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pulmonary fibrosis randomized controlled trial

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https://www.readbyqxmd.com/read/28508087/the-vitamin-d-for-enhancing-the-immune-system-in-cystic-fibrosis-disc-trial-rationale-and-design-of-a-multi-center-double-blind-placebo-controlled-trial-of-high-dose-bolus-administration-of-vitamin-d3-during-acute-pulmonary-exacerbation-of-cystic-fibrosis
#1
Vin Tangpricha, Ellen M Smith, Jose Binongo, Suzanne E Judd, Thomas R Ziegler, Seth Walker, Rabindra Tirouvanziam, Susu M Zughaier, Moon Jeong Lee, Supavit Chesdachai, Wendy A Hermes, James F Chmiel, Amit Gaggar, Ruth E Grossmann, Patricia M Joseph, Jessica A Alvarez
Vitamin D deficiency is highly prevalent in children and adults with cystic fibrosis (CF). Recent studies have found an association between vitamin D status and risk of pulmonary exacerbations in children and adults with CF. The ongoing Vitamin D for enhancing the Immune System in Cystic fibrosis (DISC) study is a multi-center, double-blind, randomized, placebo-controlled trial that will test the hypothesis of whether high dose vitamin D given as a single oral bolus of 250,000 IU to adults with CF during a pulmonary exacerbation followed by a maintenance dose of vitamin D will improve time to next pulmonary exacerbation and re-hospitalization, improve survival and lung function compared to placebo and reduce the rates of pulmonary exacerbation,...
June 2017: Contemporary Clinical Trials Communications
https://www.readbyqxmd.com/read/28471697/pirfenidone-reduces-respiratory-related-hospitalizations-in-idiopathic-pulmonary-fibrosis
#2
Brett Ley, Jeffrey Swigris, Bann-Mo Day, John L Stauffer, Karina Raimundo, Willis Chou, Harold R Collard
RATIONALE: Respiratory-related hospitalizations in patients with idiopathic pulmonary fibrosis (IPF) are more frequent than acute IPF exacerbations and are associated with poor outcomes. OBJECTIVES: To compare the risk of non-elective hospitalization by type (all-cause, respiratory-related, non-respiratory related) and death after hospitalization for pirfenidone versus placebo over 52 weeks from three phase 3 IPF clinical trials. METHODS: Individual patient data was pooled from three phase 3 randomized, placebo-controlled studies of pirfenidone for IPF (CAPACITY and ASCEND) including all patients randomized to pirfenidone 2403 mg/day (n=623) or placebo (n=624)...
May 4, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28470579/does-probiotic-supplementation-affect-pulmonary-exacerbation-and-intestinal-inflammation-in-cystic-fibrosis-a-systematic-review-of-randomized-clinical-trials
#3
REVIEW
Zeinab Nikniaz, Leila Nikniaz, Nemat Bilan, Mohammad Hossein Somi, Elnaz Faramarzi
BACKGROUND: Patients with cystic fibrosis (CF) usually have abnormal intestinal microbiota due to massive exposure to antibiotics. Probiotics could modify the gut microbiota and hence may affect CF management. So the aim of present systematic review was evaluation of the efficacy and safety of probiotic supplementation for the management of cystic fibrosis. DATA SOURCES: We searched PubMed, Science Direct, Google Scholar, Springer Cochrane Library Databases until January 2016 for randomized controlled trials (RCTs) performed in pediatric or adult populations related to the study aim...
April 29, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28469716/allergic-bronchopulmonary-aspergillosis-in-patients-with-cystic-fibrosis
#4
REVIEW
Ibrahim Ahmed Janahi, Abdul Rehman, Amal Rashid Al-Naimi
Allergic bronchopulmonary aspergillosis (ABPA) is a pulmonary disorder that often occurs in patients with asthma or cystic fibrosis (CF) and is characterized by a hypersensitivity response to the allergens of the fungus Aspergillus fumigatus. In patients with CF, growth of A. fumigatus hyphae within the bronchial lumen triggers an immunoglobulin E (IgE)-mediated hypersensitivity response that results in airway inflammation, bronchospasm, and bronchiectasis. In most published studies, the prevalence of ABPA is about 8...
April 2017: Annals of Thoracic Medicine
https://www.readbyqxmd.com/read/28465702/chinese-herbal-extractions-for-relieving-radiation-induced-lung-injury-a-systematic-review-and-meta-analysis
#5
REVIEW
Bo Deng, Chao Deng, Zhiqiang Cheng
Background. Radiation induced lung injury (RILI) is one of the most common and severe side effects of thoracic radiotherapy. In this meta-analysis, the effects of Chinese herbal extractions (CHE) for preventing and treating RILI are evaluated. Methods. Randomized Controlled Trials (RCTs) from five databases were identified. Studies were evaluated and the relevant data were extracted by two authors independently. Differences were resolved by a third party. Meta-analysis was conducted using RevMan 5.0. Results...
2017: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/28459237/effects-of-feiwei-granules-in-the-treatment-of-idiopathic-pulmonary-fibrosis-a-randomized-and-placebo-controlled-trial
#6
Yang Yu, Zengtao Sun, Liqing Shi, Yanping Zhang, Zhaoshan Zhou, Shunan Zhang, Enxiang Chao
OBJECTIVE: To document the therapeutic effects of Feiwei granules (FGs) for idiopathic pulmonary fibrosis (IPF). METHODS: One hundred cases with IPF were randomized into the treatment group (80) and control group (20). Both groups were given basic treatment with prednisone. The treatment group was given FGs, and the control group was given Jinshuibao capsules (JCs). Treatment lasted for 6 months. The Medical Research Council Dyspnea Scale (MRCDS), the Saint George's Hospital Respiratory Questionnaire (SGHRQ), pulmonary function, the Traditional Chinese Medicine Syndrome Score (TCMSS), 6-min walking test (6MWT) and blood gas analyses were recorded before the study as well as 3 months and 6 months after treatment...
August 2016: Journal of Traditional Chinese Medicine, Chung i Tsa Chih Ying Wen Pan
https://www.readbyqxmd.com/read/28430622/serious-adverse-events-of-cell-therapy-for-respiratory-diseases-a-systematic-review-and-meta-analysis
#7
REVIEW
Runzhen Zhao, Zhenlei Su, Jing Wu, Hong-Long Ji
BACKGROUND: Cell therapy holds the most promising for acute and chronic deleterious respiratory diseases. However, the safety and tolerance for lung disorders are controversy. METHODS: We undertook a systematic review and meta-analyses of all 23 clinical studies of cell therapy. The outcomes were odds ratio (OR), risk difference (RD), Peto OR, relative risk, and mean difference of serious adverse events. RESULTS: 342 systemic infusions and 57 bronchial instillations (204 recipients) of cells were analyzed for acute respiratory distress syndrome (ARDS), bronchopulmonary dysplasia, pulmonary arterial hypertension, silicosis, sarcoidosis, extensively drug-resistant tuberculosis, chronic obstructive pulmonary diseases (COPD), and idiopathic pulmonary fibrosis...
May 2, 2017: Oncotarget
https://www.readbyqxmd.com/read/28403943/plasma-metabolomics-in-adults-with-cystic-fibrosis-during-a-pulmonary-exacerbation-a-pilot-randomized-study-of-high-dose-vitamin-d3-administration
#8
RANDOMIZED CONTROLLED TRIAL
Jessica A Alvarez, Elizabeth Y Chong, Douglas I Walker, Joshua D Chandler, Ellen S Michalski, Ruth E Grossmann, Karan Uppal, Shuzhao Li, Jennifer K Frediani, Rabindra Tirouvanziam, ViLinh T Tran, Vin Tangpricha, Dean P Jones, Thomas R Ziegler
BACKGROUND: Cystic fibrosis (CF) is a chronic catabolic disease often requiring hospitalization for acute episodes of worsening pulmonary exacerbations. Limited data suggest that vitamin D may have beneficial clinical effects, but the impact of vitamin D on systemic metabolism in this setting is unknown. OBJECTIVE: We used high-resolution metabolomics (HRM) to assess the impact of baseline vitamin D status and high-dose vitamin D3 administration on systemic metabolism in adults with CF with an acute pulmonary exacerbation...
May 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/28345369/pulmonary-fibrosis-part-ii-state-of-the-art-patient-management
#9
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/28319489/autogenic-drainage-in-children-with-cystic-fibrosis
#10
Lieselotte Corten, Brenda M Morrow
PURPOSE: Airway clearance is an essential part of the management of cystic fibrosis (CF) as it facilitates clearance of viscous pulmonary secretions. This review aimed to determine the effect of autogenic drainage (AD) and assisted autogenic drainage (AAD) compared with no, sham, or other types of airway clearance in children with CF. SUMMARY OF KEY POINTS: Two pediatric randomized cross-over trials were identified on the use of AD in children with CF; no studies were available on the use of AAD...
April 2017: Pediatric Physical Therapy
https://www.readbyqxmd.com/read/28287347/predicting-life-expectancy-for-pirfenidone-in-idiopathic-pulmonary-fibrosis
#11
Mark Fisher, Steven D Nathan, Christian Hill, Jade Marshall, Fred Dejonckheere, Per-Olof Thuresson, Toby M Maher
BACKGROUND: Conducting an adequately powered survival study in idiopathic pulmonary fibrosis (IPF) is challenging due to the rare nature of the disease and the need for extended follow-up. Consequently, registration trials of IPF treatments have not been designed to estimate long-term survival. OBJECTIVE: To predict life expectancy for patients with IPF receiving pirfenidone versus best supportive care (BSC) in a population that met the inclusion criteria of patients enrolled in the ASCEND and CAPACITY trials...
March 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28287346/systematic-review-and-network-meta-analysis-of-idiopathic-pulmonary-fibrosis-treatments
#12
Kelly Fleetwood, Rachael McCool, Julie Glanville, Susan C Edwards, Sandro Gsteiger, Monica Daigl, Mark Fisher
BACKGROUND: The antifibrotics pirfenidone and nintedanib are both approved for the treatment of idiopathic pulmonary fibrosis (IPF) by regulatory agencies and are recommended by health technology assessment bodies. Other treatments such as N-acetylcysteine are used in clinical practice but have not received regulatory approval. No head-to-head trials have been conducted to directly compare the efficacy of these therapies in IPF. OBJECTIVE: To compare the efficacy of treatments for IPF...
March 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28266906/pirfenidone-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#13
Peter M George, Athol U Wells
Idiopathic pulmonary fibrosis (IPF) is a diffuse parenchymal lung disease with no cure. Up until recently, no treatment had been proven to alter its natural history as judged by rate of lung function decline. In 2014 however, the emergence of two novel anti-fibrotic agents, Pirfenidone and Nintedanib revolutionized the management of this condition. Both have demonstrated the ability to deliver a major reduction in the rate of chronic IPF progression. Areas Covered: This review article focuses on Pirfenidone - a pyridone derivative initially designed as an analgesic and anti-pyretic agent...
May 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28258928/inhaled-dry-powder-mannitol-in-children-with-cystic-fibrosis-a-randomised-efficacy-and-safety-trial
#14
K De Boeck, E Haarman, J Hull, L C Lands, A Moeller, A Munck, J Riethm├╝ller, H Tiddens, S Volpi, J Leadbetter, B Charlton, A Malfroot
INTRODUCTION: Inhaled mannitol has beneficial effects on lung function, mucociliary clearance, quality of life and sputum properties. This trial examined the efficacy of inhaled mannitol in children with cystic fibrosis (CF). METHODS: The efficacy of inhaled mannitol in children with CF aged 6-17years was assessed in a phase 2, randomised, placebo-controlled crossover study. Subjects were randomly assigned to mannitol 400mg every 12h or matching placebo for 8weeks, followed by an 8week washout and an 8week period with the alternate treatment...
May 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28137504/etiology-and-treatment-of-cough-in-idiopathic-pulmonary-fibrosis
#15
REVIEW
Christine L Vigeland, Andrew H Hughes, Maureen R Horton
Idiopathic pulmonary fibrosis (IPF) is a progressive disease of dysregulated wound healing leading to unremitting scarring and loss of lung function. The predominant symptoms are dyspnea on exertion and a persistent dry cough. For patients with IPF, cough is more than just bothersome; it has a significant negative impact on quality of life and is a marker of disease severity and progression. The etiology of cough in IPF is unclear but may be due to architectural distortion of the lungs, increased sensitivity of the cough reflex, airway inflammation, or changes in mucus production and clearance...
February 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28125158/chest-physiotherapy-can-affect-the-lung-clearance-index-in-cystic-fibrosis-patients
#16
Joerg Grosse-Onnebrink, Uwe Mellies, Margarete Olivier, Claudius Werner, Florian Stehling
OBJECTIVES: The lung clearance index (LCI) is determined by multiple-breath washout lung function (MBW). It is increasingly used as an endpoint in clinical trials. Chest physiotherapy (CP) is part of routine cystic fibrosis (CF) care. Whether the LCI is useful in detecting short-term treatment effects of CP has not been sufficiently investigated. We assessed the short-term influence of CP with highly standardized high-frequency chest wall oscillation (HFCWO) on the LCI in CF patients...
May 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28114922/a-prospective-pilot-study-of-home-monitoring-in-adults-with-cystic-fibrosis-home-cf-protocol-for-a-randomised-controlled-trial
#17
Jocelyn Choyce, Karen L Shaw, Alice J Sitch, Hema Mistry, Joanna L Whitehouse, Edward F Nash
BACKGROUND: Home monitoring has the potential to detect early pulmonary exacerbations in people with cystic fibrosis (CF), with consequent improvements in health outcomes and healthcare associated costs. This study aims to assess the effects of home monitoring on hospital admissions, quality of life, antibiotic requirements, exacerbation frequency, lung function, nutritional outcomes, anxiety, depression, costs and health outcomes, as well as the qualitative effects on the patient experience...
January 23, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28102546/ataluren-and-similar-compounds-specific-therapies-for-premature-termination-codon-class-i-mutations-for-cystic-fibrosis
#18
REVIEW
Aisha A Aslam, Colin Higgins, Ian P Sinha, Kevin W Southern
BACKGROUND: Cystic fibrosis is a common life-shortening genetic disorder in the Caucasian population (less common in other ethnic groups) caused by the mutation of a single gene that codes for the production of the cystic fibrosis transmembrane conductance regulator protein. This protein coordinates the transport of salt (and bicarbonate) across cell surfaces and the mutation most notably affects the airways. In the lungs of people with cystic fibrosis, defective protein results in a dehydrated surface liquid and compromised mucociliary clearance...
January 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28011037/assessment-of-safety-and-efficacy-of-long-term-treatment-with-combination-lumacaftor-and-ivacaftor-therapy-in-patients-with-cystic-fibrosis-homozygous-for-the-f508del-cftr-mutation-progress-a-phase-3-extension-study
#19
Michael W Konstan, Edward F McKone, Richard B Moss, Gautham Marigowda, Simon Tian, David Waltz, Xiaohong Huang, Barry Lubarsky, Jaime Rubin, Stefanie J Millar, David J Pasta, Nicole Mayer-Hamblett, Christopher H Goss, Wayne Morgan, Gregory S Sawicki
BACKGROUND: The 24-week safety and efficacy of lumacaftor/ivacaftor combination therapy was shown in two randomised controlled trials (RCTs)-TRAFFIC and TRANSPORT-in patients with cystic fibrosis who were aged 12 years or older and homozygous for the F508del-CFTR mutation. We aimed to assess the long-term safety and efficacy of extended lumacaftor/ivacaftor therapy in this group of patients in PROGRESS, the long-term extension of TRAFFIC and TRANSPORT. METHODS: PROGRESS was a phase 3, parallel-group, multicentre, 96-week study of patients who completed TRAFFIC or TRANSPORT in 191 sites in 15 countries...
February 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28000919/antibiotic-treatment-for-nontuberculous-mycobacteria-lung-infection-in-people-with-cystic-fibrosis
#20
REVIEW
Valerie Waters, Felix Ratjen
BACKGROUND: Nontuberculous mycobacteria are mycobacteria, other than those in the Mycobacterium tuberculosis complex, and are commonly found in the environment. Nontuberculous mycobacteria species (most commonly Mycobacterium avium complex and Mycobacterium abscessus) are isolated from the respiratory tract of approximately 5% to 40% of individuals with cystic fibrosis; they can cause lung disease in people with cystic fibrosis leading to more a rapid decline in lung function and even death in certain circumstances...
December 19, 2016: Cochrane Database of Systematic Reviews
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