恶性高温症和Dantrolene无法使用的困境:一个案例报告
Abdullah Mahmood1, Muhammad Faizan Abbasi1, Lubna Shafiq2
1Department of Plastic Surgery and Burn Unit, Ayub Teaching Hospital, Abbottabad, Pakistan.
JPMA. The Journal of the Pakistan Medical Association
|June 11, 2025
概括
恶性高温症 (MH) 是一种罕见的遗传疾病,如果不及时使用Dantrolene治疗,可能会致命. 这一案例凸显了丹特罗林缺乏对巴基斯坦患者存活率的重大影响.
科学领域:
- 麻醉学 麻醉学
- 药理学 药理学是指药理学的学科.
- 遗传学 是一个遗传学.
背景情况:
- 恶性高温症 (MH) 是一种罕见的,危及生命的骨肌肉的药物遗传疾病.
- 在易受影响的个体中,由挥发性麻醉剂和顺胆触发.
- 用丹特烯及时治疗对生存至关重要,但其在全球范围内的可用性有限,特别是在巴基斯坦.
研究的目的:
- 报告巴基斯坦一名儿科患者患有恶性高温症的致命病例.
- 强调丹特罗林在管理MH危机中的关键作用.
- 突出在资源有限的环境中面临的挑战.
主要方法:
- 一个5岁女孩的病例报告,她在麻醉后发展了MH.
- 在十切手术过程中用异氨酸维持麻醉.
- 支持性护理和复苏措施迅速启动.
主要成果:
- 患者在康复期间出现了高度发烧和肌肉硬.
- 尽管立即转移到ICU和支持性治疗,但患者没有存活.
- 死亡的主要原因是Dantrolene的不可用.
结论:
- 这一案例强调了丹特罗林不适用于MH治疗的严重后果.
- 迫切需要采取战略,以确保在巴基斯坦和类似地区的丹特罗林可用性.
- 改善全球供应链和本地提供MH治疗是必不可少的.
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