
A Study of the Safety and Clinical Effects of Interferon Gamma-1b in Patients With Idiopathic Pulmonary...
Idiopathic Pulmonary FibrosisStudy GIPF-002 is a phase 2 study designed to characterize the biologic and clinical effects of IFN-g 1b. The objective of the Study is to characterize the biologic and clinical effects of IFN-g 1b administered to patients with idiopathic pulmonary fibrosis (IPF). The Study will be conducted at multiple sites and enroll 30 patients with IPF who have failed treatment with corticosteroids.

Study of INS37217 Inhalation Solution in Mild to Moderate Cystic Fibrosis Lung Disease
Cystic FibrosisThe purpose of this study is to assess the safety and effectiveness of multiple dosages of INS37217 compared to placebo over 28 days in subjects with mild to moderate cystic fibrosis (CF) lung disease. Study drug will be administered through a nebulizer (a device that delivers medication as a mist by breathing it in).

Oral TheraCLEC™ - Total in Cystic Fibrosis Subjects With Exocrine Pancreatic Insufficiency
Cystic FibrosisPancreatic InsufficiencyThe purpose of this study is to determine the most effective dose of a new enzyme therapy on oral nutrient absorption in cystic fibrosis (CF) subjects with exocrine pancreatic insufficiency.

Vasoconstrictors as Alternatives to Albumin After Large-Volume Paracentesis (LVP) in Cirrhosis
AscitesCirrhosisThis clinical trial compares a combination of two drugs that constrict blood vessels (Octreotide LAR and Midodrine) to albumin after large volume paracentesis. Subjects have cirrhosis and ascites.

Study Evaluating the Safety and Efficacy of Etanercept in Patients With Idiopathic Pulmonary Fibrosis...
Pulmonary FibrosisThe objective of the study is to evaluate the safety and efficacy of etanercept in comparison with placebo in a double-blind, parallel, randomized fashion in subjects with idiopathic pulmonary fibrosis (IPF) who failed previous therapy. The treatment period will be up to 1 year. The primary objective is evaluation of safety and efficacy. Secondary: The secondary objective is to evaluate quality of life (QoL) and pharmacokinetics (PK).

The Effects of Long Term Inhalation of Hypertonic Saline in Subjects With Cystic Fibrosis
Cystic FibrosisThe effect of long term inhalation of hypertonic saline in subjects with cystic fibrosis on lung function, incidence of respiratory tract infections, quality of life, quantitative microbiology and sputum cytokine profile. The hypothesis is that regular inhalation of nebulised hypertonic saline will have a beneficial effect on lung function and other clinical outcomes with no adverse effects on infection and inflammation in adults and children with cystic fibrosis.

Study of GC1008 in Patients With Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic Pulmonary FibrosisThis study is designed to investigate whether GC1008, an antibody that neutralizes TGFb, is safe in treating patients with idiopathic pulmonary fibrosis. The highest dose without excessive side effects will be identified. Tests will determine how long GC1008 is in the body and how it is excreted.

Study of Alendronate to Prevent and Treat Osteoporosis in Cystic Fibrosis Patients
Cystic FibrosisOsteoporosis2 moreThe primary objective of this study is to determine efficacy of 70 mg alendronate once weekly compared to placebo. This will be measured by percent changes in lumbar spine(LS) bone mineral density(BMD) in adult cystic fibrosis(CF)patients after one year of treatment. The investigators hypothesize that in adult CF patients with osteopenia or osteoporosis, alendronate 70 mg once weekly will produce a mean increase from baseline in lumbar spine BMD that is greater than that observed with placebo at 12 months.

Study of Ibuprofen to Preserve Lung Function in Patients With Cystic Fibrosis
Cystic FibrosisOBJECTIVES: I. Determine the effect of different doses of ibuprofen on neutrophil (polymorphonuclear leukocyte; PMN) delivery to a mucosal surface (the oral mucosa) in patients with cystic fibrosis and healthy controls. II. Determine the duration of effect (and possible rebound effect) of ibuprofen on PMN delivery to a mucosal surface in these patients.

Oral Pirfenidone for the Pulmonary Fibrosis of Hermansky-Pudlak Syndrome
AlbinismInborn Errors of Metabolism3 moreHermansky-Pudlak Syndrome (HPS) is an inherited disease that results in decreased pigmentation (oculocutaneous albinism), bleeding problems due to a platelet abnormality (platelet storage pool defect), and storage of an abnormal fat-protein compound (lysosomal accumulation of ceroid lipofuscin). The disease can cause poor functioning of the lungs, intestine, kidneys, or heart. The most serious complication of the disease is pulmonary fibrosis and typically causes death in patients 40 - 50 years old. The disorder is common in Puerto Rico, where many of the clinical research studies on the disease have been conducted. Neither the full extent of the disease nor the basic cause of the disease is known. There is no known treatment for HPS. The drug pirfenidone blocks the biochemical process of inflammation and has been reported to slow or reverse pulmonary fibrosis in animal systems. In this study researchers will select up to 40 HPS patients diagnosed with pulmonary fibrosis. The patients will be randomly divided into 2 groups. The patients will not know if they are taking pirfenidone or a placebo "sugar pill". Group one will be patients who will receive pirfenidone. Group two will be patients who will receive a placebo "sugar pill" The major outcome measurement of the therapy will be a change in the lung function (forced vital capacity). The study will be stopped if one therapy proves to be more effective than the other.