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idiopathic lung fibrosis

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https://www.readbyqxmd.com/read/28743305/prognosis-and-longitudinal-changes-of-physical-activity-in-idiopathic-pulmonary-fibrosis
#1
Thomas Bahmer, Anne-Marie Kirsten, Benjamin Waschki, Klaus F Rabe, Helgo Magnussen, Detlef Kirsten, Marco Gramm, Simone Hummler, Eva Brunnemer, Michael Kreuter, Henrik Watz
BACKGROUND: Physical activity (PA) is associated with disease severity in idiopathic pulmonary fibrosis (IPF), but longitudinal studies evaluating its prognostic value and changes over time are lacking. METHODS: We measured PA (steps per day, SPD) in a cohort of 46 IPF-patients (mean age, 67 years; mean FVC, 76.1%pred.) by accelerometry at baseline, recorded survival status during 3 years follow-up and repeated measurements in survivors. We compared the prognostic value of PA to established mortality predictors including lung function (FVC, DLCO) and 6-min walking-distance (6MWD)...
July 25, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28739343/inhibition-of-cell-apoptosis-and-amelioration-of-pulmonary-fibrosis-by-thrombomodulin
#2
Kentaro Fujiwara, Tetsu Kobayashi, Hajime Fujimoto, Hiroki Nakahara, Corina N D'Alessandro-Gabazza, Josephine A Hinneh, Yoshinori Takahashi, Taro Yasuma, Kota Nishihama, Masaaki Toda, Masahiro Kajiki, Yoshiyuki Takei, Osamu Taguchi, Esteban C Gabazza
Pulmonary fibrosis is the terminal stage of a group of idiopathic interstitial pneumonias, of which idiopathic pulmonary fibrosis is the most frequent and fatal form. Recent studies have shown that recombinant human thrombomodulin improves exacerbation and clinical outcome of idiopathic pulmonary fibrosis, but the mechanism remains unknown. This study evaluated the mechanistic pathways of the inhibitory activity of recombinant human thrombomodulin in pulmonary fibrosis. Transgenic mice overexpressing human transforming growth factor β1 that develop spontaneously pulmonary fibrosis and wild-type mice treated with bleomycin were used as model of lung fibrosis...
July 21, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28734935/outcome-of-lung-transplantation-in-idiopathic-pulmonary-fibrosis-with-previous-anti-fibrotic-therapy
#3
Gabriela Leuschner, Florian Stocker, Tobias Veit, Nikolaus Kneidinger, Hauke Winter, René Schramm, Thomas Weig, Sandhya Matthes, Felix Ceelen, Paola Arnold, Dieter Munker, Friederike Klenner, Rudolf Hatz, Marion Frankenberger, Jürgen Behr, Claus Neurohr
BACKGROUND: Anti-fibrotic drugs may interfere with wound-healing after major surgery, theoretically preventing sufficient bronchial anastomosis formation after lung transplantation (LTx). The aim of this study was to assess the impact of previous treatment with pirfenidone and nintedanib on outcomes after LTx in patients with idiopathic pulmonary fibrosis (IPF). METHODS: All patients with IPF undergoing LTx at the University of Munich between January 2012 and November 2016 were retrospectively screened for previous use of anti-fibrotics...
July 5, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28732835/idiopathic-pulmonary-fibrosis-clinical-behavior-and-aging-associated-comorbidities
#4
REVIEW
Ivette Buendía-Roldán, Mayra Mejía, Carmen Navarro, Moisés Selman
Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and usually lethal lung disease of unknown etiology. Once considered as a relatively homogeneous, slowly progressive disease, is now recognized that the clinical behavior shows substantial heterogeneity, including an accelerated variant, and the presence of acute exacerbations. In addition, since IPF largely affects individuals over 60 years of age, the patients are at increased risk of several comorbidities that in turn have a remarkable clinical impact on the disease and increases mortality rate...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28732832/current-approaches-to-the-management-of-idiopathic-pulmonary-fibrosis
#5
REVIEW
Ganesh Raghu, Luca Richeldi
Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal lung disease associated with dyspnoea, cough and impaired quality of life. Currently, the aims of patient care are to improve outcomes for patients by slowing the progression of the disease, extending life, and improving quality of life. A prompt, accurate diagnosis is important to enable patients to receive treatment early in the course of the disease and to be considered for lung transplantation. Two anti-fibrotic drugs, nintedanib and pirfenidone, have been shown to reduce decline in lung function in patients with IPF...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28731277/tnf-%C3%AE-induced-nf-%C3%AE%C2%BAb-activation-promotes-myofibroblast-differentiation-of-lr-mscs-and-exacerbates-bleomycin-induced-pulmonary-fibrosis
#6
Jiwei Hou, Tan Ma, Honghui Cao, Yabing Chen, Cong Wang, Xiang Chen, Zou Xiang, Xiaodong Han
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and irreversible lung disease of unknown cause. It has been reported that both lung resident mesenchymal stem cells (LR-MSCs) and tumor necrosis factor-α (TNF-α) play important roles in the development of pulmonary fibrosis. However, the underlying connections between LR-MSCs and TNF-α in the pathogenesis of pulmonary fibrosis are still elusive. In this study, we found that the pro-inflammatory cytokine TNF-α and the transcription factor nuclear factor kappa B (NF-κB) p65 subunit were both upregulated in bleomycin-induced fibrotic lung tissue...
July 21, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28728146/chronic-kidney-disease-predicts-survival-in-patients-with-idiopathic-pulmonary-fibrosis
#7
Kohei Ikezoe, Tomohiro Handa, Kiminobu Tanizawa, Hideki Yokoi, Takeshi Kubo, Kensaku Aihara, Akihiko Sokai, Yoshinari Nakatsuka, Seishu Hashimoto, Kiyoshi Uemasu, Susumu Sato, Shigeo Muro, Sonoko Nagai, Motoko Yanagita, Kazuo Chin, Toyohiro Hirai, Yoshio Taguchi, Michiaki Mishima
BACKGROUND: The prevalence of chronic kidney disease (CKD) increases with age as with idiopathic pulmonary fibrosis (IPF). OBJECTIVES: We assessed the prevalence of CKD (stages 3-5) and investigated the relationship of CKD to clinical features and outcomes in patients with IPF. METHODS: This study comprised 123 patients with IPF; 61 subjects with chronic obstructive pulmonary disease (COPD), which was reportedly associated with CKD, were also enrolled as a disease control...
July 21, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28726637/microrna-101-attenuates-pulmonary-fibrosis-by-inhibiting-fibroblast-proliferation-and-activation
#8
Chaoqun Huang, Xiao Xiao, Ye Yang, Amarjit Mishra, Yurong Liang, Xiangming Zeng, Xiaoyun Yang, Dao Xu, Michael R Blackburn, Craig A Henke, Lin Liu
Aberrant proliferation and activation of lung fibroblasts contribute to the initiation and progression of idiopathic pulmonary fibrosis (IPF). However, the mechanisms responsible for the proliferation and activation of fibroblasts are not fully understood. The objective of the current study was to investigate the role of miR-101 in the proliferation and activation of lung fibroblasts. miR-101 expression was determined in lung tissues from patients with IPF and mice with bleomycin-induced pulmonary fibrosis...
July 18, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28722352/serpine-1-induces-alveolar-type-ii-cell-senescence-through-activating-p53-p21-rb-pathway-in-fibrotic-lung-disease
#9
Chunsun Jiang, Gang Liu, Tracy Luckhardt, Veena Antony, Yong Zhou, A Brent Carter, Victor J Thannickal, Rui-Ming Liu
Senescence of alveolar type 2 (ATII) cells, progenitors of the alveolar epithelium, is implicated in the pathogeneses of idiopathic pulmonary fibrosis (IPF), an aging-related progressive fatal lung disorder with unknown etiology. The mechanism underlying ATII cell senescence in fibrotic lung diseases, however, remains poorly understood. In this study, we report that ATII cells in IPF lungs express higher levels of serpine 1, also known as plasminogen activator inhibitor 1 (PAI-1), and cell senescence markers p21 and p16, compared to ATII cells in control lungs...
July 19, 2017: Aging Cell
https://www.readbyqxmd.com/read/28718917/proliferation-of-elastic-fibers-in-idiopathic-pulmonary-fibrosis-a-whole-slide-image-analysis-and-comparison-with-pleuroparenchymal-fibroelastosis
#10
Yoshiaki Kinoshita, Kentaro Watanabe, Hiroshi Ishii, Hisako Kushima, Masaki Fujita, Kazuki Nabeshima
BACKGROUND: We occasionally encounter patients with idiopathic pulmonary fibrosis (IPF) who have similar imaging patterns to pleuroparenchymal fibroelastosis (PPFE) in the upper lung fields but are not diagnosed as PPFE clinically. The clinicopathological features and intrapulmonary distribution of elastic fibers and collagen fibers in these patients have not been fully elucidated. METHODS: We retrospectively reviewed the medical records of patients with a clinical diagnosis of IPF and selected the consecutive patients who received autopsy or pneumonectomy for lung transplantation...
July 18, 2017: Histopathology
https://www.readbyqxmd.com/read/28717640/lung-microbiome-and-host-immune-tone-in-subjects-with-idiopathic-pulmonary-fibrosis-treated-with-inhaled-interferon-%C3%AE
#11
Jing Wang, Melissa Lesko, Michelle H Badri, Bianca C Kapoor, Benjamin G Wu, Yonghua Li, Gerald C Smaldone, Richard Bonneau, Zachary D Kurtz, Rany Condos, Leopoldo N Segal
Therapies targeting inflammation reveal inconsistent results in idiopathic pulmonary fibrosis (IPF). Aerosolised interferon (IFN)-γ has been proposed as a novel therapy. Changes in the host airway microbiome are associated with the inflammatory milieu and may be associated with disease progression. Here, we evaluate whether treatment with aerosolised IFN-γ in IPF impacts either the lower airway microbiome or the host immune phenotype. Patients with IPF who enrolled in an aerosolised IFN-γ trial underwent bronchoscopy at baseline and after 6 months...
July 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28714804/cellular-proliferation-and-differentiation-induced-by-single-layer-molybdenum-disulfide-and-mediation-mechanisms-of-proteins-via-the-akt-mtor-p70s6k-signaling-pathway
#12
Wei Zou, Xingli Zhang, Mengyang Zhao, Qixing Zhou, Xiangang Hu
Single-layer molybdenum disulfide (SLMoS2) is a novel kind of 2D nanosheet that has attracted great attention regarding its use in biosensors, drug delivery, tissue engineering and therapy. However, our results demonstrated that SLMoS2 accelerated proliferation and promoted myogenic differentiation and epithelial-mesenchymal transition (EMT) in human embryonic lung fibroblasts (HELFs). The abnormal proliferation and differentiation of HELFs contribute to idiopathic pulmonary fibrosis. Specifically, SLMoS2 significantly stimulated the expression of myofibroblast- and mesenchymal-associated genes and proteins...
July 17, 2017: Nanotoxicology
https://www.readbyqxmd.com/read/28705305/clinical-characteristics-of-japanese-candidates-for-lung-transplant-for-interstitial-lung-disease-and-risk-factors-for-early-death-while-on-the-waiting-list
#13
Hisao Higo, Takeshi Kurosaki, Eiki Ichihara, Toshio Kubo, Kentaroh Miyoshi, Shinji Otani, Seiichiro Sugimoto, Masaomi Yamane, Nobuaki Miyahara, Katsuyuki Kiura, Shinichiro Miyoshi, Takahiro Oto
BACKGROUND: Lung transplants have produced very favorable outcomes for patients with interstitial lung disease (ILD) in Japan. However, because of the severe donor lung shortage, patients must wait approximately 2.5 years before they can undergo transplantation and many candidates die before allocation. We reveal the clinical characteristics of Japanese patients with ILD who are candidates for lung transplants and the risk factors for early death while on the waiting list. METHODS: We retrospectively reviewed the clinical data of patients registered in the Japan Organ Transplant Network from Okayama University Hospital who are candidates for cadaveric lung transplants for ILD between 1999 and 2015...
July 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28704913/treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-the-pulmonologist-s-point-of-view
#14
REVIEW
So-My Koo, Soo-Taek Uh
Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be a significant cause of mortality in patients with CVD-associated ILD (CTD-ILD). There is a lack of randomized, clinical trials assessing pharmacological agents for CTD-ILD, except in cases of ILD-associated systemic sclerosis (SSc). This may be due to the lack of CTD cases available, the difficulty of histological confirmation of ILD, and the various types of CTD and ILD...
July 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28701304/hif1a-up-regulates-the-adora2b-receptor-on-alternatively-activated-macrophages-and-contributes-to-pulmonary-fibrosis
#15
Kemly Philip, Tingting Weng Mills, Jonathan Davies, Ning-Yuan Chen, Harry Karmouty-Quintana, Fayong Luo, Jose G Molina, Javier Amione-Guerra, Neeraj Sinha, Ashrith Guha, Holger K Eltzschig, Michael R Blackburn
Idiopathic pulmonary fibrosis (IPF) is a deadly chronic lung disease. Extracellular accumulation of adenosine and subsequent activation of the ADORA2B receptor play important roles in regulating inflammation and fibrosis in IPF. Additionally, alternatively activated macrophages (AAMs) expressing ADORA2B have been implicated in mediating adenosine's effects in IPF. Although hypoxic conditions are present in IPF, hypoxia's role as a direct modulator of macrophage phenotype and identification of factors that regulate ADORA2B expression on AAMs in IPF is not well understood...
July 12, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28701252/idiopathic-pulmonary-fibrosis-a-case-discussion
#16
Meghan K Felton, Brian Bautista, Lee E Morrow, Mark Malesker
OBJECTIVE: The purpose of this report is to describe the case of a 68-year-old man who was treated for idiopathic pulmonary fibrosis (IPF), a chronic and fatal lung disease that is characterized by progressive deterioration of pulmonary function. He was initially prescribed pirfenidone and developed significant gastric distress. The treatment was transitioned to nintedanib. This article will provide the pharmacist with a therapeutic overview of IPF, as well as review the unique process involved with drug acquisition, dosing, patient education, and monitoring of pirfenidone and nintedanib...
July 1, 2017: Consultant Pharmacist: the Journal of the American Society of Consultant Pharmacists
https://www.readbyqxmd.com/read/28696800/effect-of-nintedanib-on-airway-inflammation-and-remodeling-in-a-murine-chronic-asthma-model
#17
Hwa Young Lee, Jung Hur, In Kyoung Kim, Ji Young Kang, Hyoung Kyu Yoon, Sook Young Lee, Soon Suk Kwon, Young Kyoon Kim, Chin Kook Rhee
INTRODUCTION: Nintedanib is a multi-tyrosine kinase receptor inhibitor recently approved for treatment of idiopathic pulmonary fibrosis. Although angiogenesis is a key process involved in airway structural changes in patients with bronchial asthma, the effect of nintedanib targeting the angiokinase pathway on airway inflammation and remodeling has not been evaluated. METHODS: We used a 3-month ovalbumin (OVA) challenge mouse model of airway remodeling. Nintedanib was orally administrated during the challenge period, and the effects were examined based on the percentage of airway inflammatory cells, airway hyper-reactivity (AHR), peribronchial goblet cell hyperplasia, total lung collagen and smooth muscle area...
July 11, 2017: Experimental Lung Research
https://www.readbyqxmd.com/read/28688632/ecmo-support-in-lung-transplantation-a-contemporary-analysis-of-hospital-charges-in-the-united-states
#18
J W Awori Hayanga, Norihisa Shigemura, Jonathan K Aboagye, Chris Ensor, Mary Amanda Dew, Heather K Hayanga, Stephen A Esper, Kyla Joubert, Matthew R Morrell, Jonathan D'Cunha
BACKGROUND: There is little in the literature pertaining to cost associated with the use of extracorporeal membrane oxygenation (ECMO) in lung transplantation. We sought to evaluate charges associated with the index hospitalization among recipients of a lung transplant who required ECMO to identify factors that increase hospital charges in these patients. METHODS: With the use of the Nationwide Inpatient Sample, we reviewed data pertaining to patients who received a lung transplant between 2000 and 2011 and stratified them into ECMO and non-ECMO groups based on use of ECMO...
July 6, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28688167/inhibition-of-hyaluronan-synthesis-attenuates-pulmonary-hypertension-associated-with-lung-fibrosis
#19
Scott D Collum, Ning-Yuan Chen, Adriana M Hernandez, Ankit Hanmandlu, Heather Sweeney, Tinne C J Mertens, Tingting Weng, Fayong Luo, Jose G Molina, Jonathan Davies, Ian P Horan, Nick W Morrell, Javier Amione-Guerra, Odeaa Al-Jabbari, Keith Youker, Wenchao Sun, Jayakumar Rajadas, Paul L Bollyky, Bindu H Akkanti, Soma Jyothula, Neeraj Sinha, Ashrith Guha, Harry Karmouty-Quintana
BACKGROUND AND PURPOSE: Group III Pulmonary hypertension (PH) is a highly lethal and widespread lung disorder that is a common complication in idiopathic pulmonary fibrosis (IPF) where it is considered to be the single most significant predictor of mortality. While increased levels of hyaluronan have been observed in IPF patients, hyaluronan-mediated vascular remodelling and the hyaluronan-mediated mechanisms promoting PH associated with IPF are not fully understood. EXPERIMENTAL APPROACH: Explanted lung tissue from patients with IPF with and without a diagnosis of PH was used to identify increased levels of hyaluronan...
July 8, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28687632/increased-levels-of-gab1-and-gab2-adaptor-proteins-skew-il-4-signaling-toward-m2-macrophage-driven-pulmonary-fibrosis-in-mice
#20
Xiaohong Guo, Tingting Li, Yun Xu, Xiayan Xu, Zhengyi Zhu, Yun Zhang, Jiaqi Xu, Kaihong Xu, Hongqiang Cheng, Xue Zhang, Yuehai Ke
M2-polarized macrophages, also known as alternatively activated macrophages, have long been associated with pulmonary fibrosis; however, the mechanism has not been fully defined. Gab1 and Gab2 proteins belong to the GAB family of adaptors and are integral components of the signal specificity in response to various extracellular stimuli. In this report, we found that levels of both Gab1 and Gab2 were elevated in M2-polarized macrophages isolated from bleomycin-induced fibrotic lungs. In vitro Gab1/2 deficiency in bone marrow-derived macrophages abrogated IL-4-mediated M2 polarization...
July 7, 2017: Journal of Biological Chemistry
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