42 - Paediatric Haematology

Question 1

% of HbF at birth?

Answer 1

55-65%

Site of haematopoeisis varies

Question 2

chromosome 16 can make what

Answer 2

zeta -> alpha

Question 3

chromosome 11 can make what

Answer 3

epsilon -> gamma -> beta -> theta

Question 4

4-14 / 40 week gestation transition

Answer 4

Hb Gower-1 -> Hb Portland -> Hb Gower-2

Question 5

> 14/40 gestation

Answer 5

HbF (a2gamma2)

Question 6

neonatal stage

Answer 6

Hb A = a2b2

a2theta2 = HbA2

Question 7

Which Ig crosses the placenta?

Answer 7

IgG

Question 8

What does breast milk contain?

Answer 8

IgA, IgD, IgE, IgG, IgM -> passive immunity

Question 9

When do people start producing antibodies

Answer 9

2-3 months out of 12

Question 10

When are satisfactory immune responses made

Answer 10

6 out of 12 months

Question 11

When are immunisations given to babies?

Answer 11

3 months in

Question 12

Reach adult numbers of platelets when? and why?

Answer 12

18 weeks out of 40 gestation

to survive birth! bloody process!

Question 13

Platelets are functionally different at birth in what respect

Answer 13

Hyporesponsive to certain agonists

Hyperresponsive to vWF

Question 14

Clotting factors at birth

Answer 14

Present at birth but ineffective
Coagulation proteins CANNOT cross the placenta
This is to prevent clotting of placenta

Question 15

What clotting factors are normal at birth

Answer 15

Fibrinogen, FV, FVIII, FXIII are normal at birth

Question 16

When do haemostatic parameters reach adult values by?

Answer 16

six months

Question 17

Vitamin K dependent proteins

Answer 17

FII, FVII, FIX, FX, protein C, protein S are vit K dependent

Question 18

Haemorrhagic disease of new born

Answer 18

Babies just bleeding constantly from birth

Prevented by routine neonatal vit K

Question 19

Why is mothers having anti-convulsants a problem?

Answer 19

Uses same pathway at vitamin K so incites haemorrhagic new born

Question 20

Congenital anaemias in childhood

Answer 20

haemoglobinopathy
bone marrow failure syndromes
bone marrow infiltration
peripheral destruction
blood loss

Question 21

Causes of peripheral destruction in neonates

Answer 21

Rh/ABO or other incompatibility

Membrane defect: hereditary spherocytosis

Enzyme defects: G6PD, PK deficiency

Infection

Question 22

Blood loss in neonates

Answer 22

Twin to twin transfusion where one twin has bled into another twin

Question 23

Anaemia in childhood

Answer 23

Acquired: iron, B12, folate deficiency

Bone marrow failure
Bone marrow infiltration
Peripheral destruction: haemolysis
Blood loss

Question 24

B12 deficiency - common or?

Answer 24

Not really
Massive stores in body
Can last years after switching to vegan for example

Question 25

Folate deficiency - common or?

Answer 25

More common