Active clinical trials for "Cystic Fibrosis"

This study was designed to investigate the relationship between upper extremity muscle strength and endurance, functional capacity, and quality of life child and adolescent with cystic fibrosis

A case of a patient with cystic fibrosis with bowel obstruction due to a proximal intestinal obstruction syndrome (PIOS) is presented.This syndrome can be diagnosed with the DIOS definition, with the only distinction of a more proximal location in the gastrointestinal tract, such as the stomach, the duodenum, or the jejunum.

Cystic fibrosis (CF) is autosomal recessive, genetic disorder cause of cystic fibrosis transmembrane regulatory (CFTR) gene mutation. CF often is observed in caucasian population. CFTR protein in cell apical membrane is canal responsible of transport sodium and clorid ions. Impaired sodium ion transport causes production viscous mucus. Disease include problems such as mucus, breathlessness and coughing. Blood glucose levels fluctuation are observed. This study aims comparison between lung function, functional capacity, muscle strength, physical activity, physical fitness and activities of daily living activities in cystic fibrosis with and without abnormal glucose tolerance

The goal of this clinical trial is to investigate the etiopathogenesis of isolated hypoglycemia and hypoglycemia with abnormal glucose tolerance in children with Cystic Fibrosis (CF) and to evaluate the role of glucagon and pancreatic insufficiency on hypoglycemia in CF. The main questions it aims to answer are: Do isolated hypoglycemia and hypoglycemia with abnormal glucose tolerance have different etiopathogenesis? What is the role of pancreatic insufficiency in these two conditions? Participants were asked to perform 3-h OGTT and to take blood samples. Researchers compared with healthy peers to see if there is isolated hypoglycemia in OGTT and how is the glucagon response to OGTT in healthy peers.

Recently, the respiratory microbiota characterisation of a Cystic Fibrosis (CF) patients' cohort has highlighted the potential role of anaerobes, and specially species belonging to the genus Porphyromonas, in the first P. aeruginosa colonization. The aim of this project is to describe the bacterial anaerobic population in the respiratory microbiota of a CF cohort. At the end of this study, an inventory of the anaerobic microbiota in CF respiratory samples will be establish in relation to the patients' pulmonary function and P. aeruginosa colonization status in order to speculate about the pulmonary anaerobes roles, still unknown. The innovative aspect of the ANA-MUCO study is the use of a specific sample kit designed for the study which allows preserving anaerobic bacteria in sputum according to the recommendations of the International Human Microbiome Standards (IHMS). Extended-culture and molecular approaches will be performed to identify and describe the anaerobic bacteria which could be involved in the pulmonary homeostasis in CF respiratory samples.

Cystic fibrosis (CF) is a chronic multiorgan disorder caused by mutations in the CFTR (cystic fibrosis transmembrane conductance regulator) gene. Chronic airway infection by bacterial pathogens accounts for the progressive, suppurative pulmonary disease that leads to significant morbidity and mortality in patients with CF. Neutrophil recruitment to the lungs accounts the most important contributor to pulmonary destruction. However, there is evidence that platelets may also have an important role in the pathogenesis of inflammation. To our knowledge, there is few information in platelet levels in patients with cystic fibrosis during pulmonary exacerbation, chronic airway colonization and when stable.

In patients with cystic fibrosis (CF) the commonest cause of death is respiratory failure. Respiratory failure can have many causes. However, in patients with CF a major contributor is the impairment of the muscles required for breathing (respiratory muscles). Respiratory muscle impairment can result from poor nutrition. Lung function declines particularly during adolescence whilst body composition also changes at the same time. Thus, the investigators plan to study the relationship of nutrition and body composition to respiratory muscle strength and lung function in children and young people with CF aged between 12-18 years. The body mass index (BMI) is currently used in the clinical setting to measure nutritional status in CF. At King's College Hospital (KCH) there are portable devices to assess both respiratory muscle function and lung function. The research team will use a Bioelectrical Impedance Analysis (BIA) device to assess body composition, including BMI and lean body mass (LBM). The aim of the study is primarily to assess whether measurements of LBM impairment may better relate to poor lung function compared to BMI and secondly to examine whether lung and respiratory muscle function correlates with exercise tolerance.

The objective in this study is collecting post-marketing information on the safety and efficacy of Ciproxan injection under the routine clinical practice.

identify and validate biomarkers that might reflect partial restoration of CFTR function and can be used to monitor disease progression, and ii) evaluate the mechanistic effects of CFTR modulators and other relevant therapies in individuals with CF

Shortness of breath (dyspnea) during exercise is a major source of distress and is a commonly reported symptom in patients with cystic fibrosis (CF). A recent treatment option known as Orkambi, which combines the drugs lumacaftor and ivacaftor, may be used in patients with CF to help improve lung health. However, the effects of this combination therapy on dyspnea and exercise performance, a known predictor of survival in CF, are not clear. The investigators aim to understand the effects of Orkambi on these symptoms and to gain new insight into the potential health improvements in CF from using this treatment option.