MedlinePlus Medical Encyclopedia: Hypogonadotropic hypogonadism

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Hypogonadotropic hypogonadism

Contents of this page:
Illustrations Alternative names Definition Causes, incidence, and risk factors Symptoms Signs and tests	Treatment Expectations (prognosis) Complications Calling your health care provider Prevention

Illustrations

Endocrine glands

The pituitary gland

Pituitary hormones

Alternative names Return to top

Gonadotropin deficiency; Kallmann syndrome; Secondary hypogonadism

Definition Return to top

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

Causes, incidence, and risk factors Return to top

An area of the brain called the hypothalamus secretes hormones (GnRH) which stimulate the pituitary gland. In response to these hormones, the pituitary gland (located near the brain) 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 or cascade 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 Return to top

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 Return to top

levels of various hormones in the blood
GnRH stimulation test (measuring hormone levels after stimulation by injected hormones)
MRI of the head

Treatment Return to top

Treatment depends on the source of the defect.

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

Expectations (prognosis) Return to top

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

Complications Return to top

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 Return to top

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

Prevention Return to top

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.

Update Date: 1/26/2004

Updated by: John Goldenring, M.D., M.P.H, Department of Pediatrics, Children's Hospital, San Diego, CA. Review provided by VeriMed Healtchare Network.

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