Porphyria natural history

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Complications

• Patients with hepatic porphyrias (PCT, AIP, HCP, VP) are at increased risk over their life for hepatocellular carcinoma (primary liver cancer) and may require monitoring. Other typical risk factors for liver cancer need not be present, such as hepatitis B or C, iron overload or alcoholic cirrhosis.
• The neuropathic effects can progress and become severe leading to irreversible neurologic damage
  • Guillian-Barre type syndrome with paralysis,
  • Bulbar dysfunction,
  • Respiratory failure
  • Death