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American Journal of Critical Care. 2009;18: 180-178 doi:10.4037/ajcc2009196
Copyright © 2009 by the American Association of Critical-Care Nurses.
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Cases of Note features peer-reviewed case reports and case series that document clinically relevant findings from critical and high acuity care environments. Cases that illuminate a clinical diagnosis or a management issue in the treatment of critically and acutely ill patients and include discussion of the patient’s experience with the illness or intervention are encouraged. Proposals for future Cases of Note articles may be e-mailed to ajcc{at}aacn.org.

Acute Intermittent Porphyria as a Cause of Respiratory Failure: Case Report

By Folkert W. Asselbergs, MD, PhD, Ton K. Kremer Hovinga, MD, PhD, Cees Bouwsma, MD and Jan van Ingen, MD, PhD. Folkert W. Asselbergsis a fellow in the Department of Cardiology, University Medical Center Groningen, Groningen, the Netherlands. Ton K. Kremer Hovingais a nephrologist in the Department of Internal Medicine, Cees Bouwsmais a neurologist in the Department of Neurology, and Jan van Ingenis an intensivist in the Department of Intensive Care Medicine, Martini Hospital, Groningen, the Netherlands.

Corresponding author: Folkert W. Asselbergs, Department of Cardiology, University Medical Center Groningen, Hanzeplein 1, 9713 GZ, Groningen, the Netherlands (e-mail: fwasselbergs{at}hotmail.com).

A 45-year-old man with end-stage renal disease due to polycystic kidney disease was admitted to the hospital because of recurrent abdominal pain, progressive peripheral motor neuron neuropathy, and respiratory failure. The diagnosis of acute intermittent porphyria was confirmed by an elevated porphyrin concentration in the urine and the presence of an R167Q mutation in the porphobilinogen deaminase gene. Use of hydroxyzine, weight loss, and/or a mild upper respiratory viral infection might have been the provoking factor of the acute intermittent porphyria. Treatment with intravenous hemin (3 mg/kg) and a high-carbohydrate diet (3000 kcal/d) had no clinical effect. Tetraplegia and chronic respiratory insufficiency developed, and the patient needed a pacemaker because of a symptomatic sinus bradycardia due to autonomic dysfunction. The patient died 10 months after the first manifestation of acute intermittent porphyria.






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Copyright © 2009 by the American Association of Critical-Care Nurses.