View clinical trials related to Cystic Fibrosis.
Filter by:This study will demonstrate feasibility and collect pilot data via a pilot randomized trial of 20 participants on the effectiveness of a web-based delivery system of behavioral plus nutrition intervention for parents of children with Cystic Fibrosis (CF) ages 4 to 9 years of age that has been found to be efficacious in face to face delivery.
This study is a multiple within participant crossover study to evaluate the effect of ivacaftor on lung function in participants aged 12 years and older with cystic fibrosis (CF) who have phenotypic or molecular evidence of residual CF transmembrane conductance regulator (CFTR) function.
This was a randomized, double-blind, placebo-controlled, dose escalation study to assess the safety and tolerability of 100 mg and 200 mg of inhaled Alpha-1 HC administered once a day for three weeks in subjects aged 18 years and older with cystic fibrosis (CF). The treatment duration in this study was intended to provide multi-dose safety information prior to proceeding to longer durations of exposure.
Cystic Fibrosis (CF) is a fatal, recessive genetic disorder characterized by progressive inflammation and lung damage. It is unclear whether current treatment strategies, which focus on detection and eradication of pathogenic microorganisms in the lung, are the best way to prevent the initiation of early inflammation and lung damage. This study asks how early acquisition of microbial flora occurs in infants with CF and healthy baby controls, and whether this process initiates or influences early inflammation and clinical disease progression in CF.
The purpose of this study is to compare the tolerability and acceptability of a formulation containing Hypertonic saline 7% (HS) alone and a formulation containing HS and Hyaluronic acid 0.1% in a population of Cystic Fibrosis (CF) patients who already showed poor tolerance to HS.
The purpose of this study is to describe two cohorts of cystic fibrosis patients treated in 2 different decades in terms of FEV1 (Forced Expiratory Volume in one second) maintenance.
This study is to investigate whether using a continuous alternating therapy (CAT) regimen of 2 antibiotics of different classes and with different mechanisms of action may provide the clinical benefits of reducing acute pulmonary exacerbations, maintaining lung function, and controlling respiratory symptoms for cystic fibrosis (CF) patients while minimizing the risk of emergence of antibiotic-resistant Pseudomonas aeruginosa (PA) strains. After screening, eligible participants will be enrolled into the study and begin a 28-day run in period of tobramycin inhalation solution (TIS) twice daily. After the run-in, participants will return to the clinic and be randomized to either the Aztreonam for Inhalation Solution (AZLI) arm or the placebo arm. The AZLI arm has 3 cycles of AZLI three times daily for 28 days alternating with TIS twice daily for 28 days. The placebo arm has 3 cycles of placebo three times daily for 28 days alternating with 3 cycles of TIS twice daily for 28 days. Participants will return to the clinic for evaluation after each cycle of antibiotics for evaluation. There will be 9 scheduled study visits per participant.
The purpose of the study is to further evaluate the safety and efficacy of EUR-1008 as compared to Kreon® in the treatment of exocrine pancreatic insufficiency associated with Cystic Fibrosis in subjects 12 years of age and older.
The major causes of morbidity and mortality in Cystic Fibrosis (CF) are linked to the process of chronic inflammatory of the airway, leading to the progressive damage of the small bronchioles and subsequently to the proximal bronchi. A connection between weaknesses of respiratory muscles in CF and deficits of CFTR in the muscle has been established. Insufficient cough in CF patients may advance re-current respiratory infections. A voluntary cough flow volume (C-FVC) profile incorporates the characteristics of the forced expiratory flow volume curve (FE-VC). The study aims to explore the correspondence of voluntary cough-flow-volume and maximum expiratory flow-volume maneuvers in relation to disease complications in CF patients.
Inflammation is present in the Cystic fibrosis (CF) airway from the time of infancy, and worsens with the onset of chronic infection. Therapies with proven benefit are associated with decreased airway inflammation. Thus, sensitive and reproducible biomarkers of airway inflammation have long been sought as a necessary component to improved clinical care and to facilitate therapeutic trials for CF. FEV1, the standard outcome measure in CF, is recognized as an insensitive outcome measure. the investigators have identified a panel of 10 genes which sensitively predict resolution of pulmonary inflammation, in response to therapy of an acute pulmonary exacerbation. With the goal of yielding a technically simple but unique CF biomarker assay, the investigators have tested whether proteins signified by these genes show large changes in expression following treatment of acute pulmonary exacerbations. Protein quantifications are among the most common measurements performed in clinical laboratories around the world. Based on preliminary findings that changes in white blood cell proteins mirror changes seen in the genes, the investigators propose to identify top candidate proteins, from the investigators gene panel, which change in response to exacerbation therapy. Once identified, these proteins will be quantified directly with a new blood test which is inexpensive and simple to perform. The investigators propose to validate this blood test in a single site trial. If successful, this proposal will yield a biomarker assay that will be available to validate in a multi-site trial and provide unique insights into mechanisms that regulate white blood cell activation and recruitment in CF lung disease.