Active clinical trials for "Fibrosis"

Background: - Liver disease is a leading cause of death in people who have the human immunodeficiency virus (HIV). It especially affects those who have both HIV and hepatitis B or C viruses. Most research on HIV-related liver disease has been conducted in North America and Europe. However, HIV-related liver disease in Uganda and other African nations may involve other diseases that are not common in the West, and may not involve hepatitis B or C. Researchers want to study HIV-related liver disease in Uganda to learn more about the differences between Western and African trends of this disease. Objectives: - To study HIV-related liver disease in rural Uganda. Eligibility: Individuals at least 18 years of age who were tested for possible liver disease. Some participants will have HIV infection; others will be uninfected. All participants will be from rural areas of Uganda. Design: Participants will have at least two study visits. Participants will have a physical exam and medical history. They will complete a questionnaire about health and quality of life. Blood, urine, and stool samples will be collected. Participants will also have a liver scan to check for liver scarring, and an ultrasound to take images of the liver. Participants who may have liver disease will visit a local hospital for more tests. A liver biopsy will be performed to collect liver tissue samples.

The purpose of this study is to determine whether ivacaftor, a recently FDA-approved CFTR potentiator, improves bone micro-architecture and strength in patients with cystic fibrosis with at least one G551D CFTR mutation.

This study is an observational and retrospective study of patients with pulmonary fibrosis associated or not with telomerase mutation. The purpose of this study is to describe in detail the cases with telomerase mutation in terms of features on CT scan, respiratory function and evolution, in comparison to control subjects with idiopathic pulmonary fibrosis and no telomerase mutation identified or family history.

Pulmonary exacerbations of CF are an important cause for the experienced disability of patients, respiratory symptoms, and decreases in lungfunction, which require antibiotic therapy at home or in the hospital. Therefore, prevention of exacerbations in CF is important. The aim of this study was to assess the predictive properties of inflammatory markers in exhaled breath for pulmonary exacerbations in children with CF. In addition the reliability of home monitor assessments of symptoms and lungfunction was investigated.

In the planned study, 60 patients with mild cystic fibrosis (CF) with and without the involvement of small airways (small airway disease - SAD) are to be compared with a historical control group matched in age and gender. During the first study visit subjects are asked to perform a pulmonary function test (spirometry, body plethysmography with helium, determination of "Trapped Air") and exhaled nitric oxide (eNO) and exhaled carbon monoxide (eCO) measurements will be done in exhaled air. In addition, a blood sample is drawn to describe inflammatory status. Sputum is induced as well. During the second study visit, a non-specific bronchial provocation test(methacholine PD20 FEV1) is performed. The aim of the study is to get a characterization of the bronchial and systemic inflammation (IL-1ß, IL-6, IL-8, IL-17, TNF-α, NFKB, and recognition structures like TLR2 and TLR4) in CF patients with and without the involvement of the small airways, which may point to new treatment strategies.

Elevated fecal calprotectin is a biomarker for intestinal inflammation in cystic fibrosis

We wish to measure the inspiratory flow and volumes generated by the subjects inhaling from a spirometer with a high resistance dry powder inhaler in series in subjects with cystic fibrosis.

Our general aim is to determine the prevalence of diabetic microvascular complications in CFRD patients with and without fasting hyperglycemia, and to explore whether the presence of these complications is related to diabetes or CF factors. This cross-sectional study will provide pilot data for a longitudinal study of diabetes complications in CF.

Background Chronic airway inflammation is present in cystic fibrosis. Non-invasive inflammometry may be useful in disease management. Objective We studied 1) the ability of fractional exhaled nitric oxide and inflammatory markers (acidity, nitrite, nitrate, hydrogen peroxide, 8-isoprostane, interferon-γ, tumor necrosis factor-α, interleukin-2,-4,-5,-10) in exhaled breath condensate, to discriminate between cystic fibrosis and control children, and, 2) the relationship of biomarkers with control and severity of cystic fibrosis. Methods In 98 children (48 cystic fibrosis / 50 controls), condensate was collected using a glass condenser. Exhaled nitric oxide was measured using the NIOX®.

Cystic Fibrosis patients continue to have bowel problems even after adequate pancreatic enzyme supplementation. There may be pathology of the lining of the bowel. Capsule endoscopy will be used to photograph the entire bowel.