Bacteriological Link Between Upper and Lower Airways in Cystic Fibrosis and Primary Ciliary Dyskinesia...
Cystic FibrosisPrimary Ciliary DyskinesiaCytobacteriological examination of sputum and bacteriological sampling in the middle meatus.
Urinary and Anorectal Functional Disorders and Their Impact on CF Adults (PerineoMucoRMO)
Cystic FibrosisThis observational study evaluates prevalence of functional perinea disorders (stress urinary incontinence, overactive bladder syndrome, dysuria, anal incontinence) on CF adults patients of the North-West CF Network. Its aims are to measure the severity of urinary and anorectal symptoms ; assess their impact on patients' quality of life, sexuality, care and social life and relationships ; identify the medical and demographic factors associated with the severity of urinary and anorectal disorders and their impact and determine the relationship between the severity of these disorders, various repercussions, and risk factors.
Asthma With Hypersecretion-associated Gene for Cystic Fibrosis
AsthmaThe combination of asthma and being a carrier of genetic variants (mutations and / or polymorphisms) in the CFTR gene variant would cause bronchial asthma with mucus hypersecretion. This phenotype is characterized by a more severe disease, in terms of control, quality of life, exacerbations and lung function, and a different asthma the bronchial hypersecretion without inflammatory phenotype.
Induction of Neutrophilic Traps ( NETs ) by the Environment and Infectious Inflammatory Lung Cystic...
Cystic FibrosisThe study is defined in 3 stages of in vitro assays : Phase 1 : Secretion of NETs in response to lung disease and their secretion of factors. This is a transversal study in which we compare : Producing NETs (DNA and proteases ) by neutrophils purified from sputum and blood of patients Producing NETs (DNA and proteases ) by purified neutrophils in the blood of patients and blood of control subjects Phase 2: Mechanisms of activation of NETs secretory pathways . This phase will identify receptors (TLR ) involved in the secretion of NETs and analyze the involvement of dependent transduction pathways calcium and PKC . It will be performed on control blood neutrophils. Phase 3: Analysis of cell death ( NETose ) This is a transversal study that will analyze : the mode of cell death of neutrophils purified blood and sputum of patients and control blood neutrophils by flow cytometry and transmission electron microscopy .
Initial and Chronic Methicillin Resistant Staphylococcus Aureus (MRSA) Infection in Cystic Fibrosis...
Methicillin-Resistant Staphylococcus AureusCystic FibrosisThis study aims to examine features of MRSA that are associated with chronic MRSA infection and bacterial persistence despite IV antibiotic therapy. Subjects are asked to expectorate sputum and complete CF symptom diaries both at beginning and end of IV therapy.
The Use of Home Oral Glucose Tolerance Test Kit in Screening Cystic Fibrosis Related Diabetes
Cystic Fibrosis Related DiabetesCystic FibrosisThe aim of this study is to establish if the self-administered electronic Oral Glucose Tolerance Test kit can increase the annual uptake of screening for CFRD in children who are between 10 and 17 years of age with CF.
Detection of Reductions in Cystic Fibrosis Airway Inflammation While Using Aztreonam Lysine Solution...
Cystic Fibrosis in AdultsChronic Colonization With Pseudomonas AeruginosaIn cystic fibrosis, there is a critical need for better predictors of treatment response. The investigators have identified a panel of white blood cell biomarkers which can be directly measured as a blood test in subjects with cystic fibrosis. These biomarkers predict reduction of airway inflammation and infection more accurately than lung function testing, in patients receiving intravenous antibiotic therapy. In the current study, we hypothesize that this panel of gene biomarkers which can be readily measured from peripheral blood will sensitively predict changes in inflammation when patients receive inhaled antibiotic therapy, specifically Cayston (or inhaled aztreonam lysine). Patients enrolled in the study will have blood drawn before and after a month of inhaled Cayston, in order to test whether genes predict response to Cayston therapy more robustly than do standard measures such as lung function tests.
Registry to Monitor the Susceptibility to Aztreonam of Pseudomonas Aeruginosa Isolates From Cystic...
Cystic FibrosisThis is a prospective, longitudinal, 5-year study that will enroll participants from the existing Cystic Fibrosis Foundation (CFF) patient registry. Each enrolled participant will provide samples for microbiological evaluation, obtained upon enrollment and then once per year thereafter for 5 years.
A Study of Sweat Testing Using a Quantitative Patch
Cystic FibrosisThe primary aim of the study is to determine if the CF Quantum Sweat Test provides sweat chloride results that are equivalent to the results from traditional sweat testing. The secondary aim of the study is to assess the quantity not sufficient rate of the CF Quantum Sweat Test compared to traditional sweat testing.
A Prospective, Multicenter, Collaborative Study to Determine the Prevalence of Nontuberculous Mycobacteria...
Cystic FibrosisPediatricsTo determine the prevalence of nontuberculous mycobacterial infection (NTM) in pediatric patients with cystic fibrosis in the State fo Florida. HYPOTHESIS: There is high prevalence of NTM in CF pediatric population in the State of Florida, and likely higher than in the rest of the country.