Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome
- Author
- Arnaud Vanlander (UGent) , PG Jorens, Joél Smet (UGent) , Boel De Paepe (UGent) , W Verbrugghe, GG Van den Eynden, F Meire, P Pauwels, N Van der Aa, S Seneca, W Lissens, JG Okun and Rudy Van Coster (UGent)
- Organization
- Abstract
- 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.
- Keywords
- GENERAL ANESTHETIC 2, 6-DIISOPROPYLPHENOL, HEREDITARY OPTIC NEUROPATHY, RAT-LIVER MITOCHONDRIA, LACTIC-ACIDOSIS, RESPIRATION, CHILDREN, DISORDERS, DIAGNOSIS, MUTATION, FAILURE
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Citation
Please use this url to cite or link to this publication: http://hdl.handle.net/1854/LU-2130516
- MLA
- Vanlander, Arnaud, et al. “Inborn Oxidative Phosphorylation Defect as Risk Factor for Propofol Infusion Syndrome.” ACTA ANAESTHESIOLOGICA SCANDINAVICA, vol. 56, no. 4, 2012, pp. 520–25, doi:10.1111/j.1399-6576.2011.02628.x.
- APA
- Vanlander, A., Jorens, P., Smet, J., De Paepe, B., Verbrugghe, W., Van den Eynden, G., … Van Coster, R. (2012). Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome. ACTA ANAESTHESIOLOGICA SCANDINAVICA, 56(4), 520–525. https://doi.org/10.1111/j.1399-6576.2011.02628.x
- Chicago author-date
- Vanlander, Arnaud, PG Jorens, Joél Smet, Boel De Paepe, W Verbrugghe, GG Van den Eynden, F Meire, et al. 2012. “Inborn Oxidative Phosphorylation Defect as Risk Factor for Propofol Infusion Syndrome.” ACTA ANAESTHESIOLOGICA SCANDINAVICA 56 (4): 520–25. https://doi.org/10.1111/j.1399-6576.2011.02628.x.
- Chicago author-date (all authors)
- Vanlander, Arnaud, PG Jorens, Joél Smet, Boel De Paepe, W Verbrugghe, GG Van den Eynden, F Meire, P Pauwels, N Van der Aa, S Seneca, W Lissens, JG Okun, and Rudy Van Coster. 2012. “Inborn Oxidative Phosphorylation Defect as Risk Factor for Propofol Infusion Syndrome.” ACTA ANAESTHESIOLOGICA SCANDINAVICA 56 (4): 520–525. doi:10.1111/j.1399-6576.2011.02628.x.
- Vancouver
- 1.Vanlander A, Jorens P, Smet J, De Paepe B, Verbrugghe W, Van den Eynden G, et al. Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome. ACTA ANAESTHESIOLOGICA SCANDINAVICA. 2012;56(4):520–5.
- IEEE
- [1]A. Vanlander et al., “Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome,” ACTA ANAESTHESIOLOGICA SCANDINAVICA, vol. 56, no. 4, pp. 520–525, 2012.
@article{2130516,
abstract = {{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.}},
author = {{Vanlander, Arnaud and Jorens, PG and Smet, Joél and De Paepe, Boel and Verbrugghe, W and Van den Eynden, GG and Meire, F and Pauwels, P and Van der Aa, N and Seneca, S and Lissens, W and Okun, JG and Van Coster, Rudy}},
issn = {{0001-5172}},
journal = {{ACTA ANAESTHESIOLOGICA SCANDINAVICA}},
keywords = {{GENERAL ANESTHETIC 2,6-DIISOPROPYLPHENOL,HEREDITARY OPTIC NEUROPATHY,RAT-LIVER MITOCHONDRIA,LACTIC-ACIDOSIS,RESPIRATION,CHILDREN,DISORDERS,DIAGNOSIS,MUTATION,FAILURE}},
language = {{eng}},
number = {{4}},
pages = {{520--525}},
title = {{Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome}},
url = {{http://doi.org/10.1111/j.1399-6576.2011.02628.x}},
volume = {{56}},
year = {{2012}},
}
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