11 beta-Hydroryandrostenedione and Delta(5)-androstenediol as markers of adrenal androgen production in patients with 21-hydroxylase-deficient nonclassic adrenal hyperplasia

Objective: To determine the sensitivity of 11 beta-hydroxyandrostenedione ( 11-OHA4) and Delta(5)-androstenediol (ADIOL) as markers of excessive adrena l androgen production. Design: Prospective study. Setting: Academic medical centers. Patient(s): Thirteen women with untreated 21-hydroxylase-deficient nonclass ic adrenal hyperplasia (NCAH) and 18 healthy, eumenorrheic, nonhirsute cont rols matched for age and body mass index. Intervention(s): All subjects were studied before and after acute adrenal s timulation with 0.25 mg of IV adrenal ATCH-(1-24) Main Outcome Measure(s): Basal levels of total testosterone, sex hormone-bi nding globulin, DHEAS, and free testosterone were measured. Levels of andro stenedione (A4), DHEA, 11-OHA4, and ADIOL were determined before (Steroid(0 )) and 60 minutes after (Steroid(60)) acute ACTH-(1-24) stimulation. Result(s): Patients with NCAH had higher median basal levels of DHEAS and t otal and free testosterone than controls. Patients with NCAH had higher med ian A4(0), A4(60), DHEA(0), DWEA(60), 11-OHA4(0), ADIOL(0), and ADIOL(60) l evels but similar 11-OHA4(60) levels compared with controls. Among patients with NCAH, 30%, 54%, 15%, and 85% had 11-OHA4(0), ADIOL(0), 11-OHA4(60), a nd ADIOL(60) levels, respectively, above the 95th percentile of controls. Conclusion(s): Overall, serum levels of 11-OHA4 did not appear to be a very sensitive marker of excessive NCAH.Although ACTH-stimulated ADIOL levels w ere elevated ill 85% of the patients studied, they did not appear to have a ny advantage over the measurement of A4 or DHEA levels. ((C)1999 by America n Society for Reproductive Medicine.).