Test Catalog

Test Id : OHPG

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17-Hydroxyprogesterone, Serum

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Overview

Test Catalog

Useful For
Suggests clinical disorders or settings where the test may be helpful

Screening test for congenital adrenal hyperplasia (CAH), caused by either 11- or 21-hydroxylase deficiency, when used in combination with testing for cortisol and androstenedione

 

As part of a battery of tests to evaluate women with hirsutism or infertility

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

For more information see Steroid Pathways

Method Name
A short description of the method used to perform the test

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

NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.

Yes

Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test

17-Hydroxyprogesterone, S

Aliases
Lists additional common names for a test, as an aid in searching

17 Alphahydroxyprogesterone

17 Hydroxy Progesterone, Serum

Hydroxyprogesterone

Progesterone, 17-Hydroxy

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

For more information see Steroid Pathways

Specimen Type
Describes the specimen type validated for testing

Serum Red

Ordering Guidance

The preferred screening test for congenital adrenal hyperplasia caused by 21-hydroxylase deficiency is CAH21 / Congenital Adrenal Hyperplasia (CAH) Profile for 21-Hydroxylase Deficiency, Serum, which allows the simultaneous determination of 17-hydroxyprogesterone, androstenedione, and cortisol.

Necessary Information

Patient's age and sex are required.

Specimen Required
Defines the optimal specimen required to perform the test and the preferred volume to complete testing

Collection Container/Tube: Red top (serum gel/SST are not acceptable)

Submission Container/Tube: Plastic vial

Specimen Volume: 0.6 mL

Collection Instructions: Centrifuge and aliquot serum into plastic vial.

Special Instructions
Library of PDFs including pertinent information and forms related to the test

Forms

If not ordering electronically, complete, print, and send General Test Request (T239) with the specimen.

Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the Testing Laboratory

0.25 mL

Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected

Gross hemolysis OK
Gross lipemia Reject
Gross icterus OK

Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included

Specimen Type Temperature Time Special Container
Serum Red Refrigerated (preferred) 28 days
Frozen 28 days
Ambient 7 days

Useful For
Suggests clinical disorders or settings where the test may be helpful

Screening test for congenital adrenal hyperplasia (CAH), caused by either 11- or 21-hydroxylase deficiency, when used in combination with testing for cortisol and androstenedione

 

As part of a battery of tests to evaluate women with hirsutism or infertility

Testing Algorithm
Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

For more information see Steroid Pathways

Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test

Congenital adrenal hyperplasia (CAH) is caused by inherited defects in steroid biosynthesis. The resulting hormone imbalances with reduced glucocorticoids and mineralocorticoids and elevated 17-hydroxyprogesterone (OHPG) and androgens can lead to life-threatening, salt-wasting crisis in the newborn period and incorrect gender assignment of virtualized female patients. Adult-onset CAH may result in hirsutism or infertility in women.

 

The adrenal glands, ovaries, testes, and placenta produce OHPG. It is hydroxylated at the 11 and 21 position to produce cortisol. Deficiency of either 11- or 21-hydroxylase results in decreased cortisol synthesis, and feedback inhibition of adrenocorticotropic hormone (ACTH) secretion is lost. Consequent increased pituitary release of ACTH increases production of OHPG. But, if 17-alpha-hydroxylase (which allows formation of OHPG from progesterone) or 3-beta-hydroxysteroid dehydrogenase type 2 (which allows formation of 17-hydroxyprogesterone formation from 17-hydroxypregnenolone) are deficient, OHPG levels are low with possible increase in progesterone or pregnenolone respectively.

 

OHPG is bound to both corticosteroid binding globulin and albumin, and total OHPG is measured in this assay.

OHPG is converted to pregnanetriol, which is conjugated and excreted in the urine. In all instances, more specific tests are available to diagnose disorders or steroid metabolism than pregnanetriol measurement.

 

Most (90%) cases of CAH are due to variants in the steroid 21-hydroxylase gene (CYP21A2). CAH due to 21-hydroxylase deficiency is diagnosed by confirming elevations of OHPG and androstenedione (ANST / Androstenedione, Serum) with decreased cortisol (CINP / Cortisol, Mass Spectrometry, Serum). By contrast, in 2 less common forms of CAH, due to 17-hydroxylase or 11-hydroxylase deficiency, OHPG and androstenedione levels are not significantly elevated and measurement of progesterone (PGSN / Progesterone, Serum) and deoxycorticosterone (DOCS / 11-Deoxycorticosterone, Serum), respectively, are necessary for diagnosis.

 

For more information see Steroid Pathways

Reference Values
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.

Children:

Preterm infants

Preterm infants may exceed 630 ng/dL, however, it is uncommon to see levels reach 1,000 ng/dL.

Term infants

0-28 days: <630 ng/dL

Levels fall from newborn (<630 ng/dL) to prepubertal gradually within 6 months.

Prepubertal males: <110 ng/dL

Prepubertal females: <100 ng/dL 

 

Adults:

Males: <220 ng/dL

Females

Follicular: <80 ng/dL

Luteal: <285 ng/dL

Postmenopausal: <51 ng/dL

 

Note: For pregnancy reference ranges, see: Soldin OP, Guo T, Weiderpass E, et al. Steroid hormone levels in pregnancy and 1 year postpartum using isotope dilution tandem mass spectrometry. Fertil Steril. 2005;84(3):701-710

Interpretation
Provides information to assist in interpretation of the test results

Diagnosis and differential diagnosis of congenital adrenal hyperplasia (CAH) always requires the measurement of several steroids. Patients with CAH due to steroid 21-hydroxylase gene (CYP21A2) variants usually have very high levels of androstenedione, often 5- to 10-fold elevations. 17-hydroxyprogesterone (OHPG) levels are usually even higher, while cortisol levels are low or undetectable. All 3 analytes should be tested.

 

In the much less common CYP11A1 variant, androstenedione levels are elevated to a similar extent as in CYP21A2 variant, and cortisol is also low, but OHPG is only mildly, if at all, elevated.

 

In the also very rare 17-alpha-hydroxylase deficiency, androstenedione, all other androgen-precursors (17-alpha-hydroxypregnenolone, OHPG, dehydroepiandrosterone sulfate), androgens (testosterone, estrone, estradiol), and cortisol are low, while production of mineral corticoid and its precursors, in particular progesterone, 11-deoxycorticosterone, and 18-hydroxycorticosterone, are increased.

 

The goal of CAH treatment is normalization of cortisol levels and, ideally, also of sex-steroid levels. Traditionally, OHPG and urinary pregnanetriol or total ketosteroid excretion are measured to guide treatment, but these tests correlate only modestly with androgen levels. Therefore, androstenedione and testosterone should also be measured and used to guide treatment modifications. Normal prepubertal levels may be difficult to achieve, but if testosterone levels are within the reference range, androstenedione levels of up to 100 ng/dL are usually regarded as acceptable.

 

For more information see Steroid Pathways .

Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances

At birth the hypothalamic-pituitary-adrenal axis and the hypothalamic-pituitary-gonadal axis are activated, and adrenal and sex steroid levels are high. In preterm infants, the elevations can be even more pronounced due to illness and stress. As a result, preterm infants may occasionally have 17-hydroxyprogesterone levels of up to 1000 ng/dL. Term infants (0-28 days) will have levels less than 630 ng/dL. These then fall over the following 1 to 6 months to prepubertal levels of less than 110 ng/dL (males) and less than 100 ng/dL (female patients).

Clinical Reference
Recommendations for in-depth reading of a clinical nature

1. Von Schnakenburg K, Bidlingmaier F, Knorr D. 17-hydroxyprogesterone, androstenedione, and testosterone in normal children and in prepubertal patients with congenital adrenal hyperplasia. Eur J Pediatr. 1980;133(3):259-267

2. Sciarra F, Tosti-Croce C, Toscano V. Androgen-secreting adrenal tumors. Minerva Endocrinol. 1995;20(1):63-68

3. Collett-Solberg P. Congenital adrenal hyperplasia: from genetics and biochemistry to clinical practice, part I. Clin Pediatr. 2001;40(1):1-16

4. Speiser PW, Azziz R, Baskin LS, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2010;95(9):4133-4160

5. Nordenstrom A, Falhammar H. Management of Endocrine Disease: Diagnosis and management of the patient with non-classic CAH due to 21-hydroxylase deficiency. Eur J Endocrinol. 2019;180(3):R127-R14

6. Young WF Jr. Primary aldosteronism: A common and curable form of hypertension. Cardiol Rev. 1999;7(4):207-214

7. Young WF Jr. Pheochromocytoma and primary aldosteronism: diagnostic approaches. Endocrinol Metab Clin North Am. 1997;26(4):801-827

8. Wudy SA, Hartmann M, Svoboda M. Determination of 17-hydroxypregnenolone in plasma by stabile isotope dilution/benchtop liquid chromatography-tandem mass spectrometry. Horm Res. 2000;53(2):68-71

9. Therrell BL. Newborn screening for congenital adrenal hyperplasia Endocrinol Metab Clin North Am. 2001;30(1):15-30

10. Bachega TA, Billerbeck AE, Marcondes JA, Madureira G, Arnhold IJ, Mendonca BB. Influence of different genotypes on 17-hydroxyprogesterone levels in patients with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Clin Endocrinol. 2000;52(5):601-607

11. Kao P, Machacek DA, Magera MJ, Lacey JM, Rinaldo P. Diagnosis of adrenal cortical dysfunction by liquid chromatography-tandem mass spectrometry. Ann Clin Lab Sci. 2001;31(2):199-204

12. Young WF Jr. Management approaches to adrenal incidentalomas-a view from Rochester, Minnesota. Endocrinol Metab Clin North Am. 2000;29(1):159-185

13. Ibanez L, DiMartino-Nardi J, Potau N, Saenger P. Premature adrenarche-normal variant or forerunner of adult disease? Endocr Rev. 2000;21(6):671-696

14. Allolio B, Arlt W. DHEA treatment: myth or reality? Trends Endocrinol Metab 2002;13(7):288-294

15. Lin CL, Wu TJ, Machacek DA, Jiang NS, Kao PC. Urinary free cortisol and cortisone determined by high-performance liquid chromatography in the diagnosis of Cushing's syndrome. J Clin Endocrinol Metab. 1997;82(1):151-155

16. Findling JW, Raff H. Diagnosis and differential diagnosis of Cushing's syndrome. Endocrinol Metab Clin North Am 2001;30(3):729-747

17. Buchman Al. Side effects of corticosteroid therapy. J Clin Gastroenterol. 2001;33(4):289-297

18. Dodds HM, Taylor PJ, Cannell GR, Pond SM. A high-performance liquid chromatography-electrospray-tandem mass spectrometry analysis of cortisol and metabolites in placental perfusate. Anal Biochem. 1997;247(2):342-347

19. Cengiz H, Demirci T, Varim C, Cetin S. Establishing a new screening 17 hydroxyprogesterone cut-off value and evaluation of the reliability of the long intramuscular ACTH stimulation test in the diagnosis of nonclassical congenital adrenal hyperplasia. Eur Rev Med Pharmacol Sci. 2021;25(16):5235-5240. doi:10.26355/eurrev_202108_26537

Method Description
Describes how the test is performed and provides a method-specific reference

Deuterated stable isotopes (d4-cortisol, d7-androstenedione, d8 17-hydroxyprogesterone) are added to the serum sample as internal standards. Cortisol, androstenedione, 17-hydroxyprogesterone, and the internal standards are extracted from specimens online using a guard cartridge. The analytes are transferred online to an analytical column and are analyzed by liquid chromatography tandem mass spectrometry.(Unpublished Mayo method)

PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information

No

Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.

Monday through Friday

Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.

2 to 5 days

Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded

14 days

Performing Laboratory Location
Indicates the location of the laboratory that performs the test

Rochester

Fees
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Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.

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
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.

CPT codes are provided by the performing laboratory.

83498

LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.

Test Id Test Order Name Order LOINC Value
OHPG 17-Hydroxyprogesterone, S 1668-3
Result Id Test Result Name Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
9231 17-Hydroxyprogesterone, S 1668-3

Test Setup Resources

Setup Files
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Excel | PHP Pdf | CMS Pdf

Sample Reports
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Normal Reports | Abnormal Reports

SI Sample Reports
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SI Normal Reports | SI Abnormal Reports