Document Detail


Acute intermittent porphyria as a cause of respiratory failure: case report.
MedLine Citation:
PMID:  19255109     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Folkert W Asselbergs; Ton K Kremer Hovinga; Cees Bouwsma; Jan van Ingen
Related Documents :
3390249 - Field airway management of the trauma patient: the efficacy of bag mask ventilation.
21439419 - Analysis of five cases with neurogenic stuttering following brain injury in the basal g...
17453639 - A comparison of maximal inspiratory pressure and forced vital capacity as potential cri...
19770169 - Body mass index is associated with the development of acute respiratory distress syndrome.
10533019 - Rapid reversal of life-threatening diltiazem-induced tetany with calcium chloride.
19475349 - High-frequency oscillatory ventilation for acute respiratory distress syndrome.
16948289 - Development and testing of a new outcome measure of relationship between patients with ...
6229179 - Fc-specific reticulo-endothelial clearance in systemic lupus erythematosus and glomerul...
3149149 - Juvenile galactosialidosis in a white male: a new variant.
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    
Affiliation:
Department of Cardiology, University Medical Center Groningen, and Department of Internal Medicine, Martini Hospital, Groningen, the Netherlands. fwasselbergs@hotmail.com
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
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


Previous Document:  Medical end-of-life decisions: experiences and attitudes of belgian pediatric intensive care nurses.
Next Document:  Infections with oseltamivir-resistant influenza A(H1N1) virus in the United States.