![]() This is thought to be a result of older erythrocytes having the greatest enzyme deficiency and undergoing hemolysis first. 6 Hemolysis occurs after exposure to the stressor but does not continue despite continued infection or ingestion. 3 Medications that should be avoided in patients with G6PD deficiency are listed in Table 3, 6 and drugs that can be used safely in these patients are listed in Table 4. The variant that causes chronic hemolysis is uncommon because it is related to sporadic gene mutation rather than the more common inherited gene mutation.Īcute hemolysis is caused by infection, ingestion of fava beans, or exposure to an oxidative drug. Neonatal hyperbilirubinemia may require treatment with phototherapy or exchange transfusion to prevent kernicterus. Acute hemolysis is self-limited, but in rare instances it can be severe enough to warrant a blood transfusion. Because acute hemolysis is caused by exposure to an oxidative stressor in the form of an infection, oxidative drug, or fava beans, treatment is geared toward avoidance of these and other stressors. Different gene mutations cause different levels of enzyme deficiency, with classes assigned to various degrees of deficiency and disease manifestation. This usually is done by fluorescent spot test. The conversion of nicotinamide adenine dinucleotide phosphate to its reduced form in erythrocytes is the basis of diagnostic testing for the deficiency. ![]() Homozygotes and heterozygotes can be symptomatic, although the disease typically is more severe in persons who are homozygous for the deficiency. Approximately 400 million people are affected worldwide. This X-linked inherited disorder most commonly affects persons of African, Asian, Mediterranean, or Middle-Eastern descent. Persons with this condition also may be asymptomatic. Glucose-6-phosphate dehydrogenase deficiency, the most common enzyme deficiency worldwide, causes a spectrum of disease including neonatal hyperbilirubinemia, acute hemolysis, and chronic hemolysis. ![]()
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