Sex

Hypogonadism

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Hypogonadism

  • Definitions
  1. Hypogonadism
    1. Decreased testicular or ovarian functional activity
  • Causes
  • Hypogonadism in Children
  1. See Delayed Puberty
  2. See Primary Amenorrhea
  3. Hypogonadotropic Causes of Delayed Puberty
    1. Pituitary failure to secrete gonadotropins, results in decreased functional activity of ovaries or Testes
    2. Presents with new onset Growth Delay without pubertal growth spurt after normal growth in childhood
    3. Causes
      1. Endocrine Disorders
        1. Isolated Growth Hormone Deficiency
        2. Isolated Gonadotropin deficiency (e.g. Kallmann's Syndrome, Congenital adrenal hypoplasia)
        3. Hypothyroidism
        4. Diabetes Mellitus
        5. Glucocorticoid excess (Cushing Syndrome)
        6. Hyperprolactinemia
      2. Other Conditions
        1. CNS Infection, radiation, Head Trauma or Brain Tumor
        2. Prader-Willi Syndrome (Obesity, Short Stature, Mild Mental Retardation)
        3. Chronic conditions (e.g. CKD, Cystic Fibrosis, Sickle Cell Anemia, HIV Infection, Gaucher's Disease)
        4. Chronic Opioid use (86% of users)
        5. Nutritional deficiency OR extreme Energy Expenditure (e.g. Female Athlete Triad)
        6. Constitutional Delay in Growth and Development
  4. Hypergonadotropic Causes of Delayed Puberty
    1. Defective development of ovaries or Testes with decreased sex Hormones (Testosterone, Estradiol)
    2. Sex Hormone deficiency triggers increased gonadotropins (FSH, LH)
    3. Causes
      1. Chromosomal abnormalities
        1. Ovarian Dysgenesis (Turner's Syndrome, XO)
        2. Testicular Dysgenesis (Klinefelter Syndrome, XXY)
      2. Gonadal Toxins (Chemotherapy/Radiation)
      3. Enzyme defects
        1. Female: 17 alpha hydroxylase deficiency
        2. Male: 17 ketosteroid reductase deficiency
      4. Androgen Insensitivity (Testicular feminization)
      5. Miscellaneous (e.g. Mumps, Pelvic radiation
  5. Eugonadotropic Causes of Delayed Puberty
    1. Delayed Sexual Development and Growth Delay despite normal Pituitary Gonadotropin secretion
    2. Delayed Menarche causes
      1. Gonadal dysgenesis variants (residually functioning ovarian tissue)
      2. Abnormalities Mullerian duct development (absence of Uterus and possibly Vagina)
      3. Polycystic Ovarian disease
      4. Hyperprolactinemia