Suspected malignant hyperthermia in a child with laminin alpha2 (merosin) deficiency in the absence of a triggering agent

Paediatric Anaesthesia
Mohanad ShukryUsha Ramadhyani

Abstract

Malignant hyperthermia (MH) is an inherited disorder of the skeletal muscles that can be triggered by many anesthetic agents. MH has different presentations and manifestations that makes it difficult to diagnose. Patients with laminin alpha2 deficiency have never been reported to be susceptible to MH. We present a suspected MH episode in the absence of classic triggering agents in a 7-year-old boy with laminin alpha2 (merosin) deficiency and congenital muscular dystrophy. The episode was diagnosed using the MH clinical grading scale and responded well to prompt management with dantrolene. We conclude that patients with laminin alpha2 deficiency may be susceptible to MH, and early suspicion and rapid treatment is vital in the management of MH. Anesthesiologists should be prepared to treat MH in susceptible patients even in the absence of a classical triggering agent.

References

Aug 1, 1993·Anesthesia and Analgesia·K P Strazis, A W Fox
Aug 6, 2000·British Journal of Anaesthesia·P M Hopkins
Aug 6, 2000·British Journal of Anaesthesia·P AdnetR Krivosic-Horber
Jun 18, 2002·British Journal of Anaesthesia·C BaillardC M Samama

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Citations

Dec 23, 2006·Paediatric Anaesthesia·James Mayhew
Sep 14, 2017·Anesthesiology·Ronald S LitmanSheila Riazi
Jul 18, 2018·Current Opinion in Neurology·Bram De Wel, Kristl G Claeys
May 7, 2008·Current Opinion in Anaesthesiology·Jacques J Driessen
Jul 29, 2008·Current Opinion in Anaesthesiology·Dominic S CarolloUsha Ramadhyani
Apr 28, 2010·Current Opinion in Anaesthesiology·Francis Veyckemans
Jul 16, 2013·Paediatric Anaesthesia·Barbara W Brandom, Sheila M Muldoon
Jul 3, 2021·Brazilian Journal of Anesthesiology·Felipe Aparecido Ferreira da CruzHelga Cristina Almeida da Silva

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