Therapy of an acute porphyria attack involves removing any precipitating factors, reducing heme synthesis and addressing the patient's specific needs.
Porphyrias associated with acute attacks: AIP, ALADD, HCP, VP
Treatment of any conditions that may be triggering the attack:
(1) Discontinue and avoid any medication that may be contributing to the attack (substitute a safer alternative if the medicine is critical).
(2) Treat all significant comorbid conditions.
(3) Aggressively treat any infection.
Management of the attack:
(1) Administer a carbohydrate load at 300 grams per day (range 200 to 500). This may be in the form of intravenous dextrose.
(2) Administer intravenous hematin if the attack is severe.
(3) Maintain adequate hydration. If the syndrome of inappropriate antidiuretic hormone (SIADH) develops then restrict fluid intake.
(4) Monitor electrolytes, especially sodium and magnesium.
morphine or fentanyl
agitation and/or anxiety
chlorpromazine (50 to 400 mg/day)
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Specialty: Endocrinology, Clinical Laboratory, Gastroenterology