Dairi Abdulrhman Saleh, Dairi Ghida, Farghaly Moheyeldin A
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.
J Med Cases. 2019 Oct;10(10):293-295. doi: 10.14740/jmc3371. Epub 2019 Oct 31.
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.
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。
