Active clinical trials for "Cystic Fibrosis"

Scientist have begun to realize that many types of bacteria often live together as a complex community, and the investigators wish to apply that idea to the bacteria in the respiratory system of people with Cystic Fibrosis (CF). It is possible that the survival of the many millions of bacteria in the CF lung depends on the production of special chemicals that might be made only by very few types of bacteria. If that is true, medicines that interfere with those chemicals could treat the lung infections that cause trouble for nearly all people with CF. The investigators wish to study the production of several potentially critical chemicals by the respiratory bacteria and to examine the effect of those chemicals on the makeup of the entire community of bacteria. To detect all the bacteria in that community, the investigators will use new methods that use bacterial genetic information and can detect hundreds of different types of bacteria in respiratory samples of individual CF patients.

The purpose of this study is to determine whether 5 - methyltetrahydrofolate and vitamin B12 supplementation can ameliorate cell plasma membrane features in pediatric patients with cystic fibrosis

Life expectancy of patients with cystic fibrosis has improved dramatically the last few years. Respiratory complications remain the main contributory factor to the morbidity and mortality associated with the disease. Exercise tolerance is reduced as the disease progresses, and peak aerobic capacity seems to be linked with survival. Regular physical activity has positive benefits, including a better body image, an improvement of pulmonary function, of exercise capacity and a possible improvement of quality of life.But because of the considerable variability of the subjects, exercise programs should be tailored to individual needs, and easy included in their cumbersome treatment routines and professional activities. In the cystic fibrosis center of Strasbourg we are able to propose to the patients a one-year physical exercise program, partly supervised with coaches, at home. Electronically braked cycle ergometer and heart rate monitoring system are at patients disposal, for one year, at home. Thus, patients can choose, during the day, the best moment to work out .Subjects will be randomised in two groups:1. a control group, where subjects are asked to continue their normal daily activities and physiotherapy regime.2. a training group, where subjects are asked to exercise three times a week. For the training group, three times a week, patients will train for 30 minutes. Heart rate will be continuously monitored and send to the medical staff every week-end . A correction of exercise intensity, if needed, is weekly proposed to maintain a maximal training efficiency, and coaches can help them, if necessary. For the two groups, quality of life will be measured with a disease-specific questionnaire (CFQ14+) (Henry, 1998, Quittner, 2000), and a generic questionnaire (SF 36) (Gee, 2002) before the program, and after 6 and 12 months. After a one year training program, and compared to the control group, we should expected an improvement in aerobic capacity and peak oxygen consumption, both associated with improved prognosis in cystic fibrosis. We also expected to observe an improvement in quality of life measurement, shorter hospital stays and fewer exacerbations. With this kind of program, we also would like to improve the degree of adherence in daily life exercise.

People with cystic fibrosis (CF) often develop chronic pulmonary infections which are caused by a variety of organisms, the most predominant being Pseudomonas aeruginosa. Antibiotics are important in managing CF lung infections. Antibiotic use in CF was altered about ten years ago with the approval of inhaled tobramycin, an aminoglycoside which is effective in treating P. aeruginosa. A decade later, CF clinicians are increasingly concerned about the likelihood of induction of aminoglycoside-resistance with prolonged use of inhaled tobramycin to treat chronic P. aeruginosa airway infections. The goal of this study is to examine the current microbiology and susceptibility of organisms from CF sputum, correlate it with antibiotic use, and compare it with previous data.

This study will examine the relationship between bacterial products in lung infections in cystic fibrosis and disease severity. It will examine plasma and lung tissue from cystic fibrosis patients. Patients with cystic fibrosis and having certain genetic characteristics, who are between 9 and 65 years of age and any cystic fibrosis patient undergoing lung transplantation at INOVA Fairfax Hospital in Fairfax, Virginia, may be eligible for this study. Patients who cannot undergo apheresis may be asked to provide up to an additional 100 cc (7 tablespoons) of blood for research to look at bacterial products. Lung specimens of participating transplant patients will be collected at INOVA Fairfax Hospital. Patients who participate in the apheresis portion of the study will be admitted to the NIH Clinical Center for 2 to 3 days. Apheresis is a procedure for collecting large quantities of specific blood components. For this study, plasma-the liquid part of the blood-will be collected. For the procedure, whole blood is collected through a needle in an arm vein, similar to donating blood. The blood is separated into its components by centrifugation (spinning), the plasma and white cells are extracted and collected in a bag, and the red cells are returned to the body, either through the same needle or through another needle in the other arm. During the hospital stay, patients may also be asked to participate in other cystic fibrosis studies involving blood tests, an echocardiogram (ultrasound test of the heart), urine pregnancy test, and pulmonary function (breathing) tests.

This is a single-centre, prospective observational cohort study assessing the potential utility of the Owlstone Medical "Breath Biopsy" in early diagnosis of pulmonary infections in patients with cystic fibrosis (CF). In cystic fibrosis pulmonary infections occur frequently and are associated with decline in lung function and disease progression, therefore a cornerstone of CF management is early identification and treatment of infections. "Breath Biopsy" is a non-invasive novel technology that has been trialled extensively in diagnosis of a variety of medical conditions with promising results. The technology is based the identification of a unique profile of organic compounds in exhaled breath of patients with a certain medical condition. Making the diagnosis of pulmonary infections in patients with CF is clinically challenging and at present relies on imprecise diagnostic tests, and generally requires attendance of patients to hospital or clinic for assessment. Ultimately, this research aims to assess the feasibility of incorporating "Breath Biopsy" into this diagnostic pathway with the advantages of both improving diagnostic certainty and potentially allowing in-home diagnosis of infections related to CF. Furthermore, identification of organic compounds implicated in CF infections will improve the understanding of why these infections occur, which to date remains an area that is poorly understood. Five patients with CF-related pulmonary infections admitted to the inpatient CF unit at the Royal Papworth Hospital will be enrolled, and use "Breath Biopsy" devices provided by Owlstone medical to collect breath samples from these patients in order to determine whether a unique organic compound profile can be identified in CF exacerbations.

This is an observational study of people with severe cystic fibrosis that are eligible for Vertex's triple combination therapy through its expanded access. Pulmonary health, intestinal health, and the overall health of individuals will be tracked for a year to see how effective triple combination therapy is in these people with severe cystic fibrosis disease.

The intimate life of patients with cystic fibrosis has not been the subject of specific research, it is even left behind in favor of respiratory, digestive or endocrinological dysfunctions endangering these patients. After 16 years of practice, it is significant that this subject is problematic, painful but difficult to tackle; No doubt just as much on the side of caregivers: this question has never found its way into team exchanges, it seems to be evaded doubtless difficult or too intimate to welcome. These disorders arise from the effects of the disease on the health of the mucous epithelial tissues also located in the genital area. These difficulties are more closely approached by the difficulties of procreation; PMA allows them to get around them, nevertheless making it possible to be a parent, with the residual frustration of a satisfactory sex life: a deaf "addition", adding to many care constraints. It is therefore in terms of sexual health and quality of sexual life that it is desirable to shed light on this aspect of cystic fibrosis in women. Bibliographic research confirms this approach: this subject is not explored, the rare publications concern the vulnerability of the cervix or the difficulty on both sides of tackling this subject.

Cystic fibrosis (CF) is characterized by a decrease in mucociliary clearance, recurrent infections and airway inflammation. This inflammatory process in airway mucosa is persistent, uncontrolled, but, somewhat paradoxically, ineffective for pathogen clearance. Neutrophils are chronically recruited in the airway mucosa by proinflammatory mediators such as Interleukin (IL)-17. However, mechanisms involved in this dysregulated and persistent immune response are not well understood. In this context, a heterogeneous subpopulation of T lymphocytes called "unconventional T cells" (UTC) should deserve greater attention. UTC play a key role in orchestrating the ensuing innate and adaptive immune responses and they are endowed with numerous regulatory and effector properties. UTC mainly establish residency at mucosal sites, including the lung. To date, however, data related to implication and behavior of UTC during cystic fibrosis are extremely limited. The hypothesis is that, given UTC properties, their functions and behavior are altered in CF, and thus, these cells could be implicated in persistent inflammation and poor response to infections. The objective is to study UTC properties and functions in cystic fibrosis using blood and sputum samples of patients with CF, in correlation with comprehensive clinical and microbiological data. The study will enroll adult patients with CF followed-up at University Hospital of Tours, France. For each patient included, blood and sputum samples will be analyzed during 18 months 1/ from routine tests obtained at steady state and 2/ from tests performed during acute exacerbations. UTC will be explored in blood and sputum using flowcytometry approach, to evaluate their relative abundance, activation/inhibition profile and functions (cytokine production and cytotoxic ability). Correlation will be made with clinical status, with longitudinal comparison across the study period for each patient, and comparison with the other patients and healthy volunteers. This study will add significant knowledge in CF immunopathology by comprehensively assess UTC presence, functions and activation in CF. Indeed, UTC could be explored for disease progression marker, and, in a long-term perspective, explored for therapeutic interventions aiming at modulating their function (by activating or inhibiting UTC), to reshape lung immune response during CF.

This study evaluates the feasibility of using differentially methylated insulin DNA, a biomarker of beta cell death, in determining the time course of beta cell death and development of diabetes in people with cystic fibrosis. Study participants with cystic fibrosis and healthy control participants will have a blood sample drawn in order to measure the levels of differentially methylated insulin DNA.