11-deoxycortisol increases when ACTH levels are increased such as in Cushing's disease, ACTH producing tumours, or in 11b-hydroxylase deficiency.
Measurement of 11-deoxycortisol is indicated in the differential diagnosis of a rare form of Congenital Adrenal Hyperplasia (CAH) caused by 11b-hydroxylase deficiency. This causes blockade of 11-deoxycortisol to cortisol, and deoxycorticosterone to corticosterone leading to increased ACTH, accumulation of 11-deoxycortisol and deoxycorticosterone, and overproduction of adrenal androgens (testosterone, DHEA and androstenedione).
11-deoxycortisol also provides an index of pituitary function in suspected secondary and tertiary adrenal insufficiency when both cortisol and 11-deoxycortisol measurements are taken as part of metyrapone testing.
A normal response to metyrapone administration is a fall in cortisol levels through blockade of 11b-hyrdroxylase, triggering a rise in pituitary secretion of ACTH. The increase of ACTH triggers a rise in 11-deoxycortisol levels.
Endocrinology Lab
DCOR
Compound S
4 weeks
5303