Ease of Use and Microbial Contamination of Tobramycin Inhalation Powder (TIP) Versus Nebulised Tobramycin...
Cystic FibrosisThe purpose of this interventional Phase IV study was to explore the ease of use of TIP and prevalence of microbial contamination of the T-326 Inhaler compared with TIS and colistimethate administered via nebuliser for the treatment of Cystic Fibrosis (CF) patients chronically infected with P. aeruginosa. It was anticipated that the data from this study would provide clinicians with further guidance on the relative differences between the speed and ease of use of these treatments as well as useful information on the prevalence of microbial contamination of the inhalation devices in "real world" use.
Comparison of 2 Treatment Regimens for Eradication of P Aeruginosa Infection in Children With Cystic...
Cystic FibrosisTreatment of new Pseudomonas aeruginosa (Pa) infection in cystic fibrosis (CF) can postpone chronic infection. Aim of the study: compare 2 Pa eradication regimens in children with new Pa infection.
Evaluation of the Timing of the Nebulization Related to the Physiotherapy Session
Cystic FibrosisThe purpose of this study is to determine the optimal time for the nebulization depending on the respiratory physiotherapy session in cystic fibrosis patients.
Nasal Inhalation of Pulmozyme in Patients With Cystic Fibrosis and Chronic Rhinosinusitis
Cystic FibrosisRhinosinusitisRhinosinusitis disorders are often associated with Cystic Fibrosis. They can restrict quality of life enormously and give cause to repeated ENT surgery. The basic defect in CF is a dysfunction of chloride channels in exocrine glands, leading to retention of secretions and consecutive chronic inflammation with bacterial superinfections. The prospective placebo controlled cross-over study aims at the evaluation of a nasally inhalation of Pulmozyme with respect to mucus retention and resulting inflammation.
Ready to Use Therapeutic Food (RUTF) to Promote Growth in Cystic Fibrosis
Cystic FibrosisChildren with cystic fibrosis require increased caloric intake to maintain appropriate growth, an important determinant of long-term outcomes. This study seeks to determine the feasibility of using a novel therapeutic food to promote weight gain and growth in children with cystic fibrosis.
Cystic Fibrosis Microbiome-determined Antibiotic Therapy Trial in Exacerbations: Results Stratified...
Cystic FibrosisAntimicrobial resistance is a significant challenge facing global healthcare. The unnecessary use of antibiotics is a key driver in the development of antibiotic resistance. Cystic Fibrosis (CF) represents a unique disease model to study bacterial resistance and to explore therapeutic strategies for same, as chronic lung infection overlaps with acute lung exacerbation's caused by a multitude of organisms. With time, chronic polymicrobial infection develops, with the most dominant infecting organism being Pseudomonas aeruginosa. In acute CF infections, empiric intravenous antibiotics are usually given for two weeks. Recurrent infections and treatments result in increasing antimicrobial resistance, and alterations in pathogen host interactions in the lung and gut flora. Next-generation DNA sequencing technology now offers DNA-based personalised diagnostics and treatment strategies. Enhancing our knowledge of the microbiome allows the use of stratified targeted antibacterial therapy that can be compared with standard empirical antibacterial therapy currently used. Cystic Fibrosis Microbiome-determined Antimicrobial Therapy Trial in Exacerbations: Results Stratified (CFMATTERS) will provide a randomized multi-centre controlled trial of microbiome-derived antimicrobial treatments versus current empirical therapy.
CHaractErizing CFTR Modulated Changes in Sweat Chloride and Their Association With Clinical Outcomes...
Cystic FibrosisThis is a multicenter, cross-sectional, cohort study which will collect contemporary sweat chloride (SC) values from approximately 5000 Cystic Fibrosis (CF) patients prescribed and currently receiving commercially approved Cystic Fibrosis transmembrane conductance regulator (CFTR) modulator therapies.
Inspiratory Muscle Training in Individuals With Cystic Fibrosis
Cystic FibrosisCystic fibrosis is a genetic disease that affects some organs of the human body. Among them, the lungs tend to be the most affected due to the accumulation of mucus in the airways, which in addition to avoiding the passage of air, favors pulmonary infections. With the evolution of the condition, secondary complications arise, such as postural changes, decreased respiratory muscle strength, decreased functional capacity and, consequently, quality of life. Therefore, respiratory muscle training may be an intervention that improves the respiratory condition of these individuals, allowing an improvement in the quality of life and may delay the evolution of respiratory symptoms. Thus, this study aims to investigate a home protocol of respiratory muscle training on respiratory muscle strength, lung function, quality of life, posture and functional capacity in adolescents and adults with cystic fibrosis. The researchers believe that the training can cause an improvement in the studied variables, and can be inserted in the usual treatment of these patients.
Longitudinal Assessment of Transient Elastography in Cystic Fibrosis
Cystic Fibrosis Liver DiseaseTo determine if transient elastography (TE), when combined with ultrasound (US) pattern characterization can improve the prediction of progression to a nodular pattern on US. To confirm the feasibility of obtaining TE measurements in children with Cystic Fibrosis (CF) To prospectively assess whether TE data are associated with conventional laboratory markers of hepatic fibrosis To determine the variability of TE measurements taken at different sites in the same patient
SynRinse Irrigation Pilot (SIP) Trial
SinusitisChronic1 moreAssess if the use of SYNRINSE can improve short-term subjective and objective outcome measures after one week in patients with active Chronic Rhinosinusitis (CRS) who have had prior sinus surgery.