17078545
Descrição
Quiz por Matthew Coulson, atualizado more than 1 year ago
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Criado por Matthew Coulson
mais de 5 anos atrás
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Questão 1
Questão
Major forms of Hb:
HbA = 2 Alpha chains & 2 [blank_start]Beta[blank_end] chains
HbA2 = 2 Alpha chains & 2 [blank_start]Delta[blank_end] chains
HbF = 2 Alpha chains & 2 [blank_start]Gamma[blank_end] chains
Responda
-
Beta
-
Delta
-
Gamma
-
Delta
-
Beta
-
Gamma
-
Theta
-
Gamma
-
Delta
-
Beta
-
Theta
-
Theta
Questão 2
Questão
The most abundant Hb in the normal human body is [blank_start]HbA[blank_end]
Responda
-
HbA
-
HbA2
-
HbF
Questão 3
Questão
Alpha genes lie on chromosome 16. There are [blank_start]4[blank_end] alpha genes per cell ([blank_start]2[blank_end] on each chromosome)
Beta genes lie on chromosome 11. There are [blank_start]2[blank_end] beta genes per cell ([blank_start]1[blank_end] on each chromosome)
Responda
-
4
-
2
-
6
-
2
-
1
-
3
-
2
-
4
-
6
-
1
-
2
-
3
Questão 4
Questão
Adult levels of HbA are typically achieved by [blank_start]12 months[blank_end] of age
Responda
-
12 months
-
3 months
-
18 months
-
2 years
Questão 5
Questão
Haemoglobinopathies are hereditary conditions which affect the synthesis of [blank_start]globin[blank_end] chains, thus affecting haemoglobin production.
Responda
-
globin
Questão 6
Questão
Haemoglobinopathies whereby there is a decreased rate of globin chain synthesis are called [blank_start]thalassaemias[blank_end]
Responda
-
thalassaemias
Questão 7
Questão
Reduced rate of production of alpha globin chains is known as [blank_start]alpha thalassaemia[blank_end]
Reduced rate of production of beta globin chains is known as [blank_start]beta thalassaemia[blank_end]
Responda
-
alpha thalassaemia
-
beta thalassaemia
Questão 8
Questão
Thalassaemias lead to [blank_start]microcytic[blank_end] anaemia
Responda
-
microcytic
-
macrocytic
Questão 9
Questão
[blank_start]Alpha Thalassaemia Trait[blank_end] = 1 or 2 alpha genes missing (i.e -α/αα OR -α/-α)
[blank_start]Haemoglobin H Disease (HbH)[blank_end] = 3 alpha genes missing (i.e. (--/-α)
[blank_start]Barts Hydrops Fetalis[blank_end] = All 4 alpha genes missing (i.e. (--/--)
Responda
-
Barts Hydrops Fetalis
-
Haemoglobin H Disease (HbH)
-
Alpha Thalassaemia Trait
Questão 10
Questão
Alpha Thalassaemia Trait is the most severe form of the condition and patients rarely survive past infancy.
Responda
- True
- False
Questão 11
Questão
HbH disease causes strange 3 beta 1 alpha tetramers to form which can't carry oxygen, thus causing severe anaemia.
Responda
- True
- False
Questão 12
Questão
The skin of HbH Disease patient may be slightly discoloured as this condition is known to cause a mild [blank_start]jaundice[blank_end]
Responda
-
jaundice
Questão 13
Questão
Extramedullary haematopoiesis in HbH Disease causes the pathological enlargement of which organ?
The [blank_start]Spleen[blank_end]
Responda
-
Spleen
Questão 14
Questão
Hb Barts Hydrops Fetalis is a condition whereby HbA cannot be produced due to the inability to synthesize alpha chains. This condition is typically incompatible with life.
Responda
- True
- False
Questão 15
Questão
[blank_start]Alpha[blank_end] Thalassaemia is considered to be more dangerous that [blank_start]Beta[blank_end] Thalassaemia as the former affects HbA, HbA2 & HbF whereas the latter only affects HbA
Responda
-
Beta
-
Alpha
-
Alpha
-
Beta
Questão 16
Questão
β+/β or β0/β = Beta Thalassaemia [blank_start]Trait[blank_end]
β+/β+ or β0/β+ = Beta Thalassaemia [blank_start]Intermedia[blank_end]
β0/β0 = Beta Thalassaemia [blank_start]Major[blank_end]
Responda
-
Trait
-
Intermedia
-
Major
Questão 17
Questão
Beta Thalassaemia Trait = [blank_start]Asymptomatic[blank_end]
Beta Thalassaemia Intermedia = [blank_start]Occasional Transfusions Required[blank_end]
Beta Thalassaemia Major = [blank_start]Lifelong Transfusions Required[blank_end]
Responda
-
Asymptomatic
-
Occasional Transfusions Required
-
Lifelong Transfusions Required
Questão 18
Questão
Beta thalassaemia major present around 6-24 months of age as the individual's [blank_start]HbF[blank_end] falls
Responda
-
HbF
-
HbA
-
HbA2
Questão 19
Questão
Children with beta thalassaemia major will typically present with which two key clinical signs?
Responda
-
Pallor
-
Failure to thrive
-
Reduced consciousness
-
Reduced appetite
-
Loss of accommodation reflex
Questão 20
Questão
Beta thalassaemia major can be separated from Alpha thalassaemia by looking at the child's blood - there will be an abundance of HbF in young beta thalassaemia patients.
Responda
- True
- False
Questão 21
Questão
Regular transfusion of patients with beta thalassaemia major aims to keep the Hb level at around [blank_start]95-105[blank_end]g/L
Responda
-
95-105
-
105-15
-
115-125
Questão 22
Questão
The main cause of mortality in beta thalassaemia major patients who are well treated is [blank_start]iron[blank_end] overload from the regular blood transfusions
Responda
-
iron
Questão 23
Questão
Beta thalassaemia major patient with iron overload are typically treated via venesection
Responda
- True
- False
Questão 24
Questão
Sickle cell disease is caused by a [blank_start]point[blank_end] mutation in codon 6 of the [blank_start]beta[blank_end] globin gene that substitutes glutamine to value producing Beta Globin S
Responda
-
beta
-
point
Questão 25
Questão
When you have one normal (B) and one abnormal (BS) gene this is known as sickle [blank_start]trait[blank_end]. This is asymptomatic and makes you a carrier of sickle cell disease (not affected by the condition).
Responda
-
trait
Questão 26
Questão
Sickle cell anaemia follows which kind of inheritance pattern?
Autosomal [blank_start]Recessive[blank_end].
Responda
-
Recessive
Questão 27
Questão
Sickle Cell Anaemia causes [blank_start]Hyposplenism[blank_end] due to repeated splenic infarcts
Responda
-
Hyposplenism
-
Hypersplenism
Questão 28
Questão
A patient with known HbSS is out with friends hiking. He progressively develops chest pain, shortness of breath, headache and skin pallor. He is likely experiencing a [blank_start]sickle crisis[blank_end]
Responda
-
sickle crisis
Questão 29
Questão
Sickle cells can become stuck in arteries leading to sickle vaso-occlusion. This can cause a patient extreme [blank_start]pain[blank_end]
Responda
-
pain
-
dizziness
-
itchiness
-
nausea
Questão 30
Questão
Which of the following are common causes of sickle crises in HbSS patients?
Responda
-
Hypoxia
-
Dehydration
-
Infection
-
Stress
-
Cold Exposure
Questão 31
Questão
A Sickle Crisis should be managed by providing analgesia (if pain present, which it likely will be), hydration, oxygen and rest.
Responda
- True
- False
Questão 32
Questão
Long term management of Sickle Cell Disease includes:
- [blank_start]Folic Acid[blank_end] supplementation to increase RBC turnover
- Hydroxycarbamide to induce produce of [blank_start]HbF[blank_end]
Responda
-
HbF
-
HbA
-
HbA2
-
Folic Acid (Vitamin B9)
-
Vitamin B12
-
Vitamin A
Questão 33
Questão
Due to the risk of occlusion, sickle cell anaemia patients are at a significantly higher risk of experiencing a stroke.
Responda
- True
- False
Questão 34
Questão
Raised HbA2 is diagnostic of which type of Thalassaemia?
[blank_start]Beta Thalassaemia Trait[blank_end]
Responda
-
Beta Thalassaemia Trait
-
Alpha Thalassaemia Trait
-
HbH Disease
-
Barts Hydrops Fetalis
Questão 35
Questão
HPLC blood testing is normal in [blank_start]alpha[blank_end] thalassaemia trait and thus DNA testing is necessary to confirm the condition.
Responda
-
alpha
-
beta
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