Congenital adrenal hyperplasia (CAH) is one of the most common disorders of sex development (DSD).  DSD describes a group of conditions in which the genitals or reproductive parts do not develop in a typical way.  

CAH is a problem of the adrenal glands.  The adrenal glands sit on top of the kidneys and make three types of hormones (chemical signals): cortisol, aldosterone and androgens. 

  • Cortisol controls energy and blood sugar levels, and is needed for life. 
  • Aldosterone controls salt and water levels, and is also needed for life. 
  • Androgens (male sex hormones such as testosterone) cause male-type changes during puberty in both girls and boys.  These changes include body hair in the armpits and genital area, body odor and acne.   

CAH results when there is a problem in making one or more of these hormones.  When the adrenal glands are not able to make enough cortisol and aldosterone, the body makes too much androgen instead. 

CAH can be masculinizing, feminizing, salt-wasting or non-salt-wasting.

  • Too much male sex hormone in girls can make their genitals look more like a boy’s (masculinizing). 
  • Too little male sex hormone in boys can make their genitals look more like a girl’s (feminizing). 
  • Too little aldosterone causes salt-wasting. 
  • Some people do not have salt-wasting if they make enough aldosterone.  Usually people with non-salt-wasting CAH have a milder form of the condition.