| Acute intermittent porphyria as a cause of respiratory failure: case report. | |
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MedLine Citation:
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PMID: 19255109 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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Folkert W Asselbergs; Ton K Kremer Hovinga; Cees Bouwsma; Jan van Ingen |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: American journal of critical care : an official publication, American Association of Critical-Care Nurses Volume: 18 ISSN: 1062-3264 ISO Abbreviation: Am. J. Crit. Care Publication Date: 2009 Mar |
Date Detail:
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Created Date: 2009-03-03 Completed Date: 2009-05-11 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9211547 Medline TA: Am J Crit Care Country: United States |
Other Details:
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Languages: eng Pagination: 180, 178-9 Citation Subset: IM; N |
Affiliation:
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Department of Cardiology, University Medical Center Groningen, and Department of Internal Medicine, Martini Hospital, Groningen, the Netherlands. fwasselbergs@hotmail.com |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Chronic Disease Fatal Outcome Humans Kidney Failure, Chronic / complications Male Middle Aged Peripheral Nervous System Diseases / complications Polycystic Kidney Diseases / complications Porphyria, Acute Intermittent / etiology*, therapy Respiratory Insufficiency / etiology* |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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