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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