Glucose-6-Phosphate Dehydrogenase Deficiency Unveiled by Diabetic Ketoacidosis: A Dual Dilemma.

An 18 year old male, known case of Type 1 Diabetes Mellitus was admitted in view of diabetic ketoacidosis. With normalization of blood sugars patient developed gross reddish discoloration of urine. Urine routine microscopy did not reveal RBCs or RBC casts. Peripheral blood smear revealed bite cells, Heinz bodies and spherocytes. Thus a diagnosis of hemolytic anemia with hemoglobinuria was made. Patient's glucose-6-phosphate dehydrogenase (G6PD) levels were below the normal range. G6PD, an enzyme of the HMP shunt, is the most common enzyme defect causing hemolytic anemia. G6PD deficiency related hemolytic crisis is most commonly precipitated by infection, drugs or fava beans. Its association with DKA has been seldom reported.