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Unexpected MH Deaths Without Exposure To Inhalation Anesthetics In Pediatric Patients

Brandom, BW, & Muldoon, SM. Paediatric Anaesthesia. September 2013.

It is well documented that infants, children and adolescents with certain gene mutations are susceptible to malignant hyperthermia when exposed to certain medications including succinylcholine, a muscle relaxant, and inhalation anesthetics. However, fatal, MH-like episodes have also been documented in children not exposed to these known triggers.
This study looked at children who had MH-like episodes without being exposed to triggering agents. These children were found to have genetic mutations in the type 1 ryanodine receptor (RYR1). These events were similar to MH in that they included generalized muscle rigidity, elevated body temperature, and dangerous changes in blood pressure and heart rate. In addition to these similar features in presentation, these patients were successfully treated with standard MH treatments including giving dantrolene and other supportive measures. In addition, some family members with the same mutation were also found to have muscle biopsy results that would qualify them as MH susceptible.
Pediatric anesthesiologists must talk to the families of these children in order to diagnose underlying myopathies and connect them with the MH registry so that continued research may occur. This will help advance the diagnosis and treatment of future children and families affected. ~ Caroline So, MD – Allegheny Health Network, Pittsburgh, PA

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