Hydrocortisone replacement in disorders of sex development.

Patients with defects in the steroid biosynthesis and resulting disorders of sex development are the largest group among patients with such disorders. Many of these patients suffer from adrenal insufficiency and have to take either glucocorticoids or a combination of glucocorticoid and mineralocorticoid replacement therapy from birth to avoid life-threatening complications. In this chapter, the physiologic situation of cortisol secretion and the different possibilities of hormone replacement therapy are discussed. Further attention is given to stress-dosing of glucocorticoids, especially hydrocortisone.