G6PD (glucose 6-phosphate dehydrogenase) deficiency

Posted on: Monday, June 4th, 2018 @ 12:02 AM - By: Mohemet - Seen 501 Times

G6PD (glucose 6-phosphate dehydrogenase) deficiency or Favism

Presenting symptoms:

jaundice, pallor, tea colored urine. there might be personal or family history of the condition. ask about taking fava beans or any offending drug that may trigger the condition e.g. Metheprim.

Investigations:

CBC, BG and Rh, TSB if Hb level is <8gm/dl prepare PRBC and send for CBP with blood film, retic count, ccombs test, G6PD assay.

Treatment:

IVF maintenance (if there are signs of severe dehydration and shock due to severe anemia give shoot NS 20 ml/kg)

If Hb level is <8gm/dl give PRBC 10-15 ml/kg followed by Furosemide amp 1mg/kg

NOTICE: Do not give blood until blood has been taken for CBP and blood film!

Collect urine in bottle for color follow up

Follow up of Hb level every 12 hour

When to discharge:

if retic count normalized

urine color cleared

Filed under: Hematology