, Carlyne D Cool, Rebecca C Keith, Benjamin L Edelman, Bart P Black, Gregory P Cosgrove, Murry W Wynes, Douglas Curran-Everett, Stijn De Langhe, Luis A Ortiz, Andrew Thorburn, David W H Riches
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a progressive, fibrotic interstitial lung disease characterized by (myo)fibroblast accumulation and collagen deposition. Resistance to Fas-induced apoptosis is thought to facilitate (myo)fibroblast persistence in fibrotic lung tissues, by poorly understood mechanisms. OBJECTIVES: We tested the hypothesis that protein tyrosine phosphatase PTPN13 is expressed by IPF lung (myo)fibroblasts, promotes their resistance to Fas-induced apoptosis and contributes to the development of pulmonary fibrosis...
May 4, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29726062/expanded-acceptance-of-acute-exacerbation-of-nonspecific-interstitial-pneumonia-including-7-additional-cases-with-detailed-clinical-pathologic-correlation
#19
Atsushi Miyamoto, Amita Sharma, Michiya Nishino, Mari Mino-Kenudson, Osamu Matsubara, Eugene Jerome Mark
Acute exacerbation is uncommonly diagnosed in patients with nonspecific interstitial pneumonia (NSIP) and its pathologic features have received relatively little attention compared to idiopathic pulmonary fibrosis. We retrospectively studied 14 consecutive cases of histopathologically proven NSIP by surgical lung biopsy. The diagnosis of acute exacerbation was confirmed clinically. We analyzed whether four reported pathologic features, including organizing pneumonia lesion, alveolar hemorrhage, many fibroblastic foci, and focal hyaline membranes were present and suggestive of acute exacerbation of NSIP or not...
May 3, 2018: Pathology International
https://www.readbyqxmd.com/read/29725493/intermittent-hypoxia-increases-the-severity-of-bleomycin-induced-lung-injury-in-mice
#20
Thomas Gille, Morgane Didier, Cécile Rotenberg, Eva Delbrel, Dominique Marchant, Angela Sutton, Nicolas Dard, Liasmine Haine, Nicolas Voituron, Jean-François Bernaudin, Dominique Valeyre, Hilario Nunes, Valérie Besnard, Emilie Boncoeur, Carole Planès
Background: Severe obstructive sleep apnea (OSA) with chronic intermittent hypoxia (IH) is common in idiopathic pulmonary fibrosis (IPF). Here, we evaluated the impact of IH on bleomycin- (BLM-) induced pulmonary fibrosis in mice. Methods: C57BL/6J mice received intratracheal BLM or saline and were exposed to IH (40 cycles/hour; FiO2 nadir: 6%; 8 hours/day) or intermittent air (IA). In the four experimental groups, we evaluated (i) survival; (ii) alveolar inflammation, pulmonary edema, lung oxidative stress, and antioxidant enzymes; (iii) lung cell apoptosis; and (iv) pulmonary fibrosis...
2018: Oxidative Medicine and Cellular Longevity
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