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interstitial pulmonary fibrosis

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https://www.readbyqxmd.com/read/28727155/mucosal-associated-invariant-mait-cells-are-deficient-in-systemic-sclerosis
#1
Arsène Mekinian, Thibaut Mahevas, Mohamad Mohty, Vincent Jachiet, Sébastien Rivière, Olivier Fain, Béatrice Gaugler
OBJECTIVES: Systemic sclerosis (SSc) is an autoimmune disease, characterized by fibrosis of the skin and other organs, vascular impairment and deficient immune responses. Mucosal associated invariant T cells (MAIT) cells have been involved in various inflammatory and autoimmune diseases. The aim of this study was to determine the frequencies of MAIT cells in the blood of patients with systemic sclerosis (SSc), and to compare their distribution in the different types of SSc. PATIENTS AND METHODS: Blood samples from SSc patients and healthy controls were examined by flow cytometer to analyze the frequencies of MAIT and γδ T cells...
July 18, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28712048/photobiomodulation-therapy-improves-both-inflammatory-and-fibrotic-parameters-in-experimental-model-of-lung-fibrosis-in-mice
#2
Robson Alexandre Brochetti, Mayara Peres Leal, Raíssa Rodrigues, Renata Kelly da Palma, Luis Vicente Franco de Oliveira, Anna Carolina Ratto Tempestini Horliana, Amílcar Sabino Damazo, Ana Paula Ligeiro de Oliveira, Rodolfo Paula Vieira, Adriana Lino-Dos-Santos-Franco
Lung fibrosis (LF) is a chronic and progressive lung disease characterized by pulmonary parenchyma progressive lesion, inflammatory infiltration, and interstitial fibrosis. It is developed by excessive collagen deposition and other cellular matrix components, resulting in severe changes in the alveolar architecture. Considering the absence of effective treatment, the aim of this study was to investigate the effect of photobiomodulation therapy (PBMT) on the development of PF. For this purpose, we used C57BL6 mice subjected to induction of LF by bleomycin administration (1...
July 16, 2017: Lasers in Medical Science
https://www.readbyqxmd.com/read/28705305/clinical-characteristics-of-japanese-candidates-for-lung-transplant-for-interstitial-lung-disease-and-risk-factors-for-early-death-while-on-the-waiting-list
#3
Hisao Higo, Takeshi Kurosaki, Eiki Ichihara, Toshio Kubo, Kentaroh Miyoshi, Shinji Otani, Seiichiro Sugimoto, Masaomi Yamane, Nobuaki Miyahara, Katsuyuki Kiura, Shinichiro Miyoshi, Takahiro Oto
BACKGROUND: Lung transplants have produced very favorable outcomes for patients with interstitial lung disease (ILD) in Japan. However, because of the severe donor lung shortage, patients must wait approximately 2.5 years before they can undergo transplantation and many candidates die before allocation. We reveal the clinical characteristics of Japanese patients with ILD who are candidates for lung transplants and the risk factors for early death while on the waiting list. METHODS: We retrospectively reviewed the clinical data of patients registered in the Japan Organ Transplant Network from Okayama University Hospital who are candidates for cadaveric lung transplants for ILD between 1999 and 2015...
July 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28704913/treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-the-pulmonologist-s-point-of-view
#4
REVIEW
So-My Koo, Soo-Taek Uh
Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be a significant cause of mortality in patients with CVD-associated ILD (CTD-ILD). There is a lack of randomized, clinical trials assessing pharmacological agents for CTD-ILD, except in cases of ILD-associated systemic sclerosis (SSc). This may be due to the lack of CTD cases available, the difficulty of histological confirmation of ILD, and the various types of CTD and ILD...
July 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28690232/-progress-in-sleep-disordered-breathing-in-idiopathic-pulmonary-fibrosis
#5
Ying Zhou, Runxu Song, Danbei Diwu, Yixin Wan
Idiopathic pulmonary fibrosis (IPF) is one of the most common idiopathic interstitial pneumonia. The main symptoms for IPF are dry cough and exertional shortness of breath, which is worsen gradually with the development of pulmonary fibrosis. Patients with IPF often suffer from sleep disordered breathing (SDB). Sleep architecture for these patients including the sleep efficiency, deep sleep, rapid eye movement sleep and arousal index, are changed, which seriously affects the quality of life. In the absence of effective therapies, optimizing the quality of life may become a major therapeutic target for IPF...
June 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28688290/the-efficacy-of-plant-extract-and-bioactive-compounds-approaches-in-the-treatment-of-pulmonary-fibrosis-a-systematic-review
#6
REVIEW
Sana Bahri, Ridha Ben Ali, Anouar Abidi, Saloua Jameleddine
Pulmonary fibrosis (PF) is a lethal, chronic and progressive respiratory disease leading to interstitial lung damage and serious breathing problems. The pathogenic mechanism involves activation, migration, proliferation and differentiation of fibroblasts into myofibroblats inducing extracellular matrix accumulation that destroy lung parenchyma. Available antifibrotic treatment options are limited to Pirfenidone and Nintedanib that prevent deterioration without an improvement of this disease. The use of plant extracts and natural bioactive compounds for the treatment of PF has been known for more than thirty years in China...
July 5, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28687910/ct-findings-associated-with-survival-in-chronic-hypersensitivity-pneumonitis
#7
Jonathan H Chung, Steven M Montner, Ayodeji Adegunsoye, Justin M Oldham, Aliya N Husain, Rekha Vij, Imre Noth, Mary E Strek
OBJECTIVES: To identify CT findings in chronic hypersensitivity pneumonitis (cHP) associated with survival. MATERIAL AND METHODS: Two thoracic radiologists assessed CT scans for specific imaging findings and patterns in 132 subjects with cHP. Survival analyses were performed. RESULTS: The majority of subjects had an inconsistent with usual interstitial pneumonitis pattern on CT (55.3%,73/132). Hypersensitivity pneumonitis (HP) diagnosis on CT was less common in those with fibrosis (66...
July 7, 2017: European Radiology
https://www.readbyqxmd.com/read/28679612/functional-and-prognostic-effects-when-emphysema-complicates-idiopathic-pulmonary-fibrosis
#8
Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Maria Kokosi, Toby M Maher, Arjun Nair, Ronald Karwoski, Elisabetta Renzoni, Simon L F Walsh, David M Hansell, Athol U Wells
This study aimed to investigate whether the combination of fibrosis and emphysema has a greater effect than the sum of its parts on functional indices and outcome in idiopathic pulmonary fibrosis (IPF), using visual and computer-based (CALIPER) computed tomography (CT) analysis.Consecutive patients (n=272) with a multidisciplinary IPF diagnosis had the extent of interstitial lung disease (ILD) scored visually and by CALIPER. Visually scored emphysema was subcategorised as isolated or mixed with fibrotic lung...
July 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28673104/biopsy-in-idiopathic-pulmonary-fibrosis-back-to-the-future
#9
Giulio Rossi, Paolo Spagnolo
Idiopathic Pulmonary Fibrosis (IPF) is a relentlessly progressive, fibrosing interstitial pneumonia characterized by a radiologic and/or histologic pattern of usual interstitial pneumonia (UIP). The availability of two effective anti-fibrotic drugs in IPF has encouraged the identification and treatment of patients in early stages in order to maximize clinical benefit. The ability of high-resolution computed tomography (HRCT) to identify a 'definite' UIP pattern is suboptimal, particularly in the absence of honeycombing...
July 10, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28667660/signaling-pathways-and-their-mirna-regulators-involved-in-the-etiopathology-of-idiopathic-pulmonary-fibrosis-ipf-and-hypersensitivity-pneumonitis-hp
#10
Justyna Kiszałkiewicz, Wojciech Piotrowski, Ewa Brzeziańska-Lasota
Idiopathic pulmonary fibrosis (IPF) and hypersensitivity pneumonitis (HP) belong to heterogenic group of interstitial lung diseases (ILD). For the reason that this group of diseases present with complex clinical non-specific features, they represent a diagnostic and therapeutic challenge. In this review we focus on several crucial signaling pathways participating in inflammation, fibrosis and EMT processes, so important in the course of ILD: TNF-α/NFκβ, TGF-β/SMAD, Wnt-β-catenin and PI3K-Akt signaling...
2017: Advances in Respiratory Medicine
https://www.readbyqxmd.com/read/28667087/development-of-tools-to-facilitate-palliative-and-supportive-care-referral-for-patients-with-idiopathic-pulmonary-fibrosis
#11
Charles Sharp, Heather Lamb, Nikki Jordan, Adrienne Edwards, Rachel Gunary, Patricia Meek, Ann B Millar, Clare Kendall, Huzaifa Adamali
OBJECTIVES: Palliative care is underused in non-malignant respiratory diseases, including interstitial lung diseases (ILDs). We investigated current practices around palliative and supportive care and explored the impact of a supportive care decision aid tool. METHODS: This was a single centre study in a UK ILD centre. Retrospective analysis of hospice referrals and patients with idiopathic pulmonary fibrosis (IPF) under the Bristol ILD (BILD) service were used to identify unmet palliative and supportive care needs...
June 30, 2017: BMJ Supportive & Palliative Care
https://www.readbyqxmd.com/read/28666014/radiologic-pleuroparenchymal-fibroelastosis-like-lesion-in-connective-tissue-disease-related-interstitial-lung-disease
#12
Yasunori Enomoto, Yutaro Nakamura, Thomas V Colby, Takeshi Johkoh, Hiromitsu Sumikawa, Koji Nishimoto, Katsuhiro Yoshimura, Sayomi Matsushima, Yoshiyuki Oyama, Hironao Hozumi, Masato Kono, Tomoyuki Fujisawa, Noriyuki Enomoto, Naoki Inui, Toshihide Iwashita, Takafumi Suda
BACKGROUND: Radiologic pleuroparenchymal fibroelastosis (PPFE)-like lesion including pulmonary apical cap can be occasionally observed in clinical settings. However, the significance of radiologic PPFE-like lesion is unclear in connective tissue disease (CTD)-related interstitial lung disease (ILD). MATERIALS AND METHODS: A total of 113 patients with CTD-related ILD were enrolled and assessed for radiologic PPFE-like lesion, which was defined as bilateral, upper lobe, and subpleural dense consolidations with or without pleural thickening on chest high-resolution computed tomography...
2017: PloS One
https://www.readbyqxmd.com/read/28664834/design-of-a-randomised-placebo-controlled-clinical-trial-of-nintedanib-in-patients-with-systemic-sclerosis-associated-interstitial-lung-disease-senscis%C3%A2
#13
Oliver Distler, Kevin K Brown, Jörg H W Distler, Shervin Assassi, Toby M Maher, Vincent Cottin, John Varga, Carl Coeck, Martina Gahlemann, Wiebke Sauter, Hendrik Schmidt, Kristin B Highland
OBJECTIVES: Nintedanib is a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis (IPF). The pathological pathways involved in fibrogenesis in IPF and interstitial lung disease associated with systemic sclerosis (SSc-ILD) show commonalities; both involve fibroblast activation, myofibroblast accumulation and deposition of extracellular matrix. The SENSCIS™ trial is a randomised, placebo-controlled Phase III trial that will evaluate the efficacy and safety of nintedanib in patients with SSc-ILD (NCT02597933)...
June 29, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28654764/deep-proteome-profiling-reveals-common-prevalence-of-mzb1-positive-plasma-b-cells-in-human-lung-and-skin-fibrosis
#14
Herbert B Schiller, Christoph H Mayr, Gabriela Leuschner, Maximilian Strunz, Claudia Staab-Weijnitz, Stefan Preisendörfer, Beate Eckes, Pia Moinzadeh, Thomas Krieg, David A Schwartz, Rudolf A Hatz, Jürgen Behr, Matthias Mann, Oliver Eickelberg
RATIONALE: Analyzing the molecular heterogeneity of different forms of organ fibrosis may reveal common and specific factors and thus identify potential future therapeutic targets. OBJECTIVES: We sought to use proteome-wide profiling of human tissue fibrosis to (1) identify common and specific signatures across endstage interstitial lung disease (ILD) cases, (2) characterize ILD subgroups in an unbiased fashion, and (3) identify common and specific features of lung and skin fibrosis...
June 27, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28652456/lung-cancer-in-patients-with-severe-idiopathic-pulmonary-fibrosis-critical-aspects
#15
Elena Bargagli, Viola Bonti, Katia Ferrari, Elisabetta Rosi, Alessandra Bindi, Maurizio Bartolucci, Moroni Chiara, Luca Voltolini
Idiopathic pulmonary fibrosis (IPF) is a rare interstitial lung disease limited to the lung with an undefined etiopathogenesis and a very short life expectancy (less than 5 years). IPF susceptibility has been associated with several genetic and environmental risk factors and the prognosis is conditioned by comorbidities such as gastro-esophageal reflux, depression, venous thromboembolism, pulmonary hypertension and lung cancer. At 5 years follow-up, 15% of IPF patients develop lung cancer, which can significantly reduce their survival...
July 2017: In Vivo
https://www.readbyqxmd.com/read/28650861/microbiome-in-interstitial-lung-disease-from-pathogenesis-to-treatment-target
#16
Margaret L Salisbury, MeiLan K Han, Robert P Dickson, Philip L Molyneaux
PURPOSE OF REVIEW: This review summarizes current knowledge of the role of the lung microbiome in interstitial lung disease and poses considerations of the microbiome as a therapeutic target. RECENT FINDINGS: Although historically considered sterile, bacterial communities have now been well documented in lungs in health and disease. Studies in idiopathic pulmonary fibrosis (IPF) suggest that increased bacterial burden and/or abundance of potentially pathogenic bacteria may drive disease progression, acute exacerbations, and mortality...
June 24, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28648751/the-muc5b-promoter-polymorphism-and-telomere-length-in-patients-with-chronic-hypersensitivity-pneumonitis-an-observational-cohort-control-study
#17
Brett Ley, Chad A Newton, Isabel Arnould, Brett M Elicker, Travis S Henry, Eric Vittinghoff, Jeffrey A Golden, Kirk D Jones, Kiran Batra, Jose Torrealba, Christine Kim Garcia, Paul J Wolters
BACKGROUND: Patients with hypersensitivity pneumonitis are at risk of developing pulmonary fibrosis, which is associated with reduced survival. In families with multiple affected members, individuals might be diagnosed as having idiopathic pulmonary fibrosis (IPF) or chronic (fibrotic) hypersensitivity pneumonitis, which suggests these disorders share risk factors. We aimed to test whether the genomic risk factors associated with the development and progression of IPF are also associated with the development of fibrosis and reduced survival in people with chronic hypersensitivity pneumonitis...
June 22, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28646122/nintedanib-a-triple-tyrosine-kinase-inhibitor-attenuates-renal-fibrosis-in-chronic-kidney-disease
#18
Feng Liu, Li Wang, Hualin Qi, Jun Wang, Wei Jiang, Yi Wang, Liuqing Xu, Na Liu, Shougang Zhuang
Nintedanib (BIBF1120) is a triple kinase inhibitor of platelet derived growth factor receptor (PDGFR), fibroblast growth factor receptors (FGFR), vascular endothelial growth factor receptor (VEGFR) and Src family kinase, that has recently been approved by FDA to treat idiopathic pulmonary fibrosis. Whether it affects renal fibrosis remains unknown. Here we demonstrated that administration of nintedanib immediately or 3 days after unilateral ureteral obstruction (UUO) injury and with folic acid injection attenuated renal fibrosis and inhibited activation of renal interstitial fibroblasts...
June 23, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28642621/variants-of-the-abca3-gene-might-contribute-to-susceptibility-to-interstitial-lung-diseases-in-the-chinese-population
#19
Wei Zhou, Yi Zhuang, Jiapeng Sun, Xiaofen Wang, Qingya Zhao, Lizhi Xu, Yaping Wang
ATP-binding cassette A3 (ABCA3) is a phospholipid carrier that is mainly expressed in the alveolar epithelium. Biallelic mutations of ABCA3 has been associated with fatal respiratory distress syndrome and interstitial lung disease (ILD) in children. However, whether variations in ABCA3 have a role in the development of adult ILD, including idiopathic pulmonary fibrosis (IPF), remains to be addressed. In this study, we screened for germline variants of ABCA3 by exons-sequencing in 30 patients with sporadic IPF and in 30 matched healthy controls...
June 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28642129/fucoxanthin-inhibits-profibrotic-protein-expression-in-vitro-and-attenuates-bleomycin-induced-lung-fibrosis-in-vivo
#20
Sun Young Ma, Won Sun Park, Dae-Sung Lee, Grace Choi, Mi-Jin Yim, Jeong Min Lee, Won-Kyo Jung, Sae Gwang Park, Su-Kil Seo, Sung Jae Park, Il Yong Han, Yung Hyun Choi, Il-Whan Choi
Pulmonary fibrosis, a potentially fatal disease, results from acute and chronic interstitial lung diseases. Fucoxanthin (Fx), a carotenoid found in brown seaweed, shows a wide range of pharmacological activities. In this study, we investigated the antifibrotic effects of fucoxanthin and their underlying molecular mechanisms in transforming growth factor-beta1 (TGF-β1)-stimulated human pulmonary fibroblasts (HPFs). Thus, the effects of Fx on TGF-β1-induced expression of fibrotic factors, such as alpha-smooth muscle actin (α-SMA), type 1 collagen, fibronectin, and interleukin-6 (IL-6), in HPFs were investigated...
June 19, 2017: European Journal of Pharmacology
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