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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Ontology: Primary hypogonadism (C0948896)

Definition (NCI) Ovarian or testicular dysfunction associated with high levels of gonadotropins.(NICHD)
Concepts Disease or Syndrome (T047)
MSH D007006
SnomedCT 370999003
English Primary hypogonadism, hypergonadotropic hypogonadism, primary hypogonadism, Hypergonadotrophic hypogonadism, Primary Gonadal Failure, Hypergonadotropic hypogonadism, Primary hypogonadism (disorder), Hypergonadotropic Hypogonadism, Hypogonadism, Hypergonadotropic
Italian Ipogonadismo primitivo, Ipogonadismo ipergonadotropo
Dutch hypergonadotroop hypogonadisme, primair hypogonadisme
German hypergonadotroper Hypogonadismus, primaerer Hypogonadismus
Portuguese Hipogonadismo hipergonadotrópico, Hipogonadismo primário
Spanish Hipergonadismo hipergonadotrópico, hipogonadismo hipergonadotrófico, hipogonadismo primario (trastorno), hipogonadismo primario, Hipogonadismo primario
Japanese 高性腺刺激ホルモン性性腺機能低下, 原発性性腺機能低下, コウセイセンシゲキホルモンセイセイセンキノウテイカ, ゲンパツセイセイセンキノウテイカ
French Hypogonadisme hypergonadotrophique, Hypogonadisme primaire
Czech Hypergonadotropní hypogonadismus, Primární hypogonadismus
Hungarian Primaer hypogonadismus, Hypergonadotrop hypogonadismus