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Pulmonary fibrosis

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https://www.readbyqxmd.com/read/28441853/-differentially-expressed-genes-analysis-in-expression-profile-data-of-pulmonary-fibrosis-with-pulmonary-hypertension
#1
R Miao, D Leng, Y Wang, J F Li, J N Gong, Y Liang, Y H Yang
Objective: To analyze the differential gene expression of patients with idiopathic pulmonary fibrosis and pulmonary hypertension (IPF-PH). Methods: The expression profile data of GSE15197 was downloaded from the Gene Expression Omnibus (GEO) database. Bonferroni algorithm was used to identify the differentially expressed genes of pulmonary tissues from IPF-PH, idiopathic pulmonary arterial hypertension (IPAH) and Normal groups. Principal component analysis was used to extract the principal components of three types of samples and differentially expressed genes were obtained...
April 25, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28441669/keep-them-breathing-cystic-fibrosis-pathophysiology-diagnosis-and-treatment
#2
Sheena D Brown, Rachel White, Phil Tobin
Cystic fibrosis (CF) affects more than 30,000 people in the United States and 80,000 people worldwide. This life-threatening genetic disorder causes a buildup of thick, viscous mucus secretions in various organ systems, most commonly the gastrointestinal, pulmonary, and genitourinary systems. This article reviews the clinical manifestations, diagnosis, and monitoring of patients with CF as well as guidelines for management and emerging pharmacologic treatments.
May 2017: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/28441449/effectiveness-and-safety-of-mycophenolate-mofetil-in-idiopathic-pulmonary-fibrosis
#3
Anoop M Nambiar, Antonio R Anzueto, Jay I Peters
BACKGROUND: Currently available antifibrotic treatments may slow down disease progression in idiopathic pulmonary fibrosis (IPF), but are associated with potentially significant side effects and are costly. Mycophenolate mofetil (MMF) is well known for its potent immunosuppressive properties and possesses important antiproliferative and antifibrotic effects. The safety and effectiveness of MMF in IPF is unknown. METHODS: We performed a retrospective multicohort analysis of IPF patients treated with MMF compared to those treated with either ineffective/harmful treatments or no treatment...
2017: PloS One
https://www.readbyqxmd.com/read/28440853/antibiotic-strategies-for-eradicating-pseudomonas-aeruginosa-in-people-with-cystic-fibrosis
#4
REVIEW
Simon C Langton Hewer, Alan R Smyth
BACKGROUND: Respiratory tract infection with Pseudomonas aeruginosa occurs in most people with cystic fibrosis. Once chronic infection is established, Pseudomonas aeruginosa is virtually impossible to eradicate and is associated with increased mortality and morbidity. Early infection may be easier to eradicate.This is an update of a Cochrane review first published in 2003, and previously updated in 2006, 2009 and 2014. OBJECTIVES: To determine whether antibiotic treatment of early Pseudomonas aeruginosa infection in children and adults with cystic fibrosis eradicates the organism, delays the onset of chronic infection, and results in clinical improvement...
April 25, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28440556/direct-hemoperfusion-with-polymyxin-b-immobilized-fibre-treatment-for-acute-exacerbation-of-interstitial-pneumonia
#5
Haruhiko Furusawa, Makiko Sugiura, Chieko Mitaka, Naohiko Inase
BACKGROUND AND OBJECTIVE: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is recognized as an important cause of mortality. AE has also been reported in patients with other interstitial lung diseases such as idiopathic non-specific interstitial pneumonia (NSIP) and interstitial pneumonia associated with collagen vascular disease (CVD). Current therapies such as high-dose corticosteroid with immunosuppressive agents have provided little benefit for AE. Direct hemoperfusion (DHP) with a polymyxin B-immobilized fibre column (PMX) was originally developed for the treatment of endotoxaemia...
April 25, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28440554/tolerability-of-treatment-with-pirfenidone-or-nintedanib-for-pulmonary-fibrosis-in-the-real-world
#6
EDITORIAL
Takashi Ogura, Hideya Kitamura
No abstract text is available yet for this article.
April 25, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28440535/socioeconomic-status-and-its-relationship-to-chronic-respiratory-disease
#7
Sonu Sahni, Ankoor Talwar, Sameer Khanijo, Arunabh Talwar
Socioeconomic status (SES) is defined as an individual's social or economic standing, and is a measure of an individual's or family's social or economic position or rank in a social group. It is a composite of several measures including income, education, occupation, location of residence or housing. Studies have found a lower SES has been linked to disproportionate access to health care in many diseases. There is emerging data in pulmonary diseases such as COPD, asthma, cystic fibrosis, pulmonary hypertension and other chronic respiratory conditions that allude to a similar observation noted in other chronic diseases...
2017: Advances in Respiratory Medicine
https://www.readbyqxmd.com/read/28440314/the-peripheral-blood-proteome-signature-of-idiopathic-pulmonary-fibrosis-is-distinct-from-normal-and-is-associated-with-novel-immunological-processes
#8
David N O'Dwyer, Katy C Norman, Meng Xia, Yong Huang, Stephen J Gurczynski, Shanna L Ashley, Eric S White, Kevin R Flaherty, Fernando J Martinez, Susan Murray, Imre Noth, Kelly B Arnold, Bethany B Moore
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial pneumonia. The disease pathophysiology is poorly understood and the etiology remains unclear. Recent advances have generated new therapies and improved knowledge of the natural history of IPF. These gains have been brokered by advances in technology and improved insight into the role of various genes in mediating disease, but gene expression and protein levels do not always correlate. Thus, in this paper we apply a novel large scale high throughput aptamer approach to identify more than 1100 proteins in the peripheral blood of well-characterized IPF patients and normal volunteers...
April 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28440108/microbiology-of-the-upper-and-lower-airways-in-pediatric-cystic-fibrosis-patients
#9
Lindsay Sobin, Kosuke Kawai, Alexandria L Irace, Ozgul Gergin, Michael Cunningham, Gregory S Sawicki, Eelam A Adil
Objective To evaluate the microbiology of the upper and lower airways in pediatric cystic fibrosis (CF) patients who underwent sinus surgery. Study Design Retrospective case series with chart review. Setting Tertiary care children's hospital. Subjects and Methods A total of 201 paired sinus and pulmonary cultures from 105 CF patients were identified between 1996 and 2014. Demographics and culture results were analyzed. Results The mean age of patients was 11.2 ± 5.4 years (range, 1-27 years), and approximately one-half were female...
April 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28439630/myriocin-treatment-of-cf-lung-infection-and-inflammation-complex-analyses-for-enigmatic-lipids
#10
Anna Caretti, Michele Vasso, Fabiola Tecla Bonezzi, Andrea Gallina, Marco Trinchera, Alice Rossi, Raffaella Adami, Josefina Casas, Monica Falleni, Delfina Tosi, Alessandra Bragonzi, Riccardo Ghidoni, Cecilia Gelfi, Paola Signorelli
Our aim was to use quantitative and qualitative analyses to gain further insight into the role of ceramide in cystic fibrosis (CF). Sphingolipid ceramide is a known inflammatory mediator, and its accumulation in inflamed lung has been reported in different types of emphysema, chronic obstructive pulmonary disease and CF. CF is caused by a mutation of the chloride channel and associated with hyperinflammation of the respiratory airways and high susceptibility to ongoing infections. We have previously demonstrated that de novo ceramide synthesis is enhanced in lung inflammation and sustains Pseudomonas aeruginosa pulmonary infection in a CF murine model...
April 24, 2017: Naunyn-Schmiedeberg's Archives of Pharmacology
https://www.readbyqxmd.com/read/28439270/cytokine-regulation-of-na-k-cl-cotransporter-1-and-cystic-fibrosis-transmembrane-conductance-regulator-potential-role-in-pulmonary-inflammation-and-edema-formation
#11
REVIEW
Sarah Weidenfeld, Wolfgang M Kuebler
Pulmonary edema, a major complication of lung injury and inflammation, is defined as accumulation of extravascular fluid in the lungs leading to impaired diffusion of respiratory gases. Lung fluid balance across the alveolar epithelial barrier protects the distal airspace from excess fluid accumulation and is mainly regulated by active sodium transport and Cl(-) absorption. Increased hydrostatic pressure as seen in cardiogenic edema or increased vascular permeability as present in inflammatory lung diseases such as the acute respiratory distress syndrome (ARDS) causes a reversal of transepithelial fluid transport resulting in the formation of pulmonary edema...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28438615/clinicopathological-immunohistochemical-and-mutational-analyses-of-pulmonary-enteric-adenocarcinoma-usefulness-of-satb2-and-%C3%AE-catenin-immunostaining-for-differentiation-from-metastatic-colorectal-carcinoma
#12
Jun Matsushima, Takuya Yazawa, Masaki Suzuki, Yoko Takahashi, Satoshi Ota, Takahiro Nakajima, Ichiro Yoshino, Tomoyuki Yokose, Toru Inoue, Kunimitsu Kawahara, Yukio Nakatani
Pulmonary enteric adenocarcinoma (PEA) is a rare variant of pulmonary adenocarcinoma; it is sometimes difficult to discriminate between PEA and metastatic colorectal carcinoma (MCRC) because of their morphological and immunohistochemical resemblance. Here, we conducted clinicopathological, immunohistochemical, and mutational analyses of PEA with special focus on its differentiation from MCRC. We comparatively analyzed eight surgically resected PEA tumors (7 patients) and 20 cases of MCRC. Patients were aged 43-77years (average age, 64...
April 21, 2017: Human Pathology
https://www.readbyqxmd.com/read/28438499/rationalizing-endpoints-for-prospective-studies-of-pulmonary-exacerbation-treatment-response-in-cystic-fibrosis
#13
D R VanDevanter, S L Heltshe, J Spahr, V V Beckett, C L Daines, E C Dasenbrook, R L Gibson, R Jain, D B Sanders, C H Goss, P A Flume
BACKGROUND: Given the variability in pulmonary exacerbation (PEx) management within and between Cystic Fibrosis (CF) Care Centers, it is possible that some approaches may be superior to others. A challenge with comparing different PEx management approaches is lack of a community consensus with respect to treatment-response metrics. In this analysis, we assess the feasibility of using different response metrics in prospective randomized studies comparing PEx treatment protocols. METHODS: Response parameters were compiled from the recent STOP (Standardized Treatment of PEx) feasibility study...
April 21, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28437189/metformin-attenuates-radiation-induced-pulmonary-fibrosis-in-a-murine-model
#14
Jian Wang, Ye Wang, Jun Han, Hong Mei, Dandan Yu, Qian Ding, Tao Zhang, Gang Wu, Gang Peng, Zhenyu Lin
While radiotherapy continues to be a major cancer treatment option, its dose-limiting side effects, such as pulmonary fibrosis, severely impair the quality of life in these patients. In this study, we evaluated the radioprotective effects of metformin, a commonly used biguanide antidiabetic medication, in a murine model of pulmonary damage. Sprague Dawley(®) rats received whole lung 20 Gy irradiation with or without metformin treatment. Computed tomography (CT) was performed and Hounsfield units (HU) were determined during the observation period...
April 24, 2017: Radiation Research
https://www.readbyqxmd.com/read/28436965/a-three-dimensional-model-of-human-lung-development-and-disease-from-pluripotent-stem-cells
#15
Ya-Wen Chen, Sarah Xuelian Huang, Ana Luisa Rodrigues Toste de Carvalho, Siu-Hong Ho, Mohammad Naimul Islam, Stefano Volpi, Luigi D Notarangelo, Michael Ciancanelli, Jean-Laurent Casanova, Jahar Bhattacharya, Alice F Liang, Laura M Palermo, Matteo Porotto, Anne Moscona, Hans-Willem Snoeck
Recapitulation of lung development from human pluripotent stem cells (hPSCs) in three dimensions (3D) would allow deeper insight into human development, as well as the development of innovative strategies for disease modelling, drug discovery and regenerative medicine. We report here the generation from hPSCs of lung bud organoids (LBOs) that contain mesoderm and pulmonary endoderm and develop into branching airway and early alveolar structures after xenotransplantation and in Matrigel 3D culture. Expression analysis and structural features indicated that the branching structures reached the second trimester of human gestation...
April 24, 2017: Nature Cell Biology
https://www.readbyqxmd.com/read/28436621/risk-factors-for-mortality-before-age-18-years-in-cystic-fibrosis
#16
Susanna A McColley, Michael S Schechter, Wayne J Morgan, David J Pasta, Marcia L Craib, Michael W Konstan
BACKGROUND: Understanding early-life risk factors for childhood death in cystic fibrosis (CF) is important for clinical care, including the identification of effective interventions. METHODS: Data from the Epidemiologic Study of Cystic Fibrosis (ESCF) collected 1994-2005 were linked with the Cystic Fibrosis Foundation Patient Registry (CFFPR) demographic and mortality data from 2013. Inclusion criteria were ≥1 visit annually at age 3-5 years and ≥1 FEV1 measurement at age 6-8 years...
April 24, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28436606/early-and-late-adverse-effects-of-radioiodine-for-pediatric-differentiated-thyroid-cancer
#17
Domenico Albano, Francesco Bertagna, Maria Beatrice Panarotto, Raffaele Giubbini
BACKGROUND: Radioiodine-131 (I131) therapy for differentiated thyroid cancer (DTC) is generally a safe and effective treatment, but it has some potential side effects, which have been well described in adults but less analyzed in children. Our aim was to describe early and late adverse events of radioactive I131 in pediatric patients. METHODS: All consecutive patients ≤18 years treated for DTC in the period 1980-2015 were retrospectively analyzed for early and late side effects of radioiodine...
April 24, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28435843/mmp-7-is-a-predictive-biomarker-of-disease-progression-in-patients-with-idiopathic-pulmonary-fibrosis
#18
Yasmina Bauer, Eric S White, Simon de Bernard, Peter Cornelisse, Isabelle Leconte, Adele Morganti, Sebastien Roux, Oliver Nayler
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with poor prognosis, which is characterised by destruction of normal lung architecture and excessive deposition of lung extracellular matrix. The heterogeneity of disease progression in patients with IPF poses significant obstacles to patient care and prevents efficient development of novel therapeutic interventions. Blood biomarkers, reflecting pathobiological processes in the lung, could provide objective evidence of the underlying disease...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28435675/microbiome-effects-on-immunity-health-and-disease-in-the-lung
#19
REVIEW
Shakti D Shukla, Kurtis F Budden, Rachael Neal, Philip M Hansbro
Chronic respiratory diseases, including asthma, chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF), are among the leading causes of mortality and morbidity worldwide. In the past decade, the interest in the role of microbiome in maintaining lung health and in respiratory diseases has grown exponentially. The advent of sophisticated multiomics techniques has enabled the identification and characterisation of microbiota and their roles in respiratory health and disease. Furthermore, associations between the microbiome of the lung and gut, as well as the immune cells and mediators that may link these two mucosal sites, appear to be important in the pathogenesis of lung conditions...
March 2017: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/28435277/role-of-pirfenidone-in-the-management-of-pulmonary-fibrosis
#20
REVIEW
Keith C Meyer, Catherine A Decker
Pulmonary fibrosis is associated with a number of specific forms of interstitial lung disease (ILD) and can lead to progressive decline in lung function, poor quality of life, and, ultimately, early death. Idiopathic pulmonary fibrosis (IPF), the most common fibrotic ILD, affects up to 1 in 200 elderly individuals and has a median survival that ranges from 3 to 5 years following initial diagnosis. IPF has not been shown to respond to immunomodulatory therapies, but recent trials with novel antifibrotic agents have demonstrated lessening of lung function decline over time...
2017: Therapeutics and Clinical Risk Management
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