[sicklecell] Flashcards
1
Q
A
Beta
2
Q
A
Glutamic acid –> valine
3
Q
A
HbS
4
Q
A
africans
5
Q
A
heterozygotes (HbAS)
6
Q
A
anaemia
7
Q
A
heterozygote (HbAS)
8
Q
A
hypoxia
anaethesia/enclosed spaces etc
9
Q
A
vessels occluded by build up of sickle cells leading to painful ischaemia
10
Q
A
thalassaemia
HbC
11
Q
A
polymerisation of deformed HbS when deoxygenated
12
Q
A
sickle cells are fragile
13
Q
A
sickle cells
Target cells
14
Q
A
Hb electrophoresis
15
Q
A
allows distinction between HbSS and HbAS
16
Q
A
at birth (cord blood)
17
Q
A
pneumococcal prophylaxis
18
Q
A
sickle cell reduces spleen function i.e.immunocompromised
19
Q
A
vaso-occlusive crisis
20
Q
A
acute abdomen
21
Q
A
vaso-occlusive crisis
22
Q
A
doppler US
23
Q
A
bone
e.g. avascular necrosis of femoral head
24
Q
A
priapism
25
[sicklecell]: which virus is associated with an aplastic crisis
parvovirus b19
| invades and destroys reticulocytes
26
[sicklecell]: An aplastic crisis results in a reduction in
especially RBCs
27
[sicklecell]: why do sequestration crises usally affect children
the spleen hasnt undergone atrophy
28
[sicklecell]: what is a sequestration crisis
intrasplenic trapping of RBCs usually affecting children
29
[sicklecell]: what is the main acute medical emergency associated with SC
sequestration crisis
death in 1-2 hours due to circulatory failure
30
[sicklecell]: 40% of SC deaths are caused by .... due to splenic occlusion
infection
31
[sicklecell]: Tx: 1st line in an acute crisis?
hydroxycarbamide
32
[sicklecell]: Tx: what is the MOA of the 1st line drug used in an acute crisis?
increases the level of HbF
33
[sicklecell]: Tx: only curative treatment?
bone marrow transplant
34
[sicklecell]: Tx: for sequestration crisis
urgent transfusion
35
[sicklecell]: CRISIS: in which crisis do patients get prodromal painful crisis 2.5 days before?
acute chest syndrome
36
[sicklecell]: CRISIS: what is the pathophysiology behind
pulmonary infiltrates
37
[sicklecell]: CRISIS: acute chest syndrome symptoms (3)
tachypnoea
pain
fever
38
[sicklecell]: CRISIS: what is the Tx for acute chest syndrome (4)
O2
analgesia
emirical antibiotics
transfusion if severe (X-match)
39
[sicklecell]: CRISIS: what is the most common infiltrates into the lung in acute chest syndrome (2)
fat embolism
| infection
40
[sicklecell]: CRISIS: what are the most common infectiour organisms in acute chest syndrome (2)
chlamydia
| mycoplasma
41
[sicklecell]: Tx: what is the indication for blood transfusion? (3)
severe chest crisis
CNS event
multiorgan failure
