Active clinical trials for "Cystic Fibrosis"

The goal of this feasibility study is to evaluate the ability of Electrical Impedance Tomography (EIT) for providing regional and quantitative information about the extent and nature of bronchial obstruction in patients with cystic fibrosis. It is not being conducted to diagnose, treat, prevent, or cure any kind of disease. In electrical impedance tomography low amplitude, low frequency current is applied on electrodes, and the resulting voltage is measured and used to computed the electrical properties of the interior of the chest as they change in time. The computed properties are used to form an image, which can then be used for monitoring and diagnosis.

The proposed study is designed to characterize the pharmacokinetics of intravenous and oral tedizolid in patients with Cystic Fibrosis.

The purpose of this study is to find out whether the High Frequency Digit Triplet test can be used to screen patients with cystic fibrosis for hearing loss in conditions of health and pulmonary exacerbation. It is also designed to find out the youngest age at which a child can perform the test, the prevalence of hearing loss in a CF population and the prevalence of genetic mutations known to be associated with hearing loss in the same population.

This study plans to learn more about the effect of oral antibiotics for an outpatient pulmonary exacerbation (respiratory illness) on sputum (mucus) bacterial infections, lung function, airway inflammation and quality of life. In this study subjects will perform pulmonary function tests, provide a sputum sample and complete a questionnaire at two separate clinic visits. If a subject is unable to cough up sputum in clinic he or she will be asked to breathe in a salt water solution to help cough up sputum. The first sputum sample will be obtained at the first visit (within 48 hours of starting antibiotics). Pulmonary function testing and the questionnaire will also be completed at this time. Subjects will complete a two week course of oral antibiotics at home. During these two weeks subjects will be asked to write down the times antibiotics are taken and airway clearance (vest treatment) is performed. Within one week of completing the antibiotic course subjects will return to clinic for a second visit. At that time a sputum sample will be obtained again and if subjects are unable to cough up sputum they will again be asked to breathe in salt water solution to help cough up sputum. Pulmonary function testing and the questionnaire will also be completed at this second visit. Sputum samples will be tested for infections with bacteria and viruses. Sputum samples will also be used to measure markers of airway inflammation (swelling). The investigators hypothesize that the use of two weeks of oral antibiotics for the treatment of a pulmonary exacerbation will result in a decrease in the amount of bacteria measured in sputum. Additionally the investigators hypothesize that treatment with antibiotics will lead to an improvement in lung function, a decrease in airway inflammation and an improvement in quality of life measurements.

Inhaled tobramycin is a Cystic Fibrosis Foundation recommended effective treatment for individuals with cystic fibrosis for the management of Pseudomonas aeruginosa airway colonization and improves the FEV1 and reduces the number of acute pulmonary exacerbations of CF. Patients typically use the inhaled tobramycin for a period of 28 days. Unfortunately, the standard nebulizer method for delivering tobramycin inhaled solution (TIS) is time-consuming and may result in missed therapy doses and suboptimal care. A new inhaled formulation and delivery device, the TOBI Podhaler (TPI), is a quicker, more efficient method of administering inhaled Tobramycin. This new pocket-sized disposable inhaler is maintenance free, requires no refrigeration or power source, and should greatly increase patient mobility and improve time management.

This study is aimed at better understanding the impact of ivacaftor upon insulin and incretin secretion and glucose tolerance in patients with Cystic Fibrosis with a glycine (G551D) mutation. Investigators hypothesize that treatment with ivacaftor improves insulin secretion in individuals with CF.

Title: Duplication in Chitotriosidase (CHIT1) Gene and the Risk for Aspergillus Lung Disease in CF Patients. Aim: To evaluate the link between CHIT1 duplication in CF patients and the predisposition to ABPA or persistent Aspergillus infection. Patients: 40 CF patients. Design: Observational, single visit. Methods: All patients will be assessed for pulmonary function tests (PFT), sputum cultures, and blood tests for: CHIT1 duplication, immunoglobulin E (IgE) and Eosinophils levels. Part of the patients will be assessed for RAST, skin prick test. Primary outcome measure is the difference in CHIT1 genotyping between the groups.

Cystic fibrosis can affect organs other than the lungs. Liver disease affects about 30% of patients: its main manifestation is the development of portal hypertension (PHT). The pathophysiology of this comorbidity is still poorly understood. It was previously considered secondary to the formation of biliary cirrhosis but another hypothesis would be that of a primitive pathology of venous vessels may cause the gradual emergence of portal hypertension without cirrhosis. Evidence indiscutly suggest that cystic fibrosis is associated with a specific endothelial dysfunction, especially as the CFTR (Cystic Fibrosis Transmembrane conductance Regulator) protein is expressed on the surface of endothelial cells. The investigators hypothesize that liver disease related to PHT-associated cystic fibrosis is associated with systemic endothelial dysfunction. The aim is: To demonstrate a systemic endothelial dysfunction in patients with cystic fibrosis when associated with PHT. To study the correlations between measures of systemic endothelial function and serum markers of endothelial dysfunction and between measures of liver stiffness and systemic endothelial function.

Approximately half of the variation in the presentation and disease status of patients with Cystic Fibrosis (CF) is secondary to non-genetic variables.[1] These include household income, compliance with therapy, second hand smoke exposure and possibly exercise (although exercise has less evidence to support its influence on disease status). Recently increased attention has been focused on the role of exercise and quality of life for patients with CF. This study will evaluate the role of exercise in relation to the patient's perception of their quality of life. The primary outcome will be the overall change in the patient's Cystic Fibrosis Questionnaire - Revised (CFQ-R) over a one year period during which the participants are encouraged to exercise and use a personal fitness device to track their exercise. This is a prospective single group non-blinded study. Participants will be asked to wear a personal fitness device for 11-13 months. The change in CFQ-R score is the primary outcome and other secondary outcomes will include; (1) exacerbations of cystic fibrosis (through use of antibiotics), (2) change in subject's FEV1, (3) inpatient admission rates, (4) change in BMI and (5) change in 6 minute walk test. The study will take place at the Adult CF Clinic on the University of Oklahoma Health Sciences Center campus.

This study will compare the use of inhaled concentrated sodium chloride solution to an inhaled solution of sodium bicarbonate in an attempt to decrease the thickness and stickiness of the mucus in the lungs of a person with cystic fibrosis. Also, this study is also looking at whether or not it is possible to decrease the acidity of the airways by inhaling sodium bicarbonate through nebulizer treatments.