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3.4: G6PD Deficiency

Table of Contents

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

A: Folic acid 5mg (PO) once daily for 1 month

 

Also see

3.6: Fanconi Anemia

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