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pulmonary fibrosis randomized controlled trial

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https://www.readbyqxmd.com/read/28629920/pulmonary-hypertension-in-parenchymal-lung-diseases-any-future-for-new-therapies
#1
REVIEW
Sergio Harari, Davide Elia, Marc Humbert
Pulmonary hypertension (PH) due to chronic lung diseases is associated with a poor prognosis, regardless of the underlying respiratory condition. Updated PH guidelines recommend optimal treatment of the underlying lung disease, including long-term oxygen therapy, in patients with chronic hypoxaemia despite the lack of randomized controlled clinical trials supporting this statement. So far, randomized controlled trials on drugs approved for pulmonary arterial hypertension (PAH) have yielded discouraging results in both interstitial lung diseases (ILD) and chronic obstructive pulmonary diseases (COPD) with PH...
June 16, 2017: Chest
https://www.readbyqxmd.com/read/28622199/antifibrotic-drugs-as-treatment-of-nonidiopathic-pulmonary-fibrosis-interstitial-pneumonias-the-time-is-now
#2
Michael Kreuter, Julia Wälscher, Jürgen Behr
PURPOSE OF REVIEW: Therapeutic advances in the management of idiopathic pulmonary fibrosis (IPF) has led to improved outcomes with the use of the antifibrotic agents pirfenidone and nintedanib, with a number of randomized studies demonstrating benefits in slowing disease progression in IPF. However, treatment of other fibrosing interstitial lung diseases (ILD) remains challenging. RECENT FINDINGS: Observational and uncontrolled studies investigating pirfenidone and nintedanib in non-IPF ILDs suggest potential benefits, although the data is weak...
June 15, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28608719/home-monitoring-in-cf-to-identify-and-treat-acute-pulmonary-exacerbations-eice-study-results
#3
Noah Lechtzin, Nicole Mayer-Hamblett, Natalie E West, Sarah Allgood, Ellen Wilhelm, Umer Khan, Moira L Aitken, Bonnie W Ramsey, Michael P Boyle, Peter J Mogayzel, Ronald L Gibson, David Orenstein, Carlos Milla, John P Clancy, Veena Antony, Christopher H Goss
RATIONALE: Individuals with cystic fibrosis (CF) suffer frequent acute pulmonary exacerbations, which lead to decreased lung function and reduced quality of life. OBJECTIVES: The goal of this study was to determine if an intervention directed toward early detection of pulmonary exacerbations using home spirometry and symptom monitoring would result in slower decline in lung function compared to controls. METHODS: A multicenter randomized trial at 14 CF centers in subjects ≥14 years...
June 13, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28552099/bacteriology-and-treatment-of-infections-in-the-upper-and-lower-airways-in-patients-with-primary-ciliary-dyskinesia-adressing-the-paranasal-sinuses
#4
Mikkel Christian Alanin
The respiratory tract is lined with motile cilia that transport respiratory mucus. Primary ciliary dyskinesia (PCD) is a chronic genetic disease caused by mutations in genes responsible for ciliary structure and function. Non-functional airway cilia impair the mucociliary clearance (MCC), causing mucostasis, lung infections and destruction, chronic rhinosinusitis (CRS) and hearing impairment. It is of paramount importance to postpone chronic lung infection mainly with Gram-negative bacteria (GNB) in patients with an impaired MCC...
May 2017: Danish Medical Journal
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
REVIEW
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
#18
K De Boeck, E Haarman, J Hull, L C Lands, A Moeller, A Munck, J Riethmüller, 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
#19
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
#20
RANDOMIZED CONTROLLED TRIAL
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
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