G6PD (Glucose 6 phosphate dehydrogenase) deficiency
G6PD is a condition where there is a defect in RBC enzyme. In fact, it is the commonest RBC enzyme defect found. It is an X-linked inherited and mainly affect females. The countries that have high prevalence of this disease are in Africa, Mediterranean, Middle and Far East.
Have been diagnosed with G6PD deficiency doesn’t mean that you are sick. Most patients are asymptomatic. However, it is important to remember that you are susceptible to oxidative crisis due to decrease of glutathione production. This thing can happen during ill or exposure to the broad bean Vicia fava (favism). Some drugs like aspirin, primaquine and sulfonamides also may lead to this problem.
During oxidative crisis, you will experience rapid anaemia and jaundice (yellowish discolouration of the skin). Full blood film will show bite cells and blister cells.
In view of diagnosis, usually all the newborns will be screened for G6PD deficiency using a special enzyme assay. However, in adult, the test is not accurate if we do 2-3 weeks after the attack because young red blood cell may have sufficient enzyme and the result may appear normal.
Prevention is better than cure. If you have G6PD deficiency, you have to avoid the precipitators like the bean and some drugs. Whenever you see your doctor or buying any medications in the pharmacy, please note that you are having this condition so they will avoid prescribing any drug that may cause this crisis. In case that your symptom is severe, you may need blood transfusion and may need hospitalization till you are stable.
Reference:
1. Oxford handbook of Clinical Medicine. 7th edition. Oxford University Press. 2008.
Tags: anemia, blood transfusion, fava beans, favism, G6PD deficiency, gluthation, jaundice, oxidative crisis, x-linked
Technorati Tags: anemia, blood transfusion, fava beans, favism, G6PD deficiency, gluthation, jaundice, oxidative crisis, x-linked
I agree “Prevention is better than cure”.thanks for sharing this post..
Definitely!! I agree on that
Definitely!! I agree on that
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