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Idiopathic pulmonary disease

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https://www.readbyqxmd.com/read/28100360/-end-tidal-pco-2-for-evaluation-of-severity-of-disease-in-idiopathic-pulmonary-arterial-hypertension-and-chronic-thromboembolic-pulmonary-hypertension
#1
X Shi, G L Yang, Y Chen, J Guo, W L Yang, P Yuan, S G Gong, R Jiang, Q H Zhao, L Wang, J He, T X Chen, J M Liu
Objective: To explore the difference in end tidal PCO(2) (P(ET)CO(2)) between idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH), and to analyze the correlation between P(ET)CO(2) and the indexes of disease severity in IPAH and CTEPH patients. Methods: Data were retrieved from 68 IPAH patients and 52 CTEPH patients who all had received right-heart catheterization, pulmonary function test and cardiopulmonary exercise testing at Shanghai Pulmonary Hospital from October 2011 to October 2014...
January 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28099456/burden-of-idiopathic-pulmonary-fibrosis-progression-a-5-year-longitudinal-follow-up-study
#2
Vincent Cottin, Aurélie Schmidt, Laura Catella, Fanny Porte, Céline Fernandez-Montoya, Katell Le Lay, Stève Bénard
Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with an unpredictable course. An observational study was set up using the French hospital discharge database to describe the reasons, outcomes and costs of hospitalisations related to this disease. Patients newly hospitalised for idiopathic pulmonary fibrosis (ICD-10 code: J84.1) in 2008 were identified and followed for 5 years. As J84.1 includes other fibrotic pulmonary diseases, an algorithm excluding age<50 years and presence of a differential diagnosis in the following year was defined...
2017: PloS One
https://www.readbyqxmd.com/read/28099038/an-exome-sequencing-study-to-assess-the-role-of-rare-genetic-variation-in-pulmonary-fibrosis
#3
Slavé Petrovski, Jamie L Todd, Michael T Durheim, Quanli Wang, Jason W Chien, Fran L Kelly, Courtney Frankel, Caroline M Mebane, Zhong Ren, Joshua Bridgers, Thomas J Urban, Colin D Malone, Ashley Finlen Copeland, Christie Brinkley, Andrew S Allen, Thomas O'Riordan, John G McHutchison, Scott M Palmer, David B Goldstein
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is an increasingly recognized, often fatal lung disease of unknown etiology. OBJECTIVES: The aim of this study was to use whole-exome sequencing to improve our understanding of the genetic architecture of pulmonary fibrosis. METHODS: We performed a case-control exome-wide collapsing analysis including 262 unrelated individuals with pulmonary fibrosis; clinically classified as IPF according to ATS/ERS/JRS/ALAT guidelines (81...
January 18, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28098218/the-role-of-mir-497-5p-in-myofibroblast-differentiation-of-lr-mscs-and-pulmonary-fibrogenesis
#4
Xiang Chen, Chaowen Shi, Cong Wang, Weilin Liu, Yanhong Chu, Zou Xiang, Kebin Hu, Ping Dong, Xiaodong Han
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal fibrotic lung disease characterized by profound changes in stem cell differentiation, epithelial cell phenotypes and fibroblast proliferation. In our study, we found that miR-497-5p was significantly upregulated both during myofibroblast differentiation of lung resident mesenchymal stem cells (LR-MSCs) and in the lung tissues of a pulmonary fibrosis model. In addition, as determined by luciferase assays and Western blot analysis, reversion-inducing cysteine-rich protein with kazal motifs (Reck) was identified to be one of the target genes of miR-497-5p, and Reck could suppress the expression of matrix metalloproteinase-2 (Mmp2) and Mmp9, which could activate latent transforming growth factor-β1 (TGF-β1)...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28092235/hospital-cost-and-length-of-stay-in-idiopathic-pulmonary-fibrosis
#5
Joshua J Mooney, Karina Raimundo, Eunice Chang, Michael S Broder
OBJECTIVE: To provide a detailed picture of the economic impact of hospitalization in idiopathic pulmonary fibrosis (IPF) and to identify factors associated with cost and length of stay (LOS). METHODS: In this retrospective cross-sectional study using the Nationwide Inpatient Sample (NIS), we included hospitalizations for IPF (ICD-9-CM 516.3) with a principal diagnosis of respiratory disease (ICD-9-CM 460-519) from 2009-2011; lung transplant admissions were excluded...
January 16, 2017: Journal of Medical Economics
https://www.readbyqxmd.com/read/28090297/exhaled-nitric-oxide-in-pulmonary-arterial-hypertension-associated-with-systemic-sclerosis
#6
Zeling Cao, Stephen C Mathai, Laura K Hummers, Ami A Shah, Fredrick M Wigley, Noah Lechtzin, Paul M Hassoun, Reda E Girgis
The fractional exhaled concentration of nitric oxide (FENO) has been shown to be reduced in idiopathic pulmonary arterial hypertension (PAH) but has not been adequately studied in PAH associated with systemic sclerosis (SSc). We measured FENO at an expiratory flow rate of 50 mL/s in 21 treatment-naive patients with SSc-associated PAH (SSc-PAH), 94 subjects with SSc without pulmonary involvement, and 84 healthy volunteers. Measurements of FENO at additional flow rates of 100, 150, and 250 mL/s were obtained to derive the flow-independent nitric oxide exchange parameters of maximal airway flux (J'awNO) and steady-state alveolar concentration (CANO)...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28086844/are-risk-predicting-models-useful-for-estimating-survival-of-patients-with-rheumatoid-arthritis-associated-interstitial-lung-disease
#7
Hanna M Nurmi, Minna K Purokivi, Miia S Kärkkäinen, Hannu-Pekka Kettunen, Tuomas A Selander, Riitta L Kaarteenaho
BACKGROUND: Risk predicting models have been applied in idiopathic pulmonary fibrosis (IPF), but still not validated in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). The purpose of this study was to test the suitability of three prediction models as well as individual lung function and demographic factors for evaluating the prognosis of RA-ILD patients. METHODS: Clinical and radiological data of 59 RA-ILD patients was re-assessed...
January 13, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28079978/targeting-coagulation-factor-receptors-protease-activated-receptors-in-idiopathic-pulmonary-fibrosis
#8
REVIEW
Cong Lin, Keren Borensztajn, C Arnold Spek
Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease with a 5-year mortality rate above 50% and unknown etiology. Treatment options remain limited and, currently, only two drugs are available, nintedanib and pirfenidone. However, both of these antifibrotic agents only slow down the progression of the disease but do not remarkably prolong the survival of IPF patients. Hence, the discovery of new therapeutic targets for IPF is crucial. Studies exploring the mechanisms that are involved in IPF identified several possible targets for therapeutic interventions...
January 12, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28079851/corticosteroid-and-cyclophosphamide-in-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-single-center-experience-and-literature-review
#9
Luca Novelli, Roberto Ruggiero, Federica De Giacomi, Alice Biffi, Paola Faverio, Luca Bilucaglia, Silvia Gamberini, Grazia Messinesi, Alberto Pesci
Acute Exacerbation (AEx) is a frequent and severe complication of Idiopathic Pulmonary Fibrosis (IPF). In the absence of consensus regarding treatment, studies evaluating the efficacy of specific therapies, such as corticosteroids and immunosuppresant agents, are needed. In this case series we evaluated the outcome in terms of survival of intravenous pulse doses of high-dose corticosteroid (methylprednisolone 1000 mg per day for 3 consecutive days) followed by montlhy cyclophosphamide administration (maximum 6 doses) in a cohort of patients with AEx-IPF referred to the Respiratory Unit, San Gerardo University Hospital, Monza, Italy, from 2009 to 2013...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079846/patient-confidence-and-quality-of-life-in-idiopathic-pulmonary-fibrosis-and-sarcoidosis
#10
Jalpa Kotecha, Christopher Atkins, Andrew Wilson
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) and sarcoidosis impact significantly on health-related quality of life (HRQOL). There are few studies on the impact of patient confidence on HRQOL in these conditions. OBJECTIVES: 1. To investigate whether patient confidence is associated with HRQOL, anxiety, depression, dyspnoea or fatigue. 2. To assess if patient confidence is associated with inpatient admissions, access to community healthcare and, for IPF patients, mortality and disease severity...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079842/increased-erythrocyte-aggregation-and-oxidative-stress-in-patients-with-idiopathic-interstitial-pneumonia
#11
Erhan Ugurlu, Emine Kilic-Toprak, Goksel Altinisik, Ozgen Kilic-Erkek, Betul Cengiz, Vural Kucukatay, Hande Senol, Ismail Hakki Akbudak, Yusuf Ekbic, Melek Bor-Kucukatay
BACKGROUND: Hemorheological properties are important determinants of tissue oxygenation. Although hemorheological alterations in various lung diseases have been well-defined, no information is available about the effects of idiopathic interstitial pneumonia (IIP) on hemorheological parameters. OBJECTIVES: The aim of this study was to investigate hemorheological parameters (erythrocyte deformability, aggregation, and plasma viscosity -PV) and associated oxidative stress indices in patients with IIP...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28073522/antisynthetase-syndrome-analysis-of-11-cases
#12
Ester Zamarrón-de Lucas, Luis Gómez Carrera, Gema Bonilla, Dessiree Petit, Alberto Mangas, Rodolfo Álvarez-Sala
INTRODUCTION: Antisynthetase syndrome (ASS) is characterised by a series of clinical manifestations such as myositis, fever, mechanic's hands and diffuse interstitial lung disease (ILD), all associated with positivity to antisynthetase antibodies. The presence of ILD will be that, to a great extent it will mark the response to treatment and prognosis. PATIENTS AND METHODS: Eleven cases of patients with ASS and pulmonary involvement in monitoring at a Pulmonary monographic consult in a third level hospital consult are described...
January 7, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28070238/hematological-disorders-and-pulmonary-hypertension
#13
REVIEW
Rajamma Mathew, Jing Huang, Joseph M Wu, John T Fallon, Michael H Gewitz
Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. Several hematological disorders such as sickle cell disease, thalassemia and myeloproliferative diseases develop PH which worsens the prognosis. Associated oxidant injury and vascular inflammation cause endothelial damage and dysfunction. Pulmonary vascular endothelial damage/dysfunction is an early event in PH resulting in the loss of vascular reactivity, activation of proliferative and antiapoptotic pathways leading to vascular remodeling, elevated pulmonary artery pressure, right ventricular hypertrophy and premature death...
December 26, 2016: World Journal of Cardiology
https://www.readbyqxmd.com/read/28066605/relationship-between-survival-and-age-in-patients-with-idiopathic-pulmonary-fibrosis
#14
So-My Koo, Soo-Taek Uh, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Choon Sik Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Jong Wook Shin, Eun Joo Lee, Jin Hwa Lee, Yangin Jegal, Hyun Kyung Lee, Yong Hyun Kim, Jin Woo Song, Moo Suk Park, Young Hwangbo
BACKGROUND: There is a debate that older patients with idiopathic pulmonary fibrosis (IPF) have a worse prognosis. We evaluated whether age affects the survival of patients with IPF. METHODS: The Korean Interstitial Lung Disease (ILD) Research Group conducted a national survey to evaluate the clinical, physiological, radiological, and survival characteristics of patients with IPF. A total of 1,663 patients with IPF were stratified into three groups according to age: (I) <60 years (n=309); (II) 60-69 years (n=613); and (III) ≥70 years (n=741)...
November 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28062486/plasma-membrane-wounding-and-repair-in-pulmonary-diseases
#15
Xiaofei Cong, Rolf Dieter Hubmayr, Changgong Li, Xiaoli Zhao
Various pathophysiological conditions such as surfactant dysfunction, mechanical ventilation, inflammation, pathogen products, environmental exposures and gastric acid aspiration stress lung cells and the compromise of plasma membranes occur as a result. The mechanisms necessary for cells to repair plasma membrane defects have been extensively investigated in the last two decades, and some of these key repair mechanisms are also shown to occur following lung cell injury. As it was theorized that lung wounding and repair are involved in the pathogenesis of acute respiratory distress syndrome (ARDS) and idiopathic pulmonary fibrosis (IPF), in this review, we summarized the experimental evidence of lung cell injury in these two devastating syndromes, discuss relevant genetic, physical and biological injury mechanisms, as well as mechanisms utilized by lung cells for cell survival and membrane repair...
January 6, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28060543/inhibition-of-the-kca3-1-channel-alleviates-established-pulmonary-fibrosis-in-a-large-animal-model
#16
Louise Organ, Barbara Bacci, Emmanuel Koumoundouros, Wayne G Kimpton, Chrishan S Samuel, Cameron J Nowell, Peter Bradding, Katy M Roach, Glen Westall, Jade Jaffar, Ken J Snibson
Idiopathic pulmonary fibrosis is a chronic progressive disease of increasing prevalence marked by poor prognosis and limited treatment options. Ca2+-activated KCa3.1 potassium channels have been shown to play a key role in the aberrant activation and responses to injury in both epithelial cells and fibroblasts, both considered key drivers in the fibrotic process of IPF. Pharmacological inhibition of IPF-derived fibroblasts is able to somewhat prevent TGF-βand bFGF-dependent profibrotic responses. In the current study, we investigated whether blockade of the KCa3...
January 6, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28057004/gene-profile-of-fibroblasts-identify-relation-of-ccl8-with-idiopathic-pulmonary-fibrosis
#17
Jong-Uk Lee, Hyun Sub Cheong, Eun-Young Shim, Da-Jeong Bae, Hun Soo Chang, Soo-Taek Uh, Young Hoon Kim, Jong-Sook Park, Bora Lee, Hyoung Doo Shin, Choon-Sik Park
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is characterized by the complex interaction of cells involved in chronic inflammation and fibrosis. Global gene expression of a homogenous cell population will identify novel candidate genes. METHODS: Gene expression of fibroblasts derived from lung tissues (8 IPF and 4 controls) was profiled, and ontology and functional pathway were analyzed in the genes exhibiting >2 absolute fold changes with p-values < 0...
January 5, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28055066/association-between-antipsychotic-agents-and-risk-of-acute-respiratory-failure-in-patients-with-chronic-obstructive-pulmonary-disease
#18
Meng-Ting Wang, Chen-Liang Tsai, Chen Wei Lin, Chin-Bin Yeh, Yun-Han Wang, Hui-Lan Lin
Importance: Acute respiratory failure (ARF) is a life-threatening event that has been linked in case reports to antipsychotic use, but this association lacks population-based evidence. Particular attention should be focused on patients with chronic obstructive pulmonary disease (COPD) regarding this drug safety concern because these patients are prone to ARF and are commonly treated with antipsychotics. Objective: To determine whether the use of antipsychotics is associated with an increased risk of ARF in patients with COPD...
January 4, 2017: JAMA Psychiatry
https://www.readbyqxmd.com/read/28054535/combined-prednisolone-and-pirfenidone-in-bleomycin-induced-lung-disease
#19
Preyas J Vaidya, H S Sandeepa, Tejinder Singh, S K Susheel Kumar, Rajat Bhargava, Gopal Ramakrishnan, Prashant N Chhajed
Bleomycin is a cytostatic drug commonly employed in the treatment of Hodgkin's disease, seminomas, and choriocarcinoma. Bleomycin may induce a chronic pulmonary inflammation that may progress to fibrosis. So far, only corticosteroids have been used in the treatment of bleomycin-induced lung disease with variable results. Pirfenidone is an antifibrotic drug that has been approved for the treatment of idiopathic pulmonary fibrosis. We report two cases of bleomycin-induced lung disease treated successfully with pirfenidone and oral corticosteroids...
July 2016: Journal of Cancer Research and Therapeutics
https://www.readbyqxmd.com/read/28052583/pulmonary-hypertension-in-advanced-lung-diseases-echocardiography-as-an-important-part-of-patient-evaluation-for-lung-transplantation
#20
Jolanta Nowak, Bartosz Hudzik, Dariusz Jastrze Bski, Jacek T Niedziela, Piotr Rozentryt, Jacek Wojarski, Marek Ochman, Wojciech Karolak, Sławomir Żegleń, Marek Gierlotka, Mariusz Gąsior
INTRODUCTION: Pulmonary hypertension (PH) is common complication in advanced lung disease. Echocardiography provides additional information and may be useful to assess PH probability. OBJECTIVES: The usefulness of combination of well-known echocardiographic parameters in detecting PH in patients with advanced lung disease referred for lung transplantation was evaluated. METHODS: The study population consisted of 37 consecutive patients with idiopathic pulmonary fibrosis (IPF), 20 patients with chronic obstructive pulmonary disease (COPD), and 8 patients with other interstitial lung diseases...
January 4, 2017: Clinical Respiratory Journal
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