Expanded Access Program (EAP): Allow Patients in the US With Idiopathic Pulmonary Fibrosis Access...
Idiopathic Pulmonary FibrosisThis is an open label multi-center program to allow patients in the US with IPF access to treatment with pirfenidone.
Annual MRI Versus Biannual US for Surveillance of Hepatocellular Carcinoma in Liver Cirrhosis
Liver CirrhosisThe investigators will investigate the usefulness of biannual ultrasonography versus annual non-contrast magnetic resonance imaging for surveillance of hepatocellular carcinoma in single arm patients.
The International Imaging Registry in Liver Cirrhosis (BAVENO-VI SPSS GROUP).
Liver CirrhosisPortal Hypertension1 moreThe purpose of this study is to perform a multicentre registry of cirrhotic patients who had been submitted to an imagining technique in recent years (angio-CT scan or abdominal MRI), in order to collect anatomical and clinical information. The main objective will be focused on the study of portosystemic shunts and their relation with portal hypertension. Patient with liver cirrhosis submitted to an abdominal angio-CT scan or a MRI from year 2010 to 2014 will be included in the study. The chosen imaging technique will be angio-CT preferably, but MRI data will also be available. Patients will be identified in every hospital by means of the registry of coded diagnoses and the lists of complementary tests performed. Clinical and radiological data of every patient will be collected. The clinical variables will be obtained from reviewing the patient clinical history. The radiological parameters will be gathered by means of the systematic review of the angio-CT or MRI.
Feeding Behavior and Taste Sensitivity Before and After Liver or Renal Transplantation
CirrhosisFood Preferences3 moreThis study evaluates the influence of the taste sensitivity, of food preferences and of the reward system on the energetic balance before and after liver transplantation in cirrhotic patients and after kidney transplantation in renal failure patients
Cross Transmissions of Pseudomonas Aeruginosa Between Children From a Same Cystic Fibrosis Center....
Cystic FibrosisPseudomonas Aeruginosa1 moreCystic fibrosis is the most common hereditary autosomal recessive disease in the Caucasian population. The diseases is caused by a mutation of the gene coding for the CFTR protein (Cystic fibrosis transmembrane conductance regulator), an ion channel present at the apical pole of the epithelial cells. The channel dysfunction induces a deficit in hydration and a hyperviscosity of different exocrine secretions. Clinically, Cystic fibrosis is a multi-systemic disease. Pulmonary and pancreatic involvement are classically in the foreground. Degradation of respiratory function, associated with acute and chronic infections, represents the major cause of morbidity and mortality. Pseudomonas aeruginosa is a ubiquitous gram-negative bacillus found primarily in stagnant water. Pseudomonas aeruginosa is capable of colonizing the digestive, pulmonary and urinary mucosa and the skin. This bacterium is incriminated in many opportunistic infections including respiratory infections in patients with cystic fibrosis. Pseudomonas aeruginosa infection is the most common parenchymal lung infection in the Cystic fibrosis community. Pseudomonas aeruginosa chronic carriage represents a factor of poor prognosis associated with an increase in morbidity and mortality. Complications related to chronic carriage of Pseudomonas aeruginosa justify the implementation of strategies of eviction, screening and eradication of acute Pseudomonas aeruginosa infection. In addition to Pseudomonas aeruginosa contamination of patients via the environment, hand and airborne infections between patients with Cystic fibrosis have been reported. Measures to eliminate cross-transmissions have therefore been implemented in a majority of hospitals. The aim of the study is firstly to identify the number of Pseudomonas aeruginosa cross-transmissions between patients with Cystic fibrosis followed-up in Cystic fibrosis center of HUDERF. Investigator will use the Pulsed-Field Gel Electrophoresis to assess the possibility of cross-infection. Depending on the results, Investigator will implement new strategies to avoid future cross-contamination in our different places of care (consultation, hospitalization, physiotherapy…).
Using Cardiovascular Magnetic Resonance Tissue Characterisation and Wearable Technology to PREDICT...
Heart FailureDiffuse Fibrosis2 more150 patients admitted to University Hospital Southampton with heart failure will undergo comprehensive Cardiovascular Magnetic Resonance (CMR) imagining during their admission and continuous heart rhythm monitoring using wearable technology post discharge. We hypothesise that analysis of this outcome data will discover novel CMR tissue characterisation and heart rhythm biomarkers that can be used to predict adverse clinical outcomes in heart failure populations and how individual patients will respond to specific therapies.
Markers of Pulmonary Dysbiosis Associated With Exacerbation in Patients Followed for Cystic Fibrosis...
Cystic Fibrosis Pulmonary ExacerbationThe aim objective is to identify markers of bacterial, viral and fungal pulmonary dysbiosis, associated with the occurrence of exacerbation in patients followed for cystic fibrosis. The primary endpoint is the association between a modification of at least 10% of the relative abundance of a bacterial phylum (Proteobacteria, Firmicutes, Actinobacteria, Bacteroidetes, Fusobacteria) or fungal (ascomycetes / hemiascomycetes, basidiomycetes, zygomycetes), or viral, and the occurrence of exacerbations over a period of 12 months.
Functional MRI to Determine Severity of Cirrhosis
Liver CirrhosisLiver FibrosisThis is a pilot study to investigate the feasibility of a novel MRI technique to assess the severity of liver cirrhosis and predict complications based on functionality and perfusion measurements whilst maintaining image quality. The principal objective of this pilot study is to assess liver function and the future risk of complications in patients with cirrhosis, using novel techniques and measures based on free-breathing Dynamic Contrast Enhanced MRI. Specifically the investigators will assess: Whether sufficient data can be generated in patients with cirrhosis whilst maintaining image quality, and The dynamic range of DCE-MRI measures in patients with cirrhosis. This pilot study will, if successful, provide sufficient data to support applications for larger studies to evaluate the clinical utility of a DCE-MRI imaging biomarker in patients with cirrhosis.
Study for Evaluating the Real Use of Inhaled Aztreonam Lysine in Patients With Cystic Fibrosis
Cystic FibrosisThe aim of this observational trial is to evaluate the pulmonary function in cystic fibrosis patients that have been treated with inhaled aztreonam lysine comparing the previous 12 months before the treatment and the forward 12 months after initiating the treatment.
Reaction Time and Postural Control in Individuals With Cystic Fibrosis and Bronchiectasis
Cystic FibrosisBronchiectasisThe aim of this study is to evaluate the reaction time and postural control and to investigate the relationship between reaction time, exercise capacity, muscle oxygenation and balance in children with cystic fibrosis (CF) and non-CF bronchiectasis. 40 patients including 20 CF patients and 20 non-CF bronchiectasis and 20 healthy individuals will be included in this study. Demographic and physical characteristics' will be recorded. Lung function testing will be performed. Balance will be assessed using functional reach test, exercise capacity was measured using the incremental shuttle walking test and reaction time will be assessed using ''Fitlight TrainerTM''. Heart rate, respiratory rate, oxygen saturation, muscle oxygenation, dyspnea and fatigue perception will be measured before and after exercise test and reaction time measurement.