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https://www.readbyqxmd.com/read/28549169/regulatory-dynamics-of-11p13-suggest-a-role-for-ehf-in-modifying-cf-lung-disease-severity
#1
Lindsay R Stolzenburg, Rui Yang, Jenny L Kerschner, Sara Fossum, Matthew Xu, Andrew Hoffmann, Kay-Marie Lamar, Sujana Ghosh, Sarah Wachtel, Shih-Hsing Leir, Ann Harris
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF), but are not good predictors of lung phenotype. Genome-wide association studies (GWAS) previously identified additional genomic sites associated with CF lung disease severity. One of these, at chromosome 11p13, is an intergenic region between Ets homologous factor (EHF) and Apaf-1 interacting protein (APIP). Our goal was to determine the functional significance of this region, which being intergenic is probably regulatory...
May 26, 2017: Nucleic Acids Research
https://www.readbyqxmd.com/read/28549020/lung-density-and-pulmonary-artery-diameter-are-predictors-of-pulmonary-hypertension-in-systemic-sclerosis
#2
Margreet E Bakker, Maarten K Ninaber, Jan Stolk, Lucia J M Kroft, Anne A Schouffoer, Jeska K de Vries Bouwstra, Suzanne E van Wijngaarden, Berend C Stoel
PURPOSE: The aim was to evaluate computed tomography (CT)-measured pulmonary artery diameter (PAD) and lung density as predictors of pulmonary hypertension (PH) in subjects with systemic sclerosis (SSc). We compared these PAD values with normal values and between SSc subgroups with PH and/or interstitial lung disease (ILD). We investigated whether PAD predicts PH and whether lung densitometry, by using the 85th percentile density value (Perc85) as a measure for ILD, can predict PH. MATERIALS AND METHODS: PAD and Perc85 were measured in axial CT scans and compared between 54 SSc and 76 control subjects...
May 25, 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/28548990/outbreaks-of-nontuberculous-mycobacteria
#3
Geeta Sood, Nikki Parrish
PURPOSE OF REVIEW: The purpose of this review is to summarize the emerging literature on nontuberculous mycobacteria outbreaks in healthcare settings. As our ability to identify mycobacterial species develops, we are better able to recognize epidemiologic connections and better understand the prevalence and importance of these outbreaks and pseudo-outbreaks in healthcare settings. RECENT FINDINGS: The number of outbreaks related to nontuberculous outbreaks is increasing because of heightened awareness and better diagnostic tests for species level identification of mycobacteria...
May 25, 2017: Current Opinion in Infectious Diseases
https://www.readbyqxmd.com/read/28547678/immunosuppression-drug-therapy-in-lung-transplantation-for-cystic-fibrosis
#4
REVIEW
Pamela Burcham, Lisa Sarzynski, Sabrina Khalfoun, Kimberly J Novak, Julie C Miller, Dmitry Tumin, Don Hayes
Cystic fibrosis (CF) is a common indication for lung transplantation (LTx) in children and adults with severe and irreversible lung disease. In the setting of LTx in the CF population, immunosuppressive medications are used to prevent allograft rejection despite the majority of these patients being chronically infected with numerous, and often antibiotic-resistant, pathogens. There is limited evidence for the optimal post-LTx immunosuppression regimen in patients with CF, particularly in children. This article provides a review of immunosuppression regimens in the pediatric and adult CF post-LTx population, investigating drug dosing and monitoring, and medication combinations...
May 25, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28547381/purinergic-signalling-links-mechanical-breath-profile-and-alveolar-mechanics-with-the-pro-inflammatory-innate-immune-response-causing-ventilation-induced-lung-injury
#5
REVIEW
Djo Hasan, Paul Blankman, Gary F Nieman
Severe pulmonary infection or vigorous cyclic deformation of the alveolar epithelial type I (AT I) cells by mechanical ventilation leads to massive extracellular ATP release. High levels of extracellular ATP saturate the ATP hydrolysis enzymes CD39 and CD73 resulting in persistent high ATP levels despite the conversion to adenosine. Above a certain level, extracellular ATP molecules act as danger-associated molecular patterns (DAMPs) and activate the pro-inflammatory response of the innate immunity through purinergic receptors on the surface of the immune cells...
May 26, 2017: Purinergic Signalling
https://www.readbyqxmd.com/read/28545684/mri-of-cystic-fibrosis-lung-manifestations-sequence-evaluation-and-clinical-outcome-analysis
#6
O Scholz, T Denecke, J Böttcher, C Schwarz, H-J Mentzel, F Streitparth, M H Maurer, A Pfeil, A Huppertz, A Mehl, D Staab, B Hamm, D M Renz
AIM: To evaluate different magnetic resonance imaging (MRI) sequences for diagnosis of pulmonary manifestations of cystic fibrosis (CF) in comparison to chest computed tomography (CT), including an extended outcome analysis. MATERIALS AND METHODS: Twenty-eight patients with CF (15 male, 13 female, mean age 30.5±9.4 years) underwent CT and MRI of the lung. MRI (1.5 T) included different T2- and T1-weighted sequences: breath-hold HASTE (half Fourier acquisition single shot turbo spin echo) and VIBE (volumetric interpolated breath-hold examination, before and after contrast medium administration) sequences and respiratory-triggered PROPELLER (periodically rotated overlapping parallel lines with enhanced reconstruction) sequences with and without fat signal suppression, and perfusion imaging...
May 22, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28545478/ltb4-and-montelukast-in-transplantation-related-bronchiolitis-obliterans-in-rats
#7
Zheng-Liang Tu, Zhen-Yu Zhou, Hai-Chao Xu, Jin-Lin Cao, Peng Ye, Lu-Ming Wang, Wang Lv, Jian Hu
BACKGROUND: Lung transplantation is the only effective treatment for end-stage lung diseases. Bronchiolitis obliterans, which is known as non-infectious chronic lung allograft dysfunction (CLAD) in the new classification, is the greatest threat to long-term survival after lung transplantation. This study investigated the role of leukotriene B4 (LTB4) and montelukast in transplantation-related bronchiolitis obliterans and discussed the pathophysiological significance of LTB4 in chronic rejection...
May 25, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28544857/new-treatment-directions-for-ipf-current-status-of-ongoing-and-upcoming-clinical-trials
#8
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
#9
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/28544189/year-in-review-2016-interstitial-lung-disease-pulmonary-vascular-disease-pulmonary-function-paediatric-lung-disease-cystic-fibrosis-and-sleep
#10
REVIEW
Adelle S Jee, Tamera J Corte, Stephen J Wort, Neil D Eves, Claire E Wainwright, Amanda Piper
No abstract text is available yet for this article.
May 25, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
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
#11
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
#12
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/28543872/pai-1-gain-of-function-genotype-factors-increasing-pai-1-levels-and-airway-obstruction-the-gala-ii-cohort
#13
Michael G Sherenian, Seong H Cho, Albert Levin, Jin-Young Min, Sam S Oh, Donglei Hu, Joshua Galanter, Saunak Sen, Scott Huntsman, Celeste Eng, Jose R Rodriguez-Santana, Denise Serebrisky, Pedro C Avila, Ravi Kalhan, Lewis J Smith, Luisa N Borrell, Max A Seibold, L Keoki Williams, Esteban G Burchard, Rajesh Kumar
BACKGROUND: PAI-1 gain of function variants promote airway fibrosis, and are associated with asthma and with worse lung function in subjects with asthma. OBJECTIVE: We sought to determine if the association of a gain-of-function polymorphism in Plasminogen Activator Inhibitor -1 (PAI-1) with airway obstruction is modified by asthma status, and whether any genotype effect persists after accounting for common exposures that increase PAI-1 level. METHODS: We studied 2070 Latino children (8-21y) with genotypic and pulmonary function data from the GALA II cohort...
May 24, 2017: Clinical and Experimental Allergy: Journal of the British Society for Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28543309/adenovirus-vector-expressing-keratinocyte-growth-factor-using-cag-promoter-impaired-pulmonary-function-of-elastase-induced-emphysema
#14
Hiroshi Oki, Takuya Yazawa, Yasuko Baba, Yumi Kanegae, Hanako Sato, Seiko Sakamoto, Takahisa Goto, Izumu Saito, Kiyoyasu Kurahashi
Pulmonary emphysema impairs quality of life and increases mortality. Previous studies demonstrated that administration of KGF before elastase instillation prevented pulmonary emphysema in mice. We hypothesized that KGF could improve pulmonary function, and that the therapeutic administration of KGF would restore damaged lungs caused by elastase instillation in an animal model. We constructed KGF expressing adenovirus vector, which prevented bleomycin induced pulmonary fibrosis in the previous study. Adenovirus vector (1...
May 24, 2017: Microbiology and Immunology
https://www.readbyqxmd.com/read/28541746/the-evolving-cystic-fibrosis-microbiome-a-comparative-cohort-study-spanning-sixteen-years
#15
Nicole Acosta, Fiona J Whelan, Ranjani Somayaji, Ali Poonja, Michael G Surette, Harvey R Rabin, Michael D Parkins
RATIONALE: The cystic fibrosis (CF) airways are infected with a diverse polymicrobial community. OBJECTIVES: Understanding how changes in the CF microbiome have occurred over time, similar to the observed changes in the prevalence of cultured pathogens, is key in understanding the microbiome's role in disease. METHODS: Drawing from a prospectively collected and maintained sputum biobank, we identified 45 patients with sputum samples collected between the ages of 18-21 years in three successive cohorts of adults transitioning to our CF clinic: A(1997-2000), B(2004-2007), and C(2010-2013)...
May 25, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28540808/exposure-to-febrile-range-hyperthermia-potentiates-wnt-signalling-and-epithelial-mesenchymal-transition-gene-expression-in-lung-epithelium
#16
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/28540046/evaluation-of-a-novel-technique-in-airway-clearance-therapy-specific-cough-technique-sct-in-cystic-fibrosis-a-pilot-study-of-a-series-of-n-of-1-randomised-controlled-trials
#17
Sandra Gursli, Leiv Sandvik, Egil Bakkeheim, Bjørn Skrede, Britt Stuge
OBJECTIVES: The aim of this pilot study was to evaluate the efficacy, safety and participants' perception of a novel technique in airway clearance therapy - specific cough technique in cystic fibrosis. METHODS: We conducted randomised controlled individual trials (N-of-1 randomised controlled trials) in six adults. Each trial included 8 weeks of treatment with two interventions each week, one with specific cough technique and one with forced expiration technique...
2017: SAGE Open Medicine
https://www.readbyqxmd.com/read/28539224/inhaled-enac-antisense-oligonucleotide-ameliorates-cystic-fibrosis-like-lung-disease-in-mice
#18
Jeff R Crosby, Chenguang Zhao, Chong Jiang, Dong Bai, Melanie Katz, Sarah Greenlee, Hiroshi Kawabe, Michael McCaleb, Daniela Rotin, Shuling Guo, Brett P Monia
BACKGROUND: Epithelial sodium channel (ENaC, Scnn1) hyperactivity in the lung leads to airway surface dehydration and mucus accumulation in cystic fibrosis (CF) patients and in mice with CF-like lung disease. METHODS: We identified several potent ENaC specific antisense oligonucleotides (ASOs) and tested them by inhalation in mouse models of CF-like lung disease. RESULTS: The inhaled ASOs distributed into lung airway epithelial cells and decreased ENaC expression by inducing RNase H1-dependent degradation of the targeted Scnn1a mRNA...
May 20, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28537805/pathology-of-chronic-hypersensitivity-pneumonitis-what-is-it-what-are-the-diagnostic-criteria-why-do-we-care
#19
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/28536291/evolution-of-bacterial-frenemies
#20
Sophie E Darch, Carolyn B Ibberson, Marvin Whiteley
Chronic polymicrobial infections are associated with increased virulence compared to monospecies infections. However, our understanding of microbial dynamics during polymicrobial infection is limited. A recent study by Limoli and colleagues (D. H. Limoli, G. B. Whitfield, T. Kitao, M. L. Ivey, M. R. Davis, Jr., et al., mBio 8:e00186-17, 2017, https://doi.org/10.1128/mBio.00186-17) provides insight into a mechanism that may contribute to the coexistence of Pseudomonas aeruginosa and Staphylococcus aureus in the cystic fibrosis (CF) lung...
May 23, 2017: MBio
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