Active clinical trials for "Fibrosis"

The study will be conducted over a 6 months period. For the first three months, the child will be accompagned with a connected companion. After this period, the companion will be removed for three months in oder to prove this companion could improve treatment adherence for children suffering from cytolisis fibrosis. This study will be conducted at the University Hospital Center of Rennes and Hospital Center of Saint-Brieuc.

Patients with end stage liver disease have varying degrees of intra-abdominal hypertension (IAH) due to the presence of ascites. The perioperative events may either relieve or aggravate the intra-abdominal pressures. Intra-abdominal hypertension has damaging effect on various organ systems. There is an increase in intracranial pressures and a decrease in cerebral perfusion pressures associated with IAH . In the heart, there is an increase of right atrial pressures, increase in systemic vascular resistance and decrease in cardiac output . Pulmonary complications include increase in the peak, mean and plateau airway pressures, with decreased compliance . Renal dysfunction is an early effect of raised intra-abdominal pressure, resulting from decreased renal blood flow, shunting of blood to the medulla, mechanical compression of the kidneys and increased pressures in the renal veins . We would study the intra-abdominal pressures in liver transplant recipients and record hemodynamic, respiratory, cardiac and renal function prospectively. Follow up data for 6 days for neurological, respiratory, cardiac and renal complications will be collected, along with hospital stay, ICU stay and mortality. The association between intra-abdominal pressures and these outcomes will be analysed.

Introduction: Bronchiectasis is a chronic lung disease in which the underlying condition causes permanent damage to the conducting airways. Bronchiectasis is associated with considerable morbidity and poor quality of life. While cystic fibrosis (CF) is the most common cause of bronchiectasis in childhood, non-CF bronchiectasis is associated with a wide variety of disorders. CF bronchiectasis patients show reduced daily habitual physical activity and exercise capacity. Cardiopulmonary exercise test (CPET) is increasingly gaining importance in clinical medicine and considered the gold standard exercise test for assessing aerobic exercise capacity. The test objectively evaluates exercise physiological functions, may help assess morbidity and predict the outcome and mortality in different clinical circumstances and may serve as a basis for individualized exercise prescription within the limitation of the disease. Unlike CPET in CF, there is a paucity of data on exercise capacity using CPET in non- CF bronchiectasis patients, and on the implications of physical activity on non- CF bronchiectasis morbidity and mortality. Aim: To evaluate and compare exercise capacity in CF and non-CF bronchiectasis patients. Methods: This will be a cross-sectional retrospective/prospective study population. The retrospective study will include data analysis of patients that preformed CPET as part of their clinical evaluation. In the prospective study, patients that are scheduled to perform CPET as part of their clinical evaluation will sign (or legal guardian) informed consent prior to participation. Patients will be recruited from the exercise clinic at the Pediatric Pulmonary Institute at the Rappaport Children's Hospital. Inclusion criteria: 1. Children and adults (age >7 years, height >125cm), with CF and non CF bronchiectasis. 2. Completed a maximal CPET test according to accepted criteria; (maximal VO2 > 80% predicted, maximal heart rate > 80% predicted, acceptable RER (RER > 1.0 in children (under 18 years), RER > 1.05 for adults) or reaching a VO2 plateau..3. Evidence of bronchiectasis in computed tomography (CT). Exclusions criteria: preforming submaximal CPET, lack of data from the exercise test, exacerbation of patient's condition within three days before the exercise evaluation, relevant related chronic diseases that affecting test results.

As there are no validated tools for assessing patient reported outcomes or health related quality of life in idiopathic pulmonary fibrosis (IPF), different studies have utilised different methods. This means that comparison of the outcomes of studies is difficult or inaccurate. By collecting different quality of life tools and patient reported outcome at the same time, it will be possible to map or model the results of one tool or groups of tools onto another. 250 patients with IPF will be asked to complete the EuroQoL 5D, Kings Brief Interstitial Lung Disease questionnaire, St George's Respiratory Questionnaire, MRC dyspnoea scale, University of California, San Diego shortness of breath questionnaire and the Hospital Anxiety and Depression Scale, along with spirometry every 3 months, and undergo a 6 minute walk test every 6 months, over a 12 month period. Prognostic models will be constructed from all the clinical (questionnaire and function) measures a linear regression model.

IL-33 is a recently identified number of the IL-1 family. Hepatic over-expression of IL-33 has been recently linked to liver fibrosis. ST-2 exerts pro-inflammatory effects of IL-33. We aimed to determine ability of ST2 to predict fibrosis in chronic hepatitis B.

Primary biliary cirrhosis (PBC) is a chronic liver disease primarily affecting middle age women. It is characterized by immune-mediated damage to cells lining the tiny bile ducts within the liver. Although the underlying cause of PBC is likely to be multifactorial, the epidemiologic/population data indicate a very important role for genetic predisposition, meaning that the disease seems to run in families. Susceptibility genes for PBC have not been identified possibly due to limitations such as small sample size in prior studies. The primary objective of this study is to identify these genes. This study involves obtaining clinical and demographic data as well as collecting DNA samples from patients and their parents, and siblings to screen for a select set of candidate genes as well as the full genome for variants associated with PBC.

Gastro-oesophageal reflux (GOR) has been found to be prevalent in children with cystic fibrosis (CF)and may further worsen lung damage via reflex bronchospasm or pulmonary aspiration. Chest physiotherapy may result in increased episodes of GOR as demonstrated in children. Lung transplantation may worsen pre-existing GOR. This study will determine the prevalence, severity and significance of symptomatic and silent GOR in adults with CF before and after lung transplant using 24hr oesophageal pH monitoring, a valid symptom questionnaire, quality of life questionnaires and gastric emptying studies. This study will identify the extent of GOR in a large adult CF population and the impact on lung function and quality of life together with the effects of medical and physiotherapy treatment on gastro-oesophageal function.

The researchers are investigating a novel technique, the multi breath nitrogen washout technique, to measure airway changes in various respiratory diseases.

This a prospective observational study in Chronic Liver Disease patients admitted or seen in OPD, Department of Hepatology, Institute of Liver and Biliary Sciences, India. The study will be conducted in a period of three months starting September 2016 in sample size of 80 . A detailed proforma including history and examination and routine blood investigations will be noted. The patients will undergo close follow up at 0, 7, 15, 30, 45, 60, 90 days and similar activities will be repeated at every visit.

The purpose of this study is to use the Medication Adherence Reasons Scale (MAR-Scale) to determine the extent of non-adherence to specific medications indicated to treat cystic fibrosis, hemophilia (A or B), idiopathic pulmonary fibrosis, myasthenia gravis, and sickle cell disease, and to identify the top patient-reported reasons for non-adherence. Internal reliability of the MAR-Scale will also be assessed in each condition.