雷马唑仑和瑞芬太尼麻醉剂治疗中链酰基辅酶A脱氢酶缺乏症的儿科患者:1例报告。

IF 0.5 A&A Practice Pub Date : 2022-12-01 DOI:10.1213/XAA.0000000000001646

Masayo Kiyokawa, Junichi Saito, Kishiko Nakai, Kazuyoshi Hirota

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引用次数: 2

摘要

中链酰基辅酶a脱氢酶(MCAD)缺乏症是最常见的脂肪酸氧化障碍之一。麻醉药物的选择和血糖管理是防止代谢性失代偿的关键。一名患有MCAD缺乏症的5岁日本男孩计划接受腹股沟疝手术。围手术期持续输注葡萄糖，血糖均在正常范围内。麻醉由雷马唑仑、瑞芬太尼和间歇性罗库溴铵诱导和维持。未观察到代谢失代偿。本病例提示持续静脉输注葡萄糖的重要性，雷马唑仑可作为MCAD缺乏症患者的一线麻醉剂。

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A Remimazolam and Remifentanil Anesthetic for a Pediatric Patient With a Medium-Chain Acyl-CoA Dehydrogenase Deficiency: A Case Report.

Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is one of the most common fatty acid oxidation disorders. The choice of anesthetics and blood glucose management are crucial to prevent metabolic decompensation. A 5-year-old Japanese boy with MCAD deficiency was scheduled to undergo surgery for an inguinal hernia. Glucose was continuously infused perioperatively, and his glucose concentrations were within the normal range. Anesthesia was induced and maintained with remimazolam, remifentanil, and intermittent rocuronium. No metabolic decompensation was observed. This case indicates the importance of a continuous intravenous glucose infusion, and that remimazolam can be the first-line anesthetic for a patient with MCAD deficiency.

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A&A Practice ANESTHESIOLOGY-

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期刊介绍： A & A Case Reports, our new online journal publishing Case Reports, related Editorial Commentary, and Correspondence. Anesthesia & Analgesia 1 and Anesthesiology 2 recently announced that they were suspending publication of Case Reports. One reason is that Case Reports typically reduce the Impact Factor of a journal because they are rarely cited. Regardless of the merits of Impact Factor as a metric of journal worth, journals and their editors necessarily consider Impact Factor in strategic planning. At the same time, Case Reports are appreciated by readers for describing “real life” management of difficult or unusual cases not often encountered by practitioners. In a recent issue of Anesthesia & Analgesia, Steven Shafer1 identified many Case Reports whose publication launched productive careers dedicated to solving the puzzle posed by an unusual observation in a single patient.