
Ready to Use Therapeutic Food (RUTF) to Promote Growth in Cystic Fibrosis
Cystic FibrosisChildren with cystic fibrosis require increased caloric intake to maintain appropriate growth, an important determinant of long-term outcomes. This study seeks to determine the feasibility of using a novel therapeutic food to promote weight gain and growth in children with cystic fibrosis.

Digital Auscultation Test - IPF Data Collection
Idiopathic Pulmonary FibrosisThe aim of this study is the data collection for patients with IPF and symptom matched controls to create a database of lung auscultation sounds and basic patient characteristics.

Sofosbuvir, Daclatasvir, Ribavirin for Hepatitis C Virus (HCV) Cirrhotics
Hepatitis CCirrhosisThe investigators will treat 100 patients with cirrhosis due to hepatitis C with sofosbuvir 400 mg daily, daclatasvir 60 mg daily and weight-based ribavirin (1000 mg/d if <75 kg, 1200 mg/d if >75 kg, divided in two daily doses) for 12 weeks and calculate the sustained viral response rate at 12 weeks.

Early Use of TIPSS in Patients With Cirrhosis and Variceal Bleeding
Liver CirrhosisVariceal HaemorrhageGastro-oesophageal varices (swollen veins in the gullet and stomach) are present in 50% of patients with liver cirrhosis and are its most serious complication as their rupture results in potentially life threatening bleeding. Bleeding from these veins occurs in up to one third of patients with varices. This is associated with 20% mortality at 6 weeks. In the event of bleeding from these veins the current UK guidelines recommend certain drugs followed by early endoscopic treatment with variceal band ligation (rubber bands placed over the veins to stop them bleeding). The use of a shunt inside the liver ("TIPSS" transjugular intrahepatic portosystemic shunt) is largely reserved for cases of uncontrolled bleeding from these swollen veins. A recent randomised multicenter study carried out by Garcia Pagan and colleagues reported improved survival with early TIPSS in patients with bleeding from these swollen veins in advanced liver disease. From these guideline international guidelines now recommend consideration of early TIPSS for all high-risk patients presenting with variceal bleeding. This practice clearly has significant cost implications. To validate the findings a further randomised control trial is needed. This is a multi-center parallel-group randomized controlled trial. Both hospitals taking part in the trial will have a TIPSS service. Patients who consent to enter the trial will be randomized to either: (1) Endoscopic treatment (standard care) or (2) early TIPSS. Potential participants will be all patients with a diagnosis of liver cirrhosis presenting with an acute variceal bleed to a participating hospital who do not fulfill an exclusion criteria. All causes of cirrhosis will be included. Participants will be reviewed during their regular hepatology clinic appointments at their respective hospitals on 3 occasions over a one-year period.

Cystic Fibrosis Microbiome-determined Antibiotic Therapy Trial in Exacerbations: Results Stratified...
Cystic FibrosisAntimicrobial resistance is a significant challenge facing global healthcare. The unnecessary use of antibiotics is a key driver in the development of antibiotic resistance. Cystic Fibrosis (CF) represents a unique disease model to study bacterial resistance and to explore therapeutic strategies for same, as chronic lung infection overlaps with acute lung exacerbation's caused by a multitude of organisms. With time, chronic polymicrobial infection develops, with the most dominant infecting organism being Pseudomonas aeruginosa. In acute CF infections, empiric intravenous antibiotics are usually given for two weeks. Recurrent infections and treatments result in increasing antimicrobial resistance, and alterations in pathogen host interactions in the lung and gut flora. Next-generation DNA sequencing technology now offers DNA-based personalised diagnostics and treatment strategies. Enhancing our knowledge of the microbiome allows the use of stratified targeted antibacterial therapy that can be compared with standard empirical antibacterial therapy currently used. Cystic Fibrosis Microbiome-determined Antimicrobial Therapy Trial in Exacerbations: Results Stratified (CFMATTERS) will provide a randomized multi-centre controlled trial of microbiome-derived antimicrobial treatments versus current empirical therapy.

Validation of the Procedure of Early Liver Transplantation in Alcoholic Hepatitis Resisting to Medical...
Alcoholic HepatitisAlcoholic CirrhosisThe purpose of this study is to validate a strategy of identification of patients for early liver transplantation in severe alcoholic hepatitis. In this setting, short-term survival is very low (approx. 25% at 6 months) and a pilot study has suggested (mathurin et al. N Engl J Med 2011) that liver transplantation may be an option in very carefully selected patients who did not respond to medical treatment. This selection process deserves to be confirmed in a population of greater size. We hypothesized that patients selected with this process would have a same alcohol relapse rate after liver transplantation than patients transplanted for alcoholic cirrhosis and selected using a 6-month sobriety period

3 Airway Clearance Techniques in Non Cystic Fibrosis Bronchiectasis
BronchiectasisStudy design: a randomized, crossover trial. Each patient performed three different airway clearance techniques (Autogenic drainage, slow expiratory with glottis opened in lateral posture [ELTGOL], temporary- positive expiratory pressure [T-PEP] with not similar autonomy degree in a randomized order. Each technique were applied in 3 sessions during one week at alternate days (Monday /Wednesday/Friday or Tuesday/Thursday/Saturday). The time spent in each bronchial session was 40 minutes. Seven days were the wash-out time period between the different techniques.

AGRA Before and After Liver Transplantation
Liver CirrhosisThe immune system is impaired in liver cirrhotic patients, which is associated with a high risk for bacterial infections and worse outcome. A novel biomarker, acellular growth retardation ability (AGRA), can predict the development of severe infections in patients with liver cirrhosis and therefore identify patients at risk. It is still unclear, how this biomarker develops after liver transplantation and how valid its predictions are for post-operative infections. Therefore, AGRA will be measured before and after liver transplantation and predictive merit of AGRA for post-transplant infections will be tested.

CHaractErizing CFTR Modulated Changes in Sweat Chloride and Their Association With Clinical Outcomes...
Cystic FibrosisThis is a multicenter, cross-sectional, cohort study which will collect contemporary sweat chloride (SC) values from approximately 5000 Cystic Fibrosis (CF) patients prescribed and currently receiving commercially approved Cystic Fibrosis transmembrane conductance regulator (CFTR) modulator therapies.

Indirect Calorimeter Based Study in Patients With Liver Cirrhosis
Liver CirrhosesPart I- Observational study In the first part , investigator would investigate and compare the energy requirements and substrate utilization in healthy subjects and in patients with liver cirrhosis of various etiologies across the disease severity state viz compensated, stable decompensated, critically ill cirrhotics. The investigator would also enroll patients with chronic kidney disease, critically ill patients of acute liver failure or acute on chronic liver failure as disease controls. Part II- Randomized Controlled Study All the eligible, critically ill cirrhotic patients on mechanical ventilator support would be randomized to a control group (receiving the nutritional therapy as per the standard enteral nutritional practice in a critical care setting) or the intervention group (receiving enteral nutrition based on proposed measured requirements by Indirect calorimetry (IC) given till the patient is in ICU(Intensive care unit). The IC would be done thrice a week in both groups.