Paediatric haemotology

Question 1

what is the structure of foetal haemoglobin

Answer 1

2 alpha chains

2 gamma chains

Question 2

haematopoiesis- where does it start in a fetes and where does it end up in an adult

Answer 2

o Initially yolk sac until 2..5 months, then stops
o Liver- 1 month peaks at 4/5 months
o Spleen makes RBC again at 1 month and stops 7 months
o Bone marrow makes RBC by birth

Question 3

If you have a problem with you bone marrow which 2 organs take of the haematopoiesis function

Answer 3

spleen and liver.

Question 4

when does haemoglobin with take place from foetal to adult

Answer 4

at birth.

Question 5

chromosome 16 gives rise to which globulin chains

Answer 5

alpha, zeta

Question 6

chromosome 11 gives rise to which globulin chains

Answer 6

gamma, beta, delta, epsilon

Question 7

what chains does HB gower consist of.

Answer 7

zeta/epsilon

Question 8

what chains does Hb portalnd consist of.

Answer 8

zeta/gamma

Question 9

which immunglobulin crosses the placenta

Answer 9

IgG

Question 10

breast milk contains which antibodies

Answer 10

Ig A, IgD, IgE, IgG, IgM

Question 11

Are the WBC, same, lower or higher in children in comparison to adults

Answer 11

same.

Question 12

at what age can you make a satisfactory immune response

Answer 12

6 months.

Question 13

at what age do you start producing your own antibodies

Answer 13

2 to 3 months.

Question 14

Are platelets the same, lower or higher in children in comparison to adults.

Answer 14

higher

Birth is a bloody process to develop platelets to prevent affects of haemorrhage.

Question 15

what clotting cascade factors are normal at birth.

Answer 15

Only fibrinogen, FV, FVIII, FXIII

Question 16

at what age do all haemostatic (clotting cascade) parameters each normal.

Answer 16

by 6 months

Question 17

Is Vitamin K level the same, lower or higher in newborns

Answer 17

foetal vitamin K is 10% mother

Question 18

what is done in order to make sure that neonates are not vitamin K dependent

Answer 18

given a dose of vitamin K at birth.

Question 19

what blood thinner is teratogenic

Answer 19

warfarin

Question 20

what drug use exacerbates vitamin K deficiency

Answer 20

anti-convulsants.

Question 21

what factors are vitamin k dependent

Answer 21

2,7,9,10, protein C, protein S.

Question 22

what are the congenital causes of anaemia in childhood

Answer 22

• Haemoglobin synthesis problem- haemoglobinopathy
• Bone marrow failure syndrome.
• Bone marrow infiltration- storage disorders.
• Peripheral destruction
• Blood- losing blood.

Question 23

name 2 common haemoglobinopathies

Answer 23

• Thalassemia- cell look random shape and sizes.

* Sickle cell disease- (irreversible sickle cell)- not flexible and clog up blood vessel.

Question 24

how are haemoglobinoptathies diagnosed before the childs birth.

Answer 24

antenatal screening

Question 25

main causes of peripheral destruction blood disorders

Answer 25

• Rh/ABO or other incompatibility
• Membrane defect: hereditary spherocytosis (haemoglobin pressed into a dense compact manner causing them to be brittle)
• Enzyme defect G6PD deficiency, PK deficiency
• Infection.

Question 26

which more prevelanet prenatally ABO or Rh incompatibility and why

Answer 26

o ABO incompatibility- make IgM which cannot cross placenta, ABO on RBC after birth, not common prenatally.
o Rh- has significant effects prenatally.

Question 27

what is the prenatal treatment for Rh incompatibility

Answer 27

Given anti-D prophylaxis, so it removes antibodies that the mother develops.

Question 28

what causes acquired childhood anaemias

Answer 28

• Nutritional deficiency: iron (common in 2-4 yrs old- due to milk based diet), B12 (rare), folate (very rare).
• Bone marrow failure- Aplastic anaemia- idiopathic or liver disease associated.
• Bone marrow infiltration- stops anything else from happen in in the bone marrow.
• Peripheral destruction: haemolysis
• Blood loss.

Question 29

congential bleeding problems

Answer 29

• Platelet problems- most common, low platelet due to mothers taking medicines e.g for ITP, gestational thrombocytopenia.
• Clotting factor e.g. circumcision
• Connective tissue disorder.

Question 30

acquired bleeding problems

Answer 30

• Trauma: accidental, non accidental
• Tumour e.g. leukaemia, can cause bleeding.
• Infection: acute eg meningococcus (treatment platelets), chronic eg HIV
• Immune disorder primary- immune thrombocytopenia, TTP (similar symptoms to meningioccous)
Secondary-SLE, ALPS (Autoimmune lymphoproliferative syndrome- range of autoimmune conditions- difficult to mamage)
• Bone marrow failure
• Drug related.