Active clinical trials for "Fibrosis"

Mucus clearance is impaired in cystic fibrosis. Inhaled surfactants may reduce adhesive forces between mucus and airway surfaces and improve mucus clearance. This in turn my improve lung health. The investigators propose to measure mucus clearance before and after lucinactant or vehicle administration in patients with cystic fibrosis.

The purpose of this study is to evaluate the safety and tolerability of escalating doses of GS-9411 in healthy male volunteers. GS-9411 is a sodium channel inhibitor, that may restore airway hydration and mucociliary clearance in the lung.

Working Hypothesis: EGCG and Tocotrienol can act as genetic modifiers and increase the level of correctly spliced CFTR transcripts. Aims of the Study: To determine in patients with CF if oral administration of EGCG and Tocotrienol, both separate and in combination, modify CFTR splicing towards normal splicing as evaluated by improved Transepithelial Potential Difference (TEPD) assessment of chloride secretion. To assess the effect of EGCG and Tocotrienol, both separate and in combination, on (1) additional TEPD measures of ion channel activity, (2) levels of correctly spliced CFTR mRNA in nasal mucosa, (3) cytokine levels in sputum and (4) changes in pulmonary function over the course of the study. Potential Implications to Medicine: Alternative splicing mechanisms are a common cause of genetic disease as ~15% of all known human mutations result in defective pre-mRNA splicing. Therapies based on augmenting the levels of full length or fully functioning proteins may have a substantial impact on the treatment of patients with genetic diseases. Contribution of the expected outcome to society Today genetic diseases can be treated but not healed. This proposal may be a step in the direction of finding a cure for patients carrying splicing mutations.

The study was to evaluate the long term effects, over one year, of five airway clearance techniques used by people with cystic fibrosis (active cycle of breathing techniques, autogenic drainage, positive expiratory pressure and oscillating positive expiratory pressure (R-C Cornet and Flutter)). The primary outcome measure was forced expiratory volume in one second (FEV1)and the null hypothesis was that there are no differences among the regimens.

This is an open-label, multi-center, extension study for patients with IPF who complete a qualifying InterMune clinical trial of pirfenidone. The purpose of this study is to obtain additional safety data for pirfenidone 2403 mg/day in patients with IPF who complete a qualifying InterMune clinical trial of pirfenidone.

The pilot study aims to evaluate the effects of a treatment series of gentle joint and muscle movements (in addition to normal optimal care)on lung function, exercise capacity and posture in stable adults with cystic fibrosis.

The purpose of this trial is to evaluate the safety and effectiveness of one dose strength of Denufosol compared to placebo in patients with CF and a predicted FEV1 of greater than or equal to 75% but less than or equal to 110% predicted.

The primary objective of this study is to evaluate the safety and tolerability of a single dose of KB001 in Cystic Fibrosis patients infected with Pseudomonas aeruginosa (Pa)

The purpose of this study is to determine the short term safety and effectiveness of lubiprostone when used for constipation in adults with cystic fibrosis.

The purpose of this study is to determine the effect of pioglitazone on reducing airway inflammation in cystic fibrosis and characterize the amount and timecourse of pioglitazone elimination from the body.