Adrenogenital Syndrome
Aetiology
- Genotype is XX as should be female
- Ovaries develop normally
- Foetal Adrenal Gland is hyperactive and secretes large quantities of Adrenal Androgens, due to a 21 hydroxylase mutation.
Consequence
- Wolffian Ducts persist due to the presence of Androgens, thus allowing development of male internal and external genitalia.
- Mullarian Ducts persist due to the absence of testes, hence absence of Sertoli cell MIH secretion. This allows formation of female internal genitalia.
Morphology
- Appears male with a micropenis and scrotum
- Both male and female internal genitalia