View clinical trials related to Fibrosis.
Filter by:Cystic fibrosis is the most common severe genetic disease with autosomal recessive transmission in the Caucasian population. Its prognosis has improved considerably since the creation of Cystic Fibrosis centers (CF centers) and the improvement of symptomatic management (nutrition, antibiotic therapy, transplantation, etc.). Thus, the median survival rate is now 46 years, whereas it was 5 years in 1963. The current challenges for cystic fibrosis patients are therefore twofold: to continue to improve their survival, and to improve their quality of life (QoL) to promote "healthy ageing" with this pathology that begins in childhood. In 1980, the World Health Organization (WHO) stated that functional capacity explorations best reflected the impact of chronic disease on health-related quality of life. Impairment of physical activity is common to chronic diseases, as in cystic fibrosis, where respiratory impairment and denutrition have been shown to contribute to reduced exercise tolerance and increased dyspnoea. Measurement of the maximum oxygen consumption (VO2max) by a cardiopulmonary exercise test (CPET) is regarded as the gold standard exercise test in the measurement of aerobic exercise capacity. In 2005 Pianosi et al. found that for children with cystic fibrosis, the rate of decline of VO2max measured by CPET was predictive of poorer quality of life. Continuing to study the determinants associated with impaired aerobic fitness in cystic fibrosis offers the hope of considering appropriate therapies to further improve the quality of life of these patients. In recent years, the arrival and widespread use of CFTR protein modulators in children has been a real turning point and makes it possible to envisage a drastic change in the history of this disease and its prognosis in the long term. Thus, in this study, the investigators aimed to assess the aerobic fitness, assessed by a CPET, of children with cystic fibrosis, and to compare the results with healthy controls. Secondly, investigators wanted to identify the predictive factors of VO2max in children with cystic fibrosis.
The primary purpose of this study is to evaluate the safety and tolerability of three active doses of nebulized amikacin in a SLIT™ formulation.
The goal of this clinical trial is to learn about the safety, tolerability and pharmacokinetic profiles of SC1011,in health conditions. The main questions it aims to answer are: Safety and tolerability profiles in healthy subjects.Pharmacokinetic profiles in healthy subjects.Food effect in healthy subjects.Participants will complete the study including screening period, dosing period, and observation period. Researchers will compare the inhibitory activity of SC1011 tablets with pirfenidone capsules against the same biomarkers(e.g. blood TNFα) to see if they are different between the two drugs.
The goal of this clinical trial is to learn about the safety, tolerability and pharmacokinetic profiles of SC1011 in healthy conditions. The main questions it aims to answer are: Safety and tolerability profiles in healthy subjects and pharmacokinetic profiles in healthy subjects. Participants will complete the study including screening period, dosing period, and observation period. Investigators will compare the inhibitory activity of SC1011 tablets with pirfenidone capsules against the same biomarkers(e.g. blood TNFα) to see if they are different between the two drugs.
Cystic fibrosis (CF) is one of the most common inherited conditions in the United Kingdom (UK). There are 10,810 people living with CF in the UK, with median predicted survival now 47 years old. People with CF have multiple medical treatments to do on a daily basis, and the treatment burden is increasing. Adherence to treatment plays an important role in health outcomes and survival in CF. Online access to their own health care records gives people an increased control over their own health, greater understanding of their conditions and has a potential to improve adherence to care plans and medications. Whilst implementation of electronic records is established in primary care, there has been a much poorer roll out of electronic care records in the secondary care system. Leeds Teaching Hospitals CF Unit is a regional centre with around 650 adult and paediatric registered patients. Handwritten and typed paper records of patients under the care of the CF unit in Leeds were replaced in 2007 by electronic healthcare records (EHR; EMIS®). Patients view and obtain graphical feedback at each clinic visit including trends in parameters such as lung function, weight and inflammatory markers. In partnership with Egton Medical Information Systems (EMIS) web (EMIS®), a modification allowing secondary care access to patient records has been developed. In a structured programme of research, the Leeds Adult CF Unit have firstly evaluated the implementation of the EHR in secondary care in terms of service delivery and cost improvement. In the second phase, the investigators sought patient feedback regarding which aspects of their EHR people with CF wish to access, and their priorities for development. This has informed the third phase in which the aim is to explore the impact of patient access to their EHR. The aims of the trial are 1. To evaluate the feasibility, benefits and acceptability to patients and health care professionals of providing secure access of linked secondary care and patient's Personal Health Records in CF, and 2. To explore technological usability, future functionality and the impact of the shared records on clinical resources, communication and patient and health care professional satisfaction.
The present study evaluates the effect of nursing care based on the Self-Care Deficit Nursing Theory on the knowledge and skills related to self-care among adolescents with Cystic Fibrosis.
The goal of this clinical trial is to to assess the nutritional status of children diagnosed with cystic fibrosis between the ages of 2-14, to determine the changes in the nutrition education given to the mothers of the patients on the nutritional status of the children and the nutritional knowledge of the mothers, and to compare them with the control group.. The main questions it aims to answer are: 1. Will be better the nutritional status of children of mothers in the education group will be better compared to the control group? 2. Will the macro and micronutrient Recommended Dietary Allowance (RDA) and diet quality of children with cystic fibrosis in education group increase after nutrition education? 3. Will the nutrition knowledge test scores of mothers of children with cystic fibrosis in education group on "Nutrition in Cystic Fibrosis" increase after nutrition education? Mothers of children diagnosed with cystic fibrosis will participate in the study as a result of the call of the Cystic Fibrosis Association in Turkey. Mothers participating in the study will be divided into 2 groups. While the mothers in the first group will be given nutrition education 3 times a week, 1 hour a day, no education session will be given to the mothers in the control group. The nutritional status and nutrient consumption of children in the training group and the control group will be compared.
The aim of the study is to describe the evolution of caloric intake in patients with cystic fibrosis with an indication to start treatment with Elexacaftor/Tezacaftor/Ivacaftor according to the Marketing Authorization, between the start of treatment and at 12 months.
The goal of this observational study is to assess the main clinical and anamnestic characteristics, and frailty syndrome in an adult Cystic Fibrosis population. The main question it aims to answer is the possible association of the frailty status with the main clinical, therapeutical characteristics, including the genotyping classification of Cystic Fibrosis patients.
Based on preclinical data, investigators hypothesize that apoptosis resistance in monocyte-derived macrophages (MDMs) have a decisive role in the development of idiopathic pulmonary fibrosis (IPF). Specifically, macrophages from subjects with IPF have increased expression of Bcl-2 in mitochondria. In preclinical models of IPF, a conditional deletion of Bcl-2 in MDMs reverses established fibrosis by inducing apoptosis. Additional evidence to suggest that Bcl-2 expression in MDM mitochondria is a therapeutic target for IPF as administration of the Bcl-2 inhibitor, ABT-199 (Venetoclax), showed marked efficacy in preclinical models of IPF by inducing apoptosis of MDMs and reversing established fibrosis. ABT-199 is an orally available mimetic of the BH3 domain of Bcl-2, which is the domain the anchors Bcl-2 in the mitochondria to inhibit apoptosis. ABT-199 has shown therapeutic efficacy and good safety and tolerability in patients with chronic lymphocytic leukemia. Investigators anticipate that treatment with ABT-199 could result in significant benefit for IPF patients that have a life expectancy of 3-5 years. As there is no curative therapy for IPF, this clinical trial has the potential to substantially alter treatment approaches in patients with IPF.