
Backpack Carrying in Children With Cystic Fibrosis
Cystic FibrosisCystic Fibrosis is a hereditary, chronic respiratory illness. Cystic Fibrosis leads to a progressive decline in lung function. School-aged children with cystic fibrosis experience backpack carrying everyday. Backpack carrying induce a restrictive effect responsible for lower lung function. Respiratory muscle strength is also impaired. No studies assessed aerobic capacities during children's gait while carrying a backpack. The investigators hypothesized that backpack carrying will induce an acute decline in lung function in children with cystic fibrosis compared to healthy children. Investigators also hypothesized that aerobic capacities will be impaired.

Anticoagulation for Non-occlusive Portal Vein Thrombosis in Patients With Liver Cirrhosis
Liver CirrhosisPortal Vein Thrombosis1 moreThe purpose of this study is to determine the safety and efficacy of anticoagulation in the treatment of non-occlusive portal vein thrombosis in patients with liver cirrhosis.

A Multiple Dose Study to Evaluate the Safety, Tolerability, Pharmacokinetics and Pharmacodynamics...
FibrosisThe purpose of this study is to assess the safety and tolerability of BMS-986263 in healthy volunteers.

Oral Fecal Transplant in Cirrhosis
Hepatic EncephalopathyCirrhosis1 moreTo evaluate the safety and tolerability of oral fecal transplant in patients with cirrhosis and hepatic encephalopathy

Simplification of CF-related Diabetes Screening at Home
Cystic FibrosisCystic Fibrosis-related DiabetesCystic fibrosis (CF)-related diabetes (CFRD) is the most common complication after pulmonary complications. This specific form of diabetes is associated with an increased morbidity and mortality. CFRD prevalence at the age of 10 is 10% and reaches 40 to 50% in adulthood, while another 35% of adult patients presents impaired glucose tolerance. In order to identify patients at risk and to implement early therapeutic measures, an annual CFRD screening test should therefore be undertaken for CF patients after 10 years of age. The 2-hour oral glucose tolerance test (OGTT) with a sweet beverage is the recommended screening test. However, participation rates for screening tests are far from optimal. For examples, in 2015, the investigators observed that only 47% of non-diabetic patients attended to planned screening despite large availability and advertisement (unpublished data). Comparable low levels of screening for CFRD, usually below 33%, have been reported by various teams. Several reasons could explain these low adherence rates. Some factors are related to patients perceptions and experience: OGTT is perceived as an additional medical burden requiring a scheduled appointment (several weeks after the last exacerbation); overnight fasting followed by the intake of a large glucose load within 5 minutes can lead to nausea, headache, bloating and fatigue; some patients fear multiple blood sampling, etc. In addition, in case of CFRD diagnosis, recommended capillary blood glucose monitoring, nutritional advice and treatment (insulin) are perceived as extremely invasive and complex, thus some patients prefer avoiding screening test. To date, no alternative screening method has demonstrated its effectiveness to screen for CFRD. The investigators of this study believe that a simplified version of the OGTT would be more attractive, would make it more acceptable for patients and has the potential to improve their adherence to screening tests, simplify CF-team works and reduce costs. By allowing appropriate education and introduction of treatment in a timely manner, improved adherence to annual screening for dysglycemia has the potential to minimize or prevent clinical deterioration observed in years preceding CFRD onset.

Long Term Efficacy and Safety of Inhaled Colistimethate Sodium in Bronchiectasis Subjects With Chronic...
Non Cystic Fibrosis BronchiectasisThe primary objective of the trial is to investigate the effect of the use of inhaled colistimethate sodium, administered twice daily via the I-neb for 12 months, compared to placebo in subjects with non-cystic fibrosis bronchiectasis (NCFB) chronically infected with P. aeruginosa on the frequency of pulmonary exacerbations.

Effect of Vivomixx® on Neuroinflammation in Patients Withs Cirrhosis
CirrhosisLiverStudy Design: Double-blind randomized placebo-controlled clinical trial Study Duration: 2 years Study Center: Single center Hospital de la Santa Creu i Sant Pau, Barcelona Objectives: To assess the effect of Vivomixx® on neuroinflammation and systemic inflammatory response in patients with cirrhosis

A Dose-finding Study of Inhaled OligoG vs Placebo in Patients With Cystic Fibrosis
Cystic FibrosisRandomized, double blind, placebo controlled study. The study has two parts: Dose-finding part, followed by longer term follow-up (6 months)

The Effectiveness of the Jamboxx Respiratory Therapy Device: Study 2
Cystic Fibrosis in ChildrenThe Effectiveness of the Jamboxx Respiratory Therapy Device in Treatment of Patients with Decreased Respiratory Function is a proposal for investigation of the application of gaming to improving respiratory health. The Jamboxx device combines gaming with traditional incentive spirometry to provide users with a fun experience to keep them engaged in their respiratory therapy routine. The device allows users to play a series of mini-games that walk them through their routines. The Jamboxx also records airflow and lung parameters with an external mouthpiece attachment to provide users with real time feedback, and helps to assess increases or decreases in relative lung function over time. The Jamboxx has the potential to significantly impact the field of respiratory therapy by being one of the first gaming devices for patient therapy, and the first respiratory therapy gaming device that is accessible to users with limited mobility. Jamboxx provides a fun and engaging, low cost alternative to the traditional therapy techniques used and aims to improve patient compliance. This study addresses the ongoing challenge of clearing the burden of bronchial secretions resulting from cystic fibrosis. These patients are dependent upon mechanical devices to help clear secretions. A device autonomous means for clearing secretions is well defined in the literature (autogenic drainage) but is difficult to learn. This study proposes to teach cystic fibrosis patients to master autogenic drainage, and seeks to determine at what age it can be taught. As it would be expected that gaming could easily teach adults this procedure, the study will include children for whom learning autogenic drainage based upon conceptualization would be expected to be very difficult.

A Study of Oral Vismodegib for the Treatment of Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic Pulmonary FibrosisThis is a randomized, multicenter, double-blind, placebo-controlled, parallel-group study of vismodegib in patients with IPF. Eligible patients will be randomized in a 2:1 ratio to one of two treatment arms: vismodegib or placebo. The duration of treatment will be 52 weeks. Study drug will be administered daily by the oral route. An 8-week safety follow-up period is included for all patients who receive at least one dose of study drug.