Adrenogenital Syndrome

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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