Hypogonadism (Hypogonadism of the Gonads)

Definition

Hypogonadism refers to an underfunction of the gonads (testes, ovaries) with impaired formation or regression of sexual characteristics

Symptoms

Children:

  • Failure to develop puberty

Adolescents:

  • Stagnation of pubertal development
  • Gynecomastia (enlargement of the male mammary gland) and cryptorchidism (undescended testis) in male adolescents
  • Primary amenorrhea (absence of menstrual periods) in girls.
  • Low development of primary and secondary sexual characteristics.
  • Lack of development of sexual interests

Adults:

  • With sufficient masculinization or feminization, the clinical signs may be little pronounced.

Males:

  • Decrease / loss of libido
  • Impotence
  • Absence of mature male sperm cells in the ejaculate (azoospermia).
  • Atrophy of the testicles
  • Osteoporosis
  • Failure of secondary hair

Women:

  • Decrease / loss of libido
  • Estrogen deficiency: secondary amenorrhea, genital atrophy.
  • Osteoporosis
  • Failure of secondary hair

Views

History

  • Most forms of hypogonadism are well treatable
  • Therapy with gonadotropins can treat infertility in secondary hypogonadism.

Causes

Hypergonadotropic (primary) hypogonadism:

  • Disorder at the level of the gonads (testes, ovaries) with compensatory increase in circulating gonadotropins.
  • Klienfelter syndrome
  • Castillo syndrome
  • Traumatic injury
  • Castration
  • Maldescensus testis
  • Orchitis (testicular inflammation)
  • Ovariectomy (removal of the ovaries).
  • Congenital malformation: gonadal agenesis, gonadal dysgenesis.
  • Chemotherapy, radiotherapy
  • Mutations in the LH and/or FSH receptor.
  • Turner syndrome
  • Premature menopause
  • Anorchia (complete inability to function or absence of both testes from birth).
  • Cryptorchidism (undescended testis)
  • Galactosemia (too much galactose in the blood).
  • Noonan syndrome

Hypogonadotropic (secondary) hypogonadism:

  • Malfunction of the neurons that produce GnRH.
  • Inflammatory, tumorous, vascular, or traumatic alteration of the anterior lobe of the hypothalamus/pituitary gland
  • Malnutrition, anorexia nervosa (anorexia).
  • Kallermann syndrome

Mixed form:

  • Age hypogonadism

Complications

  • Infertility
  • Impotence
  • Osteoporosis
  • Cardiovascular disease

Risk factors

  • Kallermann syndrome
  • Klienfelter syndrome
  • Chemotherapy, radiotherapy
  • Malnutrition
  • Genetic predisposition

Diagnosis

  • Diagnosis is made by the physician taking into account the symptoms and the determination of FSH, LH, prolactin, testosterone (male) and estradiol (female) levels in the blood. This allows the doctor to better assess the pituitary situation and hormone conversion.

Differential diagnosis

  • Tumors
  • Underlying diseases that need to be treated in other ways

Non-drug therapy

Surgery (very rare):

  • In women: Because of the significant risk of gonadoblastoma or carcimoma, gonadal tissue should be removed in women who have Turner syndrome.

Drug therapy

  • Treatment of hypogonadism in both sexes consists of substitution of the appropriate sex hormones

In men: androgen substitution

  • Testosterone (oral: Andriol, intramuscular Testoviron Depot).

In women: estrogen substitution:

  • Ethinyl estradiol
  • Estradiol

Progestin substitution:

  • Medroxyprogesterone

If fertility desired:

  • Gonadotropin substitution (GnRH pumps for pulsatile release).

Prevention

  • Prevention depends on the causes
  • A balanced and healthy diet can prevent malnutrition and thus secondary hypogonadism.

Advice

  • The risks of testosterone substitution therapy are increase in prostate volume as well as induction of prostate carcinoma, increase in hematocrit, development of edema and gynecomastia. For this reason, the treatment of hypogonadism in men with testosterone requires a detailed history (prostate cancer) and a physical examination.
  • Hypogonadism in children is usually asymptomatic and is only discovered by the lack of onset of puberty.
  • Diagnosis of adult hypogonadism is often difficult because of nonspecific symptoms.