Porphyria natural history: Difference between revisions
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* 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. | * 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 | * The neuropathic effects can progress and become severe leading to irreversible neurologic damage | ||
** [[Guillian-Barre]] type syndrome with paralysis | ** [[Guillian-Barre]] type syndrome with [[paralysis]] | ||
** [[Bulbar dysfunction]] | ** [[Bulbar dysfunction]] | ||
** [[Respiratory failure]] | ** [[Respiratory failure]] | ||
** [[Death]] | ** [[Death]] |
Revision as of 13:40, 22 August 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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