粘多糖病麻醉的安全性——600多例回顾性队列比较研究

IF 3.7 2区生物学 Q2 ENDOCRINOLOGY & METABOLISM

Molecular genetics and metabolism Pub Date : 2025-04-11 DOI:10.1016/j.ymgme.2025.109108

Simon Moser , Paul Harmatz , William Rhoads , Richard Rowe , Florian B. Lagler

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引用次数: 0

摘要

背景：粘多糖病（MPS）患者经常需要全身麻醉，并且围手术期并发症的风险很高。到目前为止，还没有强有力的建议和对照研究。方法：我们对660例麻醉过程进行了单中心图表回顾，并与已发表的队列进行了比较。报告了人口统计学、MPS类型、麻醉方法、外科手术种类、发生率和并发症类型（如呼吸、神经或心脏并发症）的定量分析。其中包括2010年至2022年间在UCSF奥克兰贝尼奥夫儿童医院接受全身麻醉的所有MPS病例。我们将我们的结果与另一份著名的报告（Dohrmann et al.）进行了比较，该报告包括67名患者进行了269次麻醉手术（Dohrmann et al.）。结果在本研究中，我们分析了83例MPS患者的660种麻醉方法。最常用的气道类型是声门上气道（77%），其次是插管（15%）和面罩气道（8%）。并发症仅7例（1.06%）。这些病例需要3次或更多次插管尝试。其他出版物通常报告的比率要高得多。Dohrmann et al. 2020报道了25.6%的并发症发生率，其中最常见的是55%的气道管理问题和35%的呼吸不良事件，最严重的并发症是一例缺氧性心脏骤停导致死亡（Dohrmann et al., 2020）。讨论与结论这是一项关于MPS患者麻醉的大型回顾性研究。本报告为如何提高MPS麻醉的安全性提供了重要的假设。将所有MPS病例分配给一名麻醉师，熟练使用纤维插管，确保患者在气道装置放置期间保持自主呼吸，额外的安全标准和备用计划可能会降低并发症发生率，从而对结果产生重要影响。

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Safety of anesthesia in mucopolysaccharidoses - A comparative retrospective cohort study on more than 600 cases

Background

Mucopolysaccharidosis (MPS) patients frequently require general anesthesia and have a high risk of perioperative complications. Strong recommendations and controlled studies are missing so far.

Methods

We conducted a mono-center chart review of 660 anesthesia procedures, with comparison to a published cohort. Reported are quantitative analysis of demographics, MPS types, anesthesia methods, kinds of surgical procedures, rates and types of complications (e.g. respiratory, neurologic or cardiologic complications). Included were all MPS cases that had general anesthesia at the UCSF Benioff Children's Hospital Oakland between 2010 and 2022. We compared our results to another well-known report of 67 patients that underwent 269 anesthesia procedures (Dohrmann et al.) comprising 67 patients that underwent 269 anesthesia procedures.

Results

In this study, we analyzed 660 anesthetic procedures on 83 MPS patients. The most frequently used airway type was the supraglottic airway (77 %), followed by intubation (15 %) and mask airway (8 %). Complications occurred in only 7 cases (1.06 %). These cases required 3 or more intubation attempts. Other publications generally report much higher rates. Dohrmann et al. 2020 reported a 25.6 % complication rate, with 55 % airway management problems and 35 % respiratory adverse events as the most common as well as a case of hypoxic cardiac arrest resulting in death as the most serious complications (Dohrmann et al., 2020).

Discussion and conclusion

This is a large retrospective study on anesthesia for MPS patients. This report provides important hypotheses on how the safety of anesthesia in MPS could be improved. Allocation of all MPS cases to one anesthetist, proficient use of fiberoptic intubation, ensure patients maintain spontaneous breathing during airway device placement, additional safety standards and backup plans may reduce the complication rate and thus have an important impact on the outcome.

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来源期刊

Molecular genetics and metabolism 生物-生化与分子生物学

CiteScore

5.90

自引率

7.90%

发文量

621

审稿时长

34 days

期刊介绍： Molecular Genetics and Metabolism contributes to the understanding of the metabolic and molecular basis of disease. This peer reviewed journal publishes articles describing investigations that use the tools of biochemical genetics and molecular genetics for studies of normal and disease states in humans and animal models.