The prevalence of non-classic adrenal hyperplasia due to 11β-hydroxylase deficiency among hirsute women in a Turkish population

OBJECTIVE  The present study was designed to determine the prevalence of 11β-hydroxylase deficiency in adult women with hirsutism in a Turkish population.
DESIGN AND PATIENTS  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.
MEASUREMENTS  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 11β-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 11β-hydroxylase deficiency.