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Test Code DCORT 11-Deoxycortisol, Serum

Useful For

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

Testing Algorithm

For more information see Steroid Pathways.

Special Instructions

Method Name

Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)

Reporting Name

11-Deoxycortisol, S

Specimen Type

Serum


Necessary Information


Indicate if specimen was collection before or after metyrapone administration.



Specimen Required


Supplies: Sarstedt Aliquot Tube, 5 mL (T914)

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 0.5 mL

Collection Instructions:

1. Morning (8 a.m.) specimen is preferred.

2. Centrifuge and aliquot serum into a plastic vial.


Specimen Minimum Volume

0.4 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 28 days
  Ambient  28 days
  Frozen  28 days

Reference Values

≤18 years: <344 ng/dL

>18 years: 10-79 ng/dL

 

For International System of Units (SI) conversion for Reference Values, see www.mayocliniclabs.com/order-tests/si-unit-conversion.html.

Interpretation

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 corticotropin (previously adrenocorticotropic hormone)(1-24) stimulation.(1)

 

Serum 11-deoxycortisol levels below 1700 ng/dL when measured 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.

 

For more information see Steroid Pathways.

Clinical Reference

1. Tonetto-Fernandes V, Lemos-Marini SH, Kuperman H, et al. Serum 21-deoxycortisol, 17-hydroxyprogesterone, and 11-deoxycortisol in classic congenital adrenal hyperplasia: clinical and hormonal correlations and identification of patients with 11 beta-hydroxylase deficiency among a large group with alleged 21-hydroxylase deficiency. J Clin Endocrinol Metab. 2006;91(6):2179-2184

2. Lashanske G, Sainger P, Fishman K, et al. Normative data for adrenal steroidogenesis in a healthy pediatric population: age- and sex-related changes after adrenocorticotropin stimulation. J Clin Endocrinol Metab. 1991;73(3):674-686

3. Holst JP, Soldin SJ, Tractenberg RE, et al. Use of steroid profiles in determining the cause of adrenal insufficiency. Steroids. 2007;72(1):71-84

4. Berneis K, Staub JJ, Gessler A, et al. Combined stimulation of adrenocorticotropin and compound-S by single dose metyrapone test as an outpatient procedure to assess hypothalamic-pituitary-adrenal function. J Clin Endocrinol Metab. 2002;87(12):5470-5475

5. Idkowiak, J, Cragun, D, Hopkin RJ, Arlt W. Cytochrome P450 oxidoreductase deficiency. In: Adam MP, Feldman J, Mirzaa GM, et al, eds. Gene Reviews [Internet]. University of Washington, Seattle; 2005. Updated August 3, 2017. Accessed May 2, 2024. Available at www.ncbi.nlm.nih.gov/sites/books/NBK1419/

6. Held PK, Bird IM, Heather NL. Newborn screening for congenital adrenal hyperplasia: review of factors affecting screening accuracy. Int J Neonatal Screen. 2020;6(3):67. doi:10.3390/ijns6030067

Day(s) Performed

Tuesday

Report Available

3 to 10 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information

82634

LOINC Code Information

Test ID Test Order Name Order LOINC Value
DCORT 11-Deoxycortisol, S 1657-6

 

Result ID Test Result Name Result LOINC Value
46923 11-Deoxycortisol, S 1657-6