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Hypogonadotropic hypogonadism

Hypogonadism is a condition in which the male testes  or the female ovaries produce little or no sex hormones.

Hypogonadotropic hypogonadism (HH) is a form of hypogonadism that is due to a problem with the pituitary gland or hypothalamus.

Alternative Names

Gonadotropin deficiency; Secondary hypogonadism

Causes

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).

Normally:

  • 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).
 

Symptoms

Children:

  • 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)

Adults:

  • 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

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

Outlook (Prognosis)

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.

Possible Complications

  • Delayed puberty
  • Early menopause (in females) 
  • Infertility
  • 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

References

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.

Updated: 8/1/2013

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.


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