
Sit-to-stand Test in Cystic Fibrosis Children and Adolescents
Cystic FibrosisCystic fibrosis (CF) is a chronic hereditary respiratory disease. Exercise testing is part of CF patients regular assessment. Cardio-Pulmonary Exercise Testing (CPET) is currently considered as the gold standard to assess physical capacities. However, simple field tests are emerging. These tests are easier to perform especially in a population of CF children and adolescents. The 1minute Sit-To-Stand test have recently been evaluated in CF adults. This test correlates with maximal oxygen consumption during CPET. The investigators hypothesized that this test also correlates with 6-Minute Walking distance (during a 6-min Walk Test), quadriceps strength, respiratory muscles strength and health-related quality of life.

Characterization of Myocardial Interstitial Fibrosis and Cardiomyocyte Hypertrophy by Cardiac MRI...
Heart FailureThe investigators hypothesised that novel MRI metrics derived from myocardium post-gadolinium T1 mapping analysis will improve the current knowledge about the role interstitial fibrosis and cardiomyocyte hypertrophy in the development of left ventricular (LV) remodelling and clinical Heart Failure (HF). The investigators believe that these recently described variables will be associated with prognostically important indices in HF development.

Idiopathic Pulmonary Fibrosis (IPF) and the 3 Minutes Sit-to-stand Test
Idiopathic Pulmonary FibrosisThis study is designed to evaluate the reproductibility and the performance of the 3 minutes sit-to-stand test in patients with idiopathic pulmonary fibrosis. To do this, the investigators are recruiting 40 patients with idiopathic pulmonary fibrosis in 2 centers (Grenoble university hospital and Lyon university hospital). Patients had to achieve an effort test on a cycle ergometer. 2 visits are planned in the hospital. During each visit, patients will perform a 3 minutes sit-to-stand test, a 1 minute sit-to-stand test and a 6 minutes walk test. During the second visit, patients will also perform a 3 minutes sit-to-stand test with measurement of oxygen uptake. The investigators will then analyse the results by comparing numbers of cycle, functional response and symptoms during the 3 minutes sit-to-stand test of the 2 visits. The investigators will also compare the functional response obtained during the 3 minutes sit-to-stand test, the 1 minute sit-to-stand test and the 6 minutes walk test. Finally, the investigators will compare the maximal values of oxygen uptake, respiration rate and expired volume obtained during the 3 minutes sit-to-stand test to the effort test on cycle ergometer.

Clinical Trial to Assess Influence of MyCyFAPP Use on GI Related QOL in Children With Cystic Fibrosis...
Cystic Fibrosis in ChildrenInterventional trial to study the influence of the use of MyCyFAPP (mobile application) on the gastro-intestinal related quality of life. This mobile APP has been developed during previous workpackages of the Horizon2020 Project and contains several modules: mathematical prediction model to calculate the needed dose for pancreatic enzyme replacement therapy educational games and other educational material communication with doctor/dietician through professional webtool diary to register symptoms and data on nutrition. The app will be introduced and used during 6 months. Primary outcome parameter will be change in modified PedsQL GI after 3 months. PedsQL GI is an existing questionnaire that evaluates gastro-intestinal related quality of life in children. We validated it for use in cystic fibrosis in a previous observational study.

Validation of Fibrosis Quantification Using T1 Mapping Against Histology as Reference and Comparison...
Myocardial FibrosisMyocardial fibrosis is recognized as the pathologic entity of extracellular matrix remodeling. Diffuse, reactive fibrosis is increasingly recognized in a variety of conditions despite the absence of ischemia. Regardless of the etiology, fibrosis leads to increased myocardial stiffness thereby promoting cardiac dysfunction. This dysfunction may present clinically with symptoms of cardiac failure although this is often a subclinical disease. Various imaging modalities and collagen biomarkers have been used as surrogate markers to assess the presence, extent, and turnover of myocardial fibrosis. Techniques using echocardiography, cardiac magnetic resonance, and nuclear imaging have been developed to detect early features of systolic and diastolic left ventricular dysfunction and impaired contractile reserve. Further identification of diffuse reactive fibrosis may be possible with evolving cardiac magnetic resonance and molecular techniques. The goal of this protocol is to validate cardiac magnetic resonance imaging as a new tool for fibrosis quantification against histology as standard of reference.

Fecal Microbiota Transplantation in Cirrhosis
CirrhosisLiverPatients with cirrhosis were recruitted and divided into cintrol group and FMT group. Patients in FMT group were carried by fecal microbiota transplantation, and biochemical indexes, intestinal flora and intestinal HIF expression were observed before and after FMT respectively.

Study of Obeticholic Acid(OCA) Combination With Ursodeoxycholic Acid (UDCA) in Patients With Primay...
Primary Biliary CirrhosisObecholic acid is a modified bile acid and Farnesoid X receptor (FXR) agonist. FXR is a key regulator of bile acid synthesis and transport. Bile acids are used by the body to help with digestion. Conventional therapy with obecholic acid will improve liver function of patients with PBC.

Various Insulin Regimens for Diabetic Inpatients With Cirrhosis Trial
Type 2 DiabetesLiver CirrhosisLiver disease is an important cause of death in type 2 diabetes. In the population-based Verona Diabetes Study cirrhosis was the fourth leading cause of death and accounted for 4.4% of diabetes-related deaths. In another prospective cohort study , cirrhosis accounted for 12.5% of deaths in patients with diabetes. In Egypt hepatitis C virus the commonest of cirrhosis here has a prevalence of 9.8% in the population with the greatest burden over national health care bills. Patients with cirrhosis & type 2 diabetes mellitus are always showing up in all hospital wards without a clear consensus of best management of their hyperglycemia.

Use of Methacetin Breath Test to Predict Liver Failure in Patients With Cirrhosis
CirrhosisThe methacetin breath test (MBT) is a non-invasive liver function test which measures the ability of the liver to metabolize a tracer dose of a compound to carbon dioxide, which is exhaled. The study hypothesis is that measurement of the MBT will allow earlier detection of a decline in liver function in patients with cirrhosis who are awaiting liver transplantation.

Evaluation of Probiotics in the Treatment of Portal Hypertension
Liver CirrhosisPortal Hypertension1 moreThis study is to evaluate the role of probiotics in the treatment of portal hypertension. In particular the role of probiotics on gut microbiota in liver cirrhosis patients will be studied and compared with cytokines and other substances implicated in the pathogenesis of portal hypertension. The hypothesis whether probiotics may change the prognosis of patients with portal hypertension will be studied. The hypothesis whether probiotics may halt the pathologic cascade of events leading to various complications (e.g. hepato-renal syndrome, spontaneous bacterial peritonitis, bleeding varices) will be reviewed.