Congenital Adrenal Hypoplasia

What is congenital adrenal hypoplasia?

The small adrenal glands sit on top of each kidney. They produce hormones needed to live, including cortisol and sex hormones. Cortisol controls our response to stress and many other functions.

Congenital Adrenal Hypoplasia affects boys. It occurs when the adrenal glands do not fully develop and cannot produce the level of hormones the body needs. Children with adrenal hypoplasia show signs of the condition as infants or in early childhood.

What are the signs and symptoms of congenital adrenal hypoplasia?

Symptoms include:

• Vomiting
• Difficulty feeding
• Dehydration
• Extremely low blood sugar
• Low sodium levels
• Shock

If untreated, these symptoms can be life-threatening.

Boys with adrenal hypoplasia may lack sex hormones, which can delay puberty and cause infertility.

How is congenital adrenal hypoplasia treated?

Initial treatments include intravenous saline and dextrose to treat dehydration and bring blood sugar and sodium levels up to normal. Other treatments can include:

• Glucocorticoids to stop inflammation
• Hormone replacements