Shukry Mohanad, Guruli Zurab V, Ramadhyani Usha
Oklahoma University Health Sciences Center/Children's Hospital, Oklahoma City, OK 73104, USA.
Paediatr Anaesth. 2006 Apr;16(4):462-5. doi: 10.1111/j.1460-9592.2005.01742.x.
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.
恶性高热（MH）是一种遗传性骨骼肌疾病，可由多种麻醉剂诱发。MH有不同的表现形式，这使其难以诊断。从未有过关于层粘连蛋白α2缺乏症患者易患MH的报道。我们报告了一名7岁患有层粘连蛋白α2（merosin）缺乏症和先天性肌营养不良的男孩，在没有经典触发剂的情况下发生疑似MH发作的病例。该发作通过MH临床分级量表进行诊断，并对丹曲林的及时治疗反应良好。我们得出结论，层粘连蛋白α2缺乏症患者可能易患MH，早期怀疑和快速治疗对MH的管理至关重要。即使在没有经典触发剂的情况下，麻醉医生也应准备好治疗易感患者的MH。
