II. Definition: Hypergonadotropic Hypogonadism
- Defective development of ovaries or Testes
- Associated with excess Pituitary Gonadotropin secretion
- Results in delayed Sexual Development and Growth Delay
III. Etiology
- Gonads deficient in sex Hormone production
- Testes produce less Testosterone
- Ovaries produce less estogen
-
Hypothalamus and pituitary responds
- Increased gonadotropin release (FSH, LH)
IV. Causes
- Variants of Ovarian and Testicular Dysgenesis
- Male: Klinefelter Syndrome (XXY)
- Female: Turner's Syndrome (XO)
- Gonadal Toxins (Chemotherapy/Radiation)
- Cytotoxic drugs (e.g. Chemotherapy)
- Glucocorticoids
- Enzyme defects
- Female: 17 alpha hydroxylase deficiency
- Male: 17 ketosteroid reductase deficiency
- Androgen Insensitivity (Testicular feminization)
- Miscellaneous
- Mumps
- Pelvic radiation
- Gonadal failure (in adults)
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Related Studies
| 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, ｺｳｾｲｾﾝｼｹﾞｷﾎﾙﾓﾝｾｲｾｲｾﾝｷﾉｳﾃｲｶ, ｹﾞﾝﾊﾟ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 |
