View clinical trials related to Cystic Fibrosis.
Filter by:The effect of long term inhalation of hypertonic saline in subjects with cystic fibrosis on lung function, incidence of respiratory tract infections, quality of life, quantitative microbiology and sputum cytokine profile. The hypothesis is that regular inhalation of nebulised hypertonic saline will have a beneficial effect on lung function and other clinical outcomes with no adverse effects on infection and inflammation in adults and children with cystic fibrosis.
Aim of the study is to detect the prevalence of chronic rhinosinusitis and the colonisation with Pseudomonas aerug. in the upper airways in patients with cystic fibrosis.
Rhinosinusitis disorders are often associated with Cystic Fibrosis. They can restrict quality of life enormously and give cause to repeated ENT surgery. The basic defect in CF is a dysfunction of Chloride channels in exocrine glands, leading to retention of secretions and consecutive chronic inflammation with bacterial superinfections. The prospective placebo controlled cross-over study aims at the evaluation of the efficacy of a nasally inhalation of Pulmozyme with respect to mucus retention and resulting inflammation.
Cystic fibrosis (CF) is a catabolic condition, and children affected with this disease frequently have problems with growth, despite adequate nutrition. We hypothesize that the anabolic effects of growth hormone (GH) will improve the height and weight of CF patients and thereby improve their clinical status and their quality of life. We further hypothesize that these effects will be sustained for at least one year after GH treatment is complete.
Individuals with cystic fibrosis (CF) have persistent infection in the airways, which the body attempts to fight by recruiting immune cells (neutrophils) to the lung. The immune system and neutrophils are unable to completely kill the bacteria, and the response to the infection leads to inflammation (swelling) of the airways and lung damage. Nitric oxide (NO) has anti-bacterial and anti-inflammatory properties in the lung. NO production is decreased in CF patients, and may contribute to the persistent infection and inflammation. Increasing the production of NO in the airways of CF patients may help decrease this inflammation and infection. Rho GTPases are molecules in the cells that line the airways that decrease the protein that makes nitric oxide (NOS). Rho proteins also increase inflammation in these cells. Rho proteins are increased in CF cells, and may partially explain the low NO and high inflammation seen in CF. Blocking the Rho protein in CF cells increases NOS, which can then produce more NO. The Rho protein can be inhibited with a drug, simvastatin (Zocor®). Simvastatin is used by millions of people to lower their cholesterol, is very safe, has few side-effects and is approved for use in children greater than 10 years of age. We propose that treating CF patients with simvastatin will increase NO produced (exhaled NO), and may decrease airway inflammation. If simvastatin has these expected effects in CF, it would be another drug that has potential to become a new therapy to fight the debilitating lung damage of the disease.
Many cystic fibrosis patients die of lung failure caused by repeated lung infections from thick, sticky mucus. Past studies have shown Bronchitol inhalation may help to facilitate the clearance of mucus by altering its rheology and replenishing the airway surface liquid layer in these patients, thereby enhancing the shift of stagnant mucus from the lungs. The study aim is to determine the optimal dose of mannitol to generate clinical improvement in patients with cystic fibrosis.
The objective of this study is to determine the effect of airway surface liquid (ASL) volume on mucociliary clearance in cystic fibrosis (CF). A two-isotope nuclear medicine technique will be utilized. This pilot trial will include the imaging of n=7 CF subjects and n=7 healthy subjects. The trial will include one study visit per subject that will take approximately 3 hours. Hypothesis: The simultaneous imaging of both a "floating" and a "penetrating" radioisotope tag will allow the relative effect of airway surface liquid volume on mucociliary clearance to be determined when evaluated in CF and normal subjects.
The purpose of this study is to evaluate the incidence of venous thrombosis occurring on totally implantable vascular access devices in cystic fibrosis patients who need a new device (it can be the first one or a subsequent one) and to study the genetic risk factors of thrombosis adjusted to the acquired ones. It is a nationwide cohort study planned for two years with a six month follow up period. The expected number of inclusion is 50 patients each year, that is to say 100 for the whole study. In cystic fibrosis, pulmonary exacerbations necessitate repeated intravenous antibiotics, but the peripheral blood accesses become precarious with time, leading to the indication of a central venous device. It is important to take a lot of precautions to protect vascular access. This allows the patient to have a dramatic improvement in life expectancy with such life-long devices (ONM, French National Observatory France 2003 : median at 36 years). Venous thrombosis can cause a superior cava syndrome, a pleural effusion or a pulmonary embolism. The risk of thrombosis is significant; retrospectively, it has been evaluated to be between 4 and 16% in the publications. This rate may be higher due to the fact that venous thrombosis may remain asymptomatic, and therefore silent, but they lead to the same risk of vascular access loss.
The primary objective of this NIH funded clinical trial is to conduct a multi-center, randomized, controlled trial comparing two interventions: a behavioral plus nutrition intervention to a nutrition intervention. This study will (a) determine the impact of the behavioral intervention on energy intake and weight gain; (b) examine the durability of the behavioral intervention's impact on growth (weight and height) one year following treatment; and (c) explore the relation between physical activity and growth.
- History of clinical breast pain for at least the last six months. - At least six days of moderate or severe breast pain per cycle. - Fibrosis, cysts, nodules involving at least 25% of the surface of one breast. - Euthyroid with no prior history of thyroid disease. - Six months of daily therapy with molecular iodine. - Placebo controlled vs active (1:1).