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Anesth Analg 2009; 109:1070-1072
© 2009 International Anesthesia Research Society
doi: 10.1213/ane.0b013e3181ad63b4
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PEDIATRIC ANESTHESIOLOGY

Malignant Hyperthermia-Like Syndrome and Carnitine Palmitoyltransferase II Deficiency with Heterozygous R503C Mutation

Kirk J. Hogan, MD, JD*, and Georgirene D. Vladutiu, PhD{dagger}

From the *Department of Anesthesiology, School of Medicine and Public Health, University of Wisconsin, Madison, Wisconsin; and {dagger}Department of Pediatrics, Neurology, and Pathology & Anatomical Sciences, School of Medicine and Biomedical Sciences, State University of New York at Buffalo, Buffalo, New York.

Address correspondence to Kirk J. Hogan, MD, JD, Department of Anesthesiology, School of Medicine and Public Health, University of Wisconsin, B6/319 Clinical Sciences Center, 600 Highland Ave., Madison, WI 53792-3272. Address e-mail to khogan{at}facstaff.wisc.edu.

Abstract

We describe a child who developed a malignant hyperthermia-like syndrome after exposure to succinylcholine and halothane. Many features of a typical malignant hyperthermia episode were present, including tachydysrhythmia, tachypnea, and fever in association with metabolic acidosis, hyperCKemia, myglobinemia, and rapid recovery without residual effects upon administration of dantrolene, sodium bicarbonate, and active cooling. Muscle rigidity, hypercarbia, and hyperkalemia were not observed. The patient was found to be heterozygous for a mutation in the carnitine palmitoyltransferase II gene (CPT2) encoding an arginine to cysteine substitution at amino acid 503 (R503C) with reduced activity of the enzyme.




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Anesth. Analg., October 1, 2009; 109(4): 1001 - 1003.
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J. Benca and K. Hogan
Malignant Hyperthermia, Coexisting Disorders, and Enzymopathies: Risks and Management Options
Anesth. Analg., October 1, 2009; 109(4): 1049 - 1053.
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Lippincott, Williams & Wilkins Anesthesia & Analgesia® is published for the International Anesthesia Research Society® by Lippincott Williams & Wilkins and Stanford University Libraries' HighWire Press®. Copyright 2009 by the International Anesthesia Research Society. Online ISSN: 1526-7598   Print ISSN: 0003-2999 HighWire Press
Copyright © 2009 by the International Anesthesia Research Society.