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Acute Intermittent Porphyria

George H. Sack Jr, MD, PhD
JAMA. 1990;264(10):1290-1293. doi:10.1001/jama.1990.03450100080031.
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CASE PRESENTATION  Vincent J. Pompili, MDA 24-YEAR-OLD white woman was known to have acute intermittent porphyria (AIP). She completed a normal pregnancy and delivery 16 weeks ago; 14 weeks later, 2 weeks prior to admission to the hospital, she stopped breast-feeding. On the day before admission she had the acute onset of neck, back, and arm pain and weakness. These symptoms worsened over 18 hours, accompanied by nausea, vomiting, and shortness of breath. Breathing became more difficult and respiratory muscle weakness developed. She was intubated, given assisted respiratory ventilation, and admitted to the hospital.Her medical history is notable for three attacks of AIP with abdominal pain and vomiting. She took no medications. There were no known Swedish relatives, but her maternal grandfather, a maternal aunt, and her mother had AIP.On admission she was lethargic but oriented. Her vital signs were as follows: blood pressure, 120/70 mm Hg;


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