Paediatric haemotology Flashcards Preview

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Flashcards in Paediatric haemotology Deck (30):
1

what is the structure of foetal haemoglobin

2 alpha chains
2 gamma chains

2

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

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

3

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

spleen and liver.

4

when does haemoglobin with take place from foetal to adult

at birth.

5

chromosome 16 gives rise to which globulin chains

alpha, zeta

6

chromosome 11 gives rise to which globulin chains

gamma, beta, delta, epsilon

7

what chains does HB gower consist of.

zeta/epsilon

8

what chains does Hb portalnd consist of.

zeta/gamma

9

which immunglobulin crosses the placenta

IgG

10

breast milk contains which antibodies

Ig A, IgD, IgE, IgG, IgM

11

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

same.

12

at what age can you make a satisfactory immune response

6 months.

13

at what age do you start producing your own antibodies

2 to 3 months.

14

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

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

15

what clotting cascade factors are normal at birth.

Only fibrinogen, FV, FVIII, FXIII

16

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

by 6 months

17

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

foetal vitamin K is 10% mother

18

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

given a dose of vitamin K at birth.

19

what blood thinner is teratogenic

warfarin

20

what drug use exacerbates vitamin K deficiency

anti-convulsants.

21

what factors are vitamin k dependent

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

22

what are the congenital causes of anaemia in childhood

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

23

name 2 common haemoglobinopathies

• Thalassemia- cell look random shape and sizes.
• Sickle cell disease- (irreversible sickle cell)- not flexible and clog up blood vessel.

24

how are haemoglobinoptathies diagnosed before the childs birth.

antenatal screening

25

main causes of peripheral destruction blood disorders

• 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.

26

which more prevelanet prenatally ABO or Rh incompatibility and why

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

27

what is the prenatal treatment for Rh incompatibility

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

28

what causes acquired childhood anaemias

• 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.

29

congential bleeding problems

• 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.

30

acquired bleeding problems

• 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.