The Effects of Pulmonary Rehabilitation in Patients With Non-cystic Fibrosis Bronchiectasis
Non-cystic Fibrosis BronchiectasisNumber of Patients: Study group - Bronchiectasis that is not attributable to Cystic fibrosis Group 1 - Pulmonary Rehabilitation Group 2 - Standard care Sample size - 20 in each arm Study Design: Randomised controlled trial (RCT) All patients who qualify for the study will undergo a detailed evaluation. Baseline assessment will include the following parameters: Anthropometry Pulmonary Function Tests and Respiratory muscle strength Cardiopulmonary Exercise Testing (CPET) Six Minute Walk Test (6MWT) Severity of dyspnoea (Dyspnoea scale) Limb muscle strength Inflammatory markers in the serum - C-reactive protein Quality of Life Patients will then be randomized (using table of random numbers) to either the pulmonary rehabilitation group or the standard arm group. After 8 weeks of pulmonary rehabilitation, patients will again be reassessed by the aforementioned tools.
ObeSity Related Colorectal Adenoma Risk
Colorectal AdenomaColorectal Neoplasm5 moreIn the UK, around 1 in 16 men and 1 in 20 women will develop bowel cancer at some point in their lives. Most bowel cancers happen when a type of growth in the bowel called an adenoma eventually becomes cancerous. Cutting out adenomas reduces the risk of developing bowel cancer. Certain people are more likely to have adenomas than others, for example people who are overweight. People who are overweight are also more likely to develop liver disease by laying too much fat down in the liver. Studies in Asia have shown that people with fatty liver disease are more likely to have adenomas and these are more commonly found in the part of the bowel (right colon) furthest from the bottom end. Information on the link between obesity, fatty liver disease and adenomas is very limited, particularly in the Western population. The investigators will assess the link between body weight, fatty liver and adenomas in the UK population. 1430 patients will be invited; some through the bowel cancer screening programme and some with symptoms such as low blood count, bleeding or changed bowel habit. These patients will already have been referred for a camera test looking into the bowel, called a colonoscopy. Information including height, weight and some health questions will be taken. Blood samples will be taken. The investigators will compare the number of patients with adenomas who have liver disease or who are overweight with those who don't. This information will be used to develop a scoring system to predict risk of adenomas. This will help the investigators to decide if undertaking colonoscopies in these patients will identify those at increased risk of bowel cancer.
Primary Nasal Cell Culture as a Tool for Personalized Therapy in Cystic Fibrosis
Cystic Fibrosischaracterization of CFTR function and expression in nasal primary cells collected from patients with cystic fibrosis in comparison to their parents, healthy heterozygotes and healthy controls
Bacterial Infections in Patients With Cirrhosis in Argentina: Clinical and Microbiological Characteristics...
Bacterial InfectionsCirrhosis2 moreIn recent years, there has been an increasing prevalence of bacterial infections caused by multiresistant and extremely resistant organisms in patients with cirrhosis. These infections are associated with a worse prognosis, generate difficulties in the management of the patient during hospitalization and increase health costs. The main objective of this project is to estimate the prevalence of infections by multiresistant bacteria in patients with cirrhosis. Additionally, the prevalence of other antibiotic resistance patterns and morbi-mortality in the study population will be evaluated. For these purposes, a multicenter prospective cohort study will be carried out, including patients with cirrhosis who present bacterial infections at the time of admission, or during hospitalization. Performing a study in Argentina on the clinical and microbiological characteristics of bacterial infections in patients with cirrhosis could be very useful to develop new strategies for prevention and treatment of this severe complication.
Portal Hypertension and Systemic Endothelial Function
Cystic FibrosisPortal HypertensionCystic fibrosis can affect organs other than the lungs. Liver disease affects about 30% of patients: its main manifestation is the development of portal hypertension (PHT). The pathophysiology of this comorbidity is still poorly understood. It was previously considered secondary to the formation of biliary cirrhosis but another hypothesis would be that of a primitive pathology of venous vessels may cause the gradual emergence of portal hypertension without cirrhosis. Evidence indiscutly suggest that cystic fibrosis is associated with a specific endothelial dysfunction, especially as the CFTR (Cystic Fibrosis Transmembrane conductance Regulator) protein is expressed on the surface of endothelial cells. The investigators hypothesize that liver disease related to PHT-associated cystic fibrosis is associated with systemic endothelial dysfunction. The aim is: To demonstrate a systemic endothelial dysfunction in patients with cystic fibrosis when associated with PHT. To study the correlations between measures of systemic endothelial function and serum markers of endothelial dysfunction and between measures of liver stiffness and systemic endothelial function.
Transfer Factor Efficacy in the Management of Cirrhosis-associated Immune Dysfunction
CirrhosisLiver FailureThis study is aimed to assess the efficacy of Human derived Transfer factor ( T-lymphocytes homogenate that contains small molecular weight (10 kDa) molecules: various IFNs, ILs, chemokines, endorfins, heat shock proteins) in decreasing rate and/or severity of infections in acute or chronic decompensations of liver cirrhosis and acute on chronic liver failure..
Cirrhosis Medical Home
CirrhosisLiverTo address the health care system's lack of care coordination, the Institute of Medicine and Centers for Medicare and Medicaid Services recommend the development of collaborative care models (CCM) in a wide range of clinical settings. CCMs are intended to provide coordinated, personalized care pragmatically using care coordinators. CCMs have successfully improved care in multiple patient populations, ranging from frail older adults to depression. In contrast, for patients with cirrhosis, there is a paucity of data to support the benefit of CCM in this medically complex and vulnerable population. At Indiana University, researchers have over 20 years of experience in developing, testing, and implementing CCMs successfully for patients living with dementia or depression. Building on these successes, we have customized the CCM to best meet the unique and complex biopsychosocial needs of patients with cirrhosis: the Cirrhosis Medical Home.
Real Life Evaluation of the Multi-organ Effects of Lumacaftor/Ivacaftor on F508del Homozygous Cystic...
Cystic FibrosisBackground: 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).
Avatrombopaq In Patients With Cirrhosis
Thrombocytopenia; DrugsCirrhosis2 moreThrombocytopenia is a frequent issue in patients with cirrhosis undergoing various types of procedures (e.g. liver biopsy, endoscopy and minor surgical interventions). Thrombocytopenia < 50*10^9/L increases the risk of perioperative and postoperative bleedning and might prevent patients with cirrhosis to undergo important procedures. Doptelet is a small molecular trombopoetin agonist, which results in proliferation and differentiation of megakaryocytes in the bone marrow resulting in increased levels of thrombocytes. It is given orally as a pill and is used to increase platelet count in patients with severe thrombocytopenia (< 50*10^9/L) and cirrhosis and thus not to normalize platelet count. This study investigates the safety and efficacy of Doptelet in patients with cirrhosis and thrombocytopenia (< 50*10^9/L) undergoing minor procedures like Transjugular Adjusted Liver Biopsy (TJALB) and gastroscopy.
Feasibility of a Goal-based Agenda Setting Intervention
Cystic FibrosisChronic DiseaseThis study aims to assess the feasibility of a new, brief intervention, the 'coopeRATE Prompt', for informing conversations between patients and physicians in routine tele-health and in-person adult Cystic Fibrosis (CF) care. The coopeRATE Prompt is two questions designed to elicit patients' concerns and goals to facilitate collaborative goal setting within the health care visit. This is a prospective single arm study that will be conducted at four CF care centers in the United States.