G6PD Deficiency

 G6PD : Glucose -6- Phosphate -Dehydrogenase is an enzyme manifested in the X chromosome, one of whose function is to defend red blood cells from oxidative stress. Inadequate levels of this enzyme is referred to as G6PD deficiency.

As we are though in elementary biology class, humans have X and Y chromosomes. Men have XY chromosomes and women have XX chromosomes. To make a baby, biological parents donate chromosomes. When Y chromosome is donated (from father) to form a baby (mothers can only donate X chromosomes), a male child is the result.

G6PD deficiency is a deficiency in the X chromosome.  Since the likelihood of both donated X chromosomes (from father and mother) is not very high, females are less likely to suffer from severe GPD deficiency than male. That is in female, even if one X chromosome the other is likely to be healthy and compensate. But in males, if the X chromosome is G6PD deficient, that is it.

G6PD deficiency is a spectrum disease in that it varies in severity, and that fact is essential because it directly affects severity of risk.

The most obvious implication of G6PD deficiency is the consequent hemolysis in the presence of oxidative triggers.

Oxidative triggers could include disease conditions from infections such as the flu, food items such as fava beans and some mushrooms, and medications such as chloroquine and aspirin.

Hemolysis is basically the early death of red blood cells, which leads to anemia. When prolong, this condition could lead to organ damage, failure and death.

Early diagnosis, preferably at birth, is the best place to start defending against the consequence of a condition that is not preventable after conception. 

A good measure of ongoing hemolysis is the distribution width of red blood cells or erythrocytes (RDW) blood test. This value is usually obtained during CBC or complete blood count test.  Normal range of RDW in adult males is 11.8 to 14.5 and 12.2 to 16.1 in adult females. Younger (newer) cells have larger width than older one. The expected age of an erythrocyte is 6 weeks. When the rate of destruction or death of erythrocytes is quicker than the rate of birth or creation of new cells, the consequence is diminishing red blood cells or anemia. The bigger this difference, the more at risk a person becomes. 

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