
Prospective Surveillance of Lung Development During Childhood, Adolescence and Adulthood in Healthy...
Cystic FibrosisHealthyCystic fibrosis (CF) is the most common lethal inherited disease in Caucasian populations. To improve survival, it is essential to understand the development, progression and treatment of CF lung disease throughout early childhood. Therefore the overall objective is to prospectively assess the clinical utility of novel and non-invasive measuring methods, namely Multiple Breath Washout and functional lung MRI in the longitudinal clinical surveillance of patients with CF and compare the results to those of healthy controls.

Genetic Epidemiology of Primary Biliary Cirrhosis (PBC)
Primary Biliary CirrhosisPrimary Biliary Cirrhosis (PBC) is a progressive liver disorder of unknown cause. Current evidence suggests that genes, the genetic material we inherit from our parents, in combination with environmental factors, likely play an important role in the development of PBC. This study is being done to investigate whether genes make people more likely to develop PBC. Discovery of these proposed genes will help us better understand how PBC developes, and subsequently, to apply new approaches for its prevention, diagnosis and treatment.

Resection for Multiple Hepatocellular Carcinomas (HCC)
Hepatocellular CarcinomaMultiple Tumors1 moreSurgical resection in patients with multiple hepatocellular carcinomas (HCC) remains controversial. The aim of this study was to investigate the outcome of a consecutive cohort of patients resected for multiple HCC, without macrovascular invasion, in order to identify clinically reliable parameters to select patients for surgery.

The Use of Visual Feedback in Airway Clearance
Cystic FibrosisCystic Fibrosis (CF) patients perform airway clearance incorporating various breathing strategies, to clear secretions from their lungs. Hand held devices may aid mucus expectoration, and also motivate the patient to manage by themselves. Our aims was to study if resistive expiration through "volumetric incentive spirometer" (VISex) can improve lung function in the short term in Cystic Fibrosis (CF) patients.

Spironolactone in Adult Congenital Heart Disease
Congenital Heart DiseaseHeart Failure1 moreThe purpose of this study is to see if the study drug called spironolactone reduces fibrous (stiffening) in heart muscle tissue and improves heart function. Subjects from the study titled "Heart Failure in Congenital Heart Disease: the role of myocardial fibrosis" who have evidence of heart dysfunction and/or evidence of fibrosis (stiffening) in the heart muscle will be asked to take part in this study.

Liver Transplantation Versus Alternative Therapies for Patients With Pugh B Alcoholic Cirrhosis...
CirrhosisLiver transplantation has been universally recognized to improve survival of patients suffering from end-stage (Pugh C) alcoholic cirrhosis. However, for Pugh B patients, the benefit of liver transplantation remains to be demonstrated. The aim of the present study was to compare the outcome of Pugh B patients with alcoholic cirrhosis randomly assigned for immediate liver transplantation (group 1) or standard treatments (group 2).

Pharmacokinetics of Inhaled Mannitol in Cystic Fibrosis Patients
Cystic FibrosisThe general objective of the study is to estimate the systemic pharmacokinetics of mannitol after single and multiple dosing of IDPM 400 mg to adult and paediatric cystic fibrosis patients.

Deposition of Inhaled Prolastin in Cystic Fibrosis Patients
Cystic FibrosisThe objective of this trial is to determine the optimal region of the lung for depositing Prolastin (alpha-1 antitrypsin; AAT) by inhalation in order to treat cystic fibrosis (CF). The AKITA® nebulizer has settings which can be varied to target the inhaled drug to either the deep lung or to the upper airways in a one to one randomization. The study will measure how much of the activity of the enzyme elastase is inhibited by AAT.

Diabetes Therapy to Improve BMI and Lung Function in CF
Cystic FibrosisDiabetes MellitusTo recruit 150 adult patients with cystic fibrosis related diabetes (CFRD) without fasting hyperglycemia for a multi-center, twelve month, placebo-controlled intervention trial testing the ability of insulin or repaglinide to improve body mass index (BMI) and stabilize pulmonary function in cystic fibrosis (CF).

Vacuum Myofascial Therapy Device in Treatment of Muscle Fibrosis
Muscle InjuryWe present the case of a 24-year-old professional soccer player, with no personal history of interest, who came to the X-Ray diagnosis service due to severe pain in the right quadriceps muscle after a 10-month evolution of muscle rupture occurred during sports practice.