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idiopathic lung fibrosis

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https://www.readbyqxmd.com/read/28544857/new-treatment-directions-for-ipf-current-status-of-ongoing-and-upcoming-clinical-trials
#1
Francesco Macagno, Francesco Varone, Paolo Maria Leone, Pier-Valerio Mari, Loredana Panico, Ludovica Berardini, Luca Richeldi
The main objective of this review is to explore the wide and expanding field of new clinical trials in IPF. Recent trials have confirmed the efficacy of the approved drugs pirfenidone and nintedanib; nonetheless, the discovery of new biological pathways has opened new horizons in this field. Areas Covered: New strategies against matrix deposition are under study and so is for the role of immunity and autoimmunity. Recent advances in the use of stem cells are opening new possibilities for the recovery of damaged lung tissues...
May 25, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28544468/analysis-of-the-patients-with-simultaneous-bilateral-spontaneous-pneumothorax
#2
Tevfik Ilker Akcam, Onder Kavurmaci, Ayse Gul Ergonul, Sercan Aydin, Kutsal Turhan, Alpaslan Cakan, Ufuk Cagirici
BACKGROUND: Simultaneous bilateral spontaneous pneumothorax (SBSP) is an uncommon condition with limited data on its incidence in the literature. In this study, we aimed to describe the bilaterality in both primary and secondary spontaneous pneumothorax cases, and the clinical approach in simultaneous disease and prognosis of these patients. METHODS: A total of 16 patients who were followed with the diagnosis of bilateral spontaneous pneumothorax between January 2005 and January 2017 were retrospectively analyzed...
May 20, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28544079/cough-is-less-common-and-less-severe-in-systemic-sclerosis-associated-interstitial-lung-disease-compared-to-other-fibrotic-interstitial-lung-diseases
#3
Jasmine Z Cheng, Pearce G Wilcox, Ian Glaspole, Tamera J Corte, Darra Murphy, Cameron J Hague, Christopher J Ryerson
BACKGROUND AND OBJECTIVE: The objectives of this study were to determine the prevalence and characteristics of cough in idiopathic pulmonary fibrosis (IPF), chronic hypersensitivity pneumonitis (HP) and systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: Cough severity was measured in consecutive patients with IPF (n = 77), HP (n = 32) and SSc-ILD (n = 67) using a 10-cm visual analogue scale (VAS). Dyspnoea and quality of life were measured using established questionnaires...
May 23, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28543987/modified-gap-index-for-prediction-of-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-in-non-small-cell-lung-cancer
#4
Haruki Kobayashi, Shota Omori, Kazuhisa Nakashima, Kazushige Wakuda, Akira Ono, Hirotsugu Kenmotsu, Tateaki Naito, Haruyasu Murakami, Masahiro Endo, Toshiaki Takahashi
BACKGROUND AND OBJECTIVE: Predicting the incidence rate of acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) and its prognosis in patients with non-small cell lung cancer (NSCLC) and IPF is difficult. The aim was to study the incidence of IPF-AE during the clinical course of the disease and its prognosis in patients with both NSCLC and IPF. METHODS: In this retrospective study, we compared the incidence rate of AE during the clinical course of the disease as well as the 1-year survival rate and overall survival (OS) of patients with NSCLC and IPF using a modified gender, age and physiology (mGAP) staging system based on gender, age and percent predicted forced vital capacity...
May 23, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28540808/exposure-to-febrile-range-hyperthermia-potentiates-wnt-signalling-and-epithelial-mesenchymal-transition-gene-expression-in-lung-epithelium
#5
Ratnakar Potla, Mohan E Tulapurkar, Irina G Luzina, Sergei P Atamas, Ishwar S Singh, Jeffrey D Hasday
BACKGROUND: As environmental and body temperatures vary, lung epithelial cells experience temperatures significantly different from normal core temperature. Our previous studies in human lung epithelium showed that: (i) heat shock accelerates wound healing and activates profibrotic gene expression through heat shock factor-1 (HSF1); (ii) HSF1 is activated at febrile temperatures (38-41 °C) and (iii) hypothermia (32 °C) activates and hyperthermia (39.5 °C) reduces expression of a subset of miRNAs that target protein kinase-Cα (PKCα) and enhance proliferation...
April 26, 2017: International Journal of Hyperthermia
https://www.readbyqxmd.com/read/28537805/pathology-of-chronic-hypersensitivity-pneumonitis-what-is-it-what-are-the-diagnostic-criteria-why-do-we-care
#6
Andrew Churg, AnaMaria Bilawich, Joanne L Wright
CONTEXT: - Chronic hypersensitivity pneumonitis (CHP) has emerged from obscurity during the past 15 years and is now recognized as a very common form of fibrosing interstitial pneumonia but one that is frequently misdiagnosed both clinically and on surgical lung biopsy as usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) or fibrotic nonspecific interstitial pneumonia. OBJECTIVE: - To review the pathologic features of CHP. DATA SOURCES: - Clinical, pathology, and radiology literature were used...
May 24, 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28534660/radiation-induced-pulmonary-fibrosis-as-a-model-of-progressive-fibrosis-contributions-of-dna-damage-inflammatory-response-and-cellular-senescence-genes
#7
Tyler A Beach, Carl J Johnston, Angela M Groves, Jacqueline P Williams, Jacob N Finkelstein
Purpose/Aim of Study: Studies of pulmonary fibrosis (PF) have resulted in DNA damage, inflammatory response, and cellular senescence being widely hypothesized to play a role in the progression of the disease. Utilizing these aforementioned terms, genomics databases were interrogated along with the term, "pulmonary fibrosis," to identify genes common among all 4 search terms. Findings were compared to data derived from a model of radiation-induced progressive pulmonary fibrosis (RIPF) to verify that these genes are similarly expressed, supporting the use of radiation as a model for diseases involving PF, such as human idiopathic pulmonary fibrosis (IPF)...
May 23, 2017: Experimental Lung Research
https://www.readbyqxmd.com/read/28533489/sirtuin-6-inhibits-epithelial-to-mesenchymal-transition-during-idiopathic-pulmonary-fibrosis-via-inactivating-tgf-%C3%AE-1-smad3-signaling
#8
Kunming Tian, Panpan Chen, Zhiping Liu, Shutian Si, Qian Zhang, Yong Mou, Lianyong Han, Qin Wang, Xue Zhou
Sirt6 which is implicated in the control of aging, cancer, and metabolism, has been shown to have anti-fibrosis function in heart and liver. However, whether Sirt6 inhibits idiopathic pulmonary fibrosis remains elusive. Epithelial to mesenchymal transition has been found to be involved in the pathogenesis of idiopathic pulmonary fibrosis. In the present study, forced expression of Sirt6 significantly abrogated TGF-β1-induced epithelial to mesenchymal transition-like phenotype and cell behaviors in A549 cells...
May 9, 2017: Oncotarget
https://www.readbyqxmd.com/read/28532459/mechanical-ventilation-in-idiopathic-pulmonary-fibrosis-a-nationwide-analysis-of-ventilator-use-outcomes-and-resource-burden
#9
Joshua J Mooney, Karina Raimundo, Eunice Chang, Michael S Broder
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is associated with increased risk of respiratory-related hospitalizations. Studies suggest mechanical ventilation (MV) use in IPF does not improve outcomes and guidelines recommend against its general use. Our objective was to investigate MV use and association with cost and mortality in IPF. METHODS: This retrospective study, using a nationwide sample, included claims with IPF (ICD-9-CM: 516.3) in 2009-2011 and principal respiratory disease diagnosis (ICD-9-CM: 460-519); excluding lung transplant...
May 22, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28530639/calcium-binding-protein-s100a4-confers-mesenchymal-progenitor-cell-fibrogenicity-in-idiopathic-pulmonary-fibrosis
#10
Hong Xia, Adam Gilbertsen, Jeremy Herrera, Emilian Racila, Karen Smith, Mark Peterson, Timothy Griffin, Alexey Benyumov, Libang Yang, Peter B Bitterman, Craig A Henke
Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a prevalence of 1 million persons worldwide. The fibrosis spreads from affected alveoli into contiguous alveoli and leads to death by asphyxiation. We previously discovered that the IPF lung harbors fibrogenic mesenchymal progenitor cells (MPCs) that serve as a cell of origin for disease-mediating myofibroblasts. In a prior genomewide transcriptional analysis, we found that IPF MPCs displayed increased expression of S100 calcium-binding A4 (S100A4), a protein linked to cancer cell proliferation and invasiveness...
May 22, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28524034/-value-of-preoperative-pulmonary-artery-diastolic-pressure-on-predicting-primary-graft-dysfunction-after-bilateral-lung-transplantation-for-patients-with-idiopathic-pulmonary-fibrosis
#11
Feng Zhang, Hongyang Xu, Shuyun Jiang, Jiaqiong Li, Shunmei Lu, Dapeng Wang, Zhidong Zang, Hong Pan, Jingyu Chen
OBJECTIVE: To analyze the value of the potential risk factors on predicting primary graft dysfunction (PGD) after bilateral lung transplantation for the patients with idiopathic pulmonary fibrosis (IPF). METHODS: A retrospective study was conducted. Fifty-eight patients with IPF who underwent the bilateral lung transplantation admitted to Wuxi People's Hospital Affiliated to Nanjing Medical University from June 2014 to March 2017 were enrolled. The grade 3 PGD happened within 72 hours after transplantation was taken as the outcome event, and these patients were divided into PGD and non-PGD groups...
May 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/28521775/genome-wide-association-study-of-subclinical-interstitial-lung-disease-in-mesa
#12
Ani Manichaikul, Xin-Qun Wang, Li Sun, Josée Dupuis, Alain C Borczuk, Jennifer N Nguyen, Ganesh Raghu, Eric A Hoffman, Suna Onengut-Gumuscu, Emily A Farber, Joel D Kaufman, Dan Rabinowitz, Karen D Hinckley Stukovsky, Steven M Kawut, Gary M Hunninghake, George R Washko, George T O'Connor, Stephen S Rich, R Graham Barr, David J Lederer
BACKGROUND: We conducted a genome-wide association study (GWAS) of subclinical interstitial lung disease (ILD), defined as high attenuation areas (HAA) on CT, in the population-based Multi-Ethnic Study of Atherosclerosis Study. METHODS: We measured the percentage of high attenuation areas (HAA) in the lung fields on cardiac CT scan defined as voxels with CT attenuation values between -600 and -250 HU. Genetic analyses were performed in MESA combined across race/ethnic groups: non-Hispanic White (n = 2,434), African American (n = 2,470), Hispanic (n = 2,065) and Chinese (n = 702), as well as stratified by race/ethnicity...
May 18, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28515040/a-systematic-review-of-overlapping-microrna-patterns-in-systemic-sclerosis-and-idiopathic-pulmonary-fibrosis
#13
REVIEW
Gianluca Bagnato, William Neal Roberts, Jesse Roman, Sebastiano Gangemi
Lung fibrosis can be observed in systemic sclerosis and in idiopathic pulmonary fibrosis, two disorders where lung involvement carries a poor prognosis. Although much has been learned about the pathogenesis of these conditions, interventions capable of reversing or, at the very least, halting disease progression are not available. Recent studies point to the potential role of micro messenger RNAs (microRNAs) in cancer and tissue fibrogenesis. MicroRNAs are short non-coding RNA sequences (20-23 nucleotides) that are endogenous, evolutionarily conserved and encoded in the genome...
June 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28508572/clinical-characteristics-and-factors-associated-with-mortality-in-idiopathic-pulmonary-fibrosis-an-experience-from-a-tertiary-care-center-in-pakistan
#14
Ali Bin Sarwar Zubairi, Huzaifa Ahmad, Maryam Hassan, Sajjad Sarwar, Aamir Abbas, Talha Shahzad, Muhammad Irfan
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease (ILD) that predominantly affects older adults. IPF has the highest mortality burden of all ILDs. Data on mortality in patients with IPF is limited in developing countries. OBJECTIVES: To identify factors associated with mortality in patients with IPF at a tertiary care center in Pakistan. METHODS: A retrospective chart review was conducted at the Aga Khan University Hospital (AKUH) in Karachi, Pakistan from January 2005 to December 2015...
May 15, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28507166/matrix-stiffness-regulates-migration-of-human-lung-fibroblasts
#15
Shuichi Asano, Satoru Ito, Kota Takahashi, Kishio Furuya, Masashi Kondo, Masahiro Sokabe, Yoshinori Hasegawa
In patients with pulmonary diseases such as idiopathic pulmonary fibrosis and severe acute respiratory distress syndrome, progressive pulmonary fibrosis is caused by dysregulated wound healing via activation of fibroblasts after lung inflammation or severe damage. Migration of fibroblasts toward the fibrotic lesions plays an important role in pulmonary fibrosis. Fibrotic tissue in the lung is much stiffer than normal lung tissue. Emerging evidence supports the hypothesis that the stiffness of the matrix is not only a consequence of fibrosis, but also can induce fibroblast activation...
May 2017: Physiological Reports
https://www.readbyqxmd.com/read/28502419/the-performance-of-the-gap-model-in-patients-with-rheumatoid-arthritis-associated-interstitial-lung-disease
#16
Julie Morisset, Eric Vittinghoff, Bo Young Lee, Roberto Tonelli, Xiaowen Hu, Brett M Elicker, Jay H Ryu, Kirk D Jones, Stefania Cerri, Andreina Manfredi, Marco Sebastiani, Andrew J Gross, Brett Ley, Paul J Wolters, Talmadge E King, Dong Soon Kim, Harold R Collard, Joyce S Lee
BACKGROUND: Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is associated with significant morbidity and mortality. Similarities have been observed between patients with idiopathic pulmonary fibrosis (IPF) and the UIP (usual interstitial pneumonia) form of RA-ILD. The GAP (gender, age, physiology) model has been shown to predict mortality in patients with IPF, but its ability to predict mortality in RA-ILD is not known. METHODS: We identified 309 patients with RA-ILD at 4 academic centers with ongoing longitudinal cohorts of patients with ILD...
June 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28502413/understanding-the-determinants-of-health-related-quality-of-life-in-rheumatoid-arthritis-associated-interstitial-lung-disease
#17
Jake G Natalini, Jeff J Swigris, Julie Morisset, Brett M Elicker, Kirk D Jones, Aryeh Fischer, Harold R Collard, Joyce S Lee
RATIONALE: Health-related quality of life (HRQL) is impaired among patients with interstitial lung disease (ILD). Little is understood about HRQL in specific subtypes of ILD. OBJECTIVES: The aim of this study was to characterize and identify clinical determinants of HRQL among patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD) and compare them to patients with idiopathic pulmonary fibrosis (IPF). METHODS: We identified patients with a diagnosis of RA-ILD and IPF from an ongoing longitudinal cohort of ILD patients...
June 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28498407/respiratory-syncytial-virus-infection-accelerates-lung-fibrosis-through-the-unfolded-protein-response-in-a-bleomycin-induced-pulmonary-fibrosis-animal-model
#18
Lina Wang, Wei Cheng, Zhimin Zhang
Emerging evidence has demonstrated that endoplasmic reticulum stress (ER) is involved in the pathogenesis of idiopathic pulmonary fibrosis, however, the underlying mechanism remains unclear. Viral infection often triggers a hyperinflammatory response by an expansion of the ER. The present study was designed to observe the role of respiratory syncytial virus infection (RSV)‑induced ER stress on lung fibrosis. In order to determine the role of ER stress on the onset and progression of pulmonary fibrosis, mice received an intratracheal combined injection of RSV and bleomycin on day 0...
May 10, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28495619/the-respire-trials-two-phase-iii-randomized-multicentre-placebo-controlled-trials-of-ciprofloxacin-dry-powder-for-inhalation-ciprofloxacin-dpi-in-non-cystic-fibrosis-bronchiectasis
#19
Timothy Aksamit, Tiemo-Joerg Bandel, Margarita Criollo, Anthony De Soyza, J Stuart Elborn, Elisabeth Operschall, Eva Polverino, Katrin Roth, Kevin L Winthrop, Robert Wilson
The primary goals of long-term disease management in non-cystic fibrosis bronchiectasis (NCFB) are to reduce the number of exacerbations, and improve quality of life. However, currently no therapies are licensed for this. Ciprofloxacin Dry Powder for Inhalation (Ciprofloxacin DPI) has potential to be the first long-term intermittent therapy approved to reduce exacerbations in NCFB patients. The RESPIRE programme consists of two international phase III prospective, parallel-group, randomized, double-blinded, multicentre, placebo-controlled trials of the same design...
May 8, 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28495571/role-of-iron-in-the-pathogenesis-of-respiratory-disease
#20
REVIEW
Md Khadem Ali, Richard Y Kim, Rafia Karim, Jemma R Mayall, Kristy L Martin, Ali Shahandeh, Firouz Abbasian, Malcolm R Starkey, Veronique Loustaud-Ratti, Daniel Johnstone, Elizabeth A Milward, Philip M Hansbro, Jay C Horvat
Iron is essential for many biological processes, however, too much or too little iron can result in a wide variety of pathological consequences, depending on the organ system, tissue or cell type affected. In order to reduce pathogenesis, iron levels are tightly controlled in throughout the body by regulatory systems that control iron absorption, systemic transport and cellular uptake and storage. Altered iron levels and/or dysregulated homeostasis have been associated with several lung diseases, including chronic obstructive pulmonary disease, lung cancer, cystic fibrosis, idiopathic pulmonary fibrosis and asthma...
May 7, 2017: International Journal of Biochemistry & Cell Biology
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