What Medicines Should Be Avoided with G6PD Deficiency?

Discussion Glucose 6-phosphate dehydrogenase deficiency (G6PD) is an X-linked disease that has multiple mutations. Each mutation causes a different amount of the enzyme to be produced within cells and therefore not all mutations will produce disease. Glucose 6-phosphate dehydrogenase is an enzyme that is critical to the metabolism of all aerobic cells as it catalyzes the rate-limiting step of the pentose phosphate pathway reducing NADP to NADPH. NADPH is important for nucleic acid replication and therefore cell division. G6PD is the only source of NADPH within the cell, so deficiency makes red blood cells susceptible to hemolysis if they are under oxidative stress. Populations at higher risk for G6PD include those who their ethnic heritage is from the Mediterranean, Africa, Middle East and Asia. Newborn screening testing is not routinely done in the United States. Infants are often identified because of the early timing, persistent or extreme levels of hyperbilirubinemia. Although the levels of hyperbilirubinemia in this patient were not extreme (i.e. > 20 mg/dL), G6PD is a common reason for extreme hyperbilirubinemia. Causes of severe unconjungated hyperbilirubinemia specifically due to problems with red blood cells can be found here. A review of general causes of neonatal unconjugated hyperbilirubinemia can be found here. Learning Point Hemolytic episodes in persons with G6PD can be triggered by infections, severe stress, certain foods (such as fava beans), and certain...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news