Role of Innate Immunity in Non-Cystic Fibrosis Bronchiectasis
BronchiectasisThe purpose of this study is to demonstrate that 1) the secretion of mucins, antimicrobial peptides and Toll-like receptors is altered in certain patients with non-CF bronchiectasis, which makes them more susceptible to be infected by potentially pathogenic bacteria (PPB); and 2) the electronic nose is able to detect patterns of specific Volatile Organic Compounds (VOC) for patients with non-CF bronchiectasis colonized by PPB.
The PROspective German NOn-CF bronchiectaSIS Patient Registry
BronchiectasisThe purpose of this study is to study the epidemiology of non-CF bronchiectasis (NCFB) and to provide an estimate of the distribution (prevalence) of NCFB etiologies across all different levels of health care as well as real-life data regarding the current management of NCFB in Germany.
WGS of Korean Idiopathic Bronchiectasis
Bronchiectasis IdiopathicCystic Fibrosis1 moreWhole genome sequencing of Korean patients with idiopathic bronchiectasis and their family will perform to identify disease-causing variants.
Role of Pseudomonas Aeruginosa Biofilms in Exacerbations in Patients With Bronchiectasis With and...
BronchiectasisMicrobial Colonization2 moreExacerbations, in particular during chronic Pseudomonas aeruginosa (PA) infection, are very important in the prognosis of patients with non-cystic fibrosis bronchiectasis (BE). In Cystic Fibrosis patients, PA biofilms are associated with chronic respiratory infections and are the primary cause of their increased morbidity and mortality. However, the presence and role in exacerbations of PA biofilms, microbiome dysbiosis and inflammatory biomarkers has not been studied in depth in BE patients. Our aim is to determine the association between PA chronic infection and its biofilms with the number of exacerbations in the next year (primary outcome), time until next exacerbation, quality of life, FEV1 and inflammatory biomarkers (secondary outcomes) in BE patients with or without chronic obstructive pulmonary disease (COPD). The investigators will include and follow up during 12 months post study inclusion, 48 patients with BE and 48 with BE-COPD, with a positive sputum culture of PA. During stability and follow up (and in each exacerbation) The investigators will collect 4 sputum, 4 serum samples, perform spirometry, and quality of life tests every three months. For the biomarkers subproject, 4 additional serum samples will be collected at: exacerbation, 3-5 days after treatment, at 30 days and three months post-exacerbation. Biomarkers will be measured by commercial kits and Luminex. The investigators will quantify PA colony forming units (CFU)/mL, their resistance pattern, their mutation frequency and isolate mucoid and non-mucoid colonies. In each sputum, the investigators will analyze by Confocal Laser Scanning Microscopy (CLSM) and Fluorescent in situ Hybridizatrion (FISH) PA biofilms, their size, bacterial density and their in situ growth rate. Specific serum antibodies against PA will be determined through Crossed Immunoelectrophoresis. In addition, the investigators will indentify potential respiratory microbiome and gene expression patterns predictive for exacerbations, or with a protective role against chronic PA infection, as well as their association with biofilms. Microbiome analysis will be performed through the Illumina Miseq platform. Finally, the investigators will explore the antimicrobial activity of novel combinations of antibiotics against PA, both in in vitro planktonic cultures and in a biofilm model, and will include testing of antibiotic-containing alginate nanoparticles.
Exercise Capacity in Patients With Cystic Fibrosis vs. Non-cystic Fibrosis Bronchiectasis
BronchiectasisIntroduction: Bronchiectasis is a chronic lung disease in which the underlying condition causes permanent damage to the conducting airways. Bronchiectasis is associated with considerable morbidity and poor quality of life. While cystic fibrosis (CF) is the most common cause of bronchiectasis in childhood, non-CF bronchiectasis is associated with a wide variety of disorders. CF bronchiectasis patients show reduced daily habitual physical activity and exercise capacity. Cardiopulmonary exercise test (CPET) is increasingly gaining importance in clinical medicine and considered the gold standard exercise test for assessing aerobic exercise capacity. The test objectively evaluates exercise physiological functions, may help assess morbidity and predict the outcome and mortality in different clinical circumstances and may serve as a basis for individualized exercise prescription within the limitation of the disease. Unlike CPET in CF, there is a paucity of data on exercise capacity using CPET in non- CF bronchiectasis patients, and on the implications of physical activity on non- CF bronchiectasis morbidity and mortality. Aim: To evaluate and compare exercise capacity in CF and non-CF bronchiectasis patients. Methods: This will be a cross-sectional retrospective/prospective study population. The retrospective study will include data analysis of patients that preformed CPET as part of their clinical evaluation. In the prospective study, patients that are scheduled to perform CPET as part of their clinical evaluation will sign (or legal guardian) informed consent prior to participation. Patients will be recruited from the exercise clinic at the Pediatric Pulmonary Institute at the Rappaport Children's Hospital. Inclusion criteria: 1. Children and adults (age >7 years, height >125cm), with CF and non CF bronchiectasis. 2. Completed a maximal CPET test according to accepted criteria; (maximal VO2 > 80% predicted, maximal heart rate > 80% predicted, acceptable RER (RER > 1.0 in children (under 18 years), RER > 1.05 for adults) or reaching a VO2 plateau..3. Evidence of bronchiectasis in computed tomography (CT). Exclusions criteria: preforming submaximal CPET, lack of data from the exercise test, exacerbation of patient's condition within three days before the exercise evaluation, relevant related chronic diseases that affecting test results.
An Expanded Access Study to Assess Brensocatib for Participants With Non-Cystic Fibrosis Bronchiectasis...
Non-Cystic Fibrosis BronchiectasisThe purpose of this study is to allow early access to brensocatib for participants with non-cystic fibrosis bronchiectasis (NCFBE) who have successfully completed the INS1007301 ASPEN Clinical trial.
Etiologies, Investigations and Outcomes of Patients Presenting With Hemoptysis
HemoptysisHaemoptysis19 moreThe study consist of a retrospective analysis of the etiologies, investigations and outcomes of patients presenting between 2005 to 2010 with hemoptysis in a North-American Tertiary center.
Reaction Time and Postural Control in Individuals With Cystic Fibrosis and Bronchiectasis
Cystic FibrosisBronchiectasisThe aim of this study is to evaluate the reaction time and postural control and to investigate the relationship between reaction time, exercise capacity, muscle oxygenation and balance in children with cystic fibrosis (CF) and non-CF bronchiectasis. 40 patients including 20 CF patients and 20 non-CF bronchiectasis and 20 healthy individuals will be included in this study. Demographic and physical characteristics' will be recorded. Lung function testing will be performed. Balance will be assessed using functional reach test, exercise capacity was measured using the incremental shuttle walking test and reaction time will be assessed using ''Fitlight TrainerTM''. Heart rate, respiratory rate, oxygen saturation, muscle oxygenation, dyspnea and fatigue perception will be measured before and after exercise test and reaction time measurement.
Prevalence of Bronchiectasis in COPD Patients
BronchiectasisChronic Obstruct Airways Disease1 moreThe identification of bronchiectasis in COPD has been defined as a different clinical COPD phenotype with greater symptomatic severity, more frequent chronic bronchial infection and exacerbations, and poor prognosis. A causal association has not yet been proven, but it is biologically plausible that COPD, and particularly the infective and exacerbator COPD phenotypes, could be the cause of bronchiectasis without any other known etiology, beyond any mere association or comorbidity.
The Incidence of Nontuberculous Mycobacterial Pulmonary Infection in Bilateral Bronchiectasis and...
BronchiectasisBronchiolitisNontuberculous mycobacteria (NTM) are ubiquitous organisms in the environment and are now increasingly being recognized as significant causes of chronic pulmonary infection in immunocompetent individuals (1). The most frequently encountered NTM lung disease worldwide is caused by Mycobacterium avium-intracellular complex (MAC) (2-4). In several studies with chest computed tomography (CT), researchers have demonstrated that the presence of bilateral multifocal bronchiolitis (well-defined small nodules and branching centrilobular nodules, or tree-in-bud pattern) and bronchiectasis distributed mainly in the right middle lobe and lingular segment are indicative of NTM pulmonary infection (7-11). Accordingly, it is believed that radiologic findings of bilateral bronchiolitis and bronchiectasis on chest CT scans specifically suggest NTM pulmonary infection (1). These CT findings, however, may not be specific for NTM pulmonary infection. CT patterns of bronchiectasis and bronchiolitis in the pulmonary infections caused by various NTM organisms have been reported, and these organisms include Mycobacterium kansasii, Mycobacterium xenopi, and rapidly growing mycobacteria such as Mycobacterium abscessus, Mycobacterium fortuitum, and Mycobacterium chelonae (12-14). In addition, not all patients with bronchiectasis and bronchiolitis have NTM pulmonary infection. Two recent studies showed that only about 50% of patients with such CT features have MAC pulmonary infection (9,15). To the best of our knowledge, however, there is no report about the incidence of NTM in patients with bronchiectasis or bronchiolitis in countries with low incidence of TB. Thus, the purpose of our study was to determine the frequency of NTM pulmonary infection in patients with bilateral bronchiectasis and bronchiolitis at chest CT and to investigate whether these CT findings are specifically indicative of MAC infection or other specific pathogen.