II. Definition: Hypergonadotropic Hypogonadism

  1. Defective development of ovaries or Testes
  2. Associated with excess pituitary gonadotropin secretion
  3. Results in delayed Sexual Development and Growth Delay

III. Etiology

  1. Gonads deficient in sex Hormone production
    1. Testes produce less Testosterone
    2. Ovaries produce less estogen
  2. Hypothalamus and pituitary responds
    1. Increased gonadotropin release (FSH, LH)

IV. Causes

  1. Variants of Ovarian and Testicular Dysgenesis
    1. Male: Klinefelter Syndrome (XXY)
    2. Female: Turner's Syndrome (XO)
  2. Gonadal Toxins (Chemotherapy/Radiation)
    1. Cytotoxic drugs (e.g. Chemotherapy)
    2. Glucocorticoids
  3. Enzyme defects
    1. Female: 17 alpha hydroxylase deficiency
    2. Male: 17 ketosteroid reductase deficiency
  4. Androgen Insensitivity (Testicular feminization)
  5. Miscellaneous
    1. Mumps
    2. Pelvic radiation
  6. Gonadal failure (in adults)
    1. Hypogonadism in Men
      1. Androgen Decline in the Aging Male
      2. Testicular Failure
    2. Hypogonadism in Women
      1. Menopause
      2. Premature Ovarian Failure

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