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

被引:19
作者
Shukry, M
Guruli, ZV
Ramadhyani, U
机构
[1] Univ Oklahoma, Hlth Sci Ctr, Childrens Hosp, Dept Anesthesiol, Oklahoma City, OK 73104 USA
[2] Tulane Univ, Hlth Sci Ctr, Dept Anesthesiol, New Orleans, LA 70118 USA
关键词
malignant hyperthermia; laminin alpha 2 (merosin) deficiency; congenital muscular dystrophy; dexmeditomidine;
D O I
10.1111/j.1460-9592.2005.01742.x
中图分类号
R614 [麻醉学];
学科分类号
100217 ;
摘要
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 alpha 2 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 alpha 2 (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 alpha 2 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.
引用
收藏
页码:462 / 465
页数:4
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