View clinical trials related to Cystic Fibrosis.
Filter by:The goal of this study is to identify the immunological factors that influence a patient's response to the presence of the fungus Aspergillus fumigatus (A. fumigatus) in the lungs. In patients with cystic fibrosis (CF), this fungus is not known to cause damage to the lungs, but some patients respond with an allergic reaction that may cause wheeze, cough, or difficulty breathing. Approximately 230 patients will be enrolled with an additional 60 people who do not have CF and who do not have a history of asthma to serve as a comparison group.
In this research, we will use established surveys to look at the relationship between various styles of coping with a disease, religious coping styles, treatment compliance, locus of control, broad measures of mental health and adjustment, and basic health data (e.g., PFTs, recent hospitalizations or antibiotics within the past year, lung microbiology, CFTR mutations, and co-morbid conditions such as diabetes, depression, and liver disease). While the research is correlational, it should suggest relationships (both positive and negative) between various coping styles and desired outcomes (compliance and well-being).
*The purpose of this study is to develop a more accurate, reliable, specific and more acceptable alternative clinical test to the 72-hour stool and diet collection for quantifying fat malabsorption in people with CF and pancreatic insufficiency.
The primary objective of the trial is to assess the safety and tolerability of inhaled nitric oxide (NO) when administered by nasal cannula over a 44 hour period to clinically stable Cystic Fibrosis (CF) subjects. Toxicity is to be defined as a drop in oxygen saturations, a decline in forced expiratory volume in one second (FEV1), or an increase in methemoglobin.
This is a study to determine the safety and tolerability of 28 days of daily dosing of 560 mg of Arikayce™ versus placebo and daily dosing of 70 mg and 140 mg of Arikayce™ versus placebo in patients who have Cystic fibrosis (CF) and chronic infection due to pseudomonas aeruginosa.
This study will assess whether rhDNase treatment improves ventilation inhomogeneity as assessed by lung clearance index (LCI) in patients with cystic fibrosis (CF).
The purpose of this research study is to determine if multiple doses of two inhaled drugs will help Cystic Fibrosis patients whose lungs are infected with a bacteria called Burkholderia dolosa. The names of these drugs are tobramycin solution for inhalation and amiloride solution for inhalation. Currently, treating patients with Burkholderia dolosa infections is challenging because the bacteria is resistant to antibiotics. Therefore, researchers are looking for drugs which, when taken with an antibiotic, will help the antibiotic to work more effectively.
This is an open-label pilot study of the safety and tolerability of 7% hypertonic saline inhaled twice daily for 14 days in infants with CF, 12 to 30 months of age.
The objective of this overall project is to develop a new aerosol-based technique for quantifying liquid absorption in the airways of subjects with cystic fibrosis(CF) that can be used to help develop new therapies. In CF, mutations in the CF gene result in dysfunction of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) ion channel on the cells that line the airway epithelium, causing improper fluxes of ions such as sodium, chloride, and bicarbonate. The so called "low volume" hypothesis of CF pathogenesis contends that abnormal absorption of ions from the airways causes excessive absorption of liquid, resulting in an airway surface liquid layer that is dehydrated and difficult to clear. Here we are measuring the absorption rate of a radiolabeled small molecule (DTPA) from the lungs of cystic fibrosis patients and healthy controls. We hypothesize that the molecule will absorb more quickly in cystic fibrosis patients. Further studies will be performed to determine if DTPA absorption is related to liquid absorption in the airways.
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 a nasally inhalation of Pulmozyme with respect to mucus retention and resulting inflammation.