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.

Gonadotropin deficiency; Secondary hypogonadism

HH is caused by a lack of hormones that normally stimulate the ovaries or testes: 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 medications

Kallmann syndrome is an inherited form of HH that typically occurs with a loss 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)

Tests that may be done include:

• Blood tests to measure hormone levels such as FSH, LH, and TSH
• 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) 
• Infertility
• Low bone density
• Low self-esteem due to late start of puberty (emotional support may be helpful)
• Sexual problems such as low libido

Call your health care provider if:

• Your child does not start puberty
• You are a woman and your menstrual cycles are irregular
• You have lost armpit or pubic hair
• You are a man and you have lost muscle mass

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.