Active clinical trials for "Cystic Fibrosis"

The general aim of this project is to conduct a randomized, double-blind, placebo-controlled clinical trial of azithromycin to determine whether treatment from infancy is safe and will prevent the onset of bronchiectasis. One hundred and thirty infants will be recruited from CF clinics in Australia and New Zealand and treated from 3 months to three years of age. The primary outcome will be the proportion with radiologically-defined bronchiectasis at 3 years of age. Safety and mechanistic evaluations will also be undertaken.

Cystic fibrosis (CF) is a lethal genetic condition that affects 30,000 children and adults in the United States. Although CF management has improved substantially over the past two decades, there is still no cure and most patients with CF die before reaching their 50th birthday, largely due to lung failure. There is growing evidence that excess lung and blood inflammation that occurs in response to infections in the lungs cause CF patients to be sicker. Simvastatin is a drug that is used to lower cholesterol, but many researchers have found that this drug may also treat blood and lung inflammation. In this study, we will determine whether or not simvastatin can treat blood and lung inflammation in patients with CF and most importantly determine whether or not it can make these patients feel better and have better lung function.

The purpose of this study is to determine the pharmacokinetics and safety of inhaled vancomycin in patients with cystic fibrosis.

Multicenter prospective cohort of approximately 1000 cystic fibrosis patients followed in the Ile de France during the 2009-2010 influenza season with the main objective to assess the effectiveness of antiviral vaccination (H1N1). All subjects will be included, without excluding factor. In particular populations at risk are additional infants 6 to 23 months, pregnant women and lung transplant will also be included. These people will be vaccinated with un-adjuvanted vaccine. If flu symptoms until the results of PCR to confirm or refute the diagnosis of influenza, oseltamivir is administered in accordance with official recommendations and based on the results of the pharmacokinetic study.

The purpose of this study is to evaluate the safety and tolerability of GS-9411 in patients with Cystic Fibrosis. GS-9411 is a sodium channel inhibitor, that may restore airway hydration and mucociliary clearance in the lung.

Early diagnosis of liver fibrosis is useful for the follow-up and treatment of chronic liver disease. At present, the unique validated method to evaluate the liver fibrosis in children, is the liver biopsy which is an invasive method. If the elastometry method is proved to be a good method to evaluate the fibrosis in children, a numerous liver biopsy could be avoided.

This clinical trial will attempt to determine whether we can improve clinical outcomes for patients with cystic fibrosis who are infected with a fungus called Aspergillus fumigatus.

We hypothesize use of the insulin pump will improve body weight, lean body mass, whole body protein turnover, hepatic glucose production (HGP), and blood sugar control in CF patients with impaired glucose tolerance or patients with CF related diabetes (CFRD). We further hypothesize that HGP is also elevated in children/adolescents with type 1 diabetes and that the insulin pump will result in decreased HGP.

Cystic fibrosis (CF) is a catabolic condition, and children affected with this disease frequently have problems with growth, despite adequate nutrition. We hypothesize that the anabolic effects of growth hormone (GH) will improve the height and weight of CF patients and thereby improve their clinical status and their quality of life. We further hypothesize that these effects will be sustained for at least one year after GH treatment is complete.

The overall aim of this study is to find out whether taking regular inhaled steroids (eg Pulmicort, Flixotide, Becotide, Becloforte) is good for the lungs of children and adults with cystic fibrosis (CF). Some patients are put on inhaled steroids because they are wheezy despite taking regular bronchodilators (inhaled medicines that help open up the airways eg Ventolin, Bricanyl). Occasionally young children are put on them when they wheeze with colds, and have simply remained on them ever since. However many CF patients have been put onto inhaled steroids because their doctors thought it might reduce the inflammation in the lungs and help improve lung function. This inflammation (which is swelling of the lining of the airways) is known to be important in CF and results from recurrent chest infections. Although it is believed, in theory, that inhaled steroids should be useful for most CF patients, we are not sure how well they work in CF and it has not yet been possible to prove this with standard studies. This would normally involve starting inhaled steroids in patients who have not been taking them. We have therefore taken a different approach, namely to withdraw them from some patients who have been on them for a long time, to see if there is any effect of stopping them. It is important that we answer this question, as we do not want CF patients taking medicines that may be unnecessary. CF patients already have to take many oral and inhaled medicines and if we can cut down this burden, it would be helpful for everyone. Of course, we may find that patients do need these medicines but at least we will then be certain that it is for a good reason. The main hypothesis is that withdrawing inhaled steroids is not associated with an earlier onset of acute chest exacerbations.