极长链酰基辅酶A脱氢酶缺乏症患者的麻醉管理
Anesthesia management in a patient with very long-chain acyl-Coenzyme A dehydrogenase deficiency.
作者信息
Yuasa Haruyuki, Onoda Yukio, Kitaura Atsuhiro, Mino Takashi, Tsukimoto Shota, Nakao Shinichi
机构信息
Department of Anesthesiology, Faculty of Medicine, Kindai University, 377-2 Ohonohigashi, Osakasayama, Osaka, 589-8511, Japan.
Onoda Clinic, 1917-1 Yuasa Yuasa-cho Arita-gun, Wakayama, 643-0004, Japan.
出版信息
JA Clin Rep. 2020 Sep 16;6(1):72. doi: 10.1186/s40981-020-00379-8.
BACKGROUND
In a patient with very long-chain acyl-Coenzyme A dehydrogenase (VLCAD) deficiency, metabolism of fatty acids is impaired and a supply of alternative energy is limited when glucose level is insufficient on starvation.
CASE PRESENTATION
A 37-year-old woman with VLCAD deficiency was diagnosed with an ovarian cyst and was scheduled for laparoscopic ovarian cystectomy. Glucose was administered intravenously with the start of fasting. Anesthesia was induced with remifentanil, midazolam, and thiamylal, maintained with desflurane and remifentanil. Body temperature was maintained at 36.2-36.7 °C. During anesthesia, hypoglycemia did not occur, creatine kinase levels were in the normal range, and myoglobinuria was not detected. No shivering was observed after extubation.
CONCLUSIONS
Glucose was administered to avoid perioperative hypoglycemia. Body temperature was controlled to avoid shivering, which would otherwise increase skeletal muscle energy needs. Blood creatine kinase did not increase, and myoglobinuria was not detected; thus, rhabdomyolysis was unlikely to develop.
背景
在患有极长链酰基辅酶A脱氢酶（VLCAD）缺乏症的患者中，脂肪酸代谢受损，在饥饿时葡萄糖水平不足时，替代能量的供应受到限制。
病例报告
一名37岁患有VLCAD缺乏症的女性被诊断出患有卵巢囊肿，并计划进行腹腔镜卵巢囊肿切除术。禁食开始时静脉输注葡萄糖。使用瑞芬太尼、咪达唑仑和硫喷妥钠诱导麻醉，用地氟醚和瑞芬太尼维持麻醉。体温维持在36.2-36.7°C。麻醉期间，未发生低血糖，肌酸激酶水平在正常范围内，未检测到肌红蛋白尿。拔管后未观察到寒战。
结论
输注葡萄糖以避免围手术期低血糖。控制体温以避免寒战，否则会增加骨骼肌能量需求。血液肌酸激酶未升高，未检测到肌红蛋白尿；因此，不太可能发生横纹肌溶解。
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