Acute intermittent porphyria

Management

• Analgesia
• Glucose load
  • Decreases porphyrin production
  • Typical protocol is D10W 3-4 liters daily x 4 days
  • Risk of hyponatremia given significant free water load
• Hemin
  • Decreases porphyrin production, significantly more potent than glucose
  • Recommended for most cases requiring hospitalization, or any with neurologic symptoms
  • 3-4 mg/kg daily for 4 days
  • Can cause significant infusion site phlebitis - minimize by reconstituting in 25% albumin; consider central venous administration
  • Very expensive - around $8000 per 313 mg vial