By ULY CLINIC
G6pd Deficiency
G6PD is an inherited X-linkded recessive genetic disorder, haemolysis results from oxidative damage to RBCs due to loss of protective effect of the enzyme G6PD.
Clinical Features:
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Usually asymptomatic but liable to haemolysis if infection, incriminated drugs or foods are taken (e.g. sulphonamides, fava beans, tabs chloroquine or proguanil).
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Pallor, jaundice and dark urine(Coca-colored urine)
Laboratory findings
Anaemia, peripheral smear-normocytic normochromic, spherocytes, bite cell, Reticlocytosis, Heinz bodies, Positive Ham’s test
Non-pharmacological Treatment
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Avoid incriminated agents/foods or drugs
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Transfusion of packed red blood cells in severe anaemia. Give 10ml/kg body weight over a period of 8 hours. Then assess the level of haemoglobin.
Pharmacological Treatment
Folic acid 5mg (PO) once daily for 1 month
Updated on, 27.10.2020
References
1. STG page number 10-12