Compliance with the treatment must be ensured, growth closely monitored, and bone age readings obtained yearly. Affected female infants may require surgical reconstruction with reduction clitoroplasty and construction of a vaginal opening. Often, further surgery is required in adulthood, but with appropriate care and attention to psychosexual issues, a normal sex life and fertility may be expected. Because of deoxycorticosterone's mineralocorticoid activity, patients exhibit salt retention and hypertension with hypokalemic alkalosis. Plasma renin activity is low.
Virilization also occurs.
This very rare disorder results in accumulation of DHEA, which is peripherally converted to testosterone in extraglandular tissue. Treatment is also with a glucocorticoid and with a mineralocorticoid when necessary. No abnormalities of sexual differentiation exist. All rights reserved.