Department of Anesthesiology, University Hospital of Crete, Herakllion, Greece.
Department of Pediatric Surgery, University Hospital of Crete, 71110, Herakllion, Greece.
BMC Anesthesiol. 2017 Aug 29;17(1):116. doi: 10.1186/s12871-017-0400-9.
Mitochondria are the energy producing organelles practically in every human cell except erythrocytes. Indeed mitochondria are widespread in high energy requiring organs like brain, heart and muscles. Currently there are no clinical trials supporting with clear evidence which is the most suitable surgical or anesthetic management of a patient with known mitochondrial disease presenting with surgical disorders. This condition poses possible hazardous problems to the medical attention of those patients.
A case of an 8 year old child with known Electron Transfer Flavoprotein Dehydrogenase deficiency (ETFDH deficiency) requiring surgery for acute appendicitis is presented. Our approach for anesthesia revealed a combination of fentanyl, low dose propofol and nitrous oxide.
The choice of the safest pharmacological anesthetic agents for patients with ETFDH deficiency is challenging given that most of the general anesthetic medications have multiple effects on mitochondria, fatty acids metabolism and striated muscles. Anesthetists are expected to individualize anesthetic care for the patient based on current publications for similar cases, medical history and knowledge of pharmacology and physiology.
线粒体是除红细胞以外几乎所有人类细胞的能量产生细胞器。实际上,线粒体广泛存在于需要高能量的器官中,如大脑、心脏和肌肉。目前,没有临床试验提供明确的证据支持已知患有线粒体疾病并出现手术障碍的患者最适合的手术或麻醉管理。这种情况可能会给这些患者的医疗带来潜在的危险问题。
介绍了一例 8 岁儿童,患有已知的电子传递黄素蛋白脱氢酶缺乏症(ETFDH 缺乏症),需要手术治疗急性阑尾炎。我们的麻醉方法包括芬太尼、小剂量异丙酚和一氧化二氮。
鉴于大多数全身麻醉药物对线粒体、脂肪酸代谢和横纹肌有多种影响,因此为 ETFDH 缺乏症患者选择最安全的药理麻醉剂具有挑战性。麻醉师应根据当前类似病例的出版物、病史以及药理学和生理学知识,为患者制定个体化的麻醉护理方案。