Ver el código de «Acute intermittent porphyria»

==Background==
*Related to defect(s) in heme synthesis causing a buildup of porphyrins
*Autosomal dominant, but poor penetrance

==Clinical Features==

==Differential Diagnosis==

==Triggers==
*Tobacco, EtOH

==Diagnosis==
*Unlikely to diagnose first episode in ED given rarity of disease
*Can check spot urine porphobilinogen (PBG) - sendout at most hospitals
==Management==
*Analgesia
*Avoid offending meds
**Most seizure meds contraindicated. [[Benzodiazepines]], [[gabapentin]], [[levetiracetam]], and [[vigabatrin]] OK
**Avoid [[reglan]]
*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/

==References==
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