View clinical trials related to Fibrosis.
Filter by:Idiopathic pulmonary fibrosis (IPF) is a progressive disease for which there is no effective treatment. Interferon-gamma is a medication that has been used for other lung diseases to decrease scarring and fibrosis. Studies of interferon-gamma injected under the skin did not show any improvement in survival in patients with IPF. We hypothesize that giving interferon-gamma as a nebulized mist directly into the lungs can affect the immune system in a way that decreases fibrosis.
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.
The Pennsylvania Idiopathic Pulmonary Fibrosis State-wide Research Registry (PA-IPF) is a cooperative project between five medical centers to coordinate a team of investigators. The aim of this registry will be: 1) To assess the extent of lung fibrosis in the commonwealth of Pennsylvania 2) To provide better access of patients with pulmonary fibrosis in all regions of Pennsylvania to standard of care and diagnosis 3) To facilitate the translation of new therapeutic interventions from the bench to the bedside.
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.
The purpose of this study is to investigate how QAX576 affects levels of interleukin 13 (IL-13) in patients with idiopathic pulmonary fibrosis (IPF).
It is well known that an association exists between the acquisition of some respiratory pathogens and prognosis of CF people. About 57 % of patients has a lung infection due to P.aeruginosa (PA). Transmission of PA between CF patients is possible and several prevention measures are recommended. Among these measures, all international guidelines recommend that CF people maintain a minimum distance of 1 meter between them. However, this recommendation is not supported by specific studies and scientific evidence. In other words, the investigators don't know if this measure is sufficient or excessive, as it is based only on a theoretical rationale. This study aims at measuring experimentally the distance that can be reached by PA emitted from airways of colonized CF patients during cough and during conversation. To this purpose, the investigators will evacuate the presence of PA on surfaces placed at 4 different distances from patients, through the collection of 8 swabs, 4 of which following cough and 4 following conversation. Swabs will be collected on sterile surfaces comprised between tra 0 - 0,5 mt; 0,5 -1,00 mt.; 1,00 - 1,5 mt and 1.5-2 mt. All the PA-positive CF patients of the CF Centre of Tuscany aged 14 or more will be recruited.