Pädiatrie und Therapeutik
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Glucose-6-Phosphate Dehydrogenase Deficiency Unmasked by Hyperglycemia

Francesca Silvestri, Silvia Ortuso, Daniela Dicanio and Francesco Costantino

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked disease responsible for moderate to severe hemolytic anemia. This is the most common erythrocyte enzyme disorder, often overpassed. A 14-year-old male patient was admitted to emergency department with hyperglycemia. He was treated initially with fluid therapy, after two hours with subcutaneous ultra-rapid insulin. After five days from hospitalization he showed scleral and skin jaundice were made diagnosis of hemolytic anemia by G6PD deficiency. There was no significant family or prior medical/ drug history. Interestingly, the hemolytic features were evidenced when blood glucose levels were returning to normal values. The insulin mediated NADPH loss may have resulted in increased erythrocyte oxidant sensitivityand a loss of sulfhydryl group’s availability, causing hemolysis to manifest.

G6PD deficiency is usually linked to drug which induced oxidative stress. Association with diabetes mellitus is infrequently reported. This case wants to emphasize that the G6PD deficiency has been unmasked by hyperglycemia until now unknown without signs and symptoms.