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Pulmonary fibrosis idiopathic

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https://www.readbyqxmd.com/read/27933058/skewed-lung-ccr4-to-ccr6-cd4-t-cell-ratio-in-idiopathic-pulmonary-fibrosis-is-associated-with-pulmonary-function
#1
Ayodeji Adegunsoye, Cara L Hrusch, Catherine A Bonham, Mohammad R Jaffery, Kelly M Blaine, Meghan Sullivan, Matthew M Churpek, Mary E Strek, Imre Noth, Anne I Sperling
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal lung disease. While it has been suggested that T cells may contribute to IPF pathogenesis, these studies have focused primarily on T cells outside of the pulmonary interstitium. Thus, the role of T cells in the diseased lung tissue remains unclear. OBJECTIVE: To identify whether specific CD4(+) T cell subsets are differentially represented in lung tissue from patients with IPF. METHODS: CD4(+) T cell subsets were measured in lung tissue obtained from patients with IPF at the time of lung transplantation, and from age- and gender-matched organ donors with no known lung disease...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27932335/multicentre-randomised-placebo-controlled-trial-of-oral-anticoagulation-with-apixaban-in-systemic-sclerosis-related-pulmonary-arterial-hypertension-the-sphinx-study-protocol
#2
Alicia Calderone, Wendy Stevens, David Prior, Harshal Nandurkar, Eli Gabbay, Susanna M Proudman, Trevor Williams, David Celermajer, Joanne Sahhar, Peter K K Wong, Vivek Thakkar, Nathan Dwyer, Jeremy Wrobel, Weng Chin, Danny Liew, Margaret Staples, Rachelle Buchbinder, Mandana Nikpour
INTRODUCTION: Systemic sclerosis (SSc) is a severe and costly multiorgan autoimmune connective tissue disease characterised by vasculopathy and fibrosis. One of the major causes of SSc-related death is pulmonary arterial hypertension (PAH), which develops in 12-15% of patients with SSc and accounts for 30-40% of deaths. In situ thrombosis in the small calibre peripheral pulmonary vessels resulting from endothelial dysfunction and an imbalance of anticoagulant and prothrombotic mediators has been implicated in the complex pathophysiology of SSc-related PAH (SSc-PAH), with international clinical guidelines recommending the use of anticoagulants for some types of PAH, such as idiopathic PAH...
December 8, 2016: BMJ Open
https://www.readbyqxmd.com/read/27932290/the-diagnosis-of-idiopathic-pulmonary-fibrosis-current-and-future-approaches
#3
REVIEW
Fernando J Martinez, Alison Chisholm, Harold R Collard, Kevin R Flaherty, Jeffrey Myers, Ganesh Raghu, Simon L F Walsh, Eric S White, Luca Richeldi
With the recent development of two effective treatments for patients with idiopathic pulmonary fibrosis, an accurate diagnosis is crucial. The traditional approach to diagnosis emphasises the importance of thorough clinical and laboratory evaluations to exclude secondary causes of disease. High-resolution CT is a critical initial diagnostic test and acts as a tool to identify patients who should undergo surgical lung biopsy to secure a definitive histological diagnosis of usual interstitial pneumonia pattern...
December 5, 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27925428/measuring-sedentary-behaviours-in-patients-with-idiopathic-pulmonary-fibrosis-using-wrist-worn-accelerometers
#4
Chris Atkins, Mark Baxter, Andrew Jones, Andrew Wilson
Introduction - Idiopathic pulmonary fibrosis (IPF) patients suffer increasing functional limitation with disease worsening disease. Increasing time in sedentary behaviour has been associated with poorer quality of life. Determining thresholds for activity in patients with respiratory disease is difficult due to variable cardiorespiratory limitations between individuals. Measuring sedentary behaviour is not confounded by this limitation and may be a better measurement of activity in patients with respiratory disease...
December 7, 2016: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/27922260/acute-exacerbation-of-idiopathic-pulmonary-fibrosis-when-bronchoalveolar-lavage-becomes-a-lethal-weapon
#5
Gaetano Rea, Giuseppe Fiorentino, Antonio Corcione, Maurizia Lanza, Francesco Perna, Anna A Stanziola
No abstract text is available yet for this article.
December 6, 2016: Minerva Anestesiologica
https://www.readbyqxmd.com/read/27916096/-mir-21-promotes-pulmonary-fibrosis-in-rats-via-down-regulating-the-expression-of-adamts-1
#6
Lijing Liu, Huiming Yin, Minjiang Huang, Jianbin He, Gaozhong Yi, Zaiyan Wang, Hong Qian
Objective To observe the effect of miR-21 on bleomycin-induced pulmonary fibrosis in rats, and explore the related mechanism. Methods Peripheral blood was collected from idiopathic pulmonary fibrosis (IPF) patients (n=20) and healthy adults (n=20). Fluorescence quantitative real-time PCR was then used to measure miR-21 expression. Forty-five SD rats were randomly divided into control group, miR-21 agomir group and miR-21 antagomir group. Each group included 15 rats. After establishment of pulmonary fibrosis models by intratracheal administration with bleomycin A5, rats in control group, miR-21 agomir group and miR-21 antagomir group were injected at caudal vein with normal saline, miR21 agomir and miR21 antagomir, respectively...
December 2016: Xi Bao Yu Fen Zi Mian Yi Xue za Zhi, Chinese Journal of Cellular and Molecular Immunology
https://www.readbyqxmd.com/read/27916030/-short-term-outcomes-of-lung-transplant-recipients-using-organs-from-brain-death-donors
#7
W X He, C Jiang, X G Liu, W Huang, C Chen, L Jiang, B Yang, K Wu, Q K Chen, Y Yang, Y M Yu, G N Jiang
Objective: To assess short-term outcomes after lung transplantation with organs procured following brain death. Methods: Between April 2015 and July 2016, all 17 recipients after lung transplantation using organs from brain death donors (DBD) at Department of Thoracic Surgery, Shanghai Pulmonary Hospital, Tongji University School of Medicine were enrolled in this study. All patients were male, aging (60±7) years, including 11 chronic obstructive pulmonary disease, 5 idiopathic pulmonary fibrosis, 1 silicosis...
December 1, 2016: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/27910075/idiopathic-pulmonary-fibrosis-are-any-of-the-morphological-molecular-markers-useful-in-clinical-management
#8
REVIEW
Francesca Lunardi, Elisabetta Balestro, Nazarena Nannini, Stefania Edith Vuljan, Federico Rea, Fiorella Calabrese
Idiopathic pulmonary fibrosis (IPF), the most common form of chronic interstitial lung disease, is a severe progressive fibrotic disorder of unknown aetiology. The disease has a heterogeneous clinical course, with frequent poor prognosis, similar to malignant disease. Correctly diagnosing IPF has become particularly important in view of the availability of more precise therapeutic indications, thus avoiding steroid treatment and allowing new approaches with novel drugs. To date we have limited information about biomarkers predictive of progressive disease and associated complications...
December 2, 2016: Histology and Histopathology
https://www.readbyqxmd.com/read/27909724/hsp27-regulates-tgf-%C3%AE-mediated-lung-fibroblast-differentiation-through-the-smad3-and-erk-pathways
#9
Gang Wang, Hao Jiao, Jun-Nian Zheng, Xia Sun
Idiopathic pulmonary fibrosis (IPF) is a chronic lethal interstitial lung disease with unknown etiology. Recent studies have indicated that heat-shock protein 27 (HSP27) contributes to the pathogenesis of IPF through the regulation of epithelial-mesenchymal transition (EMT). However, the expression and role of HSP27 in fibroblasts during pulmonary fibrogenesis has not been investigated to date, at least to the best of our knowledge. In this study, we examined the expression of HSP27 in fibrotic lung tissue and fibroblasts from bleomycin (BLM)-challenged mice and human lung fibroblasts treated with transforming growth factor-β (TGF-β)...
November 28, 2016: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/27905849/pathways-to-precision-medicine-in-idiopathic-pulmonary-fibrosis-time-to-relax
#10
Nathan Hambly, Martin Kolb
No abstract text is available yet for this article.
December 1, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27905295/desensitization-of-idiopathic-pulmonary-fibrosis-fibroblasts-to-alternaria-alternata-extract-mediated-necrotic-cell-death
#11
Jintaek Im, Kyutae Kim, Ji Young Yhee, Scott M O'Grady, Richard S Nho
Alternaria alternata is an allergenic fungus and known to cause an upper respiratory tract infection and asthma in humans with compromised immunity. Although A. alternata's effect on airway epithelial cells has previously been examined, the potential role of A. alternata on lung fibroblast viability is not understood. Since lung fibroblasts derived from patients with idiopathic pulmonary fibrosis (IPF) display a distinct phenotype that is resistant to stress and cell death inducing conditions, the investigation of the role of Alternaria on pathological IPF fibroblasts provides a better understanding of the fibrotic process induced by an allergenic fungus...
November 2016: Physiological Reports
https://www.readbyqxmd.com/read/27899769/the-role-of-club-cell-phenoconversion-and-migration-in-idiopathic-pulmonary-fibrosis
#12
Jutaro Fukumoto, Ramani Soundararajan, Joseph Leung, Ruan Cox, Sanjay Mahendrasah, Neha Muthavarapu, Travis Herrin, Alexander Czachor, Lee C Tan, Nima Hosseinian, Priyanshi Patel, Jayanthraj Gone, Mason T Breitzig, Young Cho, Andrew J Cooke, Lakshmi Galam, Venkata Ramireddy Narala, Yashwant Pathak, Richard F Lockey, Narasaiah Kolliputi
Idiopathic pulmonary fibrosis (IPF) is an age-related multifactorial disease featuring non-uniform lung fibrosis. The decisive cellular events at early stages of IPF are poorly understood. While the involvement of club cells in IPF pathogenesis is unclear, their migration has been associated with lung fibrosis. In this study, we labeled club cells immunohistochemically in IPF lungs using a club cell marker Claudin-10 (Cldn10), a unique protein based on the recent report which demonstrated that the appearance of Cldn10 in developing and repairing lungs precedes other club cell markers including club cell secretory protein (CCSP)...
November 29, 2016: Aging
https://www.readbyqxmd.com/read/27896060/diffuse-bronchiectasis-as-the-primary-manifestation-of-endobronchial-sarcoidosis
#13
Paul D Hiles, Kenneth R Kemp, Jean M Coviello
Sarcoidosis is an idiopathic disease that most commonly involves the lungs and is characterized by granulomatous inflammation. Bronchiectasis is one pulmonary manifestation of sarcoidosis, although it is almost always observed as traction bronchiectasis in the setting of fibrotic lung disease. A 50-year-old woman was evaluated for chronic cough and bronchiectasis with a small amount of peripheral upper lobe honeycombing and no significant pulmonary fibrosis or lymphadenopathy. After an extensive laboratory and imaging evaluation did not identify a cause of her bronchiectasis, bronchoscopy was performed to assess for primary ciliary dyskinesia and revealed a diffuse cobblestone appearance of the airway mucosa...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27894300/pleural-inhibition-of-the-caspase-1-il-1%C3%AE-pathway-diminishes-profibrotic-lung-toxicity-of-bleomycin
#14
Olivier Burgy, Pierre-Simon Bellaye, Sebastien Causse, Guillaume Beltramo, Guillaume Wettstein, Pierre-Marie Boutanquoi, Françoise Goirand, Carmen Garrido, Philippe Bonniaud
BACKGROUND: Idiopathic and toxic pulmonary fibrosis are severe diseases starting classically in the subpleural area of the lung. It has recently been suggested that pleural mesothelial cells acquire a myofibroblast phenotype under fibrotic conditions induced by TGF-β1 or bleomycin. The importance and role of inflammation in fibrogenesis are still controversial. In this work, we explored the role of IL-1β/caspase-1 signaling in bleomycin lung toxicity and in pleural mesothelial cell transformation...
November 29, 2016: Respiratory Research
https://www.readbyqxmd.com/read/27890713/allogeneic-human-mesenchymal-stem-cells-in-patients-with-idiopathic-pulmonary-fibrosis-via-intravenous-delivery-aether-a-phase-i-safety-clinical-trial
#15
Marilyn K Glassberg, Julia Minkiewicz, Rebecca L Toonkel, Emmanuelle S Simonet, Gustavo A Rubio, Darcy Difede, Shirin Shafazand, Aisha Khan, Marietsy V Pujol, Vincent F LaRussa, Lisa H Lancaster, Glenn D Rosen, Joel Fishman, Yolanda N Mageto, Adam Mendizabal, Joshua M Hare
BACKGROUND: Despite recent FDA approval of two new drugs for idiopathic pulmonary fibrosis (IPF), curative therapies remain elusive and mortality remains high. Pre-clinical and clinical data support the safety of human mesenchymal stem cells as a potential novel therapy for this fatal condition. The AETHER trial was the first study designed to evaluate the safety of a single infusion of bone marrow-derived mesenchymal stem cells in patients with idiopathic pulmonary fibrosis. METHODS: Nine patients with mild to moderate IPF were sequentially assigned to one of three cohorts and dosed with a single intravenous infusion of 20, 100, or 200 x 10(6) human bone marrow-derived mesenchymal stem cells per infusion from young, unrelated, male donors...
November 24, 2016: Chest
https://www.readbyqxmd.com/read/27888985/azathioprine-response-in-patients-with-fibrotic-connective-tissue-disease-associated-interstitial-lung-disease
#16
Justin M Oldham, Cathryn Lee, Eleanor Valenzi, Leah J Witt, Ayodeji Adegunsoye, Scully Hsu, Lena Chen, Steven Montner, Jonathan H Chung, Imre Noth, Rekha Vij, Mary E Strek
BACKGROUND: Azathioprine is a commonly prescribed therapy for connective tissue disease-associated interstitial lung disease (CTD-ILD). Combination therapy that included azathioprine was recently shown to increase the risk of death and hospitalization in patients with idiopathic pulmonary fibrosis. Whether azathioprine increases the risk of adverse outcomes in patients with fibrotic CTD-ILD, including those with CTD-associated usual interstitial pneumonia (UIP), remains unknown. METHODS: A retrospective cohort analysis was performed to determine the combined incidence rate of death, transplant and respiratory hospitalization associated with azathioprine exposure...
December 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27886850/cellular-senescence-and-autophagy-in-the-pathogenesis-of-chronic-obstructive-pulmonary-disease-copd-and-idiopathic-pulmonary-fibrosis-ipf
#17
REVIEW
Kazuyoshi Kuwano, Jun Araya, Hiromichi Hara, Shunsuke Minagawa, Naoki Takasaka, Saburo Ito, Kenji Kobayashi, Katsutoshi Nakayama
Aging is associated with impairments in homeostasis. Although aging and senescence are not equivalent, the number of senescent cells increases with aging. Cellular senescence plays important roles in tissue repair or remodeling, as well as embryonic development. Autophagy is a process of lysosomal self-degradation that maintains a homeostatic balance between the synthesis, degradation, and recycling of cellular proteins. Autophagy diminishes with aging; additionally, accelerated aging can be attributed to reduced autophagy...
November 2016: Respiratory Investigation
https://www.readbyqxmd.com/read/27884593/idiopathic-interstitial-pneumonia-associated-pulmonary-hypertension-a-target-for-therapy
#18
Steven D Nathan, Jürgen Behr, Vincent Cottin, Harold R Collard, Marius M Hoeper, Fernando J Martinez, Athol U Wells
Advances in the treatment of idiopathic interstitial pneumonia (IIP) represent an urgent, unmet medical need for patients with this category of diffuse parenchymal lung disease. IIPs involve varying combinations of fibrosis and inflammation of unknown cause and may be associated with pulmonary hypertension (PH). When it occurs, PH is associated with higher oxygen needs, greater functional impairment, and increased mortality. However, whether or when PH is a maladaptive versus adaptive phenomenon remains to be determined...
November 5, 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27883184/epigenetics-in-reactive-and-reparative-cardiac-fibrogenesis-the-promise-of-epigenetic-therapy
#19
REVIEW
Asish K Ghosh, Rahul Rai, Panagiotis Flevaris, Douglas E Vaughan
Epigenetic changes play a pivotal role in the development of a wide spectrum of human diseases including cardiovascular diseases, cancer, diabetes, and intellectual disabilities. Cardiac fibrogenesis is a common pathophysiological process seen during chronic and stress-induced accelerated cardiac aging. While adequate production of extracellular matrix (ECM) proteins is necessary for post-injury wound healing, excessive synthesis and accumulation of extracellular matrix protein in the stressed or injured hearts causes decreased or loss of lusitropy that leads to cardiac failure...
November 24, 2016: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/27881157/plasma-micrornas-are-associated-with-acute-exacerbation-in-idiopathic-pulmonary-fibrosis
#20
Haiyan Min, Shanshan Fan, Shiyu Song, Yi Zhuang, Hui Li, Yongzheng Wu, Hourong Cai, Long Yi, Jinghong Dai, Qian Gao
BACKGROUND: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has high short-term mortality with unknown causes. To predict this malignant condition in clinics is challenging. In this study, we aim to demonstrate whether there are miRNAs that differ between AE-IPF and stable IPF, which may be served as reliable biomarker for AE-IPF prediction. METHODS: Human fibrotic-associated miRNAs arrays were designed to detect miRNAs expression in plasma of 3 AE-IPF patients, 3 Stable-IPF (S-IPF) patients and 3 normal controls (NC)...
November 23, 2016: Diagnostic Pathology
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