隨著特效藥物進入臨床使用,惡性高熱的死亡率已降至2%至3%左右。
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日前,一名中年男性患者在上海市公共衛生臨床中心接受骨科手術時,術中突發惡性高熱,情況十分危急。在麻醉科主任王利的指揮下,麻醉醫護團隊默契配合,經過4個多小時的全力救治,患者最終轉危為安。
該患者正值壯年,原本以為只是一臺常規外科手術,不想術中出現了突發危急狀況——心率驟增,短時間內超過了140次/分鐘,隨之監護儀顯示患者呼氣末二氧化碳分壓已超過110毫米汞柱,同時血壓驟降。
經過快速鑒別排查,王利發現患者體溫已接近40度,臨床診斷指向了最兇險的術中麻醉危象——惡性高熱。這是一種常染色體顯性遺傳疾病,通常由吸入性麻醉藥物及去極化肌肉松弛劑誘發,臨床表現為體溫急劇升高、心動過速、心律失常、低血壓等,患者常在短時間內因多器官功能衰竭導致死亡。一旦發病,極其兇險,搶救窗口期極短。
時間就是生命,全體在院的麻醉科醫護全員到位。他們各司其職:管理呼吸、開放中心靜脈通道、建立高級血流動力學監測、實施全身降溫、檢測生化及內環境指標、升壓糾酸降鉀。
在實施急救的同時,市公衛中心立即向兄弟醫院申請調用惡性高熱的特效急救藥物——丹曲林鈉。隨著藥物緩慢注入患者體內,兇險的惡性高熱被徹底制服。當晚9時許,患者轉危為安,送往重癥監護室后續觀察治療。第二天,患者意識完全清醒,生命體征平穩,順利脫機拔管,未發生任何不良后果。
據醫生介紹,惡性高熱是全球麻醉界談之色變的致命麻醉并發癥,極為罕見但致死率極高。作為一種基因遺傳性代謝異常綜合征,該病發病率僅約五萬分之一。患者平時無異常表現,一旦誘發將出現持續性肌肉代謝亢進,產熱急劇增加,核心體溫急劇升高和重度酸中毒。
患者體溫可迅速升至42℃以上且常規降溫措施無效,同時產生大量乳酸和二氧化碳;病情惡性發展,出現嚴重酸中毒、低氧血癥、高血鉀和心律失常等一系列變化,最終死于多器官功能衰竭。
大多數病例與RYR1基因突變有關,當存在這種基因缺陷時,患者暴露于某些誘發藥物下,會觸發細胞內鈣離子濃度失控性增加,導致肌肉纖維持續興奮并產熱。隨著特效藥物進入臨床使用,惡性高熱的死亡率已降至2%至3%左右。
目前對于惡性高熱易感人群已有了針對性較強的基因篩查技術,可以及時做出術前診斷,若家中曾有親人在接受麻醉過程中出現不明原因意外高熱,也可提前告知麻醉醫生,以便其更好地選擇麻醉方法及麻醉藥物,保證患者的麻醉安全。
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