Pead Haemo Flashcards

1
Q

where are the various haematopoiesis sites in the foetus

A

yolk sac - liver - spleen - bone marrow

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

why does foetal HbF have gamma chains

A

HbF has higher affinity for O2 so binds more strongly - take from mothers blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

which Hb chains do chromosome 16 and 11 code for

A

16 - zeta or alpha chains

11 - epsilon gamma or beta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

in the first trimester what does the Hb start as and what can that turn into

A

Hb- gower- 1 = Hb zeta2/epsilon2

turns into Hb Portland (if the epsilon changes to gamma) or Hb-gower-2 (if the zeta changes to alpha)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what happens to Hb after the first trimester

A

changes from Hb portland (Hb zeta2/gamma2) and Hb-gower2 (Hb alpha2/epsilon2) to HbF resulting in both being (alpha2/gamma2)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what happens to Hb at the neonatal stage

A

Hb changes from HbF to HbA by switching the gamma to beta = Hb alpha2/beta2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the compensation of WBC in child vs adult

A

higher numbers of WBC in children but they are not as effective

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are the differences for platelets in adult vs child

A

adult numbers reached by 18th week gestation
large but then shrink at brith
functionally different but same effectiveness
ie baby hyper responsive to vWf but hypo to certain agonists

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what assistance does the child immunity have

A

IgG cross placenta
IgA/D/E/M pass in breast milk
antibodies produced at 2-3 months
usually normal by 6 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

why are there bigger platelets made for compensation in the child

A

coagulation factors don’t cross placenta easily

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

which coagulation factors are present in normal amounts at birth

A

fibrinogen, factors V, VIII, XIII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

which factors / protein are vit K dependant

A

II, VII, IX, X protein C/S

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is haemorrhage disease of the new born

A

immature liver makes insufficient vit K = lack of vit k dependent coagulation factors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

why should you not use warfarin in children

A

teratogenic due to vit K depletion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what are examples of congenital childhood anaemias

A
haemoglobinophathy 
bone marrow failure syndromes 
bone marrow infiltration 
peripheral destruction 
blood loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what haemoglobinopathies causes anaemia in childhood

A

thalassaemia and sickle cell

17
Q

which childhood congenital anaemias cause peripheral destruction

A

Rh/ABO or the incompatibility
infection
membrane defect (hereditary spherocytosis)
enzyme defects

18
Q

what is an example of blood loss congenital anaemia in childhood

A

twin to twin transfusion - one twin takes the other blood

19
Q

what are 5 examples of acquired anaemia in childhood

A

iron, B12 or folate deficiency
bone marrow failure / infiltration (malignancy)
peripheral destruction
blood loss

20
Q

what are the differences in congenital vs acquired bleeding/bruising disorders

A

congenital - platelet problem, clotting factor problem, connective tissue disorder

acquired - trauma, tumour, infection, immune disorder, bone marrow failure, drug related