View clinical trials related to Cystic Fibrosis.
Filter by:Exploratory comparative evaluation of exhaled breath composition in cystic fibrosis patients with age and gender-matched healthy adults in order to identify a disease-specific exhaled breath pattern as well as to gain insight into pathophysiological and microbial contributions to exhaled breath composition.
Anyone who practices clinical medicine will understand that socially disadvantaged children will have worse health outcomes, no matter what the underlying condition might be. There is limited prospective data on the effects of social deprivation on children in BC and there is none concerning the effects of social deprivation on children with chronic diseases. In order to generate relevant data for those who manage children with chronic diseases in BC, the investigators wish to perform an observational study of the relationship between questionnaire-derived social variables and measured outcomes in children with cystic fibrosis, type 1 diabetes, and chronic kidney disease. Our working hypothesis is that there is an association between social determinants of health (income, education, race) and health outcomes in children with cystic fibrosis, type 1 diabetes and chronic renal failure, that is independent of access to health care (assessed by distance to nearest specialty clinic and number of clinic visits in the last year).
Cystic fibrosis-related diabetes (CFRD) occurs in almost 20% of teens and 50% of adults. The investigators' long term goal is to determine the cause of CFRD in order to translate this knowledge into therapies aimed at preventing CFRD. Since CFRD and type 2 diabetes share several clinical features and since oxidative stress is a key factor in the development of type 2 diabetes, the investigators explored the role of oxidative stress in CFRD. The investigators discovered a unique CF biochemical signature that they believe could be implicated in the development of CFRD. The investigators found that glucose ingestion in CF teens and young adults causes an acute and profound systemic redox imbalance to the oxidizing state. The degree of redox imbalance was quite severe and would be expected to damage the insulin producing cells as these cells are particularly vulnerable to oxidative stress. Thus, these findings could prove to be a critical factor in the pathogenesis of CFRD. This proposal will test the hypothesis that glucose-induced redox imbalance is an intrinsic, metabolic defect in CF. In addition, because CF people are required to consume a high calorie diet to maintain their weight, the investigators also hypothesize that certain high caloric foods will recapitulate the redox imbalance induced by ingesting glucose and thus hasten the development of CFRD. Specifically, the investigators aim to: - Determine whether young children with CF have glucose-induced redox imbalance - Determine whether eating a meal with a high glycemic index induces acute redox imbalance - Determine whether commonly consumed beverages containing simple sugars (i.e., soda or fruit juice) induce acute redox imbalance
The purpose of this study is to determine the optimal time for the nebulization depending on the respiratory physiotherapy session in cystic fibrosis patients.
The progression of lung disease in cystic fibrosis (CF) results inevitably in a reduction in exercise capacity. The assessment of fitness and exercise capacity in CF is an important measure of the impact of the disease process, particularly if it is repeated over time. With recent advances in clinical management, CF lung disease in children can be relatively mild and exercise tolerance good. The currently available field tests e.g. 3 minute step test, are often completed too easily. These tests provide limited information relating to maximal exercise performance. By contrast, the maximal CardioPulmonary Exercise Test (CPET), a progressive, incremental, gold standard exercise test with breath by breath analysis of expired gas, has proved to be a valuable means of assessing exercise response in patients with CF. Its only limitation is the requirement for specialist laboratory facilities, equipment and staff. A new field test for evaluating exercise capacity in children is needed. This should be portable, easy to administer and simple to perform by young children, while providing a higher intensity of exercise which correlates with day to day activity patterns of children, and clinically relevant information in the short term and longitudinally. This test needs to be a good surrogate measure of exercise capacity when formal CPET is unable to be undertaken. By providing accurate and useful information the results can be used to prescribe and train individuals with CF safely and effectively and can also be used in the short and long term for guidance of the medical management of these complex patients. The aim of this study is to develop and validate the use of a new incremental step test to assess exercise tolerance/capacity in children with CF, compare this with the gold standard CPET and to provide normative healthy control comparison data The main objectives of the study are 1. To develop an incremental step test to assess exercise tolerance / capacity in children with CF. 2. To compare the incremental step test with the gold standard CPET 3. To assess the level of exercise response produced by the incremental step test 4. To assess the correlation between independent variables of lung function measurements, age, weight and height with VO2peak and other exercise test outcomes 5. To assess the repeatability and evaluate the normal variability of the new incremental step test 6. To provide healthy control normative data for comparison
To 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.
The purpose of this study is to determine whether the treatment with Ivacaftor remains effective and safe in the patients with cystic fibrosis (and at least one G551D CFTR mutation) in the real life setting, after the drug has been approved by the Health authorities.
This study is designed to assess the safety, tolerability, pharmacokinetics and preliminary pharmacodynamics (proof of concept) of QBW251 in healthy subjects and cystic fibrosis patients following single and multiple doses. This first-in-human and proof of concept study will consist of 4 parts, with Parts 1 and 2 in healthy volunteers and Parts 3 and 4 in cystic fibrosis patients.
The goal of this study is to identify chemical compounds in the blood and sputum (i.e., biomarkers) that are associated with objective measurements of health status in patients with cystic fibrosis (CF). This study builds upon observations that blood levels of hepcidin-25, a protein that regulates how the body uses and stores iron, vary during CF pulmonary exacerbation (CFPE).
Real-world adherence to inhaled and oral therapies for cystic fibrosis (CF) patients remains discouragingly low, ranging between 31-53% for inhaled antibiotics and 41-72% for hypertonic saline. Programs to enhance adherence, including comprehensive behavioral interventions with adolescents, have met with mixed success. Advances in therapy, treatment delivery systems, and data capture technology offer the potential for enhancing adherence by providing immediate and more frequent feedback to the patient regarding his or her fidelity to the prescribed treatment regimen. We propose to conduct a proof-of-concept study to evaluate a systematic approach to linking treatment and feedback components to enhance adherence.