Non Classical Cah Treatment in Menopause

Congenital adrenal hyperplasia, or CAH, is a syndrome of the adrenal system that is hereditary. The syndrome is caused by a genetic mutation that prevents the production of enzymes of the adrenal hormones. Classical CAH occurs in infancy, while the non-classical form of the syndrome is milder, more common and occurs at any age and, in women, persists until past menopause.

Non-classical CAH

• Patients with non-classical CAH experience only a partial enzyme deficiency, which means better production of cortisol and aldosterone. Unlike classical CAH, these patients do not experience the adrenal "crisis" that is known to be present in the classical form. They are also better able, with treatment, to keep the syndrome under reasonable control.

Treatment in Women

• Women with non-classical CAH are treated with a glucocorticoid such as prednisone or dexamethasone. Because the syndrome causes a deficiency of aldosterone, patients are given fludrocortisone, which mimics the missing aldosterone and aids in salt retention. They are also encouraged to eat a salty diet. Oral contraceptives or antiandrogen therapy can prevent acne and hirsutism. No additional treatment is recommended for women of menopausal or post-menopausal age.

Bone Mineral Density

• Because of long-term treatment with glucocorticoids as well as the salt deficiency, women may be at increased risk of a reduction in bone mineral density. Patients with non-classical CAH, particularly those in the menopausal or post-menopausal age group, should undergo regular bone density scans to ensure that bone mineral density is being adequately maintained.