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Background
11-deoxycortisol is a steroid hormone. It can be produced in the adrenal glands. It is an intermediate in the synthesis of cortisol. 17-hydroxyprogesterone is converted to 11-deoxycortisol by the enzymes of 11β-hydroxylase (CYP11B1). The 11-deoxycortisol converts into cortisol. If there is an inhibition in the activity of CYP11B1 enzyme, 11-deoxycortisol accumulates. Cortisol can not be produced. This can cause CAH (congenital adrenal hyperplasia). CYP11B1 enzyme is a key marker to diagnose this disease.
CAH is a group of inherited disease. It can cause due to defect in the biosynthesis of cortisol which can lead to overproduction of ACTH (adrenocorticotropic hormone). It can cause secondary adrenal hyperplasia. About 5 to 8 % of the CAH cases can occur because of the defect in CYP11B1 enzyme. Patients who have excess androgen, like ambiguous genitalia in newborns female and premature development of sexual organs in male children. In severe deficiency of CYP11B1, hypertension may develop in about 2/3 of patients in the 1st few years.
The metyrapone test is used to check the function of HPA axis. Any abnormality in the HPA axis may lead to abnormal increased levels of 11-deoxycortisol in the response of stimulation of metyrapone. This test is used to diagnose the secondary impairment of adrenal. The patient is administrated 30 mg/kg oral metyrapone to test the stimulation of metyrapone. Before the dosage time and after the 8 hours of the dosage, blood samples are taken.
As per the study of London, statins can decrease the level of specific precursors which are found in the pathway of steroidogenic like 11-deoxyxortisol. It can lead to patients who have subclinical impairment of adrenal. After a single dosage of pitavastatin in 24 hours, patient has showed a reduced levels in corticosterone, cortisone, and 11-deoxycortsiol.
Indications/Applications
Indications to perform the 11-deoxycortisol test are:
To diagnose CAH because of the deficiency of 11-β-hydrolase: This disease can lead to increased production of androgen. Increased levels of 11-deoxycortisol confirm the disease.
metyrapone test to check the function of HPA axis for secondary adrenal impairment of tertiary adrenal impairment: It is a provocative test. It is used to check adrenal impairment by inhibiting the 11- β-hydrolase. It blocks the production of cortisol and cause accumulation of 11-deoxycortisol.
To evaluate adrenal tumor and Cushing’s syndrome: Elevated levels of 11-deoxycortisol is because of dysregulation of steroidogenesis.
Symptoms to perform 11-deoxycorticosol test are weight loss, dehydration, nausea, diarrhea, and vomiting.
Reference Range
The normal range of 11-deoxycortisol is:
Children below 18 years: Less than 344 ng/dL
Adults above 18 years: 10 to 79 ng/dL
The level is reduced with age.
Normal range of serum, 11-deoxycortisol is less than 1700 ng/dL after 1 dosage overnight metyrapone test.
Interpretation
Patients who are suspected of CAH, increased levels of 11-deoxycortisol indicate the deficiency of 11-beta hydroxylase. Patients may have symptoms of deficiency of cortisol, excess androgen, and hypertension because of excessive deoxycorticosterone.
Blockage of 11-β-hydroxylase along with metyrapone stimulates the increased levels of 11-deoxycortisol. An abnormal increased levels of 11-deoxycortisol after 1 dosage of metyrapone for overnight stimulation test is linked with adrenal insufficiency like:
Primary adrenal insufficiency: Adrenal adenoma, Addison disease, CAH
Secondary adrenal insufficiency: Hypothalamic tumor, pituitary microadenoma, head trauma, pituitary apoplexy
Tertiary adrenal unstuffiness: Hypothalamic diseases with reduced level of CRF corticotropin-releasing factor
Adrenal tumor: Adrenal tumor or adenomas can lead to over production of 11-deoxycortisol specifically in diseases which are linked with overproduction of androgens or cortisol.
Cushing’s syndrome: 11-deoxycortisol levels are increased in the ACTH-dependent Cushing’s syndrome. Overactive response of steroidogenic increased the levels of ACTH.
Collection And Panels
Sample type: Blood serum
Sample collection container: Red top tube, vacutainer
Sample collection method: Venipuncture
Sample volume: 0.3 mL
Sample minimum volume: 0.25 mL
Sample collection instruction: Collect the sample in morning, like 8 AM
Indicate the sample before and after the administration of metyrapone for the metyrapone test
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