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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