What Are the Treatments for Hyperplastic Adrenal Glands?

Hyperplastic adrenal glands, often referred to as adrenal hyperplasia, is a group of conditions characterized by the over production of the steroid hormone testosterone and underproduction of aldosterone and cortisol by the adrenal glands (located just above the kidneys). Some patients are born with it while others develop it later on in life. There are a few types of the disorder depending on the affected hormone. The type you have would dictate the type of treatment you would receive. Treatments are aimed at correcting the hormonal imbalance and in some cases correcting genital abnormalities that may result.

Symptoms

• The most common form is caused by a defective enzyme called 21 hydroxylase (90 to 95 percent of cases), according to the US National Library of Medicine. This leads to an excess production of testosterone and a decreased production of cortisol and aldosterone. The result in children is early sexual development, uncontrolled libido and aggressiveness in boys and genital abnormalities in girls. Adult women can suffer from unwanted growth of hair on the face and body, irregular or absent periods, infertility and acne.

General Management

• Your doctor is likely to advise that you attend regular checkups to make sure that your condition is well controlled. The Adrenal Hyperplasia Network advises that if you are a woman, you have a check up two to three times per year to maintain fertility and regular periods and to control testosterone levels. For men, once per year is usually enough to control libido, aggressiveness and sperm count if fertility is an issue.

Cortisol Treatment

• Cortisol therapy may be prescribed for you to help to minimize the overproduction of testosterone. Examples include hydrocortisone, prednisolone and dexmethasone. Side effects include nausea, headache and water retention. The dose may be adjusted in a controlled way by your doctor in times of stress or if you get an infection like flu.

Salt Losers

• Eighty percent of children with this condition are "salt losers," meaning they produce little or no aldosterone so they can be vulnerable to the effects of excessive salt loss like low blood pressure and dehydration. Treatment is aimed at minimizing salt loss to avoid acute adrenal crisis, which requires urgent medical attention, administration of fluids into a vein and possibly steroid injections. Ongoing treatment is with fludrocortisone which needs to be very carefully adjusted according to your blood pressure and salt levels. Too much can lead to headaches and increased blood pressure and too little can lead to low blood pressure and dizziness.

Non Salt Losers

• The remaining 20 percent of children are relatively healthy but girls may be born with enlarged or fused labia and enlarged clitoris due to excess testosterone. Once the girl reaches adulthood, surgery may be offered to reduce clitoral size or enlarge a small vaginal opening. Boys typically do not exhibit symptoms until age 4 or 5 when they show an abnormal growth of pubic hair and abnormal penis growth, at which time you should speak to your doctor about possible treatments.

Late Onset

• In women with late onset adrenal hyperplasia, often treatment with estrogen is enough to balance out the effect of testosterone. In men, sometimes the only effect is a reduced sperm count.