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Congenital Adrenal Hyperplasia

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Treatment

Treatment of congenital adrenal hyperplasia (CAH) includes replacement of the substances that are not being adequately produced by the body because of the enzyme deficiency and suppression of excess androgen production. This may include:

  • Glucocorticoid – a steroid such as hydrocortisone to replace cortisol and suppress ACTH secretion and excess androgen production
  • Mineralocorticoid – to replace aldosterone if someone has a salt-wasting form of CAH; salt supplementation may also be recommended for some infants.

Doses of steroids must be adjusted to avoid overtreatment as this can cause symptoms associated with Cushing syndrome. People will frequently require additional amounts of steroids during periods of stress and illness.

An adrenal crisis can be life-threatening and is typically treated with intravenous (IV) injections of glucocorticoids and large volumes of intravenous saline solution with the sugar dextrose. This treatment usually brings rapid improvement.

Surgery is sometimes indicated to modify external sex organs. It may be performed on a young female and is also sometimes required as a young adult.

Certain symptoms, such as excess facial hair and acne, may be addressed with specific treatments, but these should be in addition to, not instead of, CAH treatments.

There are a range of other treatments that may also be prescribed, for example, to block the effects of androgens, control blood pressure, stimulate growth, suppress puberty, and slow bone growth. People should talk to their doctors and to specialists as needed to determine the right therapies for their child (or themselves) at different stages of their development and to learn more about CAH.

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