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

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. J Med Cases. 2019;10(10):293-295 doi: https://doi.org/10.14740/jmc3371