
Probiotics on Intestinal Inflammation in Cystic Fibrosis
Cystic FibrosisCystic fibrosis (CF) is a complex systemic disease that mainly involves the respiratory and gastrointestinal (GI) tracts. The polymicrobial community composition of respiratory and GI tracts is influenced by both genetic and environmental factors. Children with CF may harbor an abnormal intestinal microflora, because of altered Cystic fibrosis transmembrane conductance regulator (CFTR) function and heavy drug load (antibiotics, pancreatic enzymes and acid suppressors). The investigators previously demonstrated that intestinal inflammation is highly frequent in CF children, being a major feature of intestinal involvement. In addition, specific probiotics significantly improved airway and GI inflammation in a preliminary trial. The aim of the study is to characterize intestinal and respiratory microflora in CF patients and to investigate the effects of daily Lactobacillus GG (LGG) supplementation on both GI and airway microflora and the eventual relationship between probiotic assumption and clinical and inflammation markers. The aim is to study the effect of microflora modification on intestinal and extraintestinal inflammation to eventually improve the quality of life of CF patients, who often suffer from intestinal and respiratory progressive disease, through a non invasive intervention consisting in the supplementation of probiotic bacteria.

Non Invasive Assessment of Liver Fibrosis in Fatty Liver Disease
Non Alcoholic Fatty Liver DiseaseThe purpose of this randomized clinical trial is to evaluate the utility of noninvasive markers for the detection of advanced fibrosis in patients newly diagnosed with Non-alcoholic Fatty Liver Disease (NAFLD) by ultrasound. The primary objective is to determine the effectiveness of noninvasive markers for detect of advanced fibrosis in patients with diagnosis of fatty liver disease. The secondary objectives are: To determine the increase in health care with the specialist (gastroenterologist or endocrinologist). To determine which noninvasive evaluation strategy favors any treatment of fatty liver disease.

Hepatic Encephalopathy: Lactulose or Polyethylene Glycol (H.E.L.P.)
Hepatic EncephalopathyHE3 moreThe purpose of this study is to evaluate whether the use of polyethylene glycol is superior and more safe in treating hepatic encephalopathy compared to lactulose and also to determine if treatment with polyethylene glycol will reduce the duration of hospital stay.

Treatment of Dysglycemia Using Sitagliptin in Adolescents With Cystic Fibrosis
Cystic FibrosisThis pilot study will be conducted in adolescents with cystic fibrosis (CF) without diabetes but with abnormal glucose tolerance, and will assess the effects of sitagliptin on glucose regulation. An oral glucose tolerance test (OGTT) and a mixed meal tolerance test (MMTT), will be performed at baseline and again ~4 weeks after treatment with study drug. We will also look at blood sugars throughout the day using a continuous glucose monitor (CGM) before each time the MMTT/OGTT are performed. Several hormones that may affect the way the body regulates blood sugars will be measured in blood when the OGTT and MMTT are done. We will assess the effect this medicine has on blood sugars (using CGM) and the effect the medicine has on the hormones measured during the OGTT and MMTT.

Near Patient Microbial Testing in Cystic Fibrosis
Cystic FibrosisTo identify whether home monitoring of multiple physiological parameters and biomarkers in sputum could provide advanced warning of an infective exacerbation or treatment failure before changes in patient-reported symptoms.

Magnetic Resonance Imaging in the Evaluation of Liver Fibrosis
Liver FibrosisThe main purpose of this pilot study is to evaluate non-invasive magnetic resonance imaging (MRI) techniques in the detection and grading of liver fibrosis, so that the investigators can reduce the need of invasive techniques such as liver biopsy and transjugular hepatic venous portal pressure gradient (HVPG) measurements to assess the degree of liver scarring and portal hypertension.

Fenofibrate in Combination With Ursodeoxycholic Acid (UDCA) in Primary Biliary Cirrhosis
Primary Biliary CirrhosisThe purpose of this study is to determine if the combination of ursodeoxycholic acid (UDCA) and fenofibrate is more effective than UDCA alone in the treatment of primary biliary cirrhosis.

Multi-national Cirrhosis Study to Characterise the Association Between the Pharmacokinetics of NRL972...
Hepatic CirrhosisThis is a multi-centre, multi-national, open study to assess the pharmacokinetics of NRL972 in patients with hepatic cirrhosis CTP-classes A, B, and C (histologically confirmed by liver biopsy). The pharmacokinetics of NRL972 will be referenced to a Clinical Staging Matrix obtained during a clinical work-up of patients with hepatic cirrhosis. Patients to be studied will have histologically established hepatic cirrhosis or confirmed hepatic cirrhosis by an objective imaging study without confounding end-stage co-morbidity. Within 14 days of confirming eligibility, the investigations will be conducted over 2-5 days with the test procedures (clinical laboratory tests, ultrasound (US)-investigations, gastroscopy, NRL972- and MEGX'-test). Up to one week after the NRL972-test, a follow-up telephone call will be made.

Vitamin D3 for the Treatment of Low Vitamin D in Cystic Fibrosis
Cystic FibrosisVitamin D DeficiencyVitamin D deficiency is common in cystic fibrosis. Vitamin D deficiency frequently persists despite aggressive treatment with ergocalciferol, a vitamin D preparation also known as vitamin D2. Cholecalciferol, a vitamin D preparation also known as vitamin D3,may work better to increase vitamin D levels. Vitamin D is important for absorption of calcium from the diet and bone health. Vitamin D more recently has been found to play a role in regulating the normal inflammatory process. Since cystic fibrosis is a state of excessive inflammation, vitamin D may be playing a role in cystic fibrosis. We hypothesize: cholecalciferol will work better to increase vitamin D levels in patients iwth cystic fibrosis and that it will have an effect on markers of inflammation.

Use of Formula Fortified With DHA in Infants With Cystic Fibrosis
Cystic FibrosisThe hypothesis of this study is that feeding infants diagnosed with CF via newborn screening a formula enhanced with a specific fish-oil fatty acid known as DHA will improve growth and decrease pancreatic dysfunction (as measured by human fecal elastase-1 in stool) over the first year of life. Briefly, infants diagnosed with CF in the first month of life whose parents chose not to breast feed their babies will be invited to enroll in a study comparing a standard commercial infant formula (Enfamil) with a formula enriched with arachidonic acid (AA) and docosahexaenoic acid (DHA). The study formula has 3 times the amount of DHA available in commercially available formulas. Infants will have monthly tests of stool elastase and blood work at entry, 3, 6, 9 and 12 months of age.