
Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of QAU145 in Patients With Cystic Fibrosis...
Cystic FibrosisThis study will investigate the safety, tolerability, pharmacokinetic, and pharmacodynamic response of patients with cystic fibrosis to administration of QAU145 via intranasal spray.

Safety and Efficacy of Pioglitazone as an Anti-inflammatory for the Treatment of Cystic Fibrosis...
Cystic FibrosisStudy Hypothesis: Pioglitazone may decrease inflammation in cystic fibrosis lung disease. Primary outcomes: Markers of inflammation (neutrophils, elastase, cytokines and bacteria)will be measured in induced sputum specimens before and after a 4 week treatment period with pioglitazone in clinically stable CF patients.

Albumin Administration in Patients With Cirrhosis and Infections Unrelated to Spontaneous Bacterial...
CirrhosisSpontaneous bacterial peritonitis (SBP) present in cirrhotic patients induces severe circulatory dysfunction, which results in renal failure in up to 30% of the patients. Renal failure is an important prognostic marker, representing the major predictive factor of in-hospital mortality. Recent studies have shown that plasma volume expansion with albumin associated with cefotaxime in patients with SBP is more efficient to prevent renal failure than cefotaxime treatment alone. The in-hospital and three-month mortality rates, furthermore, were significantly lower in the group treated with albumin. It is not known if other bacterial infections unrelated to SBP represent a risk factor for the development of renal failure among cirrhotic patients. The researcher's group has recently performed a study to evaluate the incidence, characteristics and outcome, of renal failure in patients with cirrhosis and bacterial infections unrelated to SBP associated with the systemic inflammatory response syndrome (Terra, unpublished results). Among a total of 106 patients, 29 (27%) presented renal failure during the course of infection. Renal failure was characterized by intense renal vasoconstriction (intrarenal resistive index of 0.83 +/- 0.09, measured by Doppler ultrasound), reduction of mean arterial pressure and an important activation of endogenous vasoconstriction systems. The three-month survival probability of patients with infection and renal failure was 34 %, much lower than that of patients with infection but not presenting renal failure (87%, p<0.0001). These results suggest that the development of renal failure in patients with cirrhosis and bacterial infections different from SBP, associated with signs of a systemic inflammatory response, is very frequent and results in a very poor prognosis. Taken as a whole, these data strongly indicate the need to consider these patients as candidates for liver transplantation and to plan strategies for its prevention. The objective of this project, therefore, is to evaluate if the plasma volume expansion with albumin, associated with conventional antibiotic therapy, can prevent the development of renal failure and increase survival rates in cirrhotic patients with bacterial infections unrelated to spontaneous bacterial peritonitis.

Open-Label Phase III Long-Term Safety Trial of Liprotamase
Cystic FibrosisExocrine Pancreatic InsufficiencyThis is an open-label, single-arm clinical study investigating the long-term safety of ALTU-135 treatment in patients with cystic fibrosis (CF)-related exocrine pancreatic insufficiency (PI).

Zinc Homeostasis and Kinetics in Children With Cystic Fibrosis (CF)
Cystic FibrosisZinc deficiency has occasionally been reported in CF, but the need for additional zinc is unclear. We will examine the effect of additional zinc supplements on zinc absorption, zinc excretion, zinc balance and zinc kinetics in children with CF. Twelve children with CF will receive additional zinc, and twelve will not. We will also compare the children with CF to 12 healthy children without CF. We hypothesize that children with CF will have poor zinc absorption and high zinc excretion, leading to poor zinc balance and abnormal zinc kinetics. We hypothesize that those children with CF who are given zinc supplements will have higher zinc balance and more normal zinc kinetics than those without supplementation, and that zinc balance and zinc kinetics will be similar to healthy children.

Safety and Tolerability Study of FG-3019 in Participants With Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary FibrosisThe main purpose of this study is to evaluate the safety, tolerability, pharmacokinetics, and immunogenicity of FG-3019, a therapeutic antibody designed to block the pro-fibrotic activity of connective tissue growth factor (CTGF). CTGF triggers the production of collagen and fibronectin, which cause scarring and thickening of the lungs. Participants will be assigned sequentially to one of the 3 dose group: Low, medium, and high FG-3019.

Efficacy of Albumin Dialysis to Treat Patients With Hepatic Encephalopathy Using The Molecular Adsorbent...
Hepatic EncephalopathyLiver Failure3 moreThe primary objective of the study was to compare the efficacy, safety and tolerability of Extracorporeal Albumin Dialysis (ECAD) using the Molecular Adsorbent Recirculating System (MARS®) device in improving severe HE by 2 grades compared to Standard Medical Therapy (SMT) in patients with chronic End Stage Liver Disease (ESLD) during a 5 day study period.

Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary FibrosisThe purposes of this study are to assess the efficacy of treatment with pirfenidone 2403 milligrams per day compared with placebo in patients with idiopathic pulmonary fibrosis (IPF)and to assess the safety of treatment with pirfenidone 2403 milligrams per day compared with placebo in patients with idiopathic pulmonary fibrosis.

A Comparison of the Drug Telbivudine (LdT) and Lamivudine in Adults With Decompensated Chronic Hepatitis...
Hepatitis BCirrhosisThis trial is being done to see if the investigational drug, LdT (Telbivudine), is safe and effective in the treatment of hepatitis B infection. In addition to this, we will be looking at the comparison of the effects (good and bad) of LdT and lamivudine.

Whole Body Vibration - a New Therapeutic Approach to Improve Muscle Function in CF
Cystic FibrosisThe purpose of this study is to evaluate the effects of whole body vibration (WBV) on muscle function in adult CF-patients.