View clinical trials related to Cystic Fibrosis.
Filter by:The purpose of this research study is to begin an exercise program for patients with a cystic fibrosis (CF) exacerbation.
The purpose of this study was to evaluate physical activity, exercise capacity, quality of life, cognitive status level and coronavirus phobia level of adult cystic fibrosis patients and compare with the findings of healthy subjects with teleconference during COVID-19 pandemic.
The purpose of this study is to compare the performances of ultra-low dose computed tomography (CT) and lung magnetic resonance imaging (MRI) for morphological assessment of cystic fibrosis-related lung disease and to compare their performances to conventional low dose CT
Patients and parents of patients will be asked to answer a questionnaire on paper or online, by the CRCMs and through communication campaigns distributed by the association Vaincre La Mucoviscidose (VLM). This questionnaire includes about fifty items that will take 5 to 12 minutes to complete. The study aims to interview 250 patients / parents of patients, users and non-users of essential oils. Thirty patients or volunteer parents will then meet with a health anthropologist for qualitative interviews to document very precisely the modalities of use of essential oils: which ones, at what dose, advised by whom, the dedicated budget. Relationships with CRCM doctors, the effects felt, the side effects will also be explored. The global data of the two phases will be integrated and put into perspective with the scientific data currently available on essential oils.
The purpose of this study is to develop and evaluate the usefulness of hyperpolarized (HP) 129Xe gas MRI for regional assessment of pulmonary function.
Background: Lumacaftor/Ivacaftor (LUM-IVA), a CFTR corrector-potentiator combination, was found to improve lung function and reduce pulmonary exacerbations (PEx). However, cystic fibrosis (CF) is a multi-organ disease and therefore there is a need for more information on the systemic effects of CFTR modulators. Aim: To evaluate pancreatic function, bone metabolism and respiratory changes through a year of LUM-IVA treatment. Methods: A prospective real world, one-year study on F508del homozygous adult CF patients who commenced treatment with LUM-IVA. Visits were scheduled on the first day of treatment and every 3 months evaluating: symptoms, Body Mass Index (BMI), spirometry, laboratory tests and Quality of life. At baseline and at 12 months, the patients underwent sweat test, oral glucose tolerance test (OGTT), chest CT and dual-energy X-ray absorptiometry (DEXA).
Cystic fibrosis (CF) affects men and women equally, but after the onset of puberty, women with CF have a lower life expectancy than men with CF. Despite these known differences, the link between CF symptom trends and the menstrual cycle remains critically understudied. To address this gap, this study will investigate changes in CF-specific symptoms among women with CF to evaluate whether and how they correlate with their menstrual cycle. Specifically, the investigators hope to examine whether CF-related symptoms change throughout the menstrual cycle, what the impact of those symptoms is on quality of life, and how feasible it is to use a period tracking app to track CF-related symptoms throughout the menstrual cycle. Investigators are asking women ages 18-45 with CF, who have regular menstrual cycles, to participate. Study procedures, including online surveys, period tracking, and interview, will take approximately 3 months.
An observational study of patients with cystic fibrosis (CF) starting treatment with Kaftrio (Elexacaftor / Tezacaftor / Ivacaftor) as part of routine clinical care, following EMA licensing (approved end of Aug 2020). - Patients with CF who are p.Phe508del homozygotes will already be receiving the less effective CFTR modulator drug Symkevi (Tezacaftor / Ivacaftor) and will switch to KaftrioTM. - Patients who are who are compound heterozygotes with at least 1 copy of p.Phe508del currently have access to no effective CFTR modulator and will be starting a CFTR modulator (Kaftrio) for the first time. Participants attend a study visit before Kaftrio treatment commences, followed by visits at 12 and 24 weeks after starting treatment. At each visit they will be scanned before and after standardised meals in the morning and mid-day (11 scans in total over 6 hours). No intravenous contrast or bowel preparation will be used. Participants will complete questionnaires on gastrointestinal symptoms as well as providing stool and sputum samples for assessment of microbiome and stool for inflammatory mediators and pancreatic function (elastase). **Following an extension, participants had a further visit at 76 weeks post starting Kaftrio, updated in detailed description**
This is a prospective, multi-center observational study. The study is designed to measure the clinical effectiveness of elexacaftor, tezacaftor and ivacaftor (ETI) triple combination therapy in children (6-11 years of old) with one or more copies of the F508del mutation, study the effects of ETI across a number of CF disease manifestations, and collect specimens for future research. Subjects in the study will have one "before ETI" visit within 30 days before initiation of the therapy and five "after ETI" visits over a 24-month follow-up period. Participants who have participated in the original PROMISE Pediatric Sub-Study have the option of participating in a long-term extension with annual visits performed at the 36- and 48-month timepoints. The durability of the clinical and biological changes in the PROMISE Pediatric Sub-Study can be assessed with extended follow-up, which would enable the sub-studies to consider potential clinical consequences of the biological or physiological effects being studied. This work will help to inform long term prognosis and feasibility of certain clinical trials outcomes for interventional studies and may be useful when considering research priorities in drug development. The duration of participation for each subject is 24 months (with an additional 24 months if participants agree to the optional long-term extension). NOTE: FDA has granted approval for elexacaftor, tezacaftor and ivacaftor in the 6-11 age group.
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.