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Many patients with 6-phosphogluconate dehydrogenase deficiency are carriers and have no symptoms. It has been discovered that symptoms manifest more frequently in female carriers. Depending on how severe the deficiency is, it has been demonstrated that enzyme activity is reduced by 35–65%. [citation needed]
Methemoglobinemia can also arise in patients with pyruvate kinase deficiency due to impaired production of NADH – the essential cofactor for diaphorase I. Similarly, patients with glucose-6-phosphate dehydrogenase deficiency may have impaired production of another co-factor, NADPH. [16]
Most individuals with G6PD deficiency are asymptomatic.When it induces hemolysis, it is usually is short-lived. [5]Most people who develop symptoms are male, due to the X-linked pattern of inheritance, but female carriers can be affected due to unfavorable lyonization or skewed X-inactivation, where random inactivation of an X-chromosome in certain cells creates a population of G6PD-deficient ...
Among American ethnicities, G6PD is most prevalent among African Americans, with a prevalence of about 12.2% (males) and 4.1% (females). [27] During the Korean War , many black soldiers developed acute hemolytic anemia after primaquine for treatment or prophylaxis of malaria, which led to early understanding of this kind of anemia.
If drug-induced nonautoimmune hemolytic anemia occurs secondarily to drug induced methemoglobinemia, methylene blue can be used as a first-line therapy. However methylene blue should be avoided in those with a concomitant G6PD deficiency as methylene blue may cause further oxidative hemolysis.
GSD Ia is caused by a deficiency in the enzyme glucose-6-phosphatase; GSD Ib, a deficiency in the transport protein glucose-6-phosphate translocase. Because glycogenolysis is the principal metabolic mechanism by which the liver supplies glucose to the body during fasting , both deficiencies cause severe hypoglycemia and, over time, excess ...
For example, a methemoglobin concentration of 1.5 g/dL may represent a percentage of 10% in an otherwise healthy patient with a baseline hemoglobin of 15 mg/dL, whereas the presence of the same concentration of 1.5 g/dL of methemoglobin in an anemic patient with a baseline hemoglobin of 8 g/dL would represent a percentage of 18.75%.
The most common and successful treatment used to treat patients with high levels of methemoglobinemia is the antidote methylene blue. Methylene blue is already recognized as a product of the reversible reaction fueled by NAPHD methemoglobin reductase, catalyzed by leukcomethylene, to reduce methemoglobin to hemoglobin.