Active clinical trials for "Cystic Fibrosis"

This is a research study where researchers are collecting blood to evaluate the genetic characteristics of individuals with chronic lung diseases, including asthma, chronic obstructive pulmonary disease (COPD), interstitial lung disease, cystic fibrosis, and lung cancer.

Serum Zn status of patients with cystic fibrosis at diagnosis and one year later, compared to a healthy control group

The purpose of this study is to identify modifier genes in cystic fibrosis (CF).

The purpose of this study is to determine which biological marker, or association of biological markers, best predict clinical response of cystic fibrosis patients to CFTR modulators.

Patients with cystic fibrosis aged 6-13 will be recruited and randomized into two groups: one will receive telerehabilitation sessions including postural, breathing and high-intensity interval training exercises and one will be subject to routine follow up. Exercise program will be applied three days a week for three months. Children will be assessed by pulmonary function tests, cystic fibrosis quality of life questionnaire, six minute walking test and anxiety and depression scale in children, while caregivers will be assessed by Beck depression scale and situational anxiety inventory.

Influenza (the "flu") is one of the most common respiratory viruses associated with respiratory deteriorations in children and adolescents with cystic fibrosis. These deteriorations usually mean antibiotics, hospitalizations, and worsening of pulmonary function tests. A new flu vaccine has been recently approved for use in Canada (Flumist®). What is particular about this flu vaccine is that it is a spray in the nose, which mimics how influenza usually infects us. This particular vaccine protects children and adolescents much better than the regular injectable flu shot. This new vaccine has been given to > 2,000 healthy children and to >2,000 children with asthma and well tolerated. The investigators want to know if Flumist® is well tolerated in children with CF and does not cause worsening of respiratory symptoms. The investigators will conduct a study where all participants will receive Flumist® in the nose. This study is particularly important because its results will provide safety information on a vaccine that is more efficacious for a population who needs safe and easy to administer protection against the flu.

The project is designed to test new biomarkers that are more sensitive than the current standard in detecting injury to the proximal kidney tubule and will establish better criteria for when kidney safety concerns may halt further testing of a drug in humans.

Adolescents with cystic fibrosis are particularly vulnerable to poor adherence, which negatively impacts their health status, quality of life and long term survival. CFFONE: A Cell Phone Support Program for Adolescents with Cystic Fibrosis, will make use of cutting-edge technology- a broadband capable, cellular telephone keyed into a highly-interactive informational web site. This web site will provide engaging online learning activities and resources specific to adolescents with cystic fibrosis. We believe the information and activities contained in CFFONE will improve adolescents knowledge, attitudes, and practices around cystic fibrosis and that adolescents exposed to the CFFONE program will demonstrate an increase in adherence to their treatment regimens and related improvements in their health status and quality of life.

Cystic fibrosis is a genetic disorder caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene, leading to pulmonary infections, sinus disease, pancreatic insufficiency, hepatobiliary disease and male infertility, with respiratory failure being the primary cause of death. Cystic Fibrosis Related Diabetes (CFRD) in one of the most common complication of cystic fibrosis (CF) and it's associated with a worse respiratory and nutritional state, with a negative impact on life expectancy. It differs from type 1 diabetes and type 2 diabetes for particular characteristics making this disease a separated clinical entity. To date, there is a lack of evidence on many aspects concerning this disease: the pathophysiology of the disease: decreased insulin secretion has historically been seen has the major trigger for CFRD, but data about this mechanism are scarce and conflicting. Moreover, the role of insulin-resistance seems to be not consistent, but pulmonary exacerbations are very common and, in this setting, insulin sensitivity can worsen significantly. the relationship between its development and particular genetic settings: certain CFTR genotypes are known to be most related to the risk of diabetes, and only few susceptibility genes for type 2 diabetes have been evaluated as potential predisposing factors for CFRD. the relationship between the therapeutic optimization and its impact on metabolic status and lung function: CFRD is known to be associated with worse clinical outcomes, reflected in more frequent clinical exacerbations, greater reduction in lung function, poorer nutritional status and decreased survival. It has also been demonstrated that insulin therapy can improve pulmonary function, increase body weight and reduce lung exacerbations. However, no study on the clinical impact of the optimization of insulin therapy on pulmonary outcomes and life expectancy are available in this population. finally, no data about potential predisposing pre-transplant risk factors for development of post-transplant DM are available For this reason, the investigators have structured a study with the aim to: characterize the pathophysiological process leading to CFRD, with assessment of the relative contribution of the insulin resistance and the β-cellular secretion impairment define the prevalence of CFRD in relation to the mutations of the CFTR gene and to the presence of candidate genes for the development of type 2 diabetes perform a proteomic analysis to identify potential proteomic biomarkers among CFRD patients evaluate the body composition, muscle performance and respiratory outcomes in patients on insulin therapy, before and after therapeutic optimization, in a follow-up period of 24 months. identify eventual predisposing factors for the development of post-transplant diabetes in subjects without pre-transplant CFRD.

Adult patients with Cystic Fibrosis who are seen at the specialty CF clinic at University of Virginia will be given an option to utilize telemedicine instead of in-person visits for standard clinic visits. Health information from standard of care visits including FEV1, exacerbations, leading to oral or intravenous antibiotics, laboratory results, hospitalization records, and responses to health questionnaires will be recorded for research purposes. Data collected for the research study will be compared to baseline and previous years to determine if there are any deleterious effects for those who transition to telemedicine clinic visits.