Congenital Adrenal Enzyme Deficiencies Lecture Notes

Overview

• Presented by Dirty Medicine
• Focus on understanding key adrenal enzyme deficiencies relevant for USMLE and COMLEX, not memorizing complicated diagrams.

Key Adrenal Enzyme Deficiencies

1. High-Yield Deficiencies
   • 21-Hydroxylase Deficiency
   • 17-Alpha Hydroxylase Deficiency
2. Low-Yield
   • Other deficiencies not recommended for memorization unless extra capacity.

High-Yield Information to Differentiate Between the Two Deficiencies

• Important factors:
  • Mineralocorticoids
  • Sex Hormones
  • Blood Pressure
  • Sodium
  • Potassium

Mnemonic Story for Remembering

• Characters:
  • 21-Year-Old (in college)
  • 17-Year-Old (stuck in high school)

Details of the Mnemonic

1. 21-Year-Old (21 Hydroxylase Deficiency):

   • Happy and manly
   • Increased Sex Hormones
   • Decreased Mineralocorticoids (e.g., Aldosterone)
   • Results:
     • Decreased Sodium
     • Decreased Blood Pressure
     • Increased Potassium

2. 17-Year-Old (17 Alpha Hydroxylase Deficiency):

   • Upset and not manly
   • Decreased Sex Hormones
   • Increased Mineralocorticoids (e.g., Aldosterone)
   • Results:
     • Increased Sodium
     • Increased Blood Pressure
     • Decreased Potassium

Review of Differences

• 21 Hydroxylase Deficiency:

  • Happy (not salty)
  • Increased sex hormones
  • Decreased mineralocorticoids →
    • Decreased sodium
    • Decreased blood pressure
    • Increased potassium

• 17 Alpha Hydroxylase Deficiency:

  • Salty (upset)
  • Decreased sex hormones
  • Increased mineralocorticoids →
    • Increased sodium
    • Increased blood pressure
    • Decreased potassium

Conclusion

• Use the mnemonic to differentiate the two adrenal enzyme deficiencies successfully.
• Remember to relate the age to the conditions for easy recall during exams.

Good luck!