Active clinical trials for "Cystic Fibrosis"

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.

This study will measure the inflammatory effects of digitoxin on IL-8 and neutrophil counts in induced sputum in stable Cystic Fibrosis (CF) patients and the pharmacokinetics of digitoxin in serum. Funding Source-FDA OOPD

The purpose of this study is to determine whether the nasal inhalation of Gernebcin® is effective to decrease the Pseudomonas aeruginosa bacterial count in the nasal lavage fluid.

The purpose of this study is to assess the pharmacokinetics and safety of co-administration of VX-770 and VX-809 in healthy adults.

The purpose of the study is to evaluate whether Oncoxin, a nutritional supplement, improves the clinical and ultrasonographic results in comparison with placebo during 24 weeks of treatment.

The purpose of this study is to investigate within a short delay the effect of nasal instillation of Miglustat on nasal potential difference in cystic fibrosis patients homozygous for the F508del mutation.

Individuals with cystic fibrosis (CF) have persistent infection in the airways, which the body attempts to fight by recruiting immune cells (neutrophils) to the lung. The immune system and neutrophils are unable to completely kill the bacteria, and the response to the infection leads to inflammation (swelling) of the airways and lung damage. Nitric oxide (NO) has anti-bacterial and anti-inflammatory properties in the lung. NO production is decreased in CF patients, and may contribute to the persistent infection and inflammation. Increasing the production of NO in the airways of CF patients may help decrease this inflammation and infection. Rho GTPases are molecules in the cells that line the airways that decrease the protein that makes nitric oxide (NOS). Rho proteins also increase inflammation in these cells. Rho proteins are increased in CF cells, and may partially explain the low NO and high inflammation seen in CF. Blocking the Rho protein in CF cells increases NOS, which can then produce more NO. The Rho protein can be inhibited with a drug, simvastatin (Zocor®). Simvastatin is used by millions of people to lower their cholesterol, is very safe, has few side-effects and is approved for use in children greater than 10 years of age. We propose that treating CF patients with simvastatin will increase NO produced (exhaled NO), and may decrease airway inflammation. If simvastatin has these expected effects in CF, it would be another drug that has potential to become a new therapy to fight the debilitating lung damage of the disease.

Specific Aim: To determine whether neutrophils, active elastase, and cytokines measured in sputum induced using hypertonic saline are useful screening tests for determining if a particular agent with known anti-inflammatory properties is a suitable candidate for more extensive clinical trials in patients with CF. This aim will be addressed using an anti-inflammatory agent, ibuprofen, that has been shown to have clinical benefit in CF. A "no treatment" arm will be included as the control group.