A case report of porphyria variegata management in the emergency department.
BACKGROUND
Porphyria variegata (VP) is one of the hepatic porphyrias that results from the deficiency of protoporphyrinogen oxidase, an enzyme in the heme synthesis pathway. The name porphyria variegata refers to its many presentations, which include various neuropsychiatric and cutaneous manifestations. Emergency department (ED) presentations due to VP are most commonly neuropathic abdominal pain.


METHODS
We present the case of a 57-year-old woman presenting to an ED with abdominal pain consistent with prior VP attacks. In addition to analgesics and supportive care, infusion of intravenous dextrose resulted in improvement in her symptoms.


CONCLUSIONS
Intravenous dextrose and heme administration remain the first-line treatment for abdominal pain attributable to known acute hepatic porphyria attacks. Recently, the mechanism of action of carbohydrates in treating porphyria has been elucidated. Current information on this illness and ED management are discussed.
Chen
Betty C
BC
Division of Emergency Medicine, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
Griffey
Richard T
RT
D015746:Abdominal Pain / Q000209:etiology / Q000628:therapy*; D000701:Analgesics, Opioid / Q000627:therapeutic use; D005260:Female; D005440:Fluid Therapy; D005947:Glucose / Q000627:therapeutic use; D006801:Humans; D004091:Hydromorphone / Q000627:therapeutic use; D008875:Middle Aged; D046350:Porphyria, Variegate / Q000150:complications / Q000628:therapy*
D000701:Analgesics, Opioid; D005947:Glucose; D004091:Hydromorphone