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Six-Year-Old Death in the Emergency Department

Death in the Emergency Department: An Unrecognized Awake Malignant Hyperthermia-Like Reaction in a Six-Year-Old.

This abstract was published by Anesthesia & Analgesia; click link for more information.

A healthy 6-year-old boy developed lower extremity rigidity, trismus, and fever after playing in a splash pool. On arrival in the emergency department, he appeared to be seizing. An endotracheal tube was emergently placed using succinylcholine. Cardiac arrest followed. He could not be resuscitated. Postmortem genetic analysis found a novel RYR1 variant. Family testing revealed the same variant in his father who also had muscle contracture testing diagnostic for susceptibility to malignant hyperthermia and central core disease diagnosed histologically. Because there was no exposure to volatile anesthetics before the onset of symptoms, this is a case of â??awakeâ?? malignant hyperthermia worsened by succinylcholine.

Written by: Wendy A. Lavezzi, MD*, John F. Capacchione, MD�, Sheila M. Muldoon, MD�, Nyamkhishig Sambuughin, PhD�, Saiid Bina, PhD�, Deanna Steele, MS, CGC� and Barbara W. Brandom, MD§?

From the *Office of the District 5 Medical Examiner, Leesburg, Florida; �Department of Anesthesiology, Uniformed Services University of the Health Sciences, Bethesda, Maryland; �Center for Medical Genetics and ?Department of Anesthesiology, University of Pittsburgh, Pittsburgh, Pennsylvania; and §North American Malignant Hyperthermia Registry (NAMHR) of the Malignant Hyperthermia Association of the United States.

Address correspondence to John F. Capacchione, MD, Department of Anesthesiology, Uniformed Services University of the Health Sciences, USUHS4301 Jones Bridge Rd., Bethesda, MD 20814. Address e-mail to [email protected].

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  • General
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