New guidelines for diagnosis and treatment of CAH

In congenital adrenal hyperplasia (CAH), optimal adult height is influenced by the dose of glucocorticoids used, the degree of hormonal control achieved (level of adrenal androgens suppression), the age at which glucocorticoid therapy, associated or not with mineralocorticoids, is instituted and replacement therapy compliance. Nevertheless, the cause of short adult stature in patients with CAH is unknown. We have hypothesized that the inhibition of dehydroepiandrosterone sulfate (DHEAS) induced by glucocorticoid treatment at the time when adrenarche takes place might influence the GH-IGF system and insulin sensitivity. We are proposing that, 1) at the time of adrenarche, adrenal androgens either directly or after conversion to testosterone and/or estradiol in target tissues, could be involved in the regulation of IGF-1 biological response and of insulin sensitivity, 2) DHEAS might be important during pubertal development in the regulation of IGF-1 biological response, and 3) in CAH, low DHEAS associated with high T and A serum levels suggests zona reticularis insufficiency, perhaps secondary to adrenal medulla deficiency. Finally, the alteration in GH-IGF system might contribute, among other factors, to decrease adult height, in CAH girls.