G6PD Deficiency Flashcards
1
Q
Who does it affect?
Genetics
A
It is more common in people from the Mediterranean and Africa
is inherited in an X-linked recessive fashion
2
Q
Pathophysiology
A
↓ G6PD → ↓ glutathione → increased red cell susceptibility to oxidative stress
3
Q
Triggers of haemolysis
A
anti-malarials: primaquine
ciprofloxacin
sulph- group drugs: sulphonamides, sulphasalazine,
sulfonylureas
Fava beans
4
Q
Clinical Features
A
neonatal jaundice is often seen
intravascular haemolysis
gallstones are common
splenomegaly may be present
5
Q
Investigations
A
Blood films: heinz bodies, bite cells
G6PD enzyme assay
6
Q
Management
A
Resuscitate with fluids
Folate to encourage RBC production
If Hb <7 = blood transfusion
Can consider splenectomy
