Document Detail

Acute intermittent porphyria as a cause of respiratory failure: case report.
MedLine Citation:
PMID:  19255109     Owner:  NLM     Status:  MEDLINE    
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.
Folkert W Asselbergs; Ton K Kremer Hovinga; Cees Bouwsma; Jan van Ingen
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
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:
Created Date:  2009-03-03     Completed Date:  2009-05-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9211547     Medline TA:  Am J Crit Care     Country:  United States    
Other Details:
Languages:  eng     Pagination:  180, 178-9     Citation Subset:  IM; N    
Department of Cardiology, University Medical Center Groningen, and Department of Internal Medicine, Martini Hospital, Groningen, the Netherlands.
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MeSH Terms
Chronic Disease
Fatal Outcome
Kidney Failure, Chronic / complications
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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