Hypogonadotropic hypogonadism

Definition

Abnormal ovarian function or testicular function due to insufficient hormonal stimulation from the hypothalamic-pituitary axis.

Location of hypothalamus, pituitary gland and olfactory bulb.

Causes

Hypogonadotropic hypogonadism is due to lack of the pituitary stimulating hormones such as gonadotropin-releasing hormone (GnRH), follicle stimulating hormone (FSH) and luteinizing hormone (LH).

List of causes:

• Damage to the pituitary gland or hypothalamus from surgery, injury, tumor, infection, or radiation
• Genetic defects
• High doses or long-term use of opioid or steroid (glucocorticoid) medicines
• High prolactin level (a hormone released by the pituitary)
• Severe stress
• Nutritional problems (both rapid weight gain or weight loss)
• Long-term (chronic) medical diseases, including chronic inflammation or infections
• Drug use, such as heroin or use or abuse of prescription opiate medicines
• Certain medical conditions, such as iron overload
• Kallmann syndrome is an inherited form of HH. Some people with this condition also have anosmia (loss of the sense of smell).

The genetic and molecular basis of idiopathic hypogonadotropic hypogonadism

Symptoms

Children:

• Lack of development at puberty
• In girls, a lack of breast development and menstrual periods
• In boys, no development of sex characteristics, such as enlargement of the testes and penis, deepening of the voice, and facial hair
• Inability to smell called anosmia
• Short stature

Report on the XYY chromosomal abnormality

Adults:

• Loss of libido in men
• Amenorrhea in women
• Decreased energy and interest in activities
• Loss of muscle mass in men
• Weight gain
• Mood changes
• Infertility

Exams and Tests

The health care provider will perform a physical exam and ask about your symptoms.

Tests that may be done include:

Blood tests to measure hormone levels such as FSH, LH, and TSH, prolactin, testosterone and estradiol

• LH response to GnRH
• MRI of the pituitary gland/hypothalamus (to look for a tumor or other growth)
• Genetic testing
• Blood tests to check for iron level

Treatment

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

Males

• Injections of testosterone
• Slow-release testosterone skin patch
• Testosterone gels

Females

• Estrogen and progesterone pills or skin patches (in females)
• GnRH injections
• HCG injections

Prognosis

The right hormone treatment will cause puberty to start in children and may restore fertility in adults. If the condition begins after puberty or in adulthood, symptoms will often improve with treatment.

Possible Complications

Health problems that may result from HH include:

• Delayed puberty
• Early menopause (in females)
• Infertility
• Low bone density and fractures later in life
• Low self-esteem due to late start of puberty (emotional support may be helpful)
• Sexual problems, such as low libido

ICD

N91.1

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