Active clinical trials for "Fibrosis"

In cystic fibrosis, there is a critical need for better predictors of treatment response. The investigators have identified a panel of white blood cell biomarkers which can be directly measured as a blood test in subjects with cystic fibrosis. These biomarkers predict reduction of airway inflammation and infection more accurately than lung function testing, in patients receiving intravenous antibiotic therapy. In the current study, we hypothesize that this panel of gene biomarkers which can be readily measured from peripheral blood will sensitively predict changes in inflammation when patients receive inhaled antibiotic therapy, specifically Cayston (or inhaled aztreonam lysine). Patients enrolled in the study will have blood drawn before and after a month of inhaled Cayston, in order to test whether genes predict response to Cayston therapy more robustly than do standard measures such as lung function tests.

We hypothesize that individuals with Alpha-1 Antitrypsin (AAT) deficiency have ongoing liver injury which is not detected by the usual blood tests used to look at liver function. This ongoing liver injury leads to cirrhosis in a significant number of adults with AAT deficiency.

This is a prospective, longitudinal, 5-year study that will enroll participants from the existing Cystic Fibrosis Foundation (CFF) patient registry. Each enrolled participant will provide samples for microbiological evaluation, obtained upon enrollment and then once per year thereafter for 5 years.

Patients with sickle cell disease many have a number of systemic complications, including liver problems. Some of these liver problems lead to liver fibrosis/cirrhosis, secondary to chronic blood transfusions. The purpose of this study is to investigate FibroScan readings in patients with sickle cell disease and iron overload secondary to blood transfusions, and to correlate the FibroScan results with Ferriscan. A comparison with the results of FibroScan to patients with Sickle cell without known liver disease, who have never been on chronic transfusions and with normal liver function profiles will also be made.The primary hypothesis is that the results of FibroScan will correlate with the results of Ferriscan and liver biopsy.

RESOLVE's objective is to identify and characterize validated molecular targets capable of shifting primary organ repair towards fibroproliferative wound healing. Work package 2 (WP2) of RESOLVE includes the clinical study protocols within the RESOLVE system evaluating different forms of pulmonary repair in humans ranging from normal repair over mainly inflammatory to predominantly fibroproliferative repair. Hypothesis Fibrosis of the lung is an aberrant and intensified form of wound healing. It is the result of an unresolved disturbance of both initiation and control of repair which is partly age-related. As a result of the relentlessly activated wound healing reaction, mechanisms of inflammation largely representing the condition of chronic inflammation within the peripheral bronchial tree will aggravate this abnormal form of repair. A systematic comparison of the molecular pathology of fibrotic repair representing Varying intensity of fibrosis related to the pathology of usual interstitial pneumonia (UIP), Varying inflammatory mechanisms (UIP vs. Hypersensitivity pneumonitis [HP], acute and chronic), and Varying stages of age (Normal pulmonary repair in young and old individuals vs. acute/chronic HP vs. UIP) will be able to identify molecules capable of shifting regular repair towards fibroproliferative repair and elucidate their interrelationship with other molecules forming coordinated yet misdirected metabolic responses characteristic for fibroproliferative repair.

Protein-energy malnutrition and muscle wasting are a common finding among patients with liver cirrhosis. Its prevalence may range from 50-90% depending on the methods used for nutritional assessment. Even stable cirrhotic patients referred as Child A have muscle depletion and the majority of patients classified as Child C have significant depletion. Malnutrition has been shown to be related to several complications of cirrhosis Despite the importance of nutritional status in patient's outcome, there is no gold standard for nutritional assessment. Traditional techniques used in healthy subjects to assess nutritional status cannot be used in cirrhotic patients due especially to ascites and peripheral edema, and altered rates of biochemical markers due to liver failure. Bioelectrical impedance vector analysis has emerged as a useful method to assess body composition and nutritional status especially in patients at the extremes of body weight (fluid overload, excess of adipose tissue, etc.). The aim of this study is to evaluate whether malnutrition assessed by bioelectrical impedance vector analysis is related to the development of hepatic encephalopathy

Infections are frequent life-threatening events in patients with cirrhosis, occurring at least in 35% of hospitalized patients and accounting for 20% of inpatients death. Among cirrhotics, ascitic patients have the highest risk of death for sepsis. At the admission, no clear-cut clinical and biochemical features are helpful in diagnosing and prognostically stratifying those patients with sepsis. Procalcitonin (PCT)is a breakthrough marker presenting high sensibility and specificity in diagnosing bacterial infections in different clinical settings. The purpose of this study is to evaluate PCT as a diagnostic and prognostic tool for sepsis in hospitalized cirrhotic patients with ascitic decompensation.

Due to the limitations and the invasive nature of liver biopsy, there has been extensive interest in developing non-invasive tests to measure liver fibrosis (1). These are alternatives to liver biopsy that can be used in clinical practice, with benefits in terms of cost, risk, and patient convenience (2). Clinically applicable non-invasive tests include radiological studies, transient elastography (TE), and serum markers. We aim at studying acceptability, reliability, applicability and practical aspects of invasive and noninvasive methods for assessment of hepatic fibrosis and cirrhosis among hepatologists.

To determine the prevalence of nontuberculous mycobacterial infection (NTM) in pediatric patients with cystic fibrosis in the State fo Florida. HYPOTHESIS: There is high prevalence of NTM in CF pediatric population in the State of Florida, and likely higher than in the rest of the country.

Aortic stenosis (AS) is the most common valvular heart condition in the United Kingdom and the Western world. Surgery for severe AS prior to symptom onset is controversial. Conventionally changes in valve area and gradient are used to time intervention but myocardial changes may be more predictive of surgical need. This study aims to elucidate the role of diffuse myocardial fibrosis as a prognostic marker, implementing a novel, non-invasive MRI technique to measure it. Design: The investigators will measure diffuse myocardial fibrosis in 150 patients with severe narrowing of the aortic valve before and one year after valve replacement. Expected outcomes: Diffuse myocardial fibrosis measured by MRI scanning will predict outcome after surgery in aortic stenosis. Anticipated Health Benefits: Identify patients with higher post operative morbidity and mortality, who may benefit from earlier valve replacement.