View clinical trials related to Cystic Fibrosis.
Filter by:The goal of this observational study is to learn about the impact of home monitoring in adults with cystic fibrosis. The main questions it aims to answer are: - how people with CF find using home monitoring equipment - to see if by using home monitoring data acute respiratory exacerbations (chest infections) can be detected earlier than standard care Participants will be provided with a range of home monitoring equipment - - hand held spirometer (lung function) - weighing scales - oximeter (blood oxygen levels) - activity and heart rate monitor to measure health at home several times a week. This information links to an app on a smartphone which the participant and clinicians can see.
This Phase 1 study evaluated the safety, tolerability, and pharmacokinetics of SPL84 single ascending doses (SAD) in healthy volunteers (HV)
The investigators will be evaluating bone marrow composition via magnetic resonance imaging in adolescents diagnosed with cystic fibrosis (CF) compared to healthy, matched controls. The investigators will also be assessing their bone mineral density via other imaging modalities, including dual-energy X-ray absorptiometry (DXA) and peripheral quantitative computed tomography (pQCT). This longitudinal project will focus on abnormalities in bone marrow composition, and specifically whether adolescents with diagnosed with CF exhibit increased bone marrow fat, its association with bone mineral density (BMD) and the underlying pathophysiology, including glycemic control, inflammation, and bone turnover markers.
The objective of this study is to describe the renal impact of Elexacaftor-Teacaftor-Ivacaftor, a triple modulator therapy of CFTR channel, in patients with cystic fibrosis. This new treatment acts on the CFTR channel, which is expressed at the level of the nephrons. The objective is to study the changes in plasma and urinary parameters, including metabolic explorations of urolithiasis, change in volemic parameters, renal function, urinary sediment and nutritional and glycemic parameters, in newly treated patients, through the data collected at introduction of the treatment and during the follow-up.
This is a prospective, observational study examining the impact of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators on chronic rhinosinusitis (CRS) and olfactory dysfunction (OD) in young children with cystic fibrosis (YCwCF). This study involves two groups: children 2-8 years old, inclusive at initial visit, receiving highly effective modulator therapy (HEMT), and a control group of children 2-8 years old, inclusive at initial visit, not receiving HEMT. Outcomes will include sinus magnetic resonance imaging (MRI) scans, olfactory tests, and quality of life surveys obtained over a two-year period.
Cystic fibrosis (CF) is the most common hereditary life-threatening condition in Belgium. Because of a dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR) channel, chloride is unable to move to the cell surface and mucus becomes more viscous. Consequently, CF patients are not able to clear their lungs efficiently, and trapped bacteria can lead to chronic infection and inflammation of the lungs, and ultimately respiratory failure. CF lung disease starts at birth due to muco-inflammatory processes and is associated with a significantly altered microbial colonization of the infant airways compared to infants without CF. Additionally, young children with CF suffer from viral infections as often as their healthy peers, but the episodes are more severe and often prolonged. Moreover, frequent viral infections in children with CF contribute towards a more pathogenic airway microbiome at a young age. Although this link has been previously reported, the exact mechanisms by which this occurs need to be elucidated. A pulmonary exacerbation in CF is characterized by an increase in respiratory symptoms, general symptoms and a decline in lung function. Most young children with CF suffer from a mean of 4 exacerbations per year for which antibiotics are prescribed. Despite the current novel therapies in CF, treatment of respiratory infections stay relevant and is a greater challenge with increasing survival. The key objective of this study is to gain insights into the mechanisms by which viral infections leading to pulmonary exacerbations induce a more pathogenic microbiome in young children with CF. About forty participants will be recruited at the paediatric CF clinic of the Antwerp University Hospital. Inclusion criteria are an age of less than 5 years and a diagnosis of CF. There are no exclusion criteria. Duration of the study is 1 year to cover for seasonality of clinical symptoms. Study visits are scheduled at 3-month intervals corresponding with the regular follow up, or unscheduled during an acute pulmonary exacerbation. From all participants, two oropharyngeal swabs (for microbiome analysis and for immunological/mucin analysis) will be collected at set time points. For the linking of the laboratory data to the clinical characteristics, we will examine demographics, environmental exposures, and disease markers of CF. Next to the collection of the oropharyngeal swabs, a history, physical examination, and technical investigations will be performed at the study visits.
The aim of the study is to identify a link between the new CFTR modulators and physical activity in cystic fibrosis patients. The triple combination of CFTR modulators (ELEXACAFTOR / TEZACAFTOR/ IVACAFTOR) has recently changed the management of cystic fibrosis. This treatment has been shown to rapidly improve patients' respiratory function, with a gain in FEV1 at 1 month ranging from 10.4% to 13.6%. It also reduces the number of respiratory exacerbations and improves the nutritional status and quality of life of treated patients. To date, there is limited data on the impact of these new therapies on physical activity. Few studies have investigated changes in exercise or physical activity parameters under ELEXACAFTOR / TEZACAFTOR/ IVACAFTOR. The 6-minute walk test is a validated field test used routinely to assess the exercise capacity of patients with chronic respiratory diseases, including cystic fibrosis.
No studies in the literature have investigated the simultaneous effects of cognitive function, exercise capacity and arterial stiffness in adult patients with CF. The study aims to compare the cognitive function, exercise capacity, exercise muscle oxygenation, and arterial stiffness of adult patients with CF and healthy subjects.
In this exploratory study, the hormonal responses to a mixed meal will be examined in people with cystic fibrosis. The aim of this study is to find correlates with impaired glucose tolerance that is associated with this population.
CMTX-101 is a bacterial biofilm disrupting monoclonal antibody being developed as an adjunctive therapy to standard of care antibiotics. The goal of this clinical trial is to assess the safety and tolerability of CMTX-101 in people with cystic fibrosis (pwCF). The main questions the study aims to answer are: - Are single doses of CMTX-101 IV infusion safe and tolerated - What is the pharmacokinetic (PK) profile of single doses of CMTX-101 - Do single doses of CMTX-101 induce development of anti-drug antibodies (ADA) and neutralizing antibodies (Nabs)