Porphyria: treatment

Evidence-based neurology checklist on porphyria: treatment: Treatments of acute porphyria Identify and remove triggers Stop porphyrinogenic drugs Heme arginate: Intravenous 10% glucose: to suppress haem biosynthesis Betablockers: for hypertension and tachycardia Pyridoxine Fluid and electrolyte…

Treatments of acute porphyria

  • Identify and remove triggers
  • Stop porphyrinogenic drugs
  • Heme arginate:
  • Intravenous 10% glucose: to suppress haem biosynthesis
  • Betablockers: for hypertension and tachycardia
  • Pyridoxine
  • Fluid and electrolyte therapy
  • Intensive care management

Treatments of recurrent acute porphyria attacks

Treatments of acute porphyria pain

Safe antiepileptic drugs in porphyria

Unsafe antiepileptic drugs in porphyria

References

  1. Gerischer LM, Scheibe F, Nümann A, Köhnlein M, Stölzel U, Meisel A. Acute porphyrias - a neurological perspective. Brain Behav 2021; 11:e2389.
  2. Thadani H, Deacon A, Peters T. Diagnosis and management of porphyria. BMJ 2000; 320:1647-1651.
  3. Kuter DJ, Bonkovsky HL, Monroy S, et al. Efficacy and safety of givosiran for acute hepatic porphyria: Final results of the randomized phase III ENVISION trial. J Hepatol 2023 (Online ahead of print).
  4. Patel P, Midha S, Shukla S, Dhamija D, Bello AO, Khan S. Evaluating the efficacy of a small interfering ribonucleic acid molecule, givosiran, in treating acute intermittent porphyria: a systematic review. Cureus 2023; 15:e40585.

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