Hypogonadotropic hypogonadism (HH) is a form of hypogonadism that is due to a problem with the pituitary gland or hypothalamus.
Gonadotropin deficiency; Secondary hypogonadism
HH is caused by a lack of hormones that normally stimulate the ovaries or testes: gonadotropin-releasing hormone (GnRH), follicle stimulating hormone (FSH) and luteinizing hormone (LH).
- The hypothalamus in the brain releases gonadotropin-releasing hormone (GnRH).
- This hormone stimulates the pituitary gland to release FSH and LH.
- These hormones tell the female ovaries or the male testes to release hormones that lead to normal sexual development in puberty.
- Any change in this hormone release chain causes a lack of sex hormones and prevents normal sexual maturity.
There are several causes of HH:
- Damage to the pituitary gland or hypothalamus from surgery, injury, tumors, infections, or radiation
- Genetic defects
- High doses or long-term use of opioid or steroid (glucocorticoid) medications
- Severe stress
- Nutritional problems (both rapid weight gain or weight loss)
- Chronic medical diseases, including chronic inflammation or infections
Kallmann syndrome is an inherited form of HH. Some patients with Kallmann’s also have anosmia (loss of the sense of smell).
- In females, a lack of breasts and menstrual periods
- In males, no development of sex characteristics, such as enlargement of the testes and penis, deepening of the voice, and facial hair
- Inability to smell (in some cases)
- Lack of development at puberty (development may be very late or incomplete)
- Short stature (in some cases)
- Loss of sexual interest (libido) in men
- Loss of menstrual periods (amenorrhea) in women
- Decreased energy and interest
- Loss of muscle mass in men
- Weight gain
Exams and Tests
Tests that may be done include:
- Blood tests to measure hormone levels such as FSH, LH, and TSH, prolactin, testosterone and estradiol
- LH response to GnRH
- MRI of the pituitary gland/hypothalamus (to look for a tumor or other growth)
- Genetic testing
Treatment depends on the source of the problem, but may involve:
- Injections of testosterone
- Slow-release testosterone skin patch
- Testosterone gels (in males)
- Estrogen and progesterone pills (in females)
- GnRH injections
The right hormone treatment will cause puberty to start and may restore fertility. If the condition begins after puberty or in adulthood, symptoms will often improve with treatment.
- Delayed puberty
- Early menopause (in females)
- Low bone density and fractures later in life
- Low self-esteem due to late start of puberty (emotional support may be helpful)
- Sexual problems such as low libido
When to Contact a Medical Professional
Call your health care provider if:
- Your child does not start puberty at the appropriate time
- You are a woman under 40 and your menstrual cycles stop
- You have lost armpit or pubic hair
- You are a man and you have decreased sexual interest
Styne DM, Grumbach MM. Puberty: Ontogeny, neuroendocrinology, physiology, and disorders. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 25.
Reviewed By: Brent Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.