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Bronchiectasis

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19 papers 0 to 25 followers
https://www.readbyqxmd.com/read/28096286/diagnosis-of-primary-ciliary-dyskinesia-potential-options-for-resource-limited-countries
#1
REVIEW
Nisreen Rumman, Claire Jackson, Samuel Collins, Patricia Goggin, Janice Coles, Jane S Lucas
Primary ciliary dyskinesia is a genetic disease of ciliary function leading to chronic upper and lower respiratory tract symptoms. The diagnosis is frequently overlooked because the symptoms are nonspecific and the knowledge about the disease in the primary care setting is poor. Additionally, none of the available tests is accurate enough to be used in isolation. These tests are expensive, and need sophisticated equipment and expertise to analyse and interpret results; diagnosis is therefore only available at highly specialised centres...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28212153/new-therapies-for-the-prevention-and-treatment-of-exacerbations-of-bronchiectasis
#2
Hani Abo-Leyah, James D Chalmers
PURPOSE OF REVIEW: Exacerbations of bronchiectasis have a major impact on quality of life, healthcare costs, and long-term risk of complications. Preventing exacerbations is one of the major goals of treatment. Bronchiectasis is increasingly recognized and the impact of bronchiectasis exacerbations on daily clinical practice is also increasing. RECENT FINDINGS: Preventing bronchiectasis exacerbations are dependent on appropriate risk assessment, identifying the patients at highest risk to rationally target preventive therapies...
May 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28093268/bronchiectasis-rheumatoid-overlap-syndrome-bros-is-an-independent-risk-factor-for-mortality-in-patients-with-bronchiectasis-a-multicentre-cohort-study
#3
Anthony De Soyza, Melissa J McDonnell, Pieter C Goeminne, Stefano Aliberti, Sara Lonni, John Davison, Lieven J Dupont, Thomas C Fardon, Robert M Rutherford, Adam T Hill, James D Chalmers
INTRODUCTION: We studied if Bronchiectasis (BR) and Rheumatoid arthritis (RA) when manifesting as an overlap syndrome (BROS) was associated with worse outcomes than other BR aetiologies applying the Bronchiectasis Severity Index (BSI). METHODS: We interrogated the Bronchiectasis Severity Index (BSI) databases of 1716 patients across 6 centres: Edinburgh, UK (608 patients), Dundee, UK (N=286), Leuven, Belgium (N=253), Monza, Italy (N=201), Galway Ireland (N=242) and Newcastle, UK (N=126)...
January 13, 2017: Chest
https://www.readbyqxmd.com/read/28026118/mounier-kuhn-syndrome-tracheobronchomegaly-an-analysis-of-eleven-cases
#4
Recep Akgedik, Harun Karamanli, Deniz Kizilirmak, Ali Bekir Kurt, Hasan Öztürk, Berna Botan Yildirim, Lütfullah Çakir
BACKGROUND AND AIM: Mounier-Kuhn syndrome (MKS) is a congenital disorder characterized by tracheobronchomegaly resulting from the absence of elastic fibers in the trachea and main bronchi or atrophy and thinning of the smooth muscle layer. In this syndrome, dead space associated with tracheobronchomegaly increases and discharge of secretions decreases because of ineffective coughing. The most common complications are recurrent lower respiratory tract infections and bronchiectasis. We examined the clinical characteristics, radiological features, and related complications of patients with MKS...
December 27, 2016: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/27889361/adult-bronchiectasis-patients-a-first-look-at-the-united-states-bronchiectasis-research-registry
#5
Timothy R Aksamit, Anne E O'Donnell, Alan Barker, Kenneth N Olivier, Kevin L Winthrop, M Leigh Anne Daniels, Margaret Johnson, Edward Eden, David Griffith, Michael Knowles, Mark Metersky, Matthias Salathe, Byron Thomashow, Gregory Tino, Gerard Turino, Betsy Carretta, Charles L Daley
OBJECTIVE: We sought to describe the characteristics of adult bronchiectasis patients enrolled in the United States Bronchiectasis Research Registry (BRR). METHODS: The BRR is a database of non-cystic fibrosis bronchiectasis (NCFB) patients enrolled at 13 sites within the United States. Baseline demographic, spirometric, imaging, microbiologic, and therapeutic data were entered into a central web-based database. Patients were subsequently analyzed by the presence NTM...
November 23, 2016: Chest
https://www.readbyqxmd.com/read/27836958/european-respiratory-society-guidelines-for-the-diagnosis-of-primary-ciliary-dyskinesia
#6
Jane S Lucas, Angelo Barbato, Samuel A Collins, Myrofora Goutaki, Laura Behan, Daan Caudri, Sharon Dell, Ernst Eber, Estelle Escudier, Robert A Hirst, Claire Hogg, Mark Jorissen, Philipp Latzin, Marie Legendre, Margaret W Leigh, Fabio Midulla, Kim G Nielsen, Heymut Omran, Jean-Francois Papon, Petr Pohunek, Beatrice Redfern, David Rigau, Bernhard Rindlisbacher, Francesca Santamaria, Amelia Shoemark, Deborah Snijders, Thomy Tonia, Andrea Titieni, Woolf T Walker, Claudius Werner, Andrew Bush, Claudia E Kuehni
The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no "gold standard" reference test. Hence, a Task Force supported by the European Respiratory Society has developed this guideline to provide evidence-based recommendations on diagnostic testing, especially in light of new developments in such tests, and the need for robust diagnoses of patients who might enter randomised controlled trials of treatments...
January 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27820964/long-term-antibiotic-treatment-for-non-cystic-fibrosis-bronchiectasis-in-adults-evidence-current-practice-and-future-use
#7
N Goldman, M R Loebinger, R Wilson
Bronchiectasis is defined pathologically by the permanent dilation of the bronchi and bronchioles and chronic airway inflammation. This is the outcome of a number of different aetiologies but up to half of bronchiectasis cases are labelled idiopathic. It is characterised by a chronic productive cough, haemoptysis, shortness of breath and recurrent infective exacerbations. Long-term antibiotics are used with the aim of reducing symptom severity and exacerbation frequency, improving quality of life and preventing disease progression...
December 2016: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/27680603/clinical-phenotype-and-current-diagnostic-criteria-for-primary-ciliary-dyskinesia
#8
Eleonora Dehlink, Claire Hogg, Siobhan B Carr, Andrew Bush
Primary ciliary dyskinesia (PCD) is a rare, mostly autosomal-recessive disorder of motile cilia, characterized by chronic lung disease, rhinosinusitis, hearing impairment, and subfertility. PCD is still often missed or diagnosed late since symptoms overlap with common respiratory complaints, but should be considered if one or more of the cardinal clues are present. Areas covered: We provide an overview on clinical presentations of PCD and clues for when to consider PCD, these include unexplained neonatal respiratory distress, persistent rhinitis from the first days of life, situs anomalies, or otorrhoea following tympanostomy tube insertion...
October 19, 2016: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/27591924/macrolides-for-clinically-significant-bronchiectasis-in-adults-who-should-receive-this-treatment
#9
Adam T Hill
Long-term macrolide therapy offers an evidence-based treatment to reduce frequent exacerbations in stable adult patients with bronchiectasis. There is limited evidence that these agents also attenuate the decline in lung function and improve health-related quality of life. The benefits and risks of long-term macrolide use need to be clearly explored for individual patients. Further work is needed to understand the optimal drug, dose, and regimen, the mechanisms behind these benefits, appropriate patient selection, sustainability of efficacy, potential long-term risk for the lung microbiome; and their use with or without inhaled antibiotic treatment...
December 2016: Chest
https://www.readbyqxmd.com/read/27492530/challenges-in-managing-pseudomonas-aeruginosa-in-non-cystic-fibrosis-bronchiectasis
#10
REVIEW
Robert Wilson, Timothy Aksamit, Stefano Aliberti, Anthony De Soyza, J Stuart Elborn, Pieter Goeminne, Adam T Hill, Rosario Menendez, Eva Polverino
BACKGROUND: An Expert Forum was held at the 2014 European Respiratory Society International Congress to address issues involved in the management of Pseudomonas aeruginosa infection in patients with non-cystic fibrosis bronchiectasis (NCFB). Multiple studies have found that chronic P. aeruginosa infection is associated with more severe disease and higher morbidity and mortality. OVERVIEW: Participants discussed appropriate management of P. aeruginosa infection at three stages: 1) first isolation, including eradication protocols; 2) during exacerbations; and 3) during chronic infection, including long-term antibiotic therapy to reduce the severity of symptoms and frequency of exacerbations...
August 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27514592/primary-ciliary-dyskinesia
#11
REVIEW
Michael R Knowles, Maimoona Zariwala, Margaret Leigh
Primary ciliary dyskinesia (PCD) is a recessive genetically heterogeneous disorder of motile cilia with chronic otosinopulmonary disease and organ laterality defects in ∼50% of cases. The prevalence of PCD is difficult to determine. Recent diagnostic advances through measurement of nasal nitric oxide and genetic testing has allowed rigorous diagnoses and determination of a robust clinical phenotype, which includes neonatal respiratory distress, daily nasal congestion, and wet cough starting early in life, along with organ laterality defects...
September 2016: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/27296824/diagnostic-challenges-of-bronchiectasis
#12
REVIEW
Guillermo Suarez-Cuartin, James D Chalmers, Oriol Sibila
Bronchiectasis is a condition of increasing incidence and prevalence around the world. Many different diseases have been associated with bronchiectasis, and their treatment can differ widely. Recent guidelines have helped to approach aetiological diagnosis but it is still a complex process. Identifying the cause of the bronchiectasis may determine a change in the treatment of a large group of subjects. That is one of the main reasons why the aetiological diagnosis is crucial in the proper management of bronchiectasis patients...
July 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27321896/aetiology-of-bronchiectasis-in-adults-a-systematic-literature-review
#13
Yong-Hua Gao, Wei-Jie Guan, Shao-Xia Liu, Lei Wang, Juan-Juan Cui, Rong-Chang Chen, Guo-Jun Zhang
While identifying the underlying aetiology is a key part of bronchiectasis management, the prevalence and impact of identifying the aetiologies on clinical management remain unclear. We aimed to determine the etiological spectrum of bronchiectasis, and how often etiological assessment could lead to the changes in patients' management. A comprehensive search was conducted using MEDLINE (via PubMed) and EMBASE for observational studies published before October 2015 reporting aetiologies in adults with bronchiectasis...
June 19, 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/27076584/asthma-and-bronchiectasis-exacerbation
#14
Bei Mao, Jia-Wei Yang, Hai-Wen Lu, Jin-Fu Xu
Bronchiectasis and asthma are common respiratory diseases worldwide. However, the influence of asthma on bronchiectasis remains unclear. The objective of this study is to analyse the effects of asthma on bronchiectasis exacerbation.Data from inpatients diagnosed with bronchiectasis with or without asthma at Shanghai Pulmonary Hospital (Shanghai, China) between January 2013 and December 2014 were retrospectively collected and analysed. 249 patients with only bronchiectasis and 214 patients with both bronchiectasis and asthma were included in the study...
June 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/26967669/primary-ciliary-dyskinesia-and-associated-sensory-ciliopathies
#15
REVIEW
Amjad Horani, Thomas W Ferkol
Primary ciliary dyskinesia (PCD) is a genetic disease of motile cilia, which belongs to a group of disorders resulting from dysfunction of cilia, collectively known as ciliopathies. Insights into the genetics and phenotypes of PCD have grown over the last decade, in part propagated by the discovery of a number of novel cilia-related genes. These genes encode proteins that segregate into structural axonemal, regulatory, as well as cytoplasmic assembly proteins. Our understanding of primary (sensory) cilia has also expanded, and an ever-growing list of diverse conditions has been linked to defective function and signaling of the sensory cilium...
2016: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/26857775/using-cystic-fibrosis-therapies-for-non-cystic-fibrosis-bronchiectasis
#16
REVIEW
Wael ElMaraachli, Douglas J Conrad, Angela C C Wang
Non-cystic fibrosis bronchiectasis (NCFB) is an increasingly prevalent disease that places a significant burden on patients and health systems globally. Although many of the therapies used to treat NCFB were originally developed as cystic fibrosis (CF) therapies, not all of them have been demonstrated to be efficacious in NCFB and some may even be harmful. This article explores the evidence for which therapeutic strategies used to treat CF have been translated into the care of NCFB. The conclusion is that therapies for adult NCFB cannot be simply extrapolated from CF clinical trials, and in some instances, doing so may actually result in harm...
March 2016: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/26846833/clinical-phenotypes-in-adult-patients-with-bronchiectasis
#17
MULTICENTER STUDY
Stefano Aliberti, Sara Lonni, Simone Dore, Melissa J McDonnell, Pieter C Goeminne, Katerina Dimakou, Thomas C Fardon, Robert Rutherford, Alberto Pesci, Marcos I Restrepo, Giovanni Sotgiu, James D Chalmers
Bronchiectasis is a heterogeneous disease. This study aimed at identifying discrete groups of patients with different clinical and biological characteristics and long-term outcomes.This was a secondary analysis of five European databases of prospectively enrolled adult outpatients with bronchiectasis. Principal component and cluster analyses were performed using demographics, comorbidities, and clinical, radiological, functional and microbiological variables collected during the stable state. Exacerbations, hospitalisations and mortality during a 3-year follow-up were recorded...
April 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/26839727/humoral-immunity-in-bronchiectasis-finding-good-s-syndrome
#18
C Pu, S Sukhal, S Fakhran
We present a case of a 37-year-old man with a past history of a surgically removed thymoma, who presented with recurrent pulmonary infections and bronchiectasis. On further testing, he was found to have low total immunoglobulin levels, a constellation of findings known as Good's syndrome. He responded well to immunoglobulin replacement, in addition to the usual treatments for bronchiectasis. We present this case to emphasize the association of bronchiectasis, low immunoglobulins, and thymomas and the role of immunoglobulin replacement as a treatment option...
2015: Case Reports in Pulmonology
https://www.readbyqxmd.com/read/26591003/airway-clearance-techniques-for-bronchiectasis
#19
REVIEW
Annemarie L Lee, Angela T Burge, Anne E Holland
BACKGROUND: People with non-cystic fibrosis bronchiectasis commonly experience chronic cough and sputum production, features that may be associated with progressive decline in clinical and functional status. Airway clearance techniques (ACTs) are often prescribed to facilitate expectoration of sputum from the lungs, but the efficacy of these techniques in a stable clinical state or during an acute exacerbation of bronchiectasis is unclear. OBJECTIVES: Primary: to determine effects of ACTs on rates of acute exacerbation, incidence of hospitalisation and health-related quality of life (HRQoL) in individuals with acute and stable bronchiectasis...
November 23, 2015: Cochrane Database of Systematic Reviews
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