21 Questions
Which type of inheritance pattern is associated with sickle cell disease?
Autosomal recessive
What is the composition of sickle haemoglobin (HbS)?
Alpha-2 β (sickle) chains
Which term describes individuals who are carriers of sickle cell disease but are asymptomatic?
HbAS
What triggers the pathophysiology of sickle cell disease?
Vaso-occlusion
What is the chance of having an affected child if both parents are carriers of sickle cell disease?
1 in 4 chance
Which condition is characterized by a reduced or absent production of globin chains?
Thalassaemia
What is the main consequence of homozygous alpha zero thalassaemia (α0/α0)?
Hydrops Fetalis
Which of the following events can trigger a sickle crisis in sickle cell disease?
Dehydration
Which type of anaemia is present in beta thalassaemia major?
Transfusion-dependent anaemia
What is the primary management strategy for G6PD deficiency?
Avoidance of certain medications and foods
In which condition should hydration, oxygenation, and prompt treatment of infection be part of the management?
Sickle cell disease
What is the most common disease causing enzymopathy in the world?
G6PD Deficiency
Which enzyme deficiency leads to increased rigidity of red blood cells and variable severity of symptoms?
Pyruvate Kinase Deficiency
What triggers hemolysis in G6PD Deficiency?
Oxidative stress
In which part of the body is Erythropoietin synthesized in response to hypoxia?
Kidney
Which type of hemoglobin has a higher oxygen affinity than HbA?
Hb F
What is the normal percentage of Hemoglobin F found in adults?
1%
Which condition results from mutations leading to structurally abnormal globin chains?
Sickle Cell Anemia
What happens in G6PD Deficiency when triggered by oxidative stress?
Scavenging and detoxifying reactive oxygen species
Which drug can trigger hemolysis in individuals with G6PD Deficiency?
Antimalarials
What role does Hemoglobin play in gas exchange within the body?
Transporting CO2 to tissues
Test your knowledge on red cell physiology and congenital anaemias with this quiz focused on understanding anaemia, red cell production in the marrow, substances required for red cell production, and more.
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