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https://www.readbyqxmd.com/read/28339994/a-longitudinal-cohort-study-of-the-anti-synthetase-syndrome-increased-severity-of-interstitial-lung-disease-in-black-patients-and-patients-with-anti-pl7-and-anti-pl12-autoantibodies
#1
Iago Pinal-Fernandez, Maria Casal-Dominguez, Julio A Huapaya, Jemima Albayda, Julie J Paik, Cheilonda Johnson, Leann Silhan, Lisa Christopher-Stine, Andrew L Mammen, Sonye K Danoff
Objective.: The aim was to study the prevalence, rate of appearance and severity of clinical features in patients with different anti-synthetase syndrome (ASyS) autoantibodies. Methods.: All Johns Hopkins Myositis Longitudinal Cohort subjects positive for any ASyS autoantibodies were included. Clinical information, including symptoms, signs, strength, creatine kinase concentrations and pulmonary function tests, were prospectively collected. The standardized mortality and cancer rates and the rate of appearance and intensity of the different organ manifestations were assessed using univariate and multivariate analysis and compared between ASyS autoantibodies...
March 4, 2017: Rheumatology
https://www.readbyqxmd.com/read/28339739/unabated-occupational-risk-in-a-patient-with-rheumatoid-pulmonary-fibrosis
#2
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/28326180/are-physicians-in-primary-health-care-able-to-recognize-pulmonary-fibrosis
#3
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/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
#4
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
https://www.readbyqxmd.com/read/28321059/arterial-carboxyhemoglobin-measurement-is-useful-for-evaluating-pulmonary-inflammation-in-subjects-with-interstitial-lung-disease
#5
Yu Hara, Masaharu Shinkai, Soichiro Kanoh, Yuji Fujikura, Bruce K Rubin, Akihiko Kawana, Takeshi Kaneko
Objective The arterial concentration of carboxyhemoglobin (CO-Hb) in subjects with inflammatory pulmonary disease is higher than that in healthy individuals. We retrospectively analyzed the relationship between the CO-Hb concentration and established markers of disease severity in subjects with interstitial lung disease (ILD). Methods The CO-Hb concentration was measured in subjects with newly diagnosed or untreated ILD and the relationships between the CO-Hb concentration and the serum biomarker levels, lung function, high-resolution CT (HRCT) findings, and the uptake in gallium-67 ((67)Ga) scintigraphy were evaluated...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28314688/nivolumab-treatment-for-oesophageal-squamous-cell-carcinoma-an-open-label-multicentre-phase-2-trial
#6
Toshihiro Kudo, Yasuo Hamamoto, Ken Kato, Takashi Ura, Takashi Kojima, Takahiro Tsushima, Shuichi Hironaka, Hiroki Hara, Taroh Satoh, Satoru Iwasa, Kei Muro, Hirofumi Yasui, Keiko Minashi, Kensei Yamaguchi, Atsushi Ohtsu, Yuichiro Doki, Yuko Kitagawa
BACKGROUND: Nivolumab is a human monoclonal IgG4 antibody that inhibits programmed cell death protein 1 (PD-1) expressed on activated T cells. We investigated the safety and activity of nivolumab in patients with treatment-refractory oesophageal cancer. METHODS: We did an open-label, single-arm, multicentre phase 2 study. Eligible patients had advanced squamous-cell carcinoma, adenosquamous-cell carcinoma, or adenocarcinoma of the oesophagus refractory or intolerant to fluoropyrimidine-based, platinum-based, and taxane-based chemotherapy...
March 14, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28302503/the-giessen-pulmonary-hypertension-registry-survival-in-pulmonary-hypertension-subgroups
#7
Henning Gall, Janine F Felix, Franziska K Schneck, Katrin Milger, Natascha Sommer, Robert Voswinckel, Oscar H Franco, Albert Hofman, Ralph T Schermuly, Norbert Weissmann, Friedrich Grimminger, Werner Seeger, Hossein A Ghofrani
BACKGROUND: Pulmonary hypertension (PH) is a severe, progressive disease. Although 5 PH subgroups are recognized, reports on survival have focused mainly on pulmonary arterial hypertension (PAH). METHODS: Long-term transplant-free survival and its determinants were investigated in patients with PH (diagnosed by right heart catheterization) within a prospective registry at a single referral center in Giessen, Germany. RESULTS: In total, 2,067 patients were enrolled (PAH, 685 patients [33...
February 17, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28300665/grape-seed-extract-ameliorates-bleomycin-induced-mouse-pulmonary-fibrosis
#8
Qi Liu, Jun-Xia Jiang, Ya-Nan Liu, Yan Guan, Wei Zhao, Yong-Liang Jia, Xin-Wei Dong, Yun Sun, Qiang-Min Xie
Pulmonary fibrosis is common in a variety of inflammatory lung diseases, such as interstitial pneumonia, chronic obstructive pulmonary disease, and silicosis. There is currently no effective clinical drug treatment. It has been reported that grape seed extracts (GSE) has extensive pharmacological effects with minimal toxicity. Although it has been found that GSE can improve the lung collagen deposition and fibrosis pathology induced by bleomycin in rat, its effects on pulmonary function, inflammation, growth factors, matrix metalloproteinases and epithelial-mesenchymal transition remain to be researched...
March 11, 2017: Toxicology Letters
https://www.readbyqxmd.com/read/28300570/idiopathic-interstitial-pneumonia-associated-with-autoantibodies-a-large-case-series-followed-over-one-year
#9
Bridget F Collins, Charles F Spiekerman, Megan A Shaw, Lawrence A Ho, Jennifer Hayes, Carolyn A Spada, Caroline M Stamato, Ganesh Raghu
BACKGROUND: Some patients with autoimmune characteristics and idiopathic interstitial pneumonia (IIP), particularly usual interstitial pneumonia (UIP), do not fit neatly into the category of connective tissue disease associated interstitial lung disease (CTD-ILD), idiopathic pulmonary fibrosis (IPF) or recently proposed yet to be validated criteria for interstitial pneumonia with autoimmune features (IPAF). Outcomes of these patients are unknown. METHODS: Retrospective single center study; ANOVA analyses compared differences in mean change in forced vital capacity (FVC) and diffusion capacity (DLCO) over 1-year among 124 well defined patients (20 AI-ILD [positive autoantibodies with or without symptoms of CTD], 15 IPAF, 36 CTD-ILD, 53 "Lone-IPF" [patients with IPF without any autoantibodies]) RESULTS: 75% of patients with AI-ILD, 33% IPAF, and 33% CTD-ILD had UIP...
March 11, 2017: Chest
https://www.readbyqxmd.com/read/28298912/heterologous-matrix-metalloproteinase-gene-promoter-activity-allows-in-vivo-real-time-imaging-of-bleomycin-induced-lung-fibrosis-in-transiently-transgenized-mice
#10
Fabio Franco Stellari, Francesca Ruscitti, Daniela Pompilio, Francesca Ravanetti, Giulia Tebaldi, Francesca Macchi, Andrea Elizabeth Verna, Gino Villetti, Gaetano Donofrio
Idiopathic pulmonary fibrosis is a very common interstitial lung disease derived from chronic inflammatory insults, characterized by massive scar tissue deposition that causes the progressive loss of lung function and subsequent death for respiratory failure. Bleomycin is used as the standard agent to induce experimental pulmonary fibrosis in animal models for the study of its pathogenesis. However, to visualize the establishment of lung fibrosis after treatment, the animal sacrifice is necessary. Thus, the aim of this study was to avoid this limitation by using an innovative approach based on a double bleomycin treatment protocol, along with the in vivo images analysis of bleomycin-treated mice...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28289977/location-function-and-ontogeny-of-pulmonary-macrophages-during-the-steady-state
#11
REVIEW
Natalio Garbi, Bart N Lambrecht
The lung is continuously exposed to potentially hazardous environmental challenges in the form of inert material and microbes. Pulmonary macrophages are critical in maintaining a low inflammatory context in the lung to facilitate optimal gas exchange. During infection, however, they mediate the immediate response to invading microorganisms in coordination with epithelial cells and other tissue-resident immune cells including dendritic cells, innate lymphocytes and memory T cells, and pulmonary interstitial macrophages...
March 13, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28282243/differences-in-right-ventricular-functional-changes-during-treatment-between-systemic-sclerosis-associated-pulmonary-arterial-hypertension-and-idiopathic-pulmonary-arterial-hypertension
#12
Rahul G Argula, Abhijit Karwa, Abigail Lauer, David Gregg, Richard M Silver, Carol Feghali-Bostwick, Lynn M Schnapp, Kim Egbert, Bruce W Usher, Viswanathan Ramakrishnan, Paul M Hassoun, Charlie Strange
RATIONALE: Patients with scleroderma associated pulmonary arterial hypertension (SSc-PAH) continue to have an unacceptably high mortality despite the progress achieved with pulmonary arterial vasodilator therapies. OBJECTIVES: We sought to determine whether SSc-PAH is a clinically distinct pulmonary vascular disease phenotype when compared to IPAH, based on the progression of echocardiographic right ventricular (RV) dysfunction. METHODS: Retrospective analysis of echocardiographic data in 13 SSc-PAH and 11 IPAH patients was used to delineate the progression of RV dysfunction during single or combination pulmonary arterial vasodilator therapy...
March 10, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28277870/safety-and-effectiveness-of-tadalafil-in-patients-with-pulmonary-arterial-hypertension-japan-post-marketing-surveillance-data
#13
Hiroyoshi Yamazaki, Noriko Kobayashi, Masanori Taketsuna, Koyuki Tajima, Masahiro Murakami
OBJECTIVE: To evaluate the long-term safety and effectiveness of tadalafil in patients with pulmonary arterial hypertension (PAH) in real-world clinical practice. METHODS: This prospective, multicenter, noninterventional, post-marketing surveillance included patients with PAH who were observed for up to 2 years after initiation of tadalafil. Safety was assessed by analyzing the frequency of adverse drug reactions (ADRs), discontinuations due to adverse events (AEs), and serious adverse drug reactions (SADRs)...
February 21, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28275536/rheumatoid-arthritis-associated-pulmonary-hypertension-clinical-challenges-reflecting-the-diversity-of-pathophysiology
#14
Evangelia Panagiotidou, Evdokia Sourla, Serafim Xrisovalantis Kotoulas, Sofia Akritidou, Vasileios Bikos, Vasileios Bagalas, Ioannis Stanopoulos, Georgia Pitsiou
The present article reports three clinical cases in order to elucidate the diversity of the pathophysiological mechanisms that underlie rheumatoid arthritis associated pulmonary hypertension. The condition's three major causes are: interstitial lung disease, vasculitis, and chronic thromboembolic disease, but it should be noted that the multiple pulmonary manifestations of rheumatoid arthritis, can all contribute to chronic lung disease or hypoxia. The first patient in this report suffered from moderate restriction due to fibrosis and was diagnosed with pulmonary hypertension during an episode of life threatening hypoxia...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28275485/serum-kl-6-and-surfactant-protein-d-as-monitoring-and-predictive-markers-of-interstitial-lung-disease-in-patients-with-systemic-sclerosis-and-mixed-connective-tissue-disease
#15
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Yumie Yamanaka, Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Koji Okudela, Tae Iwasawa, Tamiko Takemura, Kazuyoshi Kuwano, Takashi Ogura
BACKGROUND: Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. We aimed to determine whether serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) correlate with SSc/MCTD-associated ILD activity. METHODS: We retrospectively analyzed the medical records of 40 patients with SSc/MCTD-associated ILD: 29 patients with SSc and 11 patients with MCTD...
February 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28273432/p53-and-mir-34a-feedback-promotes-lung-epithelial-injury-and-pulmonary-fibrosis
#16
Shwetha K Shetty, Nivedita Tiwari, Amarnath S Marudamuthu, Bijesh Puthusseri, Yashodhar P Bhandary, Jian Fu, Jeffrey Levin, Steven Idell, Sreerama Shetty
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease. The pathogenesis of interstitial lung diseases, including its most common form, IPF, remains poorly understood. Alveolar epithelial cell (AEC) apoptosis, proliferation, and accumulation of myofibroblasts and extracellular matrix deposition results in progressive loss of lung function in IPF. We found induction of tumor suppressor protein, p53, and apoptosis with suppression of urokinase-type plasminogen activator (uPA) and the uPA receptor in AECs from the lungs of IPF patients, and in mice with bleomycin, cigarette smoke, silica, or sepsis-induced lung injury...
March 5, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28267349/oxygen-therapy-for-interstitial-lung-disease-a-mismatch-between-patient-expectations-and-experiences
#17
Yet H Khor, Nicole Sl Goh, Christine F McDonald, Anne E Holland
RATIONALE: Domiciliary oxygen therapy is commonly prescribed for patients with interstitial lung disease and hypoxemia, either at rest or during exertion, with the aim of improving symptoms and functional status. OBJECTIVES: This study aimed to explore perspectives of adults with interstitial lung disease about domiciliary oxygen therapy, comparing insights from patients using and not using oxygen therapy. METHODS: A qualitative study using semi-structured interviews was undertaken on 24 adults residing in and near Melbourne, Australia who had a diagnosis of interstitial lung disease and met the Thoracic Society of Australia and New Zealand Guidelines for domiciliary oxygen therapy...
March 7, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28264721/densitometric-and-local-histogram-based-analysis-of-computed-tomography-images-in-patients-with-idiopathic-pulmonary-fibrosis
#18
Samuel Y Ash, Rola Harmouche, Diego Lassala Lopez Vallejo, Julian A Villalba, Kris Ostridge, River Gunville, Carolyn E Come, Jorge Onieva Onieva, James C Ross, Gary M Hunninghake, Souheil Y El-Chemaly, Tracy J Doyle, Pietro Nardelli, Gonzalo V Sanchez-Ferrero, Hilary J Goldberg, Ivan O Rosas, Raul San Jose Estepar, George R Washko
BACKGROUND: Prior studies of clinical prognostication in idiopathic pulmonary fibrosis (IPF) using computed tomography (CT) have often used subjective analyses or have evaluated quantitative measures in isolation. This study examined associations between both densitometric and local histogram based quantitative CT measurements with pulmonary function test (PFT) parameters and mortality. In addition, this study sought to compare risk prediction scores that incorporate quantitative CT measures with previously described systems...
March 7, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28243798/respiratory-symptoms-are-poor-predictors-of-concomitant-chronic-obstructive-pulmonary-disease-in-patients-with-primary-sj%C3%A3-gren-s-syndrome
#19
Victor Strevens Bolmgren, Peter Olsson, Per Wollmer, Roger Hesselstrand, Thomas Mandl
Involvement of the respiratory system, in particular dry airways and chronic obstructive pulmonary disease (COPD), is common in patients with primary Sjögren's syndrome (pSS). As respiratory symptoms are also common in pSS patients and may have different etiologies, we wanted to evaluate the amount and impact of respiratory symptoms in out-patients with pSS and to assess if such symptoms are related to concomitant COPD. The St George's Respiratory Questionnaire (SGRQ) was used to assess respiratory symptoms...
February 27, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28230051/cellular-senescence-mediates-fibrotic-pulmonary-disease
#20
Marissa J Schafer, Thomas A White, Koji Iijima, Andrew J Haak, Giovanni Ligresti, Elizabeth J Atkinson, Ann L Oberg, Jodie Birch, Hanna Salmonowicz, Yi Zhu, Daniel L Mazula, Robert W Brooks, Heike Fuhrmann-Stroissnigg, Tamar Pirtskhalava, Y S Prakash, Tamara Tchkonia, Paul D Robbins, Marie Christine Aubry, João F Passos, James L Kirkland, Daniel J Tschumperlin, Hirohito Kita, Nathan K LeBrasseur
Idiopathic pulmonary fibrosis (IPF) is a fatal disease characterized by interstitial remodelling, leading to compromised lung function. Cellular senescence markers are detectable within IPF lung tissue and senescent cell deletion rejuvenates pulmonary health in aged mice. Whether and how senescent cells regulate IPF or if their removal may be an efficacious intervention strategy is unknown. Here we demonstrate elevated abundance of senescence biomarkers in IPF lung, with p16 expression increasing with disease severity...
February 23, 2017: Nature Communications
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