Hereditary Coproporphyria Medication

Updated: Mar 22, 2021
  • Author: Thomas G DeLoughery, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications. Panhematin is the drug of choice for severe attacks and may be used long-term for patients with recurrent attacks. Narcotics are used to control pain, and gabapentin is used to control seizures.

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Blood products

Class Summary

The key treatment of porphyria is stopping heme synthesis. Hematin provides negative feedback to the heme synthetic pathway and shuts down productions of porphyrins and porphyrin precursors.

Panhematin (Hemin)

DOC for severe porphyria attacks. Enzyme inhibitor derived from processed red blood cells and an iron-containing metalloporphyrin. Was previously known as hematin, a term used to describe the chemical reaction product of hemin and sodium carbonate solution.

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GABA Analogs

Gabapentin (Gralise, Neurontin)

GABA analogue that is structurally related to neurotransmitter GABA, but has no effect on GABA binding, uptake, or degradation; presence of gabapentin binding sites throughout the brain reported; mechanism for analgesic and anticonvulsant activity unknown. Indicated for adjunctive therapy for partial seizures with or without secondary generalization.

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