G6PDH deficiency

- reduced activity of G6PDH - x linked defect - decrease enzyme lowers levels of NADPH - RBC are most affected as pentose pathways is the only way to generate NADPH - - oxidative damage occurs - haemoglobin and other proteins become crosslinked via disulphide bonds forming oxidised SH groups - form insoluble aggregates called Heinz bodies - attach to RBC membrane - kill RBC causes haemolysis - asymptomatic unless chemicals i.e. antimalarials and foods, infections or stress....