Disorders of red cell metabolism-Enzymopathies Flashcards
1
Q
how does a mature RBC produce energy?
A
emden-meyerhof pathway
2
Q
Most common enzyme deficiency of the Embden-meyerhof pathway
A
Pyruvate kinase deficiency
3
Q
Why is there a lack of clinical symptoms of anaemia in PK def?
A
as PK is positioned at the end of the emden-meyerhof pathway
4
Q
treatment of PK def
A
- folic acid
2. severe cases - splenectomy
5
Q
which type of deficiency has more severe sxs of anameia?
hexokinase or pk
A
hexokinase
6
Q
3 triggers of acute haemolysis?
A
- ingestion of oxidant drugs
- ingestion of common broad bean
- infection
