Haem/onc Flashcards
Is demand for iron high or low in early years?
High
How can giving cow’s milk too early cause anaemia?
Chronic microscopic bleeding
Are infants born with iron stores?
Yes
What iron intake do babies require to maintain stores? (daily)
400-700ng
Why is iron in breast milk well absorbed?
Lactoferrin (iron binding protein)
Are artificial formula feeds a good source of iron?
Yes generally sufficiently fortified with iron to prevent deficiency
When might babies need prophylactic iron supplementation? Across what time period?
LBW and solely breast fed
Start 4-6w until mixed feeding established.
Presentation of iron deficiency anaemia
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)
When do you investigate iron deficiency anaemia?
Only if failure to respond to treatment
What might blood film/count show in iron defic anaemia?
microcytic, hypochromic RBCs and poikilocytosis (abnormal shape RBCs)
Serum ferritin low
What investigations can you do other than blood count and film
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
Management iron defic anaemia
R/o serious cause such as thalassaemia
ONLY IF DEMONSTRABLE IRON DEFICIENT STATE: oral iron salts
Dose oral iron salts?
3-6mg/kg (max 200g) daily in 2-3 divided doses
What do you need to consider when prescribing oral iron salts?
Each salt has different content of iron so specify the iron salt and elemental iron. Also consider the iron content of formula feeds.
How else can iron be given other than oral? When should it be given?
Parenterally
If oral unsuccessful for various reasons
Is parenteral better than properly taken oral iron?
Not really
Advice for parents re diet in iron deficiency?
1 pint milk per day max
Encourage more iron rich food
Tea can limit iron absorption so avoid
How quickly does iron supplementation treatment work?
Within 1w Hb levels should start to increase. If not then check compliance or consider alternative
Draw out flow chart of anaemias
see IDA notes
What is the commonest cancer cause of death in children and young people?
Brain tumour
60% brain tumour survivors are left with _____?
Life altering disability
What are the 2 most common locations for brain tumour
Brainstem and cerebellum
How do brain tumours present?
raised ICP or neuro signs
What are two long term sequelae of brain tumour?
Endocrine and growth problems
What factor is implicated in haemophilia A?
VIII
What factor is implicated in haemophilia B?
IX
What factor is implicated in haemophilia C? Who is more likely to get it?
XI
Ashkenazi jew (both sexes)
What factor is implicated in parahaemophilia?
V
How are haemophilia A and B inherited?
X linked
Could a female carrier of haemophilia A/B be at all affected?
Yes could have mild form e.g. heavy periods
Will a man with haemophilia A/B have affected children?
Can’t have a son with haemophilia but could have carrier daughters
Can you get acquired haemophilia?
Yes- give steroids
True or false haemophilia has a high rate of new mutations?
True
Which clotting test is abnormal in haemophilia a and b?
APTT increased
What is the characteristic bleed in haemophilia?
Joint
Haemarthrosis of elbows, knees, ankles. Can lead to permanent joint damage or dysfigurement
Why might haemophiliacs get nerve palsies and compartment syndromes?
Haematomas
When might haemophilia present?
Bruising as they learn to walk
Following dental procedure, accident or surgery
Will clotting factor replacement always remain effective in haemophilia?
No they can develop immune inhibitor
What do you need to red flag for in haemophilia?
IC haemorrhage- confusion, severe headache, stiff neck, vomiting, slurred speech, double vision, poor balance- call ambulance
can you diagnose haemophilia before birth?
Yes amnio/cvs
How do you manage haemophilia pharmacologically
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
Haemophilia prognosis
Normal QoL, 10y shortened life expectancy. 1/3 die from ICH
