Different dysregulations in adrenal steroid biosynthesis as a prevalent cause of hyperandrogenism in women from southern Italy.
OBJECTIVE
To investigate the presence of a dysregulation in steroid biosynthesis in women from southern Italy.


METHODS
Controlled clinical study.


METHODS
Normal and hyperandrogenic women referred to the Endocrinology Unit of Federico II University Medical School of Naples.


METHODS
One hundred fifty untreated young hyperandrogenic women and 50 normal age-matched women.


METHODS
Morning (basal) blood samples obtained in the early follicular phase and after a long (360 minute) ACTH stimulation test.


METHODS
The adrenal maximal response was calculated as stimulus under curve areas (AUCa), and all steroids were assayed using RIA methods.


RESULTS
A dysregulation of 21-hydroxylase was found in 22 patients (14.7%), with a prevalent increase of 17 alpha-hydroxyprogesterone AUC, whereas in 9 hirsute women (6%), there was a prevalent significant increase in 11-deoxycortisol AUC. In 5 women (3.3%), DHEA and DHEAS basal and AUCs plasma levels were increased, suggesting an impaired 3 beta-olo-dehydrogenase activity. The remaining 114 hyperandrogenic women (76%) compose the nonadrenal group, with a probable diagnosis of primitive functional ovarian hyperandrogenism.


CONCLUSIONS
Considering the high prevalence of hirsutism and oligomenorrhea in our female hyperandrogenic population, we suggest an adrenal hyperresponsiveness likely due to a dysregulation in enzymes related to androgen adrenal steroidogenesis.
Valentino
R
R
University Federico II. Medical School, Naples, Italy.
Tommaselli
A P
AP
Savastano
S
S
Dorato
M
M
Rossi
R
R
Lombardi
G
G
D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000096:biosynthesis* / Q000097:blood; D000324:Adrenocorticotropic Hormone; D000328:Adult; D003350:Cortodoxone / Q000097:blood; D003687:Dehydroepiandrosterone / Q000097:blood; D019314:Dehydroepiandrosterone Sulfate / Q000097:blood; D003900:Desoxycorticosterone / Q000097:blood; D005260:Female; D006628:Hirsutism / Q000097:blood; D006706:Homeostasis; D006801:Humans; D017588:Hyperandrogenism / Q000209:etiology*; D007558:Italy; D011085:Polycystic Ovary Syndrome / Q000097:blood; D013255:Steroid 21-Hydroxylase / Q000378:metabolism*
D000305:Adrenal Cortex Hormones; D003900:Desoxycorticosterone; D003687:Dehydroepiandrosterone; D019314:Dehydroepiandrosterone Sulfate; D019326:17-alpha-Hydroxyprogesterone; D000324:Adrenocorticotropic Hormone; D013255:Steroid 21-Hydroxylase; D003350:Cortodoxone