This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Congenital adrenal hyperplasia (CAH) is due to a block in the biosynthesis of cortisol. Typically, hydrocortisone (HC) is the drug of choice for steroid hormone replacement in children with CAH. Other longer acting glucocorticoids, such as prednisone (PDN) and dexamethasone (DEX), are typically not used due to theoretical concerns of toxic effects such as suppression of linear growth and development of Cushingoid features. To date, there is little data known about the comparative pharmacokinetic effects on the hypothalamic-pituitary-adrenal or growth axes of these three glucocorticoids in children with CAH. We propose a comprehensive study that will investigate the pharmacokinetic effects of HC, PDN, and DEX in children with CAH. Each child will be studied initially and then after being on a six-week regimen of each glucocorticoid. As a result of this study, we expect that more physiologic dosing regimens for all three glucocorticoids can be achieved. Furthermore, we anticipate that we will be able to optimize glucocorticoid replacement in children with CAH and be able to avoid under treatment, which can lead to virilization and skeletal advancement, as well as over treatment, which can result in growth suppression.
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