Acute intermittent porphyria: Difference between revisions
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==External Links== | ==External Links== | ||
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Revision as of 22:01, 23 September 2015
Background
Clinical Features
Differential Diagnosis
Diagnosis
Management
- Analgesia
- Glucose load
- Decreases porphyrin production
- Typical protocol is D10W 3-4 liters daily x 4 days
- Risk of hyponatremia given significant free water load
- Hemin
- Decreases porphyrin production, significantly more potent than glucose
- Recommended for most cases requiring hospitalization, or any with neurologic symptoms
- 3-4 mg/kg daily for 4 days
- Can cause significant infusion site phlebitis - minimize by reconstituting in 25% albumin; consider central venous administration
- Very expensive - around $8000 per 313 mg vial
Disposition
See Also
External Links
http://www.porphyriafoundation.com/