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    Hypogonadotropic hypogonadism

    Gonadotropin deficiency; Secondary hypogonadism

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

    Hypogonadotropic hypogonadism (HH) is a form ofhypogonadism 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: 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.
    • Thesehormones tell the female ovariesor themale 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 opioidor steroid medications

    Kallmann syndromeis an inherited form of HH thattypically occurs with a loss of smell.

    Symptoms

    • In females, a lack of breasts and menstrual periods
    • In males, no development of sex characteristics, such asenlargement 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)

    Exams and Tests

    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

    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 andmay 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
    • 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 notstart 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

    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.

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    • Endocrine glands

      illustration

    • The pituitary gland

      illustration

    • Pituitary hormones

      illustration

      • Endocrine glands

        illustration

      • The pituitary gland

        illustration

      • Pituitary hormones

        illustration

      A Closer Look

        Tests for Hypogonadotropic hypogonadism

          Review Date: 8/16/2012

          Reviewed By: Shehzad Topiwala, MD, Chief Consultant Endocrinologist, Premier Medical Associates, The Villages, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

          The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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