GENERAL CLINICAL RESEARCH CENTER

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EXCEED THE SPACE PROVIDED. This application is for a competitive renewal of a multi-categorical General Clinical Research Center located at the Children's Hospital of Cincinnati. The Center provides inpatient, outpatient and scatter bed facilities within the Children's Hospital and a Core Laboratory supporting biochemical assays, body composition measurements, and behavioral studies at the Children's Hospital. A proposed satellite at the Veterans Administration Medical Center will help support studies in medically unstable adults who are not suitable to be studied at the Children's Hospital GCRC. Major areas of research include: 1. Cardiovascular disease: Studies are defining the potential role of differences in cholesterol absorption on cholesterol metabolism, the effects of disease states such as hypertension and chronic renal disease in children on cardiac and vascular function and anatomy. 2. Behavioral Interventions: Studies are exploring the effect of behavioral intervention in enhancing nutrient intake in cystic fibrosis, sickle cell disease, Crohn's Disease and others and assessing the impact of early traumatic brain injury on later neurocognitive development. 3. Cancer: Novel investigations are ongoing evaluating the effect of rapammune and similar compounds for the treatment of angiolipomata associated with tuberous sclerosis and lymphangioleiomyomatosis (LAMS) for whom no effective therapy other than surgery is available. 4. Obesity: Studies are defining the roles of changes in body composition in early life on the development of later obesity, examining racial disparities in body composition which occur during adolescence, and the impact of obesity on sleep disturbances and carbohydrate metabolism. 5. Gene Transfer: Gene transfer trials have been initiated at the GCRC to evaluate whether insertion of a wild type gene into the stem cells of patients with Fanconi Anemia will be safe and ultimately whether it will be efficacious in correcting their underlying bone marrow defects. 6. Diabetes Mellitus: The impact of Type 2 diabetes and obesity on cardiac and vascular structure and function in adolescents is being evaluated. The role of the insulinotropic Gl hormone glucagon-like peptide 1 (GLP-1) in gastric emptying and insulin secretion in normal and Type 2 diabetics is being examined and its role in type 2 diabetes. 7. Inborn errors of bile acid metabolism: With a long track record of research and discovery of multiple enzymatic defects in the bile acid synthetic pathways, investigators at Children's Hospital continue to enhance understanding of these defects affecting infants and children with cholestasis and fat soluble vitamin deficiencies.