Active clinical trials for "Cystic Fibrosis"

Cystic Fibrosis patients continue to have bowel problems even after adequate pancreatic enzyme supplementation. There may be pathology of the lining of the bowel. Capsule endoscopy will be used to photograph the entire bowel.

People with cystic fibrosis (CF) now frequently live into adulthood and with this extended life expectancy has come new clinical problems. Poor bone health, including osteoporosis and bone fractures, is one of these increasingly important conditions. Preventing the negative outcomes of poor bone health in later life is primarily related to ensuring optimal growth (weight and height) and obtaining maximal amount of bone mass during growth and development. This study will identify factors that influence bone health in a sample of children, adolescents and young adults as measured by dual energy x-ray absorptiometry and new bone densitometry methods (peripheral quantitative computerized tomography [p-QCT] and bone sonometer). We will also identify factors which influence changes in bone status over a 12-month follow-up period in a subsample of people with CF.

Many people with Cystic Fibrosis (CF) are troubled by symptoms from their stomach and bowels: their gastrointestinal (GI) tract. Symptoms affect quality of life and can also reduce people's ability to digest enough calories to remain healthy, leaving them undernourished and less able to deal with other health problems such as infection. Clinical tests to assess bowel function are limited. Many tests involve inserting a sensor or camera into the bowel, so they are not suitable for long periods, and can be uncomfortable. In Nottingham the investigators have developed imaging scans which can assess how someone's digestion works without any invasive device. The type of scanning the investigators use is called Magnetic Resonance Imaging, or MRI. The purpose of this study is to see if those scanning methods can be used in people with CF to understand their digestion and any problems they have.

Introduction: In recent years, since the discovery of the cystic fibrosis (CF) transmembrane conductance regulator gene in human skeletal muscle, there appears to be growing interest in the measurement of muscle function in CF. One of the most used test in other chronic pulmonary diseases is the Sit-to-Stand test (STS) which consists of simply getting up from a chair. Although the main result of the STS test is the time developed during the task, the velocity and power generated during the task are considered very important variables to detect the functional decline. However, from our knowledge, no study has previously analyzed the differences in time, velocity and muscle power developed during the STS test in patients with CF and their respective healthy controls. Objectives: To compare the values gained from handgrip strength, walking speed and STS test (time, velocity, and muscle power) in a group of patients with CF and their respective healthy controls, and to analyze if these differences (if any) are associated with lung function in patients with CF. Methods: Cross-sectional study with a sample of 60 participants (30 patients diagnosed with CF and 30 healthy subjects) between 18-65 years old. The STS test will be measured through slow-motion video recording with a smartphone device (240 images per second) which will report the time, velocity and power generated during the test. Walking speed and handgrip strength will be also measured. Additionally, the relationship between the variables obtained during the test and the lung function of patients with CF will be analized.

The goal of this study is to implement a non-invasive version of the sweat secretion test based in visualization of sweat glands following β-adrenergic stimulation of sweat. Specifically, the trial will evaluate the relative response of sweat glands to β-adrenergic and cholinergic stimulation among participants with CF, heterozygous and matched control subjects

Retrospective cohort study using routinely collected annual data on lung clearance index (LCI) in combination with clinical data to predict survival in patients with Cystic Fibrosis. The primary study endpoint is the association of LCI with the compound outcome survival or lung transplantation in patients with CF.

The purpose of this study is to investigate the utility of a continuous glucose monitor device (CGM) in screening for cystic fibrosis related diabetes. The investigators will also study how fat deposition in the pancreas and liver impacts insulin production and response, as measured by a frequently sampled oral glucose tolerance test.

The purpose of this study is to determine vaccination coverage of recommended vaccines (routine childhood vaccines and vaccines against seasonal flu and pneumococci) in children with chronic diseases (allergy, cystic fibrosis, diabetes mellitus type 1, congenital heart disease, immunocompromised and solid organ transplant patients) the level of protection against measles, mumps, rubella and pertussis in children with chronic diseases. vaccination coverage of recommended vaccines (diphtheria, tetanus, pertussis and vaccines against seasonal flu and pneumococci) in adults with chronic diseases (nephropathy, diabetes mellitus, COPD, heart failure, HIV and solid organ transplant patients) the level of protection against diphtheria, tetanus and pertussis in adults with chronic diseases.

Following the results of the research team's cross-sectional study conducted in 2014 describing insufficient gynecological care, the research team implemented an on-site gynecological consultation in the adult Cystic Fibrosis (CF) centre in 2015. The study aimed to compare the results of two surveys conducted successively in 2014 and in 2017 on a cohort of women with CF attending the Lyon CF adult referral centre. All the women attending the adult CF center in 2017 were asked to complete the same self-report written questionnaire that was completed in 2014 about their contraceptive choices, gynecological follow-up and cervical screening. Questions concerning gynecological comorbidities were added to the 2017 questionnaire. This current study aimed to evaluate the impact of the implementation of an on-site gynecological consultation on gynecological health of women with CF, as measured through 3 indicators: the proportion of women with regular gynecological follow-up, cervical screening coverage, and contraceptive coverage. The identification of specific gynecological comorbidities was the other objective of this study.

Cystic fibrosis (CF) is the most common genetically inherited disease in the Caucasian population. Bilateral lung transplantation (LUTX) is a viable option for these patients. Frequently, the surgical operation of LUTX is complicated by hemodynamic instability, intractable hypoxia and respiratory acidosis. For these reasons, Intraoperative extracorporeal life support - ECLS- is required. Data on predictors of use of intraoperative ECLS in CF patients undergoing LUTX is scarce. Aim of this retrospective observational study was 1) to find possible risk factors at the time of enlistment associated with the intraoperative use of ECLS and 2) to compare the outcomes of CF patients treated with ECLS during LUTX or not.