Active clinical trials for "Cystic Fibrosis"

Cystic fibrosis and bronchiectasis are the most seen problems in children with chronic pulmonary diseases. İt is a genetic, chronic system disease that reduces life expectancy, and life quality as well. Chronic lung disease , malnutrition, and reduced activity, caused by disease lead to postural disorders. Muscle force, endurance, activity of Daily living are adversely affected. İn the treatment pulmonary rehabilitation are using. Airway clearance technique, pulmonary exercises, upper extremity ergometer, dumbbells, elastic bands, proprioceptive neuromuscular facilitation technique are applying. Resistance training using elastic bands has become an increasingly common intervention aiming to improve function by increasing muscular strength. İn one study indicated that muscle strength can be improved through three dimensional spiral large scale resistive exercises using proprioceptive neuromuscular facilitation. İn the literature there isn't any research , uses elastic bands with proprioceptive neuromuscular facilitation for the upper extremity and evaluating pulmonary functions, posture, quality of life, muscle force. The aim of this study is giving exercises programme with proprioceptive neuromuscular facilitation and elastic bands and to evaluate pulmonary muscle force, pulmonary functions, posture, activity of daily living, quality of life, functional capacity. And to evaluate the effect of treatment programme on these parameters. The subjects were divided in two groups. An experimental group three times a week for 12 weeks will perform proprioceptive neuromuscular facilitation exercises with elastic bands, and pulmonary exercises. The control group will apply only pulmonary exercises at home programme.

The investigators aim to test the feasibility and effectiveness of a pre-meal snack to reduce the postprandial glycemia (PPG) excursion after a breakfast consumption in adult patients with cystic fibrosis (CF). The investigators also want to investigate the impact of a postprandial exercise to reduce the PPG excursion. An improvement in PPG excursions can improve the condition of CF patients primarily by delaying the onset of cystic fibrosis related diabetes (CFRD) and / or by reducing the risk of lung function loss.

The investigators will recruit 15 patients with cystic fibrosis 18 years of age and older who present with constipation. The investigators will assess baseline motility symptoms with a survey. Patients will then ingest a SmartPill (trademark) to obtain baseline motility within the GI lumen. All patients will undergo intervention with taking polyethylene glycol (PEG) or Miralax (brand name) 17 grams once daily. After two weeks of therapy, the patient will repeat the motility survey and again ingest a smart pill to assess the change in motility symptoms while on therapy.

Cystic Fibrosis (CF) is the most common autosomal recessive lethal disorders affecting 1:2.500 newborns among Caucasians. CF patients are peculiarly susceptible to infection and colonization of the respiratory tract with pathogens. In particular, Methicillin-resistant Staphylococcus aureus (MRSA) has become the third most prevalent bacterium in CF in the U.S. and has been increasing in other countries. Apart from the difficulty of treating the infection because of its antimicrobial resistances, MRSA is transmissible between individuals with and without CF. Chronic MRSA infection is associated with worse outcomes, and treatment/eradication is challenging. Antibiotic dosing and choices should be optimized to minimize further resistance and to maximize chances of successful therapy. Yet, MRSA has several mechanisms to escape clearance by the immune system and antibiotic killing. For these reasons, a better understanding of preventive measures and early therapy is of key importance. In consideration of all these assessments there is an emerging consensus that MRSA is an important pathogen in CF rather than simply a marker of severe disease. However, to date there are no guidelines or recommendations on the choice of antibiotics for MRSA in CF. Glycopeptides are an important class of antibiotics active against Gram-positive pathogens. These include teicoplanin and vancomycin, which are currently in widespread use and are active against MRSA. Teicoplanin is often preferred to vancomycin for intravenous treatment because of its better safety profile but its use in MRSA lung infection is limited by its limited lung penetration. Teicoplanin is mainly used for injection/infusion. Inhalation of anti-microbial drugs is a cornerstone in the treatment of patients with CF, since inhaled antibiotics decrease the rate of decline of lung function, improve the quality of life, and reduce the frequency of exacerbations and hospital admissions. It is expected that, using inhalation route, efficacy would be improved and risk of resistance reduced. At present, no antibiotic active against MRSA is available as an inhaled formulation. The objective of this phase I, first-in-man clinical study is to identify the dose providing, after single inhalation administration, a sputum Teicoplanin concentrations exceeding the drug concentration required to inhibit bacterial growth for at least 8 hours, while minimizing the development of resistance.

This is a Phase 2 open-label, dose-escalation study to evaluate the safety, tolerability, PK, and PD of multiple dose levels of SC administered ELX-02 with and without ivacaftor in patients with CF with at least one G542X allele. In total, up to 16 patients will be enrolled in the trial; up to 4 patients will be homozygotes for G542X, and the remaining patients will be compound heterozygotes with one G542X or phenotypically similar nonsense allele and any Class 1 or Class 2 mutation. Each patient will receive up to 5 escalating doses as follows: ELX-02 0.3 mg/kg per day SC ELX-02 0.75 mg/kg per day SC ELX-02 1.5 mg/kg per day SC An individualized dose of ELX-02, as high as 3.0 mg/kg per day SC, based on the patients observed safety and tolerability, PK at previous doses and the results of laboratory tests. ELX-02 1.5 mg/kg per day SC plus 150 mg ivacaftor every 12 bid

This study was evaluate the effect of elexacaftor (ELX)/tezacaftor (TEZ)/ivacaftor (IVA) on glucose tolerance in CF participants, 12 years of age and older who are heterozygous for the F508del mutation and a minimal function mutation (F/MF genotypes), with abnormal glucose metabolism.

Phase 1b/2a, double-blind, randomized, placebo-controlled, single and multiple ascending dose study to evaluate the safety, tolerability and phage recovery profile of AP-PA02 multi-bacteriophage therapeutic candidate administered by inhalation in subjects with cystic fibrosis and chronic pulmonary Pseudomonas aeruginosa (PA) infection.

The primary objectives of this study are to assess the safety and efficacy of MS1819 in enteric capsules vs porcine pancreatic enzyme replacement therapy (PERT) in patients with exocrine pancreatic insufficiency (EPI) due to cystic fibrosis (CF). The exploratory objective of the extension phase (EP) is to find a dose of MS1819 in immediate release capsules that is safe and results in CFA values in a therapeutic range.

This study will evaluate the efficacy and safety of elexacaftor (ELX) / tezacaftor (TEZ) / ivacaftor (IVA) triple combination (TC) in subjects 6 through 11 years of age with cystic fibrosis (CF) who are heterozygous for F508del and a minimal function (MF) mutation (F/MF genotypes).

This pilot study will evaluate the safety and tolerability of a low glycemic load dietary intervention in adult patients with cystic fibrosis (CF) in a rigorous feeding study. Specific emphasis will be placed on changes in weight, body composition, and glycemic measures obtained via continuous glucose monitor (CGM) usage.