11 beta-hydroxylase deficiency in hyperandrogenism.
OBJECTIVE
to determine the 11-deoxycortisol (S) response and incidence of 11 beta-hydroxylase deficiency in hyperandrogenism.


METHODS
Hyperandrogenic women prospectively and consecutively underwent acute adrenal stimulation studies.


METHODS
Tertiary institution.


METHODS
Two hundred sixty women complaining of hirsutism and/or hyperandrogenic oligomenorrhea were studied, excluding five unrelated families (1.9% of total) suffering from 21-hydroxylase deficient late-onset adrenal hyperplasia. Forty-one healthy premenopausal eumenorrheic women served as controls.


METHODS
Only two unrelated women (0.8%) had a poststimulation or net increment S level value greater than or equal to threefold the upper 95th percentile of controls and were presumed to suffer from 11 beta-hydroxylase deficient late-onset adrenal hyperplasia. One hundred nine (42%) of hyperandrogenic women had at least one S value above the 95th percentile of controls. These women also demonstrated higher basal (F0) and stimulated cortisol levels, but a similar increment compared with controls.


RESULTS
Patients with high S measures had higher testosterone, dehydroepiandrosterone sulfate, and androstenedione levels, but similar luteinizing hormone/follicle-stimulating hormone ratios, than hyperandrogenic cohorts with no abnormal S measures. Basal values of S (S0), F0, or S0/F0 were not useful to predict an abnormal S response to stimulation.


CONCLUSIONS
Although adrenocortical hyperactivity was present in 42% of our hyperandrogenic patients, only 0.8% were presumed to suffer from 11 beta-hydroxylase deficient late-onset adrenal hyperplasia. A systemic search for this deficiency in hyperandrogenism is probably unwarranted.
Azziz
R
R
Department of Obstetrics and Gynecology, University of Alabama, Birmingham 35294.
Boots
L R
LR
Parker
C R
CR
Bradley
E
E
Zacur
H A
HA
D000311:Adrenal Glands / Q000473:pathology; D000312:Adrenal Hyperplasia, Congenital; D000328:Adult; D000728:Androgens / Q000378:metabolism*; D003350:Cortodoxone / Q000494:pharmacology; D005260:Female; D006628:Hirsutism / Q000150:complications; D006728:Hormones / Q000097:blood; D006801:Humans; D006965:Hyperplasia; D008599:Menstruation Disturbances / Q000150:complications; D011237:Predictive Value of Tests; D011446:Prospective Studies; D012016:Reference Values
D000728:Androgens; D006728:Hormones; D003350:Cortodoxone