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

We sought to determine the prevalence of 3beta-hydroxysteroid dehydrogenase-deficient nonclassic adrenal hyperplasia among adult hyperandrogenic women with dehydroepiandrosterone sulfate excess.
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.
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).
3beta-Hydroxysteroid dehydrogenase-deficient nonclassic adrenal hyperplasia is rare even among adult hyperandrogenic patients with adrenal androgen excess.
AuthorsC Moran, H D Potter, R Reyna, L R Boots, R Azziz
JournalAmerican journal of obstetrics and gynecology (Am J Obstet Gynecol) Vol. 181 Issue 3 Pg. 596-600 (Sep 1999) ISSN: 0002-9378 [Print] UNITED STATES
PMID10486469 (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
  • 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)

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