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Testicular adrenal rest tumors.

The most important condition in which the levels of ACTH are elevated is called Congenital adrenal hyperplasia (CAH). Congenital adrenal hyperplasia is an inherited disorder affecting the steroid synthesis (Pic. 2) of the adrenal gland. In more than 90% of the cases, CAH is caused by CYP21 (21-hydroxylase) deficiency. CYP21 deficiency results in an impaired production of cortisol and mostly of aldosterone. Consequently, pituitary ACTH production is increased, leading to hyperplasia (increased size) of the adrenal glands. The impaired production of cortisol and aldosterone may lead to an Addisonian crisis with salt wasting and dehydration in early life.