Medication for Congenital Adrenal Hyperplasia

Doctors at Hassenfeld Children’s Hospital at NYU Langone manage congenital adrenal hyperplasia in babies and children with medications to replace missing hormones and salt. Treatment is started immediately for newborns with the salt-wasting type, in which low levels of the hormones cortisol and aldosterone can lead to a life-threatening drop in blood pressure and shock.

Children with the classic type of congenital adrenal hyperplasia need to take medication for life. Your child’s doctor may temporarily increase the medication dosage when your child is ill; before he or she has surgery; or after an injury, since the body requires more cortisol in stressful situations.

Hormone Replacement Therapy

Hormone replacement therapy is used to restore missing hormones in children with congenital adrenal hyperplasia. Glucocorticoid medications, also known as corticosteroids, are used to increase cortisol levels and decrease androgen production in babies and children with the classic type.

For babies and young children, our doctors prescribe a type of glucocorticoid medication called hydrocortisone, which is taken daily by mouth. Older children who are finished growing may need more potent, longer-acting corticosteroids such as prednisolone or dexamethasone, which are also taken by mouth.

Children with classic congenital adrenal hyperplasia are also treated with a synthetic mineralocorticoid medication called fludrocortisone. This medication maintains normal salt levels.

Our pediatric endocrinologists monitor children who are being treated with these medications every three to four months to ensure normal growth and development. As a child grows, the medication dosage may need to be adjusted.

Boys with congenital adrenal hyperplasia have a physical exam at every visit to look for signs of testicular adrenal rest tumors—tumors in the testes that are composed of enlarged adrenal cells—after they begin puberty.

Salt Replacement

Doctors prescribe salt replacement medications in combination with fludrocortisone for infants with salt-wasting congenital adrenal hyperplasia. These medications are taken by mouth. After age three, a child is encouraged to eat salty foods to increase sodium in the diet.