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