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interstitial pulmonary fibrosis

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https://www.readbyqxmd.com/read/28431248/spontaneous-chitin-accumulation-in-airways-and-age-related-fibrotic-lung-disease
#1
Steven J Van Dyken, Hong-Erh Liang, Ram P Naikawadi, Prescott G Woodruff, Paul J Wolters, David J Erle, Richard M Locksley
The environmentally widespread polysaccharide chitin is degraded and recycled by ubiquitous bacterial and fungal chitinases. Although vertebrates express active chitinases from evolutionarily conserved loci, their role in mammalian physiology is unclear. We show that distinct lung epithelial cells secrete acidic mammalian chitinase (AMCase), which is required for airway chitinase activity. AMCase-deficient mice exhibit premature morbidity and mortality, concomitant with accumulation of environmentally derived chitin polymers in the airways and expression of pro-fibrotic cytokines...
April 20, 2017: Cell
https://www.readbyqxmd.com/read/28426895/short-term-pulmonary-function-trends-are-predictive-of-mortality-in-interstitial-lung-disease-associated-with-systemic-sclerosis
#2
Nicole S Goh, Rachel K Hoyles, Christopher P Denton, David M Hansell, Elisabetta A Renzoni, Toby M Maher, Andrew G Nicholson, Athol U Wells
OBJECTIVE: To determine the prognostic value of pulmonary function test (PFT) trends at one and two years in interstitial lung disease associated with systemic sclerosis (SSc-ILD). METHODS: The prognostic significance of PFT trends at one year (n=162), and two years (n=140) was examined against 15 year survival. PFT trends, expressed as continuous and categorical change in separate analyses, were examined against mortality in univariate and multivariate models. SSc-ILD was defined at presentation as limited lung fibrosis or extensive lung fibrosis using the UKRSA staging system...
April 20, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28408734/mycophenolate-mofetil-and-pulmonary-fibrosis-after-kidney-transplantation-a-case-report
#3
Kazuhiro Takahashi, Pauline Go, Chad H Stone, Mohamed Safwan, Krishna G Putchakayala, William J Kane, Lauren E Malinzak, Dean Y Kim, Jason E Denny
BACKGROUND Mycophenolate mofetil (MMF) induced lung disease has been described in only a few isolated reports. We report a case of fatal respiratory failure associated with MMF after kidney transplantation. CASE REPORT A 50-year-old Hispanic male with a history of end-stage renal disease secondary to hypertension underwent deceased donor kidney transplantation. His preoperative evaluations were normal except for a chest x-ray which showed bilateral interstitial opacities. Tacrolimus and MMF were started on the day of surgery...
April 14, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28400116/the-role-of-infection-in-interstitial-lung-diseases-a-review
#4
REVIEW
Natalya Azadeh, Andrew H Limper, Eva M Carmona, Jay H Ryu
Interstitial lung diseases (ILD) comprise an array of heterogeneous parenchymal lung diseases that are associated with a spectrum of pathologic, radiologic and clinical manifestations. There are ILDs with known etiologies and those that are idiopathic, making treatment strategies challenging. Prognosis can vary according to the type of ILD but many exhibit gradual progression with an unpredictable clinical course in individual patients as seen in idiopathic pulmonary fibrosis and the phenomenon of "acute exacerbation"(AE)...
April 8, 2017: Chest
https://www.readbyqxmd.com/read/28385812/sirtuin-7-is-decreased-in-pulmonary-fibrosis-and-regulates-the-fibrotic-phenotype-of-lung-fibroblasts
#5
Anne Elizabeth Wyman, Zahid Noor, Rita Fishelevich, Virginia Lockatell, Nirav G Shah, Nevins W Todd, Sergei P Atamas
Pulmonary fibrosis is a severe condition with no cure and limited therapeutic options. Better understanding of its pathophysiology is needed. Recent studies have suggested that pulmonary fibrosis may be driven by accelerated aging-related mechanisms. Sirtuins (SIRTs), particularly SIRT1, -3, and -6, are well-known mediators of aging, however limited data exist on the contribution of sirtuins to lung fibrosis. We assessed the mRNA and protein levels of all seven known sirtuins in primary lung fibroblasts from patients with idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in comparison with lung fibroblasts from healthy controls...
April 6, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28382694/quantitative-analysis-of-hyperpolarized-129-xe-gas-transfer-mri
#6
Ziyi Wang, Scott Haile Robertson, Jennifer Wang, Mu He, Rohan S Virgincar, Geoffry M Schrank, Elianna A Bier, Sudarshan Rajagopal, Yuh Chin Huang, Thomas G O'Riordan, Craig R Rackley, H Page McAdams, Bastiaan Driehuys
PURPOSE: Hyperpolarized (129) Xe magnetic resonance imaging (MRI) using Dixon-based decomposition enables single-breath imaging of (129) Xe in the airspaces, interstitial barrier tissues, and red blood cells (RBCs). However, methods to quantitatively visualize information from these images of pulmonary gas transfer are lacking. Here, we introduce a novel method to transform these data into quantitative maps of pulmonary ventilation, and (129) Xe gas transfer to barrier and RBC compartments...
April 6, 2017: Medical Physics
https://www.readbyqxmd.com/read/28370144/lung-function-not-affected-by-asbestos-exposure-in-workers-with-normal-computed-tomography-scan
#7
Christian Schikowsky, Michael K Felten, Christian Eisenhawer, Marco Das, Thomas Kraus
BACKGROUND: It has been suggested that asbestos exposure affects lung function, even in the absence of asbestos-related pulmonary interstitial or pleural changes or emphysema. METHODS: We analyzed associations between well-known asbestos-related risk factors, such as individual cumulative asbestos exposure, and key lung function parameters in formerly asbestos-exposed power industry workers (N = 207) with normal CT scans. For this, we excluded participants with emphysema, fibrosis, pleural changes, or any combination of these...
March 30, 2017: American Journal of Industrial Medicine
https://www.readbyqxmd.com/read/28356570/idiopathic-pulmonary-fibrosis-and-a-role-for-autoimmunity
#8
REVIEW
Gerard F Hoyne, Hannah Elliott, Steven E Mutsaers, Cecilia M Prêle
Idiopathic Pulmonary Fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is typically associated with extensive and progressive fibrosis, is fatal and has limited treatment options. Characteristically IPF patients display large lymphocyte aggregates composed of CD3(+) T cells and CD20(+) B cells within the lung tissue that are located near sites of active fibrosis. In addition, IPF patients can have autoantibodies to a range of host antigens, suggesting a breakdown in immunological tolerance...
March 30, 2017: Immunology and Cell Biology
https://www.readbyqxmd.com/read/28356370/comorbidities-in-the-management-of-patients-with-lung-cancer
#9
Charlotte Leduc, Delphine Antoni, Anne Charloux, Pierre-Emmanuel Falcoz, Elisabeth Quoix
Lung cancer represents a major public health issue worldwide. Unfortunately, more than half of them are diagnosed at an advanced stage. Moreover, even if diagnosed early, diagnosis procedures and treatment can be difficult due to the frequent comorbidities observed in these patients. Some of these comorbidities have a common major risk factor, i.e. smoking, whereas others are unrelated to smoking but frequently observed in the general population. These comorbidities must be carefully assessed before any diagnostic and/or therapeutic decisions are made regarding the lung cancer...
March 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28345383/pulmonary-fibrosis-part-i-epidemiology-pathogenesis-and-diagnosis
#10
Keith C Meyer
Many forms of interstitial lung disease (ILD) can progress to extensive fibrosis and respiratory failure. Idiopathic pulmonary fibrosis (IPF), which generally has a poor prognosis, has been thoroughly studied over the past two decades, and many important discoveries have been made that pertain to genetic predisposition, epidemiology, disease pathogenesis, diagnosis, and management. Additionally, non-IPF forms of ILD can have radiologic and histopathologic manifestations that mimic IPF, and making an accurate diagnosis is key to providing personalized medicine to patients with pulmonary fibrosis...
May 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28345369/pulmonary-fibrosis-part-ii-state-of-the-art-patient-management
#11
Keith C Meyer
While many pharmacologic therapies for the treatment of idiopathic pulmonary fibrosis (IPF) have been evaluated via randomized, placebo-controlled clinical trials (RCTs) conducted over the past two decades, most therapies have been shown to be ineffective or even potentially harmful. However, a number of recently completed RCTs have shown significant efficacy for pirfenidone and nintedanib for the treatment of IPF. Areas covered: This manuscript reviews recent advances in the management of IPF and other forms of fibrosing interstitial lung disease (ILD) with an emphasis on IPF...
May 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28343230/idiopathic-pulmonary-fibrosis-in-switzerland-diagnosis-and-treatment
#12
Manuela Funke-Chambour, Andrea Azzola, Dan Adler, Constance Barazzone-Argiroffo, Christian Benden, Annette Boehler, Pierre-Olivier Bridevaux, Martin Brutsche, Christian F Clarenbach, Katrin Hostettler, Rebekka Kleiner-Finger, Laurent P Nicod, Paola M Soccal, Michael Tamm, Thomas Geiser, Romain Lazor
Idiopathic pulmonary fibrosis (IPF) is a severe progressive and irreversible lung disease. Novel antifibrotic drugs that slow disease progression are now available. However, many issues regarding patient management remain unanswered, such as the choice between available drugs, their use in particular subgroups and clinical situations, time of treatment onset, termination, combination or switch, or nonpharmacologic management. To guide Swiss respiratory physicians in this evolving field still characterized by numerous areas of uncertainty, the Swiss Working Group for interstitial and rare lung diseases of the Swiss Respiratory Society provides a position paper on the diagnosis and treatment of IPF...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28339739/unabated-occupational-risk-in-a-patient-with-rheumatoid-pulmonary-fibrosis
#13
V K Holden, S J Kligerman, T G Hastings, S E Hines
Background: This case highlights the importance of considering hypersensitivity pneumonitis (HP) in the differential diagnosis of interstitial lung disease (ILD) and of obtaining an occupational history so that remediable risk factors may be identified and managed. Aims: To report a case of a chicken sexer with severe rheumatoid arthritis (RA) who developed progressively worsening dyspnoea and restrictive lung disease associated with pulmonary fibrosis. Methods: Clinical investigation included physical examination, occupational history, pulmonary function tests (PFTs), chest imaging and bronchoalveolar lavage (BAL), as well as serological tests including standard IgE bird feather mixture and local IgG precipitin preparation to chicken excrement...
February 23, 2017: Occupational Medicine
https://www.readbyqxmd.com/read/28339528/the-utility-of-comprehensive-autoantibody-testing-to-differentiate-connective-tissue-disease-associated-and-idiopathic-interstitial-lung-disease-subgroup-cases
#14
Caroline V Cotton, Lisa G Spencer, Robert P New, Robert G Cooper
Interstitial lung disease (ILD) comprises many heterogeneous disease groups, the largest being CTD-associated and those labelled as idiopathic out of necessity. The mechanisms causing ILD are poorly understood, but most CTD- and idiopathic-ILD cases can respond to immunosuppression, clearly suggesting a pathological role for inflammation. By contrast, corticosteroid immunosuppression causes harm without benefit in the feared idiopathic pulmonary fibrosis, suggesting that inflammation plays little pathological role, and where ILD progresses rapidly to lethal outcome even with anti-fibrotic drug use...
October 20, 2016: Rheumatology
https://www.readbyqxmd.com/read/28335088/further-characterization-of-computed-tomographic-and-clinical-features-for-staging-and-prognosis-of-idiopathic-pulmonary-fibrosis-in-west-highland-white-terriers
#15
Florence Thierry, Ian Handel, Gawain Hammond, Lesley G King, Brendan M Corcoran, Tobias Schwarz
Idiopathic pulmonary fibrosis is an interstitial lung disease of unknown etiology resulting in progressive interstitial fibrosis, with a known predilection in West Highland white terriers. In humans, computed tomography (CT) is a standard method for providing diagnostic and prognostic information, and plays a major role in the idiopathic pulmonary fibrosis staging process. Objectives of this retrospective, analytical, cross-sectional study were to establish descriptive criteria for reporting CT findings and test correlations among CT, clinical findings and survival time in West Highland white terriers with idiopathic pulmonary fibrosis...
March 23, 2017: Veterinary Radiology & Ultrasound
https://www.readbyqxmd.com/read/28326312/a-comparison-of-published-multidimensional-indices-to-predict-outcome-in-idiopathic-pulmonary-fibrosis
#16
Charles Sharp, Huzaifa I Adamali, Ann B Millar
Idiopathic pulmonary fibrosis (IPF) has an unpredictable course and prognostic factors are incompletely understood. We aimed to identify prognostic factors, including multidimensional indices from a significant IPF cohort at the Bristol Interstitial Lung Disease Centre in the UK. Patients diagnosed with IPF between 2007 and 2014 were identified. Longitudinal pulmonary physiology and exercise testing results were collated, with all-cause mortality used as the primary outcome. Factors influencing overall, 12- and 24-month survival were identified using Cox proportional hazards modelling and receiver operating characteristic curve analysis...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28326180/are-physicians-in-primary-health-care-able-to-recognize-pulmonary-fibrosis
#17
Minna Purokivi, Ulla Hodgson, Marjukka Myllärniemi, Eija-Riitta Salomaa, Riitta Kaarteenaho
Background: The early diagnosis of idiopathic pulmonary fibrosis (IPF) has become increasingly important due to evolving treatment options. IPF patients experience a significant delay in receiving an accurate diagnosis, thus delayed access to tertiary care is associated with higher mortality independently from disease severity. Objective: The aims were to evaluate whether there had been a delay in the referral process, and to determine whether the referring doctors had suspected IPF or other interstitial lung disease (ILD) already during the time of referral...
2017: European Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28325283/silencing-of-carbohydrate-sulfotransferase-15-hinders-murine-pulmonary-fibrosis-development
#18
Yoshiro Kai, Koichi Tomoda, Hiroyuki Yoneyama, Masahiro Kitabatake, Atsuhiro Nakamura, Toshihiro Ito, Masanori Yoshikawa, Hiroshi Kimura
Pulmonary fibrosis is a progressive lung disorder characterized by interstitial fibrosis, for which no effective treatments are available. Chondroitin sulfate proteoglycan (CSPG) has been shown to be a mediator, but the specific component of glycosaminoglycan chains of CSPG has not been explored. We show that chondroitin sulfate E-type (CS-E) is involved in fibrogenesis. Small interfering RNA (siRNA) targeting carbohydrate sulfotransferase 15 (CHST15) was designed to inhibit CHST15 mRNA and its product, CS-E...
March 17, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28323056/fty720-promotes-pulmonary-fibrosis-when-administered-during-the-remodelling-phase-following-a-bleomycin-induced-lung-injury
#19
David R Gendron, Anne-Marie Lemay, Pascale Blais Lecours, Valérie Perreault-Vallières, Carole-Ann Huppé, Ynuk Bossé, Marie-Renée Blanchet, Geneviève Dion, David Marsolais
Fibrosis complicates numerous pathologies including interstitial lung diseases. Sphingosine analogs such as FTY720 can alleviate lung injury-induced fibrosis in murine models. Contradictorily, FTY720 also promotes in vitro processes normally leading to fibrosis and high doses in vivo foster lung fibrosis by enhancing vascular leakage into the lung. The goal of this study was to determine the effect of low doses of FTY720 on lung fibrosis triggered by an acute injury in mice. We first defined the time-boundaries delimiting the inflammatory and remodelling phases of an injury elicited by bleomycin based on neutrophil counts, total lung capacity and lung stiffness...
March 16, 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28322466/the-role-of-mir-497-eif3a-axis-in-tgf%C3%AE-1-induced-epithelial-mesenchymal-transition-and-extracellular-matrix-in-rat-alveolar-epithelial-cells-and-pulmonary-fibroblasts
#20
Ren Guo, Yu Lv, Yang Ouyang, Siyu Liu, Dai Li
Multi-cause-induced interstitial lung disease, particularly pulmonary fibrosis, is a serious clinical concern. fibroblasts have been suggested to have a major role, with it recently being revealed that some of these fibroblasts are derived from alveolar epithelial cells through epithelial-mesenchymal transition (EMT). Eukaryotic translation initiation factor 3 subunit A (EIF3A) is a protein that in humans is encoded by the EIF3A gene, and has been suggested to play roles in regulating translation of a subset of mRNAs and in regulating cell cycle progression and cell proliferation...
March 21, 2017: Journal of Cellular Biochemistry
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