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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:

  • Usually asymptomatic but liable to haemolysis if infection, incriminated drugs or foods are taken (e.g. sulphonamides, fava beans, tabs chloroquine or proguanil).

  • 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 

  • Avoid incriminated agents/foods or drugs

  • 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

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