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Test Code 17PO6 / OHPG-M 17-Hydroxyprogesterone, Serum

Reporting Name

17-Hydroxyprogesterone, S

Useful For

The analysis of 17-hydroxyprogesterone (17-OHPG) is 1 of the 3 analytes along with cortisol and androstenedione, that constitutes the best screening test for congenital adrenal hyperplasia (CAH), caused by either 11- or 21-hydroxylase deficiency.

 

Analysis for 17-OHPG is also useful as part of a battery of tests to evaluate females with hirsutism or infertility; both can result from adult-onset CAH

Testing Algorithm

See Steroid Pathways in Special Instructions.

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Specimen Type

Serum Red


Necessary Information


Patient's age and sex are required.

Specimen Required


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

Specimen Volume: 0.6 mL


Specimen Minimum Volume

0.25 mL

Specimen Stability Information

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

Special Instructions

Reference Values

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 Sept;84(3):701-710

Day(s) Performed

Monday through Friday

Test Classification

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

CPT Code Information

83498

LOINC Code Information

Test ID Test Order Name Order LOINC Value
OHPG 17-Hydroxyprogesterone, S 1668-3

 

Result ID Test Result Name Result LOINC Value
9231 17-Hydroxyprogesterone, S 1668-3

Clinical Information

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 females. Adult-onset CAH may result in hirsutism or infertility in females.

 

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 (FDOC / Deoxycorticosterone [DOC], Serum), respectively, are necessary for diagnosis.

 

CAH21 / Congenital Adrenal Hyperplasia (CAH) Profile for 21-Hydroxylase Deficiency allows the simultaneous determination of OHPG, androstenedione, and cortisol.

 

See Steroid Pathways in Special Instructions.

Cautions

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 (females).

Method Description

17-Hydroxyprogesterone and internal standard are extracted from serum. The extract is quantified using high-performance liquid chromatography-tandem mass spectrometry (LC-MS/MS).(Wudy SA, Hartmann M, Svoboda M: Determination of 17-hydroxyprogesterone in plasma by stable isotope dilution/benchtop liquid chromatography-tandem mass spectrometry. Horm Res. 2000;53[2]:68-71)

Report Available

2 to 5 days
NorDx Test Catalog Note:

From the time that the Specimen is recieved at Mayo

Reject Due To

Gross hemolysis OK
Gross lipemia Reject
Gross icterus OK

Method Name

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

Forms

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