Anaemia Flashcards
What is the commonest cause of anaemia in children?
Iron deficiency anaemia
What are the symptoms of anaemia?
Malaise and lethargy
Pallor
Breathlessness
In haemolytic anaemia:
Unconjugated jaundice
Splenomegaly
What are the different causes of haemolytic anaemia in children?
Hereditary Spherocytosis
G6PD Deficiency
Pyruvate Kinase Deficiency
Chronic bleeding
What is hereditary spherocytosis and how is it managed?
It is an autosomal dominant hereditary disorder of RBC’s which causes RBC’s to be elongated.
This gives them a greater oxygen carrying capacity but also makes them more fragile and increases breakdown.
Managed with folate supplementation in mild disease.
What is G6DP deficiency?
Commonest red cell enzyme problem worldwide. (Most common in central africa)
It is an x-linked condition but female carriers have about 50% function and are clinically normal.
G6PD usually stops oxidative damage to the cell, so when it is deficient there is increased breakdown.
How does G6DP deficiency usually present?
Neonatal jaundice in the first 3 days or acute haemolysis precipitated by infection.
In between periods of haemolysis patients may be healthy.
What is pyruvate kinase deficiency?
2nd most common cause of haemolytic anaemia and is due to a lack of an enzyme vital in the final stage of glycolysis leading to a decrease in ATP.
This causes the RBC to be more rigid and increases destruction in the spleen.
How should you investigate anaemia?
FBC
If abnormal———> Blood film
Jaundiced?
Bilirubin level + LFT’s
If G6DP suspected investigate G6PD activity in cells.
How should you manage G6PD deficiency?
Drug and food advice as certain drugs and foods can trigger haemolysis.
May need exchange transfusions in emergencies.
What are the indications of splenectomy?
Severe hereditary spherocytosis
Lymphoma with spleen involvement
Very refractive ITP
Uncontrollable spleen haemorrhage
What is the management for patient that needs a splenectomy?
Before:
Immunisations for: Pneumococcus, Hib, Meningococcus and Influenza
After prophylactic antibiotics for life.
