IV. CAH WITH TREATMENT

CAH cannot be cured, but it can be effectively treated. Modalities of treatment were established in the early 1950's at Johns Hopkins Hospital in Baltimore and at Massachusetts General Hospital in Boston. The principle of therapy is to administer an amount of cortisol approximately equal to the normal production of this steroid. The result is suppression of ACTH and consequently suppression of adrenal function. Putting the adrenal "at rest" results in total suppression of cortisol precursors (including androgens).

In salt losers, it is also necessary to administer salt-retaining hormone treatment (florinef) to compensate for the aldosterone deficiency.In all forms of 21-hydroxylase deficiency, it is most important to double or triple the baseline dose of cortisol at times of stress.