
Breath Analysis in in Adults With Cystic Fibrosis (CF)
Cystic FibrosisExploratory comparative evaluation of exhaled breath composition in cystic fibrosis patients with age and gender-matched healthy adults in order to identify a disease-specific exhaled breath pattern as well as to gain insight into pathophysiological and microbial contributions to exhaled breath composition.

Study to Evaluate the Safety and Tolerability of GBT440 Administered to Subjects With IPF
Idiopathic Pulmonary FibrosisHypoxemiaThis study is a randomized, double-blind, placebo-controlled trial in which eligible IPF subjects will be randomized to receive GBT440 or Placebo orally daily.

Comparison of the Tolerability of Two Formulations of Hypertonic Saline in Cystic Fibrosis Patients...
Cystic FibrosisThe purpose of this study is to compare the tolerability and acceptability of a formulation containing Hypertonic saline 7% (HS) alone and a formulation containing HS and Hyaluronic acid 0.1% in a population of Cystic Fibrosis (CF) patients who already showed poor tolerance to HS.

Treatment of Muscle Cramps in Patients With Liver Cirrhosis
Liver CirrhosisMuscle CrampsThis is a pilot study to see if hydroxychloroquine (HCQ) if safe and effective to use with patients having cramps due to their cirrhotic liver disease.

Phase 1 Study of L-NMMA in Patients With Liver Cirrhosis
Liver CirrhosisPhase 1 study of the effects of nitric oxide inhibition with L-NMMA in patients with liver cirrhosis and healthy controls. It is hypothesized that nitric oxide availability is increased in liver cirrhosis.

Renal and Cardiovascular Effects of Terlipressin in Patients With Cirrhosis and Ascites
CirrhosisAscitesThe purpose of this study is to investigate the effect of terlipressin on myocardial perfusion and renal function. Hypothesis: Significant improvement of renal function reflected by: glomerular filtration rate (GFR), renal blood flow (RBF), excretion of salt and metabolites in the urine and related hormones and peptides in the blood. No effect on the systolic function of the heart.

The Efficacy of Azithromycin in Treating Children With Non Cystic Fibrosis Bronchiectasis
Non Cystic Fibrosis Bronchiectasis in ChildrenBronchiectasis is characterized by a permanent and abnormal dilatation of a part of the bronchial tree. An accumulation of mucus in the respiratory tract ensues, resulting in frequent bacterial infections and eventual destruction of the lungs. Clinically, patients present with a chronic productive cough and episodes of acute respiratory exacerbations. Chronic respiratory failure can follow. Although its prevalence is largely unknown, bronchiectasis is considered to be a rare and orphan disease. There are numerous causes for this disease: sequelae of respiratory infections, immunodeficiency, genetic diseases like cystic fibrosis, primary ciliary dyskinesia….The focus of this study will be on non cystic fibrosis bronchiectasis in children. Due to a lack of pediatric clinical trials, the management of children with this disease is widely based on the management of adults or patients with cystic fibrosis or pan-bronchiolitis. The treatment is based on respiratory physiotherapy, prevention of infections, administration of inhaled corticosteroids and anti biotherapy for acute exacerbations. Recently, some studies have demonstrated the efficacy of a family of antibiotics, the macrolides, in the treatment of cystic fibrosis or pan-bronchiolitis in children. Indeed, taken 3 times a week during a long period of time, the macrolides, and specifically the azithromycin have shown some anti-inflammatory and tissue repairing properties, in addition to their antimicrobial properties. Moreover, several studies conducted in the adult population have shown that the use of azithromycin has led to significant reduction in the frequency of respiratory exacerbations as well as an improvement in the quality of life. The efficacy of azithromycin in these respiratory diseases has led to enlarge its use for the long term treatment of bronchiectasis. Yet, this type of treatment has no marketing authorization application. No studies have been conducted in children, but individual examination of patients with bronchiectasis treated with azithromycin suggests an improvement of their symptoms. Thus, the use of azithromycin in the treatment of bronchiectasis in children seems to be a promising therapy. However, its efficacy needs to be demonstrated by clinical trials led on a pediatric population with an adequate number of patients and a strong methodology in order to ensure validity and reliability of the results. Therefore the investigators decided to conduct a comparative, prospective multicenter randomized study in this population. This study intends to include 100 patients already treated by azithromycin for at least 6 months. After inclusion, they will be randomized in two groups of 50 patients each. The first group will continue the treatment and the other one will discontinue it. The patients will be followed from the inclusion period (M0) until the onset of the first exacerbation for a maximum of 6 months (M6), with an intermediate health care visit after 3 months (M3). In case of exacerbation, a health care visit will be scheduled; the patient will receive the appropriate treatment and he will be suspended from the study. The duration of follow up for each patient is dictated by the occurrence of the first respiratory exacerbation, a maximum of 6 months .The total inclusion period is 12 months and the total duration of the study is 18 months.

Inhibition of Aldosterone to Reduce Myocardial Diffuse Fibrosis in Patients With Paroxysmal and...
Atrial FibrillationA randomized, double-blinded, placebo-controlled study to evaluate the effect of spironolactone in addition to conventional treatment compared with placebo in patients with paroxysmal and persistent atrial fibrillation with preserved left ventricular ejection fraction by T1 mapping, structure and function of left atrium and ventricle assessed by transthoracic echocardiography and cardiac magnetic resonance (CMR), the number of recurrent episodes of atrial fibrillation and biomarkers measured in blood.

Evaluation of Intestinal Permeability in Cirrhotic Patients Before and After Treatment With Non-selective...
Liver CirrhosesThis study seeks to investigate whether non-selective beta blocker treatment decreases intestinal permeability in cirrhotic patients by altering the expression of genes encoding intercellular junction proteins.

Metformin Experience on Minimal Hepatic Encephalopathy
Liver CirrhosisHepatic EncephalopathyPrimary aim: -To assess the effect of metformin use on the treatment of minimal hepatic encephalopathy in patients with liver cirrhosis. Secondary aim: -To evaluate if metformin is a safety drug in patients showing liver cirrhosis.