Congenital adrenal hyperplasia medical therapy

Congenital adrenal hyperplasia main page
Overview
Classification 21-hydroxylase deficiency
11β-hydroxylase deficiency
17 alpha-hydroxylase deficiency
3 beta-hydroxysteroid dehydrogenase deficiency
Cytochrome P450-oxidoreductase (POR) deficiency (ORD)
Lipoid congenital adrenal hyperplasia
Differential Diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]

Acute Pharmacotherapies

21-Hydroxylase

• Treatment is aimed at decreasing the drive to produce ACTH (AdrenoCorticoTropic Hormone), thereby decreasing androgen secretion. Glucocorticoids are given usually as hydrocortisone 25 mg QD in divided doses. The dose of steroids must be carefully titrated so that androgen production is minimized, but growth inhibition and Cushing’s syndrome from glucocorticoid excess is avoided. Assessing bone age and growth rate, signs of androgen excess and menstrual cycling gauge response to therapy. Adjunctive therapy with flutamide, an anti-androgen and testolactone, and anti-aromatase, have been shown to minimize the effects of excess androgens, reduce the dose of glucocorticoids and normalize growth rates.

• Mineralocorticoids, with or without evidence of evidence of salt loss, should be given in the form of fludrocortisone. It decreases excessive renin and Angiotensin II (AII) production. AII stimulates early steps in the steroidogenic pathway and leads to more androgen production. Both renin and AII are elevated in the salt-wasting and virilizing forms of CAH. The usual adult dose is 0.1 mg/day.

11-Hydroxylase

Treatment is similar to 21-hydroxylase deficiency with glucocorticoid replacement. Clinical assessment of virilization, growth velocity, hair growth, menstrual function and blood pressure are necessary. Despite adequate glucocorticoid replacement, medication may be required to control blood pressure. Spironolactone is a good choice as well as calcium blockers.

References

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