Haem/onc

Question 1

Is demand for iron high or low in early years?

Answer 1

High

Question 2

How can giving cow’s milk too early cause anaemia?

Answer 2

Chronic microscopic bleeding

Question 3

Are infants born with iron stores?

Answer 3

Yes

Question 4

What iron intake do babies require to maintain stores? (daily)

Answer 4

400-700ng

Question 5

Why is iron in breast milk well absorbed?

Answer 5

Lactoferrin (iron binding protein)

Question 6

Are artificial formula feeds a good source of iron?

Answer 6

Yes generally sufficiently fortified with iron to prevent deficiency

Question 7

When might babies need prophylactic iron supplementation? Across what time period?

Answer 7

LBW and solely breast fed

Start 4-6w until mixed feeding established.

Question 8

Presentation of iron deficiency anaemia

Answer 8

Can be asymptomatic

Pallor

If severe- anorexia and irritability

Decreased neuro/intellectual functioning such as attention span and alertness (iron deficiency even in the absence of anaemia)

Question 9

When do you investigate iron deficiency anaemia?

Answer 9

Only if failure to respond to treatment

Question 10

What might blood film/count show in iron defic anaemia?

Answer 10

microcytic, hypochromic RBCs and poikilocytosis (abnormal shape RBCs)

Serum ferritin low

Question 11

What investigations can you do other than blood count and film

Answer 11

Hb electrophoresis to r/o thalassaemia etc

U&Es- renal failure can cause low RBC via reduced EPO

Blood/urine culture for chronic infection (normocytic anaemia with low reticulocytes)

Bone marrow aspirate (leukaemia)

?Lead level- lead toxicity can cause microcytic anaemia

Question 12

Management iron defic anaemia

Answer 12

R/o serious cause such as thalassaemia

ONLY IF DEMONSTRABLE IRON DEFICIENT STATE: oral iron salts

Question 13

Dose oral iron salts?

Answer 13

3-6mg/kg (max 200g) daily in 2-3 divided doses

Question 14

What do you need to consider when prescribing oral iron salts?

Answer 14

Each salt has different content of iron so specify the iron salt and elemental iron. Also consider the iron content of formula feeds.

Question 15

How else can iron be given other than oral? When should it be given?

Answer 15

Parenterally

If oral unsuccessful for various reasons

Question 16

Is parenteral better than properly taken oral iron?

Answer 16

Not really

Question 17

Advice for parents re diet in iron deficiency?

Answer 17

1 pint milk per day max

Encourage more iron rich food

Tea can limit iron absorption so avoid

Question 18

How quickly does iron supplementation treatment work?

Answer 18

Within 1w Hb levels should start to increase. If not then check compliance or consider alternative

Question 19

Draw out flow chart of anaemias

Answer 19

see IDA notes

Question 20

What is the commonest cancer cause of death in children and young people?

Answer 20

Brain tumour

Question 21

60% brain tumour survivors are left with _____?

Answer 21

Life altering disability

Question 22

What are the 2 most common locations for brain tumour

Answer 22

Brainstem and cerebellum

Question 23

How do brain tumours present?

Answer 23

raised ICP or neuro signs

Question 24

What are two long term sequelae of brain tumour?

Answer 24

Endocrine and growth problems

Question 25

What factor is implicated in haemophilia A?

Answer 25

VIII

Question 26

What factor is implicated in haemophilia B?

Answer 26

IX

Question 27

What factor is implicated in haemophilia C? Who is more likely to get it?

Answer 27

XI Ashkenazi jew (both sexes)

Question 28

What factor is implicated in parahaemophilia?

Answer 28

V

Question 29

How are haemophilia A and B inherited?

Answer 29

X linked

Question 30

Could a female carrier of haemophilia A/B be at all affected?

Answer 30

Yes could have mild form e.g. heavy periods

Question 31

Will a man with haemophilia A/B have affected children?

Answer 31

Can't have a son with haemophilia but could have carrier daughters

Question 32

Can you get acquired haemophilia?

Answer 32

Yes- give steroids

Question 33

True or false haemophilia has a high rate of new mutations?

Answer 33

True

Question 34

Which clotting test is abnormal in haemophilia a and b?

Answer 34

APTT increased

Question 35

What is the characteristic bleed in haemophilia?

Answer 35

Joint Haemarthrosis of elbows, knees, ankles. Can lead to permanent joint damage or dysfigurement

Question 36

Why might haemophiliacs get nerve palsies and compartment syndromes?

Answer 36

Haematomas

Question 37

When might haemophilia present?

Answer 37

Bruising as they learn to walk Following dental procedure, accident or surgery

Question 38

Will clotting factor replacement always remain effective in haemophilia?

Answer 38

No they can develop immune inhibitor

Question 39

What do you need to red flag for in haemophilia?

Answer 39

IC haemorrhage- confusion, severe headache, stiff neck, vomiting, slurred speech, double vision, poor balance- call ambulance

Question 40

can you diagnose haemophilia before birth?

Answer 40

Yes amnio/cvs

Question 41

How do you manage haemophilia pharmacologically

Answer 41

Desmopressin rapid IVI can increase factor VIII levels Clotting factor injections (in mild- only if injured and bleeding. In severe- regular) Injections should be given SC not IM Avoid contact sports Be careful taking aspirin and avoid anticoags Good oral hygiene and regular dentist checks

Question 42

Haemophilia prognosis

Answer 42

Normal QoL, 10y shortened life expectancy. 1/3 die from ICH