Based on current understanding of haemolysis and glucose-6-phosphate dehydrogenase deficiency, infection is thought to be a probable cause. Numerous bacterial, viral and rickettsial infections have been reported as precipitants where of particular importance are infectious hepatitis, pneumonia and typhoid fever. 4.Some of these have already been examined more closely but were found to bear no relevance. Nevertheless, patients who are suffering from glucose-6-phosphate dehydrogenase deficiency are generally held to be more prone to infections. For example is an adult male patient with glucose-6-phosphate dehydrogenase deficiency whose leukocytes had an inability to even kill two very common bacteria, Escherichia coli and Staphylococcus aureus. 19.One plausible reason is an impairment in the function of leukocytes which are responsible for the active defence of the body against invading micro-organisms. 3.
More often than not, haemolysis in glucose-6-phosphate dehydrogenase deficient patients are self-limiting, which is true for the less severe African type invariably. This is a result of the replacement of old red blood cells by new young red blood cells which are able to overcome the oxidative stress encountered. Therefore, the management of glucose-6-phosphate dehydrogenase deficiency basically involves the avoidance of precipitating factors. 4.In more severe cases the administration of high doses of antioxidants such as Vitamin E have proven beneficial. 7.It should be noted that although Vitamin C is an antioxidant, paradoxically its administration in high doses have led to the precipitation of haemolysis! Single bolus dose of desferrioxamine is helpful in reducing iron-dependent formation of damaging oxidant radicals. Folic acid should be given for 2-3 weeks following an acute haemolytic event. For patients with chronic haemolysis, a long term treatment with 5mg folic acid is called for. A rather interesting proposal for the future is the use of gene replacement therapy utilising carrier viruses, as done in the treatment of cystic fibrosis today. 4.
The greatest concern currently to the health care professions is the recognition of potential complications associated with the prescribing of drugs to those who are glucose-6-phosphate dehydrogenase deficient. Steps have been taken to lay out guidelines like those provided in the British National Formulary.(Table 3). 5.
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Prepared on 01 Jan 2008 by teekoonhien |
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