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Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome

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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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Chicago
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): 520–525.
APA
Vanlander, Arnaud, Jorens, P., Smet, J., De Paepe, B., Verbrugghe, W., Van den Eynden, G., Meire, F., et al. (2012). Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome. ACTA ANAESTHESIOLOGICA SCANDINAVICA, 56(4), 520–525.
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.
MLA
Vanlander, Arnaud, PG Jorens, Joél Smet, et al. “Inborn Oxidative Phosphorylation Defect as Risk Factor for Propofol Infusion Syndrome.” ACTA ANAESTHESIOLOGICA SCANDINAVICA 56.4 (2012): 520–525. Print.
@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{\textrangle}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, Jo{\'e}l 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},
  keyword      = {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://dx.doi.org/10.1111/j.1399-6576.2011.02628.x},
  volume       = {56},
  year         = {2012},
}

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