View clinical trials related to Fibrosis.
Filter by:This study aims to determine whether respiratory system transfer impedance (Ztr) may fill an important clinical function by providing a reproducible, valid, and sensitive measure of airway obstruction in people with CF.
Hypothesis:DCI 1020 capsules are safe and effective in treating exocrine pancreatic insufficiency in CF patients <= 2 years of age. The results of this study are intended to be submitted to the FDA as part of the NDA package for marketing approval of PANCRECARB (DCI 1020).
Pulmonary Arterial Hypertension (PAH) in the setting of Idiopathic Pulmonary Fibrosis(IPF)is a risk factor for morbidity and mortality in the peri-lung transplant(LT) setting. Currently there is no significant data to support the use of pulmonary vasodilators for PAH in the setting of interstitial lung disease such as IPF. The majority of IPF patients have PAH either at rest or during exercise. The study hypothesis is that bosentan may improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH.
Pulmonary Arterial Hypertension (PAH) in the setting of Idiopathic Pulmonary Fibrosis(IPF)is a risk factor for morbidity and mortality in the peri-lung transplant(LT) setting. Currently there is no significant data to support the use of pulmonary vasodilators for PAH in the setting of interstitial lung disease such as IPF. The majority of IPF patients have PAH either at rest or during exercise. The study hypothesis is that sildenafil may improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH.
The purposeof this study is to determine the effect of a large volume paracentesis (procedure in whihc a catheter is placed to remove fluid from the abdomen) on the severity of fatigue i patients with cirrhosis (severe scarring of the liver) and large volume ascites (fluid in the abdomen).
We hypothesize use of the insulin pump will improve body weight, lean body mass, whole body protein turnover, hepatic glucose production (HGP), and blood sugar control in CF patients with impaired glucose tolerance or patients with CF related diabetes (CFRD). We further hypothesize that HGP is also elevated in children/adolescents with type 1 diabetes and that the insulin pump will result in decreased HGP.
Our specific aims include: 1. AIM 1. Characterization of glucose tolerance, nutritional and clinical status, socioeconomic status, family history of diabetes and genotype in Hispanic CF children compared to Caucasian CF children. Each child will undergo a two-hour oral glucose tolerance test and will be categorized by glucose tolerance according to standards set forth by the 1998 CF Consensus Conference on Diabetes. Nutritional status will be determined by three-day food journals and intake will be compared to energy needs measured by indirect calorimetry. Socio-economic status will be calculated from reported family income and medical insurance coverage. Genotyping will be done at the laboratory of Dr. Arthur Beaudet at Baylor College of Medicine. Clinical status will be measured using modified NIH scores. Family history for both type 1 and type 2 diabetes will be obtained in Spanish by Dr.Vanderwel. This specific aim tests the hypothesis that glucose intolerance /frank CF related diabetes occurs at a younger age in Hispanics than in Caucasians with CF, and is correlated to family history of diabetes and clinical status. 2. AIM 2. Characterization of insulin secretion and insulin sensitivity. Previous studies in adults have described peripheral insulin resistance as a major cause of CF related diabetes, yet studies have not been conducted in children. Studies in adults and children without CF suggest that insulin resistance occurs more frequently in Hispanics. We will measure insulin secretion and insulin sensitivity using the frequently sampled intravenous glucose tolerance test (IVGTT) and the minimal model analysis of Bergman, as modified for children. This specific aim tests the hypothesis that Hispanic children with CF have worse peripheral insulin resistance, but similar insulin secretion when compared to Caucasian children with CF. 3. AIM 3. Quantification of post-absorptive gluconeogenesis and whole body protein turnover. Total hepatic glucose production (HGP) will be measured using [6,6-2H2]glucose. We will quantify gluconeogenesis by measurement of the incorporation of 2H into the 2nd, 5th and 6th carbons of glucose following 2H20 administration method of Landau). We will determine whole body protein turnover using the stable isotopes [1-13C]leucine and will measure serum amino acid levels. This specific aim tests the hypothesis that gluconeogenesis and whole body protein turnover are disproportionately higher in Hispanic children and adolescents with CF than in Caucasian CF children.
We hypothesize that the anabolic effects of growth hormone (GH) will improve the clinical status of patients with CF by improving lean body mass, osteopenia, muscle strength, pulmonary function, and quality of life. We will recruit 40 malnourished CF patients for this 12-month study. All 40 patients will be treated with recombinant human growth hormone (rhGH). Each patient will serve as his/her own control by obtaining medical records for 6 months to 1 year prior to study enrollment or by completing 6 months of study without GH prior to being treated for 1 yr. with GH.
Cystic fibrosis (CF) is a catabolic condition, and children affected with this disease frequently have problems with growth, despite adequate nutrition. We hypothesize that the anabolic effects of growth hormone (GH) will improve the height and weight of CF patients and thereby improve their clinical status and their quality of life. We further hypothesize that these effects will be sustained for at least one year after GH treatment is complete.
The purpose of this study is to create a database of demographics and samples in idiopathic pulmonary fibrosis.