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Lung fibrosis

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https://www.readbyqxmd.com/read/28108847/airway-mucus-inflammation-and-remodeling-emerging-links-in-the-pathogenesis-of-chronic-lung-diseases
#1
REVIEW
Zhe Zhou-Suckow, Julia Duerr, Matthias Hagner, Raman Agrawal, Marcus A Mall
Airway mucus obstruction is a hallmark of many chronic lung diseases including rare genetic disorders such as cystic fibrosis (CF) and primary ciliary dyskinesia, as well as common lung diseases such as asthma and chronic obstructive pulmonary disease (COPD), which have emerged as a leading cause of morbidity and mortality worldwide. However, the role of excess airway mucus in the in vivo pathogenesis of these diseases remains poorly understood. The generation of mice with airway-specific overexpression of epithelial Na(+) channels (ENaC), exhibiting airway surface dehydration (mucus hyperconcentration), impaired mucociliary clearance (MCC) and mucus plugging, led to a model of muco-obstructive lung disease that shares key features of CF and COPD...
January 20, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28108166/predictive-performance-of-different-kidney-function-estimation-equations-in-lung-transplant-patients
#2
Dov A Degen, Jyotsna Janardan, Katherine A Barraclough, Hans G Schneider, Thomas Barber, Howard Barton, Gregory Snell, Bronwyn Levvey, Rowan G Walker
BACKGROUND: There has been limited examination of the performance of glomerular filtration rate estimation (eGFR) equations in lung transplant populations. This study aimed to compare the performance of serum creatinine and cystatin C based eGFR equations with Tc-99m diethylenetriaminepentaacetic acid (DTPA) GFR measurements in individuals with end-stage lung disease, either prior to, or following, lung transplantation. METHODS: In this prospective observational study, participants underwent GFR measurements with Tc-99m Pentetate...
January 17, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28107543/quantification-of-pulmonary-fibrosis-in-a-bleomycin-mouse-model-using-automated-histological-image-analysis
#3
Jean-Claude Gilhodes, Yvon Julé, Sebastian Kreuz, Birgit Stierstorfer, Detlef Stiller, Lutz Wollin
Current literature on pulmonary fibrosis induced in animal models highlights the need of an accurate, reliable and reproducible histological quantitative analysis. One of the major limits of histological scoring concerns the fact that it is observer-dependent and consequently subject to variability, which may preclude comparative studies between different laboratories. To achieve a reliable and observer-independent quantification of lung fibrosis we developed an automated software histological image analysis performed from digital image of entire lung sections...
2017: PloS One
https://www.readbyqxmd.com/read/28107411/xenon-enhanced-dual-energy-ct-imaging-in-combined-pulmonary-fibrosis-and-emphysema
#4
Keishi Sugino, Masahiro Kobayashi, Yasuhiko Nakamura, Kyoko Gocho, Fumiaki Ishida, Kazutoshi Isobe, Nobuyuki Shiraga, Sakae Homma
BACKGROUND: Little has been reported on the feasibility of xenon-enhanced dual-energy computed tomography (Xe-DECT) in the visual and quantitative analysis of combined pulmonary fibrosis and emphysema (CPFE). OBJECTIVES: We compared CPFE with idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD), as well as correlation with parameters of pulmonary function tests (PFTs). METHODS: Studied in 3 groups were 25 patients with CPFE, 25 with IPF without emphysema (IPF alone), 30 with COPD...
2017: PloS One
https://www.readbyqxmd.com/read/28107310/the-renin-inhibitor-aliskiren-protects-rat-lungs-from-the-histopathologic-effects-of-fat-embolism
#5
Amanda N Fletcher, Agostino Molteni, Rakesh Ponnapureddy, Chirag Patel, Mark Pluym, Alan M Poisner
BACKGROUND: Fat embolism (FE) and the consequent FE syndrome occurring after trauma or surgery can lead to serious pulmonary injury, including ARDS and death. Current treatment of FE syndrome is limited to supportive therapy. We have shown in a rat model that the renin angiotensin system plays a significant role in the pathophysiology of FE because drugs interfering with the renin angiotensin system, captopril and losartan reduce the histopathologic pulmonary damage. The purpose of the current study was to determine if inhibition of renin by aliskiren, an FDA-approved drug for treating hypertension, would produce effective protection in the same model...
February 2017: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/28106859/burkholderia-cepacia-complex-regulation-of-virulence-gene-expression-a-review
#6
REVIEW
Sílvia A Sousa, Joana R Feliciano, Tiago Pita, Soraia I Guerreiro, Jorge H Leitão
Burkholderia cepacia complex (Bcc) bacteria emerged as opportunistic pathogens in cystic fibrosis and immunocompromised patients. Their eradication is very difficult due to the high level of intrinsic resistance to clinically relevant antibiotics. Bcc bacteria have large and complex genomes, composed of two to four replicons, with variable numbers of insertion sequences. The complexity of Bcc genomes confers a high genomic plasticity to these bacteria, allowing their adaptation and survival to diverse habitats, including the human host...
January 19, 2017: Genes
https://www.readbyqxmd.com/read/28106744/oligonucleotide-therapy-for-obstructive-and-restrictive-respiratory-diseases
#7
REVIEW
Wupeng Liao, Jinrui Dong, Hong Yong Peh, Lay Hong Tan, Kah Suan Lim, Li Li, Wai-Shiu Fred Wong
Inhaled oligonucleotide is an emerging therapeutic modality for various common respiratory diseases, including obstructive airway diseases like asthma and chronic obstructive pulmonary disease (COPD) and restrictive airway diseases like idiopathic pulmonary fibrosis (IPF). The advantage of direct accessibility for oligonucleotide molecules to the lung target sites, bypassing systemic administration, makes this therapeutic approach promising with minimized potential systemic side effects. Asthma, COPD, and IPF are common chronic respiratory diseases, characterized by persistent airway inflammation and dysregulated tissue repair and remodeling, although each individual disease has its unique etiology...
January 17, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/28105512/global-dna-hypomethylation-has-no-impact-on-lung-function-or-serum-inflammatory-and-fibrosis-cytokines-in-asbestos-exposed-population
#8
Min Yu, Jianlin Lou, Hailing Xia, Min Zhang, Yixiao Zhang, Junqiang Chen, Xing Zhang, Shibo Ying, Lijin Zhu, Lihong Liu, Guang Jia
PURPOSE: To examine the effect of asbestos exposure on global DNA methylation and determine whether lung function and inflammatory and fibrosis biomarkers are correlated with the methylation state. METHODS: A total of 26 healthy subjects without asbestos exposure (Group 1), 47 healthy subjects with exposure (Group 2), and 52 subjects with benign asbestos-related disorders (ARDs) (Group 3) participated in this cross-sectional study. Blood global 5-methylcytosine (5mC) and serum TNF-α, collagen IV, CCL5 and CC16 concentrations were analyzed using enzyme-linked immunosorbent assay-like assays...
January 19, 2017: International Archives of Occupational and Environmental Health
https://www.readbyqxmd.com/read/28105131/immunoglobulin-g4-related-acquired-hemophilia-a-case-report
#9
Xiaoyan Li, Wei Duan, Xiang Zhu, Jianying Xu
Acquired hemophilia A (AHA) is a relatively rare and life-threatening bleeding disorder whose pathogenesis is not completely understood. The present study reports a rare case of immunogubulin (IgG)4-related AHA with multisystemic involvement. A 55-year old male patient presented with symptoms of bronchial asthma and multiple subdermal hematomas. Chest computed tomography showed multiple diffuse nodular lesions with thickening of bronchovascular bundles, and scattered high-density spots in both lung lobes. Laboratory investigations showed increased activated partial prothrombin time (120...
December 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28104103/comparative-proteomic-analysis-of-bleomycin-induced-pulmonary-fibrosis-based-on-isobaric-tag-for-quantitation
#10
Tiejun Yang, Yanlong Jia, Yongkang Ma, Liang Cao, Xiaobing Chen, Baoping Qiao
BACKGROUND: Pulmonary fibrosis (PF) is a destructive pulmonary disease and the molecular mechanisms underlying PF are unclear. This study investigated differentially expressed proteins associated with the occurrence and development of PF in rat lung tissue with bleomycin-induced PF. METHODS: Sixteen Sprague-Dawley rats were randomly divided into 2 groups: the PF model group (n = 8) and the control group (n = 8). After successfully establishing the rat PF model induced by bleomycin, the differentially expressed proteins in the 2 groups were identified through isobaric tag for relative and absolute quantitation coupled with liquid chromatography-mass spectrometry and bioinformatics analysis...
January 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28103851/cystic-fibrosis-physicians-perspectives-on-the-timing-of-referral-for-lung-transplant-evaluation-a-survey-of-physicians-in-the-united-states
#11
Kathleen J Ramos, Ranjani Somayaji, Erika D Lease, Christopher H Goss, Moira L Aitken
BACKGROUND: Prior studies reveal that a significant proportion of patients with cystic fibrosis (CF) and advanced lung disease are not referred for lung transplant (LTx) evaluation. We sought to assess expert CF physician perspectives on the timing of LTx referral and investigate their LTx knowledge. METHODS: We developed an online anonymous survey that was distributed by the Cystic Fibrosis Foundation (CFF) to the medical directors of all CFF-accredited care centers in the United States in 2015...
January 19, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28102970/microbiomes-in-respiratory-health-and-disease-an-asia-pacific-perspective
#12
REVIEW
Sanjay H Chotirmall, Shaan L Gellatly, Kurtis F Budden, Micheál Mac Aogain, Shakti D Shukla, David L A Wood, Philip Hugenholtz, Kevin Pethe, Philip M Hansbro
There is currently enormous interest in studying the role of the microbiome in health and disease. Microbiome's role is increasingly being applied to respiratory diseases, in particular COPD, asthma, cystic fibrosis and bronchiectasis. The changes in respiratory microbiomes that occur in these diseases and how they are modified by environmental challenges such as cigarette smoke, air pollution and infection are being elucidated. There is also emerging evidence that gut microbiomes play a role in lung diseases through the modulation of systemic immune responses and can be modified by diet and antibiotic treatment...
February 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28102546/ataluren-and-similar-compounds-specific-therapies-for-premature-termination-codon-class-i-mutations-for-cystic-fibrosis
#13
REVIEW
Aisha A Aslam, Colin Higgins, Ian P Sinha, Kevin W Southern
BACKGROUND: Cystic fibrosis is a common life-shortening genetic disorder in the Caucasian population (less common in other ethnic groups) caused by the mutation of a single gene that codes for the production of the cystic fibrosis transmembrane conductance regulator protein. This protein coordinates the transport of salt (and bicarbonate) across cell surfaces and the mutation most notably affects the airways. In the lungs of people with cystic fibrosis, defective protein results in a dehydrated surface liquid and compromised mucociliary clearance...
January 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28102223/global-and-local-selection-acting-on-the-pathogen-stenotrophomonas-maltophilia-in-the-human-lung
#14
Hattie Chung, Tami D Lieberman, Sara O Vargas, Kelly B Flett, Alexander J McAdam, Gregory P Priebe, Roy Kishony
Bacterial populations diversify during infection into distinct subpopulations that coexist within the human body. Yet, it is unknown to what extent subpopulations adapt to location-specific selective pressures as they migrate and evolve across space. Here we identify bacterial genes under local and global selection by testing for spatial co-occurrence of adaptive mutations. We sequence 552 genomes of the pathogen Stenotrophomonas maltophilia across 23 sites of the lungs from a patient with cystic fibrosis. We show that although genetically close isolates colocalize in space, distant lineages with distinct phenotypes separated by adaptive mutations spread throughout the lung, suggesting global selective pressures...
January 19, 2017: Nature Communications
https://www.readbyqxmd.com/read/28100499/gremlin1-plays-a-key-role-in-kidney-development-and-renal-fibrosis
#15
Rachel H Church, Imran Ali, Mitchel Tate, Deborah Lavin, Arjun Krishnakumar, Helena M Kok, Roel Goldschmeding, Finian Martin, Derek Brazil
Grem1, an antagonist of bone morphogenetic proteins, plays a key role in embryogenesis. A highly specific temporospatial gradient of Grem1 and BMP signalling is critical to normal lung, kidney and limb development. Grem1 levels are increased in renal fibrotic conditions including acute kidney injury, diabetic nephropathy, chronic allograft nephropathy and immune glomerulonephritis. A small number of grem1-/- whole body knockout mice on a mixed genetic background (8 %) are viable, with a single, enlarged left kidney and grossly normal histology...
January 18, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28099617/case-for-diagnosis-lichen-myxedematosus
#16
Priscila Regina Orso Rebellato, Mauren Beatriz Frazon Carbonar, Nicole Iasmin Magario Tabuti, Graziela Junges Crescente Rastelli
Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. This article reports the case of a scleromyxedema patient with a recent history of acute myocardial infarction and monoclonal gammopathy...
November 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/28099456/burden-of-idiopathic-pulmonary-fibrosis-progression-a-5-year-longitudinal-follow-up-study
#17
Vincent Cottin, Aurélie Schmidt, Laura Catella, Fanny Porte, Céline Fernandez-Montoya, Katell Le Lay, Stève Bénard
Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with an unpredictable course. An observational study was set up using the French hospital discharge database to describe the reasons, outcomes and costs of hospitalisations related to this disease. Patients newly hospitalised for idiopathic pulmonary fibrosis (ICD-10 code: J84.1) in 2008 were identified and followed for 5 years. As J84.1 includes other fibrotic pulmonary diseases, an algorithm excluding age<50 years and presence of a differential diagnosis in the following year was defined...
2017: PloS One
https://www.readbyqxmd.com/read/28099038/an-exome-sequencing-study-to-assess-the-role-of-rare-genetic-variation-in-pulmonary-fibrosis
#18
Slavé Petrovski, Jamie L Todd, Michael T Durheim, Quanli Wang, Jason W Chien, Fran L Kelly, Courtney Frankel, Caroline M Mebane, Zhong Ren, Joshua Bridgers, Thomas J Urban, Colin D Malone, Ashley Finlen Copeland, Christie Brinkley, Andrew S Allen, Thomas O'Riordan, John G McHutchison, Scott M Palmer, David B Goldstein
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is an increasingly recognized, often fatal lung disease of unknown etiology. OBJECTIVES: The aim of this study was to use whole-exome sequencing to improve our understanding of the genetic architecture of pulmonary fibrosis. METHODS: We performed a case-control exome-wide collapsing analysis including 262 unrelated individuals with pulmonary fibrosis; clinically classified as IPF according to ATS/ERS/JRS/ALAT guidelines (81...
January 18, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28098218/the-role-of-mir-497-5p-in-myofibroblast-differentiation-of-lr-mscs-and-pulmonary-fibrogenesis
#19
Xiang Chen, Chaowen Shi, Cong Wang, Weilin Liu, Yanhong Chu, Zou Xiang, Kebin Hu, Ping Dong, Xiaodong Han
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal fibrotic lung disease characterized by profound changes in stem cell differentiation, epithelial cell phenotypes and fibroblast proliferation. In our study, we found that miR-497-5p was significantly upregulated both during myofibroblast differentiation of lung resident mesenchymal stem cells (LR-MSCs) and in the lung tissues of a pulmonary fibrosis model. In addition, as determined by luciferase assays and Western blot analysis, reversion-inducing cysteine-rich protein with kazal motifs (Reck) was identified to be one of the target genes of miR-497-5p, and Reck could suppress the expression of matrix metalloproteinase-2 (Mmp2) and Mmp9, which could activate latent transforming growth factor-β1 (TGF-β1)...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28096998/treatment-with-nintedanib-for-acute-exacerbation-of-idiopathic-pulmonary-fibrosis
#20
Hiromi Tomioka, Hirohito Takada
There is currently no effective treatment for acute exacerbation of idiopathic pulmonary fibrosis (IPF). We herein report the case of a patient with acute exacerbation of IPF which was treated with nintedanib, an intracellular inhibitor of tyrosine kinases, and showed improvement of the condition. An 84-year-old man with IPF was admitted to our hospital because of dry cough and worsening of dyspnoea within last 1 month. He presented with hypoxemia, and chest high-resolution computed tomography (HRCT) revealed new, bilateral multifocal ground-glass opacities superimposed on the background of lung fibrosis...
March 2017: Respirology Case Reports
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