title: Suspected malignant hyperthermia in a child with laminin alpha2 (merosin) deficiency in the absence of a triggering agent.
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.
authors.affiliation: Oklahoma University Health Sciences Center/Children's Hospital, Oklahoma City, OK 73104, USA. mohanad-shukry@ouhsc.edu
authors.forename: Mohanad
authors.initials: M
authors.lastname: Shukry
authors.forename: Zurab V
authors.initials: ZV
authors.lastname: Guruli
authors.forename: Usha
authors.initials: U
authors.lastname: Ramadhyani
mesh_terms: D000771:Anesthesia, Intravenous; D002648:Child; D003620:Dantrolene / Q000627:therapeutic use; D020927:Dexmedetomidine / Q000627:therapeutic use; D006801:Humans; D007797:Laminin / Q000172:deficiency* / Q000235:genetics*; D008297:Male; D008305:Malignant Hyperthermia / Q000188:drug therapy / Q000503:physiopathology*; D009125:Muscle Relaxants, Central / Q000627:therapeutic use; D009136:Muscular Dystrophies / Q000150:complications / Q000151:congenital; D010641:Phenotype
chemical_list: D007797:Laminin; D009125:Muscle Relaxants, Central; D020927:Dexmedetomidine; D003620:Dantrolene