Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common X-linked recessive red blood cell disease in humans. The highest prevalence of G6PD deficiency is reported to be in Africa, Southern Europe, the Middle East, Southeast Asia, and the islands of the Central and South Pacific. G6PD deficiency causes acute hemolysis upon exposure to oxidative stress. Various stress factors that can cause hemolysis include infections, fever, sepsis, various foods such as fava beans, and various medications. In this report, we describe the case of a 13-year-old child who was diagnosed with G6PD deficiency in childhood but did not experience typical complications, such as hemolysis or jaundice. This child underwent Mathieu's flip-flap surgery for the treatment of distal penile hypospadias under spinal anesthesia and underwent the procedure uneventfully, with no hemolytic complications, malignant hyperthermia, or methemoglobinemia. Therefore, the main goals of our anesthesia management are to avoid various agents that cause hemolysis, use agents with antioxidant properties, reduce the stress of surgery through appropriate pain management, and monitor for signs of hemolysis. Therefore, in our case, subarachnoid blockade was found to be a safe and effective anesthetic technique compared with general anesthesia in the treatment of children with G6PD deficiency. Dexmedetomidine has antioxidant properties, maintains upper respiratory tract patency, and has sedative effect. Therefore, in our case, it was administered intravenously for perioperative management.
Keywords: antioxidant; dexmedetomidine; glucose 6 phosphate dehydrogenase (g6pd); hemolysis; subarachanoid block (sab).
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