
Effect of Bosentan on Skin Fibrosis in Patients With Systemic Sclerosis
Systemic SclerodermaSkin Fibrosis1 moreEndothelin-1 is a potent vasoconstrictor and binds to two receptors, ET-A and ET-B, which are variable expressed on endothelial cells, smooth muscle cells, and fibroblasts. Furthermore, endothelin-1 has been found to be released in vitro by scleroderma fibroblasts and could contribute to the development of dermal fibrosis in systemic sclerosis. Bosentan is a dual receptor antagonist, that competes with the binding of endothelin-1 to both receptors and has already been approved for the treatment of pulmonary arterial hypertension in Europe, the US, and some other countries. The purpose of this study is to evaluate the effect of bosentan treatment on skin fibrosis and functionality in patients with systemic sclerosis.

Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary...
Idiopathic Pulmonary FibrosisThe objectives of this study are to assess the safety and efficacy of treatment with pirfenidone 2403 milligrams per day (mg/d) compared with placebo in patients with idiopathic pulmonary fibrosis (IPF), to assess the safety and efficacy of treatment with pirfenidone 1197 mg/d in patients with idiopathic pulmonary fibrosis and to characterize the pharmacokinetic disposition of pirfenidone in patients with idiopathic pulmonary fibrosis.

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.

Sildenafil to Increase Exercise Capacity in Individuals With Idiopathic Pulmonary Fibrosis and Pulmonary...
Pulmonary FibrosisHypertension1 moreIdiopathic pulmonary fibrosis (IPF) is a chronic lung disease that affects an individual's ability to breathe. Currently, no medications can reverse the damage to the lungs caused by IPF, but individuals are encouraged to engage in moderate exercise to strengthen lung function. This study will evaluate the effectiveness of sildenafil at improving exercise tolerance and breathing difficulties in individuals with IPF.

Cirrhotic Ascites Treatment With Satavaptan in Patients With Ascites Due to Cirrhosis of the Liver...
AscitesLiver CirrhosisPrimary:To evaluate the efficacy of satavaptan on top of conventional treatment in the treatment of clinically evident ascites in participants with cirrhosis of the liver. Secondary:To evaluate the tolerability and safety of satavaptan over a 52-week treatment period in participants with cirrhosis of the liver and ascites. The one-year double blind placebo controlled period is extended up to 2 years in a long term safety study (PASCCAL-2).

Study of Denufosol Inhalation Solution in Patients With Mild Cystic Fibrosis Lung Disease
Cystic FibrosisThe purpose of this trial is to evaluate the safety and effectiveness of a one dose strength of denufosol compared to placebo in patients with mild CF lung disease.

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

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.

Non-invasive Ventilation and Oxygen Therapy in Cystic Fibrosis Patients With Nocturnal Oxygen Desaturation...
Cystic FibrosisThe purpose of this study is to determine whether correction of low nighttime oxygen (O2) levels and/ or high carbon dioxide levels in patients with cystic fibrosis improves their quality of life. The treatments being used overnight are (1)O2 (2)pressurised air which assists breathing (non-invasive positive pressure ventilation, NIPPV)

International Safety and Efficacy Study of Aztreonam for Inhalation Solution (AZLI) in Cystic Fibrosis...
Cystic FibrosisThe purpose of this study was to evaluate the safety and efficacy of a 28-day course of aztreonam for inhalation solution (AZLI) in patients with cystic fibrosis (CF) and lung infection due to Pseudomonas aeruginosa (PA).