目的:肌病与典型的体征和症状有关,但也有可能危及生命的并发症,在紧急情况下可能需要援助。这种现象在文献中研究不足。我们旨在评估演示文稿,管理,以及可能与需要转诊至成人急诊科(ED)和住院治疗的肌肉疾病相关的临床表现的结局。
方法:使用国际疾病分类检索的匿名患者数据,回顾性分析了与4年以上肌肉疾病相关的第九次修订代码。评估医疗报告以提取人口统计学和临床变量,以及结果。根据到达时诊断出的肌肉疾病的存在(已知诊断[KD]组)或不存在(未知诊断[UD]组)来定义两组。
结果:共包括244例患者,51%的人受到已知肌病的影响,主要是肢带肌营养不良和肌强直性营养不良。KD组ED就诊的主要原因是呼吸道问题,肌肉无力的恶化,还有肠胃问题.心脏并发症不太普遍。在UD组中,27例患者在ED进入后接受了特定原发性肌肉疾病的新诊断,主要是炎症性肌病.记录了26例患者住院期间的死亡,KD组和受线粒体和炎性肌病影响的患者的发病率较高。脓毒症和呼吸困难与死亡风险增加相关。
结论:呼吸系统并发症是肌病患者进入ED的最常见原因,其次是胃肠道问题。感染是严重威胁,一旦住院,这些患者的死亡率相对较高。
OBJECTIVE: Myopathies are associated with classic signs and symptoms, but also with possible life-threatening complications that may require assistance in an emergency setting. This phenomenon is understudied in the literature. We aimed to assess the presentation, management, and outcomes of clinical manifestations potentially related to a muscle disorder requiring referral to the adult emergency department (ED) and hospitalization.
METHODS: Anonymized patient data retrieved using the International Classification of Diseases, Ninth Revision codes related to muscle disorders over 4 years were retrospectively analyzed. Medical reports were evaluated to extract demographic and clinical variables, along with outcomes. Two groups were defined based on the presence (known diagnosis [KD] group) or absence (unknown diagnosis [UD] group) of a diagnosed muscle disorder at arrival.
RESULTS: A total of 244 patients were included, 51% of whom were affected by a known myopathy, predominantly limb-girdle muscular dystrophies and myotonic dystrophies. The main reasons for ED visits in the KD group were respiratory issues, worsening of muscle weakness, and gastrointestinal problems. Heart complications were less prevalent. In the UD group, 27 patients received a new diagnosis of a specific primary muscle disorder after the ED access, mostly an inflammatory myopathy. Death during hospitalization was recorded in 26 patients, with a higher rate in the KD group and in patients affected by mitochondrial and inflammatory
myopathies. Sepsis and dyspnea were associated with increased death risk.
CONCLUSIONS: Respiratory complications are the most common reason for myopathic patients accessing the ED, followed by gastrointestinal issues. Infections are severe threats and, once hospitalized, these patients have relatively high mortality.