Hypogonadism

Clinic

• Diminished functional activity of the gonads which results in diminished production of sex hormones.
  • Hypoandrogenism (Low androgen e.g., testosterone)
  • Hypoestrogenism (Low estrogen e.g., estradiol)

• Hypogonadism commonly referred to Low testosterone and other hormones including progesterone, DHEA, anti-Müllerian hormone, activin and inhibin.
• Spermatogenesis and Ovulation may be impaired which causes infertility.
• Hypogonadism can involve just hormone production or just fertility, but most commonly involves both.
  1. Hypopituitarism and Kallmann syndrome: Hypogonadism that affect hormone production more than fertility
  2. Klinefelter syndrome and Kartagener syndrome: Hypogonadism that affect fertility more than hormone production

Types

(1) Primary hypogonadism

• LH and/or FSH are usually elevated, since the main problem is in Testicles / Ovaries (Hyper-gonatropic hypogonadism)

• Causations:
  1. Genetic: Klinefelter syndrome, Turner syndrome, Noonan syndrome, XY with SRY gene-immunity
  2. Acquired: MMP

(2) Secondary hypogonadism

• In secondary hypogonadism, LH / FSH are normal or low, suggesting the problem is in the brain
• Causations:
  1. Hypogonadotropic hypogonadism (HH) due to Hypopituitarism / Pituitary hypoplasia.
  2. Lack of hormone response is Androgen Insensitivity Syndrome
  3. Isolated hypogonadotropic hypogonadism (IHH)= Idiopathic or congenital hypogonadotropic hypogonadism (CHH): HH due to deficiency in or insensitivity to GnRH where the function and anatomy of the anterior pituitary is normal and secondary causes of HH are not present
• Examples are
  • PCOS
  • Kallmann syndrome
  • Hemochromatosis
  • Diabetes mellitus

Central Hypogonadism

• Central hypogonadism referring to CNS / Hypothalamic defects including:
  • Genetic disorders such as Kallmann syndrome (Abnormal hypothalamic development)
  • Infections including HIV
  • Pituitary disorders
  • Inflammatory diseases including sarcoidosis, MTB and histiocytosis

Other

Hypogonadism can occur in other conditions, like Prader–Willi syndrome.

Signs / Symptoms

• Women: Amenorrhea, Decreased height+ Impaired Breast development Or Cessation of menstruation + Lowered libido + Loss of body hair + Hot flashes.
• Men: Impaired muscle + Impaired body hair development + Gynecomastia + Decreased height + Erectile dysfunction + Sexual difficulties.
• Central hypogonadism: Headaches + Impaired vision / Double vision + Milky discharge from the breast (Prolactin) + Symptoms caused by other hormone problems.

Hypogonadotrophic hypogonadism

• It is a subtype of hypogonadism
• Late, incomplete or lack of development at puberty
• Sometimes short stature or the inability to smell
• In females, a lack of breasts and menstrual periods, and in males a lack of sexual development, e.g., facial hair, penis and testes enlargement, deepening voice.

Clinic

• HH is a kind of Secondary/ Central hypogonadism
• It means that problems with either the hypothalamus or pituitary gland affecting the hypothalamic-pituitary-gonadal axis (HPG axis) cause HH.
• Hypothalamic disorders result from a deficiency in the release of GnRH
• Pituitary gland disorders are due to a deficiency in the release of gonadotropins from the anterior pituitary.

Two Types

1. Congenital HH: CHH is due to genetic abnormalities resulting in non-functional GnRH secreting neurons or gonadotropic cell dysfunction in the anterior pituitary. CHH is divided into 2 subtypes depending on the condition of the olfactory system, anosmic HH (Kallman syndrome) and normosmic HH.
2. Acquired HH: AHH is an acquired form of the disease often occurring after sexual maturation and is not related to genetic defects.

Acquired hypogonadotropic hypogonadism

• AHH is a postnatal onset of a GnRH releasing disorder and/or pituitary gonadotroph cell disorder.
• There are many causes of AHH, mostly due to structural or functional abnormalities of HPG axis such as
  • Sarcoidosis
  • Lymphocytic hypophysitis
  • Pituitary adenomas, craniopharyngiomas and other CNS tumours
• Most of these patients have multiple pituitary hormone deficiencies.
• Hyperprolactinaemia is the most common cause of AHH. It is a well-established cause of infertility in both male and female mammals. Prolactin inhibits GnRH neurons and therefore inhibits the subsequent release of LH, FSH and sex steroids.

Entity / Miasms

MTB

HPV