Future Directions in the Study and Management of Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

Figure 1. In a person with normal adrenal function ( ), the adrenal gland produces both cortisol and androgen. The hypothalamic-pituitary-adrenal axis is controlled by negative feedback. In the untreated patient with CAH ( ), a block in cortisol biosynthesis leads to a buildup of cortisol precursors and lack of negative feedback. Corticotropin ( ) is oversecreted, and adrenal hyperplasia occurs. The combination of accumulated cortisol precursors and increased ACTH results in massive androgen production. In the treated patient with CAH ( ), exogenous hydrocortisone replacement reduces androgen production. Supraphysiologic doses of hydrocortisone are often necessary to adequately suppress androgen production. CRH = corticotropin-releasing hormone.
Figure 1. In a person with normal adrenal function ( ), the adrenal gland produces both cortisol and androgen. The hypothalamic-pituitary-adrenal axis is controlled by negative feedback. In the untreated patient with CAH ( ), a block in cortisol biosynthesis leads to a buildup of cortisol precursors and lack of negative feedback. Corticotropin ( ) is oversecreted, and adrenal hyperplasia occurs. The combination of accumulated cortisol precursors and increased ACTH results in massive androgen production. In the treated patient with CAH ( ), exogenous hydrocortisone replacement reduces androgen production. Supraphysiologic doses of hydrocortisone are often necessary to adequately suppress androgen production. CRH = corticotropin-releasing hormone. Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency.leftmiddleACTHright

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  1. Ann Intern Med February 19, 2002 vol. 136 no. 4 320-334
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