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pulmonary fibrosis randomized controlled trial

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
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
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
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...
2016: BMC Pulmonary Medicine
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
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
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
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
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
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)...
June 4, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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
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
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
Hiroyuki Taniguchi, Yasuhiro Kondoh
Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs...
July 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
Giovanni Ferrara, Lisa Carlson, Andreas Palm, Jonas Einarsson, Cecilia Olivesten, Magnus Sköld
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is an emerging problem in the western world, being related to increasing age and implying significant costs for the diagnosis and management of affected patients. The epidemiology of IPF is not well understood. METHODS: To allow estimates of the problem and eventually to evaluate quality of the care of IPF patients in Sweden, a national IPF Registry was started in the autumn of 2014. Data on criteria used to diagnose IPF, demographics, lung function, and quality of life (measured with the King's Brief Interstitial Lung Disease Questionnaire, K-BILD) were reported directly to the registry, based at the coordinating centre (Karolinska University Hospital, Stockholm, Sweden) via a web-based platform...
2016: European Clinical Respiratory Journal
Caitlyn T Solem, Montserrat Vera-Llonch, Sizhu Liu, Marc Botteman, Brenda Castiglione
BACKGROUND: The analysis aimed to examine the impact of pulmonary exacerbations (PEs) and lung function on generic measures of HRQL in patients with cystic fibrosis (CF) using trial-based data. METHODS: In a 48-week randomized, placebo-controlled study of ivacaftor in patients ≥12 years with CF and a G551D-CFTR mutation the relationship between PEs, PE-related hospitalizations and percent predicted forced expiratory volume in one second (ppFEV1) with EQ-5D measures (index and visual analog scale [VAS]) was examined in post-hoc analyses...
April 21, 2016: Health and Quality of Life Outcomes
Gary M Onady, Adrienne Stolfi
BACKGROUND: The Cystic Fibrosis Foundation recommends both short-term and long-acting insulin therapy when cystic fibrosis-related diabetes has been diagnosed. Diagnosis is based on: an elevated fasting blood glucose level greater than 6.94 mmol/liter (125 mg/deciliter); or oral glucose tolerance tests greater than 11.11 mmol/liter (200 mg/deciliter) at two hours; or symptomatic diabetes for random glucose levels greater than 11.11 mmol/liter (200 mg/deciliter); or glycated hemoglobin levels of at least 6...
April 18, 2016: Cochrane Database of Systematic Reviews
Els H Vandecasteele, Michel De Pauw, Guy Brusselle, Saskia Decuman, Yves Piette, Filip De Keyser, V Smith
Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by vasculopathy and progressive fibrosis of the skin and visceral organs (gastrointestinal tract, heart, kidneys and lungs). Although the prevalence is low, SSc is a disease with high morbidity and mortality. Since pulmonary arterial hypertension (PAH) associated with SSc (SSc-PAH) and clinically evident cardiac involvement is associated with increased mortality, the cardiac complications and PAH in SSc are reviewed. Both diffuse cutaneous (DcSSc) and limited cutaneous (LcSSc) subgroups are at risk for cardiac involvement and SSc-PAH...
February 2016: Acta Clinica Belgica
Larry C Lands, Sanja Stanojevic
BACKGROUND: Progressive lung damage causes most deaths in cystic fibrosis. Non-steroidal anti-inflammatory drugs (such as ibuprofen) may prevent progressive pulmonary deterioration and morbidity in cystic fibrosis. OBJECTIVES: To assess the effectiveness of treatment with non-steroidal anti-inflammatory drugs in cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, hand searches of relevant journals and abstract books of conference proceedings...
April 7, 2016: Cochrane Database of Systematic Reviews
Marcela Junqueira Brunelli, Álvaro N Atallah, Edina M K da Silva
BACKGROUND: Mucopolysaccharidosis type VI or Maroteaux-Lamy syndrome is a rare genetic disorder caused by the deficiency of arylsulphatase B. The resultant accumulation of dermatan sulphate causes lysosomal damage.The clinical symptoms are related to skeletal dysplasia (i.e. short stature and degenerative joint disease). Other manifestations include cardiac disease, impaired pulmonary function, ophthalmological complications, hepatosplenomegaly, sinusitis, otitis, hearing loss and sleep apnea...
2016: Cochrane Database of Systematic Reviews
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