The prevalence of non-classic adrenal hyperplasia due to 11 beta-hydroxylase deficiency among hirsute women in a Turkish population.
OBJECTIVE
The present study was designed to determine the prevalence of 11 beta-hydroxylase deficiency in adult women with hirsutism in a Turkish population.


METHODS
One hundred and twenty-four consecutive unselected hirsute patients were studied. An ACTH stimulation test was performed in the midfollicular phase of the cycle on the patients and 20 age-matched controls by administration of a single bolus of 0.25 mg ACTH (1-24) at 0900 h.


METHODS
Serum 11-deoxycortisol levels were measured before, 30 and 60 minutes after ACTH injection. Basal free testosterone (fT), SHBG, cortisol and androstenedione (A) were also measured. The diagnosis of 11 beta-hydroxylase deficiency has been presumed when the serum 11-deoxycortisol response to ACTH stimulation exceeded three times the 95th percentile of controls.


RESULTS
Basal hormone levels including fT and A were significantly higher in the hirsute women than in the healthy women. SHBG was significantly lower in the hirsute patients. Basal and ACTH stimulated 11-deoxycortisol levels were found to be significantly increased in the patients compared with the controls. Eight patients (6.5%) had an 11-deoxycortisol response higher than three times the upper normal limit.


CONCLUSIONS
Using stringent diagnostic criteria, we have found that 6.5% of the hirsute women in a Turkish population could be presumed to have 11 beta-hydroxylase deficiency.
Keleştimur
F
F
Department of Endocrinology, Erciyes University, Medical School Hospital, Kayseri, Turkey.
Sahin
Y
Y
Ayata
D
D
Tutuş
A
A
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*; D000328:Adult; D000735:Androstenedione / Q000097:blood; D003350:Cortodoxone / Q000097:blood; D003366:Cosyntropin; D005260:Female; D006628:Hirsutism / Q000453:epidemiology* / Q000209:etiology; D006801:Humans; D015995:Prevalence; D013739:Testosterone / Q000097:blood; D014421:Turkey / Q000453:epidemiology
D003366:Cosyntropin; D013739:Testosterone; D000735:Androstenedione; D003350:Cortodoxone