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Hypogonadotropic hypogonadism

Definition

Hypogonadotropic hypogonadism is a form of hypogonadism that is due to a problem with the pituitary gland or hypothalamus. In this condition, the male testes or the female ovaries produce little or no hormones.

Alternative Names

Gonadotropin deficiency; Secondary hypogonadism; Kallmann syndrome

Causes, incidence, and risk factors

Hypogonadotropic hypogonadism is caused by a lack of secretion of the gonadal stimulating pituitary hormones: 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 other hormones, including FSH and LH. These hormones tell the female ovaries and 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.

Failure of the hypothalamus is usually a result of Kallmann syndrome. Kallmann syndrome is an inherited form of hypogonadotropic hypogonadism that can occur with a loss of smell.

Symptoms

  • In females, absence of breasts and menstruation
  • In males, 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 incomplete or significantly delayed)
  • Short stature (in some cases)

Signs and tests

Tests that may be done include:

Treatment

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

  • Injections of testosterone
  • Slow-release testosterone skin patch
  • Testosterone gels
  • Estrogen and progesterone pills
  • GnRH injections

Expectations (prognosis)

With the right hormone treatment, the person can go through puberty, and fertility may be restored.

Complications

  • Delayed puberty
  • Infertility
  • Low self-esteem due to late start of puberty (emotional support may be helpful)

Calling your health care provider

Call your health care provider if your child does not enter puberty as expected.

Prevention

Prevention depends on the cause.

People who have a family history of inherited conditions that cause hypogonadism may benefit from genetic counseling. Preventing serious head injuries reduces the risk of hypogonadotropic hypogonadism due to pituitary injury.

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.


Review Date: 5/8/2012
Reviewed By: A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, and David R. Eltz. Previously reviewed by Nancy J. Rennert, MD, Chief of Endocrinology & Diabetes, Norwalk Hospital, Associate Clinical Professor of Medicine, Yale University School of Medicine, New Haven, CT. Review provided by VeriMed Healthcare Network (7/26/2011).
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. Call 911 for all medical emergencies. 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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