Continued support is requested for the Pediatric Clinical Research Center for both inpatient and outpatient investigation. This Center will provide facilities and partial financing for ongoing studies of a variety of illnesses and disorders of significance not only during childhood but also relating to lifetme health and quality of life. Major investigational efforts involve endocrine studies in normal and pathologic states; investigations of metabolic pathways in a variety of genetically determined and acquired metabolic disorders; a survey of hypertensive disorders in young children and adolescence in order to define etiologic factors and to develop remedial approaches including dietary alternatives and behavior modification; a study of the toxic effects of lead and trace metals in children and the development of procedures to detect, define and treat these disorders; studies on the dietary control of hyperammonemia and hyperaminoacidemia states utilizing ketoanalogues of essential amino acids; definitive evaluation of hyperlipidemic states in order to define matabolic causes and to develop appropriate therapeutic maneuvers and investigations to determine the biological significance of hemoglobin F synthesis in a variety of hematologic disorders and the possible therapeutic implications in sickle cell disease.
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