It is inherited in an x-linked recessive manner. Most children with G6PD deficiency are hematologically normal unless they undergo oxidative stress. Many pediatric patients are not diagnosed until they have an acute hemolytic crisis. In the midst of the acute crisis, it can be difficult to make the diagnosis of G6PD deficiency due to the increased amount of G6PD in reticulocytes and young red blood cells.
Oxidative stressors can be infectious agents, drugs, chemicals and certain legumes.
In G6PD deficient patients, oxidative stress exposes interior sulfhydryl groups that are oxidized and cannot be reduced, leading to irreversible denaturation of the hemoglobin with Heinz body formation. The same agents which lead to hemolysis in G-6PD deficiency can also lead to the formation of methemoglobin. The following case report illustrates the clinical severity that can occur when potent oxidizers induce methemoglobinemia and hemolysis in G6PD deficient patients. He had been in his usual state of good health until 2 days prior to admission when he developed non-bloody diarrhea and emesis. Shortly thereafter, his mother noticed his urine to be tea colored.