Porphyria: treatment
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Porphyria: treatment. Identify and remove triggers, Stop porphyrinogenic drugs, Heme arginate. Fully referenced neurology checklist.
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
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Treatments of acute porphyria pain
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Safe antiepileptic drugs in porphyria
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Unsafe antiepileptic drugs in porphyria
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References
- Gerischer LM, Scheibe F, Nümann A, Köhnlein M, Stölzel U, Meisel A. Acute porphyrias - a neurological perspective. Brain Behav 2021; 11:e2389.
- Thadani H, Deacon A, Peters T. Diagnosis and management of porphyria. BMJ 2000; 320:1647-1651.
- 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).
- 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.
