How does the degree of glucose-6-phosphate dehydrogenase (G6PD) deficiency affect its clinical presentation?

Updated: Feb 19, 2019
  • Author: Srikanth Nagalla, MBBS, MS, FACP; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

The degree of G6PD deficiency determines the clinical expression of the disorder. Individuals with minimally reduced enzyme levels do not experience hemolysis. Others with a greater degree of deficiency have episodes of brisk hemolysis triggered by infections, taking drugs that increase oxidative stress, ingesting fava beans, or ketoacidosis. Hemolysis due to oxidant stresses are usually self-limiting within 8 to 14 days due to the compensatory production of young red blood cells with high levels of G6PD. Patients with severe G6PD deficiency have chronic hemolysis and are often thought to have non-spherocytic hemolytic anemia.

Jaundice in G6PD-deficient neonates is considered to be due to an imbalance between the production and conjugation of bilirubin, with a tendency for inefficient bilirubin conjugation. Borderline premature infants are at special risk of the bilirubin production-conjugation imbalance. [11]


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