Glucose-6-phosphate dehydrogenase defficiency

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1. Introduction
  • It is an inherited disorder.
  • It is due to genetic deficiency of glucose-6-phosphate dehydrogenase.
  • Due to this, generation of NADPH is impaired.
  • This results in the accumulation of methemoglobin and peroxides in the erythrocytes which leads to red cell hemolysis (hemolytic anemia).
  • Note: As discussed in the pentose phosphate pathway, NADPH counteracts the damaging effects of oxygen free radicals; when NADPH is not available free radicals will induce hemolysis.

Clinical manifestations in G6PD deficiency
  • Most of the patients do not usually exhibit clinical symptoms.
  • Some of them, however, develop hemolytic anemia, if
    • Administered with oxidant drugs e.g. primaquine (antimalarial), acetanilide (antibiotic) or fava beans (antipyretic), or
    • Exposed to a severe infection (results in the generation of free radicals).

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