View clinical trials related to Fibrosis.
Filter by:The benefits of short-term naso-gastric feeding in patients with advanced cirrhosis remain uncertain. This was a randomised study comparing naso-gastric feeding and oral feeding in Asian patients with cirrhosis who were admitted to hospital for decompensation.
Cystic Fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The encoded protein, CFTR, is an epithelial chloride ion channel responsible for aiding in the regulation of salt and water absorption and secretion in various tissues. Although the disease affects multiple organs, the leading cause of mortality is the progressive loss of lung function. Obstruction of airways with thick mucus, chronic bacterial infection of the airways, and inflammatory response are all thought to play a role in causing lung damage. Through its function as a chloride channel, CFTR is believed to be integral in epithelial ion and water transport and hence, maintaining the normal hydration of lung secretions. VX-770 (ivacaftor) is a potent and selective potentiator of wild-type, G551D, F508del, and R117H forms of human CFTR. Based on in vitro studies and pharmacologic, pharmacokinetic (PK), and safety profiles, VX-770 has been selected for clinical development as a possible treatment for patients with CF. Hyperpolarized noble gas magnetic resonance imaging (HG-MRI) is a promising new means of assessing lung function by direct imaging of certain non-radioactive isotopes of an inert noble gas, such as helium or xenon. Through this technique, high-resolution 3-dimensional images of lung ventilation can be obtained in both pediatric and adult patients during a single short breath-hold following inhalation of the gas. This is a 2-part study to evaluate the effect of VX-770 on hyperpolarized helium-3 magnetic resonance imaging (3He-MRI), and to evaluate the safety and efficacy of VX-770 in subjects aged 12 years and older with CF who have the G551D-CFTR mutation. Part A is a single-blind, placebo-controlled study that includes 4 weeks of VX-770 treatment and 4 weeks of placebo treatment. Part B is an open-label, 48 week study of long-term effect of VX 770 on hyperpolarized 3He-MRI.
Background: - Heart failure is a common cardiovascular disorder whose incidence increases with age, affecting up to 10% of people older than 65 years of age. As the population ages, the prevalence and cost of heart failure will continue to rise. Researchers are interested in using noninvasive imaging methods to better understand the symptoms and effects of heart failure. Objectives: - To conduct a noninvasive comparative imaging study of individuals with heart failure. Eligibility: - Individuals at least 18 years of age who have been diagnosed with heart failure (with at least mild symptoms and slight limitations on physical activity). Design: - This study will last approximately 2 years and will require four visits to the National Institutes of Health Clinical Center, with one screening visit and three study visits. - Participants will be screened with a full medical history and physical examination, as well as blood and urine samples. - Participants will have the following tests during each study visit: - Physical examination - Blood and urine samples - Cardiac magnetic resonance imaging - Cardiac computerized tomography to study the blood vessels in and leading to the heart - Echocardiogram to evaluate heart function - Electrocardiogram to measure heart electrical activity - The three study visits will take place 1 year apart. Participants will also receive follow-up phone calls 6 months after the first and second visits. - No treatment will be provided as part of this protocol.
The objective of this study is to compare the lower airways inflammatory response to infection/pulmonary exacerbation among children known to have Primary Ciliary Dyskinesia (PCD) with children known to have Cystic Fibrosis (CF) as measured by the presence of inflammatory mediators in expectorated/induced sputum.
Hypothesis 1: Microaspiration, as diagnosed by bronchoalveolar lavage (BAL) pepsin, is common in patients with IPF. Hypothesis 2a: Baseline clinical variables and co-morbid conditions are risk factors for microaspiration in patients with IPF. Hypothesis 2b: Baseline biological variables reflecting alveolar epithelial injury and inflammation are markers of microaspiration in IPF. Hypothesis 3a: Microaspiration will lead to a more rapid rate of decline in pulmonary function. Hypothesis 3b: Microaspiration will lead to higher rates of urgent medical care use (i.e. unscheduled clinic visit, emergency room visit, or hospitalization).
Cystic fibrosis (CF) is the most common lethal, inherited disorder among Caucasians. Choline is an essential vitamin and as a methyl donor is critically needed to support the normal metabolism. Our previous studies have demonstrated that children with CF have depleted levels of choline. The purpose of this study is to gather data on the status of choline and related metabolites in children with Cystic Fibrosis by age and gender. The hypothesis for this study is that in children with CF, deficiency of choline and related metabolites will increase with increasing age.
The specific aims for this study are: 1. To determine if sonographic findings predict the risk of progression of liver disease to cirrhosis by comparing cystic fibrosis subjects with heterogeneous echogenicity pattern on ultrasound to those with normal echogenicity pattern on ultrasound 2. To develop a database and biorepository of serum, plasma, urine and DNA to aid the investigations in ascertaining the mechanisms, consequences, genetic risk factors and biomarkers for the development of cirrhosis 3. To determine if there are differences in health related quality of life, pulmonary or nutritional status in children with cystic fibrosis who have a heterogeneous echo pattern on ultrasound compared to those who have a normal echo pattern on ultrasound 4. To determine if Doppler velocity measurements of hepatic and splenic vessels predict an increased risk for the development of cirrhosis. 5. To determine if cirrhosis on ultrasound progresses to portal hypertension during the study period 6. To determine if homogeneous liver progresses to either cirrhosis or heterogeneous liver. 7. To determine the frequency of complications of portal hypertension during follow up in those identified with cirrhosis by year 6 of the study
Cystic fibrosis (CF) is a genetic disorder caused by a mutation in the gene that makes the cystic fibrosis transmembrane conductance regulator (CFTR) protein. A specific type of mutation called a nonsense (premature stop codon) mutation is the cause of CF in approximately 10% of patients with the disease. Ataluren is an orally delivered investigational drug that has the potential to overcome the effects of the nonsense mutation. This study is a Phase 3 extension trial that will evaluate the long-term safety of ataluren in adult and pediatric participants with nonsense mutation CF (nmCF), as determined by adverse events and laboratory abnormalities. The study will also assess changes in pulmonary function, CF pulmonary exacerbations, health-related quality of life, antibiotic use for CF-related infections, CF-related disruptions to daily living, body weight, and CF pathophysiology. Funding source for this study is the FDA OOPD.
To investigate safety of BIBF 1120 in Japanese patients with idiopathic pulmonary fibrosis (IPF), with and without pirfenidone background treatment. To assess pharmacokinetics of BIBF 1120 in Japanese patients, with and without pirfenidone background treatment. To assess pharmacokinetics of pirfenidone in Japanese patients, alone and in combination with BIBF 1120 treatment.
Phase 4, open-label, two-year, prospective, multi-center, follow-up study conducted at up 15 sites in USA. Approximately 1,000 patients with moderate-to-severe CKD will be enrolled and followed for up to 24 months.