Department of Anesthesiology and Pain Medicine, Kyungpook National University School of Medicine, Daegu, Korea.
Korean J Anesthesiol. 2010 Feb;58(2):207-10. doi: 10.4097/kjae.2010.58.2.207. Epub 2010 Feb 28.
Angelman syndrome is characterized by a partial deficit of paired autosomal chromosome 15, which contains a subunit of the GABA (Gamma-Amino Butyric Acid) receptor. Many drugs that act on the CNS (Central Nerve System) during anesthesia are believed to exert their effects via the GABA receptors. We describe the anesthesia of a 7 year-old female patient with Angelman syndrome who underwent surgery for dental caries. The basic factors that needed to be considered when administering anesthesia to this patient were epilepsy, significant dominance of the vagal tone, craniofacial abnormalities and peripheral muscular atrophy. Inhalational anesthetics (sevoflurane) were employed for this patient. The patient had an uneventful peri-operative period and was discharged home on the same day of the operation.
安琪曼氏症候群的特征是部分缺乏对偶体 15 号染色体,该染色体包含 GABA(γ-氨基丁酸)受体的一个亚单位。人们相信,麻醉期间作用于中枢神经系统(CNS)的许多药物是通过 GABA 受体发挥作用的。我们描述了一例 7 岁女性安琪曼氏症候群患者接受龋齿手术的麻醉情况。对该患者进行麻醉时需要考虑的基本因素包括癫痫、迷走神经张力显著占主导地位、颅面畸形和外周肌肉萎缩。该患者使用吸入麻醉剂(七氟醚)。患者围手术期无并发症,手术当天出院回家。