Since the introduction of glucocorticoid and mineralocorticoid replacement
therapy approximately 50 years ago, the standard treatment principles of 21
-hydroxylase deficiency have remained largely unchanged. Glucocorticoid is
given in doses sufficient to suppress adrenal androgen secretion, and miner
alocorticoid is given to normalize electrolytes and plasma renin activity.
This treatment strategy often fails to normalize the growth and development
of children with congenital adrenal hyperplasia (CAH), and management is o
ften complicated by iatrogenic Gushing's syndrome, inadequately treated hyp
erandrogenism, and, later in life, infertility. This article addresses the
clinical problems patients with CAH continue to have and outlines new ideas
for the medical treatment of this condition.