What is hypogonadotropic hypogonadism?

Hypogonadotropic hypogonadism (often called secondary hypogonadism) is a condition in which the hypothalamus or the pituitary gland do not produce the hormones that signal to the testes (in males) or the ovaries (in females) to produce sex hormones.

The hypothalamus is a small structure in the brain that controls the function of the pituitary gland. The pituitary gland is a small, pea-sized gland in the brain that controls many other glands in the body. Hormones are chemicals in the body that act as messengers that enable the body to function properly.

What are the clinical features of hypogonadotropic hypogonadism?

Clinical features of hypogonadotropic hypogonadism in children may vary depending on the time of the onset of this condition. Possible features include the following:

  • Absence or incomplete development of secondary sex characteristics (physical changes that happen during puberty, such as breast development in girls and increased size of the testes, facial hair and voice changes in boys)
  • Little development of hair in the pubic and axillary areas (under the arms) during puberty
  • Reduced muscle mass in boys
  • Reduced bone mass/density
  • Anemia (when the body does not make enough red blood cells)
  • Very low testosterone or estrogen levels

What causes hypogonadotropic hypogonadism?

Hypogonadotropic hypogonadism occurs when there is a problem in the hypothalamus or pituitary gland that prevents the secretion of sex hormones. The causes include genetic or other factors:

Genetic causes

  • Kallmann syndrome (a genetic condition that causes delayed or absent puberty and little to no sense of smell). People with Kallmann syndrome may have other physical features of this condition as well.
  • Idiopathic hypogonadotropic hypogonadism (IHH). When a condition has an unknown cause, it is called idiopathic. In this form of hypogonadotropic hypogonadism, the sense of smell is intact. Many people with IHH have genetic mutations causing this condition.

External factors

  • Low weight, excessive exercise or chronic stress
  • Damage to the pituitary gland or hypothalamus (such as from a tumor, injury, infection, inflammation or radiation)
  • Certain chronic medical conditions

What is hypothalamic amenorrhea?

Hypothalamic amenorrhea is a condition in which females do not menstruate (get their periods) because of hypogonadotropic hypogonadism resulting from low weight, excessive exercise or chronic stress. This stops both ovulation (when the ovaries release a mature egg into the fallopian tubes, the tubes that connect to the uterus) and menstruation. When ovulation and menstruation stop, females are temporarily infertile (unable to reproduce).

Rev. 3/2022. MassGeneral Hospital for Children and Massachusetts General Hospital do not endorse any of the brands listed on this handout. This handout is intended to provide health information so that you can be better informed. It is not a substitute for medical advice and should not be used to treatment of any medical conditions.