Non classical congenital adrenal hyperplasia


NCAH can cause excessive male hormones. This is a relatively mild condition that doesn’t usually require treatment.

NCAH is a congenital disease that affects the glands of the adrenals. It is present from birth.

The Adrenal Gland is a small kidney-shaped organ on top of the kidneys. They produce cortisol, a hormone released in response to an injury, aldosterone, which regulates water and salt levels; and androgens.

If you have nonclassical congenital hyperplasia of the adrenal glands, your body does not produce enough 21-hydroxylase. This enzyme affects how much hormones your body produces — usually too little cortisol and too much androgen.

What causes nonclassical congenital adrenal hyperplasia (NCCAH)?

A 21-hydroxylase defect most commonly causes NCAH. The genetic mutation that you inherit is the cause of this enzyme deficiency. The compounds used to make the hormone Aldosterone and cortisol build up in the adrenal glands when there is not enough 21-hydroxylase. Your adrenal glands convert these compounds into androgens which can cause problems.

NCAH is usually diagnosed later in life. It’s not life-threatening. You may have normal genitals, but you’ll also likely have extra male hormones.

What are the symptoms of nonclassical congenital adrenal hyperplasia?

Many people have mild or no symptoms of NCAH. It can be challenging to diagnose because symptoms often mimic other conditions. It can be confused with Polycystic Ovarian Syndrome or PCOS. PCOS is a condition where the ovaries produce too many androgens.

NCAH symptoms include

  • Extra body or face hair
  • Acne
  • Period problems
  • Infertility
  • Androgenic hair loss or male pattern hair loss
  • The risk of miscarriage is higher
  • Ovulatory Infertility or difficulty getting pregnant due to problems with the ovaries
  • Early beard development in Boys
  • Small testicles
  • Early puberty
  • Short stature but early and rapid growth
  • Oily Skin
  • Body odor

How is nonclassical congenital adrenal hyperplasia diagnosed?

Your doctor will perform tests to determine the hormone levels in your blood.

Your doctor may conduct blood tests to determine your adrenal steroid level if symptoms appear later in life. You may also be asked to take an adrenocorticotropic hormonal (ACTH) stimulation test. Your doctor will test your cortisol level and then give you ACTH. They recheck your blood after an hour.

Typical test results show high cortisol levels in response to hormones. NCAH tests reveal high levels of the compounds which produce cortisol but low cortisol levels. This means that your adrenal glands are having difficulty making hormones.

What is the treatment for nonclassical congenital adrenal hyperplasia (NCAH)?

Most people don’t need nonclassical congenital hyperplasia treatments, especially without symptoms. Your doctor may prescribe medication if you experience symptoms such as acne, period problems, or extra facial hairs. These treatments include:

  • Hydrocortisone is a type of steroid medication
  • Birth control pills
  • Antiandrogen medications such as spironolactone
  • Laser hair removal
  • Fertility medication

You can reduce your medication as you improve. It is essential to do this under the supervision of your doctor.

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