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CSI > Sicle Cell Disease > Flashcards

Sicle Cell Disease Flashcards

1
Q

What base substitution occurs in HbS

A

Charged glutamic acid changed to uncharged valine at position 6

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2
Q

What does Hb molecule consist of

A

2 alpha and 2 beta chains

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3
Q

What genetic makeup causes sickle cell anaemia

A

HbSS
Autosomal recessive

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4
Q

What genetic makeup causes sickle cell trait

A

HbAS oxygen affinity is the same
Asymptomatic

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5
Q

What happens to the reticulocyte count in SCD

A

increases as a compensatory mechanism due to increased levels of haemopoeisis

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6
Q

What is splenomegaly

A

Increase in size of spleen due to accumulation of degraded erythrocytes

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7
Q

How does sickle cell anaemia lead to vision loss

A

Sickle cells accumulate within micro vessels in retina increasing the pressure and damaging vessels

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8
Q

Vision loss

A

Sickle cell ischaemia-released chemokines-angiogenesis of collaterals to maintain oxygen to retina-spread across eye

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9
Q

What is an ischaemic stroke

A

Cerebral artery is blocked resulting in oxygen deficiency

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10
Q

What causes a sickle cell crises

A

Cold weather
Strenuous exercise
Stress
Dehydration

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11
Q

Microvascular occlusion caused by

A

When sicklesd red blood cells block small blood vessels

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12
Q

Bone crises mechanism

A

Reduced blood flow to bone marrow due to sickling reuksting in microvascukar occlusion
Oxygen deprivation-subsequent prolonged ischaemia-infarction-sickling

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13
Q

Sickle solubility test

A

Add sodium dithionite
Hb is released
HbA dissolves easily in plasma
HbS less soluble and causes solution to become turbid
Can’t differentiate between HbSS and HbAS

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14
Q

What causes gall stones

A

Billirubin release

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15
Q

Splenic sequesteration

A

Large volume of blood pooled in spleen
Sudden drop in Hb

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16
Q

In electrophoresis

A

HbA closest to anode
HbS further away as valine is uncharged so less attracted to anode
In sickle cell anaemia HbS is thick whereas not present if you don’t have the disease
Carried out on cellulose acetate gel

17
Q

Why is sickle solubility test not that effective

A

As it doesn’t differentiate between sickle cell trait and sickle cell anaemia

18
Q

What is used to screen newborns

A

Heel prick test

19
Q

Treatments

A

Blood transfusion
Hydroxycarbamide
Bone marrow transplant
Gene therapy
Antibiotics

20
Q

How does hydroxycarbamide work

A

Stimulates synthesis of HbF
Suppresses marrow production of reticulocytes and neutrophils

21
Q

Globin chains contain

A

HbA: two alpha, two beta.

HbA2: two alpha, two delta

HbF (Fetal Haemoglobin): two alpha, two gamma.

22
Q

What causes sickling

A

During deoxygenation hydrophobic valine protrudes from the surface causing interactions between valine on one molecule with phenylaline leucine and alanine

23
Q

Haemolysis

A

Repeated sickling weakens cell membrane
Results in intravascular haemolysis
Sickle cells only last for 20 days reuksting in anaemia

24
Q

Crispr

A

Cas9 enzyme can target and repair the faulty B Globin gene
Cas9 promotes the production of fetal haemoglobin by breaking a gene that encodes a repressed

25
Q

Intravascular Haemolysis

A

When a red blood cell ruptures

26
Q

Myoglobin

A

Is an oxygen binding protein found in muscle

27
Q

Positive cooperation

A

Once an oxygen molecule binds to haem it becomes easier for the next oxygen molecule to bind

28
Q

Vasocclusion

A

Blockage of capillaries

29
Q

What to they have an increased susceptibility to

A

Encapsulated bacteria

30
Q

Zolas triggers

A

Interpersonal crises eg divorce/death
Perceived interference with social/personal relations(affects social life)
Sanctioning (pressure from others)
Perceived interference with vocational/physical activity eg affecting job
Temporalizing of symptomatology eg setting deadlines

31
Q

Hellman’s folk model

A

What happened
Why has it happened
Why me
Why now
What would happen if nothing was done about this

CSI (7 decks)

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