11-Deoxycortisol
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The serum reference ranges of 11-deoxycortisol are as follows [1, 2, 3] :
Persons aged 18 years or younger: < 344 ng/dL
Adults older than 18 years: 10-79 ng/dL
Ranges decrease with age after puberty
Serum reference range of 11-deoxycortisol after a single-dose overnight metapyrone test is >1700 ng/dL
In patients with suspected congenital adrenal hyperplasia (CAH), elevated 11-deoxycortisol levels indicate possible 11-beta-hydroxylase deficiency.
Pharmacological blockade of 11-beta-hydroxylase with metyrapone normally stimulates a rise in 11-deoxycortisol levels. A subnormal rise in 11-deoxycortisol levels after a single-dose overnight metapyrone stimulation test is associated with causes of adrenal insufficiency, including the following:
Primary adrenal insufficiency: Addison disease, adrenal adenoma, CAH
Secondary adrenal insufficiency: Pituitary microadenoma, hypothalamic tumor, pituitary apoplexy, head trauma
Tertiary adrenal insufficiency: Hypothalamic disease with decrease in corticotropin-releasing factor (CRF)
Specifics for collection and panels of 11-deoxycortisol are as follows:
Specimen type: Blood serum
Container: Vacutainer, red top (see image below)
Collection method: Venipuncture
Specimen volume: 0.3 mL (minimum 0.25 mL)
Collection instructions: Morning (eg, 8 AM) collection desirable
Other instructions: For metapyrone test, indicate if drawn before or after metapyrone administration
CAH is a general term for a group of inherited disorders in which a defect in cortisol biosynthesis results in overproduction of adrenocorticotropic hormone (ACTH), with secondary adrenal hyperplasia as a consequence. An enzymatic defect in 11-beta-hydroxylase, which converts 11-deoxycortisol to cortisol, is the second most common variant of CAH and accounts for approximately 5%-8% of cases. Patients present with features of androgen excess, such as ambiguous genitalia in female newborns and premature sexual development in male children. With the severe (classic) form of 11-beta-hydroxylase deficiency, hypertension develops in approximately two thirds of patients, often in the first few years of life.
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.
Indications for testing of serum 11-deoxycortisol include the following:
Diagnostic workup of CAH for 11-beta-hydroxylase deficiency
Part of metapyrone testing to evaluate the HPA-axis in the workup of suspected secondary or tertiary adrenal insufficiency
For the overnight metapyrone stimulation test, the patient is administered oral metapyrone at a weight-based dosage (30 mg /kg). Blood samples are collected before the time of dosage (eg, at midnight) and 8 hours after dosage (eg, at 8 AM).
Burris CA, Ashwood ER, Burns DE. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. 4th ed. St. Louis: Elsevier Saunders; 2006.
McPherson RA, Pincus MR. Henry’s Clinical Diagnosis and Management by Laboratory Methods. 22nd ed. Philadelphia: Elsevier Saunders; 2011.
Williamson MA, Snyder LM, eds. Wallach’s Interpretation of Diagnostic Tests. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2011.
Rajaa Almourani, MD Resident Physician, Department of Internal Medicine, St Louis University Hospital
Rajaa Almourani, MD is a member of the following medical societies: National Arab American Medical Association, Syrian American Medical Society
Disclosure: Nothing to disclose.
Thomas M Wheeler, MD Chairman, Department of Pathology and Immunology, WL Moody, Jr, Professor of Pathology, Professor of Urology, Baylor College of Medicine
Thomas M Wheeler, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American Medical Association, American Society for Clinical Pathology, American Society of Cytopathology, American Thyroid Association, American Urological Association, College of American Pathologists, United States and Canadian Academy of Pathology, International Society of Urological Pathology, Harris County Medical Society
Disclosure: Received stock from PathXL for medical advisory board. for: PathXL, Inc.
Judy Lin, MD
Disclosure: Nothing to disclose.
11-Deoxycortisol
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