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

Abstract
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.

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