Nasally Delivered Pulmozyme for Sinusitis in Cystic Fibrosis
SinusitisCystic FibrosisChronic sinusitis is a frequent complication in cystic fibrosis. The aim of this study is to determine whether Pulmozyme(dornase alfa) would maintain sinus health (compared to placebo) in patients with cystic fibrosis who have recently undergone sinus surgery.
Safety, Pharmacokinetic and Pharmacodynamic Study of MP-376 in Patients With Cystic Fibrosis
Cystic FibrosisPatients with cystic fibrosis (CF) suffer from chronic infections of the lower respiratory tract that can be caused by one or multiple bacteria, including Pseudomonas aeruginosa, which has been particularly problematic to eradicate and been implicated as the major cause of morbidity and mortality in CF patients. Aerosol delivery of antibiotics directly to the lung increases the local concentrations of antibiotic at the site of infection resulting in improved antimicrobial effects compared to systemic administration. Bacterial resistance to current aerosol antibiotic treatments indicate a need for improved therapies to treat CF patients with pulmonary infections caused by multi-drug resistant Pseudomonas aeruginosa and other bacteria. High concentrations of MP-376 delivered directly to the lung are projected to have antimicrobial effects on even the most resistant organisms and reduce the emergence of resistant bacteria.
The Effects of Long Term Inhalation of Hypertonic Saline in Subjects With Cystic Fibrosis
Cystic FibrosisThe 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.
Nasal Inhalation of Dornase Alfa (Pulmozyme) in Patients With Cystic Fibrosis and Chronic Rhinosinusitis...
Cystic FibrosisChronic RhinosinusitisRhinosinusitis 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.
Mannitol Dose Response Study in Cystic Fibrosis
Cystic FibrosisMany 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.
PTC124 for Cystic Fibrosis
Cystic FibrosisIn some patients with cystic fibrosis (CF), the disease is caused by a nonsense mutation (premature stop codon) in the gene that makes the cystic fibrosis transmembrane regulator (CFTR) protein. PTC124 has been shown to partially restore CFTR production in animals with CF due to a nonsense mutation. The main purpose of this study is to understand whether PTC124 can safely increase functional CFTR protein in the cells of patients with CF due to a nonsense mutation.
Efficacy and Safety of 28 or 56 Day Treatment for Pseudomonas Aeruginosa in Children With Cystic...
Cystic FibrosisThis study assessed time to recurrence of infection with Pseudomonas aeruginosa following treatment of the initial infection with tobramycin nebuliser solution. The safety profile of the initial tobramycin treatment was assessed during the first 3 months of the study and patients were followed until the end of the study, month 27.
Safety of Tobramycin Inhalation Powder (TIP) vs Tobramycin Solution for Inhalation in Patients With...
Cystic FibrosisThis study compares the safety of the tobramycin solution for inhalation with the tobramycin dry powder formulation, used with a simple inhaler
Macrolide Antibiotic Therapy for Patients With Cystic Fibrosis
Cystic FibrosisThe purpose of this study is to evaluate whether Biaxin (clarithromycin) improves sputum abnormalities, lung function, and overall feeling of well-being in people with cystic fibrosis (CF). Biaxin is an antibiotic commonly used for the treatment of respiratory infections in people who do not have CF, and is sometimes used in CF patients as well. Studies done in a disease called diffuse panbronchiolitis (which is similar to CF) and some preliminary studies that have been done in CF patients suggest that Biaxin might have a beneficial effect on CF sputum in ways unrelated to its antibiotic activity.
Oral TheraCLEC™ - Total in Cystic Fibrosis Subjects With Exocrine Pancreatic Insufficiency
Cystic FibrosisPancreatic InsufficiencyThe purpose of this study is to determine the most effective dose of a new enzyme therapy on oral nutrient absorption in cystic fibrosis (CF) subjects with exocrine pancreatic insufficiency.