
The Effect of Hypertonic Saline on the Lung Clearance Index in Patients With Cystic Fibrosis
Cystic FibrosisThis study is examining the effect of hypertonic saline compared to placebo on the Lung Clearance Index in Cystic Fibrosis patients.

Study of the Effects of High-dose N-acetylcysteine (NAC) in Idiopathic Pulmonary Fibrosis (IPF)...
Pulmonary FibrosisThe purpose of this study is to determine whether NAC added to prednisone, and azathioprine has a better effect on lung function, radiology and clinical condition than placebo + prednisone in combination with azathioprine after 6 and 12 months.

Pilot Study of Imatinib Mesylate to Treat Nephrogenic Systemic Fibrosis
Nephrogenic Systemic FibrosisThe purpose of this study is to determine the efficacy of imatinib mesylate in reducing cutaneous thickening and tethering in patients with nephrogenic systemic fibrosis (NSF). The study will also work to assess the safety and tolerability of imatinib mesylate in patients with chronic kidney disease and NSF.

Safety and Efficacy Study of Aztreonam for Inhalation Solution (AZLI) in Patients With Cystic Fibrosis,...
Cystic FibrosisLung Infection1 moreThe 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), mild lung disease (forced expiratory volume in 1 second [FEV1] >75% predicted, and Pseudomonas aeruginosa (PA) infection.

Study Investigating a Delayed-Release Pancrelipase in Patients With Pancreatic Exocrine Insufficiency...
Cystic FibrosisPancreatic Exocrine InsufficiencyThis study will assess the effect of pancrelipase delayed release 12,000 unit capsules on fat and nitrogen absorption in subjects 7 - 11 with pancreatic exocrine insufficiency due to Cystic Fibrosis.

Cholecalciferol for Vitamin D in Adult Cystic Fibrosis (CF) Patients
Cystic FibrosisVitamin D DeficiencyThe main aim of the research question to test the primary hypothesis of this study, namely, Does 12 weeks of an additional 5000 IU daily of cholecalciferol increase serum 25OHD levels in adults with Cystic Fibrosis (CF) who have vitamin D deficiency relative to placebo?

Safety and Efficacy of Long-Term Treatment With Atorvastatin in Patients With Primary Biliary Cirrhosis...
Primary Biliary CirrhosisHypercholesterolemiaPrimary biliary cirrhosis (PBC) is frequently associated with hypercholesterolemia and possibly with an increased cardiovascular morbidity and mortality. Statins lower serum cholesterol levels and may thus improve the cardiovascular risk in PBC patients. The aim of our study therefore was to prospectively examine the efficacy of low-dose atorvastatin on indicators of cardiovascular risk such as dyslipidemia and vascular function as well as safety in patients with PBC.

Safety and Efficacy of Inhaled OligoG CF-5/20 for the Treatment Cystic Fibrosis
Cystic FibrosisCystic fibrosis (CF) is a disease caused by a mutation in the gene that makes the cystic fibrosis transmembrane regulator protein. As a result mucus stagnation, obstruction and plugging take place in the respiratory and gastrointestinal tract, the biliary and pancreatic duct, and in the reproductive system. The objective of this study is to determine the safety and tolerability of 3 days of daily dosing of OligoG CF-5/20 versus placebo in healthy volunteers.

Effects of Losartan on Hepatic Fibrogenesis in Chronic Hepatitis C
Chronic Hepatitis CLiver FibrosisThere is evidence on the beneficial effects of the administration of angiotensin II type 1 (AT1) receptors antagonists on liver fibrosis in hepatic stellate cells, experimental models of liver fibrosis in rodents and limited information in chronic hepatitis C with mild fibrosis. The purpose of this study is to investigate the effect of long-term administration of oral Losartan, an AT1 receptor antagonist, on liver fibrogenesis in patients with chronic hepatitis C and fibrosis F2-F3 (METAVIR score).

Comparison of Airway Clearance Efficacy of Two High Frequency Chest Wall Oscillation (HFCWO) Devices...
Cystic FibrosisPatients with cystic fibrosis (CF) must perform daily bronchial drainage therapy (BD) to keep their airways clear of secretions. Many different techniques are available to achieve this and there is currently no agreement as to which form of therapy is most effective. High frequency chest wall oscillation (HFCWO is used for this purpose by CF patients throughout the United States and abroad. To perform this therapy, the patient wears a vest which fits over the entire torso and is connected to an air compressor. The compressor generates oscillating air pulses that are transmitted to the lungs, thereby mobilizing secretions. The oscillations produced by the most commonly used device have a sinusoidal wave form (The Vest™ Airway Clearance System, Hill-Rom Inc, St Paul, MN). Previous studies indicate this form of therapy is as effective as more traditional and cumbersome forms of therapy. The design of the pulse generator has been recently modified so that the oscillations produced have a triangular wave form (Respitech Inc, MN). Studies done at the University of Minnesota found that the air flows induced in the airways can vary substantially depending on the wave form applied to the chest. At present, the impact of using a triangular wave form on mucus removal is unknown. The proposed study will compare mucous clearance using a device generating sine wave oscillations to a device generating triangular wave oscillations. Hypothesis: Bronchial drainage using a triangular wave form of HFCWO will result in superior sputum production compared to HFCWO using a sine wave form. Secondary Hypotheses: Bronchial drainage using a triangular wave form of HFCWO may result in sputum with rheologic properties distinct from sputum produced while using sine wave HFCWO. Bronchial drainage using a triangular wave form of HFCWO may result in superior post-therapy pulmonary function tests compared to pulmonary function tests obtained following therapy with sine wave HFCWO. Subjects will perceive bronchial drainage using a triangular wave form of HFCWO as more comfortable than airway clearance using sine wave HFCWO.