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11-Deoxycortisol, 11-Deoxycorticosteroid, Cortodoxone, INN, USAN, BAN, 11-Desoxycortisol, 17-hydroxy-11-deoxycorticosterone, Compound B, Compound S, Corticosterone, Deoxycortisol, Tetrahydro S, Cortoxelone
Diagnostic workup of patients with congenital adrenal hyperplasia
Part of metyrapone testing in the workup of suspected secondary or tertiary adrenal insufficiency
Part of metyrapone testing in the differential diagnostic workup of Cushing syndrome
Indicate if specimen was drawn before or after metyrapone.
Morning (8 a.m.) specimen is preferred.
Ethanol, estrogens (exogenous and pregnancy-related), barbiturates, valproic acid, phenytoin, and exogenous gluco-corticoids may cause impaired response to metyrapone.
There have been occasional reports of Addisonian crisis during 2-day metyrapone testing. For this reason, 2-day metyrapone testing probably should not be performed when plasma cortisol values are less than 3 mcg/dL.
< or =18 years: <344 ng/dL
>18 years: 10-79 ng/dL
In a patient suspected of having congenital adrenal hyperplasia (CAH), elevated serum 11-deoxycortisol levels indicate possible 11 beta-hydroxylase deficiency. However, not all patients will show baseline elevations in serum 11-deoxycortisol levels. 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. The test cannot reliably distinguish between primary and secondary or tertiary causes of adrenal failure, as neither patients with pituitary failure, nor those with primary adrenocortical failure, tend to show an increase of 11-deoxycortisol levels after metyrapone is administered.