Prevalence of 3beta-hydroxysteroid dehydrogenase-deficient nonclassic adrenal hyperplasia in hyperandrogenic women with adrenal androgen excess.

Abstract

OBJECTIVE:

We sought to determine the prevalence of 3beta-hydroxysteroid dehydrogenase-deficient nonclassic adrenal hyperplasia among adult hyperandrogenic women with dehydroepiandrosterone sulfate excess.

STUDY DESIGN:

Thirty consecutive hyperandrogenic women with hirsutism, oligomenorrhea, or both and dehydroepiandrosterone sulfate levels of >8.5 micromol/L and 24 control subjects were studied. Basal sex hormone binding globulin, total and free testosterone, dehydroepiandrosterone sulfate, 17-hydroxyprogesterone, and basal and 60-minute corticotropin-stimulated 17-hydroxypregnenolone and dehydroepiandrosterone were measured, and the increment (change from basal to 60-minute value) was calculated.

RESULTS:

Twenty-six (87%) and 25 (83%) of the 30 hyperandrogenic patients studied had 60-minute dehydroepiandrosterone and change in 0- to 60-minute dehydroepiandrosterone levels greater than the mean + 2 SD of control subjects, respectively. Six (20%) and 6 (20%) of the 30 hyperandrogenic patients had 60-minute 17-hydroxypregnenolone and 0- to 60-minute change in 17-hydroxypregnenolone levels greater than the mean + 2 SD of control subjects, respectively. However, none of the subjects had either 60-minute 17-hydroxypregnenolone levels or 60-minute dehydroepiandrosterone levels or both associated with the diagnosis of genetically proved 3beta-hydroxysteroid dehydrogenase deficiency (>5-fold of the control mean value).

CONCLUSION:

3beta-Hydroxysteroid dehydrogenase-deficient nonclassic adrenal hyperplasia is rare even among adult hyperandrogenic patients with adrenal androgen excess.

• Authors: C Moran, H D Potter, R Reyna, L R Boots, R Azziz
• Journal: American journal of obstetrics and gynecology (Am J Obstet Gynecol) Vol. 181 Issue 3 Pg. 596-600 (Sep 1999) ISSN: 0002-9378 [Print] United States
• PMID: 10486469 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Testosterone
• Dehydroepiandrosterone
• Dehydroepiandrosterone Sulfate
• 17-alpha-Hydroxyprogesterone
• Adrenocorticotropic Hormone
• 3-Hydroxysteroid Dehydrogenases

Topics

• 17-alpha-Hydroxyprogesterone (blood)
• 3-Hydroxysteroid Dehydrogenases (deficiency)
• Adolescent
• Adrenal Hyperplasia, Congenital (blood, complications, enzymology)
• Adrenocorticotropic Hormone
• Adult
• Dehydroepiandrosterone (blood)
• Dehydroepiandrosterone Sulfate (blood)
• Female
• Humans
• Hyperandrogenism (blood, complications, enzymology)
• Kinetics
• Testosterone (blood)