Hypogonadotropic hypogonadism

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

Hypogonadism

Hypogonadism occurs when the body's sex glands produce little or no hormones. In men, these glands (gonads) are the testes. In women, these glands ...

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Testes

The testes are 2 egg-shaped male reproductive organs located in the scrotum. They produce sperm and the male hormone, testosterone.

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

Causes

HH is caused by a lack of hormones that normally stimulate the ovaries or testes. These hormones include gonadotropin-releasing hormone (GnRH), follicle stimulating hormone (FSH) and luteinizing hormone (LH).

Normally:

• The hypothalamus in the brain releases 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, normal menstrual cycles, estrogen levels and fertility in adult women, and normal testosterone production and sperm production in adult men.
• Any change in this hormone release chain causes a lack of sex hormones. This prevents normal sexual maturity in children and normal function of the testicles or ovaries in adults.

There are several causes of HH:

• Damage to the pituitary gland or hypothalamus from surgery, injury, tumor, infection, or radiation
• Genetic defects
• High doses or long-term use of opioid or steroid (glucocorticoid) medicines
• High prolactin level (a different hormone released by the pituitary)
• Severe stress
• Nutritional problems (both rapid weight gain or weight loss)
• Long-term (chronic) medical diseases, including chronic inflammation or infections
• Drug use, such as heroin or use or abuse of prescription opiate medicines
• Certain medical conditions, such as iron overload

  Iron overload

  Hemochromatosis is a condition in which there is too much iron in the body. It is also called iron overload.

  

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Kallmann syndrome is an inherited form of HH. Some people with this condition also have anosmia (loss of the sense of smell).

Symptoms

Children:

• Lack of growth and sexual development at the standard age for puberty (development may be very late or incomplete)
  
• In girls, a lack of breast development and menstrual periods
• In boys, 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)
• Short stature (in some cases)

  Short stature

  A child who has short stature is much shorter than children who are the same age and sex. Your health care provider will go over your child's growth ...

  

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Adults:

• Loss of interest in sex (libido) in men
• Loss of menstrual periods (amenorrhea) in women
• Decreased energy and interest in activities
• Loss of muscle mass in men
• Weight gain
• Mood changes
• Infertility
  

  Infertility

  Infertility means you cannot get pregnant (conceive). There are 2 types of infertility:Primary infertility refers to couples who have not become preg...

  

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Exams and Tests

The health care provider will perform a physical exam and ask about your symptoms.

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

  LH response to GnRH

  LH response to GnRH is a blood test to help determine if your pituitary gland can correctly respond to gonadotropin releasing hormone (GnRH). LH sta...

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• MRI of the pituitary gland/hypothalamus (to look for a tumor or other growth)
• Genetic testing
• Blood tests to check for iron level

Treatment

Treatment depends on the source of the problem, but may involve:

• Injections of testosterone (in males)

  Testosterone

  A testosterone test measures the amount of the male hormone, testosterone, in the blood. Both men and women produce this hormone. The test described...

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• Slow-release testosterone skin patch (in males)
• Testosterone gels (in males)
• Estrogen and progesterone pills or skin patches (in females)
• GnRH injections
• HCG injections 
  

Outlook (Prognosis)

The right hormone treatment will cause puberty to start in children and may restore fertility in adults. If the condition begins after puberty or in adulthood, symptoms will often improve with treatment.

Possible Complications

Health problems that may result from HH include:

• 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

Contact your provider if:

• Your child does not start puberty at the appropriate time. 
• You are a woman under age 40 and your menstrual cycles stop.
• You have lost armpit or pubic hair.
• You are a man and you have decreased interest in sex.

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