Tofil Nancy M, Buckmaster Mark A, Winkler Margaret K, Callans Beth H, Islam Monica P, Percy Alan K
Department of Pediatrics, Division of Critical Care, University of Alabama at Birmingham, Birmingham, AL, USA.
J Child Neurol. 2006 Mar;21(3):210-3. doi: 10.2310/7010.2006.00051.
Herein we present the largest retrospective case-control series of deep sedation in patients with Rett syndrome, including discussion of the unique aspects of Rett syndrome that make these patients at high risk of sedation. Twenty-one patients with Rett syndrome and 21 control patients who received propofol for deep sedation to facilitate lumbar puncture were compared. Patients with Rett syndrome required significantly less propofol than control patients when standardized for weight and the duration of the procedure (P = .004). Seven of the 21 patients with Rett syndrome compared with none of the control patients experienced a serious adverse event, most of which were due to prolonged apnea (P = .004). All adverse events were transient, and all patients returned to their baseline after the procedure was completed. Sedation of patients with Rett syndrome is associated with a relatively high rate of complications and should not be done without appropriate personnel available who recognize the risks of sedating this unique population.
在此，我们展示了雷特综合征患者深度镇静的最大规模回顾性病例对照系列研究，包括对使这些患者处于高镇静风险的雷特综合征独特方面的讨论。比较了21例雷特综合征患者和21例接受丙泊酚深度镇静以利于腰椎穿刺的对照患者。按体重和操作持续时间标准化后，雷特综合征患者所需丙泊酚显著少于对照患者（P = 0.004）。21例雷特综合征患者中有7例发生严重不良事件，而对照患者均未发生，其中大多数是由于呼吸暂停延长（P = 0.004）。所有不良事件均为短暂性，且所有患者在操作完成后均恢复至基线状态。雷特综合征患者的镇静与相对较高的并发症发生率相关，若无认识到对这一独特人群进行镇静风险的合适人员在场，不应进行镇静操作。
