Electrical Impedance Imaging of Patients With Cystic Fibrosis
Cystic FibrosisThe 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.
BioEnergetics and Metabolomics in Cystic Fibrosis
Cystic FibrosisWith better medical care, patients with cystic fibrosis (CF), a life-threatening disease, are enjoying longer lives. As the CF life expectancy increases, conditions such as CF-related diabetes (CFRD) become more prevalent. Nutrition plays a major role in maintaining optimal health in cystic fibrosis (CF). This project is designed to investigate nutrition-related factors, such as diet and body composition, on outcomes in patients with CF. The data generated from this study will be used to inform future nutrition intervention studies in adults with CF and CFRD.
Evaluation of the High Frequency Digit Triplet Test in Cystic Fibrosis
Cystic FibrosisSensorineural Hearing LossThe 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.
Efficacy of TOBI Podhaler vs. Tobramycin Inhalation Solution
Cystic FibrosisInhaled 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.
Ivacaftor (Kalydeco) and Insulin in Cystic Fibrosis (CF)
Cystic Fibrosis Related DiabetesCystic FibrosisThis 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.
Characterization of Apolipoprotein A-I Pathways in Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary FibrosisBackground: - Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that becomes worse over time. There is currently no effective treatment for it. Researchers want to study the disease and learn new ways to treat it. Objectives: - To discover new pathways that are involved in pulmonary fibrosis. To develop new drugs that may be used to treat pulmonary fibrosis. Eligibility: People at least 18 years old with IPF. Healthy volunteers at least 18 years old. Design: Participants will be screened with medical history, questionnaire, and physical exam. They will have blood, lung, and walking tests and chest scans. All participants will have 1 study visit, including: Medical history and physical exam. Questions about their breathing. Blood tests. Breathing tests. Six-minute walk test. Pregnancy test. Chest x-ray (healthy volunteers) or chest CT scan (people with pulmonary fibrosis ). Small area of skin may be removed. Genetic tests of blood and skin samples. Participants will probably not be informed of any findings. Samples may be used to make stem cells for use in research. Participants may be contacted in the future to give consent for this research. Some participants will have repeat visits over many years, repeating many of the study tests.
Duplication in CHIT1 Gene and the Risk for Aspergillus Lung Disease in CF Patients
Cystic FibrosisChit1Title: 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.
Ultrasound Stiffness Imaging in Crohn's Disease
Crohn's DiseaseUltrasound3 moreThe purpose of this study is to determine whether ultrasound-based assessment of intestinal stiffness in patients with Crohn's disease predicts the effectiveness of medical therapy or the need for surgical resection.
Angiogenesis and Fibrosis in Myocardial Infarction
Myocardial InfarctionFibrosis2 moreAngiogenesis and fibrosis lie at the heart of a number of fundamental processes responsible for cardiovascular disease. In this proposal, the investigators intend to build upon a highly successful programme of studies exploring the cardiovascular applications of positron emission tomography. Specifically, the investigators will explore the potential role of a novel radiotracer, 18F-fluciclatide, which is a highly selective ligand for the αvβ3 and αvβ5 integrin receptors that are up regulated during angiogenesis, and tissue fibrosis and remodelling. This tracer has been successfully used to assess angiogenesis in metastatic tumours and its uptake is suppressed by anti-angiogenic therapies. The investigators here propose to describe the pattern of uptake of 18F-fluciclatide in cardiovascular diseases, specifically acute myocardial infarction and aortic atherosclerosis. The investigators will correlate 18F-fluciclatide uptake with in vivo measures of angiogenesis and fibrosis. If successful, this novel radiotracer could provide an extremely important non-invasive method of assessing in vivo angiogenesis, plaque vulnerability, and tissue remodelling as well as potential applications in developing stem cell therapies.
BCAA Supplemental Study
CirrhosisThe central hypothesis of this study is that BCAA supplementation and BCAA supplementation plus low-intensity activity will improve muscle mass and HRQOL in cirrhotic patients compared to usual care