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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1 March 2009
Cases of Note|
March 01 2009
Acute Intermittent Porphyria as a Cause of Respiratory Failure: Case Report
Folkert W. Asselbergs, MD, PhD;
Folkert W. Asselbergs, 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.
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Ton K. Kremer Hovinga, MD, PhD;
Ton K. Kremer Hovinga, 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.
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Cees Bouwsma, MD;
Cees Bouwsma, MD
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.
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Jan van Ingen, MD, PhD
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.
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Am J Crit Care (2009) 18 (2): 180.
Citation
Folkert W. Asselbergs, Ton K. Kremer Hovinga, Cees Bouwsma, Jan van Ingen; Acute Intermittent Porphyria as a Cause of Respiratory Failure: Case Report. Am J Crit Care 1 March 2009; 18 (2): 180–178. doi: https://doi.org/10.4037/ajcc2009196
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