Active clinical trials for "Pulmonary Fibrosis"

Application of artificial intelligence deep learning algorithm to analyze the relationship between hormone sensitivity of idiopathic interstitial pneumonia and imaging features of high resolution CT.

Analysis of exhaled breath condensate biomarkers and cough severity in patients with idiopathic pulmonary fibrosis.

This study measures the 4 metre gait speed (4MGS) test in patients with Idiopathic Pulmonary Fibrosis (IPF). The investigators are interested to see whether usual walking speed in IPF patients changes following pulmonary rehabilitation and if it changes, what is the smallest change that is meaningful to patients.

This study investigates whether usual walking speed, measured by the 4 metre gait speed test (4MGS), and change in usual walking speed over 6 months predicts death and hospital admissions in patients with Idiopathic Pulmonary Fibrosis.

The investigators aim to assess whether pulmonary MRI (hyperpolarised 129Xe ventilation imaging [Xe-MRI]) can detect changes in ventilation defects in patients with CF before and after treatment for a pulmonary exacerbation. The investigators will determine whether changes seen using pulmonary Xe-MRI are associated with changes in pulmonary function (spirometry, lung volumes, lung clearance index [LCI]) in patients with CF before and after pulmonary exacerbation.

The purpose of this study is to determine whether extension of the conducting airways into the distal lung, or bronchiolization, occurs early in the course of Idiopathic Pulmonary Fibrosis, a disease wherein normal lung structures are destroyed and replaced by non-functional scar tissue.

Cystic fibrosis (CF) is the most common autosomal recessive genetic disease in Caucasians. It results in lung disease that affects quality of life and causes early death. Lung damage from CF starts in infancy and continues over time. Lung damage can negatively affect how the lung functions. It would be ideal to measure lung damage in CF patients in three instances: (1) During the first year of life after diagnosis by state newborn screening programs, (2) In children and adults over long periods of time (years), and (3) During times of illness (pulmonary exacerbation), to allow for better treatment and therapy to prevent loss of lung function. The lung is made of elastin, collagen and cartilage. When the lung is damaged by CF, these components break down into pieces that can be measured in urine, sputum and blood. These products may represent markers of lung injury. We believe that the levels of these markers will be increased over time in CF patients and even higher in patients who are sick with lung symptoms. The goal of my research is to measure the amount of lung breakdown products in urine, sputum and blood in infants, children and adults with CF during times when well and also during times of illness. I also hope to use new technologies involving the study of proteins and metabolites in samples like sputum, urine and blood to help provide new information regarding CF lung disease. These studies will help us to better treat CF lung disease.

Background: - Erdheim Chester Disease (ECD) is a very rare disease in which abnormal white blood cells start growing and affect the bones, kidneys, skin, and brain. ECD can cause severe lung disease, kidney failure, heart disease, and other complications that lead to death. Because ECD is a rare disease, found mostly in men over 40 years of age, there is no standard treatment for it. More information is needed to find out what genes can cause ECD and how best to treat it. Objectives: - To collect study samples and medical information on people with Erdheim Chester Disease. Eligibility: - Individuals 2 to 80 year of age who have been diagnosed with Erdheim Chester Disease. Design: Participants will be screened with a physical exam and medical history. Participants will have a study visit to provide samples for study, including blood, urine, and skin tissue samples. Participants will also have lung, heart, and muscle function tests; imaging studies of the brain, chest, and whole body; a treadmill running stress test; an eye exam; and other tests as needed by the study doctors. Participants will be asked to return for a similar set of tests every 2 years, and to remain in contact for possible treatment options.

The objective of this study is to assess the effects of using HRQL measures in the clinical care of pre- and post-lung transplant patients. The hypotheses are that the inclusion of HRQL measures, the Health Utilities Index System Mark 2(HUI2) and Mark 3 (HUI3), in routine clinical care of pre- and post-lung transplant patients, will: 1) improve patient-clinician communication;2) affect patient management; 3) improve patients' HRQL.

Lung transplantation is a life saving procedure for patients with a terminal lung disease such as cystic fibrosis. Approximately, one in 3,500 children in the United States are born with cystic fibrosis each year with the predicted survival reaching 36.9 years in 2006. Cystic fibrosis was the third lead indication for lung transplantation in 2006. Cystic fibrosis is a genetic disease that can affect the way the body can remove salt from various organs. It results in mucus blocking the ducts of the lungs and pancreas leading to inability to handle oxygen and malabsorption of nutrients. Malabsorption is a common complication of cystic fibrosis that can affect the way the anti-rejection medications are absorbed. One medication that is utilized after transplant to prevent rejection is mycophenolate mofetil. This medication may not be absorbed adequately in this population due to their disease thus placing these patients at increased risk of rejection. At the investigators' institution, all transplant patients are initiated at the same mycophenolate dose regardless of their underlying disease. The limited available literature regarding cystic fibrosis transplant patients and mycophenolate suggests that these patients require higher doses due to their erratic absorption. The purpose of this study is to evaluate the effects of mycophenolate mofetil on the body in lung transplant patients who have cystic fibrosis in efforts to improve survival outcomes.