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George H. Sack, Jr, MD, PhD

JAMA. 1990;264(10):1290-1293.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

CASE PRESENTATION

Vincent J. Pompili, MD

A 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; . . . [Full Text PDF of this Article]

Author Affiliations
From the Departments of Medicine, Biological Chemistry, and Pediatrics, The Johns Hopkins University School of Medicine and the Kennedy Institute, Baltimore, Md.

Footnotes
Reprint requests to Division of Medical Genetics, Blalock 1008, The Johns Hopkins Hospital, Baltimore, MD 21205 (Dr Sack).

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