Serum 3α-androstanediol glucuronide measurements in sexually mature women with congenital adrenal hyperplasia during therapy

Serum 3α-androstanediol glucuronide (3α-diol G) measurements may indicate the extent of androgen metabolism and action in target tissues. To test this supposition we measured serum 3α-diol G concentrations in 23 women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, including 13 with the salt-losing and 10 with the simple virilizing form, while they were receiving glucocorticoid and, in some cases, mineralocorticoid therapy. Their mean age was 28.3 yr (range, 17.9-38.7 yr). Twenty-four-hour urinary 17-ketosteroid excretion, plasma androstenedione and testosterone levels, and serum 3α-diol G levels were measured during the follicular phase. The values were within or below the normal range in 87%, 78%, 70%, and 91% of the patients, respectively. By contrast, plasma 17-hydroxyprogesterone levels were normal in only 12% of the patients. Serum 3α-diol G levels correlated well with 24-h urinary 17-ketosteroid excretion (r = 0.75) and plasma 17-hydroxyprogesterone (r = 0.77), androstenedione (r = 0.84), and testosterone (r = 0.93) levels. The serum 3α-diol G levels were not significantly different in the women with the salt-losing form and those with the simple virilizing form. However, they were significantly lower (P<0.05) in the women with normal menses compared to those with abnormal menses. This finding underscores the validity of serum 3α-diol G measurements as indicators of androgen production and metabolism in women. The excellent correlation between the serum 3α-diol G levels and standard measures of control indicates that the former measurement may be a useful adjunct in the management of women with congenital adrenal hyperplasia.