Quiz
Haemoglobinopathies
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1Major 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- ABeta
- BDelta
- CGamma
- DDelta
- EBeta
- FGamma
- GTheta
- HGamma
- IDelta
- JBeta
- KTheta
- LTheta
2The most abundant Hb in the normal human body is [blank_start]HbA[blank_end]
- AHbA
- BHbA2
- CHbF
3Alpha 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)- A4
- B2
- C6
- D2
- E1
- F3
- G2
- H4
- I6
- J1
- K2
- L3
4Adult levels of HbA are typically achieved by [blank_start]12 months[blank_end] of age
- A12 months
- B3 months
- C18 months
- D2 years
5Haemoglobinopathies are hereditary conditions which affect the synthesis of [blank_start]globin[blank_end] chains, thus affecting haemoglobin production.
- Aglobin
6Haemoglobinopathies whereby there is a decreased rate of globin chain synthesis are called [blank_start]thalassaemias[blank_end]
- Athalassaemias
7Reduced 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]- Aalpha thalassaemia
- Bbeta thalassaemia
8Thalassaemias lead to [blank_start]microcytic[blank_end] anaemia
- Amicrocytic
- Bmacrocytic
9[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. (--/--)- ABarts Hydrops Fetalis
- BHaemoglobin H Disease (HbH)
- CAlpha Thalassaemia Trait
10Alpha Thalassaemia Trait is the most severe form of the condition and patients rarely survive past infancy.
- ARichtig
- BFalsch
11HbH disease causes strange 3 beta 1 alpha tetramers to form which can't carry oxygen, thus causing severe anaemia.
- ARichtig
- BFalsch
12The skin of HbH Disease patient may be slightly discoloured as this condition is known to cause a mild [blank_start]jaundice[blank_end]
- Ajaundice
13Extramedullary haematopoiesis in HbH Disease causes the pathological enlargement of which organ?
The [blank_start]Spleen[blank_end]- ASpleen
14Hb 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.
- ARichtig
- BFalsch
15[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
- ABeta
- BAlpha
- CAlpha
- DBeta
16β+/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]- ATrait
- BIntermedia
- CMajor
17Beta 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]- AAsymptomatic
- BOccasional Transfusions Required
- CLifelong Transfusions Required
18Beta thalassaemia major present around 6-24 months of age as the individual's [blank_start]HbF[blank_end] falls
- AHbF
- BHbA
- CHbA2
19Children with beta thalassaemia major will typically present with which two key clinical signs?
- APallor
- BFailure to thrive
- CReduced consciousness
- DReduced appetite
- ELoss of accommodation reflex
20Beta 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.
- ARichtig
- BFalsch
21Regular transfusion of patients with beta thalassaemia major aims to keep the Hb level at around [blank_start]95-105[blank_end]g/L
- A95-105
- B105-15
- C115-125
22The 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
- Airon
23Beta thalassaemia major patient with iron overload are typically treated via venesection
- ARichtig
- BFalsch
24Sickle 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
- Abeta
- Bpoint
25When 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).
- Atrait
26Sickle cell anaemia follows which kind of inheritance pattern?
Autosomal [blank_start]Recessive[blank_end].- ARecessive
27Sickle Cell Anaemia causes [blank_start]Hyposplenism[blank_end] due to repeated splenic infarcts
- AHyposplenism
- BHypersplenism
28A 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]
- Asickle crisis
29Sickle cells can become stuck in arteries leading to sickle vaso-occlusion. This can cause a patient extreme [blank_start]pain[blank_end]
- Apain
- Bdizziness
- Citchiness
- Dnausea
30Which of the following are common causes of sickle crises in HbSS patients?
- AHypoxia
- BDehydration
- CInfection
- DStress
- ECold Exposure
31A Sickle Crisis should be managed by providing analgesia (if pain present, which it likely will be), hydration, oxygen and rest.
- ARichtig
- BFalsch
32Long 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]- AHbF
- BHbA
- CHbA2
- DFolic Acid (Vitamin B9)
- EVitamin B12
- FVitamin A
33Due to the risk of occlusion, sickle cell anaemia patients are at a significantly higher risk of experiencing a stroke.
- ARichtig
- BFalsch
34Raised HbA2 is diagnostic of which type of Thalassaemia?
[blank_start]Beta Thalassaemia Trait[blank_end]- ABeta Thalassaemia Trait
- BAlpha Thalassaemia Trait
- CHbH Disease
- DBarts Hydrops Fetalis
35HPLC blood testing is normal in [blank_start]alpha[blank_end] thalassaemia trait and thus DNA testing is necessary to confirm the condition.
- Aalpha
- Bbeta
Informationen
- Beschreibung
- Talking about Thalassaemias and Sickle Cell Disease
- Autor
- Matthew Coulson
- Sprache
- English
- Aufrufe
- 69
- Schlagwörter
- Bewertung
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