进行性肌营养不良患者麻醉一例

Anesthesia in a Patient with Progressive Muscular Dystrophy

摘要: 进行性肌营养不良症(progressive muscular dystrophy, PMD)是一组原发于肌肉组织的遗传病，病理上以肌纤维变性、坏死和肌膜内移为主要特点。临床主要表现为慢性进行性肌无力、肌肉萎缩、运动发育落后或运动障碍、心肌酶谱高、肝功生化异常等，部分患者有心肌受损和不同程度的智力障碍。PMD患者存在骨骼肌、呼吸、心脏等多系统的问题，围术期可能发生麻醉相关的并发症，如麻醉诱发的横纹肌溶解(Anesthesia-induced Rhabdomyolysis, AIR)、高钾血症、恶性高热(Malignant Hyperthermia, MH)和术后呼吸衰竭等，因此给麻醉管理带来了巨大的挑战。

Abstract: The progressive muscular dystrophy (PMD) is a group of genetic disorders that originate in muscle tissue and are pathologically characterized by muscle fiber degeneration, necrosis, and sarcolem-mal membrane shift. The main clinical manifestations are chronic progressive muscle weakness, muscle atrophy, backward motor development or dyskinesia, high cardiac enzyme profile, and bio-chemical abnormalities in liver work, etc., and some patients have cardiac muscle damage and dif-ferent degrees of intellectual disability. Patients with PMD have multisystem problems involving skeletal muscles, respiration, and heart, and anesthesia related complications, such as anesthesia induced rhabdomyolysis (AIR), hyperkalemia, malignant hyperthermia (MH), and postoperative respiratory failure, may occur in the perioperative period, thus posing a significant challenge for anesthesia management.