ANDROGEN METABOLISM IN CONGENITAL ADRENAL HYPERPLASIA DUE TO 11 β-HYDROXYLASE DEFICIENCY.

The plasma concentration of androstenedione and testosterone, metabolic clearance rate of androstenedione, and in vivo conversion ratio of androstenedione to testosterone have been studied in a normotensive 5-year-old female with congenital adrenal hyperplasia due to a deficiency of 11 β-hydroxylase. Prior to glucocorticoid administration, the urinary excretion of 17-ketosteroids varied from 2.2 to 4.9 mg/24 hours, urinary excretion of pregnanetriol varied from 0.7 to 2.2 mg/24 hours, and total 17-hydroxysteroid excretion varied from 1.2 to 7.5 mg/24 hours. Urinary tetrahydro-11-deoxy cortisol (TSH) was detected at a concentration of 550 µg/24 hours. The plasma concentration of androstenedione varied from 100 to 530 mµg/100 ml and the plasma concentration of testosterone varied from 40 to 90 mµg/100 ml. These values are significantly elevated when compared to those obtained in normal prepubertal females. Urinary steroid excretion and plasma androgen concentrations fell to normal in response to glucocorticoid administration. The metabolic clearance rate of androstenedione was 890 liters per day per M² and the in vivo conversion ratio of androstenedione to testosterone was 11%. The calculated production rate of androstenedione was 4.7 mg per day per M². Virilization in congenital adrenal hyperplasia due to 11 β-hydroxylase deficiency can be explained by an elevated plasma concentration of testosterone, which can be accounted for on the basis of conversion from androstenedione. [ABSTRACT FROM AUTHOR]