Active clinical trials for "Cystic Fibrosis"

Cystic fibrosis (CF) is a genetic disease characterized by abnormal chloride transport in epithelial tissues. Inflammation is a key component contributing to the main alterations induced by the disease. The increase in life expectancy comes with a higher prevalence of CF-related comorbidities, but also with new emerging complications directly related to aging. Physical fitness and exercise practice have been previously associated to survival and better quality of life in patients with cystic fibrosis. Thus, the aim of this study is to assess the effects of a remotely supervised resistance exercise program on lung function, muscle strength, body composition, quality of life and inflammatory markers in adult patients with cystic fibrosis.

Cystic fibrosis (CF) is a genetic disease that affects many organs and systems, especially respiratory system problems due to lung damage. Patients often have difficulty in removing the sticky and viscous secretion that accumulates in the respiratory tract, and the risk of mortality increases with the development of respiratory failure. In patients with CF, exercise capacity, peripheral muscle strength, core endurance, flexibility, postural stability, physical activity level, and quality of life also decrease secondarily. Recently published guidelines recommend respiratory physiotherapy for coping with CF-related symptoms and recommend referral of patients to physical activity and exercise. Hippotherapy simulator is a mechanical exercise tool that imitates the walking movement of a real horse and is used to increase physical fitness parameters. This study aims to show the effects of exercises performed with a hippotherapy simulator in addition to respiratory physiotherapy on physical fitness, sputum production, physical activity and quality of life of children with CF.

Cystic fibrosis related diabetes (CFRD) is a major factor of morbidity and mortality at all disease stages. Insulin deficiency has serious clinical consequences by increasing malnutrition, since protein and lipid catabolism is accelerated in chronic infections. Traditionally, insulin is injected before a meal. Yet, in these patients with highly varied and often staggered nutritional intakes, insulin injection can result in an increased risk of postprandial hypoglycaemia, all the more so as CF patients exhibit decreased glucagon secretion. Recent progress in the development of new insulins mimicking the physiological secretion more closely has led to ultra-fast insulins (fast aspart), allowing for postprandial hyperglycaemia to be better controlled. In Type 1 diabetics treated with basal-bolus, faster-acting aspart insulin injected after a meal enabled metabolic control comparable to injection of aspart insulin prior to the meal. Fast apart insulin is of particular interest with regard to CFRD, wherein postprandial hyperglycaemia occurs early. In CFRD, these insulins are likewise advantageous in that they can be injected after the meal, thus permitting more flexibility in patients with highly varied diets. Moreover, the insulin dose can be adapted depending on dietary intake, thus preventing hypoglycaemia secondary to highly-varied carbohydrate intakes. Due to its flexibility, this insulin therapy is likely to be better accepted by patients with cystic fibrosis.

This study will provide important mechanistic information regarding the effect of inhaled mannitol (Bronchitol) in people with cystic fibrosis (PwCF) with moderate to severe disease who are already using elexacaftor/tezacaftor/ivacaftor (E/T/I). Many patients have already discontinued hypertonic saline and other pulmonary therapies because of the profound effect of E/T/I of their symptoms and lung function. Further, because both inhaled osmotic agents (i.e., Bronchitol, hypertonic saline [HS]) and E/T/I are believed to exert their beneficial effects through improvements in mucociliary clearance (MCC), it is unknown if the combination of these therapies might be additive or are redundant in a population with moderate to severe disease where bronchiectasis and chronic infection persists, and where eventual decline in lung function is expected over time. This study, therefore, will be the first to determine whether "add on" therapy with inhaled mannitol is able to further accelerate MCC in E/T/I patients. These data would provide some guidance regarding the use of these approved therapies in PwCF.

The main objective of the study is to evaluate the short-term effects of the airway clearance technique by the medical device Simeox (Physio Assist, France) versus Autogenic Drainage, on the pulmonary function of adult patients with stable cystic fibrosis. To analyse these effects, the investigators used forced oscillation technique measured by TremoFlo™ C-100 Airwave Oscillometry System™ (THORASYS Thoracic Medical Systems Inc. Montreal, Quebec, Canada) with an evaluation of the perceived clinical benefits.

The purpose of this study is to look at pulmonary exacerbations in people with cystic fibrosis (CF) that need to be treated with antibiotics given through a tube inserted into a vein (intravenous or IV). A pulmonary exacerbation is a worsening of respiratory symptoms in people with CF that needs medical intervention. Both doctors and CF patients are trying to understand the best way to treat pulmonary exacerbations. This study is trying to answer the following questions about treating a pulmonary exacerbation: Do participants have the same improvement in lung function and symptoms if they are treated with one type of antibiotic (called beta-lactams or β-lactams) versus taking two different types of antibiotics (tobramycin and β-lactams)? Is taking one type of antibiotic just as good as taking two types?

The main objective of this study is to determine whether closed-loop glucose control is superior to standard insulin therapy with continuous glucose monitoring (CGM) in young people (≥16 years) and adults with cystic fibrosis (CF) related diabetes. This is an open-label, multicentre, randomised, single-period, two-arm parallel design study, involving a run-in period followed by a 26 week intervention period during which glucose levels will be controlled either by a hybrid closed-loop system or by participants usual insulin therapy with continuous glucose monitoring. A total of up to 128 young people and adults (aiming for 114 completed participants) with CF related diabetes using insulin will be recruited through outpatient CF and diabetes clinics and other established methods at participating centres. Participants who drop out of the study within the first 4 weeks of the intervention period will be replaced. Participants will receive appropriate training in the safe use of the CGM and closed-loop devices. Participants will have access to the study team during the intervention phase with 24/7 telephone support. The primary outcome is time spent in target range between 3.9 and 10.0 mmol/L as recorded by CGM over the 26 week period. Other key endpoints include time above target glucose range (>10mmol/L), mean glucose, and HbA1c. Secondary outcomes include time spent with glucose levels below target as recorded by CGM, and other CGM-based metrics in addition to percent of predicted FEV1, body mass index, fasting C-peptide levels, insulin requirements and number of pulmonary exacerbations and hospitalisations. Safety evaluation comprises severe hypoglycaemic episodes, and other adverse and serious adverse events. Psychosocial outcomes include CGM & closed-loop usage, health-related quality of life questionnaires, burden of diabetes management assessment and semi-structured interviews after participants have had at least three months experience of using the technology. Data will be collected for future health economic analysis.

Cystic fibrosis (CF) is an inherited, genetic disease of the body's mucus-producing glands that primarily affects the lungs and gastrointestinal tract. There are no studies that have examined anatomical changes, the connection between structure and function in the ribcage and the effect of symptom-relieving manual treatment. The purpose of the study is therefore to investigate chest mobility in people with CF. Method The study is conducted in three parts; a / A retrospective longitudinal part whose purpose is to investigate possible changes in the chest configuration in relation to deterioration of lung volumes in a cohort of CF patients. Chest configuration will be measured standardized and blinded on computed tomography (CT) images and related to results from spirometry examinations. b / A prospective, consecutive cross-sectional study of the same cohort. The aim is to investigate the extent of stiffness and pain that is examined standardized (number of pain-free / normal moving structures) and its relation to objective examination of respiratory movements, respiratory muscle strength and spirometry. c / A randomized controlled single-blind study aimed at evaluating the effect of manual treatment for pain and reduced mobility in patients with these symptoms. The treatments consist of standardized manual therapy with passive joint mobilization without impulse and soft tissue treatment. Evaluation will be done via the examination protocol in sub-study b / as well as objective measurements of respiratory movements (primary variable), respiratory muscle strength and spirometry which will be performed by a blinded tester both before and immediately after the intervention / control period. Clinical significance When it comes to CF care, great medical advances have been made and for Swedish patients, the physiotherapeutic active treatment has proven to have very good effects. However, there are areas where care can be improved. The results from our study will provide additional breadth to strategies in CF care

This is a 3-part, single-ascending dose Phase 1a randomized, double-blind, placebo-controlled study in healthy volunteers (Part A) and multiple-ascending dose Phase 1a randomized, double-blind, placebo-controlled study in healthy volunteers (Part B), and a Phase 1b open-label study in subjects with CF (Part C) to assess the safety, tolerability, PK, and preliminary efficacy of ABCI. Subjects will be evaluated for eligibility during Screening within 30 days prior to Day 1 (Randomization; Visit 3). In Parts A and B, eligible healthy volunteers may be enrolled in the study and randomly allocated to treatment with ABCI or placebo as described below. In Part C, eligible subjects with CF may be enrolled in the study and receive treatment with ABCI as described below. Approximately 72 healthy subjects total will be randomized to 9 cohorts (48 subjects in 6 cohorts in Part A, 24 subjects in 3 cohorts in Part B) and up to 12 subjects with CF will receive the medium dose (2 sentinel subjects) or high dose (up to 10 subjects) of ABCI in Part C.

The aim of this study is to examine the effects of different exercise modalities applied with tele-rehabilitation on functional capacity, oxidative stress and respiratory parameters in children with cystic fibrosis