Acute Intermittent Porphyria (AIP)

Various triggers
  • Alcohol
  • Medications
    • Barbiturates
    • Anesthetics
    • Sulfonamides
    • Griseofulvin
    • Antimalarials
    • Phenylbutazone
  • Hormonal factors
    • Menstual cycle
    • Estrogen administration
  • Infections
  • Weight loss
  • Prolonged fasting
 
Acute attacks with no skin manifestations
  • Abdominal syndrome
    • Abdmonial pain
    • Constipation
    • Vomiting
    • Pseudoappendicitis
  • Neurological syndrome
    • Polymorphic
    • Polyneuritis
    • Peripheral and cranial nerve paralysis
    • Convulsions
    • Respiratory paralysis
  • Psychological syndrome
    • Dementia, Korsakoff, delirium tremens, confusion
 
Labs
  • Porphyrin precursors: porphbilinogen and ALA in urine
  • Urine becomes dark due to oxidation of precursors into porphyrins
 
Treatment
  • Avoid triggers
  • Glucose serum infusions and hematin