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G6pd deficiency

G6pd deficiency
G6pd deficiency
G6pd deficiency
G6pd deficiency

Introduction

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.

Risk Factors

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Signs and symptoms

• 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)

Diagnostic criteria

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Investigations

• Anaemia
• Peripheral smear-normocytic normochromic
• Spherocytes
• Bite cell
• Reticlocytosis
• Heinz bodies
• Positive Ham’s test

Treatment

  • Non-pharmacological

    • 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

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

Prevention

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Updated on,

14 Novemba 2020 11:35:36

References

    1. STG
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