Schmitt H J, Wick S, Münster T
Department of Anaesthesiology, Friedrich Alexander University Erlangen-Nuremberg, Krankenhausstrasse 12, D-91054 Erlangen, Germany.
Br J Anaesth. 2004 Apr;92(4):592-6. doi: 10.1093/bja/aeh106. Epub 2004 Feb 20.
Friedreich's ataxia is a rare hereditary neurodegenerative disease caused by a defect in the gene that encodes a mitochondrial protein called frataxin. We report the use of rocuronium 0.6 mg kg(-1) in two adolescent girls with Friedreich's ataxia undergoing propofol-sufentanil-oxygen-air anaesthesia for spinal surgery. Neuromuscular transmission was monitored using acceleromyography, and onset and recovery times were recorded. The clinical duration of rocuronium was comparable to that of children without neuromuscular disease (25% recovery T(1)=44 and 24 min for patients 1 and 2 respectively).
弗里德赖希共济失调是一种罕见的遗传性神经退行性疾病，由编码一种名为铁调素的线粒体蛋白的基因缺陷引起。我们报告了在两名患有弗里德赖希共济失调的青春期女孩中使用0.6毫克/千克的罗库溴铵，她们在接受丙泊酚-舒芬太尼-氧气-空气麻醉下行脊柱手术。使用加速度肌电图监测神经肌肉传递，并记录起效和恢复时间。罗库溴铵的临床作用时间与无神经肌肉疾病的儿童相当（患者1和患者2的25%恢复时间T(1)分别为44分钟和24分钟）。
