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Endocrine glands
Endocrine glands
The pituitary gland
The pituitary gland
Pituitary hormones
Pituitary hormones

Hypogonadotropic hypogonadism

Definition:

Hypogonadotropic hypogonadism describes absent or decreased function of the gonads -- the male testes or the female ovaries. It results from the absence of the gonadal stimulating pituitary hormones FSH (follicle stimulating hormone) and LH (luteinizing hormone).



Alternative Names: Gonadotropin deficiency; Kallmann syndrome; Secondary hypogonadism

Causes, incidence, and risk factors:

An area of the brain called the hypothalamus secretes gonadotropin-releasing hormone (GnRH), which stimulate the pituitary gland. In response to this hormone, the pituitary gland (located near the hypothalamus) secretes other hormones (FSH and LH).

These hormones, in turn, stimulate the ovaries (female) and�testes (male) to secrete hormones that are responsible for normal sexual development in puberty. Any disruption in this chain reaction causes a deficiency of the sex hormones and halts normal sexual maturation.

Failure of the hypothalamus is most commonly a result of Kallmann syndrome. Kallmann syndrome is an inherited disorder that usually includes a disorder of the sense of smell. Failure of the pituitary may result from empty sella syndrome, pituitary tumors (craniopharyngioma), head injuries, or other causes.



Symptoms:
  • lack of development at puberty, incomplete development or significant delay of pubertal development
  • prepubertal testicular size in adolescence
  • absence of secondary sexual development (for example, pubic, facial, and underarm hair)
  • short stature may be associated with some forms of hypogonadism
  • anosmia (inability to smell)


Signs and tests:
  • levels of various hormones in the blood
  • GnRH stimulation test (measuring hormone levels after stimulation by injected hormones)
  • MRI of the head


Treatment:

Treatment depends on the source of the problem.

  • intramuscular (IM) testosterone or slow-release testosterone skin patch
  • estrogen and progesterone pills
  • GnRH injections


Support Groups:



Expectations (prognosis):

With proper hormonal stimulation, puberty can be induced and fertility may be restored.



Complications:
  • delayed puberty
  • infertility
  • low self-esteem (Children who do not develop puberty changes until late may suffer from comparison with their peers. Lots of 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 is dependent on the cause. Genetic counseling may be appropriate for individuals with heritable disorders that are associated with hypogonadism. Prevention of serious head injuries reduces the risk of pituitary injury associated hypogonadotropic hypogonadism.




Review Date: 1/18/2006
Reviewed By: Nikheel S. Kolatkar, MD, Clinical and Research Fellow, Division of Endocrinology, Diabetes, and Hypertension, Brigham and Women's Hospital, Harvard Medical School, Boston, MA. Review provided by VeriMed Healthcare Network.

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