Suspected malignant hyperthermia in a child with laminin α2 (merosin) deficiency in the absence of a triggering agent

Mohanad Shukry, Zurab V. Guruli, Usha Ramadhyani

Research output: Contribution to journalArticle

21 Citations (Scopus)

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 α2 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 α2 (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 α2 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.

Original languageEnglish (US)
Pages (from-to)462-465
Number of pages4
JournalPaediatric Anaesthesia
Volume16
Issue number4
DOIs
StatePublished - Apr 1 2006

Fingerprint

Malignant Hyperthermia
Laminin
Dantrolene
Muscular Dystrophies
Anesthetics
Skeletal Muscle

Keywords

  • Congenital muscular dystrophy
  • Dexmeditomidine
  • Laminin α2 (merosin) deficiency
  • Malignant hyperthermia

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Anesthesiology and Pain Medicine

Cite this

Suspected malignant hyperthermia in a child with laminin α2 (merosin) deficiency in the absence of a triggering agent. / Shukry, Mohanad; Guruli, Zurab V.; Ramadhyani, Usha.

In: Paediatric Anaesthesia, Vol. 16, No. 4, 01.04.2006, p. 462-465.

Research output: Contribution to journalArticle

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