Paeds - haem

Question 1

What investigation MUST be done in a patient with ?ITP, before giving IVIG + steroids?

Answer 1

Bone marrow aspirate

Question 2

Common cause in IDA in 1yo child

Answer 2

Late introduction of solid foods –> low Vit C

Question 3

What kinda milk, if consumed in high quantities, is associated with IDA

Answer 3

Cow’s milk ( v low absorption of iron)

Question 4

Management of IDA? How long is it required for?

Answer 4

Dietary changes (fortified cereals, vit C, less cow's milk etc)
ORAL IRON

• continue for 3 months post-correction

Question 5

Precipitants of jaundice in G6PD deficiency

Answer 5

Infection
Drugs - antimalarials, aspirin
Moth balls/fava beans

Question 6

Dx of G6PD

Answer 6

measure g6pd activity

Question 7

Regular management of G6PD

Answer 7

Folate supplements

+ avoid triggers

Question 8

How can Sickle cell disease –> heart failure

Answer 8

untreated anaemia –> cardiac enlargement and heart failrue

Question 9

SCD - high risk of infection. How can this be prevented?

Answer 9

Daily oral pencillin

Question 10

Supplement taken by SCD patiets

Answer 10

folic acid, daily

Question 11

beta thalassemia - affected ethnic groups?

Answer 11

Indian subcontinent + Cypriots

Question 12

Diff between beta thalassemia major + intermedia?

Answer 12

Major - no HbA can be produced

Question 13

+ve result for beta thalassemia on Hb electrophoresis?

Answer 13

HbA2 >3.5%

Question 14

Microcytic anemia, FTT + massive forehead

Answer 14

beta-thalassemia (extra medullary haemotpoesis)

Question 15

Management of beta-thai major

Answer 15

Life long RBC transfusions + Desferrioxamine

Question 16

how many alpha globin genes do normal people have?

Answer 16

4

Question 17

alpha globin genes : what is the effect if one/2/3/4 are dleted

Answer 17

1/2 gene deletions: asymptomatic w hypchormic, microcytic anemia

3 gene deletions: HbH disease. mild/mod anaemia
4 gene deltions: HbBarts hydrops fetalis

Question 18

Complications of long term transfusions eg in beta thal major

Answer 18

Liver probs
Hyperpigemntation
DM
Cardiomyopathy

Question 19

Bleeding disorder that is typically diagnosed in adolescence

Answer 19

vWF deficiency (menorrhagia)

Question 20

How to differentiate between Haemophilia and vWF defcieicny

Answer 20

Haemophilia - prolonged APTT
vWF - normal APTT (and low vWF antigen, duh)

Both have low Factor8:C activity

Question 21

Management of acute bleeding episode in haemophilia?

If 1st line is unavailable, what is given

Answer 21

Recombinant factor 8/9 concentrate

or FFP/cryoprecipitate