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Treatments and drugs
By Mayo Clinic staffTo treat congenital adrenal hyperplasia, it's best to get a referral to a specialist in childhood hormonal issues (pediatric endocrinologist). Treatments include:
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Medications. In most cases, your child's doctor will prescribe replacement hormone medication to boost the levels of deficient hormones in your child and restore them to normal levels. For example, your child may take an oral drug such as hydrocortisone or dexamethasone to replace cortisol, and fludrocortisone to replace aldosterone, on a daily basis. At times, children with congenital adrenal hyperplasia need multiple drugs, with even higher doses prescribed during periods of illness or severe stress, including surgery.
Steroid-type replacement medications may cause side effects, particularly if the doses are high and are used long term. Your child will need monitoring for drug-related effects such as the loss of bone mass and impaired growth. Periodic routine blood tests throughout the course of your child's treatment will alert your child's doctor to make medication adjustments if needed. Regular visits to the doctor can serve to evaluate your child's progress, including monitoring changes in height, weight and blood pressure.
As adults, some men and women with congenital adrenal hyperplasia are able to stop taking their medications. However, others, particularly people with the classic form of the disease, may need to take medications indefinitely.
- Surgery. In some infant girls who have ambiguous external genitalia, doctors recommend reconstructive surgery to correct the appearance and function of the genitals. This procedure may involve reduction of the clitoris size and reconstruction of the vaginal opening. The surgery is typically performed between 2 and 6 months of age.
Prenatal management
Sometimes, treatment for congenital adrenal hyperplasia can begin before your child is born.
When congenital adrenal hyperplasia is diagnosed in the fetus, one option is for the pregnant mother to take a potent corticosteroid drug, such as dexamethasone, before giving birth. Corticosteroids can cross the placenta into the womb, and suppress the activity of the fetus's own adrenal glands. By reducing the secretion of male hormones (androgens), this approach may allow female genitals to develop normally. It can also reduce the masculine features that may develop later in female fetuses. When the fetus is male, doctors may recommend a shorter course of lower dose dexamethasone treatment during pregnancy to keep the adrenal glands functioning as normally as possible.
Although the use of dexamethasone can be effective, many doctors prescribe it cautiously, awaiting findings about the long-term effects of the drug's use in children with this condition. The medication can also cause side effects in the pregnant mother, including excess weight gain, mood swings and high blood pressure.
Prognosis
Many children with congenital adrenal hyperplasia can successfully manage the condition by remaining on their medications indefinitely. They grow up to lead lives in good health and with a normal life expectancy; however, they may be shorter than their parents. Both men and women may have fertility problems in adulthood.
Girls who have corrective genital surgery may need further cosmetic surgery later in life. When they become sexually active, they're more likely than are women who have not had genital surgery to experience sexual problems such as pain during intercourse and difficulty achieving orgasm.
Some advocates of children with ambiguous genitalia argue that corrective surgery shouldn't be performed until a child is old enough to understand the risks and choose his or her own gender assignment. However, most experts recommend performing corrective genital surgery when girls are very young because the surgery is technically less challenging than in later years.
Before making decisions about the best treatment approach for your daughter, talk with your doctor about these issues. Working together, you and your doctor can make informed choices that will help your daughter thrive.
- Congenital adrenal hyperplasia. Institute of Child Health and Human Development. http://www.nichd.nih.gov/health/topics/Congenital_Adrenal_Hyperplasia.cfm. Accessed Jan. 29, 2009.
- Stewart PM. The adrenal cortex. In: Kronenberg HM, et al. Kronenberg: Williams Textbook of Endocrinology. 11th ed. Philadelphia, Pa.: Saunders Elsevier; 2008:445
- Bora N. Congenital adrenal hyperplasia. In: Ferri FF. Ferri's Clinical Advisor 2009. St. Louis, Mo.: Mosby; 2008:216.
- Adrenal diseases — congenital adrenal hyperplasia (CAH): The facts you need to know. National Adrenal Diseases Foundation. http://www.nadf.us/diseases/cah.htm. Accessed Jan. 29, 2009.
- Nieman LK. Genetics and clinical presentation of nonclassic (late-onset) congenital adrenal hyperplasia due to CYP21A2 (21-hydroxylase) deficiency. http://www.uptodate.com/home/index.html. Accessed Jan. 29, 2009.
- Merke DP. Genetics and clinical presentation of classic congenital adrenal hyperplasia due to CYP21A2 (21-hydroxylase) deficiency. http://www.uptodate.com/home/index.html. Accessed Jan. 29, 2009.
- Parks JS, et al. Congenital adrenal hyperplasia and related disorders. In: Kliegman RM, et al. Kliegman: Nelson Textbook of Pediatrics. 18th ed. Saunders Elsevier; 2007:2360.
- Diamond DA. Sexual differentiation: Normal and abnormal. In: Wein AJ. Wein: Campbell-Walsh Urology. 9th ed. Saunders Elsevier; 2007:3799.
- Gastaud F, et al. Impaired sexual and reproductive outcomes in women with classical forms of congenital adrenal hyperplasia. Journal of Clinical Endocrinology and Metabolism. 2007;92:1391.
- Nieman LK. Treatment of classic congenital adrenal hyperplasia due to CYP21A2 (21-hydroxylase) deficiency in adults. http://www.uptodate.com/home/index.html. Accessed Jan. 29, 2009.
- Nippoldt TB (expert opinion). Mayo Clinic, Rochester, Minn. Feb. 4, 2009.
