This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.In patients with non classical adrenal hyperplasia (NCAH), the hypothalamic-pituitary-adrenal axis is mildly affected by the disease and only minimally further suppressed by the administration of glucocorticoid medication. It is unknown whether adrenomedullary function is also compromised in these patients. We hypothesize that these patients can mount an appropriate endogenous cortisol response to stress and that their adrenomedullary function is intact. The study purpose is: 1) To examine ACTH and cortisol responses under stress (simulated by CRH administration) in patients with NCAH, 2) To measure plasma total metanephrine, free metanephrine, and epinephrine under similar stress conditions, 3) To extrapolate from these findings as to whether glucocorticoid coverage during periods of stress is necessary for patients with NCAH.
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