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一名葡萄糖-6-磷酸脱氢酶缺乏症患者在接受低温等离子体腺样体切除术联合鼻中隔成形术和鼻甲切除术时的麻醉管理

Anesthetic Management in a Patient With Glucose-6-Phosphate Dehydrogenase Deficiency Undergoing Coblation Adenoidectomy With Septoplasty and Turbinectomy.

作者信息

Dairi Abdulrhman Saleh, Dairi Ghida, Farghaly Moheyeldin A

机构信息

Department of Anesthesia and Pain Management, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Jeddah, Saudi Arabia.

Department of Anesthesia and Pain Management, International Medical Center, Jeddah, Saudi Arabia.

出版信息

J Med Cases. 2019 Oct;10(10):293-295. doi: 10.14740/jmc3371. Epub 2019 Oct 31.

Abstract

Glucose-6-phosphate dehydrogenase (G-6-PD) is the major enzyme in the pentose phosphate pathway (PPP). The end products of this pathway are ribose-5-phosphate and nicotinamide adenine dinucleotide phosphate hydrogen (NADPH). G-6-PD deficiency is known to be the most common enzymatic deficiency in red blood cells (RBCs). Genetically, the mode of inheritance is an X-linked recessive disease. The exposure to oxidative stressors will result in hemolytic anemia including fava beans, infections, metabolic conditions such as diabetic ketoacidosis, metabolic acidosis, hyperglycemia, hypoglycemia, and hypothermia. Moreover, surgical stress and certain types of medication are known to lead to hemolytic anemia. Acute hemolytic crisis is a life-threatening situation in patients with G-6-PD deficiency. Therefore, it is extremely important to monitor the patient perioperatively. The authors present this case of successful anesthetic management in a 23-year-old lady with G-6-PD deficiency and a previous history of acute hemolytic anemia undergoing coblation adenoidectomy with septoplasty and turbinectomy.

摘要

葡萄糖-6-磷酸脱氢酶(G-6-PD)是磷酸戊糖途径(PPP)中的主要酶。该途径的终产物是5-磷酸核糖和还原型烟酰胺腺嘌呤二核苷酸磷酸(NADPH)。G-6-PD缺乏症是已知最常见的红细胞(RBC)酶缺乏症。从遗传学角度来看,其遗传方式为X连锁隐性疾病。接触氧化应激源会导致溶血性贫血,包括蚕豆、感染、糖尿病酮症酸中毒、代谢性酸中毒、高血糖、低血糖和低温等代谢状况。此外,手术应激和某些类型的药物已知会导致溶血性贫血。急性溶血危象对于G-6-PD缺乏症患者来说是危及生命的情况。因此,围手术期对患者进行监测极为重要。作者介绍了一例23岁患有G-6-PD缺乏症且有急性溶血性贫血病史的女性患者,成功接受了腺样体消融术、鼻中隔成形术和鼻甲切除术的麻醉管理病例。

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本文引用的文献

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Glucose-6-phosphate dehydrogenase deficiency and cardiac surgery.葡萄糖-6-磷酸脱氢酶缺乏症与心脏手术
Perfusion. 2010 Nov;25(6):417-21. doi: 10.1177/0267659110380770. Epub 2010 Aug 12.
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Glucose-6-phosphate dehydrogenase deficiency.葡萄糖-6-磷酸脱氢酶缺乏症
Lancet. 2008 Jan 5;371(9606):64-74. doi: 10.1016/S0140-6736(08)60073-2.

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