Department of Metabolic and Endocrine diseases, Wilhelmina Children's Hospital, UMC Utrecht, The Netherlands.
Mol Genet Metab. 2011 May;103(1):96-7. doi: 10.1016/j.ymgme.2011.01.010. Epub 2011 Jan 27.
Surgical procedures in patients with metabolic disorders require specific anesthetic measures based on the nature of the involved metabolic disorder. Illustrated by the history of two patients, the need for a specific perioperative regimen in patients with very long chain acyl-CoA dehydrogenase deficiency (VLCADD) is discussed. One patient deteriorated, the other patient did well without any specific measurements. Although perioperative metabolic decompensation can currently not be predicted, it is a severe complication which should be avoided. We therefore advise to consider certain perioperative precautions in all VLCADD patients: 1) age and weight adapted glucose infusion, 2) stress avoiding premedication, 3) avoidance of volatile anesthetics, 4) avoidance of long chain fatty acid containing anesthetics and 5) perioperative glucose and CK monitoring.
代谢紊乱患者的手术需要根据代谢紊乱的性质采取特定的麻醉措施。通过两名患者的病史，讨论了极长链酰基辅酶 A 脱氢酶缺乏症（VLCADD）患者特定围手术期方案的必要性。一名患者病情恶化，另一名患者未采取任何特殊措施但情况良好。尽管目前无法预测围手术期代谢性失代偿，但这是一种严重的并发症，应予以避免。因此，我们建议所有 VLCADD 患者考虑以下围手术期预防措施：1）根据年龄和体重调整葡萄糖输注量；2）避免应激的术前用药；3）避免使用挥发性麻醉剂；4）避免使用含有长链脂肪酸的麻醉剂；5）围手术期血糖和 CK 监测。
