Glucose-6-phosphatase dehydrogenase (G-6-PD) deficiency is the most common disease-producing enzymopathy in humans. Inherited as an X-linked disorder, G-6-PD deficiency affects 400 million people worldwide.
The G6PD enzyme catalyzes the oxidation of glucose-6-phosphate to 6-phosphogluconate while concomitantly reducing the oxidized form of nicotinamide adenine dinucleotide phosphate (NADP+) to nicotinamide adenine dinucleotide phosphate (NADPH). NADPH, a required cofactor in many biosynthetic reactions, maintains glutathione in its reduced form.
Reduced glutathione acts as a scavenger for dangerous oxidative metabolites in the cell. With the help of the enzyme glutathione peroxidase, reduced glutathione also converts harmful hydrogen peroxide to water. Red blood cells rely heavily upon G-6-PD activity because it is the only source of NADPH that protects the cells against oxidative stresses; therefore, people deficient in G-6-PD are not prescribed oxidative drugs because their red blood cells undergo rapid hemolysis under this stress.
Those with Glucose-6-phosphate dehydrogenase (G6PD) deficiency should avoid vitamin K. Those with G6PD deficiency experience a serious breakdown of red blood cells (called hemolysis) when exposed to certain infections or medications, including vitamin K.
Ascorbic acid is involved in modulating iron absorption and transport. It is highly unlikely that healthy individuals who take supplemental vitamin C will have any problem with iron overload. On the other hand, those with hemochromatosis, thalassemia, sideroblastic anemia, sickle cell anemia and erythrocyte G6PD deficiency might have such a problem if they use large amounts of vitamin C
Vitamin E injections are used for thalassemia, orally for glucose-6-phosphate dehydrogenase deficiency [G6PD] anemia and anemia caused by kidney dialysis.
DHEA blocks G6PD (glucose-6-phosphate-dehydrogenase)
Vitamin E and folic acid (both anti-oxidants) may help decrease hemolysis in G6PD-deficient individuals.
G6PD deficiency is caused by one copy of a defective G6PD gene in males or two copies of a defective G6PD gene in females. Hemolytic anemic attacks can be caused by oxidants, infection, and or by eating fava beans.
Sudden attacks of G6PD deficiency can be caused by any serious illness and certain medicines such as:
Medicines for the treatment of malaria: Chloroquine, Primaquine, Pamaquine, Pentaquine, Plasmoquine, Quinine, Quinocide
Medicines for fever and pain: Acetanilid, Acetylsalicylic acid, Aminopyrine, Antipyrine
Sulphonamides: Sulphanilamide, Sulphacetamide, Sulphapyridine, Sulphamethoxypyradizine
Sulphones: Sulphoxone, Thiazolsulfone, Diaminodiphenyl Sulphone
Nitrofurans: Nitrofurantonin, Furazolidone, Nitrofurazone
Others: Phenylhydrazine, Acetylphenylhydrazine, Probenecid, Dimercaprol, Methylene blue, Naphthalene, Vitamin K, Aminosalicylic acid, Chloramphenicol, Vitamin C (only in very high doses), Neosalvarsan
Measure the actual enzyme activity of G6PD rather than the amount of G-6-PD protein. Performing assay for G-6-PD during hemolysis and reticulocytosis may affect levels and not reflect baseline values.
Perform a CBC count with reticulocyte count to determine the level of anemia and bone marrow function.
Indirect bilirubinemia occurs with excessive hemoglobin degradation and can produce clinical jaundice.
Serum haptoglobin levels serve as an index of hemolysis and will be decreased.
Abdominal ultrasound may be useful in assessing for splenomegaly and gallstones.
Histologic Findings: Acute hemolysis from G-6-PD deficiency is associated with formation of Heinz bodies, which consist of denatured hemoglobin.
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