
Tolerability of Inhaled Hypertonic Saline in Infants With Cystic Fibrosis
Cystic FibrosisThis is an open-label pilot study of the safety and tolerability of 7% hypertonic saline inhaled twice daily for 14 days in infants with CF, 12 to 30 months of age.

The Effect of rhDNase on Ventilation Inhomogeneity in Patients With Cystic Fibrosis
Cystic FibrosisThis study will assess whether rhDNase treatment improves ventilation inhomogeneity as assessed by lung clearance index (LCI) in patients with cystic fibrosis (CF).

Use of Fenofibrate for Primary Biliary Cirrhosis
Primary Biliary CirrhosisThis is a pilot study to evaluate the safety and efficacy of fenofibrate on patients with primary biliary cirrhosis who have an incomplete response to ursodeoxycholic acid.

Scandinavian Cystic Fibrosis Azithromycin Study
Cystic FibrosisIn patients with Cystic Fibrosis, recurrent airway infection caused by Pseudomonas aeruginosa ultimately leads to chronic airway infection. The purpose of this study is to determine whether supplementary low-dose azithromycin to standard inhaled colistin and oral ciprofloxacin in the treatment of intermittent pseudomonas airway-infection can postpone the next episode of intermittent pseudomonas airway-infection and prevent development of chronic airway-infection.

Improving Vitamin D Status In Cystic Fibrosis
Cystic FibrosisThe purpose of this study is to establish an effective method to correct vitamin D deficiency in subjects with cystic fibrosis. The investigators will examine cholecalciferol, ergocalciferol and UV light.

A Phase 2 Study to Determine the Safety and Efficacy of Inhaled Dry Powder Mannitol in Cystic Fibrosis...
Cystic FibrosisCystic fibrosis is the most frequent lethal genetic disease of childhood. Causes disruption of glandular function of the pancreas, intestine, liver, lungs (causing chronic lung infection with emphysema), sweat glands and reproductive organs. We know that many CF patients die of lung failure, brought about in part by repeated lung infections caused by thick, sticky mucus that cannot be readily cleared from the lung. Inhaled mannitol is an osmotic agent that has been investigated in a number of small studies that have examined mucociliary clearance, quality of life and lung function in CF and bronchiectasis. The promising results of these studies warrant futher investigation. The aim of this study is to assess the safety and efficacy of inhaled mannitol when administered twice a day over two weeks in CF.

Dose Escalation Study of QR-010 in Homozygous ΔF508 Cystic Fibrosis Patients
Cystic FibrosisA randomized, double-blind, placebo-controlled study of single and multiple ascending doses of QR-010 in adults homozygous for ΔF508 Cystic Fibrosis.

First Time in Human (FTIH) Study of GSK3008348 in Healthy Volunteers and Idiopathic Pulmonary Fibrosis...
Idiopathic Pulmonary FibrosisGSK3008348 is an investigational drug, being developed by GlaxoSmithKline Research and Development Limited (the Sponsor, a pharmaceutical company based in the UK) for the treatment of Idiopathic Pulmonary Fibrosis (IPF). IPF is a rare and poorly understood disease that causes scarring of the lungs. The main symptoms are shortness of breath and a dry cough. Symptoms generally worsen over time and in some subjects may prove fatal. The cause of IPF is unknown. This is a First Time in Human, Phase 1, 3-part study which is being carried out on behalf of the Sponsor by Quintiles. The primary purpose of Part A is to examine the safety and tolerability of single nebulised (a medicated spray) doses of GSK3008348 following inhalation in healthy volunteers. The secondary objective is to determine how and at what rate the body absorbs, distributes, breaksdown and eliminates the drug. Parts B and C of this study will be in-patients with Idiopathic Pulmonary Fibrosis (IPF). The purpose of Part B and C is to examine the safety and tolerability, and how much of the drug binds to its target, following single nebulised (a medicated spray) doses of GSK3008348 following inhalation in patients with Idiopathic Pulmonary Fibrosis (IPF). The secondary objective is to determine how and at what rate the bodies of these patients absorbs, distributes, breaksdown and eliminates the drug. The total duration of Part A will be 65 - 87 days, Part B 62 days and Part C 43 days.

Home-Based Pulmonary Rehab for Patients With Pulmonary Fibrosis
Idiopathic Pulmonary FibrosisOutpatient (phase 2) pulmonary rehab is an important, but mostly underutilized intervention to improve physical function in patients with idiopathic pulmonary fibrosis (IPF). Of those individuals who complete phase 2 pulmonary rehab, only a small cohort continues with any type of maintenance exercise therapy (phase 3). This is largely due to personal factors, such as access to transportation, weather, scheduling difficulties, and cost. Little is known about how to enhance physical activity among patients with IPF. In addition, no studies have investigated the long-term maintenance strategies of physical training in patients with IPF. Thus, the purpose of this study is to test the feasibility and effectiveness of a home-based, pulmonary rehab program for patients with IPF. A randomized, blinded, clinical trial (RCT) design with two arms -- Wii Fit Plus exergame program and cognitive video gaming, with no active whole body movement involved - will be used to investigate the benefits of a home-based exergame program on improving pulmonary-related function and symptoms in patients with IPF.

Pilot Trial of Phototherapy for Acute Depression in Hospitalized Cystic Fibrosis Patients
DepressionCystic FibrosisThe purpose of this study is to determine whether bright light phototherapy can improve depressive symptoms in hospitalized patients with cystic fibrosis (CF) with subsequent effects on quality of life and illness recovery. It is hypothesized that phototherapy will improve depressive symptoms and decrease length of stay in depressed patients with CF who are hospitalized.