| Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome. | |
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MedLine Citation:
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PMID: 22260353 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Propofol is an anesthetic agent widely used for induction and maintenance of anesthesia, and sedation in children. Although generally considered as reliable and safe, administration of propofol can occasionally induce a potentially fatal complication known as propofol infusion syndrome (PRIS). Mitochondrial dysfunction has been implicated in the pathogenesis of PRIS. We report on an adult patient with Leber hereditary optic neuropathy (LHON) who developed PRIS. He was a carrier of the m.3460G>A mutation, one of the major three pathogenic point mutations associated with LHON. The propositus was blind and underwent propofol sedation after severe head injury. Five days after start of propofol infusion, the patient died. The activity of complex I of the oxidative phosphorylation (OXPHOS) system was severely deficient in skeletal muscle. Our observation indicates that fulminate PRIS can occur in an adult patient with an inborn OXPHOS defect and corroborates the hypothesis that PRIS is caused by inhibition of the OXPHOS system. |
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Authors:
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A V Vanlander; P G Jorens; J Smet; B DE Paepe; W Verbrugghe; G G VAN DEN Eynden; F Meire; P Pauwels; N VAN DER Aa; S Seneca; W Lissens; J G Okun; R VAN Coster |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2012-1-19 |
Journal Detail:
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Title: Acta anaesthesiologica Scandinavica Volume: - ISSN: 1399-6576 ISO Abbreviation: - Publication Date: 2012 Jan |
Date Detail:
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Created Date: 2012-1-20 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0370270 Medline TA: Acta Anaesthesiol Scand Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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© 2012 The Authors. Acta Anaesthesiologica Scandinavica © 2012 The Acta Anaesthesiologica Scandinavica Foundation. |
Affiliation:
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Department of Pediatrics, Division of Pediatric Neurology and Metabolism, Ghent University Hospital, Ghent, Belgium. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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