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Path Week 2 > Ha - Paeds Haem > Flashcards

Ha - Paeds Haem Flashcards

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1
Q

how to response to infection differ in adults and kids

A

kids = lymphocytosis
adults = neutrophilia

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2
Q

how does neonate blood count differ from children

A

neonate has higher haem F, higher Hb, lymphocyte count, neutrophil count

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3
Q

why doesn’t beta thalassaemia major affect babies

A

they have more ham F not haem B so doesnt develop until switched to haem B

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4
Q

3 causes of polycythaemia in foetus / neonate

A

twin to twin transfusion
IU hypoxia
placental insufficiency

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5
Q

what is bad about polycythaemia

A

get hyperviscosity

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6
Q

4 causes of foetal / neonatal anaemia

A

twin to twin transfusion
foetal to maternal transfusion
parvovirus infection
haemorrhage from cord / placenta

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7
Q

when does the first leukaemia genetic hit occur

A

in utero

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8
Q

who gets congenital leukaemia (aka transient abnormal myelopoiesis)

A

downs syndrome

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9
Q

how is congential leukaemia different from normal leukaemia

A

it spontaneously remits

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10
Q

what cell type is involved in congential leukaemia

A

myeloid - megakaryocytes

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11
Q

newborn babies vs adults have got what different in their blood film ?

A

a higher Hb

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12
Q

what is thalassaemia

A

reduced rate of synthesis of one type of haemaglobin

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13
Q

what is haemaglobinopathy

A

synthesis of a structurally abnormal molecule of haemaglobin
or
can include thalassaemias too

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14
Q

why do defects in alpha / beta haemaglobinopathies occur at different ages?

A

alpha synthesis starts in utero so will show up then
beta synthesis starts after birth so newborn babies wont have it

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15
Q

what chromosome has beta hb encoded

A

11

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16
Q

what chromosome has alpha hb encoded

A

16

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17
Q

how does sickle cell cause blockage of BVs

A

regular haemaglobin is donut shaped
if it has the sickle cell gene, it will turn into sickle shape when exposed to HYPOXIA
these aggregate and block BVs

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18
Q

is sickle cell trait part of sickle cell disease

A

no - it causes subclinical sx if any

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19
Q

if both parents have sickle trait, what % of children will have:
- sickle trait
- sickle cell
- normality

A

50% trait
25% sickle cell
25% normal

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20
Q

how is sickle cell diagnosed at birth

A

guthrie spot

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21
Q

why do kids get sickle crises in hand / feet but adults dont

A

kids have extension of red marrow further into limbs - these are very vascular areas so more likely to get sickle occlusion –> leads to hand-foot syndrome where thrombosis in periperhies, which you don’t get in adults

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22
Q

how can sickle cell manifest differently in kids and adults

A

hand-foot syndrome in kids - longer red marrow
splenic sequestration - spleen is still functioning, so can get sickle cells sequestering
stroke - thinner BVs in kids

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23
Q

why doesn’t splenic sequestration happen in older children / adults

A

splenic infarction occurs so much that the spleen has become small and fibrotic

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24
Q

what risk increases as splenic sequestration risk decreases

A

hyposplenism - bacterial infections etc

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25
what 2 infections are particularly bad in sickle babies
pneumococcal parvovirus
26
why does folic acid matter more in a child with sickle cell than in a normal child / adult
hyperplastic erythropoiesis and growth spurts requires folic acid
27
what is the lifespan of a sickle red cell
20 days
28
how do you manage sickle cell and complications in infant / child
accurate diagnosis educate parents - sickle cell crisis signs vaccinate - pneumococcus prescribe folic acid / penicillin
29
is stroke more common in sickle kids or adults
kids - smaller BVs in brain
30
siblings with sickle cell anaemia present together with severe anaemia, low reticulocyte count. Dx?
parvovirus b19 infection
31
6 year old afro caribean boy with chest / abdo pain. hb 63, mcv 85 and blood film shows sickle cells. is this trait/anaemia/sickle with beta thalassaemia ?
sickle cell anaemia
32
what blood film feature is seen in sickle cell with beta thalassaemia
microcytosis
33
what is beta thalassaemia
condition resulting from reduced synthesis of beta globin chain and therefore haemaglobin A
34
when does beta thalassaemia first present
3-6 months
35
what is prognosis of beta thalassaemia heterozygosity/trait
harmless but genetically important
36
what is prognosis of beta thalassaemia homozygosity
severe anaemia, that in the absence of transfusion, is fatal in first few years of life
37
what is the name for the intermediate forms of beta thalassaemia
beta thalassaemia intermedia
38
clinical effects of poorly treated thalassaemia
anaemia - HF, growth retardation erythropoietic drive - bone expansion, hepatomegaly, splenomegaly iron overload - HF, gonadal failure
39
Mx of infant / child with beta thalassaemia major
accurate diagnosis family counselling blood transfusion chelation therapy once iron overload occurs
40
name 2 chelation therapies
desferioxamine deferiprone
41
are all congenital haemolytic anaemias inherited? why?
no - can get transplacental passage of ABs causing haemolytic disease of newborn
42
what causes transplacental haemolytic anaemia
ABO or RH D ABs mismatch
43
what causes inhertied haemolytic anaemias in children
defects in: red cell membrane (spherocytosis/elliptocytosis) Hb molecule (sickle cell) red cell enzymes - glycolytic pathway (pyruvate kinase def) / pentose shunt (G6PD def)
44
signs of haemolytic anaemia in newborn
jaundice splenomegaly increased unconjugated BR
45
why is high BR an issue in babies
can lead to conikterose
46
name 5 inherited haemolytic anaemias in children
hereditary spherocytosis hereditary elliptocytosis sickle cell pyruvate kinase deficiency G6PD deficiency
47
what should be avoided in G6PD deficiency
moth balls - naphthalene fava beans (broad beans) some drugs infection
48
2 important acquired haemolytic anaemias in kids
autoimmune haemolytic anaemia haemolytic uraemic syndrome
49
characteristics of AI HA
spherocytosis positive coombs test
50
characteristics of hamolytic uraemic syndrome
haemolysis uraemia (kidney impairment) small angular fragments and microspherocytes --> schistocytes
51
what precipitates haemolytic uraemic syndrome in kids
a specific e coli
52
presentation of haemophilia in kids
boys bleeding following minor ops eg circumcision haemarthroses when starting to walk bruises
53
Mx of haemophilia in kids
accurate Dx counselling of family Tx of bleeding episodes use of prophylactic coagulation involve school / family
54
mucosal bleeding, bruises and post traumatic bleeding. Dx?
von willebrand disease
55
Tx of von willebrand factor
lower purity factor Viii concentrates
56
1 year old boy presents with joint bleeding hb, wcc, platelet normal aptt long pt, bleeding time normal Dx?
haemophilia A
57
which is more likely of the haemophilias
A
58
petechiae, bruises and blood blisters in mouth. dx?
ITP
59
Dx of ITP
history blood count and film aspirate if needed
60
Mx of ITP
observation steroids high dose IV IG IV anti Rh D (if positive)
61
what leukaemia is more common in kids
ALL
62
what leukaemia is more common in infants under 1
AML
63
Mx of hyposplenism
vaccinations prophylactic penicillin advice to parents re other risks - malaria, dog bites

Path Week 2 (18 decks)

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