17-α-Hydroxyprogesterone
| 17-α-Hydroxyprogesterone | | | |
| Number | | 004713 |
| CPT | | 83498 |
| Related Information | | Adrenocorticotropic Hormone (ACTH), Plasma |
| Synonyms | | 17-OHP |
| Special Instructions | | See the Endocrine Appendix for instructions on multiple specimen testing. |
| Specimen | | Serum or plasma |
| Volume | | 0.3 mL |
| Minimum Volume | | 0.2 mL (Note: This volume does not allow for repeat testing.) |
| Container | | Red-top tube, gel-barrier tube, or lavender-top (EDTA) tube |
| Collection | | If tube other than a gel-barrier tube is used, transfer separated serum or plasma to a plastic transport tube. |
| Storage Instructions | | Refrigerate |
Reference Interval | | See table.1,2
| Age | Male
(ng/dL) | Female
(ng/dL) | | 0-60 days | 0-440 | 0-440 | | 2 mo - 10 y | 0-120 | 0-120 | | 11-16 y | 0-240 | 0-240 | | Adults | 5-160 | | | Follicular | | 30-100 | | Luteal | | 20-290 | | Pregnant | | 40-
1540 |
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| Use | | Markedly elevated in patients with congenital adrenal hyperplasia (adrenogenital syndrome) due to 21-hydroxylase deficiency; evaluate hirsutism and/or infertility; assess certain adrenal or ovarian tumors with endocrine activity |
| Methodology | | Enzyme immunoassay (EIA) |
| Additional Information | | 17-OH-progesterone is the substrate for subsequent 21- and 11-hydroxylation to produce cortisol. The two critical enzymes, 21-hydroxylase and 11-beta-hydroxylase, participate in cortisol generation. If hydroxylation, at either position, cannot take place because of enzyme deficiency, cortisol synthesis decreases, accompanied by increased ACTH. Congenital adrenal hyperplasia and adrenogenital syndrome result from lack of normal glucocorticoids and buildup of precursors (mostly virilizing). Lack of 21-hydroxylase is the most common cause of adrenogenital syndrome. Congenital adrenal hyperplasia caused by 21-hydroxylase deficiency is the most common cause of female hermaphroditism.3 It is an autosomal recessive disorder. Basal 17-hydroxyprogesterone levels can be normal in late-onset 21-hydroxylase deficiency presenting as hirsutism. Such patients are described as having dramatically increased 17-hydroxyprogesterone response to ACTH.4 Patients with 21-hydroxylase deficiency have increased 17-ketosteroids, urine pregnanetriol, as well as high 17-hydroxyprogesterone. Prenatal diagnosis of congenital adrenal hyperplasia is possible by HLA typing, by DNA analysis, or by hormone measurements from amniotic fluid, including 17-hydroxyprogesterone.3 Some nonspecificity is seen when amniotic fluid analysis is used.5 Congenital adrenal hyperplasia with adult onset is among the causes of hirsutism and/or infertility. |
| Footnotes | | - Soldin SJ, Bailey J, Beatey J, et al, “Pediatric Reference Ranges for 17 Alpha-Hydroxy Progesterone,” Clin Chem, 1995, 41:S92.
- Tietz NW, ed, Clinical Guide to Laboratory Tests, 3rd ed, Philadelphia, PA: WB Saunders Co, 1995, 348.
- Pang SI, Pollack MS, Marshall RN, et al, “Prenatal Treatment of Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency,” N Engl J Med, 1990, 322(2):111-5.
- Chrousos GP, Loriaux DL, Mann DL, et al, “Late-Onset 21-Hydroxylase Deficiency Mimicking Idiopathic Hirsutism or Polycystic Ovarian Disease,” Ann Intern Med, 1982, 96(2):143-8.
- Lee A and Ellis G, “Serum 17-Alpha-Hydroxyprogesterone in Infants and Children as Measured by a Direct Radioimmunoassay Kit,” Clin Biochem, 1991, 24(6):505-11
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| References | | Check JH, Vaze MM, Epstein R, et al, “17-Hydroxyprogesterone Level as a Marker for Corpus Luteum Function in Aborters Versus Nonaborters,” Int J Fertil, 1990, 35(2):112-5. DiGeorge AM, “Adrenogenital Syndrome,” Nelson Textbook of Pediatrics, 13th ed, Behrman RE, Vaughn VI, and Nelson WE, eds, Philadelphia, PA: WB Saunders Co, 1987, 1220-4. Loriaux DL, “Hirsutism,” Cecil Textbook of Medicine, 18th ed, Volume 2, Wyngaarden JB and Smith LH Jr, eds, Philadelphia, PA: WB Saunders Co, 1988, 1446-8. New MI, “Clinical and Endocrinological Aspects of 21-Hydroxylase Deficiency,” Ann N Y Acad Sci, 1985, 458:1-27. Pang S, Pollack MS, Loo M, et al, “Pitfalls of Perinatal Diagnosis of 21-Hydroxylase Deficiency Congenital Adrenal Hyperplasia,” Ann N Y Acad Sci, 1985, 458:111-29. Robboy SJ, Lombardo JM, and Welch WR, “Disorders of Abnormal Sexual Development,” Blaustein's Pathology of the Female Genital Tract, 3rd ed, Kurman RJ, ed, New York, NY: Springer-Verlag, 1987, 15-35. |
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