Glucose 6 phosphate dehydrogenase(G6PD) is an enzyme that is found in the red blood cells (RBC) of our body. A deficiency of this enzyme causes premature destruction of RBC, resulting in anemia and jaundice. G6PD deficiency is a genetic disorder(X-linked recessive).
Usually, G6PD deficiency either presents in the newborn period as neonatal jaundice or it can present in infancy( or in childhood/adult life) with sudden onset anemia and jaundice ( dark color urine may be seen), after exposure to some triggers.
The trigger may be a medicine, food, or a chemical. These triggers act like a catalyst and cause sudden onset moderate to severe RBC lysis- presenting as anemia and jaundice, in a G6PD deficient individual. Hospitalization is needed in severe cases.
Note, some patients suffer from chronic hemolytic anemia due to G6PD deficiency.
in India, the G6PD enzyme is deficient in 1-10% population. As the disease is widely prevalent it is usually done in all newborns as a universal screening program. If G6PD status is not checked already in your baby, you should do it as early as possible. The test can be done at any age.
list of substances that triggers hemolysis in G6PD deficient individual
1. FOOD ITEMS
Flava beans(33% of cases of hemolysis in g6pd deficiency are caused by flava beans).
Some researchers argue to remove all legumes from the diet(such as beans, peas, lentils, and peanuts) . But it’s not clear if completely avoiding all legumes is actually necessary.
2. CHEMICAL COMPOUNDS
Mothballs(naphthalene)
Henna
Aniline dye
Food coloring agent-1‐phenyl azo‐2‐naphthol‐6‐sulphonic acid
3.INFECTION(BACTERIAL OR VIRAL)
4. DRUGS
MONTHLY INDEX OF MEDICAL SPECIALTIES (MIMS) SUMMARY TABLE FOR DRUGS TO AVOID IN G6PD DEFICIENCY
Download link-MIMS Summary Table-G6PD
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