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Paediatrics > Paeds Haem > Flashcards

Paeds Haem Flashcards

1
Q

Anaemia is Hb below normal. Anaemia in neonate = Hb below what?

A

<140 g/L in neonate

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2
Q

anaemia in <1yr = Hb below what?

A

<100g/L

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3
Q

anaemia in 1-12 yr olds = Hb below what?

A

<110g/L

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4
Q

Give me three overarching reasons why anaemia might happen.

A
  1. not enough red cells being made
  2. too many red cells being destroyed
  3. blood loss
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5
Q

What happens to HbF during first yr of life?

A

HbF high at birth then gradually replaced by HbA during first yr of life

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6
Q

One reason for anaemia is not enough red cells being made. Give me two reasons for not enough red cells being made.

A

ineffective erythropoiesis

red cell aplasia (i.e. none produced!)

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7
Q

One reason for anaemia is too many red cells being destroyed, a.k.a HAEMOLYTIC anaemia. Give me 4 reasons for too many red cells cells being destroyed.

A

membranopathies
enzymopathies
haemoglobinopathies
immune

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8
Q

too many red cells being destroyed, a.k.a.

A

haemolytic

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9
Q

Membranopathies, enzymopathies, haemoglobinopathies and immune are all examples of what reason for anaemia?

A

too many red cells being destroyed a.k.a HAEMOLYTIC

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10
Q

Give me an example of a membranopathy causing haemolytic anaemia.

A

hereditary spherocytosis

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11
Q

Give me an example of an enzymopathy causing haemolytic anaemia.

A

G6PD deficiency

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12
Q

What is haematocrit?

A

proportion of the blood that’s cellular (usually 45% cells and the rest plasma)

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13
Q

Give me 2 examples of haemoglobinopathies causing haemolytic anaemia.

A

sickle cell + thalassaemia

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14
Q

Three causes of normocytic anaemia

A

anaemia of chronic disease
pregnancy
mixed haematinic (eg iron + folate)

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15
Q

2 causes of macrocytic anaemia

A

b12/folate deficiency

alcohol

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16
Q

In which type of anaemia would there be a high reticulocyte count?

A

haemolytic

lots of new red cells having to be produced bc they’re being destroyed all the time

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17
Q

In which type of anaemia would there be a high reticulocyte count?

A

haemolytic

lots of new red cells having to be produced bc they’re being destroyed all the time

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18
Q

Give me an example of immune cause of haemolytic anaemia.

A

haemolytic disease of the newborn

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19
Q

Tell me something about ‘aplastic anaemia’.

A

it’s bone marrow failure and part of PANCYTOPENIA (low rbcs, wbcs, and plts)

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20
Q

One reason for anaemia is blood loss. Give me three examples of kids blood loss causing anaemia.

A

fetomaternal bleeding
GI e.g. meckels diverticulum
bleeding disorder e.g. von willebrands

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21
Q

One reason for anaemia is not enough red cells being made. This can happen due to ineffective erythropoiesis or red cell aplasia. Give me three examples of things that would cause ineffective erythropoiesis.

A

iron def
folate def
chronic infl

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22
Q

One reason for anaemia is not enough red cells being made. This can happen due to ineffective erythropoiesis or red cells aplasia. Give me two examples of things that would cause red cell aplasia (a.k.a rbcs not prod).

A

Diamond Blackfan

Parvovirus B19

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23
Q

Diamond Blackfan
Parvovirus B19

both causes of…

A

red cells apalsia –> not enough red cells being made

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24
Q

iron def
folate def
chronic infl

all causes of…

A

ineffective erthyropoiesis –> not enough red cells being made

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25
Q

Give me some symptoms of iron def anaemia in kids.

A

fatigue
pallor
Pica!
SOB if severe

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26
Q

What might cause iron def anaemia in kids? (x4)

A

malnutrition e.g. EBF beyond 6 months
malabsorption e.g. hookworm
rapid growth / puberty
blood loss e.g. meckels

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27
Q

exclusive breastfeeding beyond 6 months is a risk for..

A

iron def anaemia

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28
Q

What kind of anaemia do coeliac and hookworm cause?

A

iron def (malabsorption)

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29
Q

A child presents with slow feeding and tires easily. Mum has noticed he has started putting soil in his mouth. What might you see on examination of cojunctivae / tongue / palmar creases?

A

PALLOR

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30
Q

What would FBC show in iron def anaemia, in terms of:

a) Hb
b) MCV
c) MCHC

A 
iron def anaemia:
low Hb
low MCV (microcytic)
low MCHC (hypochromic)
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31
Q

what is MCHC on FBC?

A

mean corpuscular haemoglobin concentration

e.g. low MCHC =hypochromic

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32
Q

What is MCV on FBC?

A

mean corpuscular volume

e.g. low MCV = microcytic

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33
Q

13 yr old girl who has just gone into puberty and had a growth spurt presents with tiredness, pallor and SOB. You suspect iron def anaemia. What investigations do you do?

A

FBC
blood film
serum ferritin

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34
Q

A coryzal 6yr old with coeliac presents with tiredness and pallor. You check serum ferritin and it’s raised. You are confused - you expected it to be low since you’re pretty sure he has iron def anaemia due to malabsorption. Why could this result be?

A

serum ferritin is an acute phase reactant so can get raised in illness (clue = coryza)

therefore check CRP too

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35
Q

A child’s FBC and blood film shows microcytic hypochromic anaemia - you suspect iron def. What would you expect from her serum ferritin and total iron binding capacity?

A

low serum ferritin
increased total iron binding capacity

(lots of spare carriers not being filled)

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36
Q

What drug is used to treat iron def anaemia? (inc dose + freq)

A
  • ORAL FERROUS SULPHATE / fumurate 5mg/kg daily
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37
Q

What is the treatment for iron def anaemia? I want all the details.

A

oral ferrous sulphate / fumurate 5mg/kg daily

until Hb normal then continue for 3 months after

+ dietary advice!

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38
Q

How long do you continue the ferrous sulphate after the Hb has normalised?

A

3 months

don’t forget dietary advice

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39
Q

Give me three drugs that can cause folate def.

A

trimethoprim
nitrofurantoin
valproate

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40
Q

What is the treatment for folic acid def?

A

5mcg/kg folic acid

+ dietary advice

**NEVER TREAT W FOLIC ACID ALONE UNLESS B12 KNOWN NORMAL
(risk of cord degen)

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41
Q

What is important to remember about treating folate def anaemia?

A

never treat w folic acid alone unless b12 known normal

risk of cord degen

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42
Q

Give me three causes of b12 def.

A

malnutrition (vegans)
malabsorption
PERNIIIIICIOUS ANAEMIA (autoimmune, no intrinsic factor - can’t absorb)

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43
Q

What is the treatment for b12 def?

A

IM hydroxocobalamin injections

like pete has

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44
Q

what is that word for b12 injections

A

hydroxocobalamin

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45
Q

You do FBC and blood film for folate def anaemia. What do you expect to see?

A

macrocytic anaemia

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46
Q

What investigations for susepcted b12/folate anaemia?

A

FBC
blood film
serum folate / B12

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47
Q

Child presents with pallor, fatigue, GLOSSITIS and HYPOTONIA. Potentially some developmental delay. FBC and blood film show a macrocytic anaemia. What’s your diagnosis and what other test would show it?

A

b12/folate deficiency

serum b12/folate

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48
Q

Symptoms of b12 and folate def?

A

pallor, fatigue, GLOSSITIS, HYPOTONIA, dev delay

might end up with ataxia and bad vibration sense!!

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49
Q

what would expect from reticulocyte count in red cell aplasia (diamond blackfan, parvovirus b19)

A

low reticulocytes

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50
Q

If hereditary spherocytosis is very troublesome, what might you need to do?

A

splenectomy

(they’ll need lots of vaccines)

before that - try oral folic acid, blood trasnfusions and cholecystectomy if gallstones

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51
Q

Why does herediatry spherocytosis lead to too many red cells being destroyed?

A

they’re a funny shape so less deformable so get destroyed in spleen

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52
Q

Jaundice, gallstones, anaemia and splenomegaly. Blood film show lots of reticulocytes and spherocytes. what’s this?

A

hereditary spherocytosis.

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53
Q

Where is G6PD deficiency most prevalent?

A

Africa, middle + far East

54
Q

what inheritance is G6PD def?

A

X linked recessive

55
Q

Neonatal jaundice, and intermittent acute haemolytic reactions precipitated by febrile infection, fava beans, and mothballs.

A

G6PD deficiency

56
Q

What would you see on blood film in G6PD def?

A

high reticulocytes

Heinz bodies! (think beans)

57
Q

What is the most severe form of sickle cell?

A

HbSS

(least severe is ‘sickle cell trait - asymp, HbS from one parent).

58
Q

Point substitution on the B globin gene (glutamine to valine). What’s this?

A

sickle cell

59
Q

When does sickle cell usually present?

A

3 months - 6yrs

60
Q

Tell me about the pathophysiology of sickle cell.

A

HbS polymerises within rbcs making them sickle shaped and increasing blood viscosity

have shorter lifespan + get destroyed early

vaso occlusion of small vessels - ischaemia + infarction.

61
Q

Why does sickle cells usually present after 3 months?

A

run out of HbF

62
Q

I want 7 consequences of sickle cell.

A 
anaemia
infection
priapism
vaso-occlusive crisis
aplastic crisis
sequestration crisis
long term probs e.g. stroke
63
Q

Give me an example of sickle cell infection

A

salmonella osteomyelitis

64
Q

Cold, dehydration, infection and hypoxia are all precipitants of what in sickle cell?

A

vaso-occlusive crisis

65
Q

What can happen to long bones as a result of sickle cell vaso occlusive crisis?

A

avascular necrosis

66
Q

What can happen to chest as a result of sickle cell vaso-occlusive crisis?

A

“acute chest syndrome” - severe hypoxia

67
Q

What triggers aplastic crisis in sickle cell?

A

parvovirus B19

68
Q

Boy with sickle cell has sudden abdo pain + circulatory collapse. What is happening?

A

sequestration crisis

rbcs trapped in spleen / liver

69
Q

What happens in a sequestration crisis?

A

rbcs trapped in spleen / liver

– sudden abdo pain + circulatory collapse

70
Q

Give me some examples of long term problems in sickle cell.

A

stroke
heart failure
delayed puberty

71
Q

All newborns are screened for sickle cell. When are all Afro-Caribbean kids also screened?

A

before general anaesthetic.

72
Q

Why does sickle cell cause anaemia?

A

haemoglobinopathy – too many red cells being destroyed (haemolytic)

73
Q

What is the general treatment for ALL acute crises in sickle cell?

A

analgesia
fluids
oxygen
Abx if fever

74
Q

All sickle cell crises need analgesia, fluids, oxygen and Abx if fever. In aplastic crisis or sequestration crisis, they need:

A

blood transfusion

75
Q

All sickle cell crises need analgesia, fluids, oxygen, and Abx if fever. In acute chest syndrome or priapism, they need:

A

exchange transfusion!

76
Q

What does maintenance treatment involve for children with sickle cell?

A

PROPHYLAXIS

daily oral folic acid (to help make more rbcs)

bad - HYDROXYCARBAMIDE
:/ bone marrow transplant

77
Q

What 3 things does PROPHYLAXIS for sickle cell kids involve?

A

lifetime oral Pen V
vaccinations
avoid triggers

78
Q

If they survive it, this treatment is curative for sickle cell:

A

bone marrow transplant

79
Q

Where is thalassaemia most prevalent?

A

Indian subcontinent + Middle East

80
Q

In what population is sickle cell most prevalent?

A

Afro-caribbean

81
Q

Which form of thalassaemia causes hydrops + death in utero?

A

alpha thalassaemia major.

east asians

82
Q

Which two forms of thalassaemia are asymptomatic?

A

beta and alpha thalassaemia TRAITS

83
Q

Which form of sickle cell is asymptomatic?

A

sickle cell trait

84
Q

Which form of thalassaemia causes severe anaemia, faltering growth and hepatosplenomegaly in childhood?

A

Beta thalassaemia major

85
Q

What are the symptoms of beta thalasseamia major?

A

severe anaemia
faltering growth
hepatosplenomegaly

86
Q

Why does thalassaemia cause anaemia?

A

haemoglobinopathy – too many red cells being destroyed (haemolytic)

87
Q

Beta thalassaemia major is fatal without treatment. What’s treatment?

A

lifelong monthly BLOOD TRANSFUSIONS

with iron chelation to avoid haemochromatosis

88
Q

What treatment is curative for beta thalassaemia major?

A

bone marrow transplant

89
Q

The treatment of beta thalassaemia major is lifelong monthly blood transfusions. What do these need to be combined with and why?

A

iron chelation

avoid haemochromatosis

90
Q

Beta thalassaemia major is caused by mutation of what gene?

A

mutation of Beta globin chain - cant prod HbA (which needs two alpha and two beta chains)

91
Q

Which element is important in converting clotting factors?

A

calcium

92
Q

prothrombin get converted to thrombin. then what happens.

A

thrombin converts fibrinogen to fibrin

93
Q

what does plasmin do?

A

breaks down clots (fibrin) (fibrinolysis)

94
Q

what is APTT?

A

activated partial thromboplastin time

- measures activity of lots of clotting factors.

95
Q

Give me a list of investigations which might be useful in bleeding disorders?

A

FBC, PT, APTT, D-dimer, LFTs, quantitative fibrinogen assay, vwF antigen

96
Q

What is the inheritance for haemophilia?

A

X linked recessive

97
Q

Which type of haemophilia is more common?

A

haemophilia A

98
Q

What clotting factor is affected in haemophilia A?

A

factor 8 = haemophilia A (commonest)

99
Q

What clotting factor is affected in haemophilia B?

A

factor 9 = haemophilia B

100
Q

What/when is common presentation of haemophilia?

A

spontaneous bleeding into muscles and joint around 1yr of age.

neonates - intracranial haemorrhage

101
Q

Haemophilia usually presents at what age?

A

1 yr olds

102
Q

What is the treatment for haemophilia A?

A

recombinant factor 8 concentrate

by IV infusion when bleeding
prophylactic when severe

103
Q

What is the treatment for haemophilia B?

A

recombinant factor 9 concentrate

by IV infusion when bleeding
prophylactic when severe

104
Q

Two complications of haemophilia treatment.

A

antibodies to factor 8/9

vascular access :/

105
Q

von Willebrand disease usually presents at what age?

A

adolescents

106
Q

What two important things does von willebrand’s factor do?

A
  • facilitates platelet adhesion to damaged to endothelium

- carrier for factor 8

107
Q

what is the inheritance of von willebrands disease?

A

autosomal dominant

108
Q

Adolescent presents with menorrhagia, epistaxis and mucosal bleeding. No purpura. What suspect?

A

von willbrands disease

109
Q

What is key investigation in suspected von willebrands disease?

A

VWF ANTIGEN (low)

110
Q

what is the treatment for von willebrands disease?

A

mild - DDAVP (desmopressin)

severe - plasma derived factor 8 concentrate

111
Q

Name 4 ACQUIRED types of bleeding disorder.

A

vit K def
liver disease
Immune thrombocytopenia
DIC

112
Q

Which acquired bleeding disorder causes haemorrahgic disease of the newborn?

A

vit K def

113
Q

vitamin K is needed for which clotting factors? :)

A

10 9 7 2

prolonged prothrombin time

114
Q

what 3 things can cause vitamin K deficiency?

A

malnutrition
malabsorption
vit K antagonists.g. warfarin

115
Q

platelets are destroyed by IgG autoantibodies. what disease is this?

A

immune thrombocytopenia

116
Q

when does immune thrombocytopenia tend to present?

A

age 2-10

often after VIRAL INFECTION

117
Q

7 yr old presents with petechiae, purpura and superficial bruising after viral infection.

A

immune thrombocytopenia

118
Q

define thrombocytopenia.

A

thrombocytopenia = platelets <150 x10 9/L

119
Q

What is treatment for immune thrombocytopenia?

A

usually self limiting! 6-8wks

if major bleed - oral pred

120
Q

If child has major bleed in immune thrombocytopenia, how do you treat?

A

oral prednisolone

platelet transfusion if life threatening

121
Q

What are the symptoms of immune thrombocytopenia?

A

petechiae, purpura, superficial bruising

122
Q

80% of immune thrombcytopenia is self-limiting. What % are chronic, a.k.a persistent?

A

20%

might have to give rituximab

123
Q

differentials for immune thrombocytopenia ? (x3)

A

SLE
ALL
aplastic anaemia

124
Q

diffuse fibrin deposition in microvasculature and consumption of clotting factors and platelets, with severe sepsis / circulatory shock. what’s this?

A

disseminated intravascular coagulation

125
Q

what is treatment for DIC?

A

treat underlying cause

fresh frozen plasma, cryoprecipitate, platelet transfusion

126
Q

ALL, SLE and aplastic anaemia are all differentials for…

A

immune thrombocytopenia

127
Q

List me 2 inherited and 4 acquired bleeding disorders.

A

inherited - haemophilia, von willebrands disease

acquired - vit K def, liver disease, immune thrombocytopenia, DIC

128
Q

Name 3 congenital thrombophilias.

A
  • protein C def
  • protein S def
  • factor V leiden
129
Q

What is DIC?

A

diffuse fibrin deposition in microvasculature and consumption of clotting factors and platelets, with severe sepsis / circulatory shock.

130
Q

Some inherited thrombophilias are protein C def, protein S def, factor V leiden. Name some acquired thrombophilias.

A

DIC, malignancy, SLE

polycythaemia, Kawasaki

131
Q

?Acute treatment of thrombosis

A

LMWH

132
Q

?Chronic treatment of thrombosis / thrombophilia?

A

warfarin

Paediatrics (14 decks)

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