What does high 11-deoxycortisol mean?
In a patient suspected of having congenital adrenal hyperplasia (CAH), elevated serum 11-deoxycortisol levels indicate possible 11 beta-hydroxylase deficiency.
What is 11-deoxycortisol test for?
11-Deoxycortisol – 11-Deoxycortisol (Compound S) is useful in diagnosing patients with 11-beta-hydroxylase deficiency (second leading cause of congenital adrenal hyperplasia) and primary (adrenal failure) or secondary (hypothalamic-pituitary ACTH deficiency) adrenal insufficiency.
What is deoxycortisol test?
The overnight metyrapone test is used to assess the functioning of the hypothalamic-pituitary-adrenal (HPA) axis; impairment of any component of the HPA-axis results in a subnormal rise in 11-deoxycortisol levels in response to metapyrone stimulation. The test is used mainly to diagnose secondary adrenal insufficiency.
What is metyrapone test?
The metyrapone stimulation test is based upon the principle that decreasing serum cortisol concentrations normally produces an increase in corticotropin (ACTH) secretion due to a decrease in glucocorticoid negative feedback. The test is performed primarily to detect partial defects in pituitary ACTH secretion.
Does 11 Deoxycortisol have mineralocorticoid activity?
11-Deoxycorticosterone (DOC), or simply deoxycorticosterone, also known as 21-hydroxyprogesterone, as well as desoxycortone (INN), deoxycortone, and cortexone, is a steroid hormone produced by the adrenal gland that possesses mineralocorticoid activity and acts as a precursor to aldosterone.
What does a hydroxylase do?
Hydroxylases are enzymes which add an hydroxyl group to organic compounds. This addition is the first step of aerobic oxidative degradation.
What does a high 11-deoxycortisol level mean?
As a result, the level of 11-deoxycortisol is measured to diagnose impaired cortisol synthesis, to find out the enzyme deficiency that causes impairment along the pathway to cortisol, and to differentiate adrenal disorders.
How is 11-deoxycortisol synthesized?
11-Deoxycortisol is synthesized from 17α-hydroxyprogesterone by 21-hydroxylase and is converted to cortisol by 11β-hydroxylase. 11-Deoxycortisol in mammals has limited biological activity and mainly acts as metabolic intermediate within the glucocorticoid pathway, leading to cortisol.
What causes elevated 11 deoxycortisol levels after metyrapone?
In a significant proportion of cases, increases in 11-deoxycortisol levels are only apparent after adrenocorticotropic hormone (ACTH) (1-24) stimulation. (1) Serum 11-deoxycortisol levels below 1,700 ng/dL 8 hours after metyrapone administration is indicative of probable adrenal insufficiency.