麻醉和罕见的神经肌肉疾病

Jovan Z. Radeka, M. Stojanovic, Milena N. Vasilijic, Milica Randjelović, R. Jankovic
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引用次数: 0

摘要

神经肌肉疾病虽然在一般人群中很少见,但在需要接受手术和麻醉的人群中也可能存在。幸运的是,早期诊断的可能性、新的治疗方法和多学科方法的应用大大延长了这类患者的预期寿命。这种疾病的存在代表了麻醉学实践的挑战,无论是在手术准备本身,手术过程和重症监护病房的术后恢复。为了降低围手术期发病率和死亡率的风险,以减少并发症的可能性,对本病的主要障碍及其与麻醉剂本身和麻醉学中必要药物的潜在相互作用有基本的了解是必要的。罕见的肌肉疾病由于其病因和病理的多变性,很难用标准的方法进行分类。这项工作的重点是一些肌肉疾病,如Dubowitz综合征,儿童横纹肌肉瘤,脊髓性肌萎缩,肯尼迪病,先天性乌尔里希型肌肉营养不良(UCMD), Sotos综合征,多肌炎和Setleis综合征,线状棒肌病,Pompe病,emry - dreifuss肌营养不良(EDMD)。回顾了麻醉技术和围手术期并发症的医学文献。考虑到它们会对全身麻醉过程产生非常不利的影响,特别是这些疾病会导致非常严重,甚至危及生命的并发症,适当的麻醉方法对于减少上述并发症的可能性非常重要。无论是在麻醉、局部麻醉还是全身麻醉下,手术前后都必须采用多学科方法。手术过程本身必须在有足够设备治疗这类病人的机构中进行,并由有治疗经验的人员进行。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anesthesia and rare neuromuscular diseases
Neuromuscular diseases, although rare in the general population, can be present in people who need to undergo surgery and anesthesia. Fortunately, the possibility of early diagnosis, new therapeutic approaches and the application of a multidisciplinary approach have significantly extended the life expectancy of such patients. The presence of such a disorder represents a challenge in anesthesiology practice, both during the preparation for surgery itself, the operative procedure and postoperative recovery in intensive care units. A basic understanding of the main disorders of the disease and their potential interactions with the anesthetic itself and the necessary agents in anesthesiology is necessary in order to reduce the risk of perioperative morbidity and mortality, with the aim of reducing the possibility of complications. Rare muscle diseases are very difficult to classify in a standard way because of the variability of their etiology and pathology. This work focuses on a number of muscle diseases such as Dubowitz syndrome, rhabdomyosarcoma in children, spinal muscular atrophy, Kennedy's disease, Congenital muscular dystrophy of the Ullrich type (UCMD), Sotos syndrome, Polymyositis and Setleis syndrome, Nemaline rod myopathy, Pompe disease, Emery-Dreifuss muscular dystrophy (EDMD). The medical literature on anesthetic techniques and perioperative complications was reviewed. Given that they can have a very unfavorable effect on the course of general anesthesia in particular and that these diseases can cause very serious, even life-threatening complications, an adequate anesthesiological approach is very important to reduce the possibility of the mentioned complications. A multidisciplinary approach is imperative for such patients before and after surgery, whether it is performed under analgosedation, some type of regional or general anesthesia. The operative procedure itself must be performed in institutions that are adequately equipped to treat such patients and by personnel who have experience in treating them.
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