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| ==Background==
| | #REDIRECT[[Porphyria]] |
| *Acute Intermittent Porphyria is a disorder caused by the inability to produce heme, a component of hemoglobin in red blood cells.
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| *The defective enzyme is porphobilinogen deaminase.
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| *Patients typically present with dark urine, abdominal pain, and psychiatric disturbances
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| ===Triggers===
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| *[[Infection]]
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| *Metabolic stress and starvation
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| *[[ETOH]]
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| *CYP450 Inducers: [[sulfonamides]], [[barbiturates]], [[rifampin]] or [[metoclopramide]]
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| ==Clinical Features==
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| *Gastrointestinal symptoms
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| **Acute [[abdominal pain]] (85-90% of attacks)
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| **Port wine-colored urine
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| **[[Agitation]], [[confusion]], combativeness, [[seizure]]
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| ==Differential Diagnosis==
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| {{Extra-abdominal sources of abdominal pain DDX}}
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| ==Evaluation==
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| ''Consider porphyria in patients with abdominal pain that is unexplained after an initial workup has excluded common causes (appendicitis, cholecystitis, pancreatitis, etc).''
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| *Spot urinary porphobilinogen (sendout at most hospitals)
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| **Normal = 0-4mg/day
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| **acute attack, spot urine can be 20-200mg/L
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| *Recurrent attacks in a patient with proven acute porphyria are usually similar and can be diagnosed on clinical grounds without biochemical reconfirmation.
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| ==Management<ref>https://emedicine.medscape.com/article/205220-treatment</ref>==
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| *Goal to decrease heme synthesis and reduce production of porphyrin precursors
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| *High doses of glucose, e.g. [[dextrose|D5]]NS at 2L/hr x 24h
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| **Avoid D5W or D10W as may aggravate [[hyponatremia]]
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| *Severe attacks, especially with severe neuro symptoms: hematin 4mg/kg/d x 4d
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| ==Disposition==
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| ==See Also==
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| *[[Abdominal pain]]
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| *[[Porphyria]]
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| ==References==
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| ==Video==
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| {{#widget:YouTube|id=VQHz0Qu-OjA}}
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| [[Category:Heme/Onc]]
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