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Classification

Authoring team

A pathological classification of porphyrias concentrates on the site of the defect in haem synthesis:

  • defects in the liver cause hepatic porphyrias
  • defects in the marrow cause erythropoietic porphyrias

A clinically more useful classification is the division in to acute and non-acute porphyrias:

  • acute poryphrias typically present with abdominal pain and neuropsychiatric crises
  • non-acute porphyrias cause photosensitivity

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