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pulmonary fibrosis randomized controlled trial

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https://www.readbyqxmd.com/read/28137504/etiology-and-treatment-of-cough-in-idiopathic-pulmonary-fibrosis
#1
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
#2
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...
January 26, 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
#3
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
#4
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
#5
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
#6
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...
19, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27939076/efficacy-of-simtuzumab-versus-placebo-in-patients-with-idiopathic-pulmonary-fibrosis-a-randomised-double-blind-controlled-phase-2-trial
#7
Ganesh Raghu, Kevin K Brown, Harold R Collard, Vincent Cottin, Kevin F Gibson, Robert J Kaner, David J Lederer, Fernando J Martinez, Paul W Noble, Jin Woo Song, Athol U Wells, Timothy P M Whelan, Wim Wuyts, Emmanuel Moreau, Scott D Patterson, Victoria Smith, Selina Bayly, Jason W Chien, Qi Gong, Jenny J Zhang, Thomas G O'Riordan
BACKGROUND: Lysyl oxidase-like 2 (LOXL2) catalyses collagen cross-linking and is implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF). The aim of this study was to investigate the efficacy and safety of simtuzumab, a monoclonal antibody against LOXL2, in patients with IPF. METHODS: In this randomised, double-blind, phase 2 trial, we recruited patients aged 45-85 years with definite IPF diagnosed prior to 3 years of screening from 183 hospitals and respiratory clinics in 14 countries...
January 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27795804/hepatic-sarcoidosis-a-case-series
#8
Rym Ennaifer, Shema Ayadi, Hayfa Romdhane, Myriam Cheikh, Houda Ben Nejma, Wassila Bougassas, Najet Bel Hadj
Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement...
2016: Pan African Medical Journal
https://www.readbyqxmd.com/read/27764523/benzodiazepines-for-the-relief-of-breathlessness-in-advanced-malignant-and-non-malignant-diseases-in-adults
#9
REVIEW
Steffen T Simon, Irene J Higginson, Sara Booth, Richard Harding, Vera Weingärtner, Claudia Bausewein
BACKGROUND: This is an updated version of the original Cochrane review published in Issue 1, 2010, on 'Benzodiazepines for the relief of breathlessness in advanced malignant and non-malignant diseases in adults'. Breathlessness is one of the most common symptoms experienced in the advanced stages of malignant and non-malignant disease. Benzodiazepines are widely used for the relief of breathlessness in advanced diseases and are regularly recommended in the literature. At the time of the previously published Cochrane review, there was no evidence for a beneficial effect of benzodiazepines for the relief of breathlessness in people with advanced cancer and chronic obstructive pulmonary disease (COPD)...
October 20, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27576473/probiotic-supplementation-in-children-with-cystic-fibrosis-a-systematic-review
#10
REVIEW
Anitha Ananthan, Haribalakrishna Balasubramanian, Shripada Rao, Sanjay Patole
UNLABELLED: Probiotics may benefit in cystic fibrosis (CF) as gut dysbiosis is associated with gastrointestinal symptoms and exacerbation of respiratory symptoms in CF. We conducted a systematic review of randomized controlled trials (RCTs) and non-RCTs of probiotic supplementation in children with CF, using the Cochrane methodology, preferred reporting items for systematic reviews (PRISMA) statement, and meta-analysis of observational studies in epidemiology (MOOSE) guidelines. Primary outcomes were pulmonary exacerbations, duration of hospitalization and antibiotics, and all-cause mortality...
October 2016: European Journal of Pediatrics
https://www.readbyqxmd.com/read/27450411/study-protocol-rationale-and-recruitment-in-a-european-multi-centre-randomized-controlled-trial-to-determine-the-efficacy-and-safety-of-azithromycin-maintenance-therapy-for-6%C3%A2-months-in-primary-ciliary-dyskinesia
#11
RANDOMIZED CONTROLLED TRIAL
Helene E Kobbernagel, Frederik F Buchvald, Eric G Haarman, Carmen Casaulta, Samuel A Collins, Claire Hogg, Claudia E Kuehni, Jane S Lucas, Heymut Omran, Alexandra L Quittner, Claudius Werner, Kim G Nielsen
BACKGROUND: Clinical management of primary ciliary dyskinesia (PCD) respiratory disease is currently based on improving mucociliary clearance and controlling respiratory infections, through the administration of antibiotics. Treatment practices in PCD are largely extrapolated from more common chronic respiratory disorders, particularly cystic fibrosis, but no randomized controlled trials (RCT) have ever evaluated efficacy and safety of any pharmacotherapeutics used in the treatment of PCD...
July 22, 2016: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/27415821/antibiotic-treatment-for-stenotrophomonas-maltophilia-in-people-with-cystic-fibrosis
#12
REVIEW
Reshma Amin, Valerie Waters
BACKGROUND: Stenotrophomonas maltophilia is one of the most common emerging multi-drug resistant organisms found in the lungs of people with cystic fibrosis and its prevalence is increasing. Chronic infection with Stenotrophomonas maltophilia has recently been shown to be an independent predictor of pulmonary exacerbation requiring hospitalization and antibiotics. However, the role of antibiotic treatment of Stenotrophomonas maltophilia infection in people with cystic fibrosis is still unclear...
2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27378490/active-cycle-of-breathing-technique-for-cystic-fibrosis
#13
REVIEW
Naomi A Mckoy, Lisa M Wilson, Ian J Saldanha, Olaide A Odelola, Karen A Robinson
BACKGROUND: People with cystic fibrosis experience chronic airway infections as a result of mucus build up within the lungs. Repeated infections often cause lung damage and disease. Airway clearance therapies aim to improve mucus clearance, increase sputum production, and improve airway function. The active cycle of breathing technique (also known as ACBT) is an airway clearance method that uses a cycle of techniques to loosen airway secretions including breathing control, thoracic expansion exercises, and the forced expiration technique...
July 5, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27348402/high-oxygen-delivery-to-preserve-exercise-capacity-in-patients-with-idiopathic-pulmonary-fibrosis-treated-with-nintedanib-methodology-of-the-hope-ipf-study
#14
Christopher J Ryerson, Pat G Camp, Neil D Eves, Michele Schaeffer, Nafeez Syed, Satvir Dhillon, Dennis Jensen, Francois Maltais, Denis E O'Donnell, Natya Raghavan, Michael Roman, Michael K Stickland, Deborah Assayag, Jean Bourbeau, Genevieve Dion, Charlene D Fell, Nathan Hambly, Kerri A Johannson, Meena Kalluri, Nasreen Khalil, Martin Kolb, Helene Manganas, Onofre Morán-Mendoza, Steve Provencher, Warren Ramesh, J Douglass Rolf, Pearce G Wilcox, Jordan A Guenette
RATIONALE: Pulmonary rehabilitation improves dyspnea and exercise capacity in idiopathic pulmonary fibrosis (IPF); however, it is unknown whether breathing high amounts of oxygen during exercise training leads to further benefits. OBJECTIVES: Herein, we describe the design of the High Oxygen Delivery to Preserve Exercise Capacity in IPF Patients Treated with Nintedanib study (the HOPE-IPF study). The primary objective of this study is to determine the physiological and perceptual impact of breathing high levels of oxygen during exercise training in patients with IPF who are receiving antifibrotic therapy...
September 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27320420/pulmonary-rehabilitation-in-individuals-with-non-cystic-fibrosis-bronchiectasis-a-systematic-review
#15
Annemarie L Lee, Catherine J Hill, Christine F McDonald, Anne E Holland
OBJECTIVE: To examine the effect of pulmonary rehabilitation (PR) (exercise and education) or exercise training (ET) on exercise capacity, health-related quality of life (HRQOL), symptoms, frequency of exacerbations, and mortality compared with no treatment in adults with bronchiectasis. DATA SOURCES: Computer-based databases were searched from their inception to February 2016. STUDY SELECTION: Randomized controlled trials of PR or ET versus no treatment in adults with bronchiectasis were included...
June 16, 2016: Archives of Physical Medicine and Rehabilitation
https://www.readbyqxmd.com/read/27298017/efficacy-and-safety-of-lumacaftor-ivacaftor-combination-therapy-in-patients-with-cystic-fibrosis-homozygous-for-phe508del-cftr-by-pulmonary-function-subgroup-a-pooled-analysis
#16
J Stuart Elborn, Bonnie W Ramsey, Michael P Boyle, Michael W Konstan, Xiaohong Huang, Gautham Marigowda, David Waltz, Claire E Wainwright
BACKGROUND: Lumacaftor/ivacaftor combination therapy has shown clinical benefits in patients with cystic fibrosis homozygous for the Phe508del CFTR mutation; however, pretreatment lung function is a confounding factor that potentially affects the efficacy and safety of this therapy. We aimed to assess the efficacy and safety of lumacaftor/ivacaftor therapy in these patients, defined by specific categories of lung function. METHODS: Both trials (TRAFFIC and TRANSPORT) included in this pooled analysis were multinational, randomised, double-blind, placebo-controlled, parallel-group, phase 3 studies...
August 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27267518/sino-nasal-inhalation-of-isotonic-versus-hypertonic-saline-6-0-in-cf-patients-with-chronic-rhinosinusitis-results-of-a-multicenter-prospective-randomized-double-blind-controlled-trial
#17
Jochen G Mainz, Ulrike Schumacher, Katja Schädlich, Julia Hentschel, Christiane Koitschev, Assen Koitschev, Joachim Riethmüller, Freerk Prenzel, Olaf Sommerburg, Bärbel Wiedemann, Doris Staab, Wolfgang Gleiber, Rainald Fischer, James F Beck, Christin Arnold
BACKGROUND: Chronic rhinosinusitis is a hallmark of Cystic fibrosis (CF) impairing the patients' quality of life and overall health. However, therapeutic options have not been sufficiently evaluated. Bronchial inhalation of mucolytic substances is a gold standard in CF therapy. Previously, we found that sinonasal inhalation of dornase alfa as vibrating aerosol reduces symptoms of chronic rhinosinusitis more effectively than NaCl 0.9% (net treatment benefit: -5.87±2.3 points, p=0.017; SNOT-20 total score)...
November 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27177012/a-safety-evaluation-of-pirfenidone-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#18
REVIEW
Adam Anderson, Adrian Shifren, Steven D Nathan
INTRODUCTION: Pirfenidone is a novel oral anti-fibrotic agent approved for the treatment of idiopathic pulmonary fibrosis (IPF). Since IPF is a chronic and progressive disease most commonly encountered in an older population, therapeutic options should be not only effective, but also free from drug interactions and as safe and tolerable as possible. AREAS COVERED: Comprehensive data from randomized controlled trials, meta-analyses, safety studies, and post-marketing data are available to assess the efficacy and safety of pirfenidone in the treatment of IPF...
July 2016: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/27161257/safety-and-tolerability-of-acetylcysteine-and-pirfenidone-combination-therapy-in-idiopathic-pulmonary-fibrosis-a-randomised-double-blind-placebo-controlled-phase-2-trial
#19
Jürgen Behr, Elisabeth Bendstrup, Bruno Crestani, Andreas Günther, Horst Olschewski, C Magnus Sköld, Athol Wells, Wim Wuyts, Dirk Koschel, Michael Kreuter, Benoît Wallaert, Chin-Yu Lin, Jürgen Beck, Carlo Albera
BACKGROUND: Oral acetylcysteine (also known as N-acetylcysteine) is used with pirfenidone to treat idiopathic pulmonary fibrosis (IPF) in Europe. However, no randomised studies have investigated the safety and tolerability of this combination. The PANORAMA study assessed the safety and tolerability of acetylcysteine combined with pirfenidone in patients with IPF. Exploratory efficacy endpoints were also assessed. METHODS: We did a double-blind randomised trial at 48 sites in eight countries...
June 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27149365/pilot-randomized-controlled-trial-evaluating-the-effect-of-hypertonic-saline-with-and-without-hyaluronic-acid-in-reducing-inflammation-in-cystic-fibrosis
#20
Anna Brivio, Massimo Conese, Simone Gambazza, Arianna Biffi, Amedea Silvia Tirelli, Maria Russo, Michaela Foà, Carla Colombo
BACKGROUND: Hypertonic saline (HS) has been established as a therapy aimed at restoring the surface liquid of airways liquid and enhancing mucociliary clearance in patients with cystic fibrosis (CF). A formula containing 7% HS and 0.1% hyaluronic acid (HA) is also available, basing its use on the protective effects of HA against elastin injury and on its greater ease of administration (i.e., the perceived acceptability of inhalation). This study explores the effect of HA+HS in reducing the inflammation of airways, by measuring cytokine levels in sputum, its safety profile, and the prevalence of commonly reported symptoms like cough, throat irritation, and saltiness...
December 2016: Journal of Aerosol Medicine and Pulmonary Drug Delivery
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