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Non-cf bronchiectasis

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https://www.readbyqxmd.com/read/29678900/prevention-of-exacerbations-in-patients-with-stable-non-cystic-fibrosis-bronchiectasis-a-systematic-review-and-meta-analysis-of-pharmacological-and-non-pharmacological-therapies
#1
Abd Moain Abu Dabrh, Adam T Hill, Claudia C Dobler, Noor Asi, Wigdan H Farah, Qusay Haydour, Zhen Wang, Khalid Benkhadra, Larry J Prokop, Mohammad Hassan Murad
BACKGROUND: Several pharmacological and non-pharmacological therapies are used to treat stable bronchiectasis of non-cystic fibrosis (CF) aetiology. OBJECTIVE: We conducted a systematic review and meta-analysis to assess the evidence of the effectiveness of pharmacological and non-pharmacological treatment options in patients with stable non-CF bronchiectasis with a focus on reducing exacerbations. STUDY SELECTION: Multiple databases were searched through September 2017...
April 20, 2018: BMJ evidence-based medicine
https://www.readbyqxmd.com/read/29621625/ciprofloxacin-dry-powder-for-inhalation-ciprofloxacin-dpi-technical-design-and-features-of-an-efficient-drug-device-combination
#2
REVIEW
Pamela J McShane, Jeffry G Weers, Thomas E Tarara, Alfred Haynes, Preeti Durbha, Danforth P Miller, Tobias Mundry, Elisabeth Operschall, J Stuart Elborn
Bronchiectasis is a chronic respiratory disease with heterogeneous etiology, characterized by a cycle of bacterial infection and inflammation, resulting in increasing airway damage. Exacerbations are an important cause of morbidity and are strongly associated with disease progression. Many patients with bronchiectasis suffer from two or more exacerbations per year. However, there are no approved therapies to reduce or delay exacerbations in this patient population. Ciprofloxacin DPI is in development as a long-term, intermittent therapy to reduce exacerbations in patients with non-cystic fibrosis (CF) bronchiectasis and evidence of respiratory pathogens...
April 2, 2018: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29605210/a-review-of-the-etiology-and-clinical-presentation-of-non-cystic-fibrosis-bronchiectasis-a-tertiary-care-experience
#3
Ozlem Satırer, Ayse Mete Yesil, Nagehan Emiralioglu, Gökcen Dilsa Tugcu, Ebru Yalcın, Deniz Dogru, Nural Kiper, Ugur Ozcelik
INTRODUCTION: Non-cystic fibrosis(CF) bronchiectasis has been recognized in children for the past 200 years. Early childhood pneumonia and underlying conditions such as immunodeficiency, primary ciliary dyskinesia(PCD), and congenital lung pathology should be considered in the etiology. The aim of our study was to describe the clinical characteristics, laboratory, and radiological findings of a large population of patients with non-CF bronchiectasis at a tertiary center. METHODS: We analyzed the clinical findings of 187 patients diagnosed with non-CF bronchiectasis over a period of 10 years (January 2005-December 2015) at the Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology...
April 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29588830/allergic-bronchopulmonary-aspergillosis-in-patients-with-cystic-fibrosis-and-non-cystic-fibrosis-bronchiectasis
#4
Soheila Alyasin, Mozhgan Moghtaderi, Shirin Farjadian, Maryam Babaei, Saeed Hosseini Teshnizi
Background: Aspergillus sensitization (AS) and allergic bronchopulmonary aspergillosis (ABPA) can occur as a cause of permanent lung damage in patients with cystic fibrosis (CF) and non-CF bronchiectasis. Objective: The aim of this study was to determine the frequency of AS and ABPA in patients with CF and non-CF bronchiectasis in southwestern Iran. Methods: This cross-sectional study was conducted on 33 patients with CF and 27 patients with non-CF bronchiectasis from southwestern Iran who were referred to Namazi Hospital affiliated to Shiraz University of Medical Sciences from July 2015 to February 2016...
January 2018: Electronic Physician
https://www.readbyqxmd.com/read/29587336/oral-versus-inhaled-antibiotics-for-bronchiectasis
#5
REVIEW
Sally Spencer, Lambert M Felix, Stephen J Milan, Rebecca Normansell, Pieter C Goeminne, James D Chalmers, Tim Donovan
BACKGROUND: Bronchiectasis is a chronic inflammatory disease characterised by a recurrent cycle of respiratory bacterial infections associated with cough, sputum production and impaired quality of life. Antibiotics are the main therapeutic option for managing bronchiectasis exacerbations. Evidence suggests that inhaled antibiotics may be associated with more effective eradication of infective organisms and a lower risk of developing antibiotic resistance when compared with orally administered antibiotics...
March 27, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29435737/clinical-determinants-of-incremental-shuttle-walk-test-in-adults-with-bronchiectasis
#6
Sulenur Yildiz, Deniz Inal-Ince, Ebru Calik-Kutukcu, Naciye Vardar-Yagli, Melda Saglam, Hulya Arikan, Lutfi Coplu
INTRODUCTION: Exercise capacity is impaired in patients with bronchiectasis. Incremental shuttle walk test (ISWT) stresses cardiorespiratory system physiologically to symptom-limited maximal exercise capacity. The purpose of this study was to investigate the clinical determinants of ISWT in adults with non-cystic fibrosis (CF) bronchiectasis. METHODS: Forty-one clinically stable bronchiectasis patients aged 18-72 years (27 females, 14 males) participated in the study...
February 12, 2018: Lung
https://www.readbyqxmd.com/read/29355548/treating-cough-due-to-non-cf-and-cf-bronchiectasis-with-nonpharmacological-airway-clearance-chest-expert-panel-report
#7
Adam T Hill, Alan F Barker, Donald C Bolser, Paul Davenport, Belinda Ireland, Anne B Chang, Stuart B Mazzone, Lorcan McGarvey
BACKGROUND: In bronchiectasis due to cystic fibrosis (CF) and other causes, airway clearance is one of the mainstays of management. We conducted a systematic review on airway clearance by using non-pharmacological methods as recommended by international guidelines to develop recommendations or suggestions to update the 2006 CHEST guideline on cough. METHODS: The systematic search for evidence examined the question, "Is there evidence of clinically important treatment effects for non-pharmacological therapies in cough treatment for patients with bronchiectasis?" Populations selected were all patients with bronchiectasis due to CF or non-CF bronchiectasis...
April 2018: Chest
https://www.readbyqxmd.com/read/29338445/investigational-inhaled-therapies-for-non-cf-bronchiectasis
#8
REVIEW
Sabina Antonela Antoniu
Bronchiectasis not related to cystic fibrosis (non-CF bronchiectasis) are associated with a high unmet therapeutic need due to the lack of specifically authorized medications, especially via the inhalation route. In non-CF bronchiectasis chronic infection with Pseudomonas aeruginosa is common and favored by the persistent local inflammation and viscid sputum production. Therefore inhaled antibiotics, mucolytics or anti-inflammatory agents could represent appropriate therapeutic interventions in this setting Areas covered: This review herein discusses the inhaled therapies currently under investigation for non-CF bronchiectasis and their potential therapeutic positioning in exacerbation versus stable state...
February 2018: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/29327941/infection-is-not-required-for-mucoinflammatory-lung-disease-in-cftr-knockout-ferrets
#9
Bradley H Rosen, T Idil Apak Evans, Shashanna R Moll, Jaimie S Gray, Bo Liang, Xingshen Sun, Yulong Zhang, Chandler W Jensen-Cody, Anthony M Swatek, Weihong Zhou, Nan He, Pavana G Rotti, Scott R Tyler, Nicholas W Keiser, Preston J Anderson, Leonard Brooks, Yalan Li, R Marshall Pope, Maheen Rajput, Eric A Hoffman, Kai Wang, J Kirk Harris, Kalpaj R Parekh, Katherine N Gibson-Corley, John F Engelhardt
RATIONALE: Classical interpretation of cystic fibrosis (CF) lung disease pathogenesis suggests that infection initiates disease progression, leading to an exuberant inflammatory response, excessive mucus, and ultimately bronchiectasis. While symptomatic antibiotic treatment controls lung infections early in disease, life-long bacterial residence typically ensues. Processes that control the establishment of persistent bacteria in the CF lung, and the contribution of non-infectious components to disease pathogenesis, are poorly understood...
January 12, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29303285/-non-cf-bronchiectasis-of-adults-short-review-for-clinical-practice-position-paper-of-board-of-disease-with-bronchial-obstruction-czech-pulmonological-and-phthiseological-society-czech-medical-association-of-j-e-purkyne
#10
Zuzana Antušová, Libor Fila, Vladimír Herout, Eva Kočová, Kateřina Neumannová, Jaromír Zatloukal, Vladimír Koblížek
Bronchiectasis is a clinically important, but poorly understood, pulmonary condition characterized by dilated and thick-walled bronchi. Bronchiectasis remains a significant cause of morbidity and mortality around the world. Targeted effort to early high-resolution computed tomography diagnosis and detailed confirmation of causation are in the spotlight of respiratory physicians in the developed countries. The risk population consists of subjects with persistent and/or productive cough, where another clear diagnosis has not been performed...
December 0: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29221293/distance-saturation-product-of-the-6-minute-walk-test-predicts-mortality-of-patients-with-non-cystic-fibrosis-bronchiectasis
#11
Meng-Heng Hsieh, Yueh-Fu Fang, Fu-Tsai Chung, Chung-Shu Lee, Yu-Chen Chang, Yuan-Zhang Liu, Cheng-Hsien Wu, Horng-Chyuan Lin
Background: Previous surveillance methods to monitor the prognoses of patients with bronchiectasis are too complex for use in daily practice. The 6-minute walk test (6MWT) is a simple exercise test to predict the prognosis of chronic obstructive airway disease and numerous chronic lung diseases, including idiopathic pulmonary fibrosis. No studies have investigated exercise-induced oxygen desaturation (EID) and distance-saturation product (DSP) of 6MWT to predict the prognoses of patients with bronchiectasis...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29132121/the-therapeutic-potential-of-cftr-modulators-for-copd-and-other-airway-diseases
#12
REVIEW
George M Solomon, Lianwu Fu, Steven M Rowe, James F Collawn
Airways diseases, especially chronic obstructive pulmonary disease (COPD) and asthma, are common causes of morbidity and mortality worldwide. There is an ongoing unmet need for novel and effective therapies. There is an established pathophysiological link and phenotypic similarity between the chronic bronchitis phenotype of COPD and cystic fibrosis (CF). New evidence suggests that CFTR dysfunction may play a role in other common airways diseases such as COPD, non-atopic asthma and non-CF bronchiectasis. Newly approved and investigational drugs that target both mutant and wild-type CFTR channels have provided a new treatment opportunity addressing the mucus defect in pulmonary diseases that share the same pathophysiology with CF...
June 2017: Current Opinion in Pharmacology
https://www.readbyqxmd.com/read/29094263/exophiala-dermatitidis-revealing-cystic-fibrosis-in-adult-patients-with-chronic-pulmonary-disease
#13
Frédéric Grenouillet, Bernard Cimon, Heloise Pana-Katatali, Christine Person, Marie Gainet-Brun, Marie-Claire Malinge, Yohann Le Govic, Bénédicte Richaud-Thiriez, Jean-Philippe Bouchara
Cystic fibrosis (CF) is a genetic inherited disease due to mutations in the gene cystic fibrosis transmembrane conductance regulator (CFTR). Because of the huge diversity of CFTR mutations, the CF phenotypes are highly heterogeneous, varying from typical to mild form of CF, also called atypical CF. These atypical features are more frequently diagnosed at adolescence or adulthood, and among clinical signs and symptoms leading to suspect a mild form of CF, colonization or infection of the respiratory tract due to well-known CF pathogens should be a warning signal...
February 2018: Mycopathologia
https://www.readbyqxmd.com/read/29077527/the-rationale-and-evidence-for-use-of-inhaled-antibiotics-to-control-pseudomonas-aeruginosa-infection-in-non-cystic-fibrosis-bronchiectasis
#14
Rajiv Dhand
Non-cystic fibrosis bronchiectasis (NCFBE) is a chronic inflammatory lung disease characterized by irreversible dilation of the bronchi, symptoms of persistent cough and expectoration, and recurrent infective exacerbations. The prevalence of NCFBE is on the increase in the United States and Europe, but no licensed therapies are currently available for its treatment. Although there are many similarities between NCFBE and cystic fibrosis (CF) in terms of respiratory symptoms, airway microbiology, and disease progression, there are key differences, for example, in response to treatment, suggesting differences in pathogenesis...
October 27, 2017: Journal of Aerosol Medicine and Pulmonary Drug Delivery
https://www.readbyqxmd.com/read/28982600/oxidative-stress-in-early-cystic-fibrosis-lung-disease-is-exacerbated-by-airway-glutathione-deficiency
#15
Nina Dickerhof, John F Pearson, Teagan S Hoskin, Luke J Berry, Rufus Turner, Peter D Sly, Anthony J Kettle
Neutrophil-derived myeloperoxidase (MPO) is recognized as a major source of oxidative stress at the airway surface of a cystic fibrosis (CF) lung where, despite limited evidence, the antioxidant glutathione is widely considered to be low. The aims of this study were to establish whether oxidative stress or glutathione status are associated with bronchiectasis and whether glutathione deficiency is inherently linked to CF or a consequence of oxidative stress. MPO was measured by ELISA in 577 bronchoalveolar lavage samples from 205 clinically-phenotyped infants and children with CF and 58 children without CF (ages 0...
October 2, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28866642/the-quantitative-link-of-lung-clearance-index-to-bronchial-segments-affected-by-bronchiectasis
#16
Sylvia Verbanck, Gregory G King, Wenxiao Zhou, Anne Miller, Cindy Thamrin, Daniel Schuermans, Bart Ilsen, Caroline W Ernst, Johan de Mey, Walter Vincken, Eef Vanderhelst
In adult patients with cystic fibrosis (CF), the lung clearance index (LCI) derived from the multiple breath washout relates to both acinar and conductive ventilation heterogeneity. The latter component predicts an association between LCI and the number of bronchial segments affected by bronchiectasis. Here, we experimentally demonstrated this association in patients with CF, and also examined an ancillary group of patients with non-CF bronchiectasis. We conclude that lung disease severity in terms of number of bronchial segments results in an associated LCI increase, likely constituting a portion of LCI that cannot be reversed by treatment in patients with CF lung disease...
September 2, 2017: Thorax
https://www.readbyqxmd.com/read/28863927/the-role-of-non-invasive-modalities-for-assessing-inflammation-in-patients-with-non-cystic-fibrosis-bronchiectasis
#17
Stamatoula Tsikrika, Katerina Dimakou, Andriana I Papaioannou, Georgios Hillas, Loukas Thanos, Kostantinos Kostikas, Stelios Loukides, Spyros Papiris, Nickolaos Koulouris, Petros Bakakos
INTRODUCTION: Bronchiectasis is a heterogeneous entity, taking into account clinical characteristics, inflammatory response, effectiveness of treatment and frequency of exacerbations. In stable state non-cystic fibrosis (non-CF) bronchiectasis, little is known about non-invasive techniques used for evaluating airway inflammation in obstructive airway diseases. OBJECTIVES: We sought to evaluate the associations between induced sputum and clinical/radiologic characteristics, and the differences between biomarkers expressing Th1 and Th2 response in patients with non-CF bronchiectasis and to compare our findings with a previously studied population of patients with asthma and COPD...
November 2017: Cytokine
https://www.readbyqxmd.com/read/28844058/molecular-epidemiology-of-pseudomonas-aeruginosa-in-an-unsegregated-bronchiectasis-cohort-sharing-hospital-facilities-with-a-cystic-fibrosis-cohort
#18
Philip J Mitchelmore, Joanna Randall, Matthew J Bull, Karen A Moore, Paul A O'Neill, Konrad Paszkiewicz, Eshwar Mahenthiralingam, Chris J Scotton, Christopher D Sheldon, Nicholas J Withers, Alan R Brown
While Pseudomonas aeruginosa (PA) cross-infection is well documented among patients with cystic fibrosis (CF), the equivalent risk among patients with non-CF bronchiectasis (NCFB) is unclear, particularly those managed alongside patients with CF. We performed analysis of PA within a single centre that manages an unsegregated NCFB cohort alongside a segregated CF cohort. We found no evidence of cross-infection between the two cohorts or within the segregated CF cohort. However, within the unsegregated NCFB cohort, evidence of cross-infection was found between three (of 46) patients...
August 26, 2017: Thorax
https://www.readbyqxmd.com/read/28828295/role-of-vitamin-d-in-cystic-fibrosis-and-non-cystic-fibrosis-bronchiectasis
#19
REVIEW
Maria Moustaki, Ioanna Loukou, Kostas N Priftis, Konstantinos Douros
Bronchiectasis is usually classified as cystic fibrosis (CF) related or CF unrelated (non-CF); the latter is not considered an orphan disease any more, even in developed countries. Irrespective of the underlying etiology, bronchiectasis is the result of interaction between host, pathogens, and environment. Vitamin D is known to be involved in a wide spectrum of significant immunomodulatory effects such as down-regulation of pro-inflammatory cytokines and chemokines. Respiratory epithelial cells constitutively express 1α-hydroxylase leading to the local transformation of the inactive 25(OH)-vitamin D to the active 1,25(OH)2-vitamin D...
August 8, 2017: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/28540765/an-update-on-pediatric-bronchiectasis
#20
Danielle F Wurzel, Anne B Chang
The prevalence and awareness of bronchiectasis not related to cystic fibrosis (CF) is increasing and it is now recognized as a major cause of respiratory morbidity, mortality and healthcare utilization worldwide. The need to elucidate the early origins of bronchiectasis is increasingly appreciated and has been identified as an important research priority. Current treatments for pediatric bronchiectasis are limited to antimicrobials, airway clearance techniques and vaccination. Several new drugs targeting airway inflammation are currently in development...
June 5, 2017: Expert Review of Respiratory Medicine
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