How can a child’s immune response be jeopardized in the condition, G6PD?

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G6PD (glucose-6-phosphate dehydrogenase) is a common hereditary condition seen in over 400 million children worldwide. It is commonly known as Favism and caused by inherited deficiency of the enzyme G6PD. The deficiency of the enzyme triggers destruction of the red blood cells which is commonly known as haemolysis and thereby the child invariable suffers from anaemia when consuming Fava beans, a certain broad bean or while exposed to inhalation of these broad bean pollens or during intake of certain drugs. In severe cases when there is literally absence of this enzyme, the child might also be exposed to life-threatening bacterial and fungal infections. However, these conditions go unnoticed by most paediatricians until the problems and severity of the associated infections prop up.

To gain clear access of the mechanistic link between immune response and G6PD deficiency the scientists at University of Zurich and children’s research centre now reveal the entire underlying process behind the condition. G6PD enzyme plays a vital role in cell metabolism of the pentose phosphate pathway functioning. The co-factors Nicotinamide Adenine Dinucleotide (NADPH) have two primary functions in the entire mechanism:

Firstly, it guarantees sufficient glutathione that is present in the red blood cells of the body, which protect the cells with its dense antioxidant potential.

On the other hand the second function the co-factor NADPH does is to allow the production of reactive oxygen compound present in the immune system. These cells formed help in fending off bacterial and fungal infections

In the case of G6PD deficiency, the cells will not possess the protection of the co-factors, its antioxidant properties against oxidative stress, thus are prone to haemolysis. In India, where 340,000 cases of G6PD deficiency are detected annually, Newborn Screening is a real boon to help its kids from getting exposed to life-threatening conditions.

Summarising the entire study Scientist Reichenbach from Germany stated that “In order to determine the need for permanent antimicrobial prophylactic measures, the severity of the G6PD deficiency has to be determined first during the diagnosis”. He also quoted that in severe enzyme deficiency it is also good to probe into the formation of reactive oxygen compounds and DNA, to make the Newborn screening assessments more conclusive.

Once a child is detected with G6PD sufficient dietary changes is prescribed to the child in order to avoid the chemicals that cause the destruction of red blood cells. Even during intake of drugs and medication for certain infections and illnesses, there could be a need to avoid the use of certain medications that cause the destruction of red blood cells. A daily dose of antibiotics prescribed by the doctor may help in preventing  infections in these kids. If the child encounters an episode of hemolysis a blood transfusion to replenish healthy red blood cells is also recommended.

Ref: http://www.news-medical.net/news/20160726/Scientists-clarify-mechanism-of-defective-immune-defense-in-G6PD-patients.aspx

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