THE PREVALENCE OF NONCLASSIC 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 o f 11 beta-hydroxylase deficiency in adult women with hirsutism in a Tu rkish population. DESIGN AND PATIENTS One hundred and twenty-four cons ecutive unselected hirsute patients were studied. An ACTH stimulation test was performed in the midfollicular phase of the cycle on the pati ents and 20 age-matched controls by administration of a single bolus o f 0.25 mg ACTH (1-24) at 0900 h. MEASUREMENTS Serum 11-deoxycortisol l evels were measured before, 30 and 60 minutes after ACTH injection. Ba sal free testosterone (fT), SHBG, cortisol and androstenedione (A) wer e also measured. The diagnosis of 11 beta-hydroxylase deficiency has b een presumed when the serum 11-deoxycortisol response to ACTH stimulat ion exceeded three times the 95th percentile of controls. RESULTS Basa l hormone levels including fT and A were significantly higher in the h irsute women than in the healthy women. SHBG was significantly lower i n the hirsute patients. Basal and ACTH stimulated 11-deoxycortisol lev els were found to be significantly increased in the patients compared with the controls. Eight patients (6.5%) had an 11-deoxycortisol respo nse 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-hydrox ylase deficiency.