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idiopathic lung fibrosis

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https://www.readbyqxmd.com/read/28092235/hospital-cost-and-length-of-stay-in-idiopathic-pulmonary-fibrosis
#1
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/28086844/are-risk-predicting-models-useful-for-estimating-survival-of-patients-with-rheumatoid-arthritis-associated-interstitial-lung-disease
#2
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
#3
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
#4
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
#5
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
#6
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/28078713/the-novel-mtor-complex-1-2-inhibitor-p529-inhibits-human-lung-myofibroblast-differentiation
#7
Keith T Ferguson, Elizabeth E Torr, Ksenija Bernau, Jonathan Leet, Davis Sherris, Nathan Sandbo
Idiopathic pulmonary fibrosis is a progressive and deadly disorder with very few therapeutic options. Palomid 529 (8-(1-hydroxyethyl)-2-methoxy-3-(4-methoxybenzyloxy)-benzo[c]chromen-6-one; P529) is a novel dual inhibitor of mechanistic target of rapamycin complex 1/2 (mTORC1/2). In these studies, we investigated the effect of P529 on TGF-β-dependent signaling and myofibroblast differentiation. TGF-β-induced phosphorylation of the mTORC1 targets, p70 S6 kinase 1 (S6K1) and eukaryotic translation initiation factor 4E binding protein 1 (4E-BP1), were both dose dependently inhibited by P529 in human lung fibroblasts with maximal inhibition occurring between 10-20 µM...
January 11, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28073522/antisynthetase-syndrome-analysis-of-11-cases
#8
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/28067202/pulmonary-fibrosis-tissue-characterization-using-late-enhanced-mri-compared-with-unenhanced-anatomic-high-resolution-ct
#9
Lisa P Lavelle, Darragh Brady, Sinead McEvoy, David Murphy, Brian Gibney, Annika Gallagher, Marcus Butler, Fionnula Shortt, Marie McMullen, Aurelie Fabre, David A Lynch, Michael P Keane, Jonathan D Dodd
PURPOSE: We aimed to prospectively evaluate anatomic chest computed tomography (CT) with tissue characterization late gadolinium-enhanced magnetic resonance imaging (MRI) in the evaluation of pulmonary fibrosis (PF). METHODS: Twenty patients with idiopathic pulmonary fibrosis (IPF) and twelve control patients underwent late-enhanced MRI and high-resolution CT. Tissue characterization of PF was depicted using a segmented inversion-recovery turbo low-angle shot MRI sequence...
January 9, 2017: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/28066605/relationship-between-survival-and-age-in-patients-with-idiopathic-pulmonary-fibrosis
#10
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
#11
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
#12
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
#13
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/28054535/combined-prednisolone-and-pirfenidone-in-bleomycin-induced-lung-disease
#14
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
#15
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
https://www.readbyqxmd.com/read/28051231/effect-of-pistacia-lentiscus-oil-on-experimental-pulmonary-fibrosis
#16
Anouar Abidi, Raja Serairi Beji, Nadia Kourda, Samir Ennigrou, Riadh Ksouri, Saloua Jameleddine
Background - Idiopathic pulmonary fibrosis (IPF) is a chronic disease characterized by histopathological lesions in lung tissue. This is the most common and most severe idiopathic interstitial pneumonias. Current treatments are based on the combination of corticosteroids and immunosuppressants, but their effectiveness is still debated. Purpose of work - Testing the preventive effect of Pistacia Lentiscus oil, known for its antioxidant, anti-mutagenic and anti-proliferative effects, on a model of experimental lung fibrosis...
July 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/28046024/chest-fat-quantification-via-ct-based-on-standardized-anatomy-space-in-adult-lung-transplant-candidates
#17
Yubing Tong, Jayaram K Udupa, Drew A Torigian, Dewey Odhner, Caiyun Wu, Gargi Pednekar, Scott Palmer, Anna Rozenshtein, Melissa A Shirk, John D Newell, Mary Porteous, Joshua M Diamond, Jason D Christie, David J Lederer
PURPOSE: Overweight and underweight conditions are considered relative contraindications to lung transplantation due to their association with excess mortality. Yet, recent work suggests that body mass index (BMI) does not accurately reflect adipose tissue mass in adults with advanced lung diseases. Alternative and more accurate measures of adiposity are needed. Chest fat estimation by routine computed tomography (CT) imaging may therefore be important for identifying high-risk lung transplant candidates...
2017: PloS One
https://www.readbyqxmd.com/read/28039616/a-cost-effectiveness-analysis-of-nintedanib-in-idiopathic-pulmonary-fibrosis-in-the-uk
#18
C Rinciog, M Watkins, S Chang, T M Maher, C LeReun, D Esser, A Diamantopoulos
BACKGROUND: International guidelines recommend nintedanib (OFEV(®)) as an option for the treatment of idiopathic pulmonary fibrosis (IPF). OBJECTIVE: The objective of this study was to assess the cost effectiveness of nintedanib versus pirfenidone, N-acetylcysteine and best supportive care (BSC) for the treatment of IPF from a UK payer's perspective. METHODS: A Markov model was designed to capture the changes in the condition of adults with IPF...
December 31, 2016: PharmacoEconomics
https://www.readbyqxmd.com/read/28038697/correction-of-spinal-deformity-on-a-lung-transplantation-recipient
#19
José Vicente Andrés Peiró, Joan Bagó Granell, Montserrat Feliu Moret, Antonio Moreno Galdó
BACKGROUND: The coexistence of lung disease and scoliosis entails a dramatic situation. There are no papers reporting scoliosis surgery in patients who suffered lung transplantation. PURPOSE: To describe the case of a patient who underwent surgery to correct progressive spinal deformity after two consecutive lung transplants. STUDY DESIGN: Case report, including review of patient records, imaging and pulmonary function tests, and literature review...
January 2017: Spine Deformity
https://www.readbyqxmd.com/read/28035951/a-systematic-review-of-the-role-of-dysfunctional-wound-healing-in-the-pathogenesis-and-treatment-of-idiopathic-pulmonary-fibrosis
#20
REVIEW
Alan Betensley, Rabab Sharif, Dimitrios Karamichos
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disorder showcasing an interaction between genetic predisposition and environmental risks. This usually involves the coaction of a mixture of cell types associated with abnormal wound healing, leading to structural distortion and loss of gas exchange function. IPF bears fatal prognosis due to respiratory failure, revealing a median survival of approximately 2 to 3 years. This review showcases the ongoing progress in understanding the complex pathophysiology of IPF and it highlights the latest potential clinical treatments...
December 26, 2016: Journal of Clinical Medicine
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