Porphyria natural history

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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Overview

Natural History

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

Prognosis

References

Overview