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Pulmonary fibrosis

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https://www.readbyqxmd.com/read/29161089/protein-folding-and-the-challenges-of-maintaining-endoplasmic-reticulum-proteostasis-in-idiopathic-pulmonary-fibrosis
#1
Freddy Romero, Ross Summer
Alveolar epithelial type II (AEII) cells are "professional" secretory cells that synthesize and secrete massive quantities of proteins to produce pulmonary surfactant and maintain airway immune defenses. To facilitate this high level of protein synthesis, AEII cells are equipped with an elaborate endoplasmic reticulum (ER) structure and possess an abundance of the machinery needed to fold, assemble, and secrete proteins. However, conditions that suddenly increase the quantity of new proteins entering the ER or that impede the capacity of the ER to fold proteins can cause misfolded or unfolded proteins to accumulate in the ER lumen, also called ER stress...
November 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29161079/a-new-frontier-in-immunometabolism-cholesterol-in-lung-health-and-disease
#2
Michael B Fessler
The lung has a unique relationship to cholesterol that is shaped by its singular physiology. On the one hand, the lungs receive the full cardiac output and have a predominant dependence on plasma lipoprotein uptake for their cholesterol supply. On the other hand, surfactant lipids, including cholesterol, are continually susceptible to oxidation owing to direct environmental exposure and must be cleared or recycled because of the very narrow biophysical mandates placed upon surfactant lipid composition. Interestingly, increased lipid-laden macrophage "foam cells" have been noted in a wide range of human lung pathologies...
November 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29161077/mitochondrial-dysfunction-in-pulmonary-fibrosis
#3
Sunad Rangarajan, Karen Bernard, Victor J Thannickal
The aging of the human population has resulted in an unprecedented increase in the incidence and prevalence of age-related diseases, including those of the lung. Idiopathic pulmonary fibrosis is a disease of aging, and is characterized by a progressive decline in lung function and high mortality. Recent studies suggest that mitochondrial dysfunction, which can accompany aging phenotypes, may contribute to the pathogenesis of idiopathic pulmonary fibrosis. In this review, we explore current evidence for mitochondrial dysfunction in alveolar epithelial cells, fibroblasts, and immune cells that participate in the fibrotic process...
November 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29160385/usual-interstitial-pneumonia-typical-possible-and-inconsistent-patterns
#4
Pedro Paulo Teixeira E Silva Torres, Marcelo Fouad Rabahi, Maria Auxiliadora Carmo Moreira, Gustavo de Souza Portes Meirelles, Edson Marchiori
Idiopathic pulmonary fibrosis is a severe and progressive chronic fibrosing interstitial lung disease, a definitive diagnosis being established by specific combinations of clinical, radiological, and pathological findings. According to current international guidelines, HRCT plays a key role in establishing a diagnosis of usual interstitial pneumonia (UIP). Current guidelines describe three UIP patterns based on HRCT findings: a typical UIP pattern; a pattern designated "possible UIP"; and a pattern designated "inconsistent with UIP", each pattern having important diagnostic implications...
September 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/29159034/hamman-rich-syndrome
#5
Arshiya Mastan, Nilaani Murugesu, Adil Hasnain, Terry O'Shaughnessy, Vladimir Macavei
INTRODUCTION: Acute interstitial pneumonia is a rare but important diagnosis, associated with a high mortality rate and important to identify early. CASE PRESENTATION: A 76 year-old individual presented to hospital with a two-week history of shortness of breath, fevers and a non-productive cough. Treatment initially was for lower respiratory tract infection but returned to hospital three days later as her shortness of breath and peripheral oedema was worsening despite diuretic treatment...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29159032/use-of-ceftolozane-tazobactam-in-a-cystic-fibrosis-patient-with-multidrug-resistant-pseudomonas-infection-and-renal-insufficiency
#6
Katie Stokem, Jonathan B Zuckerman, David P Nicolau, Minkey Wungwattana, Edmund H Sears
We report the successful use of ceftolozane/tazobactam (C/T) to treat a pulmonary exacerbation in a 35 year old female, post lung transplant, with cystic fibrosis (CF), malnutrition, chronic kidney disease, and multi-drug resistant Pseudomonas aeruginosa infection (MDR PSA). Given the complexity of the clinical profile, we measured drug levels of C/T during treatment of her current exacerbation to determine pharmacokinetics. The patient achieved an estimated ceftolozane peak of 174.1 μg/mL and trough of 9...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29158824/dioscin-exerts-protective-effects-against-crystalline-silica-induced-pulmonary-fibrosis-in-mice
#7
Chao Li, Yiping Lu, Sitong Du, Siyi Li, Yiting Zhang, Fangwei Liu, Ying Chen, Dong Weng, Jie Chen
Inhalation of crystalline silica particles leads to pulmonary fibrosis, eventually resulting in respiratory failure and death. There are few effective drugs that can delay the progression of this disease; thus, patients with silicosis are usually only offered supportive care. Dioscin, a steroidal saponin, exhibits many biological activities and health benefits including its protective effects against hepatic fibrosis. However, the effect of dioscin on silicosis is unknown. METHODS: We employed experimental mouse mode of silicosis...
2017: Theranostics
https://www.readbyqxmd.com/read/29158503/kr%C3%A3-pple-like-factor-4-attenuates-lung-fibrosis-via-inhibiting-epithelial-mesenchymal-transition
#8
Lianjun Lin, Qian Han, Yan Xiong, Ting Li, Zhonghui Liu, Huiying Xu, Yanping Wu, Nanping Wang, Xinmin Liu
Epithelial-mesenchymal transition (EMT) plays an important role in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Krüpple-like-factor 4 (KLF4), has been suggested to play an important role in the phenotype transition. However, its function in pulmonary fibrosis and EMT of human alveolar epithelial cells (AECs) remains unclear. This study aimed to examine the role of KLF4 in pulmonary fibrosis and EMT. Decreased expression of KLF4 was first observed in human IPF lung tissues and models of bleomycin-induced pulmonary fibrosis...
November 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29157921/pooling-of-bronchoalveolar-lavage-in-children-with-cystic-fibrosis-does-not-adversely-affect-the-microbiological-yield-or-sensitivity-in-detecting-pulmonary-inflammation
#9
Paul McNally, Jennifer O'Rourke, Emmanuelle Fantino, Archana Chacko, Rishi Pabary, Andrew Turnbull, Tim Grant, Niamh O'Sullivan, Claire Wainwright, Barry Linnane, Jane C Davies, Peter D Sly
BACKGROUND: Bronchoalveolar lavage (BAL) is a potentially useful outcome measure for clinical trials in children with CF but its use is limited by variations in approach internationally. We sought to determine if pooling adversely affected the diagnostic properties of BAL. METHODS: Children undergoing bronchoscopy for clinical reasons were included. A multi-step study protocol ensured BAL was collected and analysed both separately and as a pooled fluid. RESULTS: Eighty-five children (53 CF, 32 control) were recruited...
November 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29155707/optimized-lc-ms-ms-method-for-the-high-throughput-analysis-of-clinical-samples-of-ivacaftor-its-major-metabolites-and-lumacaftor-in-biological-fluids-of-cystic-fibrosis-patients
#10
Elena K Schneider, Felisa Reyes-Ortega, Jian Li, Tony Velkov
Defects in the cystic fibrosis trans-membrane conductance regulator (CFTR) are the cause of cystic fibrosis (CF), a disease with life-threatening pulmonary manifestations. Ivacaftor (IVA) and ivacaftor-lumacaftor (LUMA) combination are two new breakthrough CF drugs that directly modulate the activity and trafficking of the defective CFTR-protein. However, there is still a dearth of understanding on pharmacokinetic/pharmacodynamic parameters and the pharmacology of ivacaftor and lumacaftor. The HPLC-MS technique for the simultaneous analysis of the concentrations of ivacaftor, hydroxymethyl-ivacaftor, ivacaftor-carboxylate, and lumacaftor in biological fluids in patients receiving standard ivacaftor or ivacaftor-lumacaftor combination therapy has previously been developed by our group and partially validated to FDA standards...
October 15, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29155027/serum-level-of-transforming-growth-factor-beta-1-is-associated-with-left-atrial-voltage-in-patients-with-chronic-atrial-fibrillation
#11
Shilu Zhao, Mingfang Li, Weizhu Ju, Lingyun Gu, Fengxiang Zhang, Hongwu Chen, Kai Gu, Bing Yang, Minglong Chen
BACKGROUND: Atrial tissue fibrosis can cause electrical or structural remodeling in patients with atrial fibrillation. Transforming growth factor beta 1(TGF-β1) signaling acts as a central role in fibroblast activation. In this report, we aimed to investigate the relationship between serum level of TGF-β1 and mean left atrial voltage in patients with chronic atrial fibrillation (CAF). METHODS: A total of 16 consecutive adult patients with CAF who underwent catheter ablation were enrolled...
November 15, 2017: Indian Pacing and Electrophysiology Journal
https://www.readbyqxmd.com/read/29154426/microvascular-dysfunction-and-cardiac-fibrosis-in-heart-failure-with-preserved-ejection-fraction-a-case-report
#12
Nikhil Narang, Diego Medvedofsky, Kathryn Dryer, Sanjiv J Shah, Charles J Davidson, Amit R Patel, John E A Blair
We report the case of a 55-year-old woman with heart failure with preserved ejection fraction (HFpEF), who presented with hypertensive urgency and pulmonary oedema. The patient was medically optimized and underwent cardiac catheterization revealing pulmonary hypertension, elevated pulmonary capillary wedge pressure, normal cardiac index, and non-obstructive coronary disease. Invasive evaluation of coronary flow revealed blunted coronary flow reserve and increased index of microvascular resistance. Cardiac magnetic resonance imaging demonstrated reduced global myocardial perfusion and diffuse interstitial fibrosis...
November 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/29154106/diagnostic-criteria-for-idiopathic-pulmonary-fibrosis-a-fleischner-society-white-paper
#13
REVIEW
David A Lynch, Nicola Sverzellati, William D Travis, Kevin K Brown, Thomas V Colby, Jeffrey R Galvin, Jonathan G Goldin, David M Hansell, Yoshikazu Inoue, Takeshi Johkoh, Andrew G Nicholson, Shandra L Knight, Suhail Raoof, Luca Richeldi, Christopher J Ryerson, Jay H Ryu, Athol U Wells
This Review provides an updated approach to the diagnosis of idiopathic pulmonary fibrosis (IPF), based on a systematic search of the medical literature and the expert opinion of members of the Fleischner Society. A checklist is provided for the clinical evaluation of patients with suspected usual interstitial pneumonia (UIP). The role of CT is expanded to permit diagnosis of IPF without surgical lung biopsy in select cases when CT shows a probable UIP pattern. Additional investigations, including surgical lung biopsy, should be considered in patients with either clinical or CT findings that are indeterminate for IPF...
November 15, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/29154105/taking-a-giant-step-in-the-diagnosis-of-idiopathic-pulmonary-fibrosis
#14
Vasilios Tzilas, Dominique Valeyre, Argyris Tzouvelekis, Demosthenes Bouros
No abstract text is available yet for this article.
November 15, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/29152045/discovery-of-indolinone-based-multikinase-inhibitors-as-potential-therapeutics-for-idiopathic-pulmonary-fibrosis
#15
Zhenhua Huang, Heran Li, Qian Zhang, Fangzheng Lu, Mei Hong, Zhigang Zhang, Xiaocui Guo, Yuanju Zhu, Sanming Li, Hongzhuo Liu
Idiopathic pulmonary fibrosis (IPF) is a serious and deadly disease for which treatment options are limited. The recent approval of antifibrosis agent nintedanib represents one of the first therapeutic approaches for the treatment of IPF. Here, we report novel indolinone-based multikinase inhibitors that target angiogenesis and fibrosis pathways and may serve as potential therapeutics for IPF. KBP-7018 is a novel, tyrosine kinase-selective inhibitor with potent effects on three fibrotic kinases (c-KIT, PDGFR, and RET)...
November 9, 2017: ACS Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/29151518/prognostic-factors-in-the-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-retrospective-single-center-study
#16
Kenichiro Atsumi, Yoshinobu Saito, Naoyuki Kuse, Kenichi Kobayashi, Toru Tanaka, Takeru Kashiwada, Minoru Inomata, Nariaki Kokuho, Hiroki Hayashi, Koichiro Kamio, Kazue Fujita, Shinji Abe, Arata Azuma, Kaoru Kubota, Akihiko Gemma
Objectives Acute exacerbation of idiopathic pulmonary fibrosis (IPF-AE) has been recognized as a fatal pulmonary disorder, but the exact prognostic factors are unknown. The aim of the present study was to analyze the clinical characteristics of patients with IPF-AE and identify the prognostic factors. Methods The medical records of 59 cases of IPF-AE were retrospectively reviewed. Clinical data, laboratory data, radiographic findings, treatment, and time from the onset of symptoms to the initiation of corticosteroid pulse therapy, i...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29151428/continuous-positive-airway-pressure-in-patients-with-obstructive-sleep-apnea-and-non-alcoholic-steatohepatitis-a-systematic-review-and-meta-analysis
#17
Gonzalo Labarca, Rodrigo Cruz, Jorge Jorquera
STUDY OBJECTIVES: Several studies have reported an association between obstructive sleep apnea (OSA) and several extra-pulmonary issues, such as arterial hypertension and insulin resistance. In recent years, the associations between OSA, non-alcoholic fatty liver disease, and non-alcoholic steatohepatitis (NASH) have been published; however, there is a gap between experimental and clinical studies regarding the efficacy of continuous positive airway pressure (CPAP) treatment in patient populations with these conditions...
November 15, 2017: Journal of Clinical Sleep Medicine: JCSM: Official Publication of the American Academy of Sleep Medicine
https://www.readbyqxmd.com/read/29151410/-effects-of-curcumin-on-pulmonary-fibrosis-and-functions-of-paraquat-challenged-rats
#18
Honggang Chen, Rongjia Yang, Yan Tang, Jun Xu, Youfan Feng, Shijie Liu, Shikui Zhang, Lijun Hou
OBJECTIVE: To observe the effects of curcumin on pulmonary fibrosis and functions on paraquat (PQ)-challenged rats, and investigate the possible mechanism. METHODS: 108 SPF Wistar rats were divided into three groups according to random number sheet: normal saline (NS) control group, PQ model group and curcumin-treatment group. The rats in each group were subdivided into three subgroups according to different time points (3, 7, 14 days), with 12 rats in each subgroup...
November 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/29151332/the-relationship-between-the-degree-of-skin-fibrosis-by-sonoelastography-and-the-degree-of-pulmonary-involvement-in-scleroderma
#19
Songül Çildağ, Mehmet Burak Çildağ
Background/aim: This study aimed to assess the relationship between skin fibrosis as determined by sonoelastography and the degree of pulmonary involvement as determined by high-resolution computed tomography (HRCT) in patients with diffuse cutaneous systemic sclerosis (dcSSc).Materials and methods: This prospective study included 40 patients with dcSSc. All patients with HRCT scans underwent conventional ultrasonography and sonoelastography to determine skin thickness and degree of fibrosis. The degree of fibrosis was classified according to color-scale sonoelastography...
November 13, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29151314/effect-of-inhaled-steroids-on-clinical-and-inflammatoryparameters-in-children-with-cystic-fibrosis
#20
Zeynep Seda Uyan, Göksenin Ünlügüzel Üstün, Goncagül Haklar, Erkan Çakır, Sedat Oktem, Refika Ersu, Bülent Taner Karadağ, Fazilet Karakoç, Elif Dağlı
Background/aim: The effectiveness of inhaled corticosteroids (ICSs) in cystic fibrosis (CF) is controversial. The aim of this study was to investigate the effect of an ICS on bronchial hyperreactivity (BHR), oxidative status, and clinical and inflammatory parameters in CF patients. Materials and methods: CF patients were randomized to receive either 2 mg/day nebulized budesonide or 0.9% normal saline as placebo for 8 weeks. Results: Twenty-nine CF patients (mean age: 10.5 ± 2.9 years) were enrolled in the study...
November 13, 2017: Turkish Journal of Medical Sciences
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