11-deoxycortisol


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11-deoxycortisol

Compound S An adrenocortical steroid marker for adrenal 11-β-hydroxylase deficiency ↑ in Congenital adrenal hyperplasia ↓ in Adrenal insufficiency or hypoplasia. See Metyrapone test.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
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The screening protocol included one sample two-tier testing with measurement of 17 [alpha]-hydroxyprogesterone (17-OHP) the most abundant substrate for the CYP21 enzyme by fluoroimmunoassay in the first step in dried blood spots obtained on the 3-5th days of life followed by steroid profiling in the same dried blood spots using liquid chromatography-tandem mass spectrometry (LC-MS) method to measure 17-OHP, 21-deoxycortisol, cortisol, 11-deoxycortisol and androstenedione as a secondtier test in those with positive initial screening.
An adequate response was defined by a rise of ACTH from baseline to >106 pg/mL (23.3 pmol/L)[3] and an 11-deoxycortisol (11DOC) rise to >208 nmol/L.
Therefore, it was perhaps unsurprising that intermediates such as 11-deoxycortisol, 17-hydroxypregenenolone, and pregnenolone provided discrimination of adrenocortical carcinoma from other adrenal lesions using serum (1).
Lack of increase in ACTH and 11-deoxycortisol levels after metyrapone administration is diagnostic of ACTH deficiency.
Pretorius, "Measurement of cortisol, cortisone, prednisolone, dexamethasone and 11-deoxycortisol with ultra high performance liquid chromatography-tandem mass spectrometry: application for plasma, plasma ultrafiltrate, urine and saliva in a routine laboratory," Journal of Chromatography B, vol.
Levels of 11-deoxycortisol and cortisone rose but did not achieve statistical significance.
On 6/18/13, her unstimulated serum 11-deoxycortisol concentration was impressively elevated at 91ng/dL (<37ng/dL) consistent with a nonclassic 11-hydroxylase deficiency.
One of the enzymes affected is 11-beta-hydroxylase , which is responsible for the conversion of 11-desoxycorticosterone to corticosterone (both hormones possessing mineralocorticoid activity and being precursors to aldosterone) and 11-deoxycortisol to cortisol (10).
The exact diagnosis of 11-[beta]-hydroxylase deficiency can be performed by the high basal levels of deoxycorticosterone and or 11-deoxycortisol serums or tetrahydrometabolites, which might be found during a 24-hour urine test.
DISCUSSION: Defective conversion of 17-hydroxyprogesterone to 11-deoxycortisol accounts for more than 95% of cases of congenital adrenal hyperplasia.