《中国康复理论与实践》

《中国康复理论与实践》 ›› 2006, Vol. 12 ›› Issue (05): 427-428.

• 临床观察 • 上一篇    下一篇

中央轴空病患者畸形矫正术中的恶性高热风险

吴士文1; 马维娅1; 于生元2; 沈定国2   

  1. 1.武警总医院神经内科 北京市 100039;2.解放军总医院神经内科 北京市 100853
  • 收稿日期:2006-01-23 出版日期:2006-05-25 发布日期:2006-05-25

Morbidity of Malignant Hyperthermia in Central Core Disease Patients Undergoing Orthopedics

WU Shi-wen, MA Wei-ya, YU Sheng-yuan, et al   

  1. Department of Neurology, General Hospital of Chinese Armed Police Force, Beijing 100039, China
  • Received:2006-01-23 Published:2006-05-25 Online:2006-05-25

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摘要： 目的探讨中央轴空病患者骨、关节畸形的发生情况,以及在畸形矫正术中的恶性高热风险。方法回顾分析28例中央轴空病患者临床及肌纤维钙离子致钙离子释放试验资料。结果22例出现明确的肢体肌无力,6例无临床症状;21例伴有骨和/或关节畸形,其中伴关节挛缩11例,关节脱位9例,脊椎侧凸12例,脊椎前弯5例,胸廓畸形1例。10例患者有畸形矫正的手术史,6例在手术麻醉中发生恶性高热或可疑恶性高热发作。8例患者经钙离子致钙离子释放试验检查诊断为恶性高热易患者。结论骨、关节畸形在中央轴空病中很常见,在畸形矫正术中有很高的恶性高热发病率。

关键词: 中央轴空病, 恶性高热, 畸形矫正

Abstract: ObjectiveTo investigate the incidence of skeletal and/or articular malformations in central core disease (CCD) patients, and their risk of malignant hyperthermia (MH) in orthopedics. Methods28 unrelated patients with central core disease collected in the past 23 years were analyzed retrospectively. Results22 patients showed limb muscle weakness, while 6 patients didn't show any clinical symptom. Skeletal and/or articular malformations appeared in 21 patients, including joint contracture in 11, joint dislocation in 9, scoliosis in 12, lordosis in 5 and thoracic deformity in 1. 6 of the 10 patients could not endure orthopedic operation because of MH attack or dubitable MH attack. Calcium-induced calcium release (CICR) test performed on other 8 patients with MH family history or MH attack showed all those patients were MH susceptibilities. ConclusionSkeletal and/or articular malformations are common in CCD, as well as MH attack in orthopedics.

Key words: central core disease, malignant hyperthermia, orthopedics