全身麻醉下一系列手术中多酰基辅酶A脱氢酶缺乏症的麻醉管理：一例报告
Anesthetic management of multiple acyl-coenzyme A dehydrogenase deficiency in a series of surgeries under general anesthesia: a case report.
作者信息
Owaki-Nakano Ryoko, Higashi Midoriko, Iwashita Kohei, Shigematsu Kenji, Toyama Emiko, Yamaura Ken
机构信息
Department of Anesthesiology, Fukuoka University Hospital, 7-45-1 Nanakuma, Jonan-ku, Fukuoka, 814-0180, Japan.
Department of Anesthesiology and Critical Care Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
出版信息
JA Clin Rep. 2021 Jul 10;7(1):54. doi: 10.1186/s40981-021-00459-3.
BACKGROUND
Glutaric acidemia is a type of multiple acyl-coenzyme A dehydrogenase deficiency, an inborn error in fatty acid metabolism. In patients with glutaric acidemia, during the perioperative period, prolonged fasting, stress, and pain have been identified as risk factors for the induction of metabolic derangement. This report describes the surgical and anesthetic management of a patient with glutaric acidemia.
CASE PRESENTATION
A 56-year-old male patient with glutaric acidemia type 2 underwent a series of surgeries. During the initial off-pump coronary artery bypass surgery, the patient developed renal failure due to rhabdomyolysis upon receiving glucose at 2 mg/kg/min. However, in the second laparoscopic cholecystectomy, rhabdomyolysis was avoided by administering glucose at 4 mg/kg/min.
CONCLUSIONS
To avoid catabolism in patients with glutaric acidemia, appropriate glucose administration is important, depending on the surgical risk.
背景
戊二酸血症是多种酰基辅酶A脱氢酶缺乏症的一种，是脂肪酸代谢的先天性缺陷。在戊二酸血症患者的围手术期，长期禁食、应激和疼痛已被确定为诱发代谢紊乱的危险因素。本报告描述了一名戊二酸血症患者的手术及麻醉管理情况。
病例介绍
一名56岁的2型戊二酸血症男性患者接受了一系列手术。在最初的非体外循环冠状动脉搭桥手术中，患者在以2mg/kg/min的速度输注葡萄糖时因横纹肌溶解而出现肾衰竭。然而，在第二次腹腔镜胆囊切除术中，通过以4mg/kg/min的速度输注葡萄糖避免了横纹肌溶解。
结论
为避免戊二酸血症患者发生分解代谢，根据手术风险进行适当的葡萄糖输注很重要。
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