Updated: 7/22/2020

Androgen Insensitivity Syndrome / Testicular Feminization

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Introduction
  • 46,XY 
  • XR disorder with defect in androgen receptor 
    • results in normal-appearing female with male genotype
  • Loss of negative feedback results in ↑ testosterone and LH
    • ↑ estrogen due to conversion of excess testosterone via aromatase
  • Y chromosome contains the sex determining region of Y (SRY) 
    • encodes the gene for Müllerian Inhibitory Factor (MIF)  
      • produced by Sertoli cells
      • results in degeneration of internal female sex organs
    • promotes testosterone expression
      • produced by Leydig cells
      • can be converted to dihydrotestosterone (DHT) by 5-alpha reductase
  • Lack of DHT effect (due to defective receptor) results in no external male sex organs
Presentation
  • Physical exam
    • rudimentary vagina
    • presents with no sexual hair
    • cryptorchid testes
      • often found in labia majora or inguinal canal
      • surgically removed to prevent malignancy

 

 

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